Cases in Pediatric Acute Care

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Cases in Pediatric Acute Care

Strengthening Clinical Decision Making

Edited by

Andrea M. Kline-Tilford, PhD, CPNP-AC/PC, FCCM, FAAN

C.S. Mott Children’s Hospital
Michigan Medicine
Ann Arbor, MI, USA

Catherine M. Haut, DNP, CPNP-AC/PC, FAANP, CCRN

Nemours/Alfred I. duPont Hospital for Children
Wilmington, DE, USA;
Mednax Pediatrix Medical Group
Baltimore, MD, USA
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This edition first published 2020
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Library of Congress Cataloging-in-Publication Data

Library of Congress Cataloging-in-Publication Data

Names: Kline-Tilford, Andrea M., editor. | Haut, Catherine, editor.
Title: Cases in pediatric acute care : strengthening clinical decision
making / edited by Andrea M. Kline-Tilford, Catherine Haut.
Description: Hoboken, NJ : Wiley-Blackwell 2020. | Includes bibliographical
references.
Identifiers: LCCN 2019056731 (print) | LCCN 2019056732 (ebook) |
ISBN 9781119568223 (paperback) | ISBN 9781119568209 (adobe pdf) |
ISBN 9781119568216 (epub)
Subjects: MESH: Critical Care–methods | Child | Critical
Illness–diagnosis | Critical Illness–therapy | Diagnosis, Differential |
Infant | Adolescent | Case Reports
Classification: LCC RJ370 (print) | LCC RJ370 (ebook) | NLM WS 366 | DDC
618.92/0028–dc23
LC record available at https://lccn.loc.gov/2019056731
LC ebook record available at https://lccn.loc.gov/2019056732

Cover Design: Wiley

Cover Images: Female doctor examining kid little child boy © Oksana Kuzmina/Shutterstock, Smiling doctor holding baby © JGI/Jamie Grill/
Getty Images, Pretty woman holding a newborn baby in her arms © Trendsetter Images/Shutterstock, Baby in mother’s arms in white blanket
© Herlanzer/Shutterstock, Paediatrician checking baby boys heart using stethoscope © Emely/Getty Images

Set in 9.5/12.5pt STIXTwoText by SPi Global, Pondicherry, India

Printed and bound by CPI Group (UK) Ltd, Croydon, CR0 4YY

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v

Contents

Preface xi
List of Contributors xiii

1 Complication of Pneumonia 1
Ji-Yeon Kim
2 Infant with Diaphoresis during Feedings 5
Kristen Brown and Kristen Nelson McMillan
3 Hypotension in the Operating Room 7
Ann Marie Felauer
4 Toddler with Vomiting and Diarrhea 11
Kimberly L. DiMaria
5 Infant with Fever and Cold Symptoms 15
Cheryl Bartke
6 Toddler with Pineoblastoma and Bone Marrow Transplant 19
Cathryn Freeman
7 A 5-Year-Old with Altered Mental Status 23
Yu-shan Tseng and Richard Garcia
8 An 8-Year-Old with Abdominal Trauma 29
Julie Resler
9 Child with Left Eye Swelling 33
Amy Graf
10 Billing and Coding 37
Dawn M. Silverman
11 Language Delay 47
Jolene Dickerson
12 A 6-Month-Old Infant with RSV and Respiratory Failure 51
Catherine M. Haut
13 Adolescent Abdominal Pain after Cardiac Surgery 55
Cathy Woodward
14 Young Infant with Vomiting 57
Sarah Martin
15 School-age Child with Bruising 61
Lisa Hovingh and Candie Ritsema
16 Infant with Shunted Hydrocephalus 65
Mark Weber
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vi Contents

17 Newborn with Poor Oral Intake and Irritability 69

Elaine Geary
18 An 8-Week-Old Infant with a Cough 73
Sarah Jane Garraty
19 Desaturation in Postoperative Cardiac Surgery Infant 77
Lisa Kohr
20 Pre-school Child with Seizures 81
Heather Herrera and Rachel Luthen
21 Child with Blood in Stool 85
Brittany Cook and Darby Tozer
22 Osteomyelitis Case Study 89
Kim Steanson
23 A 2-Year-Old with Liver Transplant and Fever 93
Louise Flynn
24 Respiratory Decompensation in Trached Infant 97
Jennifer Helman
25 Neonatal Hypoglycemia 101
Janice L. Wilson
26 Chronic Otitis Media 105
Kimberly Young-Conner
27 Complex Patient with Recurrent Lower Respiratory Tract Infection 109
Ann Marie Ramsey
28 Child with Racing Heart 115
Bradley Goettl
29 Teen with Headache and Seizure 119
Valarie Eichler
30 A 4-Year-Old with Bloody Diarrhea 125
Tamara Hill
31 Child with Persistent Fever 129
Misty Ellis
32 Quality Improvement Case Study 133
Peggy Dorr
33 Newborn Immediately Post Delivery 139
Heather Schniepp
34 A 5-Year-Old with Poor Weight Gain and Dehydration 143
Kimberly Young-Conner
35 Toddler with Complex Chronic Condition 147
Jennifer Wright
36 Pre-adolescent with Knee Pain 151
Katelyn Hamann and Shannon Konieczki
37 Infant with Increased Fussiness and Decreased Oral Intake 155
Stephanie Haughey
38 Infant with Prolonged Jaundice 161
Aimee Bucci
39 Child with Rash and Drooling 165
Mark Weber
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Contents vii

40 Child with Fever and Sore Throat 169

Stephanie Robell and Danielle Van Damme
41 Teenager with Sickle Cell Disease and Fever 173
Shannon Konieczki and Katelyn Hamann
42 Ethical Case Study 177
Jamie Tumulty
43 Infant with Neurologic Changes and Respiratory Failure 183
Blythe Pollack and Stacey Sears
44 Acute Hyperglycemia 191
Jeanne Little
45 Infant with Progressive Lethargy and Poor Feeding 195
Kathleen P. Thompson
46 Neonatal Tachypnea 199
Janet Dutcher
47 Diplopia and Esotropia 203
Lindsay Chapman
48 Acetaminophen Ingestion 207
Aaron Carpenter
49 A 3-Year-Old with Sudden Onset of Breathing Difficulty 211
Judy Verger
50 A 2-Month-Old with Acute Abdomen 215
Christyne Kyper
51 Henoch–Schönlein Purpura Nephritis 219
Tamara Hill
52 Toddler with Tachypnea and Cool Extremities 223
Cathy Woodward
53 Teen with Intractable Headache 227
Tara Pezzuto
54 Seizures in School-age Child 231
Ann Marie Felauer
55 An 18-Year-Old with Upper Respiratory Symptoms, Headache and Fever 235
Misty Evans
56 Testicular Torsion 239
Amanda Mandel
57 Teen with Fever and Rash 243
Christine Renke and Elizabeth Hoppe
58 Preterm Infant Unable to Wean from Mechanical Ventilation 247
Michelle Dokas
59 Toddler with Acute Respiratory Failure 251
Ahmed Aly and Bradley Tilford
60 Adolescent with Head Injury 255
Jennette Firlein
61 Newborn with Ambiguous Genitalia 261
Barbara Wise
62 Anemia in a Toddler 265
Dyane Bunnell
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viii Contents

63 A 2-Year-Old with Fever and Cough 269

Jessica Strohm Farber
64 Research Review 273
Lauren R. Sorce
65 Toddler with Fever and Redness Behind Ear 277
Amanda Lewis Weir
66 A 5-Year-Old with Right-Sided Weakness 281
Suzette Meinke and Jessica Spruit
67 Postoperative Heart Block 285
Cathy Woodward
68 Recognizing Human Trafficking 287
Christine DiPaolo
69 A Toddler With Stridor 291
Mari Welch
70 Delirium in a 9-Year-Old Boy 295
Heather Herrera
71 Feeding Challenge in a Child with Spinal Muscular Atrophy 299
Nicole Fragale
72 A 5-Year-Old with Osteomyelitis, Electrolyte Disturbance and Headaches 303
Kimberly L. DiMaria
73 Child with Scald Burn Injury 307
Amarilis Martin and Bradley Tilford
74 Parvovirus Requiring Blood Transfusion 313
Ashley Thibodeau
75 A Teen With High Fever, Rash and Lethargy 317
Jessica Strohm Farber
76 A 9-Year-Old With Scoliosis 321
Alicia McCarthy
77 An Unresponsive Infant 325
Charlene Pringle
78 A Toddler With Abdominal Pain, Diarrhea and Lethargy 331
Jessica Pech
79 Sore Throat and Shortness of Breath 335
Amy Manion
80 Oral Presentation and Communication 339
Jill Siegrist Thomas
81 A 7-Year-Old with Migratory Joint Pain 345
Christin Diller
82 Adolescent with Murmur and Chest Flutter 349
Natalie Hecht
83 Outpatient Management of Asthma 353
Jolene Dickerson
84 A Newborn with Abnormal Movements 357
Maura Bernardo
85 Postoperative Infant with Tachycardia 361
Cathy Woodward
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Contents ix

86 Brain Death 363

Maria C. Woosley
87 Postoperative Spinal Fusion with Complications 367
Jaime Panton and Melissa C. Varrone
88 Orthopnea in a 10-Year-Old 371
Max Pizzo
89 Complication of Traumatic Brain Injury 375
Christine Schindler
90 Newborn with Hypothermia 379
Marshay James
91 Back Pain in an Active Teenager 383
Alicia McCarthy
92 Teen with Fever and Abdominal Pain 387
Sarah Martin
93 Complication in a 6-Year-Old with a Kidney Transplant 391
Alison Hewitt Torres
94 Acute Hypertension and Seizures in a 17-Year-Old Male 395
Alison Hewitt Torres
95 Jittery 2-Week-Old Infant 399
Christopher D. Newman
96 Child with Leg and Back Pain 403
Jill Marks
97 Fussy, Tired Infant 407
Charlene Pringle
98 Tics and Something More 411
Judy Adelizzi-DeLany
99 Lightheaded Adolescent 415
Cathy Woodward
100 Infant with Hyponatremia 419
Sarah Pihl
101 Bone Marrow Transplant in Sickle Cell Disease 423
J. Paige Rieckhoff
102 Child with Respiratory Failure and Transaminitis 427
Kim Steanson
103 A Serious Football Injury 431
Julie Resler
104 Infant with Generalized Weakness 435
Blythe Pollack
105 A 3-Day-Old with Irritability 441
Christopher D. Newman
106 Child with Fever and Rash 445
Misty Evans
107 A Teen With Vomiting and Diarrhea 449
Christyne Kyper
108 Acute Jaundice in a 7-Year-Old Child 453
Roseann Hausmann
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x Contents

109 Idiopathic Nephrotic Syndrome 457

Tamara Hill
110 School-age Child with Hives and Lip Swelling 461
Jay M. Hunter
111 A 10-Year-Old with Asthma in the PICU 463
Catherine M. Haut
112 Skin Wound in Postoperative Neonate 467
Hayley Redmond
113 Provider Burnout and Resiliency 471
Jill Siegrist Thomas
114 Kidney Transplant Complication 475
Cathy C. McAdams
115 Infant with Influenza A and New Fever 479
Lauren Flagg
116 A 3-Year-Old with Fever and Painful Urination 483
Roseann Hausmann

Index 487

Preface
The healthcare landscape is rapidly changing and will
continue to evolve, providing challenges for patients and
families, but also for those providing care. Preventative
care for children forms the framework for minimizing
hospitalization, injury and illness, but when children do
become ill, providing care with the goal of a quick return
to function and home must be collaborative and timely.
Children are basically healthy and the incidence of illness
and injury is small as compared to adults, but the chal-
lenges of diagnosis and management can be dramatic.
Children bring unique and unusual genetic problems,
chronic healthcare issues, trauma, and acute and critical
presentations to inpatient and outpatient areas. It is espe-
cially important for pediatric providers to recognize diag-
noses and potential clinical outcomes in the first few
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xi
minutes of their encounters with these children and their
families. It is also imperative for pediatric providers to
engage the most recent evidence-based practice para-
meters to substantiate care while incorporating the unique
needs of the child and utilizing critical thinking for
decision-making. In this book, pediatric cases are presented
with an opportunity for the reader to answer questions about
assessment, diagnosis and management, interpret diagnostic
studies as well as to incorporate practical considerations
such as billing for services and participating in research at
the point of care. System-based, contemporary, common
as well as unusual clinical cases are included that will
challenge the reader and add knowledge. We thank the
contributors for sharing their patient stories and expertise
in creating this book.
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xiii

List of Contributors

Judith Adelizzi-DeLany, DNP, PPCNP-BC Kristen M. Brown, DNP, CPNP-AC, CHSE-A

Nurse Practitioner Assistant Professor
Nemours/Alfred I. duPont Hospital for Children Advanced Practice Simulation Coordinator
Wilmington, DE John Hopkins University School of Nursing
USA Baltimore, MD
Chapter 98 Tics and Something More USA
Chapter 2 Infant with Diaphoresis during Feedings

Sheree H. Allen, DNP, APRN, CPNP-PC/AC Aimee Bucci, DNP, CPNP-AC

Director, Pediatric Acute Care Nurse Practitioner Specialty Lead Advanced Practice Provider
Vanderbilt University School of Nursing Phoenix Children’s Hospital
Nashville, TN Phoenix, AZ;
USA Faculty Associate
Section Editor: Infectious Disease Arizona State University
Edson College of Nursing and Health Innovation
Phoenix, AZ
Ahmed Aly, MD USA
Pediatric Critical Care Fellow Chapter 38 Infant with Prolonged Jaundice
Children’s Hospital of Michigan Dyane Bunnell, MSN, APRN-BC, AOCNS, CPON
Detroit, MI Clinical Nurse Specialist
USA Nemours/Alfred I. duPont Hospital for Children
Chapter 59 Toddler with Acute Respiratory Failure Wilmington, DE
USA
Chapter 62 Anemia in a Toddler
Cheryl Bartke, MSN, PPCNP, CPNP-AC
Nurse Practitioner Aaron Carpenter, DNP, M.Div., CPNP-PC
Pediatric Critical Care Senior Director Ambulatory and Advanced
Seattle Children’s Hospital Practice Nursing
Seattle, WA Nemours/Alfred I. duPont Hospital for Children
USA Wilmington, DE
Chapter 5 Infant with Fever and Cold Symptoms USA
Chapter 48 Acetaminophen Ingestion

Maura Bernardo, MSN, CPNP Lindsay Chapman, MSN, CPNP-PC

Nurse Practitioner Nurse Practitioner
Nemours/Alfred I. duPont Hospital for Children St. Jude Children’s Research Hospital
Wilmington, DE Memphis, TN
USA USA
Chapter 84 A Newborn with Abnormal Movements Chapter 47 Diplopia and Esotropia
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xiv List of Contributors

Brittany Cook, MSN, FNP-BC Michelle Dokas, MSN, CPNP-AC

Nurse Practitioner Nurse Practitioner
Pediatric Emergency Medicine Pediatric Cardiovascular Surgery
Beaumont Hospital Children’s Hospital of Michigan
Troy, MI Detroit, MI
USA USA
Chapter 21 Child with Blood in Stool Chapter 58 Preterm Infant Unable to Wean from Mechanical
Ventilation
Jolene Dickerson, MSN, CPNP-PC
Nurse Practitioner
Nemours/Alfred I. duPont Hospital for Children Peggy Dorr, DNP, CPNP-PCNP
Wilmington, DE Clinical Program Manager, Women & Children’s Services
USA Nurse Practitioner, Pediatric Sedation Service
Chapter 11 Language Delay; Chapter 83 Outpatient University of Maryland Medical Center
Management of Asthma Baltimore, MD
USA
Christin Diller, DNP, CPNP-AC Chapter 32 Quality Improvement Case Study
Nurse Practitioner
Cincinnati Children’s Hospital Medical Center Janet Dutcher, DNP, NNP, APRN-BC
Cincinnati, OH; Neonatal Nurse Practitioner
Nursing Instructor Nemours/Alfred I. duPont Hospital for Children
Vanderbilt University College of Nursing Wilmington, DE
Nashville, TN USA
USA Chapter 46 Neonatal Tachypnea
Chapter 81 A 7-Year-Old with Migratory Joint Pain

Colleen Ditro, DNP, CPNP Valarie Eichler, MSN, CPNP-AC

Orthopedic APRN Supervisor, Skeletal Dysplasia Nurse Practitioner
Program Coordinator Dallas Children’s Hospital
Nemours/Alfred I. duPont Hospital for Children Dallas, TX
Wilmington, DE USA
USA Chapter 29 Teen with Headache and Seizure
Section Editor: Trauma, Musculoskeletal, Toxicology
Misty Ellis, DNP, CPNP-AC/PC
Kimberly L. DiMaria, MSN, CPNP-AC, CCRN
Lead Cardiac Nurse Practitioner
Heart Institute Clinical Practice Specialist
Norton Children’s Hospital
Children’s Hospital Colorado
Louisville, KY
Aurora, CO
USA
USA
Chapter 31 Child with Persistent Fever
Chapter 4 Toddler with Vomiting and Diarrhea;
Chapter 72 A 5-Year-Old with Osteomyelitis,
Electrolyte Disturbance and Headaches Misty Evans, DNP, CPNP-AC
Nurse Practitioner
Christine DiPaolo, NP Vanderbilt College of Nursing
Nurse Practitioner Nashville, TN
Nemours/Alfred I. duPont Hospital for Children USA
Wilmington, DE Chapter 55 An 18-Year-Old with Upper Respiratory
USA Symptoms, Headache and Fever; Chapter 106 Child with
Chapter 68 Recognizing Human Trafficking Fever and Rash
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List of Contributors xv

Jessica Strohm Farber, DNP, CPNP-AC Cathryn Freeman, MSN, CPNP-PC

Nurse Practitioner Nurse Practitioner
Children’s Hospital of Philadelphia Pediatric Hematology/Oncology
Philadelphia, PA Children’s Hospital of Michigan
USA Detroit, MI
Chapter 63 A 2-Year-Old with Fever and Cough; USA
Chapter 75 A Teen With High Fever, Rash and Lethargy Chapter 6 Toddler with Pineoblastoma and Bone
Marrow Transplant
Ann Marie Felauer, DNP, CPNP-AC/PC
Assistant Professor and Director
Pediatric Acute and Primary Care Nurse Practitioner Richard U. Garcia, MD
Specialties Assistant Professor Department of Pediatrics
UMB CURE Anatomy and Chemistry Curriculum Tracks, Division of Cardiology and Critical Care
Program Manager Wayne State University
University of Maryland School of Nursing Detroit, MI
Baltimore, MD USA
USA Chapter 7 A 5-Year-Old with Altered Mental Status
Chapter 3 Hypotension in the Operating Room;
Chapter 54 Seizures in School-age Child
Section Editor: Endocrinology Sarah Jane Garraty, MSN, CPNP
Nurse Practitioner
Jennette Firlein, MSN, CPNP-PC Beacon Pediatrics
Nurse Practitioner Rehoboth Beach, DE
Nemours/Alfred I. duPont Hospital for Children USA
Wilmington, DE Chapter 18 An 8-Week-Old Infant with a Cough
USA
Chapter 60 Adolescent with Head Injury
Elaine Geary, MSN, CRNP
Lauren Flagg, MSN, CPNP-AC Nurse Practitioner
Nurse Practitioner Nemours/Alfred I. duPont Hospital for Children
Yale New Haven Hospital Wilmington, DE
New Haven, CT USA
USA Chapter 17 Newborn with Poor Oral Intake and Irritability
Chapter 115 Infant with Influenza A and New Fever

Louise Flynn, MSN, CCTC Bradley Goettl, DNP, AGACNP-BC, FNP-C, ENP-C
Clinical Nurse Specialist Assistant Professor/Clinical
Nemours/Alfred I. duPont Hospital for Children University of Texas Health
Wilmingon, DE San Antonio, TX
USA USA
Chapter 23 A 2-Year-Old with Liver Transplant and Fever Chapter 28 Child with Racing Heart

Nicole Fragale, MPH, RD, CSP, LDN, CNSC

Registered Dietitian Amy K. Graf, MSN, DNP, FNP-C, CPNP-AC
Nemours/Alfred I. duPont Hospital for Children Nurse Practitioner
Wilmington, DE Norton Women and Children’s Hospital
USA Louisville, KY
Chapter 71 Feeding Challenge in a Child with Spinal USA
Muscular Atrophy Chapter 9 Child with Left Eye Swelling
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xvi List of Contributors

Katelyn Hamann, MSN, CPNP-AC Heather Herrera, MSN, CPNP-AC/PC

Nurse Practitioner Nurse Practitioner
Pediatric Hematology/Oncology Children’s Hospital of San Antonio
Children’s Hospital of Michigan San Antonio, TX
Detroit, MI USA
USA Chapter 20 Pre-school Child with Seizures;
Chapter 36 Pre-adolescent with Knee Pain; Chapter 70 Delirium in a 9-Year-Old Boy
Chapter 41 Teenager with Sickle Cell Disease and Fever
Tamara Hill, DNP, CPNP-AC
Stephanie Haughey, MSN, CPNP-AC Nurse Practitioner
Nurse Practitioner UMSON Faculty Associate/Adjunct Faculty
Spectrum Health, Helen DeVos Children’s Hospital University of Maryland Medical Center
Grand Rapids, MI Baltimore, MD
USA USA
Chapter 37 Infant with Increased Chapter 30 A 4-Year-Old with Bloody Diarrhea;
Fussiness and Decreased Oral Intake Chapter 51 Henoch–Schönlein Purpura Nephritis;
Chapter 109 Idiopathic Nephrotic Syndrome
Roseann Hausmann, MSN, CPNP-AC/PC Section Editor: Genitourinary/Renal
Nurse Practitioner
Seattle Children’s Hospital
Seattle, WA Elizabeth Hoppe, MSN, CPNP-AC
USA Nurse Practitioner
Chapter 108 Acute Jaundice in a 7-Year-Old Child; Ann & Robert H. Lurie Children’s Hospital of Chicago
Chapter 116 A 3-Year-Old with Fever and Painful Urination Chicago, IL
USA
Catherine M. Haut, DNP, CPNP-AC/PC, FAANP, CCRN Chapter 57 Teen with Fever and Rash
Coordinator of Nursing Research and Evidence Based Care
Nemours/Alfred I. duPont Hospital for Children, Lisa Hovingh, CPNP-AC
Wilmington, DE; Nurse Practitioner
Nurse Practitioner Pediatric Hematology/Oncology
Mednax-Pediatrix Medical Group Helen DeVos Children’s Hospital
Richardson, TX Grand Rapids, MI
USA USA
Chapter 12 A 6-Month-Old Infant with RSV and Respiratory Chapter 15 School-age Child with Bruising
Failure; Chapter 111 A 10-Year-Old with Asthma in the PICU
Section Editor: Respiratory Jay M. Hunter, DNP, CPNP-AC, CCRN, CPN
Book Editor: Cases in Pediatric Acute Care: Strengthening Nurse Practitioner and Assistant Clinical Professor
Clinical Decision Making UCSF Benioff Children’s Hospital and
UCSF School of Nursing
Natalie Hecht, MSN, CPNP-AC San Francisco, CA
Nurse Practitioner USA
Pediatric Cardiac Surgery Chapter 110 School-age Child with Hives
C.S. Mott Children’s Hospital and Lip Swelling
Ann Arbor, MI
USA
Marshay James, DNP, MSNEd, CPNP-AC, CPEN, CNE
Chapter 82 Adolescent with Murmur and Chest Flutter
Instructor
Jennifer Helman, MSN, CPNP-AC/PC Vanderbilt University School of Nursing Nashville, TN;
Nurse Practitioner Advanced Practice Provider Manager
Pediatric Otolaryngology Department of Critical Care Medicine
C.S. Mott Children’s Hospital St. Jude Children’s Research Hospital
Ann Arbor, MI Memphis, TN
USA USA
Chapter 24 Respiratory Decompensation in Trached Infant Chapter 90 Newborn with Hypothermia
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List of Contributors xvii

Jennifer Joiner, MSN, CPNP-AC/PC Jeanne Little, DNP, PNP-AC/PC

Assistant Professor Baylor College of Medicine Assistant Professor
Lead Nurse Practitioner Pediatric Intensive Care Unit Rush University College of Nursing
Children’s Hospital of San Antonio Chicago, IL
San Antonio, TX USA
USA Chapter 44 Acute Hyperglycemia
Section Editor: Neurology

Ji-Yeon Kim, MD Rachel Luthen, MSN, CPNP-AC

Pediatric Critical Care Nurse Practitioner
Children’s Hospital of Michigan Children’s Hospital of San Antonio
Detroit, MI San Antonio, TX
USA USA
Chapter 1 Complication of Pneumonia Chapter 20 Pre-school Child with Seizures

Andrea M. Kline-Tilford, PhD, CPNP-AC/PC, FCCM, FAAN

Nurse Practitioner Manager Amanda Mandel, CPNP
C.S. Mott Children’s Hospital Urology Nurse Practitioner
Michigan Medicine Nemours/Alfred I. duPont Hospital for Children
Ann Arbor, MI Wilmington, DE
USA USA
Book Editor: Cases in Pediatric Acute Care: Strengthening Chapter 56 Testicular Torsion
Clinical Decision Making

Lisa M. Kohr, MSN, CPNP-AC, MPH Amy Manion, PhD, RN, CPNP-PC
Nurse Practitioner Nurse Practitioner
Acute Care Cardiology Associate Professor
Cincinnati Children’s Hospital Medical Center Rush University College of Nursing
Cincinnati, OH Chicago, IL
USA USA
Chapter 19 Desaturation in Postoperative Chapter 79 Sore Throat and Shortness of Breath
Cardiac Surgery Infant

Jill Marks, DNP, CPNP-AC

Shannon Konieczki, MSN, CPNP-AC
Nurse Practitioner
Children’s Hospital of Michigan
Detroit, MI
USA
Chapter 36 Pre-adolescent with Knee Pain;
Chapter 41 Teenager with Sickle Cell Disease and Fever
Senior Instructor
University of Colorado Pediatric Neurology, Boulder, CO;
Faculty Acute Care Pediatric Nurse Practitioner Program
Rush University College of Nursing
Chicago, IL
USA
Chapter 96 Child with Leg and Back Pain

Christyne Kyper, MSN, CPNP AC/PC

Nurse Practitioner Amarilis Martin, MD
Alaska Native Tribal Health Consortium Pediatric Critical Care Fellow
Anchorage, AK Children’s Hospital of Michigan
USA Detroit, MI
Chapter 50 A 2-Month-Old with Acute Abdomen; USA
Chapter 107 A Teen With Vomiting and Diarrhea Chapter 73 Child with Scald Burn Injury
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xviii List of Contributors

Sarah Martin, MS, CPNP-AC/PC, CCRN Osei Owusu, MD

Nurse Practitioner
Department of Pediatric Surgery
Ann & Robert H. Lurie Children’s Hospital
Chicago, IL
USA
Chapter 14 Young Infant with Vomiting;
Chapter 92 Teen with Fever and Abdominal Pain
Pediatric Critical Care Medicine
Mednax-Pediatrix Medical Group
The Herman and Walter Samuelson Children’s Hospital
Baltimore, MD
USA
Section Editor: Respiratory

Cathy C. McAdams, PPCNP-BC Jaime Panton, DNP, CPNP-AC/PC

Nurse Practitioner Assistant Professor of Nursing
Nemours/Alfred I. duPont Hospital for Children PNP Program Director
Wilmington, DE Columbia School of Nursing
USA New York, NY
Chapter 114 Kidney Transplant Complication USA
Chapter 87 Postoperative Spinal Fusion with Complications
Alicia McCarthy, MSN, CPNP-AC
Nurse Practitioner
Jessica Pech, MSN, CPNP-PC
Nemours/Alfred I. duPont Hospital for Children
Nurse Practitioner
Wilmington, DE
Ann & Robert H. Lurie Children’s Hospital of Chicago
USA
Chicago, IL
Chapter 76 A 9-Year-Old With Scoliosis;
USA
Chapter 91 Back Pain in an Active Teenager
Chapter 78 A Toddler With Abdominal Pain,
Diarrhea and Lethargy
Kristen Nelson McMillan, MD
Associate Professor
Pediatric Cardiac Critical Care
Tara Pezzuto, DNP, CPNP
Advocate Children’s Pediatric Heart Institute
Headache Program
University of Chicago Comer Children’s Hospital
Nemours/Alfred I. duPont Hospital for Children
Chicago, IL
Wilmington, DE
USA
USA
Chapter 2 Infant with Diaphoresis during Feedings
Chapter 53 Teen with Intractable Headache

Suzette Meinke, MSN, PNP-BC

Nurse Practitioner Sarah Pihl, MSN, CPNP-AC
Pediatric Hematology Nurse Practitioner and Senior Instructor
Children’s Hospital of Michigan Children’s Hospital of Colorado
Detroit, MI Aurora, CO
USA USA
Chapter 66 A 5-Year-Old with Right-Sided Weakness Chapter 100 Infant with Hyponatremia

Christopher D. Newman, PA-C, FCCM

Physician Assistant Max Pizzo, MS, CPNP-AC
Children’s Hospital of Colorado Nurse Practitioner
Aurora, CO Pediatric Critical Care Medicine
USA C.S. Mott Children’s Hospital
Chapter 95 Jittery 2-Week-Old Infant; Ann Arbor, MI
Chapter 105 A 3-Day-Old with Irritability; USA
Section Editor: Fluid and Electrolytes Chapter 88 Orthopnea in a 10-Year-Old

Blythe Pollack, MSN, CPNP-AC
Nurse Practitioner
Pediatric Critical Care Medicine
C.S. Mott Children’s Hospital
Ann Arbor, MI
USA
Chapter 43 Infant with Neurologic Changes and
Respiratory Failure;
Chapter 104 Infant with Generalized Weakness
Charlene Pringle, MSN, CPNP-AC/PC
Nurse Practitioner
Pediatric Critical Care Medicine
University of Florida
Gainesville, FL
USA
Chapter 77 An Unresponsive Infant;
Chapter 97 Fussy, Tired Infant
Ann Marie Ramsey, RN, MSN, CPNP
Pediatric Nurse Practitioner
Pediatric Home Ventilator Program
C.S. Mott Children’s Hospital
Michigan Medicine
Ann Arbor, MI
USA
Chapter 27 Complex Patient with Recurrent Lower
Respiratory Tract Infection
Hayley Redmond, DO, FAAP
Pediatric Critical Care Fellow
Norton Children’s Hospital
Louisville, KY
USA
Chapter 112 Skin Wound in Postoperative Neonate
Christine Renke, MSN, CPNP-AC
Nurse Practitioner
Pediatric Critical Care Medicine
C.S. Mott Children’s Hospital
Ann Arbor, MI
USA
Chapter 57 Teen with Fever and Rash
Julie Resler, CPNP-AC
Nurse Practitioner
Riley Hospital for Children
Indianapolis, IN
USA
Chapter 8 An 8-Year-Old with Abdominal Trauma;
Chapter 103 A Serious Football Injury
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List of Contributors xix
J. Paige Rieckhoff, MSN, CPNP-AC
Nurse Practitioner
C.S. Mott Children’s Hospital
Ann Arbor, MI
USA
Chapter 101 Bone Marrow Transplant in Sickle Cell Disease
Candie Ritsema, MSN, CPNP-AC, CPON
Nurse Practitioner
Pediatric Hematology/Oncology
Helen DeVos Children’s Hospital
Grand Rapids, MI
USA
Chapter 15 School-age Child with Bruising
Stephanie Robell, MSN, CPNP-AC
Nurse Practitioner
Pediatric Critical Care Medicine
C.S. Mott Children’s Hospital
Ann Arbor, MI
USA
Chapter 40 Child with Fever and Sore Throat
Christine Schindler, PhD, RN, CPNP-PC/AC
Clinical Associate Professor Coordinator
Pediatric Acute Care Graduate Option
Marquette University College of Nursing
Milwaukee, WI;
Advanced Practice Provider Director
Critical Care/Palliative Care Advanced Practice
Provider Program
Medical College of WI/Children’s Hospital of WI
Milwaukee, WI
USA
Chapter 89 Complication of Traumatic Brain Injury
Heather Schniepp, DNP, CPNP-AC/PC
Nurse Practitioner
University of Texas Health
San Antonio, TX
USA
Chapter 33 Newborn Immediately Post Delivery
Stacey Sears, MSN, CPNP-AC
Nurse Practitioner
Pediatric Critical Care Medicine
C.S. Mott Children’s Hospital
Ann Arbor, MI
USA
Chapter 43 Infant with Neurologic Changes
and Respiratory Failure
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xx List of Contributors

Danielle Sebbens, DNP, CPNP-AC/PC Kim Steanson, DNP, CPNP-AC/PC

Clinical Associate Professor Nurse Practitioner
Coordinator Acute Care Pediatric Nurse Practitioner Monroe Carell Jr. Children’s Hospital at Vanderbilt
Program Nashville, TN;
Arizona State University Instructor
Edson College of Nursing and Health Innovation Vanderbilt University School of Nursing
Phoenix, AZ; Nashville, TN
Alaska Native Medical Center USA
Nurse Practitioner Chapter 22 Osteomyelitis Case Study;
Anchorage, AK Chapter 102 Child with Respiratory Failure
USA and Transaminitis
Section Editor: Gastrointestinal

Ashley Thibodeau, DNP, CPNP-PC

Shari Simone, DNP, CPNP-AC, FAANP, FCCM, FAAN
Nurse Practitioner
Senior NP Clinical Program Manager
Seattle Children’s Hospital
Women & Children’s Services
Seattle, WA
Nurse Practitioner
USA
University of Maryland Medical Center
Chapter 74 Parvovirus Requiring Blood Transfusion
Baltimore, MD
USA
Section Editor: Professional Issues
Jill Siegrist Thomas, MSN, CPNP-AC
Nurse Practitioner
Dawn M. Silverman, DNP, CRNP, AGACNP-BC University of Maryland Children’s Hospital
Nurse Practitioner University of Maryland Medical Center
University of Maryland Children’s Hospital Baltimore, MD
University of Maryland Medical Center USA
Baltimore, MD Chapter 80 Oral Presentation and Communication;
USA Chapter 113 Provider Burnout and Resiliency
Chapter 10 Billing and Coding

Lauren R. Sorce, PhD, CPNP-AC/PC, CCRN, FCCM Kathleen P. Thompson, MPAS, PA-C
Founders Board Nurse Scientist Assistant Professor
Pediatric Critical Care APRN Baylor College of Medicine,
Ann & Robert H. Lurie Children’s Hospital of Chicago Houston, TX;
Chicago, IL; Physician Assistant Program & Department of Pediatrics
Assistant Professor, Division of Critical Care Medicine Pediatric Critical Care Medicine, Texas Children’s Hospital
Northwestern University Feinberg School of Medicine Houston, TX
Chicago, IL USA
USA Chapter 45 Infant with Progressive Lethargy
Chapter 64 Research Review and Poor Feeding

Jessica Spruit, DNP, CPNP-AC, CPHON, BMTCN Bradley Tilford, MD

Assistant Professor, Clinical Pediatric Intensivist
Wayne State University College of Nursing Children’s Hospital of Michigan
Detroit, MI Detroit, MI
USA USA
Chapter 66 A 5-Year-Old with Right-Sided Weakness Chapter 59 Toddler with Acute Respiratory Failure;
Section Editor: Hematology/Oncology Chapter 73 Child with Scald Burn Injury
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List of Contributors xxi

Alison Hewitt Torres, MSN, CPNP-AC Judy Verger, ARNP, PhD, FAAN, FCCM
Nurse Practitioner University of Iowa School of Nursing
Johns Hopkins Children’s Center Iowa City, IA
Baltimore, MD USA
USA Chapter 49 A 3-Year-Old with Sudden
Chapter 93 Complication in a 6-Year-Old with a Kidney Onset of Breathing Difficulty
Transplant; Chapter 94 Acute Hypertension and Seizures
Mark Weber, CPNP-AC, FCCM
in a 17-Year-Old Male
Nurse Practitioner
Children’s Hospital of Philadelphia
Darby Tozer, MSN, CPNP-AC Philadelphia, PA
Nurse Practitioner USA
Pediatric Emergency Medicine Chapter 16 Infant with Shunted Hydrocephalus;
Beaumont Hospital Chapter 39 Child with Rash and Drooling
Troy, MI
USA Amanda Lewis Weir, CPNP-AC
Chapter 21 Child with Blood in Stool Nurse Practitioner
Norton Children’s Hospital
Louisville, KY
Yu-shan Tseng, MD
USA
Pediatric Critical Care Fellow
Chapter 65 Toddler with Fever and Redness Behind Ear
Children’s Hospital of Michigan
Detroit, MI Mari Welch, MSN, APRN, CPNP-AC
USA Nurse Practitioner
Chapter 7 A 5-Year-Old with Altered Mental Status Nemours/Alfred I. duPont Hospital for Children
Wilmington, DE
USA
Jamie Tumulty, MSN, CPNP-AC
Chapter 69 A Toddler With Stridor
Nurse Practitioner
University of Maryland Children’s Hospital Janice L. Wilson, DNP, NNP-BC
University of Maryland Medical Center Assistant Professor and NNP Specialty Director
Baltimore, MD University of Maryland School of Nursing
USA Baltimore, MD
Chapter 42 Ethical Case Study USA
Chapter 25 Neonatal Hypoglycemia
Danielle Van Damme, DNP, CPNP-AC
Nurse Practitioner Barbara Wise, PhD, CPNP-AC/PC
Norton Children’s Hospital Assistant Professor
University of Louisville University of Maryland School of Nursing
Louisville, KY Baltimore, MD
USA USA
Chapter 40 Child with Fever and Sore Throat Chapter 61 Newborn with Ambiguous Genitalia
Section Editor: Immunology/Rheumatology/HEENT/
Cathy Woodward, DNP, APRN, CPNP-AC
Dermatology
Professor
Long School of Medicine
Melissa C. Varrone, MSN, CPNP, CCRN University of Texas Health
Pediatric Orthopedic Nurse Practitioner San Antonio, TX
Morgan Stanley Children’s Hospital of New York, NY; USA
New York Presbyterian Hospital, Columbia University Chapter 13 Adolescent Abdominal Pain after Cardiac
Irving Medical Center Surgery; Chapter 52 Toddler with Tachypnea and Cool
New York, NY Extremities; Chapter 67 Postoperative Heart Block;
USA Chapter 85 Postoperative Infant with Tachycardia;
Chapter 87 Postoperative Spinal Fusion Chapter 99 Lightheaded Adolescent
with Complications Section Editor: Cardiovascular
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xxii List of Contributors

Maria C. Woosley, DNP, CPNP-AC Kimberly Young-Conner, ARNP-C

Faculty Clinical Instructor Nurse Practitioner
Long School of Medicine Nemours/Alfred I. duPont Hospital for Children
University of Texas Health Wilmington, DE
San Antonio, TX USA
USA Chapter 26 Chronic Otitis Media;
Chapter 86 Brain Death Chapter 34 A 5-Year-Old with Poor Weight Gain
and Dehydration
Jennifer Wright, MS, RN, CPNP-PC
Nurse Practitioner
Stepping Stones Pediatric Palliative Care
C.S. Mott Children’s Hospital
Ann Arbor, MI
USA
Chapter 35 Toddler with Complex Chronic Condition

Complication of Pneumonia
Ji-Yeon Kim
Children’s Hospital of Michigan, Detroit, MI, USA
A 2-year-old previously healthy female is admitted to the
pediatric intensive care unit (PICU) with tachypnea, grunt-
ing, and nasal flaring. She is hypoxic despite receiving sup-
plemental oxygen with a high-flow nasal cannula (HFNC)
at 12 L/min and FiO2 0.7.
History of Present Illness
This is a 2-year-old female with no significant past medical
history who presented with 2 days of fever, cough, and dif-
ficulty breathing. Her mother reports that for the past
2 weeks the patient has had intermittent low-grade fever
with cough. Difficulty breathing started 2 days ago with
fever of 40 C (104 F). She has been eating and drinking less
and having decreased wet diapers. She has been in close
contact with her older brother who has also had symptoms
of cough, rhinorrhea, and congestion. Her parents decided
to bring her to the emergency department (ED) when her
fever did not defervesce with acetaminophen and she con-
tinued to have difficulty breathing.
Past Medical History
The patient was born at 39 weeks gestational age. Family
denies history of hospitalizations or outpatient visits for ill-
ness. Immunizations are up-to-date for age, but she has not
received the influenza vaccine this year.
Past Surgical History
Family denies any previous surgical procedures.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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1
Family History
Parents are healthy. There is a 6-year-old sibling who had a
cold and fever about a week ago. Grandparents are alive
and well. Paternal grandmother has a history of breast can-
cer. Denies family history of asthma or allergies.
Current Status
On arrival in the ED, the patient is noted to have difficulty
breathing, with grunting and nasal flaring. She is tachy-
cardic, with heart rate of 160 bpm, and tachypneic, with
respiratory rate of 56 breaths per minute. On examination
the patient has diminished breath sounds over the left
lung field. The patient is placed on 50% oxygen using
HFNC at 8 L/min but remains hypoxic with pulse oxim-
eter reading of 90% saturation. A basic metabolic panel
and complete blood count are obtained with values as
noted in Tables 1.1 and 1.2. The chest X-ray is shown in
Figure 1.1.
Questions
Answer the following questions using the details provided.
1 What are the pathologic findings on the X-ray?
2 What are the most common pathogens causing pneu-
monia in infants and children?
3 What are the recommended antimicrobial therapies for
bacterial pneumonia?
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2 Cases in Pediatric Acute Care

Table 1.1 Complete blood count. There is complete obliteration of the lateral hemithorax
and the left lung base. The right lung is clear. Gas-distended
White blood cell count 18 900/mm3
bowel loops are noted in the left upper abdomen.
Hemoglobin 13 g/dL
Hematocrit 32%
Platelet count 303 000/mm3 What are the most common pathogens causing
pneumonia in infants and children?
Neutrophils 63%
Lymphocytes 11% See Table 1.3.
Monocytes 20%
Bands 4% What are the recommended antimicrobial
therapies for bacterial pneumonia?

Table 1.2 Basic metabolic profile. Treatment of suspected bacterial pneumonia is based on
presumed pathogen, age, and clinical appearance of the
Sodium 130 mEq/L patient. For a mildly ill child who does not require hospi-
Potassium 4.5 mEq/L talization, amoxicillin is recommended. For school-age
Chloride 97 mEq/L children or clinical features concerning for atypical pneu-
Carbon dioxide 18 mEq/L monia (Mycoplasma pneumoniae or Chlamydophila
Blood urea nitrogen 35 mg/dL pneumoniae), azithromycin is recommended. For those
who require hospitalization, the antibiotic of choice is
Creatinine 0.83 mg/dL
cefotaxime or ceftriaxone. If clinical features suggest Staph-
Glucose 135 mg/dL
ylococcus aureus (pneumatoceles, empyema or bronchopul-
C-reactive protein (CRP) 4412 mg/L monary fistula) initial antibiotic therapy should include
clindamycin or vancomycin.
If viral pneumonia is suspected, it is reasonable to with-
hold antibiotic therapy especially in patients who are clin-
ically stable and present with clinical evidence suggesting a
viral infection. Optimal total duration of antibiotic therapy
should not be less than 10–14 days, unless azithromycin is
used (duration of 5 days total).
Continuation of case: The child is admitted to the PICU
and continues to have oxygen desaturations despite an
increase to 70% oxygen using HFNC at 12 L/min. She
becomes lethargic with increased work of breathing. The
patient is intubated and a chest tube is placed in the left
pleural space. About 400 mL of frank pus is drained from
the chest tube.
4 What are the most common causes of pleural effusion?
5 What are the characteristics of an exudative effusion?
6 What is the management of pleural empyema?
Figure 1.1 Chest X-ray.

What are the most common causes of pleural

Rationale and Evidence-based Practice effusion?
Explanation See Table 1.4.

What are the pathologic findings on the X-ray?

What are the characteristics of an exudative
There is a large left pleural effusion with atelectasis of the
effusion (empyema)?
left lung. Atelectasis is suspected because of shifting of
the mediastinum toward the left suggesting volume loss. See Table 1.5.
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Chapter 1 Complication of Pneumonia 3

Table 1.3 Pathogens causing pneumonia in infants and children.

Cause Age and epidemiology Common complications

Bacterial
Group B streptococci Neonates Shock, respiratory failure, apnea
Streptococcus pneumoniae 3 weeks to 4 years, asplenia, Consolidation, pneumatoceles, empyema, shock
immunosuppressed
Staphylococcus aureus Infants Pneumatoceles, empyema
Mycoplasma pneumoniae >5 years; summer to fall Atypical pneumonia
Group A streptococci >5 years Empyema, pneumatoceles
Chlamydophila pneumoniae Adolescents Atypical pneumonia
Chlamydia trachomatis Neonates Associated with conjunctivitis
Mixed anaerobes Underlying pulmonary disease Aspiration and necrotizing pneumonia, pneumatoceles
Gram-negative enterics Neonates Hospital-acquired pneumonia, necrotizing pneumonia,
(Pseudomonas) shock

Viral: infants and children <5 years

Respiratory syncytial virus Winter Bronchiolitis, apnea
Parainfluenza types 1–3 Fall Croup, wheezing, pneumonitis, tracheitis
Influenza A, B Winter High fever, bacterial superinfection, apnea, seizures,
Guillain–Barré syndrome, Reye syndrome
Human metapneumovirus Winter Similar to respiratory syncytial virus

Others
Foreign body, food or gastric acid aspiration, hypersensitivity to inhaled material (hydrocarbon or lipoid substances), drug- or
radiation-induced pneumonitis

Table 1.4 Causes of pleural effusion.

Category Causes

Infection (exudates) Bacterial, viral, fungal, mycobacterial

Cardiovascular Congestive heart failure, constrictive pericarditis, superior vena cava obstruction, postcardiac surgery
Pulmonary Pulmonary infarction, atelectasis, pulmonary embolism, drug-induced pleuritic reaction
Intra-abdominal Abdominal surgery, pancreatitis, hepatitis, peritonitis, subdiaphragmatic or intrahepatic abscess, cirrhosis
with ascites
Renal Uremia, urinary tract obstruction, nephrotic syndrome, peritoneal dialysis
Neoplastic Lymphoma, leukemia, carcinoma
Collagen vascular Rheumatoid arthritis, systemic lupus erythematosus, Sjögren syndrome, Wegener granulomatosis,
disease myxedema, sarcoidosis
Iatrogenic/ Extravascular central venous catheter placement (i.e. misplaced catheter), radiation therapy, hemothorax,
miscellaneous chylothorax, hypoalbuminemia, esophageal rupture

What is the management of empyema?
Treatment of empyema is based on stage of disease
(exudative, fibrinopurulent, and organizing) which can
be assessed using imaging studies such as ultrasonography
and computed tomography (CT). In stage 1 (exudative),
fibrinous exudates, which are clear and sterile, form on
the surface of the pleura. In stage 2 (fibrinopurulent),
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4 Cases in Pediatric Acute Care

Table 1.5 Characteristics of an exudative effusion (empyema).

Characteristic Transudate Empyema

Appearance Clear Cloudy

Cell count (per mm3) <1000 Usually >50 000
Cell type Lymphocytes, monocytes Neutrophils
Lactate dehydrogenase (LDH) <200 U/L >1000 U/L
Pleural fluid LDH/serum LDH <0.6 >0.6
Protein <3 g Rare Frequent
Pleural fluid protein/serum protein <0.5 >0.5
Glucose Normal (>60 mg/dL) Low (<40 mg/dL)
pH Normal (7.40–7.60) <7.30
Gram stain Negative Sometimes positive

the exudates become thick and purulent. Septations are
formed creating infected pleura that are loculated. In stage
3 (organizing), granulation tissues are created from
proliferation of the fibroblast-creating areas of abscess
cavities surrounding the lung. Treatment involves antibi-
otic therapy and drainage with tube thoracostomy. In
order to debride or lyse adhesions and drain loculated
areas of pus, additional approaches could be utilized
including intrapleural fibrinolytic therapy (streptokinase,
urokinase, or tissue plasminogen activator) and surgical
options (selected video-assisted thoracoscopy and
decortication).

Further Reading
Brunow de Carvalho, W., Johnston, C., and Fonseca, M.C.
(2015). Bronchiolitis and pneumonia. In: Rogers’ Textbook of
Pediatric Intensive Care (ed. D.H. Shaffner and D.G.
Nichols), 716–730. Philadephia, PA: Lippincott Williams &
Wilkins.
Cashen, K. and Petersen, T.L. (2017). Pleural effusions and
pneumothoraces. Pediatrics in Review 38: 170. doi: 10.1542/
pir.2016-0088.
Pasala, S., Storm, E.A., Stroud, M.H. et al. (2017). Pediatric
vascular access and centeses. In: Pediatric Critical Care
(ed. B.P. Fuhrman and J.J. Zimmerman), 175–178.
Philadelphia, PA: Elsevier.
Sandora, T.J. and Sectish, T.C. (2011). Community-acquired
pneumonia. In: Nelson Textbook of Pediatrics
(ed. R. Kliegman), 1474–1479. Philadelphia, PA: Elsevier
Saunders.
Winnie, G.B. and Lossef, S.V. (2011). Pleurisy, pleural
effusions, and empyema. In: Nelson Textbook of Pediatrics
(ed. R. Kliegman), 1505–1509. Philadelphia, PA: Elsevier
Saunders.

Infant with Diaphoresis during Feedings
Kristen Brown and Kristen Nelson McMillan
Johns Hopkins University School of Nursing, Baltimore, MD, USA
A 10-week-old infant presents to the emergency depart-
ment (ED) with lethargy, described as sleeping more and
being less playful, associated with reduced oral intake
and increased work of breathing.
History of Present Illness
She has become diaphoretic and tachypneic with feedings
and requires multiple rest periods to finish a bottle.
Past Medical History
This infant was full term and delivered via vaginal delivery.
Birthweight was 3.5 kg. Immunizations are up to date for
age. Formula fed and on multivitamins every day.
Past Surgical History
No prior surgeries.
Family History
The parents have no significant medical history. The
5-year-old sibling is healthy. No one is sick at home.
Current Status
On arrival at the ED, axillary temperature is 36.8 C (98.2 F),
respiratory rate 65 breaths per minute, heart rate 165 bpm in
normal sinus rhythm, blood pressure 92/68 mmHg, and pulse
oximeter 99% on room air. Weight is 4.3 kg. The baby is mildly
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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5
thin, pale, lethargic, and tachypneic, with mild retractions.
Her skin is clear with no rashes or other significant skin
lesions. Sinus tachycardia is noted on the monitor. On auscul-
tation, a grade II/VI holosystolic murmur at the mid lower left
sternal border with radiation to the cardiac apex is noted. An
S4 gallop is noted at the cardiac apex. There are no rubs or
clicks. The abdomen is soft, non-distended, and non-tender.
Liver edge is palpable 3–4 cm below the right costal margin.
There are no palpable masses or splenomegaly. Bowel
sounds are normal. Extremities are symmetric and cool,
with peripheral pulses 1+ in all extremities. Capillary refill
is 3–4 seconds.
Questions
Answer the following questions using the details provided.
1 Based on the case presentation, what is your differential
diagnosis?
2 Which diagnostic studies are indicated?
Continuation of case: Initial diagnostic studies show the
following results. The complete metabolic profile is unre-
markable. The complete blood count demonstrates a white
blood cell count of 9000/mm3 and hemoglobin of 12.4 g/dL.
The chest radiograph shows moderate cardiomegaly with
mild pulmonary edema. The 12-lead electrocardiogram
(EKG) shows sinus tachycardia, and normal PR and QTc
intervals with left axis deviation. Voltage evidence of biven-
tricular hypertrophy is present. No significant Q waves or
ST-segment changes are noted.
3 What additional studies are needed?
4 Which therapies and medications are appropriate in the
management of this child?

6 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
Based on the case presentation, what is your
differential diagnosis?
This patient has signs of cardiac failure: tachycardia, poor
capillary refill, hepatomegaly, poor weight gain, and leth-
argy. Differential diagnosis for this child includes conges-
tive heart failure secondary to structural heart disease,
cardiomyopathy, myocarditis, arrhythmia, and anemia.
The initial differential diagnosis also includes an intercur-
rent respiratory illness (e.g. respiratory syncytial virus).
Which diagnostic studies are indicated?
The initial diagnostic studies will focus on evaluation of car-
diac function and of infection. A complete blood count and
respiratory viral panel will help determine if an infectious eti-
ology is present; hemoglobin status is also evaluated. Blood
and urine cultures may also be ordered to evaluate for infec-
tion. A complete metabolic panel can be obtained to evaluate
hydration status and renal and hepatic function. A chest radi-
ograph is needed to evaluate lung fields and heart size and an
EKG is required to evaluate rhythm and voltages.
What additional studies are needed?
An echocardiogram is necessary to evaluate underlying
structural disease due to physical examination findings
and cardiomegaly on chest radiograph. The echocardio-
gram demonstrates a large perimembranous ventricular
septal defect (VSD) with non-restrictive left-to-right shunt-
ing. All cardiac chambers are dilated. Left ventricular con-
tractility is at the lower range of normal. There is no
pericardial effusion. This acyanotic cardiac lesion is associ-
ated with increased pulmonary blood flow and left-to-right
shunting, resulting in pulmonary edema and increased
work of breathing (congestive heart failure).
Which therapies and medications are appropriate
in the management of this child?
Therapy is targeted at improving congestive heart failure.
Pharmacologic strategies may include diuretics (e.g. furose-
mide), afterload-reducing agents, and digoxin. Diuretics are
often indicated to reduce total body water, alleviating cardiac
workload and reducing pulmonary edema. Afterload
reduction may be considered to decrease systemic vascular
Further Reading
Park, M.K. (2014). Park’s Pediatric Cardiology for Practitioners,
6th edn. Philadelphia, PA: Elsevier Saunders.
Ungerleider, R.M., Meliones, J.N., Nelson-McMillan, K., Cooper,
D.S., and Jacobs, J.P. (ed.) (2019). Critical Heart Disease in
Infants and Children, 3rd edn. Philadelphia, PA: Elsevier.
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resistance and augment cardiac output. Most often, an angio-
tensin-converting enzyme (ACE) inhibitor is used to decrease
systemic vascular resistance and decrease the renin–
angiotensin–aldosterone system which is continuously acti-
vated with CHF. Spironolactone, a weak diuretic, is often
added to the regimen for potassium-sparing effect and to
augment lowering of the renin–angiotensin–aldosterone sys-
tem. Milrinone is often used in the intensive care setting in
acute new-onset systemic ventricular dysfunction to improve
cardiac output and reduce left ventricular afterload. Care must
be taken to avoid hypotension as milrinone results in systemic
vasodilation and may also result in some pulmonary vasodila-
tion given its general vascular dilation effect. Digoxin may be
used to treat cardiac failure in place of or to augment ACE
inhibitor use. The action of digoxin is mediated through inhi-
bition of Na+/K+-ATPase pumps, which leads to a slight
increase in the force of contraction (inotropy).
Oxygen is not necessary for this infant; however, if respira-
tory support is needed, consider non-invasive respiratory sup-
port such as continuous positive airway pressure (CPAP)
or HFNC at the lowest oxygen concentration to maintain nor-
mal saturations. Oxygen can result in an increase in left-to-
right shunting as it causes lowering of pulmonary vascular
resistance, which can result in worsening of pulmonary
edema; thus, oxygen is used cautiously in this presentation.
To promote growth, frequent high-calorie feedings deliv-
ered either orally or by nasogastric tube are initiated in
patients awaiting surgical management. Support from a
dietitian is often needed for these children.
Infants with a large VSD defect and congestive heart fail-
ure are candidates for surgical repair. Medical management
with diuretics, ACE inhibitors, and/or digoxin are often
needed to promote growth prior to VSD repair, typically per-
formed between 3 and 6 months of age. Over time, unre-
paired, large left-to-right shunts (e.g. VSD) result in
pulmonary artery hypertension, increased pulmonary vascu-
lar resistance, and right ventricular overload, ultimately
resulting in reversal of shunt direction and Eisenmenger syn-
drome. Traditionally, VSDs have been closed surgically, but
advances in catheter intervention procedures have expanded
candidacy for transcatheter closure of perimembranous
VSDs. In infants failing to achieve adequate growth/weight
gain on optimized medical management and tube feedings
who are not yet surgical candidates, a pulmonary artery band
may be placed to restrict pulmonary blood flow and augment
systemic blood flow until the child is ready for VSD closure.
You, T., Yi, K., Ding, Z.-h., et al. (2017). Transcatheter closure,
mini-invasive closure and open-heart surgical repair for
treatment of perimembranous ventricular septal defects in
children: a protocol for a network meta-analysis. BMJ Open
7: e015642. doi: L10.1136/bmjopen-2016-015642.

Hypotension in the Operating Room
Ann Marie Felauer
University of Maryland School of Nursing, Baltimore, MD, USA
A 12-year-old female, weighing 45 kg, is admitted to the
pediatric intensive care unit (PICU) following a ruptured
appendix requiring open appendectomy with washout.
During the intraoperative period she developed hypoten-
sion that was unresponsive to fluid resuscitation and
required initiation of dopamine to maintain arterial systolic
blood pressure (SBP) of 80 mmHg. The need for inotropic
support and concern for continuing hypotension was the
rationale for PICU admission.
History of Present Illness
This is a previously healthy 12-year-old female who began
complaining of abdominal pain and fevers 4 days prior to
admission. At that time she was seen by her primary care
provider and referred to the local emergency department
(ED). Abdominal ultrasound was negative at that time
and she was discharged home with a diagnosis of viral gas-
troenteritis. She presented to the ED last evening with
worsening pain at McBurney’s point and positive Rovs-
ing’s sign. Because of concerns for appendicitis she was
admitted to surgical team and taken to the operating room
for a laparoscopic appendectomy and had an easy mask
induction with oxygen and sevoflurane. She was given
rocuronium, fentanyl, and ondansetron and was intubated
with a 7.0 cuffed endotracheal tube. End-tidal carbon
dioxide (CO2) recording was obtained and maintained
throughout the procedure and bilateral breath sounds
were auscultated. Once asleep she had two peripheral
intravenous catheters placed in her left and right hand
as well as a left radial 20-gauge arterial line placed. Anes-
thesia was maintained throughout the case with sevo-
flurane, and with intermittent doses of fentanyl and
rocuronium. Antibiotics that were started on admission
in the ED were continued during the case. The appendix
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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7
ruptured during the surgery and an open appendectomy
with washout was completed. She required two fluid
boluses of 20 mL/kg for a total of 1800 mL of normal
saline due to hypotension. An infusion of dopamine was
initiated for continued SBP of 68–78 mmHg. Dopamine
was titrated to 10 μg/kg per min to maintain SBP above
80 mmHg and mean arterial pressure (MAP) above
60 mmHg. All other vital signs remained within accepted
parameters. Despite hypotension, the remainder of the pro-
cedure went well. At the conclusion of the case, the patient
was given glycopyrrolate and neostigmine, anesthesia was
reversed, and the child was extubated without difficulty.
The patient received a total of 1800 mL of crystalloid fluid,
and there was urine output of 160 mL, with overall mini-
mal blood loss with the surgical procedure. Because of the
need for inotropic support and continued hypotension, the
patient was transferred to the PICU for continued care.
Past Medical History/Past
Surgical History
This patient was born at term by spontaneous vaginal deliv-
ery and was discharged home with her mother. She has had
all required vaccines along with annual influenza. Her
weight is at the 44% percentile for gender and age and
has been consistently in this trajectory, with a current body
mass index (BMI) of 18.1. There are no known chronic ill-
nesses and no prior surgical procedures or hospitalization.
Family History
The patient’s mother has migraine headaches and her
father has type 2 diabetes mellitus, treated with oral
anti-hyperglycemic agents.
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8 Cases in Pediatric Acute Care

Current Status Table 3.2 Complete blood count.

White cell count 22 000/mm3

On arrival at the PICU, the patient is alert and talking, cur-
Hemoglobin 11 g/dL
rently on oxygen by nasal cannula at 1 L/min and with pulse
oximetry at 99–100%. Current vital signs are as follows: Hematocrit 33%

••
Platelet count 195 000/mm3
weight documented prior to surgery, 45 kg;
Neutrophils 79%
heart rate 82–89 bpm;

• arterial blood pressure 72/46 to 84/57 mmHg; MAP

55–64 mmHg;
Lymphocytes
Monocytes
9%
4%

•• temperature 38.3 C (100.9 F) via Foley catheter;

respiratory rate 14–18/min;
Eosinophils 1%

• central venous pressure (CVP) 5–8 mmHg measured via

right subclavian double-lumen central line catheter, cur-
Questions
rently infusing dopamine at 10 μg/kg per min, plus dextrose
5% in 0.9% normal saline with KCl 20 mEq/L at 85 mL/hour;

• piperacillin/tazobactam 3 g given immediately post-

operatively and continued every 6 hours due to concerns
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
for peritonitis;

•
intake/output since the operating room: intake 2055 mL;
output 240 mL urine (1.33 mL/kg per hour).
Physical examination showed the following.
noses for this child?
2 What additional diagnostic studies and/or consults
should be obtained?
3 What is the best way to maximize blood pressure and

•
what is the goal MAP?
General: awake, alert, interactive, talking, without
Continuation of case: After discussion with the surgical
reported pain present.

• Respiratory: lungs clear to auscultation, no retractions,

full aeration.
team, the patient received another 20 mL/kg fluid bolus
on arrival at the PICU for continued hypotension and

• Cardiac: regular rate and rhythm, normal S1, S2, no

murmurs noted, capillary refill <3 seconds, pulses
was started on an epinephrine infusion at 0.03 μg/kg per
min, titrated up to a goal of maintaining MAP above
70–75 mmHg. The patient’s oxygen and respiratory effort
2+ throughout, extremities warm and well perfused.

• Abdomen: soft, tender to palpation, no organomegaly

noted, midline abdominal incision dressing intact, drain
remained stable and she is continuously monitored.
The PICU elected to obtain mixed venous oxygen saturation
level via the subclavian line with a repeat CBC, lactate level,
in place with minimal serosanguineous drainage and
and BMP, with results showing SvO2 72%, CBC and BMP sta-
absent bowel sounds.

• Neurologic: alert and oriented ×3.

A basic metabolic panel (BMP), complete blood count

ble, and lactate 1.4 mmol/L. The surgical team was reluctant to
initiate norepinephrine due to concerns for perfusion to the
surgical site, so epinephrine was titrated to 0.09 μg/kg per
(CBC) and lactate were obtained, as well as ionized calcium min with SBP maintained at 88–90 mmHg. SvO2 remained
and blood culture (see Tables 3.1 and 3.2). at 70–75% and urine output was 0.8–1.1 mL/kg per hour.
4 What evidence guides the decision to use epinephrine
Table 3.1 Basic metabolic panel. or norepinephrine?
5 Would you obtain any other diagnostic studies?
Sodium 134 mEq/L
Potassium 3.8 mEq/L
Chloride 101 mEq/L
Carbon dioxide 24 mEq/L Rationale and Evidence-based Practice
Blood urea nitrogen 12 mg/dL Explanation
Creatinine 0.6 mg/dL
Based on the data given, what are the differential
Glucose 53 mg/dL
diagnoses for this child?
Calcium 110 mg/dL
Ionized calcium 4.5 mg/dL Differential diagnoses for this child include persistent
shock due to significant fluid loss, fluid shifts or ascites,
Serum lactate 2.8 mmol/L
cardiac dysfunction, cardiac tamponade or tension

pneumothorax, or catecholamine resistance. Given this
child’s physical examination, cardiac dysfunction can be
excluded. According to the “early goal-directed therapy
for treatment of shock” protocol, the patient has received
appropriate treatment with fluid boluses and initiation
of inotropic support. However, given the other measures
of perfusion are reassuring, evaluation for adrenal
insufficiency should be considered at this point. Adrenal
insufficiency in this patient is most likely due to a proin-
flammatory response related to septic shock which has
caused decreased cortisol production and synthesis. This
dysfunction of the hypothalamic–pituitary–adrenal axis
can occur during a critical illness and results in an inade-
quate response to stress.
What additional diagnostic studies and/or
consults should be obtained?
Prior to treating for adrenal insufficiency, assessment of
adrenocortical function should be completed. Obtaining a
morning cortisol level (at 8 a.m.) would be ideal; however,
in situations such as seen with surgery there may be an
alteration in cortisol levels due to the stress of the procedure
alone. An adrenocorticotropic hormone (ACTH) stimula-
tion test also evaluates adrenal gland function and is more
reasonable in this circumstance. Measurements of serum
cortisol levels are obtained at baseline and then 60 minutes
after an infusion of synthetic ACTH. Recommended dosing
is 250 μg for children over 2 years of age, given over
1 minute. Serum cortisol levels are then evaluated in com-
parison to baseline levels, with a normal response being a
minimum of >18 μg/dL and at least 7–10 μg/dL greater
than the baseline.
Treatment of catecholamine-resistant shock requires
stress dose hydrocortisone 50–100 mg/m2 (maximum 100
mg/dose) followed by 50 mg/m2 per 24 hours (maximum
100 mg/dose) divided every 4–6 hours until the patient is
hemodynamically stable and able to be weaned from vaso-
active medications.
Further Reading
Bornstein, S.R., Allolio, B., Arlt, W., et al. (2016). Diagnosis and
treatment of primary adrenal insufficiency: an Endocrine
Society Clinical Practice Guideline. Journal of Clinical
Endocrinology and Metabolism 101(2): 364–389. doi:
10.1210/jc.2015-1710.
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Chapter 3 Hypotension in the Operating Room 9
What is the best way to maximize blood pressure
and what is the goal MAP?
In addition to capillary refill of 2 seconds or less and nor-
malization of blood pressure, goals for this patient include
urine output above 1 mL/kg per hour, normal mental sta-
tus, normalization of serum lactate (beyond the operative
period), maintaining normal perfusion pressure (MAP –
CVP for age), and SvO2 greater than 70%. Administration
of hydrocortisone will improve the hypotension and the
vasoactive infusions can be weaned in order of initiation,
with epinephrine discontinued first. Goals should continue
to be normalization of perfusion, cardiac output as well as
oxygenation and ventilation.
What evidence guides the decision to use
epinephrine or norepinephrine?
Epinephrine is used in the presence of cold shock, which in
this case was determined by hypotension as the primary
symptom with the knowledge that a ruptured appendix
can contribute to peritonitis, or an abdominal infection,
causing sepsis. Norepinephrine is primarily used for man-
agement of “warm shock,” during which time blood pres-
sure may be normal or low in children.
Would you obtain any other diagnostic studies?
Given the fact that this patient developed adrenal insuffi-
ciency after a ruptured appendix versus longer-term critical
illness and is at risk for further hypothalamic–pituitary–
adrenal dysfunction, an endocrinology consult would be
helpful for evaluation. Continued monitoring of electrolytes
while the patient is NPO and frequent intake and output are
part of PICU care. Avoidance of hypocalcemia as well as
hypoglycemia/hyperglycemia is important for preventing
secondary complications of critical illness. The patient pro-
gressed to spend another day in the PICU for weaning of
inotropic support, was stabilized, and was transferred to
the pediatric general unit on day 3 of hospitalization.
Davis, A.L., Carcillo, J.A., Aneja, R.K., et al. (2017). American
College of Critical Care Medicine clinical practice
parameters for hemodynamic support of pediatric and
neonatal septic shock. Critical Care Medicine 45(6):
1061–1093. doi: 10.1097/CCM.0000000000002425.

Toddler with Vomiting and Diarrhea
Kimberly L. DiMaria
Children’s Hospital Colorado, Aurora, CO, USA
A full-term previously healthy 18-month-old female pre-
sents to the emergency department (ED) with 2 days of
diarrhea, emesis, and low-grade fever.
History of Present Illness
The toddler was in her usual state of health until 3 days
prior to presentation at which time she developed
decreased appetite and intermittent complaints of abdom-
inal pain. The following day, she developed a tactile tem-
perature and had frequent bowel movements. Her
mother reports that the stools were loose. The day prior
to presentation, she developed emesis and was unable to
keep any food or liquids down. The mother reported fre-
quent trips to the bathroom for vomiting and diarrhea.
The mother encouraged fluids but was only able to get
the child to take sips throughout the day. Overnight, she
was restless, febrile to 38.3 C (100.9 F), and complaining
of a worsening “tummy ache.” This morning, the mother
noticed that the child appeared tired and pale, so she was
brought to the ED for further evaluation.
Past Medical History
Born at full term via scheduled cesarean section. She had
APGAR scores of 7 and 9 after birth and was discharged
home with mother on day of life 3. She was closely followed
by primary care physician (PCP) for slow weight gain dur-
ing the first 3 months of life that improved after initiation
of formula supplementation. She has had multiple upper
respiratory tract infections and one episode of acute
otitis media that required antibiotics. She is otherwise
healthy, meeting developmental milestones and tracking
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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11
consistently at the 25th percentile on the growth curve.
Her vaccinations are up to date, including seasonal
influenza vaccine. She has no known drug allergies. No
hospitalizations.
Past Surgical History
No previous surgeries.
Family History
Aunt with colon cancer, mother with asthma. Three
healthy older siblings.
Current Status
She is a well-developed toddler sleeping in her mother’s lap.
Vital signs in ED triage as follows: temperature 37.9 C
(100.2 F), heart rate 130 bpm, respiratory rate 35 breaths
per minute, blood pressure 92/55 mmHg, oxygen satura-
tions 96% on room air.
She appears lethargic, but irritable with examination.
Her eyes are slightly sunken and mucous membranes are
dry. No tears are noted when crying during examination.
Her capillary refill time is 4 seconds and she has 2+ pulses
in all extremities. She cries out when the examiner attempts
to palpate her abdomen, but all four quadrants are palpated
without evidence of tenderness. Her abdomen is round,
soft, and non-distended. No hepatosplenomegaly. Hyperac-
tive bowel sounds are present.

12 Cases in Pediatric Acute Care
Questions
Answer the following questions using the details provided.
1 Based on the data given, what is the differential diagno-
sis and the most likely diagnosis for this child?
2 What are the essential questions to ask while obtaining
the history?
3 What are the initial steps of management?
4 How would you evaluate the child’s hydration status?
5 What is your management plan?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what is the differential
diagnosis and the most likely diagnosis for
this child?
The initial differential diagnosis is broad and includes
gastroenteritis, appendicitis, central nervous system infec-
tion, bowel obstruction, bowel perforation/acute abdomen,
diabetes ketoacidosis, and ingestion. When further consid-
ering the differential diagnosis, appendicitis is on the list
because of fever, vomiting, diarrhea, and lethargy, though
is less likely due to absence of rebound tenderness or tender
abdomen. Central nervous system infection is considered
on the differential diagnosis due to fever, emesis, and
altered mental status (lethargic and irritable); a thorough
neurologic examination including assessment for nuchal
rigidity is needed. Bowel obstruction is considered in the
differential due to frequent emesis coupled with abdominal
pain, though is less likely because the emesis is not bilious
and the patient is having diarrhea. A bowel perforation/
acute abdomen is unlikely because of reassuring abdominal
examination. Diabetic ketoacidosis is considered in the
differential diagnosis due to presenting emesis, evidence
of dehydration, and mental status changes, but is not
accompanied by Kussmaul breathing or polyuria. Toxic
ingestion should also be considered, though is less likely
due to low-grade fever and prolonged illness.
The most likely diagnosis for this patient is gastroenteritis
because of the symptoms of diarrhea, low-grade fever,
abdominal pain, and emesis in the absence of a rigid and
tender abdomen and without focal neurologic symptoms.
Gastroenteritis is defined as acute onset of diarrheal illness
(e.g. increase in stool frequency and/or decrease in stool
consistency) that can occur either in isolation or in combi-
nation with nausea, emesis, fever, and abdominal pain.
Acute gastroenteritis typically lasts less than 7 days. It is
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a common illness, especially in children under 5 years of
age. The highest incidence of dehydration is in children
under 6 months of age. Most commonly, gastroenteritis is
the result of infection with rotavirus, norovirus, adenovi-
rus, Salmonella, Campylobacter, and Clostridium difficile.
Etiology varies by geographic location and vaccination
against rotavirus. Diarrheal illness characterized by fre-
quent small amounts of stool, bloody stool, high fever,
and abdominal pain are most likely to be associated with
a bacterial etiology.
What are essential questions to ask while
obtaining the history?
Essential elements of the history include onset and dura-
tion of the emesis, including color (bloody, bilious) and
timing (immediately after eating?); onset and color
(blood, mucus) of diarrhea; fluid intake and urinary out-
put (dysuria?); immunization history (specifically rotavi-
rus vaccination); and contact with others with similar
symptoms.
What are the initial steps of management?
Priority is given to evaluation for presence of hypovolemic
shock. If present, this must be treated immediately, as it can
be life-threatening. The child’s level of dehydration should
be evaluated and treatment targeted to level of dehydration
and replacement of ongoing losses. Other steps in
management include initiating an age-appropriate diet,
determining the disposition (e.g. does the child require
hospitalization?), and considering an antiemetic if emesis
prohibits effective oral rehydration therapy or for patient
comfort.
In general, children with acute gastroenteritis do not
require diagnostic evaluation with stool studies except
when the child has underlying chronic conditions, pro-
longed symptoms, bloody diarrhea, travel history, or during
outbreaks.
How would you evaluate the child’s hydration
status?
The largest driver of morbidity and hospitalization rates
associated with acute gastroenteritis is dehydration. The
exact proportion of a patient’s dehydration is thought to
be represented by the difference in their pre-illness weight
and their weight at time of presentation. This assessment is
limited in its utility because most parents are unsure of the
patient’s exact pre-illness weight. Because of this limitation,
most clinicians rely on a clinical dehydration scale which
incorporates presenting symptoms to categorize the patient

Table 4.1 Clinical dehydration scale.
Score 0 1
General appearance Normal Thirsty, restless or lethargic, but irritable when touched
Eyes Normal Slightly sunken
Mucous Moist Sticky
membranes
Tears Tears Decreased tears
Total score: 0, no dehydration; 1–4, some dehydration; 5–8, moderate/severe dehydration.
Source: Guarino, A., Ashkenazi, A., Gendrel, D., et al. (2014). European Society for Pediatric Gastroenterology, Hepatology, and Nutrition/
European Society for Pediatric Infectious Diseases evidence-based guidelines for the management of acute gastroenteritis in children in Europe:
update 2014. Journal of Pediatric Gastroenterology and Nutrition 59(1): 132–152. Reproduced with permission of Wolters Kluwer Health, Inc.
as having minimal (<3% loss of body weight), mild to mod-
erate (3–9% loss of body weight), or severe (>9% of body
weight) dehydration. Multiple clinical dehydration scales
have been developed by several organizations (Table 4.1).
The various scales vary in their reliability in predicting
actual dehydration, but can give a healthcare provider an
estimate of a patient’s dehydration status that will assist
with creating a management plan. All scoring tools must
be used in conjunction with the individual clinical exami-
nation and history. Newer methods for estimating dehydra-
tion status have been evaluated, including ultrasound to
measure inferior vena cava diameter, the ratio of inferior
vena cava to aorta diameter, and bedside hand-held bladder
ultrasound scanning and digital videography to measure
capillary refill time.
According to the clinical dehydration scale, this child is
classified as having moderate dehydration (restless, irrita-
ble, tachypneic, sunken eyes, dry mucous membranes).
What is your management plan?
The management plan for this child is to initiate oral rehy-
dration, administering 80–100 mL/kg of oral rehydration
fluid over 2–4 hours. Oral rehydration therapy is recom-
mended for mild to moderate cases of acute gastroenteritis.
She warrants close observation and may require intrave-
nous fluid therapy. She should be offered an oral rehydra-
tion fluid instead of water, juice, or a sports electrolyte
drink. She has lost electrolytes because of her diarrhea
and emesis and therefore water would not meet her electro-
lyte replacement needs. A high-glucose solution (most com-
mon in juice or sports drinks) can exacerbate diarrhea.
Ideally, an oral rehydration fluid would have a 1 : 1 glucose
to sodium ratio. She should be offered 50–100 mL/kg body
weight of oral rehydration fluid over 2–4 hours to replace
the estimated fluid deficit. Caregivers should offer small
amounts of oral rehydration therapy via spoon or syringe,
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Chapter 4 Toddler with Vomiting and Diarrhea 13
2
Drowsy, limp, cold or sweaty ± comatose
Extremely sunken
Dry
Absent tears
allowing her to drink more if she appears thirsty. In breast-
fed infants, breastfeeding should continue through oral
rehydration therapy. An age-appropriate diet can be
initiated after the initial 4–6 hours of rehydration. Modified
milk formula and dilution of formula is generally not
needed. Enteral hydration by nasogastric route is preferred
over intravenous hydration when oral rehydration is not
feasible.
The patient requires assessment of ongoing fluid losses
and replacement as needed. An antiemetic such as ondan-
setron can be prescribed if the child is unable to drink the
prescribed amount of fluid without continued emesis.
Probiotic therapy has been associated with reduction in
the duration of diarrhea, but there is limited evidence of
its impact on duration of hospitalization.
The patient does not meet hospital admission criteria
at this time. However, if she were unable to complete
oral rehydration therapy because of ongoing emesis or
diarrhea, if oral rehydration therapy at home was unsuc-
cessful, or if she developed signs of hypovolemic shock,
she would warrant either prolonged observation in the
ED or hospital admission. No laboratory studies or intra-
venous fluids are indicated currently. Additionally, a
basic metabolic panel to assess electrolytes, specifically
sodium, as well as blood urea nitrogen and creatinine
to assess renal function and severity of dehydration,
could be considered if she was refractory to oral rehy-
dration therapy and developed severe dehydration. Anti-
biotics are not a recommended therapy for acute
gastroenteritis.
The patient requires ongoing monitoring of end-organ
perfusion and level of hydration, and for symptoms of acute
abdominal process such as appendicitis, bowel obstruction,
or perforation, which are less common. If she improves
after tolerating oral rehydration therapy, she can be safely
discharged home with strict return instructions for deteri-
oration in status.
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14 Cases in Pediatric Acute Care

Further Reading
Guarino, A., Ashkenazi, A., Gendrel, D., et al. (2014). Hendrickson, M.A., Zaremba, J., Wey, A.R., Gaillard, P.R., and
European Society for Pediatric Gastroenterology, Kharbanda, A.B. (2018). The use of triage-based protocol for
Hepatology, and Nutrition/European Society for Pediatric oral rehydration in a pediatric emergency department.
Infectious Diseases evidence-based guidelines for the Pediatric Emergency Care 34(4): 227–232.
management of acute gastroenteritis in children in Europe: Onyon, C. and Dawson, T. (2018). Gastroenteritis. Paediatrics
update 2014. Journal of Pediatric Gastroenterology and and Child Health 28(11): 527–532. doi: https://doi.org/
Nutrition 59(1): 132–152. doi:10.1097/ 10.1016/j.paed.2018.08.010.
MPG.000000000000000375.
Hartman, S., Brown, E., Loomis, E., and Russell, H. (2019).
Gastroenteritis in children. American Family Physician
99(3): 159–165.

Infant with Fever and Cold Symptoms
Cheryl Bartke
Seattle Children’s Hospital, Seattle, Washington, USA
A 10-week-old with fever and respiratory symptoms is
admitted to the pediatric floor.
History of Present Illness
This 10-week-old infant has had 3 days of rhinorrhea,
congestion, and cough. In the past 12 hours, he has
developed post-tussive emesis, tactile fever, increased
work of breathing, and retractions.
Past Medical History
He was born at term, is uncircumcised, and went home
with his mother. He had his 2-month vaccines last month.
Past Surgical History
Other than circumcision, he has not had any surgeries.
Family History
Non-contributory. His older sibling is in daycare with
current upper respiratory illness.
Current Status
On arrival in the emergency department (ED), he had tach-
ypnea to 80 breaths per minute, a congested cough, subcla-
vicular, intercostal and subcostal retractions, tracheal
tugging, head bobbing, and nasal flaring. He was wheezing,
and hypoxic with an oxygen saturation of 86% on room air.
He was treated with nasal suctioning and supplemental
oxygen at 100% via blow-by and a trial of albuterol, which
did not decreasing his wheezing.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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15
He is admitted to the acute care floor from the ED with a
nasal cannula at 2 L/min and a saturation of 92%. He is
breathing at a rate of 60 breaths per minute with moderate
retractions and is still noted to have significant nasal
congestion. He continues to have intermittent wheezing.
A chest radiograph is completed (Figures 5.1 and 5.2).
Rapid laboratory testing for respiratory viral panel is
pending.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what is the differential diagno-
sis and the most likely diagnosis for this child?
2 What is the best way to increase this infant’s oxygena-
tion and how would you order this?
3 Would you consider albuterol at the same time?
4 How would you determine if the therapy was effective?
5 Would you obtain any other diagnostic studies?
6 What other therapies would be recommended?
7 What anticipatory guidance should be given to the
parents?
Rationale and Evidence-based
Practice Explanation
Based on the data given, what is the differential
diagnosis and the most likely diagnosis for
this child?
The differential diagnoses for this child include viral bron-
chiolitis, viral or bacterial pneumonia, pertussis, and reac-
tive airway disease.

16 Cases in Pediatric Acute Care
Figure 5.1 Anteroposterior (AP) chest radiograph.
Figure 5.2 Lateral chest radiograph.
The most likely diagnosis for this child is viral bronchio-
litis due to the infant’s history and presenting symptoms of
rhinorrhea, congestion, cough, fever, and tachypnea. Bron-
chiolitis is a leading cause of illness in the first year of life
and is often caused by respiratory syncytial virus, although
other viruses such as human metapneumovirus and rhino-
virus can be the offending pathogen. Most children tolerate
bronchiolitis and can be managed as outpatients with sup-
portive measures; however, it remains a leading cause of
hospitalization in infants.
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Infants are obligatory nose breathers and find it difficult
to clear their nasal airways without assistance. A cardinal
manifestation of bronchiolitis in infants is copious and
tenacious airway mucus.
What is the best way to increase this infant’s
oxygenation and how would you order this?
The best way to increase oxygenation in an infant with
bronchiolitis is non-invasive nasal suctioning for airway
clearance. Infants are obligate nose breathers and have
difficulty clearing their nasal airway of the copious thick
mucus of bronchiolitis without assistance. The use of
normal saline during suctioning should be considered
to facilitate airway clearance. Deeper suctioning may
be required; however, it can lead to trauma and
increased airway edema which can further occlude the
upper airways.
Guidelines suggest that supplemental oxygen does not
need to be administered unless the saturation is less than
90%. If this decision is made, there are many methods to
deliver oxygen. In some cases, non-invasive positive pres-
sure ventilation or high-flow nasal cannula may decrease
the patient’s work of breathing.
Would you consider albuterol at the same time?
Guidelines include strong recommendations against the
use of bronchodilators in infants with wheezing in the set-
ting of bronchiolitis. Given this patient has no history of
wheezing, the likelihood of improving his symptoms with
albuterol are minimal. In addition, the likelihood of side
effects including tachycardia are not insignificant.
How would you determine if the therapy
was effective?
Frequent physical examinations are recommended.
Determination of improvement is based on clinical signs
and symptoms of decreasing respiratory distress. These
include normalization of respiratory rate and heart rate,
and decreasing retractions, nasal flaring and head
bobbing
Would you obtain any other diagnostic studies?
The diagnosis of bronchiolitis is based on physical examina-
tion and history. Routine radiographic and laboratory test-
ing should not be performed. However, if there is suspicion
of another process, they should be explored in a targeted
manner. Infants under 12 weeks of age with a history of pre-
maturity, cardiopulmonary disease or immunodeficiency
concerns place the child at higher risk for increased severity

of illness and require further exploration of potential causes
of the symptoms.
The young age of this patient prompted the original chest
X-ray, showing no focal abnormalities and an expected
heart size.
Electrolyte and complete blood count testing may be con-
sidered, but usually do not provide diagnostic or manage-
ment assistance in the setting of viral bronchiolitis.
Viral testing is unlikely to assist in the clinical care and
management of the patient. However, it may provide an
explanation of the symptoms and assist with the cohorting
of patients in the inpatient setting. In the case of the pre-
senting patient, respiratory syncytial virus was detected
on the rapid respiratory panel.
Further Reading
American Academy of Pediatrics (2014). Clinical practice
guideline: The diagnosis, management, and prevention of
bronchiolitis. Pediatrics 184(5): 1474–1502.
Dean, S. and Runde, D. (2015). Bronchiodilators for
bronchiolitis. American Family Physician 92(5): 349–350.
Fleisher, G. (2006). Infectious disease emergencies. In:
Textbook of Pediatric Emergency Medicine, 5th edn
(ed. G. Fleisher, S. Ludwig and F. Henretig), 813–814.
Philadelphia, PA: Lippincott Williams & Wilkins.
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Chapter 5 Infant with Fever and Cold Symptoms 17
What other therapies would be recommended?
Ensure the infant is appropriately hydrated. Given his
recent post-tussive vomiting, insensible fluid losses in res-
piratory distress, and the likelihood that he has had a
decreased oral intake prior to presentation, an assessment
of his fluid status should be completed. Even slight dehy-
dration can lead to increased tenacity of the airway secre-
tions, potentially further occluding his airway.
Antipyretic administration may decrease the respiratory
and heart rates and help to improve the overall symptoms.
Antimicrobials are not recommended unless there is evi-
dence or a strong suspicion of a concomitant bacterial infec-
tion. Other therapies not recommended include chest
physiotherapy and systemic corticosteroids.
Louisdon, P. and Pringle, E. (2018). Diagnosis and
management of the child with acute upper and lower
airway disease. In: Pediatric Fundamental Critical
Care Support, 3rd edn (ed. M.A. Madden), 125–126.
Mount Prospect, IL: Society of Critical Care Medicine.
Norris, C., O’Neal, P., Adams, E., and Wyatt, T. (2018).
Nasal airway clearance for bronchiolitis. American
Journal of Maternal Child Nursing 43(6): 318–323.

Toddler with Pineoblastoma and Bone Marrow Transplant
Cathryn Freeman
Children’s Hospital of Michigan, Detroit, MI, USA
History of Present Illness
A 3-year-old African American male with a history of
metastatic pineoblastoma presented to clinic for scheduled
follow-up 32 days after his most recent bone marrow trans-
plant (BMT). He had been discharged from the hospital
2 weeks prior, following his second of three-tandem autol-
ogous stem cell transplants. His father reported that he felt
warm to the touch beginning that morning and appeared to
have decreased energy, although his temperature was not
measured at that time. He ate breakfast and tolerated
his morning medications without nausea or vomiting.
He denies any cough, nasal congestion, or known sick
contacts. He has been stooling twice daily, described as soft,
non-bloody, without abdominal pain. He is not toilet
trained, with no change in urine output or characteristics
reported. His family denied bruising, bleeding, headaches,
or change in mental status.
Past Medical History
He was born full term and was a previously healthy male.
He is up to date on immunizations through age 2 years. One
week after his third birthday, he presented to the
emergency department (ED) with a 3-week history of eye
crossing, decreased hand–eye coordination, altered gait,
once-weekly emesis, and regression in toileting. Computed
tomography (CT) of his head revealed obstructive
hydrocephalus and calcified mass concerning for pineo-
blastoma. He underwent a gross total resection of the pineal
lesion. Postsurgical complications included seizures, and
he was placed on levetiracetam. He currently has a
ventriculoperitoneal (VP) shunt. He received three cycles
of induction chemotherapy, followed by peripheral blood
stem cell harvest. He then went on to receive high-dose
chemotherapy with stem cell rescue.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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19
Allergies include etoposide and blood products requiring
premedication prior to platelet transfusions. Current home
medications include levetiracetam, magnesium carbonate,
potassium phosphate, sodium phosphate, polyethylene
glycol, and docusate as needed for constipation.
Past Surgical History
Gross total resection of pineal lesion, VP shunt placement,
power port placement, and apheresis catheter placement.
Family History
His maternal grandfather has hypertension and heart dis-
ease. Remainder of family history unremarkable.
Social History
He lives with his parents and 1-year-old brother. He is cared
for in the home by his parents and does not attend day care.
Current Status
On initial examination, he is sitting on his father’s lap,
appearing lethargic. He is answering questions appropri-
ately for age, at his neurologic baseline.
Vital signs are as follows: oral temperature 38.4 C
(101.4 F), heart rate 136 bpm, blood pressure 106/57 mmHg,
respiratory rate 36 breaths per minute, and oxygen
saturation 100% on room air.
There is no source of fever evident on physical examina-
tion. He has 2+ peripheral pulses, capillary refill <3 sec-
onds, and his extremities are well perfused. Standard
post-transplant laboratory studies are collected, including
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20 Cases in Pediatric Acute Care

Table 6.1 Basic metabolic profile. 3 Looking at the laboratory results, what abnormalities
require immediate attention? What additional testing
Sodium 131 mEq/L
should be performed?
Potassium 3.9 mEq/L
Chloride 98 mEq/L
Continuation of case: Within the first 24 hours the blood
cultures from both lumens of his apheresis catheter result
Carbon dioxide 25 mEq/L
positive for Enterobacter cloacae. He remains stable with
Blood urea nitrogen 6 mg/dL
persistent fever, maximum temperature 39.4 C (102.9 F).
Creatinine 0.3 mg/dL
Glucose 87 mg/dL
4 With the findings of his blood cultures and persistent
fever, should his antibiotic regimen be adjusted? When
Magnesium 2.0 mg/dL
should additional coverage be added?
Phosphorus 2.1 mg/dL
Continuation of case: The following morning his clinical
course begins to deteriorate. Vital signs reveal a blood pres-
Table 6.2 Complete blood count. sure of 62/28 mmHg, respiratory rate 38 per minute, heart
rate 142 bpm, temperature 39.6 C (103.3 F), SpO2 92% on
White blood cell count 1300/mm3 room air, and flash capillary refill. He is flushed with pete-
Hemoglobin 7 g/dL chiae to his lower extremities, and lethargic.
Hematocrit 20.3%
5 What immediate interventions are required?
Platelet count 8000/mm3
Neutrophils 30%
Eosinophils 1% Rationale and Evidence-based Practice
Monocytes 33% Explanation
Lymphocytes 31%
Bands 0% Based on the information given, what are the
Absolute neutrophil count (ANC) 400 potential differential diagnoses?
Febrile neutropenia, systemic inflammatory response syn-
a complete blood count (CBC), reticulocyte count, basic drome (SIRS), and respiratory, urinary and/or bloodstream
metabolic panel, magnesium, phosphorus, bilirubin, alka- infections are all possible differential diagnoses. A fever
line phosphatase, alanine aminotransferase, aspartate ami- may be the only sign of infection in neutropenic patients,
notransferase, lactate dehydrogenase, immunoglobulin, due to the decreased neutrophil-mediated component of
and type and screen (see Tables 6.1 and 6.2). Additionally, the inflammatory response. The Infectious Diseases Society
blood cultures from both lumens of his apheresis catheter of America defines febrile neutropenia as a single oral tem-
and Mediport are collected, and a 0.9% normal saline fluid perature of 38.3 C (101 F) or above, or a temperature of
bolus of 20 mL/kg is infused. Cefepime is initiated within 38.0 C (100.4 F) or above sustained over a 1-hour period
30 minutes of identification of fever. He is admitted to in a patient with an absolute neutrophil cell count below
the BMT unit for further monitoring. 500/mm3. SIRS is a non-specific inflammatory state that
Following admission, a sodium and phosphorus bolus is may be seen with infection, requiring the presence of two
infused to correct for his hyponatremia and hypophospha- of the following criteria: temperature >38 or <36 C
temia. He is transfused with one unit of packed red blood (>100.4 or <96.8 F), tachycardia, tachypnea, and white cell
cells and platelets after receiving premedication with aceta- count >12 000/mm3 or <4000/mm3. Bloodstream infec-
minophen and diphenhydramine. tions, specifically Gram-negative organisms, are a frequent
complication of neutropenia in oncologic patients, requir-
ing prompt evaluation and empirical antibiotic therapy.
Questions
What sources of infection must be considered
Answer the following questions using the details provided. given this patient’s medical and surgical history?
1 Based on the information given, what are the potential In immunocompromised patients, one must consider all
differential diagnoses? major systems and sources of infection, including bacterial,
2 What sources of infection must be considered given this viral, and fungal etiologies. Respiratory and urinary tract
patient’s medical and surgical history? infections may be present in asymptomatic neutropenic

patients. All implanted devices, including this patient’s
apheresis catheter, Mediport, and VP shunt, are possible
reservoirs of infection.
Looking at the laboratory results, what
abnormalities require immediate attention? What
additional testing should be performed?
This patient is anemic and thrombocytopenic requiring
transfusion of packed red blood cells and platelets to
replenish his volume, improve oxygen-carrying capacity,
and allow for improved coagulation and reduced risk of
bleeding complications. Infection results in decreased pro-
duction of red blood cells and platelets and increased plate-
let consumption. Therefore, a daily CBC is required to
monitor the need for ongoing transfusions. Peripheral
blood cultures should be considered in addition to central
line cultures as they increase the identification of bactere-
mia; however, collection should not delay the initiation of
antibiotics. Chest radiography should only be obtained in
patients who present with or develop respiratory signs
and symptoms. A clean-catch midstream urine sample
for urinalysis and urine culture should also be considered
when feasible given the patient’s toileting abilities and
clinical status.
With the findings of his blood cultures and
persistent fever, should his antibiotic regimen be
adjusted? When should additional coverage
be added?
The clinical practice guidelines recommend continuation
of the initial empiric antibacterial coverage in children
who are persistently febrile and stable. Monotherapy with
an antipseudomonal beta-lactam, fourth-generation cepha-
losporin, or a carbapenem remains the gold standard
Further Reading
Freifeld, A.G., Bow, E.J., Sepkowitz, K.A., et al. (2011). Clinical
practice guideline for the use of antimicrobial agents in
neutropenic patients with cancer: 2010 update by the
Infectious Diseases Society of America. Clinical Infectious
Diseases 52(4): e56–e93. doi: 10.1093/cid/cir073.
Lehrnbecher, T., Robinson, P., Fisher, B., et al. (2017).
Guideline for the management of fever and neutropenia in
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Chapter 6 Toddler with Pineoblastoma and Bone Marrow Transplant 21
empiric treatment for pediatric febrile neutropenia.
A second antibiotic to cover Gram-negative organisms or
a glycopeptide should be added when a patient becomes
clinically unstable, in suspected resistant Gram-negative,
Gram-positive and anaerobic bacterial infection, or in hos-
pitals with a high rate of resistant organisms.
What immediate interventions are required?
It is evident that this child is now developing sepsis from
Gram-negative bacteremia. Prompt recognition and inter-
vention is critical to prevent the progression of sepsis to
shock. Support the child’s airway, oxygenation, and venti-
lation, and continue to monitor vital signs. Rapid fluid
resuscitation with isotonic crystalloid to restore the intra-
vascular volume and improve tissue perfusion is the first
step in correcting these findings. Reevaluate the patient’s
physiologic response after each 20 mL/kg bolus to deter-
mine the need for further fluid boluses and assess for pul-
monary edema as a consequence of resuscitation.
Following the Pediatric Advanced Life Support algorithm,
if signs of shock persist including warm extremities, flash
capillary refill, and/or low blood pressure, initiation and
titration of norepinephrine is required. Norepinephrine is
the vasopressor of choice for fluid-refractory septic shock
due to its potent α-adrenergic vasoconstricting effects.
Case Resolution
The patient required transfer to the pediatric intensive care
unit for management of bacteremia and support with high-
flow nasal cannula and a vasopressor. His clinical state
improved after 48 hours and he was transferred back to
the BMT unit a few days later to continue his therapy.
children with cancer and hematopoietic stem cell
transplantation recipients: 2017 update. Journal of Clinical
Oncology 35(18): 2082–2094.
Prusakowski, M.K. and Chen, A.P. (2017). Pediatric sepsis.
Emergency Medicine Clinics of North America 35(1):
123–138.

A 5-Year-Old with Altered Mental Status
Yu-shan Tseng and Richard Garcia
Children’s Hospital of Michigan, Wayne State University, Detroit, MI, USA
A 5-year-old is evaluated in the emergency department
(ED) for decreased activity and lethargy. The child was
found lying on the floor of the grandparents’ home with
emesis, and appeared to have altered mental status with
abnormal respiration. After initial resuscitation in the
ED, she was admitted to the pediatric intensive care unit
(PICU) with persistent hypotension and bradycardia
despite several fluid boluses and initiation of inotropic
support.
History of Present Illness
This child was previously healthy, with no history of past
medical conditions. As per grandparents she arrived to
their home 2 days ago and was acting fine until they found
her altered on the floor today in the early afternoon.
In the ED, the patient was found to have heart rate of
60 bpm, blood pressure 75/45 mmHg, and glucose 272
mg/dL. After becoming progressively obtunded, the child
was intubated for airway protection. She received multiple
fluid boluses and calcium, and was started on inotropic sup-
port, which quickly escalated to three vasopressors (norep-
inephrine, epinephrine, and phenylephrine) at high doses
for persistent hypotension.
Past Medical History
Non-significant past medical history.
Past Surgical History
None.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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23
Social History
Parents are divorced. The patient frequently stays at the
grandparents’ house for care and attends school and
kindergarten.
Current Status
Patient arrived in the intensive care unit intubated and
sedated. She is bradycardic and hypotensive, with cool
extremities and delayed capillary refill. A basic metabolic
panel indicates an elevated creatinine of 1.75 mg/dL and
hyperglycemia with glucose of 312 mg/dL. Arterial blood
gas analysis indicates metabolic acidosis with a serum lac-
tate of 11.5 mg/dL.
Initial laboratory results are shown in Table 7.1. The
electrocardiogram (EKG) and chest X-ray are shown in
Figures 7.1 and 7.2, respectively. Chest X-ray showed mild
vascular congestion bilaterally with normal heart silhouette.
Questions
Answer the following questions using the details provided.
1 Based on the data provided, what is the most likely diag-
nosis for this child?
2 Would you obtain any other diagnostic studies?
Continuation of case: The echocardiogram reveals nor-
mal four-chamber intracardiac anatomy with mildly
depressed function bilaterally. There is no pericardial effu-
sion. The patient is emergently placed on veno-arterial
extracorporeal membrane oxygenation (ECMO) due to per-
sistent hemodynamic instability, and to prevent further
end-organ damage.
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24 Cases in Pediatric Acute Care

Table 7.1 Basic metabolic panel (BMP) and arterial blood Would you obtain any other diagnostic studies?
gas (ABG) analysis.
In addition to the basic laboratory tests obtained in this
BMP case, one should also obtain serum and urine drug screen-
Sodium 147 mEq/L ing tests, since multiple drug ingestions are relatively com-
Potassium 3.2 mEq/L mon. Obtaining an EKG is a must as it gives valuable
Chloride 109 mEq/L information such as conduction abnormalities leading to
several degrees of atrioventricular block and sinus node
Bicarbonate 14 mEq/L
dysfunction. An echocardiogram should be obtained to
Total calcium 8.5 mg/dL
investigate for pericardial effusion and myocardial dysfunc-
Blood urea nitrogen 15 mg/dL
tion, and evaluate for congenital pathology. Furthermore, a
Creatinine 1.75 mg/dL chest X-ray can explore for pulmonary edema, a common
Glucose 312 mg/dL occurrence in CCB toxicity or help to demonstrate other
Ionized calcium 1.12 mmol/L lung pathology.
Lactate 11.5 mg/dL At the cellular level, CCBs almost exclusively block the
L-type voltage-gated calcium channel located on the sur-
ABG
face membrane. The primary consequences are depressed
inotropic and negative chronotropic effects, and peripheral
pH 7.28
vasodilation. The depressed inotropy and peripheral vaso-
PCO2 27 mmHg
dilation are mediated by interfering with phase 2 of the
PO2 162 mmHg action potential in myocytes and vascular smooth muscle,
Bicarbonate 12.6 mEq/L which involves the slow influx of extracellular calcium via
Base excess –12.5 the L-type calcium channels. Specifically blocking the
L-type calcium channels stops the release of calcium from
the intracellular sarcoplasmic reticulum, a process known
3 What other therapies would be recommended at as calcium-induced calcium release, which is essential for
this time? muscle contraction. Moreover, the L-type calcium channels
4 What are the signs and symptoms of calcium channel are responsible for impulse generation and conduction by
blocker toxicity? allowing the slow influx of calcium during phase 4 of the
5 Why does the patient have persistent hemodynamic action potential in pacemaker cells located in the sinoatrial
instability despite multiple inotropes and fluid boluses? and atrioventricular nodes, which creates a negative chron-
6 What other therapies would be recommended in a case otropic effect.
of calcium channel blocker poisoning? Is there benefit CCBs can be divided into two major categories. Dihy-
to the use of hemodialysis? dropyridines, such as nifedipine and amlodipine, prefer-
entially block vascular smooth muscle L-type calcium
channels, producing potent vasodilatory effects. Non-
dihydropyridines, such as verapamil and diltiazem,
Rationale and Evidence-based Practice
selectively inhibit L-type calcium channels in the myo-
Explanation cardium, therefore causing delayed conduction and
depressed myocardial contractility. As dihydropyridines
Based on the data provided, what is the most likely
generally produce peripheral vasodilation and have less
diagnosis for this child?
influence on cardiac chronotropy and inotropy, reflex
During the discussion when the child was admitted, it was sinus tachycardia can be seen during vasodilatory shock.
noted that the grandmother’s purse had been found close to However, this CCB selectivity can be lost in cases of
the child on the floor and her medication bottles were large overdose. Although calcium is also critical for skel-
opened. Grandmother is taking diltiazem and amlodipine, etal muscle contraction, CCBs primarily affect cardiac
both calcium channel blockers (CCBs). Given this history and vascular smooth muscle.
of missing pills from the grandmother’s purse and the In addition, insulin secretion by pancreatic beta cells is
patient’s presentation with bradycardia, hypotension, poor gated by L-type calcium channels on the cell membrane.
perfusion, metabolic acidosis, acute kidney injury, and By blocking these L-type channels, CCBs prevent the
hyperglycemia, the most likely diagnosis would be CCB- release of insulin, inducing insulin resistance that results
induced shock. in a hyperglycemic state.

Figure 7.1 12-lead EKG.
Figure 7.2 Chest radiograph.
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Chapter 7 A 5-Year-Old with Altered Mental Status 25
What other therapies would be recommended
at this time?
While on ECMO support, the patient was started on hyper-
insulinemic euglycemia (HIE) therapy and calcium drip.
Her inotropes were weaned significantly. Within the next
couple of days, acidosis resolved and urine output improved
with down-trending creatinine. Her epinephrine, calcium,
and high insulin infusions were weaned off and her EKG
normalized to sinus rhythm. She was decannulated suc-
cessfully on day four of admission and extubated soon after.
What are the signs and symptoms of calcium
channel blocker toxicity?
The severity of CCB ingestion varies greatly; most children
are asymptomatic after accidental exposure. However, case
reports of cardiac arrests and deaths after one to two tablets
have been documented in the literature. Significant CCB
toxicity manifests in cardiovascular instabilities including

26 Cases in Pediatric Acute Care
hypotension, bradycardia, and arrhythmias from conduc-
tion abnormalities. Patients may also present with symp-
toms of end-organ hypoperfusion such as dizziness,
syncope, altered mental status, seizures, cerebrovascular
ischemia, acute kidney injury, shock liver, nausea and vom-
iting, bowel ischemia, and metabolic acidosis. It is notewor-
thy to mention that both CCBs and beta-blockers present
with the detrimental combination of bradycardia and hypo-
tension; however, the presence of hyperglycemia in CCB
intoxication helps to distinguish it from beta-blocker inges-
tion, which generally presents with hypoglycemia.
Finally, acute pulmonary edema can occur following
CCB ingestion. Pulmonary edema can be secondary to
heart failure, but non-cardiogenic pulmonary edema has
also been reported. The mechanism behind it is yet to be
discovered; currently, it is thought to be secondary to selec-
tive precapillary vasodilation and aggressive fluid
resuscitation.
Why does the patient have persistent
hemodynamic instability despite multiple
inotropes and fluid boluses?
Because CCBs act at a cellular level, the cardiogenic and
vasodilatory shock seen in CCB toxicity are often refractory
to initial resuscitation with isotonic fluid boluses and intra-
venous vasoactive medications. It is preferred to limit fluid
boluses to 60 mL/kg as patients are usually euvolemic in
CCB ingestion, and aggressive fluid resuscitation carries
the risk of acute pulmonary edema. Also, bradycardia tends
to be refractory to atropine administration because ulti-
mately the effects of atropine are mediated at the cellular
level by the L-type calcium channels.
What other therapies would be recommended in a
case of calcium channel blocker poisoning? Is
there benefit to the use of hemodialysis?
Asymptomatic patients need to be monitored using telem-
etry; patients who ingest immediate-release formulations
should be observed for at least 6–8 hours whereas those
who ingest sustained-release formulations should be mon-
itored for at least 24 hours. Timely gastrointestinal decon-
tamination with activated charcoal is recommended in all
patients with CCB ingestion with active bowel sounds,
since activated charcoal adheres well to CCBs and
decreases their absorption. Given a secure airway, activated
charcoal should be administered within 1–2 hours of inges-
tion of a short-acting CCB. In cases when an extended-
release drug is the culprit, it can be done up to 4–6 hours
after a documented ingestion. Furthermore, whole bowel
irrigation with polyethylene glycol can be considered in
patients with clear mentation when extended-release
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preparations are involved, particularly if the drug is vera-
pamil or diltiazem. If the patient becomes symptomatic,
urgent admission to an intensive care unit is warranted.
There is no unique management algorithm in children
with CCB-induced shock refractory to conventional medi-
cal therapy. Timely consultation with the institution toxi-
cologist or regional poison control center is prudent.
Different treatments with possible benefits have been used
in CCB toxicity, including continuous intravenous calcium
infusion, intravenous HIE therapy, glucagon therapy, lipid
emulsion therapy, cardiac pacing, and veno-arterial ECMO
support. Frequently, these therapies are provided simulta-
neously or in rapid succession since patients can rapidly
deteriorate to cardiac arrest and death.
Intravenous calcium is used to counteract the negative
cardiovascular effects of CCBs. Calcium chloride is pre-
ferred to calcium gluconate because of the higher potency.
Calcium chloride should be administered through central
venous access, if possible, to avoid calcium extravasation
and skin necrosis. Intravenous calcium boluses can be
repeated every 10–20 minutes or followed by an infusion.
HIE therapy has shown promising results in case reports
and animal models. CCBs appear to disrupt myocardial
fatty acid metabolism and switch it to carbohydrate metab-
olism, but at the same time CCBs create a relative insulin
resistance within the myocardium by preventing the
release of insulin from the pancreatic beta cells. Insulin
at high doses (1–10 units/kg per hour) has positive ino-
tropic properties and assists in myocardial uptake of
glucose, mitigating the myocardial depression seen in
patients with CCB toxicity. Electrolytes including potas-
sium and glucose should be measured frequently, at least
every 15–30 minutes initially until the levels are stable.
Titrate dextrose infusions and give potassium supplementa-
tions as necessary. As CCB overdose often results in
hyperglycemia, patients with hyperglycemia refractory to
high-dose insulin may not require supplemental dextrose
infusion.
Glucagon activates adenylate cyclase and increases the
production of cyclic AMP, the intracellular second messen-
ger downstream of β-adrenergic receptors, and hence has
inotropic, chronotropic, and dromotropic effects. However,
it offers little advantage beyond the use of common vasopres-
sors. Lipid emulsion therapy has been used to treat poison-
ings involving lipophilic medications in recent years, and
preliminary data have demonstrated some benefits in CCB
intoxication. Although the mechanism behind lipid emul-
sion therapy remains unclear, it is theorized that lipid emul-
sion acts as a “lipid sink” by sequestering the lipophilic
drugs, reducing the amount of available free drug in the
intravascular compartment. Another proposed mechanism
is that by increasing fatty acid availability, lipid emulsion

may reverse the CCB-induced lipid to glucose metabolism in
the myocardium. Temporary transesophageal or intrave-
nous pacing can be attempted to counter bradycardia. How-
ever, it is difficult to propagate the depolarization current
through the blocked L-type calcium channels by activating
the myocardial action potential artificially.
Healthcare providers should be aware of the high mor-
bidity and mortality of CCB toxicity. Veno-arterial ECMO
should be initiated immediately if a patient presents with
shock refractory to pharmacotherapy. Veno-arterial ECMO
placement should be performed before end-organ dysfunc-
tion develops and generally support is only needed for a few
days while the drug is metabolized and excreted.
Most calcium channel blockers are highly protein
bound and have a large volume of distribution; therefore
Further Reading
Arroyo, A.M. and Kao, L.W. (2009). Calcium channel blocker
toxicity. Pediatric Emergency Care 25(8): 532–538; quiz
539–540. doi: 10.1097/PEC.0b013e3181b0a504.
Krentz, J.R. and Kaakeh, Y. (2018). An overview of
hyperinsulinemic–euglycemic therapy in calcium
channel and beta blocker overdose. Pharmacotherapy
38(11): 1130–1142. doi: 10.1002/phar.2177.
Reuter-Rice, K.E. and Peterson, B. (2016). Conventional and
unconventional lifesaving therapies in an adolescent with
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Chapter 7 A 5-Year-Old with Altered Mental Status 27
dialysis is typically ineffective in removing a significant
amount of the drug.
Case Resolution
While on ECMO support, she was started on hyperinsulinemia-
euglycemia (HIE) therapy and calcium drip. Her ino-
tropes were weaned significantly. Within the next couple
days, acidosis resolved and urine output improved with
down trending creatinine. Her epinephrine, calcium,
and high insulin infusions were weaned off and her elec-
trocardiogram normalized to sinus rhythm. She was
decannulated successfully on day four of admission and
extubated soon after.
amlodipine ingestion. Critical Care Nurse 36(4): 64–69. doi:
10.4037/ccn2016524.
St-Onge, M., Anseeuw, K., Cantrell, F.L., et al. (2017).
Experts consensus recommendations for the management
of calcium channel blocker poisoning in adults. Critical Care
Medicine 45(3): e306–e315. doi: 10.1097/
CCM.0000000000002087.

An 8-Year-Old with Abdominal Trauma
Julie Resler
Riley Hospital for Children, Indianapolis, IN, USA
An 8-year-old girl is admitted to the pediatric intensive care
unit (PICU) on the trauma service for a grade IV liver
laceration.
History of Present Illness
The child was an improperly restrained (lap belt only)
front seat passenger involved in a head-on motor vehicle
collision at 55 m.p.h. There was no known loss of con-
sciousness and she was able to extricate herself from the
vehicle. She had a noted seat belt abrasion and abdominal
pain with emesis at the scene.
Past Medical History
The patient was born at term and discharged home with the
mother. She is in the 75th percentile for height and weight.
She is up to date on vaccinations and did not receive the
seasonal flu vaccine. Home medications include acetamin-
ophen as needed for pain and fever.
Past Surgical History
No previous surgical procedures.
Family History
The patient’s parents, mother and father are both healthy.
Father is currently incarcerated. The child has a 5-year-old
and 2-year-old siblings, both reported healthy.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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29
Current Status
On arrival in the emergency department (ED), the primary
survey reveals an intact airway, breath sounds clear and equal
bilaterally, strong rapid pulse in arms and legs, Glasgow
Coma Scale (GCS) score 15, and body warm with clothing
in place. She is in a cervical collar and on a spine board with
total spine precautions in place. Initial survey reveals tem-
perature of 37.2 C (99 F), heart rate 144 bpm, respiratory rate
22/minute, oxygen saturation 98% on room air, and blood
pressure 76/40 mmHg. Peripheral intravenous access is
placed and complete blood count (CBC), comprehensive met-
abolic profile, amylase, and lipase are obtained with values as
noted in Tables 8.1 and 8.2. A 20 mL/kg fluid bolus of normal
saline is given with little response in blood pressure. A second
20 mL/kg fluid bolus of normal saline is given with similar
response. CBC reveals a hemoglobin of 7 g/dL and hematocrit
of 21%. A transfusion of 10 mL/kg of packed red blood cells
is given to support blood pressure, which increases to 96/
50 mmHg. Secondary survey reveals pain to palpation in right
upper quadrant of the abdomen and pain with inspiration.
Chest radiograph indicates right 10th, 11th, and 12th rib
fractures. Radiographs of the pelvis are negative. Computed
tomography (CT) of the abdomen and pelvis with intrave-
nous contrast is obtained and notes a grade IV liver laceration.
CT imaging is shown in Figure 8.1. The child is admitted to
the PICU on the trauma service. She is on bedrest and is
nil by mouth with intravenous fluids running at a mainte-
nance rate. Serial hemoglobin and hematocrits are ordered.
Questions
Answer the following questions using the details provided.
1 Why are young children at greater risk for serious liver,
spleen or kidney injury from blunt abdominal trauma?
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30 Cases in Pediatric Acute Care

Table 8.1 Complete blood count.

White blood cell count 10 300/mm3

Hemoglobin 7 g/dL
Hematocrit 21.1%
Platelet count 350 000/mm3
Neutrophils 41%
Lymphocytes 40%
Monocytes 0.5%
Eosinophils 4%

Table 8.2 Complete metabolic panel, including amylase

and lipase.

Sodium 138 mEq/L

Potassium 3.9 mEq/L
Chloride 108 mEq/L
Carbon dioxide 14 mEq/L
Figure 8.1 Computed tomography of abdomen/pelvis.
Blood urea nitrogen 11 mg/dL
Creatinine 0.4 mg/dL
Glucose 108 mg/dL larger-size organs, less overlying fat, and weaker abdominal
muscles. The liver has a dual blood supply via the hepatic
Calcium 9.5 mg/dL
arteries and portal vein, which adds to the possibility of
Albumin 3.0 mg/dL
rapid blood loss if these vessels are damaged along with
Total protein 6.0 g/dL damage to the parenchyma of the liver.
Aspartate aminotransferase 480 U/L
Alanine aminotransferase 385 U/L
Alkaline phosphatase 133 U/L What is the best treatment plan for this child?
Total bilirubin 0.5 mg/dL Many blunt abdominal organ injuries can be treated non-
Amylase 38 U/L operatively, including involvement of the liver. Pediatric
Lipase 35 U/L facilities have protocols for length of bedrest, frequency
of lab draws, and length of activity restriction. This allows
for stabilization of the laceration/hematoma and reabsorp-
2 What is the best treatment plan for this child? tion of the blood. However, the newest guidelines, pub-
3 What signs would prompt escalation of interventions lished by the Pediatric Trauma Society, utilize ATOMAC
for this child? (Arizona–Texas–Oklahoma–Memphis–Arkansas Consor-
4 What additional imaging would be helpful to diagnose tium), which consists of a group of Level I pediatric trauma
complications? centers across the United States who have participated in
research to improve outcomes for children who suffer
trauma injuries. This publication from 2015 offers a prac-
Rationale and Evidence-based Practice
tice guideline with four highlights. Guidelines include
Explanation managing liver and spleen trauma based on hemodynamic
stability versus grade of injury, a period of as little as
Why are young children at greater risk for serious
24 hours bedrest, and limiting transfusions to those who
liver, spleen or kidney injury from blunt abdominal
have hemoglobin levels of less than 7 g/dL. Management
trauma?
includes urgent surgery or embolization or continued
Liver, spleen, and kidney injuries resulting from blunt non-surgical intervention. In 2015, PECARN (Pediatric
trauma occur more frequently in children than adults pri- Emergency Care Applied Research Network) published
marily because of the physical features of the child, which data in regard to management of liver trauma and found
include a protuberant abdomen, more pliable ribcage and that only 5% of liver injuries required surgical intervention

or embolization and 11% required blood transfusion, leav-
ing the majority of patients on minimal therapies. There
continues to be some debate about overall hospitalization
time, length of bedrest, and recurring blood work, despite
published guidelines. For this patient, serial abdominal
examinations and serial checks of hemoglobin and hemat-
ocrit until stabilized will help guide treatment. A diet can be
started and advanced as tolerated. Follow-up with the
trauma surgeon may be necessary if the child is or becomes
hemodynamically unstable.
What signs would prompt escalation of
interventions for this child?
Serial abdominal examinations will provide insight into
subtle changes in the patient’s condition. Location of the
liver laceration may also raise suspicion for the possibility
of developing a bile leak. Fever, tachycardia, tachypnea,
Further Reading
Linnaus, M.E., Langlais, C.S., Garcia, N.M., et al. (2017).
Failure of nonoperative management of pediatric blunt liver
and spleen injuries. Journal of Trauma and Acute Care
Surgery 82(4): 672–679.
Notrica, D.M., Eubanks, J.W., Tuggle, D.W. et al. (2015).
Nonoperative management of blunt liver and spleen injury
in children: evaluation of the ATOMAC guideline using
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Chapter 8 An 8-Year-Old with Abdominal Trauma 31
change in abdominal examination, and decrease in appetite
are all signs warranting further investigation.
What additional imaging would be helpful
to diagnose complications?
Many pediatric trauma centers attempt to limit radiation in
pediatric patients due to the lifetime increase in cancer risk
with ongoing exposure. However, if there is suspicion for
development of a bile leak, repeat CT imaging may be nec-
essary for diagnosis. Endoscopic retrograde cholangiopan-
creatography (ERCP) is a diagnostic tool that may not be
available at all treatment centers. It allows the practitioner
to directly identify duct transection/injury and stent or per-
form a sphincterotomy. Last, fluoroscopy and interven-
tional radiology facilitates drain placement in order to
divert fluid and pressure away from the site of injury.
The patient may require follow-up imaging to assess for res-
olution of the injury.
GRADE. Journal of Trauma and Acute Care Surgery 79(4):
683–693.
Wisner, D.H., Kuppermann, N., Cooper, A., et al. (2015).
Management of children with solid organ injuries after
blunt torso trauma. Journal of Trauma and Acute Care
Surgery 79(2): 206–214.

Child with Left Eye Swelling
Amy Graf
Norton Children’s Hospital, Louisville, KY, USA
A 3-year-old female presents to the emergency department
(ED) for evaluation of left eye swelling.
History of Present Illness
A 3-year-old female developed symptoms of rhinosinusitis
4 days ago. On day 3 of illness, she developed left upper eye-
lid erythema, edema, eye pain, and diplopia that has
become progressively worse over the past 24 hours.
Past Medical History
She has trisomy 21 and was born at 37 weeks’ gestation. She
spent 6 weeks in the neonatal intensive care unit (NICU).
She has pulmonary hypertension, requiring 1.5 L/min of
oxygen continuously per nasal cannula. She also has a
history of gastroesophageal reflux, hypothyroidism, and
hypotonia. Her immunizations are up to date for age,
including influenza vaccine.
Past Surgical History
She has had a G-tube placement, Nissen fundoplication,
closure of patent ductus arteriosus and patent foramen
ovale, multiple cardiac catheterizations, and tonsillectomy
and adenoidectomy for obstructive sleep apnea.
Family History
Her mother has hypothyroidism and her father has a
history of hypertension. Her older brother was diagnosed
with reactive airway disease at age 4 years.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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33
Current Status
On arrival at the ED, she is febrile to 38.9 C (102.1 F), heart
rate is 115 bpm, and oxygen saturation 94% on 1.5 L/min of
oxygen per nasal cannula. She appears relatively well,
except for rhinorrhea and guarding her left eye. She has
periorbital edema and is unable to open her eye
(Figure 9.1). On examination, her pupils are equal round
and reactive to light and she has conjunctival chemosis,
but it is not possible to assess extraocular movements due
to lack of cooperation.
Questions
Answer the following questions using the details provided.
1 What are the differential diagnoses and the most likely
diagnosis for this child?
2 What diagnostic tests are recommended to assist in the
diagnosis of this eye condition?
3 What specialists may be consulted to manage this left
eye condition?
4 What are the most common etiologies of eye swelling?
5 What is the most appropriate treatment for this
condition?
Rationale and Evidence-based Practice
Explanation
What are the differential diagnoses and the most
likely diagnosis for this child?
Possible diagnoses for this condition are broad and
include preseptal cellulitis, orbital cellulitis, allergic res-
ponse, and tumors of the eye including neuroblastoma,

34 Cases in Pediatric Acute Care
Figure 9.1 Periorbital edema. Source: Courtesy of Robin
Scarbrough.
retinoblastoma, rhabdomyosarcoma and orbital pseudotu-
mor. Additionally, conjunctivitis, cavernous sinus throm-
bosis, granulomatosis with polyangiitis of the orbit
(Wegner disease), Graves disease, viral infections including
herpes simplex or varicella zoster, periocular dermoid cyst,
posterior scleritis, sinus mucocele with superimposed infec-
tion, thrombosed orbital varix, and tuberculosis of the orbit
can all be included on the list of differential diagnoses.
Both a preseptal cellulitis and orbital cellulitis present
with similar examination findings, including eyelid edema,
erythema, and ocular pain. However, orbital cellulitis must
be differentiated from preseptal cellulitis as orbital cellulitis
may result in catastrophic outcomes. Orbital cellulitis may
cause abscesses of the brain, orbit or subperiosteal area,
vision loss or even death. Clinical manifestations of orbital
cellulitis include fever, leukocytosis with a neutrophil
predominance, pain with eye movements, and proptosis.
Chemosis and opthalmoplegia with diplopia are more com-
monly seen in orbital cellulitis than in preseptal cellulitis.
The primary distinction is that preseptal cellulitis causes
inflammation only in the eyelids, whereas orbital cellulitis
causes extraocular muscle inflammation, fat stranding,
and anterior displacement of the globe. The leading diagno-
sis for this patient is orbital cellulitis, given the examination
findings of erythema, edema, pain, and chemosis.
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Orbital cellulitis is an infection involving the orbital con-
tents including the ocular muscles and fat. It is an infection
that is more frequently seen in young children rather than
older children and adults. Rhinosinusitis is commonly the
source of orbital cellulitis as 86–98% of cases have a coexist-
ing rhinosinusitis. The most common types of sinusitis that
lead to an orbital cellulitis are pansinusitis and ethmoid
sinusitis.
What diagnostic tests are recommended to assist
in the diagnosis of this eye condition?
Computed tomography (CT) of the orbits with intravenous
contrast and magnetic resonance imaging (MRI) with intra-
venous contrast are beneficial studies in diagnosing orbital
cellulitis. However, CT scan of the orbits with intravenous
contrast is frequently the initial diagnostic study of choice
due to availability of the test and duration of the study. MRI
is superior to CT of the orbits when evaluating progression
of soft tissue disease. The Infectious Disease Society of
America recommends laboratory studies including a com-
plete blood count (CBC) with differential, blood cultures
and a C-reactive protein (CRP) in patients with soft tissue
infections who have signs and symptoms of systemic
toxicity (see Tables 9.1 and 9.2).
CT of the orbits with intravenous contrast shows left peri-
orbital and orbital cellulitis with small early abscess medi-
ally and myositis of medial rectus muscle. The CT also notes
sinus disease in the left ethmoid and left maxillary sinuses,
as well as left otomastoid disease (Figure 9.2).
What specialists may be consulted to manage the
eye condition?
A pediatric ophthalmologist is consulted to evaluate visual
acuity and extraocular eye movements. An otolaryngologist
is consulted if surgery is necessary to drain the abscess.
Table 9.1 Complete blood count.
White blood cell count 9290/mm3
Hemoglobin 11.2 g/dL
Hematocrit 32.6%
Platelet count 202 000/mm3
Neutrophils 75%
Eosinophils 2%
Monocytes 4%
Lymphocytes 18.3%
Bands 0%
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Chapter 9 Child with Left Eye Swelling 35

Table 9.2 Additional results.

Erythrocyte sedimentation rate (ESR) 49 mm/hour

C-reactive protein (CRP) 3.3 mg/dL
Blood culture Pending

What are the most common etiologies of eye

swelling?
Staphylococcus aureus and streptococci are the most com-
monly identified organisms in bacterial orbital cellulitis.
Haemophilus influenzae was previously a common patho-
gen but incidence has declined due to immunization with
the Hib vaccine. Orbital cellulitis may also be polymicrobial
and, rarely, fungal infections such as Mucorales and Asper-
gillus may cause orbital cellulitis in immunocompro-
mised hosts.

What is the most appropriate treatment for this

condition? Figure 9.2 Orbital CT scan. Source: Courtesy of Dr. Karen Moeller.

Antibiotics are necessary. Initial therapy often includes

vancomycin and a second- or third-generation cephalospo- recommended dose of antibiotics is warranted. If positive
rin or piperacillin-tazobactam. If intracranial extension of cultures are obtained, antibiotics should be tailored to
the orbital cellulitis is a concern, then use of the highest identified organism.

Further Reading
Bedwell, J. and Bauman, N. (2011). Management of pediatric
orbital cellulitis and abscess. Current Opinion in
Otolaryngology and Head and Neck Surgery 19(6): 467–473.
Infectious Disease Society of America (2014). Practice
guidelines for the diagnosis and management of skin and
soft tissue infections: 2014 update by the Infectious Disease
Society of America. Clinical Infectious Disease 59(2):
e10–e52.
Upile, N., Munir, N., Leong, S., and Swift, A. (2012). Who
should manage acute periorbital cellulitis in children?
International Journal of Pediatric Otorhinolaryngology 78(8):
1073–1077.
Wong, S. and Levi, J. (2018). Management of pediatric orbital
cellulitis: a systematic review. International Journal of
Pediatric Otorhinolaryngology 110: 123–129.

10
Billing and Coding
Dawn M. Silverman
University of Maryland Children’s Hospital, University of Maryland Medical Center, Baltimore, MD, USA
Billing for healthcare services is an imperative part of
learning the provider role in any setting and it is important
to have proficiency in billing practices to ensure reimburse-
ment. Key information related to reimbursement, billing,
and coding for pediatric outpatient and inpatient services
are discussed in this chapter to provide an overview of
professional billing practices for the acute care provider.
In addition, critical care criteria are defined with a case
presentation to provide an example of critical care billing.
There are many providers who are able to bill for services,
but this chapter focuses on the the acute care nurse
practitioner.
Questions
Please answer the following questions.
1 As a new provider for the inpatient cardiology service,
what steps are required to be a billable provider?
2 Once a pediatric nurse practitioner (PNP) is creden-
tialed, how does a PNP’s work generate a charge?
3 What documentation elements are required for billing?
4 What codes are used to bill for services?
Rationale and Evidence-based
Practice Explanation
As a new provider for the inpatient cardiology
service, what steps are required to be a
billable provider?
The Centers for Medicare and Medicaid Services (CMS)
requires a PNP to be a graduate of an advanced practice
nursing program, certified by a recognized national certify-
ing body, and licensed as a registered professional nurse
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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37
in the state where the delivery of care is performed
(Wound, Ostomy, and Continence Nurses Society, 2012).
Additionally, services must be physician services, medically
necessary, and within the scope of practice of the PNP
(Munro, 2013).
The CMS also issues National Provider Identifier
(NPI) numbers to providers of all disciplines. This unique
10-digit number identifies the professional as a healthcare
provider. It is often used when prescribing medications
and always used for billing medical services. Some group
practices also hold an NPI number. Individual contracts
will determine whether the PNP’s services are billed under
an individual or group NPI number (Wound, Ostomy, and
Continence Nurses Society, 2012). PNPs can apply for
an NPI number online at https://nppes.cms.hhs.gov/#/.
Once a PNP is licensed, board certified, and has obtained
an NPI, the organization for whom the PNP is employed
will complete credentialing and privileging. Documents
demonstrating evidence of a diploma, state license, board
certification, NPI, Drug Enforcement Administration
license, and state-issued controlled dangerous substance
license must be shared with the credentialing body. Curric-
ulum vitae and professional references are also required.
Privileges are established and granted to the PNP based
on role descriptions and state-based scope of practice
delineations.
Providers may also be required to enroll with third-party
payers. The healthcare organization and associated billing
entity will facilitate this process. It is important to note
that while PNPs are typically able to claim 85–100% of a
physician’s usual and customary charge, PNPs are not
recognized as credentialed providers by all insurers. The
recognition of a PNP as a credentialed provider is based
on individual states’ practice authorities and agreements
with insurers. Current federal laws and regulations do not
require private insurers to credential or reimburse PNPs.

38 Cases in Pediatric Acute Care
Once the PNP is credentialed, how does a PNP’s
work generate a charge?
Once the PNP is ready to practice and is credentialed and
granted hospital privileges, it is helpful to understand the
revenue cycle by which services are billed. The cycle is
initiated by services rendered. As previously mentioned,
these services must be services that a physician would typ-
ically provide and within the scope of practice of the PNP.
The PNP will engage in an encounter with a patient, pro-
vide services, and then complete documentation. In most
cases, documentation is entered into an electronic medical
record (EMR) or health record. Standardized note tem-
plates assist the provider with the flow and content required
for documentation and billing.
Some healthcare organizations require providers to
assign codes to their documentation; however, in many
cases, professional medical coders review documentation
and assign appropriate current procedural terminology
(CPT) codes and diagnosis codes. The US Department of
Health and Human Services designates CPT codes, which
are the most widely accepted medical nomenclature for
procedures and services. The World Health Organization
••
compiles the International Statistical Classification of Dis-
eases and Related Health Problems (ICD) codes. The ICD
codes are assigned based on the patient’s diagnoses. To
ensure accurate and appropriate coding of services and
diagnoses, coders may send queries to providers with ques-
tions or requests for more detail or additional information
necessary to establish a claim.
Professional medical billers assign fees based on the
documented codes and agreements with insurers. Billers
enter the codes into billing software and report claims to
insurers, who in turn generate an explanation of benefits.
The claim is either paid or denied. Denials can be resub-
mitted with explanation; however, there are fees associated
with denial challenges so it is important for providers to
supply as much detail and evidence of medical necessity
in the documentation.
What documentation elements are required
for billing?
The adage “If it is not documented, it didn’t happen” is par-
ticularly relevant when it comes to billing. Documentation
must not only identify the patient, the setting, and the pro-
vider, it must also contain sufficient detail in the assess-
ment, diagnosis, and plan of care to meet standards for
billing and medical necessity. Concise and detailed docu-
mentation leads to better patient care and outcomes. Doc-
umentation in an EMR is a communication tool. It provides
the author and subsequent readers with a chronological
record of the health and disease of a patient so that
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providers can deliver immediate and appropriate treatment
and track a patient’s health over time (https://www.cms.
gov/). Of course, it is also important to reimbursement.
What codes are used to bill for services?
Evaluation and management (E/M) documentation is the
foundation of medical documentation. It is employed in
the ambulatory and inpatient setting and is appropriate
for preventive care, primary care, and critical care. The
CPT codes assigned to documentation are based on the
patient type (new or established) or encounter type (initial
or subsequent), the setting of service (outpatient, inpatient,
emergency department, nursing facility, etc.), and level of
complexity. The complexity is based on the depth and detail
of history taking, the physical examination, and medical
decision-making (https://www.cms.gov/). While no dis-
tinct number of minutes is associated with each level of
E/M, there should be a correlation between the level of
E/M and the time spent with the patient.
Seven elements are used to define the level of E/M
services:
history
physical examination
••
medical decision-making
counseling
••
coordination of care
nature of presenting problem
•
time associated with delivery.
History, physical examination, and medical decision-
making are the most important elements when designating
the level of E/M (Table 10.1) (https://www.cms.gov/).
Four types of history are utilized for E/M documentation.
These histories are problem focused, expanded, detailed,
and comprehensive. Table 10.2 shows the required compo-
nents for each type of history.
Outpatient Case Documentation
Example
A 10-year-old male presents to the clinic with complaints of
runny nose, itchy eyes, and sneezing. Example of level 3
E/M documentation (CPT 99203) for this patient follows.
Patient
James Doe, date of birth 1 January 2010, medical record
number (MRN) xxxxx0123.
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Chapter 10 Billing and Coding 39

Table 10.1 Level of coding by patient type and complexity.

Service CPT History and physical Medical decision-making Average time in minutes

New patient: outpatient 99201 Problem focused Straightforward 10

99202 Expanded Straightforward 20
99203 Detailed Low complexity 30
99204 Comprehensive Moderate complexity 45
99205 Comprehensive High complexity 60
Established patient: outpatient 99211 — — 5
99212 Problem focused Straightforward 10
99213 Expanded Low complexity 15
99214 Detailed Moderate complexity 25
99215 Comprehensive High complexity 40
Inpatient 99221 Detailed Low complexity 30
99222 Comprehensive Moderate complexity 50
99223 Comprehensive High complexity 70
Source: Data from https://www.cms.gov, 2017.

Table 10.2 History component documentation requirements for coding.

Type of history Chief complaint History of present illness Review of systems Past, family and social history

Problem focused Required Brief — —

Expanded problem focused Required Brief Problem pertinent —
Detailed Required Extended Extended Pertinent
Comprehensive Required Extended Extended Complete
Source: Data from https://www.cms.gov, 2017.

Chief complaint Past medical history

Runny nose, itchy eyes, and sneezing. Distal radius fracture in 2018 requiring casting (associated
with skateboarding injury), frequent otitis media as an
infant and toddler.
History of present illness
The patient was brought to clinic today by his mother who Past surgical history
reports that James has been suffering from a “runny nose,
Myringotomy and tube placement in 2012.
itchy eyes, and sneezing” for 3 weeks. James’s symptoms
are worse when he is outside. His symptoms are relieved
with cold compresses to his eyes and after his mother Family history
administers diphenhydramine. However, the mother is
Father has a history of seasonal allergies and takes an
concerned that James is sleepy from the medication and
antihistamine during the spring and summer.
his symptoms distract him at school. James reports, “I hate
my allergies. I can’t stop itching my face.”
Review of systems
Ears: negative for decreased hearing or pain
Medications
Nose: negative for epistaxis or pain
Multivitamin one tablet by mouth daily, Benadryl 25 mg by Mouth and throat: negative for pain, but complains of
mouth as needed for allergy symptoms. “itchiness in back of throat”

40 Cases in Pediatric Acute Care
Eyes: negative for visual changes, but complains of “itchi-
ness and tears”
Pulmonary: negative for cough, hemoptysis, and shortness
of breath
Physical examination
General: well developed, no acute distress, average height
and weight for age
Vital signs: blood pressure 110/65 mmHg, heart rate
85 bpm, respiratory rate 14/min, SpO2 99% on room air
Head: atraumatic, no sinus tenderness
Neck: supple, trachea midline, no lymphadenopathy
Eyes: mildly injected superficial blood vessels in medial
sclerae bilaterally, no discharge or crusting
Nose: mildly injected nasal mucosa, unremarkable septum
and turbinates
Mouth, throat: normal palate, no oral or pharyngeal ery-
thema, tonsils present and unremarkable
Ears: pearly tympanic membrane bilaterally, normal
hearing
Lungs: clear to auscultation, no cough or discolored
sputum
Cardiovascular: S1S2, normal rate and rhythm, no murmurs
or rubs
Extremities: well developed, palpable pulses, no edema
Assessment
Allergic rhinitis in an otherwise healthy patient.
Plan
1) Over-the-counter non-drowsy antihistamine daily
2) Saline nasal spray b.i.d.
3) Antihistamine eye drops for relief of allergic conjuncti-
vitis b.i.d.
4) Follow-up in 4 weeks, call if no symptom relief
within 1 week
5) Referral to allergy specialist if over-the-counter reme-
dies do not alleviate symptoms
Total time
31 minutes
Questions
Answer the following questions using the details provided.
1 What is the difference between ambulatory and inpa-
tient billing encounters and which of the PNP services
are billable?
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2 Is critical care management different from E/M docu-
mentation and billing?
3 Are critical care management codes different for pedi-
atric patients?
4 A critically ill 5-year-old is transferred to a larger hospi-
tal for more advanced services. What critical care code
does the receiving PNP report and what code should the
transferring PNP report?
5 Do time-based critical care management codes have dif-
ferent documentation requirements than pediatric crit-
ical care management codes?
6 Which services are bundled into pediatric critical
care codes?
7 What elements need to be documented for pediatric
critical care billing?
8 What services do not merit critical care manage-
ment codes?
Rationale and Evidence-based
Practice Explanation
What is the difference between ambulatory and
inpatient billing encounters and which of the PNP
services are billable?
In addition to the level of E/M, another important consid-
eration in documentation and billing is collaboration. PNPs
often work on multidisciplinary teams. Individual state leg-
islators establish PNP practice authority. However, even in
full practice authority states, PNPs often collaborate with
physician partners. If both a PNP and a collaborating
physician see a patient (face-to-face encounter) and
contribute to the evaluation and management, then the
encounter can be split or shared. Shared E/M encounters
can be reimbursed at 100% of the physician fee schedule
if both the PNP and the physician see the patient on the
same calendar date. The PNP and physician may see the
patient jointly or independently, both providers must
document, and documentation should be linked in some
fashion. Finally, documentation must support the level of
service (Munro, 2013).
Another type of collaborative encounter is the incident to
service. “Incident to” encounters can also be reimbursed at
100% of the physician fee schedule. However, “incident to”
encounters are exclusive to the ambulatory setting and the
following criteria must be met.
• PNP sees the patient under the physician’s direct
supervision.
•
The physician must be immediately available.

• The physician must perform the initial visit or
consultation.
• The physician is not required to see the patient at
every visit.
• The physician must contribute to the plan of care.
Is critical care management different from E/M
documentation and billing?
Critical care management is the highest level of E/M. The
rules regarding the seven key elements of E/M do not apply
to critical care management documentation. Critical care
management documentation requires evidence of “a criti-
cal illness or injury acutely impairing one or more vital
organ systems such that there is a high probability of immi-
nent or life threatening deterioration in the patient’s condi-
tion” (Britton et al., 2019, pp. 15–46). Further, as per CPT,
critical care “involves high complexity decision making to
assess, manipulate, and support vital system function(s)
to treat single or multiple vital organ system failure and/
or to prevent further life threatening deterioration of the
patient’s condition” (Britton et al., 2019, pp. 15–46).
The overriding principle in documenting critical care
E/M is to demonstrate, with data and a narrative of interval
events, a description of organ system(s) impairments; the
rationale of interventions to assess, manipulate, and sup-
port vital organ function; and the patient’s response to
treatment. A review of systems and most histories, unless
pertinent, are not required.
Are critical care management codes different for
pediatric patients?
Critical care codes for neonate and pediatric patients
(29 days to 5 years) are per diem. Once a patient reaches
his or her sixth birthday, time-based codes are reported.
Pediatric critical care codes are exclusive to the inpatient
setting. If critical care is initiated in a clinic or emergency
department, time-based critical care codes are used.
While only one pediatric critical care code can be
reported per patient per calendar day, it is not uncommon
for multiple providers to provide critical care to a single
patient in a calendar day. For example, a pediatric patient
with septic shock and seizures might be seen by both a pedi-
atric intensivist and a neurologist on the same day. The
intensivist is managing vasoactive medications, resuscita-
tion, antibiotics, and ventilator settings while the neurolo-
gist is interpreting the electroencephalogram and providing
recommendations related to anti-seizure medications. One
physician, likely the intensivist, will report the pediatric
critical care code and the neurologist will report a time-
based critical care code.
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Chapter 10 Billing and Coding 41
A critically ill 5-year-old is transferred to a larger
hospital for more advanced services. What critical
care code does the receiving PNP report and what
code should the transferring PNP report?
Pediatric critical codes include initial inpatient encounter
codes and subsequent day codes. Only one initial inpatient
critical care encounter code can be reported per patient per
hospital stay. Every additional critical care code reported
during of the hospital stay should be a subsequent day crit-
ical care code. If a patient were to leave the intensive care
unit (ICU) and later decompensate and return to the ICU, a
subsequent day code would again be reported.
The child’s age also determines the critical care code
reported (Table 10.3). Critical care provided to children
aged 29 days to 24 months utilizes CPT code 99471 (initial
inpatient encounter) and 99472 (subsequent days)
(Lesnick, 2010). CPT code 99475 is reported for initial inpa-
tient critical care encounter for children aged 2 through
5 years and CPT code 99476 is reported for subsequent
day critical care (Lesnick, 2010).
When neonates or children under 6 years of age are
transferred from one hospital to another, the receiving pro-
vider should report an initial per diem critical care code
(99471 or 99475) and the transferring provider should
report time-based codes for the day of transfer.
Do time-based critical care management codes
have different documentation requirements than
pediatric critical care management codes?
Time-based codes are reported for pediatric and adult
critical care services depending on the circumstance
(Table 10.4). If a patient is aged 29 days to 5 years, per diem
codes are used first. Conversely, if additional critical care is
rendered by a second provider from a different group or
specialty, time-based codes are then reported. If the patient
is 6 years or older, time-based codes are always reported.
CPT code 99291 represents the first 30–74 minutes of crit-
ical care provided by a single provider. CPT code 99292 may
be reported for each additional 30 minutes (beyond 74 min-
utes) of critical care provider by an initial provider or a
Table 10.3 Pediatric critical care codes.
CPT code Age of child Per admission occurrence
99471 29 days to 2 years ×1 per admission
99472 29 days to 2 years Daily as appropriate
99475 2–5 years ×1 per admission
99476 2–5 years Daily as needed
Source: Lesnick (2010). Reproduced with permission of Elsevier.

42 Cases in Pediatric Acute Care
Table 10.4 Time-based codes.
Minutes CPT codes
30–74 99291 ×1
75–104 99291 ×1, 99292 ×1
105–134 99291 ×1, 99292 ×2
135–164 99291 ×1, 99292 ×3
165–195 99291 ×1, 99292 ×4
>194 99291 ×1, 99292 × appropriate number
Source: Data from Mazza (2010).
member of the provider’s group. The time reported may be
continuous or aggregate, but the total number of minutes
must reflect a single calendar day. However, if a clinician
provides continuous critical care that was initiated before
midnight and extends into the next calendar date, the time
is attributed to the date the services were initiated. Some
insurers may require a start and stop time (e.g. 9:15 a.m.
to 10:10 a.m.) while others accept a cumulative total of min-
utes per day.
Time-based codes can be reported wherever critical care
services are delivered, including clinics, emergency depart-
ments, non-telemetry rooms, and ICUs (Painter, 2013).
Two providers from a single group cannot report critical
care for the same time period. Thus, it is important to doc-
ument start and stop times when multiple providers are
delivering critical care. Finally, time-based critical care
codes must be rendered in exclusive attention to a single
patient (Painter, 2013). The provider may not assess, deliver
care, or consult on other patients during the minutes time-
based critical care codes are reported.
Which services are bundled into pediatric critical
care codes?
Many procedures and services are bundled into pediatric
and time-based codes (Table 10.5). Bundled services are
assumed to be included in routine critical care and cannot
be billed separately.
What elements need to be documented for
pediatric critical care billing?
Documentation for critical care services should include
patient information, including name, MRN, and date of
birth; a history of present critical event; summary of inter-
val events, including description of unstable or failing
organ system(s), medical necessity, and response to treat-
ment; corresponding diagnoses; summary of communica-
tion with patient, family, surrogates and other providers;
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time in minutes (if reporting a time-based code); and
a provider attestation (if reporting time-based codes)
(Pohlig, 2013).
The critical care attestation is a statement necessary for
time-based codes. It is a declaration attesting that the
reporting provider was in exclusive attention to the criti-
cally ill patient, physically present on the unit, and the time
reported is exclusive of unbundled procedures and services.
The EMR provides clinicians with an opportunity to
develop templates to ensure the documentation includes
necessary billing elements and the appropriate verbiage
for the attestation.
What services do not merit critical care
management codes?
It is important to note that admission to an ICU does not
necessarily merit critical care management codes. Exam-
ples of situations that will not satisfy medical necessity
criteria for critical care are listed here.
• The patient is admitted to the ICU for 24 hours of
ventilator management before a planned extubation
the following day. Routine postoperative ventilator
management does not meet the standard for critical care.
• The patient is admitted to the ICU for administration
of a thrombolytic for treatment of a pulmonary embo-
lism. The patient is otherwise stable. Admissions
due to institution rules requiring ICU admission for
particular treatments do not meet the standard for
critical care.
• The patient is admitted to the ICU because no other hos-
pital beds are available.
PICU Case Study
A 7-year-old African American male was admitted to the
pediatric intensive care unit (PICU) with a diagnosis of
bacterial pneumonia at 7 p.m. The patient was on sup-
plemental oxygen via high-flow nasal cannula 20 L/
min and FiO2 40%. At 8:15 p.m. the patient experienced
increased work of breathing and oxygen desaturation to
80%. PaO2 on arterial blood gas analysis is 67 mmHg.
A decision is made to intubate the patient, which is
accomplished by the PNP. The patient becomes hypoten-
sive requiring fluid resuscitation and the insertion of
a non-tunneled central venous catheter. A nasogastric
tube is also inserted. A bronchoscopy is performed at
the bedside by a pulmonologist. Thick purulent secre-
tions are observed in addition to a mucous plug in the
right middle lobe, which is successfully removed via
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Chapter 10 Billing and Coding 43

Table 10.5 Bundled services.

Services bundled into per diem pediatric Services bundled into time-based
critical care codes critical care codes

Peripheral vessel catheterization √

Introduction of needle or intracatheter, upper or √
lower extremity artery
Arterial catheterization for sampling, monitoring, or √
transfusion
Umbilical venous catheterization √
Central vessel catheterization √
Vascular access procedures √
Vascular puncture √
Umbilical arterial catheterization √
Endotracheal intubation √
Ventilation management √ √
Bedside pulmonary function test √ √
Surfactant administration √
Continuous positive airway pressure √ √
Monitoring or interpretation of blood gases or √ √
oxygen saturation
Car seat evaluation √
Transfusion of blood components √ √
Oral or nasogastric tube placement √ √
Suprapubic bladder aspiration √ √
Bladder catheterization √ √
Lumbar puncture √
Interpretation of cardiac output measures √ √
Chest radiography √ √
Collection and interpretation of physiologic data √ √
Temporary transcutaneous pacing √ √
Vascular access procedures √
Source: Data from Grider (2019).

suction. A bronchoalveolar lavage sample is sent for 5 What is the critical care billing for a child less than
culture. The endotracheal tube and nasogastric tube 5 years of age?
are verified by portable chest and abdominal X-ray 6 Must the PNP know every rule and regulation related to
respectively. billing?

Questions Rationale and Evidence-based

Practice Explanation
Answer the following questions using the details provided.
What is the critical care time that can be billed?
1 What is the critical care time that can be billed?
2
3
What
What
services are bundled and cannot be billed?
services are unbundled and can be billed?
• Time spent providing care in exclusive attention, as well
as direct and indirect care, to this critically ill and unsta-
4 What billing codes are appropriate for this case? ble patient: 8:15 p.m. to 11:05 p.m.
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44 Cases in Pediatric Acute Care

• The patient is greater than 5 years old so time-based critical

care codes are reported. Critical care time: 170 minutes
•• Insertion of a nasogastric tube
Review of chest X-ray and abdominal X-ray

What services are bundled and cannot be billed?

•• Endotracheal intubation
Insertion of non-tunneled central venous catheter

• Coordinating care and ordering laboratory and imaging

investigations Unbundled procedures

• Review of arterial blood gas (and other laboratory

investigations)
• Bronchoscopy (25 minutes)

•• Insertion of a nasogastric tube

Review of chest X-ray and abdominal X-ray Appropriate billing codes

• 99475 ×1 for initial critical care time for child aged

2–5 years

•
What services are unbundled and can be billed?

•
31622 ×1 for bronchoscopy
Unbundled procedures include intubation (15 minutes
spent on intubation), central venous catheter insertion
(30 minutes) and bronchoscopy (20 minutes). Total time: Must the PNP know every rule and regulation
170 – 15 – 30 – 20 = 105 minutes

••
related to billing?
The PNP bills for the procedures performed
Healthcare is a regulated business and, as such, providers
The PNP can also bill for the critical care time if the inten-
must comply with rules and regulations. The US Office of
sivist is not present and the PNP delivered the services
Inspector General enforces regulations related to medical
during the total minutes reported in exclusive attention
billing and reimbursement in the United States. The regu-
to this patient
lations are in place to prevent fraud by healthcare providers
and billers. It is therefore important that documentation is
What billing codes are appropriate for this case? accurate and reflects the services the provider rendered and

•• 99291 ×1 for first 74 minutes

99292 ×1 for additional 30 minutes
a patient’s factual state of health. There should never be an
effort to “up-code” if the facts and documentation do not

•• 31500 ×1 for endotracheal intubation

36556 ×1 for insertion of non-tunneled centrally inserted
support the higher level of billing. While details and accu-
racy help facilitate optimal billing potential, it is incumbent
upon the PNP to always accurately document.
venous catheter age 5 or older

• 31622 ×1 for bronchoscopy

The Health Insurance Portability and Accountability Act
(HIPAA) was established in 1996. The rules and regulations
put forth in the HIPAA legislation guarantees rights to
What is the critical care billing for a child less than patients with regard to their healthcare and medical record.
5 years of age? It also safeguards patient confidentiality and a secure med-

• Time spent providing exclusive attention, as well as direct

and indirect care, to this critically ill and unstable patient
ical record, whether it is paper or electronic.
Every healthcare organization should have a compliance
office. Compliance officers provide clinicians with educa-
(if he were under 5 years old): 8:15 p.m. to 11:05 p.m.

• Critical care time: 170 minutes

tion, advice and support related to rules and regulations.
In addition, billing entities often provide education and
resources to providers regarding documentation and
Bundled procedures required billing elements. PNPs should familiarize them-

• Coordinating care and ordering laboratory and imaging

investigations
selves with resources and know appropriate contacts within
an organization that can provide guidance and support

• Review of arterial blood gas (and other investigations) related to documentation and compliance.

References
Britton, F., Brown, D., Cachecho, R., et al. (2019). Coding and Grider, D. (2019). Revisiting neonatal and pediatric critical
Billing for Critical Care: A Practice Tool. Prospect, IL: Society care services. Critical Care Connections (May/June/July),
of Critical Care Medicine. 30–31.

Lesnick, B. (2010). Bundled babies and bundled billing: how
to properly use the new pediatric critical care codes. Chest
137(3): 701–704. doi: 10.1378/chest.09-1059.
Mazza, L.E. (2010). Understanding and coding for critical
care services. JustCoding News: Inpatient, 6 January 2010.
Available at https://www.hcpro.com/HIM-244419-3288/
Understanding-and-coding-for-critical-care-
services.html.
Munro, N. (2013). What an acute care nurse practitioner
should know about reimbursement. AACN Advanced
Critical Care 24(2): 110–113. doi: 10.1097/
NCI.0b013e31828c8890.
Further Reading
Center for Medicare and Medicaid Services (2019). National
Plan and Provider Enumeration System. https://nppes.cms.
hhs.gov/#/ (accessed 30 March 2019).
Medicare Learning Network (2017). Evaluation and
Management Services. https://www.cms.gov/Outreach-and-
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Chapter 10 Billing and Coding 45
Painter, J. (2013). Critical care in the surgical global period.
Chest 143(3): 851–855. doi: 10.1378/chest.09-0359.
Pohlig, C. (2013). How to avoid Medicare denials for critical-
care billing. The Hospitalist (October). Available at https://
www.the-hospitalist.org/hospitalist/article/125598/health-
policy/how-avoid-medicare-denials-critical-care-billing
(accessed 31 March 2019).
Wound, Ostomy, and Continence Nurses Society (2012).
Reimbursement of advanced practice registered nurse
services: a fact sheet. Journal of Wound, Ostomy, and
Continence Nurse 39(2 Suppl): S7–S16. doi: 10.1097/
WON.0b013e3182478df0.
Education/Medicare-Learning-Network-MLN/
MLNProducts/Downloads/eval-mgmt-serv-guide-
ICN006764.pdf (accessed 10 April 2019).
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
47

11

Language Delay
Jolene Dickerson
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

A 15-month-old boy presents with his mother to the Family History

primary care office for his well child visit. He is healthy
with no previous concerns. There is no significant family medical history. There is a
6-year-old brother and an 8-year-old sister who are both
healthy and live with the family. Parents are healthy. There
History of Present Illness
are no family members with learning disabilities or other
developmental delays.
The child’s mother reports she has no concerns for today’s
visit; however, on being asked routine developmental
screening questions, she reveals that he is not saying any
words and this does concern her.
Current Status
Past Medical History
The toddler appears well nourished and happy. His physi-
cal examination is unremarkable. A brief developmental
The toddler was born at a gestation of 38 weeks 4 days via
assessment finds that he is not speaking any words, just
spontaneous vaginal delivery. There were no complications
babbling. He does not say “mama” or “papa” specifically.
during the pregnancy or labor and delivery. His birthweight
He follows directions (such as putting trash in the trash
was 3.23 kg and he passed the newborn hearing screen, car-
can) and will point to what he wants. He is walking, climb-
diac screen, and metabolic testing. He is up to date on his
ing and beginning to run. He uses a spoon to feed himself
routine vaccinations, and he will receive 15-month vaccines
and scribbles with a crayon. Per mother’s report, he is
today. Weight is at the 40th percentile, height is at the 50th
always busy and rarely sits still.
percentile, and head circumference is at the 90th percentile.
The Pediatric Evaluation of Developmental Status (PEDS)
screening tool was administered at 9 months of age and
was negative for developmental delay. Other screening
questions at each previous visit have been within normal Questions
limits. His past medical history is significant for transient
tyrosinemia, which resolved in the newborn period, and Answer the following questions using the details provided.
two episodes of otitis media, both treated and resolved.
1 Based on the data provided, what are the differential
diagnoses and the most likely diagnosis for this child?
Past Surgical History 2 What is the best way to address this child’s developmen-
tal concerns?
Only surgical history includes circumcision during 3 What other referrals or diagnostic studies would be
newborn hospitalization. indicated at this time?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

48 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
Based on the data provided, what are the
differential diagnoses and the most likely
diagnosis for this child?
Differential diagnoses for this child include language delay,
autism, and hearing loss. Language delay is the most likely
diagnosis. Language development begins early: infants in
the early months of life will make cooing sounds, and begin
to vocalize and babble. By one year of life, an infant should
make sounds to get attention and has one to two words that
are usually most understandable to parents. By age 2, a
toddler should be able to put some words together in short
sentences.
A diagnosis of autism does include lack of or limited
language, but also will involve other social findings, includ-
ing avoidance of eye contact and hand flapping, Congenital
hearing loss is less likely as the child passed the hearing
screen at birth and the other forms of hearing loss would
be associated with illness; for example, conductive hearing
loss would occur as a result of otitis media and/or fluid in
the middle ear. The most likely diagnosis for this child is
language delay, as language development has begun;
however, autism and hearing loss must also be ruled out.
What is the best way to address this child’s
developmental concerns?
Early language screening is important for identifying
autism and hearing loss, but also language delay as a
primary diagnosis. Recent research indicates that children
with language delay present with middle ear ventilation
disorders, have family histories of language disorders,
and fall into categories representing expressive language
delay, mixed receptive and expressive language delay, delay
with cognitive impairment or intellectual disability, and
autism (Singleton, 2018). Another consideration which is
more prevalent today is the issue of English as a second lan-
guage, and the many children who will grow up in bilingual
families. Challenges to the evaluation of these children,
who may be “late talkers,” include the linguistic variations
between the child’s language, variations of timing of
learning each language, and potential cultural differences
in screeners and the child/family (Singleton, 2018).
At any well child visit, the care provider should first
address the concerns of the parent. The Bright Futures
recommendations for the 15-month visit include evaluating
social skills and communication, sleep patterns and issues,
oral hygiene, and safety. This child’s mother is concerned
that he is not speaking, so a few more specific questions
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are asked about his language and social development,
which appears to be normal as he is interactive and does
respond to directions and questions. Referral to a pediatric
developmental specialist will help identify any specific
delays or concerns to determine what services can be uti-
lized to help him. Early intervention is key to improving
outcomes in children with developmental delay (Bright
Futures Steering Committee, 2006, pp. 405–406).
Collaboration with the developmental specialist and
others involved in his care, such as the speech therapist,
will assist in determining if the child is meeting milestones.
He will also be assessed at each well child visit, using formal
developmental assessment tools, to ensure he is making
progress and receiving all needed services. The primary
care provider should receive reports from the pediatric
developmental specialist and support the family in utilizing
recommendations.
What other referrals or diagnostic studies would
be indicated at this time?
This young boy is referred to the state developmental
assessment and support program, where he will undergo
complete evaluation by a developmental pediatrician. He
will have an assessment to determine any social, gross
motor, fine motor and/or language problems. Recom-
mended therapy and/or other specialty referrals will be
identified. The developmental pediatrician and hearing
specialist determine that he has language delay and
recommend speech therapy and an audiology referral to
rule out hearing loss. The primary care provider will con-
tinue to follow this child’s developmental progress and
his social/adaptive skills at each well child visit to deter-
mine if any other referrals are warranted.
An autism evaluation is also recommended by the state
program, and therefore the child is referred to a pediatric
psychologist who specializes in developmental pediatrics
and autism. It can be difficult to quickly refer to an autism
program or evaluation specialist, and it may be hard to
diagnose autism at this age, but there are also office-based
tools to utilize such as M-Chat, which is a parent-response
tool that identifies typical features and behaviors of
children with autism.
Case Resolution
At his 18-month visit, the child’s mother answers
another PEDS screening assessment. The American
Academy of Pediatrics recommends routine developmen-
tal screening at the 9-, 18-, and 30-month well child visits
using a standardized screening tool such as the PEDS

screening assessment, which is a 10-question survey of
development for parents to complete and which can be
used for young ages (Bright Futures, 2006, p. 406). This
screen asks general questions with regard to speech,
behavior, getting along with others, and use of hands
References
Bright Futures Steering Committee (2006). Identifying infants
and young children with developmental disorders in the
medical home: an algorithm for developmental surveillance
and screening. Pediatrics 118(1): 405–420.
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Chapter 11 Language Delay 49
and fingers among others. This child underwent 18-month
screening with use of the PEDS questionnaire. Mother has
concerns that he is not speaking, but he receives speech
therapy twice weekly and he is also undergoing an autism
assessment.
Singleton, N.C. (2018). Late talkers: why the wait and see
approach is outdated. Pediatric Clinics of North America 65:
13–29. doi: 10.1016/j.pcl.2017.08.018

12
A 6-Month-Old Infant with RSV and Respiratory Failure
Catherine M. Haut1,2
1
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
2
Mednax-Pediatrix Medical Group, Richardson, TX, USA
A 6-month-old, breast-fed, term baby with a 3-day history
of nasal congestion and cough presents to the primary care
provider for evaluation.
History of Present Illness
She has had a fever to 38.8 C (101.8 F) and decreased appe-
tite, but temperature responds well to acetaminophen and
she is happy and appears comfortable. Evaluation by the
primary care provider indicates scattered expiratory
wheezes and mild tachypnea. The parents are instructed
to use saline nose drops for congestion, feed her more fre-
quently in smaller amounts, and monitor her work of
breathing.
Two days later, the primary care provider is informed that
the infant is working harder to breath and the parents are
worried, so the child is taken to the emergency department
for further care. The baby continues to be tachypneic with
a respiratory rate of 70 breaths per minute and is hypoxic with
oxygen saturation of 86% on room air. She is placed on a high-
flow nasal cannula (HFNC) at a flow rate of 4 L/min, but is
still working hard with diffuse wheezing throughout her lung
fields. During observation, she is noted to have a very short
episode of apnea, which occurs again within 15 minutes.
Other than respiratory findings, the remainder of her physical
examination is normal, except for the presence of a right otitis
media and a temperature of 38.3 C (100.9 F).
Past Medical History
The patient has received all required vaccines up to
6 months, along with one influenza vaccine and her growth
and development are appropriate. She has no other medical
problems.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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51
Past Surgical History
No previous surgical procedures.
Family History
A 3-year-old sibling had one episode of wheezing with a
cold at age 18 months. There is no history of asthma in
the family. Parents are healthy and both sets of grandpar-
ents are alive and healthy. The infant attends home daycare
with her sibling.
Questions
Answer the following questions using the details provided.
1 Based on the history and physical examination, what
are the potential differential diagnoses?
2 What diagnostic work-up is warranted and why?
3 What would be the next therapy for management of res-
piratory distress and why?
4 What other therapeutic modalities are recommended at
this time?
Continuation of case: This baby girl could not maintain
oxygenation and comfortable respirations on nasal cannula
at 4 L/min, so care is escalated to HFNC at 14 L/min and
40% oxygen, and then to intubation and ventilation as
she had additional episodes of apnea. She also could not
maintain sufficient oxygenation during nasopharyngeal
suction, and her secretions were thick and obstructive.
Because of respiratory failure, she is admitted to the pedi-
atric intensive care unit (PICU) for further monitoring
and care. A dose of nebulized albuterol is given with little
improvement, so no further medication is administered

52 Cases in Pediatric Acute Care
other than the antibiotic. The infant continues to need
higher oxygen levels and higher flow rates, so a decision
is made to intubate her in order to improve oxygenation
and for more efficient suctioning. She is started on intrave-
nous dexmedetomidine and fentanyl infusions for sedation
and analgesia. An intravenous catheter was in place and
she is now nil by mouth and receiving fluids at
maintenance.
5 What is the rationale and benefit to using HFNC for
treatment of respiratory distress? When would intuba-
tion and ventilation be appropriate?
6 When is weaning from HFNC indicated?
7 What criteria are used for transfer from the PICU to the
pediatric inpatient unit?
Rationale and Evidence-based Practice
Explanation
Based on the history and physical examination,
what are the potential differential diagnoses?
Based on the history, presenting findings and timeline, the
most likely diagnosis would be respiratory syncytial virus
(RSV) bronchiolitis. Other possible diagnoses include aspi-
rated foreign body, reactive airways disease, and pneumo-
nia. In order to evaluate for pneumonia, a chest radiograph,
both anteroposterior and lateral, would be recommended.
Other symptoms of pneumonia include decreased appetite,
persistent fever, and respiratory distress as tachypnea with
oxygen required, which all exist for this patient. There is no
history of the infant choking and she only recently started
baby foods and has not had any table foods as yet, so a
foreign body aspiration is unlikely. Wheezing is an
indication of reactive airways disease, but in such a child
the first episode of wheezing at less than 1 year of age
defines bronchiolitis.
Bronchiolitis is a common diagnosis in young infants and
occurs seasonally in most areas of the United States.
Bronchiolitis is usually caused by a viral process and there
are many known viral etiologies, with the most common,
RSV, accounting for 50–80% of all hospitalizations from
bronchiolitis (Meissner, 2016). Rhinovirus, parainfluenza,
enterovirus, coronavirus, and human metapneumovirus
are others that can cause bronchiolitis. Recent data indicate
that infants who are hospitalized for bronchiolitis are usu-
ally term infants between the ages of birth and 5 months,
with risks higher among infants who have coexisting pro-
blems such as prematurity, chronic lung disease of
prematurity, and cardiac disease. Diagnostic criteria for
bronchiolitis are based on history and physical examination
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and include the presence of respiratory illness in children
less than 2 years of age. Clinical symptoms include rhinor-
rhea, cough, tachypnea, wheezing, crackles, and respira-
tory distress at varied levels, with the possibility of
grunting, nasal flaring and retractions.
RSV is an enveloped, non-segmented, negative-stranded
RNA virus with two subtypes, with subtype A causing more
severe illness. An RSV infection begins when the virus is
replicated in the upper airway, typically causing initial
cold-like symptoms. The virus spreads to the bronchiolar
epithelium of the small airways, becoming a lower airway
disease process. Edema of the airways, along with increased
production of mucus, results in small airway obstruction,
air trapping, and increased resistance. Wheezing is noted
when air cannot pass easily through narrowed pathways.
In addition, the damaged epithelial cells initially necrose
in the process of regeneration, producing additional debris
to further obstruct air movement.
What diagnostic work-up is warranted and why?
The American Academy of Pediatrics (AAP) has published
guidelines for the diagnosis and management of bronchio-
litis outside of the intensive care unit. There are no
documented recommended medications or therapies that
hasten resolution of symptoms of bronchiolitis, so support-
ive care is the treatment. Nebulized saline treatments are
the only therapy which can be “considered” per the AAP
guidelines. Radiographs, laboratory studies and viral panels
are not routinely recommended for diagnosis. A patient
who requires intensive care may require additional diag-
nostic studies to determine comorbidities.
What would be the next therapy in management
of respiratory distress and why?
Hospitalization for infants with bronchiolitis is indicated
when they are unable to manage feeding to maintain
hydration and/or have an oxygen requirement or respi-
ratory distress that requires support. If oxygenation can-
not be maintained and the infant is tachypneic with
respiratory distress, then the next recommendation for
care is to use a non-invasive ventilation mode, in the
form of continuous positive airway pressure (CPAP),
bilevel positive airway pressure (BiPAP), or high-flow
nasal cannula (HFNC), which can be more comfortable,
stays in place more easily, and allows oxygen to be
titrated. An additional level of support with intubation
and ventilation is reserved but sometimes indicated
when babies cannot maintain an adequate open airway
and/or oxygenation.
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Chapter 12 A 6-Month-Old Infant with RSV and Respiratory Failure 53

What other therapeutic modalities are oxygenation. Also, using high humidity, HFNC prevents
recommended at this time? thickening of secretions, decreasing some of the obstructive
components of bronchiolitis.
Antibiotic treatment can be recommended for this infant,
The use of non-invasive ventilation in the PICU has
due to the presence of otitis media with fever. Ceftriaxone
many advantages: decreased risk of ventilator-associated
is not first-line therapy for otitis media, but can be given
pneumonia, decreased need for sedation, decreased length
either intravenously or intramuscularly if oral medications
of stay, and attenuation of the laryngeal and tracheal injury
are not tolerated. Ceftriaxone is broad spectrum and the
and barotrauma associated with intubation and mechani-
dosing for otitis media is 75 mg/kg once daily for 3 days.
cal ventilation. HFNC has resulted in higher nurse and
With worsening status, though, treatment can be
patient satisfaction, increased mobility for the child, and
continued, especially if there is concern for a bacterial
overall lower hospital costs of care.
pneumonia.
Infants with RSV bronchiolitis can present with apnea
and hypoxia. Because of fatigue, increased secretions and
What is the rationale and benefit to using HFNC for
respiratory failure, the next level of respiratory support is
treatment of respiratory distress? When would
intubation and mechanical ventilation.
intubation and ventilation be appropriate?
Non-invasive ventilation can be either CPAP/BiPAP or
HFNC. CPAP/BiPAP employs a set pressure to deliver When is weaning from HFNC indicated?
positive end-expiratory pressures. These systems work by
There are no standard weaning criteria for HFNC and there
using pressure that distends end-airways at the alveolar
are no evidence-based feeding protocols, although some
level, improving oxygenation. HFNC is a mode of non-
institutions have developed their own pathways. There
invasive ventilation which is not as well defined. Heated
are some that utilize a small percentage of decrease in
HFNC or high-velocity nasal insufflation supplies humidi-
HFNC flow over time, and others that wean quickly. Based
fied and blended air/oxygen via nasal cannula at different
on the nature of bronchiolitis and the variability of illness,
flow rates (≥1–2 L/min), delivering both high concentra-
weaning time frame can also vary. In this child, weaning
tions of oxygen and potentially continuous distending pres-
was slow, but after 3 days in the PICU it became easier
sure. HFNC utilizes a small, thin tapered nasal cannula,
to wean the high flow, and when she was on 8 L/min
which facilitates the velocity of flow. It is unknown exactly
she was transferred to the pediatric inpatient unit.
how much pressure is utilized based on flow rate, but this
treatment has been used successfully in neonates and
infants (Beggs et al., 2014).
What criteria are used for transfer from the PICU to
Bronchiolitis can result in the collection of intraluminal
the pediatric inpatient unit?
debris and mucus in the lower airways, causing obstruc-
tion, contributing to tachypnea, and resulting in muscle Discharge criteria include ability to feed and gain weight
fatigue and upper airway collapse. Non-invasive ventilation and no further need for oxygen. Discharge teaching should
assists in providing distending pressure to the lower air- include information that bronchiolitis can result in a cough
ways, offering a mechanism of airway conductance and which lasts a longer time and that it can also recur.
pulmonary compliance. HFNC decreases dead space in Research indicates that infants who develop bronchiolitis
the airway with washout of carbon dioxide and increased have a higher risk of wheezing or developing asthma later
fraction of oxygen in the alveoli, thus increasing in childhood.

References
Beggs, S., Wong, Z.H., Kaul, S., Ogden, K.J., and Walters, J.A.
(2014). High-flow nasal cannula therapy for infants with
bronchiolitis. Cochrane Database of Systematic Reviews (1):
CD009609.
Meissner, H.C. (2016). Viral bronchiolitis in children. New
England Journal of Medicine 374: 62–72. doi: 10.1056/
NEJMra1413456.

Further Reading
Ralston, S.L., Lieberthal, E.S., Meissner, H.C., et al. (2014). prevention of bronchiolitis. Pediatrics 134(5): e1474–e1502.
Clinical Practice Guideline: the diagnosis, management and doi: 10.1542/peds.2014-2742.
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55

13

Adolescent Abdominal Pain after Cardiac Surgery

Cathy Woodward
Long School of Medicine, University of Texas Health San Antonio, San Antonio, TX, USA

A 12-year-old was admitted to the step-down unit 2 days Current Status

after a repair of coarctation of the aorta.
The child develops severe abdominal pain and the regis-
tered nurse pages the healthcare provider to the bedside
to evaluate the patient. On arrival, the patient is pale, dia-
History of Present Illness phoretic and complaining of mid-epigastric abdominal
pain. The child has vomited twice in the past 2 hours,
The child was diagnosed with coarctation of the aorta by
but denies diarrhea. On further investigation, the registered
echocardiogram as part of a work-up conducted after a
nurse reports that the child has not passed stool since prior
pre-sports physical revealed hypertension. The surgery
to the surgery. The mother reports that the child, who was
and immediate postoperative course were uneventful with
on a full liquid diet, ate several slices of pizza when visited
the exception of hypertension initially treated postopera-
by family members earlier in the evening. On examination,
tively with intravenous antihypertensives, but later transi-
his abdomen is full, distended and tender to palpation over
tioned to the oral angiotensin-converting enzyme (ACE)
the epigastric area with no bowel sounds noted. His skin is
inhibitor enalapril. The child was weaned off all intrave-
slightly diaphoretic, pulses are 2+ in all extremities, and the
nous medications and continued on oral acetaminophen
thoracotomy site is clean and dry with no drainage or
and ibuprofen, as needed for pain, when admitted to the
erythema.
step-down unit.
Vital signs at the time of examination are as follows: tem-
perature 37.2 C (98.9 F), heart rate 110 bpm with regular
rhythm, respiratory rate 18 breaths per minute, blood pres-
Past Medical History sure 138/80 mmHg, and oxygen saturation 100% on
room air.
The child is up to date on immunizations; height and
weight at the 75th percentile.

Questions
Past Surgical History Answer the following questions using the details provided.

No previous surgeries or hospitalizations. 1 What diagnoses are included in your differential?

2 What diagnostic studies or imaging would you order?
3 What finding is demonstrated on the abdominal flat
plate radiograph?
Family History 4 Based on the information provided, which is the most
probable diagnosis?
The patient lives with mother and two older siblings. 5 What treatments should you initiate?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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56 Cases in Pediatric Acute Care

Rationale and Evidenced-based Practice

Explanation

What diagnoses are included in your differential?

The differential diagnoses include gastric reflux, dyspepsia,
post-coarctectomy syndrome, pancreatitis, gastrointestinal
viral infection, and food poisoning.
Post-coarctectomy syndrome can occur in children after
repair of coarctation of the aorta due to return of pulsatile
blood flow distal to the coarctation including to the mesen-
teric arteries. This sudden reperfusion to arteries that have
not seen pulsatile flow can cause reflex vasoconstriction
and ischemia to the organs and tissues perfused by the mes-
enteric vessels, causing abdominal pain and distension,
vomiting, and ileus. This is the most likely diagnosis for this
patient. If it progresses, perforation with bloody stools and
possibly shock can be seen. The syndrome may be associ-
ated with early feeding and increasing oxygen requirements
of the abdominal organs. A common preventive treatment
is to avoid early feeding and progress the diet slowly
48 hours after repair of coarctation of the artery. Figure 13.1 Abdominal flat plate radiograph.

What diagnostic studies or imaging would

you order?
A complete blood count, electrolytes, C-reactive protein
(CRP), liver enzymes, amylase and lipase are ordered as well
as an abdominal flat plate radiograph (see Figure 13.1).
C-reactive protein is found to be abnormal (150 mg/L;
normal, 1–3 mg/dL).
What finding is demonstrated on the abdominal
flat plate radiograph?
Gas-filled loops indicative of ileus. No pneumatosis or free
air noted.
Based on the information provided, which is the
most probable diagnosis?
Ileus due to post-coarctectomy syndrome.
What treatments should you initiate?
Nil by mouth status and initiation of intravenous fluids at
maintenance rate until pain resolves and bowel sounds
return is indicated. A clear liquid diet can be initiated after
bowel sounds return. Avoidance of opioid analgesic medi-
cations, monitoring of abdominal girths and ambulation
should be encouraged. Monitoring is needed for signs of
worsening ileus, peritonitis, pain, fever, abdominal disten-
sion and shock. If ileus occurs, returning to nil by mouth
status until bowel function returns, using non-opioid
analgesics and encouraging ambulation are the most com-
mon measures taken to reduce further complications. The
placement of a nasogastric tube may not be warranted in
this case; however, if the ileus worsens it may become nec-
essary. If perforation occurs, a laparotomy and bowel re-
section may be needed.
Case Resolution
This patient’s ileus resolved with conservative management
and he was discharged 3 days later with adequate bowel
function and persistent hypertension.

Further Reading
Roeleveld, P.P. and Zwijsen, E.G. (2017). Treatment
strategies for paradoxical hypertension following surgical
correction of coarctation of the aorta in children. World
Journal of Pediatric and Congenital Heart Surgery 8(2):
321–331.
Sapin, S.O., Rosengart, R.M., and Salem, M.M. (2002). Chest
pain during stenting of a native aortic coarctation: a case
study for acute intercostal muscle ischemia and
rhabdomyolysis. Catheterization and Cardiovascular
Interventions 57: 217–220.

14
Young Infant with Vomiting
Sarah Martin
Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA
A 6-week-old male infant, weighing 3.6 kg, has a 3-day
history of projectile, non-bloody, non-bilious, postprandial
emesis.
History of Present Illness
Parents bring their 6-week-old male infant to the emergency
department (ED) with a 3-day history of projectile, non-
bloody, non-bilious, postprandial emesis. The infant has
been afebrile, stooling daily, and has had three wet diapers
a day, last voided 8 hours ago. He has been breastfeeding
eagerly every 3 hours; however, following the feeds the
infant has been having large projectile emesis. There have
been no sick contacts and the infant does not attend daycare.
Past Medical History
This infant was born full-term by normal vaginal delivery
and was discharged home with mother on the second day
of life. The infant has no past medical or surgical history,
except for circumcision at birth without any complications
including bleeding.
Family History
The infant has a healthy 2-year-old brother and several
young cousins, but no history of surgery in the first
6 months of life. Both parents and grandparents are
healthy. There are no known family members with a his-
tory of anesthetic complications or bleeding disorders.
Current Status
On arrival in the ED the infant is normothermic, with a
heart rate of 170 bpm, respiratory rate of 32 breaths per
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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57
minute, and blood pressure 80/44 mmHg. His physical
examination reveals a quiet infant sucking vigorously on
a pacifier. The infant’s cardiac and respiratory examination
are unremarkable. His abdominal examination reveals a
soft, rounded, non-tender abdomen. There are no abdomi-
nal masses, hernias, or hepatosplenomegaly appreciated.
The infant is awake with normal tone and is moving all
extremities well. His anterior fontanel is flat. The infant’s
extremities are pink, warm, and dry with a capillary refill
time of 2–3 seconds. His radial and posterior tibial pulses
are palpable and are 3+/4+.
To further evaluate the infant a complete blood count,
basic metabolic panel (BMP) and an abdominal ultrasound
are performed. The results of the BMP are detailed in
Table 14.1.
Questions
Answer the following questions using the details provided.
1 Based on the presentation and initial laboratory values,
what is the most likely diagnosis and two other
differentials?
Continuation of case: The abdominal ultrasound reveals
a thickened pyloric muscle measuring up to 0.6 cm and an
elongated pyloric channel measuring 2.2 cm. These ultra-
sound findings are consistent with the diagnosis of hyper-
trophic pyloric stenosis (HPS) or pyloric stenosis (PS).
2 What is the physiologic basis for the electrolyte
derangements seen in infants with pyloric stenosis?
3 What are the clinical criteria and diagnostic evaluation
for the diagnosis of pyloric stenosis?
4 What is the treatment for infants with pyloric stenosis?
5 Why would anesthesia not be administered to this
infant with the electrolyte derangements noted at
admission?

58 Cases in Pediatric Acute Care
6 What feeding regimen should be offered to an infant
following a pyloromyotomy?
7 What are the signs and symptoms that would be con-
cerning for a postoperative complication following a
laparoscopic pyloromyotomy?
Rationale and Evidence-based Practice
Explanation
Based on the presentation and initial laboratory
values, what is the most likely diagnosis and two
other differentials?
The most likely diagnosis for this 6-month-old infant is
pyloric stenosis, a gastric outlet obstruction that typically
occurs in young infants between the ages of 4 and 8 weeks
of life and is the most common surgical condition in this age
group. Previously thought to be a congenital disorder,
pyloric stenosis occurs with thickening and hypertrophy
of the pyloric muscles, causing an obstructive process that
results in vomiting, often projectile in nature. Pyloric steno-
sis occurs more frequently in males than in females and can
result in weight loss, dehydration and electrolyte abnorm-
alities, especially hypochloremic alkalosis.
Two other common differential diagnoses include
milk-protein allergy and gastroesophageal reflux disease
(GERD). A true milk-protein allergy can result in emesis,
but the most common symptom is bloody diarrhea. GERD
is also a common finding in young infants, and can be asso-
ciated with weight loss and dehydration, but even though
vomiting can occur, it is usually not projectile in nature.
Neither milk-protein allergy or GERD results in metabolic
alkalosis. If a child with either of these problems has dehy-
dration, metabolic acidosis is the typical presentation.
What is the physiologic basis for the electrolyte
derangements seen in infants with pyloric
stenosis?
With the progressive vomiting of HPS, infants become
dehydrated and can present with a hypochloremic, hypoka-
lemic metabolic alkalosis with a paradoxical aciduria.
These metabolic derangements used to be seen universally
in infants with PS; however, with earlier ultrasound diag-
nosis these abnormalities are only seen in a small percent-
age of infants at diagnosis. Persistent emesis results in the
loss of hydrogen and chloride ions and the pancreas is not
stimulated to secrete bicarbonate as hydrogen ions are not
reaching the duodenum, with a resultant metabolic alkalo-
sis (Jacobs et al., 2019). In an effort to retain sodium and
water, the kidney excretes potassium and hydrogen ions
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leading to paradoxical aciduria. An elevated unconjugated
hyperbilirubinemia related to transient impairment of glu-
curonyltransferase activity may also be present.
What are the clinical criteria and diagnostic
evaluation for the diagnosis of pyloric stenosis?
Pyloric stenosis can be diagnosed using a combination of
clinical and physical examination and laboratory findings.
Projectile vomiting following feeding, with continued rav-
enous appetite, weight loss and dehydration are reported
symptoms of PS. A common physical examination finding,
though sometimes difficult to ascertain, is the olive sign,
which is a small round palpable mass located in the right
upper quadrant of the abdomen. Although this physical
finding may not be noted preoperatively in the awake hun-
gry infant when assessed for at the time of surgery, it can be
palpated once the infant is asleep.
Ultrasound is the gold standard for making the diagnosis
of HPS. Ultrasound findings of a pylorus muscle wall thick-
ness of 0.3 cm or more, and a pyloric channel length of
1.5 cm or more is diagnostic for HPS with 100% sensitivity
and specificity (Jobson et al., 2016). In addition, there are
specific measurements that apply to the diagnosis of HPS
in premature infants. Other radiologic studies may be
employed and include an upper gastrointestinal study,
which may be of value if there are concurrent suspicions
for malrotation and GERD. A string sign (elongated and
thickened pyloric channel), beak sign (filling of the proxi-
mal pylorus), or double shoulder sign (thickened pylorus
compressing the antrum of the stomach) may be noted
when HPS is present.
What is the treatment for infants with pyloric
stenosis?
An early surgical consult is recommended once there is a
suspicion of HPS. Preoperative care for the infant with
HPS includes nil by mouth status, intravenous hydration,
and correction of electrolyte derangements if present. The
definitive treatment for HPS is operative intervention with
a pyloromyotomy. Rammstedt pioneered the procedure,
which involves the longitudinal extramucosal splitting of
the muscle. A pyloromyotomy can be performed with either
an open technique or via a laparoscopic approach.
Why would anesthesia not be administered to this
infant with the electrolyte derangements noted at
admission?
Even though surgical intervention is the treatment for HPS,
this is not an emergent procedure and many infants require
stabilization with fluid administration and electrolyte
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Chapter 14 Young Infant with Vomiting 59

Table 14.1 Laboratory values. infants with persistent emesis in the postoperative period
or delayed ad lib feeds. Breastfeeding is considered safe
Preoperative values and appropriate and should be encouraged. If infants
Admission values (2 days later) require a regimented feeding plan, the feeds are increased
by minutes with each feeding as tolerated. Often infants
Sodium 140 mEq/L 141 mEq/L
with HPS are on specialized formulas preoperatively due
Potassium 2.6 mEq/L 3.8 mEq/L
to their emesis, but it is not necessary to continue this fol-
Chloride 89 mEq/L 106 mEq/L lowing surgery.
Carbon dioxide 36.3 mEq/L 24.7 mEq/L Postoperative vomiting is common and occurs due to gas-
Glucose 88 mg/dL 89 mg/dL tric distension and atony. Postoperative feeding intolerance
Blood urea nitrogen 20 mg/dL 8 mg/dL with emesis is more commonly seen in infants with severe
Creatinine 0.43 mg/dL 0.28 mg/dL electrolyte derangements.
The majority of infants tolerate ad lib feeds (volume of at
least 60 mL) within 24–48 hours of the operation. Prior to
correction prior to undergoing surgery. Surgery is done discharge it is desirable to have the infant tolerate two 60-
when the child is hydrated and electrolytes have been nor- mL feeds without emesis. Persistent vomiting beyond 72–96
malized. For infants with derangements as severe as in hours postoperatively may occur in cases of incomplete
this case, it can take several days for needed correction, pyloromyotomy and an upper gastrointestinal series may
as severe alkalosis can place the infant at risk for apnea be valuable to determine if this complication is present.
following extubation after general anesthesia. It is desirable
to have a carbon dioxide level less than 30 mEq/L prior to
the surgical procedure. Judicious and daily laboratory mon- What are the signs and symptoms that would be
itoring is appropriate because correction can take several concerning for a postoperative complication
days (see Table 14.1). following a laparoscopic pyloromyotomy?
Infants with PS should be placed on a cardiac and respi-
What feeding regimen should be offered to an
ratory monitor, as subtle trends in vital sign measure-
infant following a pyloromyotomy?
ment can identify significant complications. Gastric
Varying postoperative feeding regimens are used, such as mucosal tears or duodenal perforation are life-
ad lib feeds starting immediately after surgery to 6 hours threatening complications and these infants may have
postoperatively or an incremental feeding plan often tachycardia as an early sign of peritonitis that will
referred to as a “pyloric regimen.” Markel et al. (2017) develop with perforation. A temperature elevation should
reported on a prospective randomized trial comparing an be brought to the provider’s attention because this sign
incremental feeding regimen to ad lib feeds and found may be seen with a mucosal tear. Persistent vomiting
the infants that took feeds ad lib reached goal feeds sooner. beyond 72 hours may be investigated over concern for
The pyloric regimen is more commonly prescribed for an incomplete pyloromyotomy.

References
Jacobs, C., Johnson, K., Khan, F.A., et al. (2019). Life-
threatening electrolyte abnormalities in pyloric stenosis.
Journal of Pediatric Surgery Case Reports 43: 16–18.
doi: 10.1016/j.epsc.2019.01.002.
Jobson, M. and Hall, N.J. (2016). Contemporary management
of pyloric stenosis. Seminars in Pediatric Surgery 25:
219–224. doi: 10.1053/j.sempedsurg.2016.05.004.
Markel, T.A., Scott, M.R., Stokes, S.M., et al. (2017). A
randomized trial to assess advancement of enteral feedings
following surgery for hypertrophic pyloric stenosis. Journal
of Pediatric Surgery 52: 534–539. doi: 10.1016/j.
pedsurg.2016.09.069.

15
School-age Child with Bruising
Lisa Hovingh and Candie Ritsema
Helen DeVos Children’s Hospital, Grand Rapids, MI, USA
A 6-year-old male presents to the pediatric emergency
department (ED) with fever, extensive bruising, and
enlarged lymph nodes to his neck.
History of Present Illness
This previously healthy 6-year-old boy developed nasal con-
gestion and low-grade fevers about 2 weeks prior to presenta-
tion; these symptoms resolved within a few days. About 5 days
ago, he was noted to have bruising to his lower extremities.
The bruising and petechial rash have worsened over the last
few days. He was also found to have palpable lymph nodes to
his neck and axilla. He reports some pain in his legs that the
family attributed to growing pains. Today, he was febrile to
39.1 C (102.4 F) at home. He has had no cough, no difficulty
breathing, no bleeding symptoms, no fatigue, and no diffi-
culty sleeping. He has had no known exposure to illness.
Past Medical History
He was born at term weighing 3.23 kg and was discharged
home with his mother on day of life 2. All immunizations
are up to date, including annual influenza vaccines. He has
a body mass index (BMI) of 14.81. He was diagnosed with a
concussion at 15 months of age following a witnessed fall
off a coffee table. At 4 years, he was seen in the ED with
dysphagia, throat pain, and refusal to swallow oral secre-
tions after swallowing a quarter. Fiberoptic endoscopic
removal of foreign body was successfully performed.
Past Surgical History
None.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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61
Family History
No history of cancer or blood disease including bleeding or
thrombotic disorders in parents or grandparents. No immune
disorders. He has an 8-year-old sister who is currently healthy.
Current Status
Vital signs on presentation to ED: temperature 38.1 C
(100.6 F), heart rate 124 bpm, respiratory rate 28 breaths
per minute, blood pressure 108/66 mmHg, and oxygen
saturation of 98% on room air. Peripheral blood culture, com-
plete blood count (CBC), and comprehensive metabolic panel
are obtained. Results are shown in Tables 15.1 and 15.2.
Questions
Answer the following questions using the details provided.
1 Based on the data provided, what are the differential
diagnoses for this patient?
2 What is your management plan for this patient?
3 How will you continue to monitor this patient to
determine if this treatment was effective?
Rationale and Evidence-based Practice
Explanation
Based on the data provided, what are the
differential diagnoses for this patient?
Differential diagnoses for this patient include infection,
inflammation, asplenia, hemolytic disease, myeloproli-
ferative disorder, hereditary or chronic neutrophilia, or a
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62 Cases in Pediatric Acute Care

Table 15.1 Basic metabolic profile. and inducing high urine output, decreasing the likelihood
of uric acid or calcium phosphate precipitation in the renal
Sodium 138 mEq/L
tubules. The recommended rate in both children and adults
Potassium 3.9 mEq/L is 2–3 L/m2 per day, or 200 mL/kg per day in children
Chloride 99 mEq/L weighing less than 10 kg.
Carbon dioxide 26 mEq/L With the elevated uric acid on presentation, he will need
Blood urea nitrogen 12 mg/dL both rasburicase and allopurinol. Rasburicase will rapidly
Creatinine 0.48 mg/dL break down serum uric acid that has already accumulated
Glucose 75 mg/dL
by promoting catabolism of uric acid. Recommended intra-
venous dosing of rasburicase is 0.1–0.2 mg/kg over 30 min-
Uric acid 10.4 mg/dL
utes. It is important to ask about the child’s family history
Lactate dehydrogenase >2500 U/L
prior to administration of rasburicase, as it is contraindicated
Phosphorus 5.5 mg/dL in patients with glucose 6-phosphate dehydrogenase defi-
ciency and can cause severe hemolysis with this condition.
Allopurinol will not break down serum uric acid but will
Table 15.2 Complete blood count. decrease the formation of new uric acid. Recommended dos-
ing of allopurinol is 10 mg/kg divided twice daily.
White blood cell count 180 850/mm3 With the risk for worsening electrolyte imbalances
Hemoglobin 9.6 g/dL secondary to tumor lysis syndrome, prevention of hyperka-
Hematocrit 28.9% lemia is imperative. Hydration should not contain potas-
Platelet count 18 000/mm3 sium due to its intracellular release into the circulation
Neutrophils 6% as cells break down. Hyperkalemia is a dangerous
Eosinophils 0% component of tumor lysis syndrome as it can cause sudden
death and cardiac dysrhythmia. An electrocardiogram and
Monocytes 2%
continuous cardiac monitoring, along with frequent labora-
Lymphocytes 15%
tory evaluations, are necessary.
Blasts 77%
Oftentimes, children with leukemia may present with a
mediastinal mass, necessitating a chest radiograph. This
malignant process such as leukemia or lymphoma. The should be obtained prior to any sedation for imaging studies
most likely diagnosis for this child is leukemia with or procedures as the presence of a mediastinal mass will
developing tumor lysis syndrome. Leukocytosis, anemia, increase the risk for airway compromise. Consultation with
thrombocytopenia, and the presence of blasts indicate a oncology is important in determining the diagnosis and
malignant process. Other clinical findings of leukemia treatment plan for the patient’s disease. Further information
include fever, lymphadenopathy, hepatosplenomegaly, regarding the diagnosis can be obtained from pathology
and bone pain. review and flow cytometry of his peripheral blood. A bone
The severity of his electrolyte abnormalities is due to marrow biopsy and aspirate is also needed for additional
tumor lysis syndrome, which can develop before treatment information regarding his type of leukemia and any addi-
is initiated. Tumor lysis syndrome is a group of metabolic tional cytogenetic or molecular information. A lumbar punc-
abnormalities resulting from the rapid release of intracellu- ture will also be necessary to determine if he has leukemia in
lar components. This process can cause hyperuricemia, his central nervous system. Establishing an accurate diagno-
hyperkalemia, hyperphosphatemia, renal injury, arrhyth- sis will determine the best treatment for this patient and
mias, seizures and even death. The immediate need is guide therapy per a protocol or enrollment in a clinical trial.
correction of electrolytes to prevent renal injury in the It is important to initiate therapy as soon as possible to treat
setting of hyperuricemia and hyperphosphatemia. his underlying leukemia. However, treatment will increase
cell turnover, increasing the risk for worsening tumor lysis
syndrome and subsequent kidney injury.
What is your management plan for this patient?
The priority for this patient is hydration, management of
How will you continue to monitor this patient to
his electrolyte abnormalities, and preservation of renal
determine if this treatment was effective?
function. Given the patient’s laboratory values and
extremely elevated white cell count, his tumor lysis will Frequent ongoing assessment of electrolytes including
worsen and close monitoring is imperative. Aggressive monitoring for hyperkalemia, hyperphosphatemia, hyper-
hydration is the cornerstone for preventing renal injury uricemia, hypocalcemia, elevated lactate dehydrogenase,
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Chapter 15 School-age Child with Bruising 63

and metabolic acidosis are needed. These abnormalities are Table 15.3 Repeat basic metabolic profile.
the result of excessive cell turnover and breakdown.
Sodium 138 mEq/L
Because of his risk of impaired renal function from hyper-
uricema and hyperkalemia, his urine output, fluid balance, Potassium 4.2 mEq/L
and weight should be closely monitored. He should also Chloride 101 mEq/L
have regular cardiac and neurologic assessments due to Carbon dioxide 23 mEq/L
his associated risks. Blood urea nitrogen 15 mg/dL
Creatinine 0.4 mg/dL
Continuation of case: Diagnostic testing reveals the
following results. Glucose 135 mg/dL
Peripheral blood smear shows severe leukocytosis with Uric acid 1.6 mg/dL
marked predominance of blast-type cells, with very few Lactate dehydrogenase >2500 U/L
background neutrophils and microcytic anemia. There is Phosphorus 4 mg/dL
also severe thrombocytopenia. Peripheral flow cytometry
indicates T-lymphoblastic leukemia (90% blasts).
Chest radiograph shows interval marked enlargement Table 15.4 Repeat complete blood count.
of the cardiothymic silhouette involving both the cardiac
silhouette and hilar adenopathy, enlargement or uplifting White blood cell count 171 000/mm3
of the left pulmonary artery, perihilar interstitial promi- Hemoglobin 9 g/dL
nence, bibasilar atelectasis, small pleural effusions, and Hematocrit 27%
no pneumothorax (Figure 15.1). Platelet count 16 000/mm3
The patient is admitted to the pediatric oncology inpa-
Neutrophils 5%
tient service and continued on intravenous fluids at
Eosinophils 0%
Monocytes 1%
Lymphocytes 17%
Blasts 76%

3 L/m2 daily. He is given one dose of rasburicase and is

started on allopurinol. His diagnosis is confirmed as
T-lymphoblastic leukemia per flow cytometry of peripheral
blood. He is scheduled for bone marrow evaluation and
lumbar puncture to complete his diagnostic work-up
prior to initiating treatment. Repeat laboratory studies
are shown in Tables 15.3 and 15.4.
4 As the uric acid is normal, would you stop allopurinol?

As the uric acid is normal, would you stop

Figure 15.1 Chest X-ray. Source: Courtesy of Catherine M. Haut,
CPNP, Herman and Walter Samuelson Children’s Hospital at Sinai,
Baltimore, MD.
allopurinol?
The child’s uric acid responded well to rasburicase, but due
to ongoing tumor burden anticipate significant cell turno-
ver. This may worsen once chemotherapy is initiated, so
he should continue to receive allopurinol, aggressive hydra-
tion, and frequent monitoring of electrolytes (every 4–6
hours). In order to obtain accurate uric acid results follow-
ing rasburicase, blood must be collected in pre-chilled
tubes, immediately placed on ice, and assay completed in
less than 4 hours. Rasburicase will cause enzymatic degra-
dation of uric acid at room temperature so this is an impor-
tant step to ensure adequate results.

64 Cases in Pediatric Acute Care
Further Reading
Cairo, M.S., Coiffier, B., Reiter, A., et al. (2010).
Recommendations for the evaluation of risk and prophylaxis
of tumour lysis syndrome (TLS) in adults and children with
malignant diseases: an expert TLS panel consensus. British
Journal of Haematology 149: 578–586.
Coiffier, B., Altman, A., Pui, C., et al. (2008). Guidelines for the
management of pediatric and adult tumor lysis syndrome:
an evidence-based review. Journal of Clinical Oncology 26:
2767–2778.
Engorn, B. and Flerlage, J. (ed.) (2015). The Johns Hopkins
Hospital. The Harriet Lane Handbook, 20th edn.
Philadelphia, PA: Elsevier Saunders.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Howard, S., Jones, D., and Pui, C. (2011). The tumor lysis
syndrome. New England Journal of Medicine 364:
1844–1854.
Wilson, P.F. and Berns, J. (2014). Tumor lysis syndrome: new
challenges and recent advances. Advances in Chronic Kidney
Disease 21(1): 18–26.
Prescribing information for rasburicase is available online at
http://products.sanofi-aventis.us/elitek/elitek.html
(accessed on 4 June 2019).

16
Infant with Shunted Hydrocephalus
Mark Weber
Children’s Hospital of Philadelphia, Philadelphia, PA, USA
A 3-month-old twin 35-week gestation male with a past
medical history of shunted hydrocephalus presents to the
pediatric intensive care unit (PICU) with fever, erythema,
and swelling at his ventriculoperitoneal (VP) shunt site.
History of Present Illness
The infant was in his usual state of health when 5 days prior
to admission his mother noticed that his head circumfer-
ence appeared to have increased. She also noticed that he
had erythema, swelling, and clear drainage at the incision
site where his VP shunt was revised 15 days prior to pres-
entation. Two days before admission he developed a fever
of 38.6 C (101.5 F). He had no change in his mental status
or decreased oral intake. His mother called the neurosur-
gery clinic and she was advised to bring him to the local
emergency department (ED) for further evaluation. He
was then transferred to a local children’s hospital for fur-
ther management.
Past Medical History
He was born premature as twin A at 35 weeks’ gestation.
His hydrocephalus, diagnosed on a prenatal ultrasound,
was due to aqueductal stenosis. He was delivered via
planned cesarean section in a high-risk obstetrics unit
and transferred to the neonatal intensive care unit where
he was admitted for 3 weeks. He was found to have no
genetic abnormalities. He was discharged with a nasogas-
tric (NG) tube to augment his oral feeds. He has been gain-
ing weight well on his feeding regimen of oral/NG feeds. He
has a seizure disorder that was diagnosed during the time of
his previous VP shunt malfunction. He has remained on
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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65
maintenance levetiracetam with good seizure control. His
immunizations are up to date for age.
Past Surgical History
A VP shunt was placed at day 6 of life. He required a shunt
revision roughly 2 months later due to a proximal shunt
obstruction.
Family History
There is no significant family history. His twin has
remained healthy.
Current Status
On arrival at the ED, he is hypothermic to 35.7 C (96.3 F).
He is noted to be mottled so an intravenous catheter is
placed and he is given a 20 mL/kg bolus of 0.9% sodium
chloride. He is started on vancomycin at a dose of 15 mg/kg
every 6 hours and cefepime 50 mg/kg every 8 hours, both
at meningitic dosing. While in ED, he is awake with stable
mental status. Neurosurgery performs a shunt tap after a
0.05 mg/kg dose of morphine is administered for analgesia.
Laboratory samples are sent for blood culture, complete
blood count (CBC), basic metabolic panel, erythrocyte sed-
imentation rate (ESR), C-reactive protein (CRP), and a type
and screen (see Tables 16.1 and 16.2). The laboratory eval-
uation shows a leukocytosis of 18% on CBC and a CRP of
17 mg/L (normal range 0–0.9 mg/L). Computed tomogra-
phy (CT) of the brain shows soft tissue swelling and similar
levels of dilation of his lateral and third ventricles as his
most recent head CT (Figure 16.1).
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66 Cases in Pediatric Acute Care

Table 16.1 Basic metabolic profile. Table 16.3 Cerebrospinal fluid.

Sodium 137 mEq/L Color Xanthochromic

Potassium 4.5 mEq/L
Chloride 109 mEq/L White blood 358/mm3
cell count
Carbon dioxide 17 mEq/L
Red blood cell 1690/mm3
Blood urea nitrogen 13 mg/dL count
Creatinine 0.3 mg/dL Differential Segs 71%
Glucose 86 mg/dL Monocytes 15%
Lymphocytes 13%
Glucose <20 mg/dL (normal range 40–85 mg/dL)
Protein 1165 mg/dL (normal range 14–45 mg/dL)
Table 16.2 Complete blood count.

White blood cell count 23 500/mm3

Hemoglobin 8.6 g/dL Questions
Hematocrit 26.9%
Answer the following questions using the details provided.
Platelet count 655 000/mm3
Neutrophils 66% 1 Based on the data given, what is the differential diagno-
Eosinophils 2% sis and the most likely diagnosis for this child?
Monocytes 8% 2 What therapies are indicated for this child?
3 What laboratory studies will be useful in confirming
Lymphocytes 23%
your diagnosis?
4 What other factors will be important to manage in a
child with an externalized ventricular drain awaiting
shunt re-internalization?

Rationale and Evidence-based

Practice Explanation

Based on the data given, what is the differential

Figure 16.1 Head CT shows soft tissue swelling and similar
dilation of lateral and third ventricles. The shunt remains in good
condition within the lateral ventricle.
The cerebrospinal fluid (CSF) results (Table 16.3) are
concerning for infection. A preliminary Gram stain of the
CSF shows Gram-positive cocci in pairs and chains with
a CSF culture pending. He is admitted to PICU for close
neurologic monitoring and further management.
diagnosis and the most likely diagnosis for
this child?
Differential diagnoses for this child would include VP shunt
infection, VP shunt failure/obstruction, peritonitis, local
abscess, seroma or other source of sepsis not yet identified.
The most likely diagnosis for this child would be a VP
shunt infection due to the history of fever, erythema, swell-
ing, and known CSF leakage at the VP shunt insertion site.
A recent (<1 month) surgical intervention on his shunt also
puts him at increased risk of infection. Other common risk
factors for VP shunt infection include age under 5 years,
two or more previous shunt procedures, prematurity, a
neoplastic etiology for hydrocephalus, and postoperative
CSF leak.
What therapies are indicated for this child?
The mainstays of therapy for a VP shunt infection include
antibiotics and removal of the infected device. The choice
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Chapter 16 Infant with Shunted Hydrocephalus 67

of antibiotics can be guided by a history of previous infec- What other factors will be important to manage in
tions and culture results. Gram-positive organisms, includ- a child with an externalized ventricular drain
ing methicillin-resistant Staphylococcus aureus (MRSA), awaiting shunt re-internalization?
can be covered with vancomycin. The recommended dose
While awaiting shunt re-internalization the pending cul-
is 15 mg/kg every 6 hours. This dosing regimen will
tures should be monitored. The growth of any bacteria will
ensure sufficient penetration into the CSF, as vancomycin
help guide the choice and duration of antibiotics. If the
tends to have poor CSF penetration. A vancomycin trough
patient requires a prolonged course of intravenous antibio-
should be checked prior to the fourth dose and target
tics, the placement of a peripherally inserted central cath-
levels between 15 and 20 μg/mL are typical. The patient
eter (PICC) or other means of secure intravenous access
should also be placed on an antibiotic to cover Gram-
should be considered.
negative organisms. If the patient has been frequently
During this period the importance of maintaining the
hospitalized, an antibiotic that would cover Pseudomonas
child’s nutrition should not be forgotten. In smaller chil-
aeruginosa should be used. Either ceftazidime (third-
dren who have a significant amount of CSF drainage from
generation cephalosporin) or cefepime (fourth-generation
their external ventricular drain, serum sodium levels
cephalosporin) would be acceptable choices.
should be followed. The loss of sodium through the CSF
Another organism that should be considered is Cutibac-
can lead to hyponatremia. Hyponatremia can lower the
terium acnes (lipophilic anaerobic Gram-positive bacte-
child’s seizure threshold, making them more prone to sei-
rium; common skin commensal). This organism is
zures. If there is concern for seizure activity, an electroen-
known to be slow-growing and may take up to 10 days to
cephalogram and antiepileptic management would be
grow in culture. Cutibacterium acnes responds well to
indicated. If the serum sodium level is falling, the CSF out-
vancomycin and third-generation cephalosporins. The
put can be replaced with intravenous sodium chloride to
treatment time should be extended to 14 days if this
maintain a normal serum sodium level. Although very rare
bacterium is isolated in culture.
in cases of VP shunt infections, other sodium dysregulation
Device removal should also be considered for infected VP
syndromes may occur, such as syndrome of inappropriate
shunts. Removal of the device and placement of a tempo-
antidiuretic hormone (SIADH), diabetes insipidus, and
rary external ventricular drain will be required until the
cerebral salt wasting.
CSF cultures become clear.
Additionally, since the child’s hospital stay will likely be
greater than 1 week, physical therapy/occupational therapy
What laboratory studies will be useful in
and child life support will be integral in promoting a rapid
confirming your diagnosis?
recovery and successful transition back to home.
Several laboratory studies are important in the diagnosis of
an infected VP shunt. First, Gram stain and culture of the
CSF, preferably from the VP shunt, will be useful in iden-
tifying the causative organism. If possible, the culture Case Resolution
should be drawn from the VP shunt prior to the administra-
tion of antibiotics, although antibiotics should not be The following morning his VP shunt was removed and an
delayed in order to successfully obtain a culture. A cell external ventriculostomy drain was placed in the operating
count, protein and glucose from the CSF can also be helpful room. The cultures from the CSF grew methicillin-sensitive
in revealing a possible infection. CSF with an elevated Staphylococcus aureus (MSSA). He was treated with oxacil-
white blood cell count, elevated protein, and low glucose lin for 2 weeks via a PICC. His CSF cultures were followed
is suggestive of a bacterial infection. A blood culture with and trended for the 2 weeks to assure that the MSSA was
a serum CBC, CRP, and procalcitonin may reveal signs of cleared and that a secondary infection was not present.
an active infection. Additionally, an infectious disease con- At the completion of the 2 weeks a new VP shunt was
sult is recommended as it can be difficult to determine placed and he was discharged to home the following day.
appropriate antibiotic selection and optimal timing of
shunt re-internalization.

Further Reading
Conen, A., Walti, L.N., Merlo, A., et al. (2008). Characteristics associated infections in adults: a retrospective analysis over
and treatment outcome of cerebrospinal fluid shunt- an 11-year period. Clinical Infectious Diseases 47: 73–82.

68 Cases in Pediatric Acute Care
Erps, A., Roth, J., Constantini, S., Lerner-Geva, L., and
Grisaru-Soen, G. (2018). Risk factors and epidemiology of
pediatric ventriculoperitoneal shunt infection. Pediatrics
International 60: 1056–1061.
Liu, C., Bayer, A., Cosgrove, S.E., et al. (2011). Clinical practice
guidelines by the Infectious Diseases Society of America for
the treatment of methicillin-resistant Staphylococcus aureus
infections in adults and children: executive summary.
Clinical Infectious Diseases 52: 285–292.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Tamber, M.S., Klimo, P., Mazzola, C.A. et al. (2014). Pediatric
hydrocephalus: systematic literature review and evidence-
based guidelines. Part 8: Management of cerebrospinal
fluid shunt infection. Journal of Neurosurgery Pediatrics 14
(Suppl 1): 60–71.
Tunkel, A.R., Hasbun, R., Bhimraj, A. et al. (2017). Infectious
Diseases Society of America’s clinical practice guidelines for
healthcare-associated ventriculitis and meningitis. Clinical
Infectious Diseases 64: e34–e65.

17
Newborn with Poor Oral Intake and Irritability
Elaine Geary
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 15-day-old term infant is brought to the emergency
department (ED) with a 48-hour history of decreased oral
intake and irritability.
History of Present Illness
The infant is accompanied by mother. She was seen by her
primary care provider (PCP) for a well child visit 2 days
prior to presentation. A murmur was noted on ausculta-
tion, otherwise the examination was normal. Because of
the heart murmur, the infant was scheduled to see a
cardiologist the following day. The night following the
PCP visit, the infant was fussier and uncharacteristically
hard to settle, acting hungry but eating less. The infant
vomited the last three feeds.
Prior to presenting, the mother denied tachypnea, poor
perfusion, or poor growth. The infant had been taking
57–85 g (2–3 ounces) of Similac Advance every 3–4 hours,
enjoyed eating, fed effortlessly and has been gaining
weight. The mother denies any recent fever, cough,
congestion, vomiting, diarrhea or ill exposures.
Past Medical History
Delivered vaginally at term after an unremarkable gestation.
The infant went home with her and had no jaundice, is on no
medications, and has no allergies. Hepatitis B was adminis-
tered prior to discharge and baby passed a hearing screen.
Family and Social History
Unremarkable. Lives at home with both parents and a
healthy 3-year-old brother. No pets and no smoking in
the home.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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69
Current Status
In the ED, the infant’s electrocardiogram (EKG) demon-
strated a narrow-complex tachycardia at 280 bpm with a
regular rate. The examination demonstrated a blood
pressure in the right upper extremity of 72/38 mmHg, tach-
ypnea, irritability and pale skin, but no acute distress.
Pulses were palpable, capillary refill was approximately
3 seconds, and oxygen saturation was 89% on room air.
The cardiology team was called to evaluate the patient.
Questions
Answer the following questions using the details provided.
1 What is your differential diagnosis?
2 What would be your interventions to reduce the
heart rate?
3 What additional diagnostic studies or laboratory studies
should be ordered?
Rationale and Evidence-based Practice
Explanation
What is your differential diagnosis?
Differential diagnoses include sinus tachycardia, supraven-
tricular tachycardia including atrioventricular reentrant
tachycardia (AVRT) such as WPW syndrome, and atrioven-
tricular nodal reentry tachycardia (AVNRT)
What would be your interventions to reduce the
heart rate?
The infant was placed on a nasal cannula delivering oxygen
at 2 L/min. Intravenous (IV) access was initiated and vagal

70 Cases in Pediatric Acute Care
maneuvers (knees to chest, ice to the face) attempted. Nei-
ther maneuver was successful in reducing the heart rate.
What additional diagnostic studies or laboratory
studies should be ordered?
An EKG is obtained. A basic metabolic panel, complete
blood count, and glucose level were obtained at time of
IV placement (see Tables 17.1 and 17.2).
Continuation of case: The next set of vital signs included
temperature 37.2 C (99 F), heart rate 280 bpm, respiratory
rate 72 breaths per minute, oxygen saturation 94% on room
air, and blood pressure 68/35 mmHg. Rhythm on 12-lead
EKG (Figure 17.1) was a narrow-complex tachycardia
indicative of supraventricular tachycardia (SVT). Glucose
measured at 70 mg/dL. She was attached to the monitor
and defibrillation pads were placed.
4 Which medication(s) should be given?
5 What are the features of the EKG that would indi-
cate SVT?
6 What would have been the course of action had the
infant quickly decompensated or if the second dose
of adenosine had not been effective?
7 Why is it important to have a rhythm strip running
while the adenosine is administered?
8 What is the indication for the echocardiogram?
9 How do we know this patient does not have Wolff–
10
Parkinson–White (WPW) syndrome?
What about secondary prevention?
11 What is the pathophysiology of SVT?
Which medication(s) should be given?
While a rhythm strip was running, adenosine 100 μg/kg
rapid IV push was given with an immediate rapid normal
saline flush of 10 mL. There was a 2-second pause on the
EKG strip, then SVT resumed at 285 bpm. By this time,
the infant’s blood pressure had dropped to 55/30 mmHg,
her capillary refill was sluggish, and she was getting sleepy.
Table 17.1 Basic metabolic profile.
Sodium 134 mEq/L
Potassium 3.5 mEq/L
Chloride 101 mEql/L
Carbon dioxide 23 mEq/L
Blood urea nitrogen 11 mg/dL
Creatinine 0.2 mg/dL
Glucose 72 mg/dL
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Table 17.2 Complete blood count.
White blood cell count 15 000/mm3
Hemoglobin 14.2 g/dL
Hematocrit 39%
Platelet count 481 000/mm3
A second dose of adenosine was given at double the orig-
inal dose, 200 μg/kg rapid IV push followed by an immedi-
ate normal saline flush of 10 mL. After the second dose of
adenosine, there was a brief pause in rhythm followed by
sinus rhythm at 150 bpm.
Echocardiogram showed normal systolic and diastolic
function, patent foramen ovale (PFO), and peripheral pul-
monic stenosis (PPS).
Her heart murmur was consistent with PPS, a common
innocent murmur of the neonate associated with flow
acceleration across the branch pulmonary arteries. The
PFO, a small communication between the upper filling
chambers of the heart, is a remnant of fetal circulation
and is considered an incidental finding. Neither of these
findings would result in cardiovascular symptoms and nei-
ther are associated with SVT.
What are the features of the EKG that would
indicate SVT?
•• Heart rate greater than 220 bpm in an infant
Lack of rate variation, constant R–R interval
• Tachycardia unrelated to hydration status, analgesia,
or fever
• Sudden change in heart rate with conversion to sinus
rhythm
What would have been the course of action had
the infant quickly decompensated or if the second
dose of adenosine had not been effective?
In unstable patients (e.g. depressed mental status, hypoten-
sion, signs of shock) without IV access, or without response
to IV adenosine, the appropriate treatment is immediate
synchronized cardioversion with 0.5–1 J/kg. If this is not
effective, increase to 2 J/kg. The elimination half-life of
adenosine is 10 seconds, and therefore it should be given
in an IV line as close to the heart as possible. If the adeno-
sine is administered too slowly or with inadequate saline
flush, less of the medication may reach the heart, decreas-
ing its efficacy. For these same reasons, intraosseous
(injecting directly into the marrow of bone) may not be a
reliable route for adenosine administration.

Figure 17.1 12-Lead EKG. Source: Courtesy of Dr. Richard Garcia.
Why is it important to have a rhythm strip running
while the adenosine is administered?
It is vital for diagnosis and medical management of these
patients to record the rhythm prior to, during, and after
adenosine administration. These strips will yield informa-
tion on the exact mechanism of SVT when it terminates;
this information has implications for both treatment and
prognosis.
What is the indication for the echocardiogram?
There are two indications for the echocardiogram in this
infant. First, it is not known how long she has been in
SVT. An otherwise healthy infant with a structurally nor-
mal heart can tolerate SVT of rates to 220 bpm for hours
at a time. Sustained SVT, lasting longer than 6–12 hours,
will typically be associated with signs of congestive heart
failure, specifically irritability, tachypnea, poor feeding,
and pallor. If the tachycardia continues to go unrecognized,
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Chapter 17 Newborn with Poor Oral Intake and Irritability 71
it will eventually lead to tachycardia-induced cardiomyop-
athy, a life-threatening myocardial dysfunction. The second
indication for echocardiography is her murmur. On auscul-
tation, the murmur is consistent with PPS but, given that
SVT is more common in children with congenital heart dis-
ease, an echocardiogram is warranted to rule out significant
structural heart disease.
How do we know this patient does not have WPW
syndrome?
There are no delta waves noted in the EKG after cardiover-
sion, suggesting the accessory pathway conducts only retro-
grade. This is referred to as a “concealed pathway” because
the EKG during sinus rhythm appears normal.
What about secondary prevention?
There are significant practice variations in secondary pre-
vention in this population. SVT may not be promptly
detected in infants. They cannot complain of palpitations

72 Cases in Pediatric Acute Care
and they can tolerate high heart rates for a significant
period of time. When they present to medical attention,
there are generally some signs or symptoms of cardiac fail-
ure. While having caregivers check the heart rate may be
effective, it often adds significant stress and ownership to
the parents of the infant. For these reasons, many centers
use medications. Based on the available evidence, propran-
olol is most often used as the initial prophylactic therapy for
infants with SVT.
What is the pathophysiology of SVT?
SVT is an abnormally rapid heart rhythm originating above
the ventricles. It is the most common tachyarrhythmia seen
in the pediatric age group, with an estimated incidence
from 1 in 25 000 to as high as 1 in 250. The two most com-
mon forms of SVT in children are AVRT, which includes
the WPW syndrome, and AVNRT.
A reentrant rhythm involves two distinct pathways for
conduction with unidirectional block in one of the two
pathways. This creates a circuit through which an electrical
impulse can cycle repetitively in one direction (antegrade
or retrograde), with consequent rapid and regular ventric-
ular contractions. When the accessory pathway conducts
only retrograde it is referred to as a “concealed pathway”
because the EKG during sinus rhythm appears normal.
This is the type of dysrhythmia in this case presentation.
The hallmark feature of SVT is a very fast rhythm with
no rate variability.
The clinical presentation of this infant is typical, with
reports of atypical fussiness and poor feeding with no asso-
ciated fever or illness. Supplemental oxygen was instituted
to support the myocardium and the oxygen saturations
were low at 88% on room air. Tracheal intubation was
Further Reading
Chu, P., Hill, K.D., Clark, R.H., Smith, B., and Hornik, C.P.
(2015). Treatment of supraventricular tachycardia in
infants: analysis of a large multicenter database. Early
Human Development 91(6): 345–350.
Drago, F., Battipaglia, I,, and Di Mambro, C. (2018). Neonatal
and pediatric arrhythmias: clinical and electrocardiographic
aspects. Cardiac Electrophysiology Clinics 10(2): 397–412.
Park, M.K. (2014). Park’s Pediatric Cardiology for Practitioners,
6th edn, 414–418. Philadelphia, PA: Elsevier Saunders.
Schlechte, E.A., Boramanand, N., and Funk, M. (2008).
Supraventricular tachycardia in the pediatric primary care
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not indicated. While she was tachypneic, her respirations
were unlabored and effective. She was placed on the cardi-
orespiratory monitor and defibrillator patches were also
applied. This provided the method to record her rhythm
and enabled synchronized cardioversion if necessary. Vagal
maneuvers (glove containing ice applied to the face, above
the nose and mouth for 15–30 seconds; knees to chest) were
attempted while the IV was being placed but they were
unsuccessful. Treatment should not be delayed to adminis-
ter vagal maneuvers in unstable patients.
Serum was obtained at the time of IV placement to check
glucose and electrolytes, and to assess for evidence of
infection.
Adenosine has a very short half-life, its effects lasting
approximately 10 seconds. If the first bolus of adenosine
does not convert the SVT, two subsequent boluses of double
the initial dose may be administered 2 minutes apart. The
patient must be monitored for adverse drug reactions,
which may include facial flushing, hypotension, heart
block, bradycardia, asystole, atrial fibrillation, broncho-
spasm and, in the older patient, chest pressure.
If the SVT fails to respond to adenosine and the patient
remains hemodynamically stable, an electrophysiologist
will prescribe drug therapy depending on the type of
narrow-complex SVT and the patient’s heart function.
Synchronized cardioversion is the treatment of choice
anytime a patient appears compromised.
In patients who present with SVT in early infancy,
symptoms often resolve by 1 year of age and therapy
beyond that time may not be needed. However, some
patients may have recurrence later in childhood. By con-
trast, SVT tends to persist in children who present after
the age of 2 years.
setting: age-related presentation, diagnosis, and
management. Journal of Pediatric Health Care 62(9):
289–299.
Wackel, P.L. (2018). Palpitations and arrhythmia. In: Common
Cardiac Issues in Pediatrics (ed. J.N. Johnson and D.M.
Kamat), 154–156. Itasca, IL: American Academy of
Pediatrics.
Zimetbaum, P. (2016). Cardiac arrhythmias with
supraventricular origin. In: Goldman-Cecil Medicine, 25th
edn (ed. L. Goldman and A.I. Schafer), 356–367.
Philadelphia, PA: Elsevier Saunders.
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
73

18

An 8-Week-Old Infant with a Cough

Sarah Jane Garraty
Beacon Pediatrics, Rehoboth Beach, DE, USA

An 8-week-old female presents to her pediatrician’s office Past Surgical History

with signs and symptoms of progressively increasing work
of breathing, cough, runny nose, and wheezing. Frenectomy completed in the newborn nursery.

History of Present Illness Family History

This patient is an 8-week-old female who was brought to The infant’s mother has a history of anxiety and depression,
her pediatrician’s office for increasing work of breathing cholestasis, and asthma. She also has a history of cocaine
and wheezing over a 12-hour period. Her symptoms were and tobacco use. Her father had asthma as a child and
preceded by a 4-day history of nasal congestion and cough. her older brother has mild intermittent asthma.
The patient’s mother reports that she has been afebrile, but
has had decreased oral intake, with only one wet diaper in
the past 12 hours. Her older sibling, who is in daycare, has Current Status
had a runny nose and cough for the past week.
On presentation at her pediatric primary care office, she
weighed 3.97 kg and her heart rate was 170 bpm. Upon
inspection, she had intercostal and subcostal retractions
Past Medical History
and accessory muscle use. She had a regular rate and
rhythm on cardiac examination. On pulmonary examina-
The infant was born after a 40-week gestation, via sponta-
tion, her respiratory rate was 80 breaths per minute, and
neous vaginal delivery, with a cleft palate and mild neona-
her oxygen saturation was 92% on room air at rest. Wheez-
tal abstinence, not requiring medical therapy. Maternal
ing, rhonchi, and crackles were auscultated bilaterally on
history includes cocaine and tobacco use during preg-
lung examination.
nancy, and positivity for group B Streptococcus, but ade-
The patient was transported via emergency medical ser-
quately treated prior to delivery. The infant’s early
vices to the local community hospital. During transport, the
neonatal period was significant for tachypnea and poor
infant receives two doses of albuterol, with no significant
feeding. Tachypnea resolved rapidly, but she continued
improvement in her respiratory status.
to require feeding support because of a cleft palate and
tight lingual frenulum. She was observed in the neonatal
intensive care unit (NICU) for 24 hours. No further desa-
Questions
turations were noted during sleep or feedings during her
NICU stay. She was then transferred to the newborn nurs-
Answer the following questions using the details provided.
ery for further observation and feeding support prior to
discharge. 1 What is the differential diagnosis and the most likely
She has recently received 2-month vaccines. diagnosis?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

74 Cases in Pediatric Acute Care
2 What is the pathophysiology of the underlying
diagnosis?
3 What are the different management modalities and
what is their effectiveness in treating the underlying
disorder?
4 Are there any preventive or prophylactic treatment
modalities?
Rationale and Evidence-based Practice
Explanation
What is the differential diagnosis and the most
likely diagnosis?
The differential diagnoses include bronchiolitis, congenital
heart disease/heart failure, pneumonia, bronchomalacia,
tracheal/vascular rings, and gastroesophageal reflux dis-
ease (GERD).
The most likely diagnosis of this infant is bronchiolitis
due to the infant’s age (less than 12 weeks of age puts the
infant at risk for severe disease), reported symptoms of
upper respiratory tract infection over the last week, and
adventitious respiratory findings and signs of respiratory
distress that included tachypnea, intercostal and subcostal
retractions, wheezing, and crackles.
The acute nature of this presentation, without any previ-
ous history of stridor or wheezing, makes the diagnoses of
tracheal/vascular rings and bronchomalacia unlikely. Non-
specific chest radiograph findings, with the presence of
peribronchial cuffing, make bacterial pneumonia improba-
ble as the diagnosis. The diagnosis of heart failure or con-
genital heart disease is also not supported by the history of
weight gain, the acute nature of the presentation, chest
radiograph findings, improvement on high-flow nasal can-
nula, and no murmurs heard on cardiac examination.
What is the pathophysiology of the underlying
diagnosis?
Bronchiolitis is a disorder that most commonly occurs in
children younger than 2 years of age. The disorder is char-
acterized in the beginning by symptoms such as rhinorrhea
and cough, followed by inflammation and infection of the
lower respiratory tract (bronchioles). Bronchiolitis occurs
in the terminal and respiratory bronchioles. Bronchioles
are small airways (<2 mm in diameter) and lack cartilage
and submucosal glands. Bronchiolar injury from infection,
and the subsequent inflammatory response, lead to
increased mucus secretion, with mucous debris causing
bronchial obstruction and constriction, and air trapping
that leads to atelectasis. This results in reduced ventilation,
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causing a ventilation–perfusion mismatch, and also
increased work of breathing in an effort to overcome the
obstruction.
The clinical findings of this lower airway obstructive dis-
ease include wheezing, crackles/rales, and signs of
increased respiratory effort (tachypnea, grunting, nasal
flaring, intercostal retractions, subcostal retractions).
The etiology of bronchiolitis can be summarized as
follows:
• one-third of hospitalized cases involve two or more
viruses;
• respiratory syncytial virus (RSV) is involved in 75%
of cases;
•• parainfluenza virus;
influenza virus;
••
adenovirus;
rhinovirus is usually observed as a co-infection.
What are the different management modalities
and what is their effectiveness in treating the
underlying disorder?
Albuterol: Most randomized controlled trials do not pro-
vide significant evidence to indicate consistent benefit from
the use of β-adrenergic agents, such as albuterol, in the
management and treatment of bronchiolitis. Several
meta-analyses and systematic reviews have shown that
bronchodilators may provide short-term symptom
improvement but have little impact on overall outcome.
Children with severe bronchiolitis or with respiratory
failure were generally excluded from the trials, and there-
fore evidence cannot be generalized to these situations.
Epinephrine: This is an adrenergic agent with both α- and
β-receptor agonist activity. It has been used to treat upper
and lower respiratory tract illnesses and can be adminis-
tered as a nebulized solution. Evidence suggests that epi-
nephrine should not be routinely administered to
children hospitalized for bronchiolitis, except potentially
as a rescue agent in severe disease.
Corticosteroids: Although there is sufficient evidence to
indicate effectiveness of corticosteroids in the treatment
of respiratory diseases such as asthma and croup, there is
no evidence to indicate clinical effectiveness of corticoster-
oid use in the treatment of bronchiolitis.
High-flow nasal cannula: Heated humidified air and oxy-
gen is delivered through a nasal cannula (tubes) at higher
flow rates than can be achieved using conventional dry oxy-
gen delivery. This allows the comfortable delivery of high
flow rates of an air/oxygen blend which may improve ven-
tilation. This may lead to a reduced need for invasive respi-
ratory support (e.g. intubation) and may have a clinical

advantage over other treatments by preventing drying of
the upper airway.
In one study of 1472 patients, among infants with bron-
chiolitis who were treated with oxygen, those who received
high-flow oxygen therapy had significantly lower rates of
escalation of care due to treatment failure than those in
the group that received standard oxygen therapy. High-flow
nasal cannula use is becoming widely accepted because
of its relative safety and possible benefits (Franklin
et al., 2018).
Are there any preventive or prophylactic
treatment modalities?
Palivizumab is a monoclonal antibody produced by recom-
binant DNA technology. It is used in the prevention of RSV
infections. It is recommended for infants that are high risk
because of prematurity or other medical problems such as
congenital heart disease. Clinicians should not administer
palivizumab to otherwise healthy infants with a gestational
age of 29 weeks 0 days or greater.
Clinicians should administer palivizumab during the first
year of life to infants with hemodynamically significant
heart disease or chronic lung disease of prematurity defined
Reference
Franklin, D., Babl, F.E., Schlapbach, L.J., et al. (2018). A
randomized trial of high-flow oxygen therapy in infants with
Further Reading
American Academy of Pediatrics Committee on Infectious
Diseases and Bronchiolitis Guidelines Committee (2014).
Updated guidance for palivizumab prophylaxis among
infants and young children at increased risk of
hospitalization for respiratory syncytial virus infection.
Pediatrics 134(2): 415–420. doi: 10.1086/ahr.113.3.752.
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Chapter 18 An 8-Week-Old Infant with a Cough 75
as preterm infants less than 32 weeks 0 days’ gestation who
require greater than 21% oxygen for at least the first 28 days
of life. Clinicians should administer a maximum of five
monthly doses (15 mg/kg per dose) of palivizumab during
the RSV season (typically November to March) to infants
who qualify for palivizumab in the first year of life.
Case Progression and Resolution
At the local community hospital, the infant tested positive
for RSV and had a chest radiograph completed that indi-
cated peribronchial thickening with no evidence of focal
pneumonia. She was started on heated humidified high-
flow nasal cannula therapy (Vapotherm) and following
intravenous access was started on administration of intra-
venous fluids, consisting of a bolus of normal saline
20 mL/kg, followed by dextrose 5% and normal saline at
maintenance. Her symptoms improved with these mea-
sures, and she was transferred to the pediatric intensive
care unit (PICU) for further care. After 5 days in the PICU,
she was ready for transfer back to the general ward and was
discharged home on hospital day 9.
bronchiolitis. New England Journal of Medicine 378:
1121–1131.
Ralston, S.L., Lieberthal, A.S., Meissner, H.C., et al. (2014).
Clinical practice guideline: The diagnosis, management, and
prevention of bronchiolitis. Pediatrics 134(5): e1474–e1502.
doi: 10.1542/peds.2014-2742.

19
Desaturation in Postoperative Cardiac Surgery Infant
Lisa Kohr
Cincinnati Children’s Hospital, Cincinnati, OH, USA
History of Present Illness
This is a 3-day-old, 2.6-kg infant with a postnatal diagnosis
of total anomalous pulmonary venous drainage who is now
postoperative day 3 following complete repair.
Past Medical History
The infant (36 1/7 gestational age) was born via urgent
cesarean section due to fetal decelerations. The birthweight
was 2.54 kg. The Apgar scores were 4 at 1 minute, 6 at 5 min-
utes, and 7 at 10 minutes. The infant was placed on nasal
continuous positive airway pressure (CPAP) during line
placement; however, the cyanosis continued and the infant
was intubated and mechanically ventilated. A murmur was
noted and the local pediatric cardiac intensive care unit was
called. An echocardiogram was performed that demon-
strated obstructed total anomalous pulmonary venous
return to the hepatic veins. He was transported to the inten-
sive care unit, prepared for surgery, and then urgently
taken to the operating room where he underwent a com-
plete repair with ligation of the vertical vein and a suture-
less repair of the pulmonary veins to the left atrium.
He returned intubated and sedated on dopamine and
milrinone infusions as well as inhaled nitric oxide 20 ppm.
Family History
No history of heart disease, lung disease, blood diseases,
reactions to anesthesia or unexpected deaths. No ill contacts.
Current Status
During handoff 8 hours after return from the OR, you are
informed that the infant has been stable all day. The infant
returned from the operating room on a dopamine infusion
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77
that was weaned off 4 hours ago and a milrinone infusion
that was weaned by 50% 2 hours ago. The plan for that day
was to wean towards extubation. This has been a slow proc-
ess due to the infant being “sleepy,” so the sedation infu-
sions were also weaned. Since he has been awake, he has
experienced intermittent desaturation episodes responsive
to low-dose morphine and temporarily increasing the
FiO2 through the ventilator circuit.
You are called urgently to the bedside because the infant’s
oxygen saturation has fallen to 60%. The infant is currently
on an FiO2 of 0.5, ventilator rate of 15 breaths per minute,
pressure control of 22 cmH2O, positive end-expiratory
pressure (PEEP) of 3 cmH2O, pressure support of 8 cmH2O,
and inhaled nitric oxide of 10 ppm.
The vital signs and hemodynamics reveal heart rate of
189 bpm, respiratory rate 24 breaths per minute, blood pres-
sure 58/34 mmHg, right atrial pressure (RAP) 10 mmHg,
oxygen saturation (SpO2) 70%, cerebral near-infrared spec-
troscopy (NIRS) 43%, and regional cerebral infrared spec-
troscopy (rSO2 NIRS) 65%.
On examination, the infant is pale and cool, with +1
peripheral pulses. He is agitated with nasal flaring and
intercostal retractions. Breath sounds are heard through-
out. During the exam, the infant suddenly stops moving.
Vital signs are as follows: heart rate 89 bpm, respiratory rate
24 breaths per minute, blood pressure 52/32 mmHg, RAP
12 mmHg, SpO2 55%, cerebral NIRS 32%, rSO2 NIRS 45%.
Questions
Answer the following questions using the details provided.
1 Based on the data, what is your differential diagnosis?
2 What additional studies would be helpful?
3 What interventions should be implemented?
4 What is the pathophysiology of pulmonary hyperten-
sion crisis?

78 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
Based on the data, what is your differential
diagnosis?
The differential diagnoses for this desaturation event
include pulmonary hypertension crisis, obstructed pulmo-
nary veins, pulmonary edema, mechanical issues with the
ventilator, pulmonary parenchymal issue (e.g. pneumotho-
rax, effusion), cardiac tamponade, and seizures.
Based on the findings during this scenario, the most
likely diagnosis is pulmonary hypertension crisis.
What additional studies would be helpful?
An arterial blood gas, hemoglobin/hematocrit and lactate is
ordered and the results are shown in Tables 19.1 and 19.2.
A chest X-ray can be considered if there is concern for lung
disease, signs of intercurrent illness, or suspected malposi-
tion of endotracheal tube.
What interventions should be implemented?
The primary goal should be to decrease PVR and support the
hemodynamics. Bagging the patient with 100% oxygen may
increase HR and oxygen saturation as well as simultaneously
considering mechanical issues triggering the event. Addi-
tional interventions that should occur in rapid succession
include optimizing mechanical ventilation (e.g. increase
ventilatory rate or tidal volume), initiation of an epinephrine
infusion, administration of a muscle relaxant agent, escala-
tion of sedation and analgesia, increasing inhaled nitric
oxide to 40 ppm, preparing code medications, and adminis-
tration of sodium bicarbonate, calcium, and a fluid bolus.
Table 19.1 Arterial blood gas analysis.
pH 7.16
PCO2 76 mmHg
PaO2 32 mmHg
Bicarbonate 14 mEq/L
Base excess –11
Lactate 8 mmol/L
Ionized calcium 1 mmol/L
Table 19.2 Hemoglobin/hematocrit.
Hemoglobin 15.3 g/dL
Hematocrit 45%
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What is the pathophysiology of pulmonary
hypertension crisis?
Pulmonary hypertension is defined as a mean pulmonary
artery pressure (PAP) above 25 mmHg with a normal pul-
monary capillary wedge pressure or a pulmonary artery
pressure greater than half systemic. A pulmonary hyper-
tensive crisis occurs when elevated PAP progresses to
the point of causing significant ventricular dysfunction,
leading to bradycardia and eventually pulseless electrical
activity. There is an increase in left atrial pressure due
to the interventricular septum shifting from right to left
as a result of the elevated right ventricular pressure. This
results in acute left ventricular diastolic dysfunction. There
is decreased end-tidal CO2 (found to be 18 mmHg in this
scenario) due to poor gas exchange from limited pulmo-
nary blood flow. The diagnosis is dependent on the past
medical history, physical examination, and hemodynamic
data.
Risk factors include the diagnosis and weight. Obstructed
pulmonary veins cause pulmonary congestion due to the
inability of the pulmonary blood flow to return to the left
atrium. Initial signs of a pulmonary hypertensive event
include tachycardia, an elevation in RAP due to an increase
in right ventricular afterload, hypotension due to decreased
cardiac output, and desaturation due to constriction of pul-
monary vasculature. Triggers of a pulmonary hypertensive
event include acidosis, agitation, pain, hypothermia, suc-
tioning, and suboptimal ventilation. In this scenario, the
infant awoke from sedation and developed agitation, which
may have triggered the event.
While the infant is at risk for experiencing the other diag-
noses, generally, obstructed pulmonary veins occurs later
in the postoperative trajectory. Pulmonary edema may be
present, but there is no evidence except for desaturation
and an elevation in RAP. Mechanical issues with the ven-
tilator and pulmonary parenchymal issues may be present
but would generally be reflected in the ventilatory flow
loops. Cardiac tamponade and seizures generally occur
within the first 24–48 hours after surgery.
The treatment plan for a pulmonary hypertensive crisis is
targeted at reducing pulmonary vascular resistance and
supporting cardiac output. Management for pulmonary
hypertension crisis should be immediate and aggressive
to avoid further cardiac deterioration. The management
of pulmonary hypertension includes the administration
of pulmonary vasodilators such as oxygen and nitric oxide,
which stimulates cyclic GMP. Sedation, analgesia, and neu-
romuscular blockade will assist in eradicating the trigger
for the event. Cardiac output can be augmented with cal-
cium chloride boluses, epinephrine for inotropy, fluid
boluses to augment preload, and sedation/analgesia/muscle
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Chapter 19 Desaturation in Postoperative Cardiac Surgery Infant 79

relaxation to decrease stress sympathetic stimulation. Vaso-
active medications are used to avoid systemic hypotension
and support coronary perfusion. Tachycardia should be
avoided to allow for adequate fill and ejection of the right
ventricle. The milrinone infusion should be increased
once adequate preload and blood pressure is achieved. Mor-
phine and nitric oxide are given for pulmonary vasodilation.
Sodium bicarbonate is administered to correct acidosis.
Ventilation goals should include maintaining the PEEP
and mean airway pressures at lowest levels to provide
functional residual capacity (FRC). If the patient continues
to deteriorate, extracorporeal membrane oxygenation sup-
port should be anticipated.
Case Resolution
Following your interventions, the infant eventually stabi-
lizes following aggressive medical management.

References
Kohr, L. and Nieves, J. (2015). Pulmonary hypertension. In: Oishi, P. and Fineman, J.R. (2016). Pulmonary hypertension.
Pediatric Cardiac Intensive Care Handbook (ed. M. Jones, Pediatric Critical Care Medicine 17(8 Suppl 1): S140–S145.
D. Klugman, R. Fitzgerald, et al.), 39–41. Washington, DC:
Pediatric Intensive Care Books.

20
Pre-school Child with Seizures
Heather Herrera and Rachel Luthen
Children’s Hospital of San Antonio, San Antonio, TX, USA
A 22-month-old male with a history of hypoxic ischemic
injury at birth with subsequent cerebral palsy and seizure
disorder presents with seizures and respiratory distress.
History of Present Illness
Approximately 2 days prior to presentation, this child had
upper respiratory infection (URI) symptoms of congestion
and cough without fever. Today, he began to experience
twitching of the right upper extremity every 10 minutes,
which began mid-morning. Approximately 30 minutes later,
mother administered home rectal diazepam 1 mg to abort
the seizures. Despite administration of diazepam, he devel-
oped generalized convulsions 15 minutes after administration
of diazepam and the mother called the emergency medical
services (EMS). EMS providers administered a second dose
of diazepam 1 mg without effect. Upon arrival to the children’s
emergency department, an intravenous catheter was placed
and lorazepam 0.1 mg/kg administered. He received a total
of four doses of lorazepam without successful resolution of
the seizures, so he was subsequently given a loading dose of
phenobarbital. It was noted at this time that he had been
having convulsive seizures for 35 minutes. Despite the
phenobarbital load, he continued in status epilepticus. He
developed respiratory failure with hypotension and required
endotracheal intubation and a fluid bolus of 20 mL/kg of
isotonic fluid. He was then transferred to the pediatric inten-
sive care unit (PICU) where he was immediately placed on
continuous electroencephalogram (EEG) monitoring and
care was continued.
Past Medical History
He was a term delivery to a 20-year-old mother with an
uncomplicated pregnancy. However, his delivery was
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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81
complicated by meconium aspiration and hypoxia with
subsequent development of hypoxic ischemic encephalop-
athy. He required a 3-week stay in the neonatal intensive
care unit. A neurology consult was completed and he ulti-
mately required initiation of phenobarbital, clonazepam,
and levetiracetam to achieve seizure control. The child con-
tinued on this regimen with intermittent adjustments for
growth with adequate seizure control until approximately
1 month ago when he developed breakthrough seizures.
He is up to date on his immunizations and he has been
in close contact with his older sibling who attends daycare
and always has URI symptoms.
Past Surgical History
Circumcised at birth.
Family History
Parents are healthy and there is no history of seizures.
Current Status
On arrival at the PICU, the patient was having subclinical
seizures, noted by the neurologist as non-convulsive refrac-
tory status epilepticus on EEG. A propofol infusion is
initiated with the goal of burst suppression to abort the
seizures. During this time, the patient requires two addi-
tional boluses of isotonic fluid 20 mL/kg and a norepineph-
rine drip to maintain adequate blood pressure. After several
titrations of the propofol infusion, cessation of status
epilepticus is achieved. A basic metabolic profile and com-
plete blood count are obtained (see Tables 20.1 and 20.2).
No meningeal signs or history of fever are noted; lumbar
puncture is not completed. Other results are as follows.
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82 Cases in Pediatric Acute Care

Table 20.1 Basic metabolic profile. considered, including complex febrile seizures, meningitis,
encephalitis, traumatic brain injury, syncope, and meta-
Sodium 139 mEq/L
bolic disorders. With a history of seizures and the need
Potassium 3.8 mEq/L for three antiepileptic drugs (AEDs), the most likely diag-
Chloride 109 mEq/L nosis for this child is status epilepticus secondary to his
Carbon dioxide 21 mEq/L known seizure disorder.
Blood urea nitrogen 12 mg/dL
Creatinine 0.4 mg/dL What are the essential pieces of the initial
Glucose 221 mg/dL
evaluation?
An initial quick physical examination at presentation
should focus and assess respiratory and cardiac status.
Table 20.2 Complete blood count.
An adequate airway should be established if there is
White blood cell count 14 200/mm3 evidence of respiratory compromise and supportive care
Hemoglobin 11.2 g/dL must be initiated. Blood and urine studies should be sent
Hematocrit 34.2%
for serum glucose (as well as point-of-care glucose), serum
electrolytes, blood gas and pH, a complete blood count,
Platelet count 320 000/mm3
urine and blood toxicology studies, and serum AED levels.
An evidence-based review found that almost one-third of

•• Blood culture: pending.

Respiratory viral panel: rhinovirus/enterovirus positive.
children who presented in status epilepticus had drug levels
that were subtherapeutic. These findings can impact both

• Toxicology: levetiracetam level therapeutic, phenobarbi-

tal level subtherapeutic.
management and outcome. Critical laboratory studies
should be completed to identify any electrolyte abnormal-

• Urine toxicology screen: benzodiazepines positive,

remainder negative.
ities and can also reveal metabolic disorders that can pres-
ent with seizures. Key electrolyte abnormalities that can

• EEG (read by neurology): new-onset refractory status epi-

lepticus with persistent generalized periodic epileptiform
discharges (GPED) as well as right periodic lateralizing
epileptiform discharges (PLED), then bilateral PLEDs
which have been difficult to suppress (see Figure 20.1).
Questions
Answer the following questions using the details provided.
1 Based on the data described, what are the differential
diagnoses and the most likely diagnosis for this child?
2 What are the essential pieces of the initial evaluation?
3 What is the best way to treat this problem?
4 Would you obtain any other diagnostic studies?
5 What is the definition of status epilepticus?
Rationale and Evidence-based Practice
Explanation
Based on the data described, what are the
differential diagnoses and the most likely
diagnosis for this child?
When a child presents to the emergency department with
prolonged seizures, several differential diagnoses must be
lead to seizures are hyponatremia, hypoglycemia, hypo-
magnesemia, and hypocalcemia. Metabolic studies should
be considered if there is suspicion of inborn errors of metab-
olism and children with these disorders can present with
altered mental status and seizures and may have elevated
ammonia levels, acidosis, or hypoglycemia. Many children
with acute seizures will have elevated blood glucose levels
that do not require intervention, but acute hypoglycemia
should be treated promptly (except in those patients who
are on a ketogenic diet). Brain glucose levels may be low-
ered with continued seizures, and when hypoxemia is also
present brain lactic acidosis and neuronal damage may be
worsened.
What is the best way to treat this problem?
The goal of therapy for status epilepticus is rapid termina-
tion of both clinical and electrical seizure activity. Timely
cessation of status epilepticus reduces the associated mor-
bidity and mortality. Appropriate treatment may be hin-
dered by therapies aimed only at reduction in seizures
rather than cessation, by the use of ineffective therapies
such as sedatives and/or paralytics, and by the administra-
tion of inadequate doses of anticonvulsant medications.
The initial phase for treatment of seizures begins when
seizure duration reaches 5 minutes. A benzodiazepine is
recommended as the initial therapy of choice. This includes
intravenous lorazepam, intramuscular midazolam, and

Figure 20.1 EEG. Source: Courtesy of Jennifer Joiner.
intravenous diazepam as these medications have demon-
strated efficacy, safety and tolerability. Although intrave-
nous phenobarbital is an established medication with
good tolerability, it is not recommended as first-line ther-
apy as it requires a slower rate of administration when com-
pared to the benzodiazepines. In the pre-hospital setting or
in situations where the recommended benzodiazepines are
not available, rectal diazepam, intranasal midazolam, and
buccal midazolam are reasonable therapy options. Initial
therapy should consist of single, adequate full doses rather
than smaller multiple doses. Intravenous lorazepam and
diazepam can both be repeated at full doses if needed.
The second phase of therapy begins when seizure dura-
tion reaches 20 minutes and ends with cessation of seizures
or when seizures last for 40 minutes despite continuation of
treatment. In second-line therapy, fosphenytoin, valproic
acid, and levetiracetam are recommended. There is no evi-
dence to support one of these medications being superior to
the others. Intravenous phenobarbital is also a reasonable
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Chapter 20 Pre-school Child with Seizures 83
alternative if none of the other recommended second-line
medications are available.
The third phase of seizure treatment begins when sei-
zures have lasted 40 minutes or longer. There is no clear
evidence to guide therapy in this phase. When compared
with initial therapy, second-line therapy is not as effective,
just as third-line therapy is also less effective than second-
line therapy. Third-line treatment choices include repeat-
ing second-line therapy or using anesthetic doses of
thiopental, midazolam, pentobarbital, or propofol, with
continuous EEG monitoring being recommended for all
third-line therapies. A patient is considered to have refrac-
tory epilepsy when seizures continue despite first- and sec-
ond-line treatments, the seizure has lasted longer than
1 hour, or general anesthesia is required. Throughout all
phases of treatment, until the patient returns to baseline,
supportive symptomatic care should be provided.
When treating seizures with benzodiazepines and other
AEDs, the most common treatment-related adverse events

84 Cases in Pediatric Acute Care
are respiratory and cardiac depressive symptoms. Cardiac
monitoring with frequent vital sign checks should be insti-
tuted. In most cases, status epilepticus itself does not cause
hypotension; however, many of the medications that are
used to treat it do. If hypotension is observed prior to the
administration of AEDs, consideration should be given to
a possible underlying infectious process, meningitis or sep-
sis. The rate of respiratory depression in patients with con-
vulsive status epilepticus treated with benzodiazepines is
lower than in patients with convulsive status epilepticus
treated with placebo, which indicates that respiratory pro-
blems are an important consequence of untreated convul-
sive status epilepticus. Many children will develop
hypotension or bradycardia secondary to AED therapy
and vasopressor and fluid support will be initiated.
If there is concern for meningitis or other infectious eti-
ology prompting seizure activity, antibiotics should be
initiated promptly. Antibiotics should not be held until a
lumbar puncture can be performed if there is concern for
systemic or central nervous system infection.
Would you obtain any other diagnostic studies?
There are no other diagnostic studies indicated for this
child at this time. To evaluate for diagnoses beyond an eti-
ology of status epilepticus secondary to known seizure dis-
order, a lumbar puncture could be done to determine
meningitis or encephalitis. Neuroimaging is indicated in
children for whom status epilepticus is the first presenta-
tion of epilepsy as well as in those children who do not fol-
low a typical recovery course after treatment for status
epilepticus or if trauma is suspected or known. Computed
tomography (CT) can be done quickly and should be per-
formed in patients with concern for infection to evaluate
for an intracranial mass or for increased intracranial pres-
sure prior to a lumbar puncture. Magnetic resonance ima-
ging (MRI) has better yield for determining the underlying
etiology of seizures and can be done once the patient has
been stabilized and seizure control has been achieved.
Further Reading
Alford, E.L., Wheless, J.W., and Phelps, S.J. (2015). Treatment
of generalized convulsive status epilepticus in pediatric
patients. Journal of Pediatric Pharmacology and
Therapeutics 20(4): 260–289. doi: 10.5863/1551-6776-
20.4.260.
Glauser, T., Shinnar, S., Gloss, D., et al. (2016). Evidenced-
based guideline: treatment of convulsive status epilepticus
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What is the definition of status epilepticus?
Brief short seizures are those seizures that last less than
5 minutes, while prolonged seizures are defined as those
lasting between 5 and 30 minutes. Status epilepticus is
defined as more than 30 minutes of either continuous sei-
zure activity or two or more sequential seizures without full
recovery of consciousness between seizures. The 30-minute
definition of status epilepticus is based on the duration of
status epilepticus that may potentially lead to permanent
neuronal injury. Once a seizure lasts more than 5 minutes,
it is likely to become prolonged. Seizure treatment proto-
cols have used a 5-minute definition to minimize the risk
of seizures reaching 30 minutes and the potential adverse
events associated with unnecessarily intervening on brief
self-limiting seizures.
Status epilepticus can present in several different
forms: convulsive status epilepticus consisting of repeated
generalized tonic–clonic seizures with persistent postictal
depression of neurological function between seizures;
non-convulsive status epilepticus in which seizures pro-
duce a continuous “epileptic twilight” state; or repeated
partial seizures that manifest as focal motor signs, focal
sensory symptoms, or focal impairment of function that
is not associated with altered awareness.
Case Resolution
The toddler had home medications restarted on hospital
day 2 with increase in phenobarbital dosing determined
by neurology. Blood cultures were negative at 48 hours
and the respiratory viral panel was positive for rhinovirus
infection. Blood pressure normalized once the propofol
was stopped and he was extubated on hospital day 3. He
was discharged home one week after admission with return
to baseline mental status.
in children and adults. Report of the guideline committee of
the American Epilepsy Society. Epilepsy Currents 16(1):
48–61.
Trinka, E. and Kalviainen, R. (2016). 25 years of advances in
the definition, classification, and treatment of status
epilepticus. Seizure 44: 65–73. doi: 10.1016/j.
seizure.2016.11.001.

21
Child with Blood in Stool
Brittany Cook and Darby Tozer
Beaumont Hospital, Troy, MI, USA
A 4-year-old male with a history of constipation presented
to the emergency department (ED) with concern for blood
in his stool.
History of Present Illness
The child had one episode of blood streaks in his stool on the
day of presentation. He had also been experiencing intermit-
tent abdominal cramping as well as two episodes of non-
bloody watery stools. No fever, chills, vomiting or appetite
changes. No cough, nasal congestion, sore throat or rash.
His sibling was recently ill with vomiting. No recent travel.
Past Medical History
He was born full-term without complications. He has a his-
tory of bladder spasms and constipation. His medications
include oxybutynin daily for bladder spasms and controls
constipation with diet modifications and daily polyethylene
glycol. He has never been hospitalized and does not take
any other daily medications. His immunizations are up to
date for his age, excluding annual influenza vaccine.
Past Surgical History
Bilateral myringotomy tubes placed at 2 years of age for
recurrent otitis media.
Family History
His father has irritable bowel syndrome. His parents deny a
family history of Crohn’s disease, colitis, or other gastroin-
testinal disorders.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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85
Current Status
On arrival at the ED, he is alert and interactive and
appears well. On palpation, his abdomen is soft and
non-tender with active bowel sounds throughout. Stool
guaiac test is positive. His rectal examination is without
fissures or hemorrhoids. An abdominal radiograph is
obtained which is suggestive of a focal ileus as there are
dilated loops of small bowel in the right lower quadrant
with an air–fluid level. The patient tolerates oral fluids
in the ED without complaints of pain or emesis. He is
therefore deemed safe for discharge with instructions to
follow up with his primary care provider (PCP) and a
diagnosis of viral gastroenteritis. Four days later, he
returns to the ED for daily non-bloody episodes of diar-
rhea since discharge. He has also had two episodes of
non-bloody, non-bilious emesis over the past 2 days with
new-onset fever on the day of presentation, with a maxi-
mum temperature of 38.3 C (101 F). He received a
weight-appropriate dose of acetaminophen just prior to
arrival to ED. Mother states that he was refusing to drink
fluids or eat food on the day of presentation. He was eval-
uated at PCP’s office earlier in the day and sent to the ED
for further evaluation. On arrival at the ED, he is afebrile
with a pulse of 120 bpm, blood pressure 118/96 mmHg,
respiratory rate of 24 breaths per minute, and pulse oxim-
etry of 97% on room air. He appears pale with dry mucous
membranes, and delayed capillary refill of 3–4 seconds.
He is noted to have significant pallor. A small bruise is
noted on the right shin. His abdominal examination is
unchanged from his prior visit, revealing a soft, non-
distended abdomen without tenderness or flank pain.
A complete metabolic panel, complete blood count, and
urine analysis were obtained, with laboratory results as
noted in Tables 21.1–21.3.
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86 Cases in Pediatric Acute Care

Table 21.1 Complete metabolic profile. 2 Based on the patient’s history and laboratory work,
what is the most likely diagnosis for this child?
Sodium 134 mEq/L
3 What is the most common cause of this condition?
Potassium 6.2 mEq/L 4 What are some other causes of this condition?
Chloride 96 mEq/L 5 What treatments should be initiated in the ED for
Carbon dioxide 13 mEq/L this child?
Blood urea nitrogen 167 mg/dL 6 What additional testing and therapies should be
Creatinine 3.9 mg/dL considered?
Glucose 68 mg/dL
Aspartate aminotransferase 146 U/L
Alanine aminotransferase 52 U/L Rationale and Evidence-based
Bilirubin (total) 1.8 mg/dL Practice Explanation

What differential diagnoses should you consider

Table 21.2 Complete blood count.
for this case?
Differential diagnoses for this child include hemolytic
White blood cell count 9000/mm3
uremic syndrome, infectious colitis, inflammatory
Hemoglobin 6.2 g/dL bowel disease, disseminated intravascular coagulation,
Hematocrit 18.3% and intussusception.
Platelet count 91 000/mm3
Neutrophils 65% Based on the patient’s history and laboratory
Eosinophils 0% work, what is the most likely diagnosis for
Monocytes 13% this child?
Lymphocytes 22% The most likely diagnosis of this patient’s clinical presenta-
Bands 0% tion is shiga-like toxin-producing Escherichia coli (STEC)
hemolytic uremic syndrome (HUS). Shiga-like toxin HUS
is a disorder that results most commonly from an infection
in the gastrointestinal tract that produces toxic substances.
Table 21.3 Urinalysis.
This results in a combination of microangiopathic hemo-
Color Dark yellow lytic anemia, thrombocytopenia, and acute kidney injury.
Clarity Cloudy The child’s laboratory results displayed a triad of anemia,
Bilirubin Positive
thrombocytopenia, and acute kidney injury, with a hemo-
globin of 6.2 g/dL, platelets 91 000/mm3, an elevated blood
Ketone 30 mg/dL
urea nitrogen of 167 mg/dL, and creatinine of 3.9 mg/dL, all
Specific gravity 1.020
consistent with HUS. The patient also exhibited clinical
Blood 3+ manifestations associated with a prodromal illness that is
Protein >300 mg/dL consistent with STEC-HUS, including abdominal pain,
Nitrites Negative vomiting, and bloody diarrhea. Other signs of HUS include
Leukocyte esterase 1+ fever, bruising, low urine output, pallor, and petechiae. In
Red blood cells 3–10 cells per high-power field cases of significant metabolic derangements, encephalopa-
Casts, hyaline >20 cells per high-power field thy or seizures can also be present.
Bacteria 2+
What is the most common cause of this condition?
The most common cause of STEC-HUS is associated with
Questions
gastrointestinal infection with Escherichia coli 0157:H7.
In this case, the patient’s mother reported that 5 days prior
Answer the following questions using the details provided.
to presentation, the patient was at an apple orchard where
1 What differential diagnoses should you consider for he consumed unpasteurized cider, which can harbor E. coli
this case? bacteria. Risk of exposure to infecting agents that can lead

to HUS can result from ingestion of raw meat, unpasteur-
ized foods, swimming in contaminated bodies of water,
and contact with farm animals.
What are some other causes of this condition?
While STEC is the most common cause of pediatric HUS,
other infections are associated with HUS. Streptococcus
pneumoniae is a cause of HUS and can be seen in children
diagnosed with pneumonia, meningitis, sinusitis, and otitis
media. HUS may also present with coexisting diseases or
conditions, such as transplantation, malignancy, autoim-
mune diseases, or kidney disease including malignant
hypertension or nephropathy. In the past two decades, an
additional form of HUS caused by dysregulation of the com-
plement pathway, called atypical HUS, has been described.
What treatments should be initiated in the
ED for this child?
There is no therapy to stop the cascade of HUS; therapy is
supportive. The treatments for HUS that would be initiated
in the ED include strict fluid and electrolyte management,
blood and/or platelet transfusion, and transfer to the inten-
sive care unit or a higher-level care facility. There is evi-
dence to support the role of volume expansion to
enhance outcomes and minimize renal replacement ther-
apy requirements in patients with HUS. This child received
a normal saline bolus of 10 mL/kg while in the ED, using a
conservative volume due to concern for fluid overload from
associated renal failure. Blood products were held in the ED
per the recommendation of the hematology service, who
advised that the patient would receive them on arrival to
the pediatric intensive care unit (PICU). Stool and urine
Further Reading
Ardissino, G., Tel, F., Possenti, I., et al. (2017). Early volume
expansion and outcomes of hemolytic uremic syndrome.
Pediatrics 137(1): e20152153. doi: 10/1542/peds.2015-2153.
Cody, E.M. and Dixon, B.P. (2019). Hemolytic uremic
syndrome. Pediatric Clinics of North America 66(1):
235–246.
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Chapter 21 Child with Blood in Stool 87
cultures were obtained to assist in identifying the inciting
bacterial agent.
What additional testing and therapies
should be considered?
Supportive care including maintenance of circulating vol-
ume and correction of electrolyte imbalances is a priority
in HUS. Close monitoring and management of blood pres-
sure is required. Because of the degree of azotemia, dialysis
should be discussed at the receiving facility. Although his-
torically contraindicated, use of antibiotics remains a con-
troversial topic, requiring consideration of risks and
benefits associated with therapy. Nutritional support with
parenteral nutrition due to prolonged colonic inflamma-
tion may be needed, or nutritional support with nasogastric
tube feedings if prolonged appetite suppression is present
after functional intestinal recovery is achieved. Prior to
any invasive procedures (e.g. insertion of a hemodialysis
catheter), the platelet count should be evaluated and the
patient transfused if indicated to reduce the risk of bleed-
ing. Depending on the procedure and associated risk of
bleeding, a higher baseline platelet count may be desired.
Case Resolution
The patient required placement of a dialysis catheter and
hemodialysis for 5 days. His urine output, fluid balance,
electrolytes, and hematologic laboratory measures steadily
improved over his 12-day PICU hospitalization. He was dis-
charged home after 18 days of hospitalization.
Fakhouri, F., Zuber, J., Bacchi, V.F., et al. (2017). Haemolytic
uraemic syndrome. Lancet 390(10095): 681–696.
Grisaru, S., Xie, J., Samuel, S., et al. (2016). Associations
between hydration status, intravenous administration, and
outcomes of patients infected with shiga toxin-producing
Escherichia coli. JAMA Pediatrics 171(1): 68–76.
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89

22

Osteomyelitis Case Study

Kim Steanson
Vanderbilt University College of Nursing, Nashville, TN, USA

A 13-year-old boy presents with a 6-day history of fever and
increasing pain to his left ankle.
History of Present Illness
This 13-year-old boy has had 7 days of progressively worsen-
ing left ankle pain with fever. He is experiencing tenderness
over the left ankle and inability to bear weight and ambulate.
portion of his distal tibia. The rest of his physical examina-
tion is unremarkable.
Vital signs in the ED include temperature 37.4 C (99.3 F),
heart rate 86 bpm, respiratory rate 20 breaths per minute,
and blood pressure 118/62 mmHg. A complete blood count,
C-reactive protein (CRP), and blood culture are ordered.
A plain film radiograph is obtained of the left ankle. Results
of complete blood count are shown in Table 22.1. Other
results are as follows.

••
He has been treating the pain with acetaminophen and
ibuprofen, but the over-the-counter medications are no C-reactive protein: 67 mg/dL.
Blood culture: pending.

•
longer providing adequate pain relief. He has not had any
ill contacts or recent trauma to the ankle/extremity. He
has had a poor appetite and malaise over the last 3 days.
Left ankle plain film radiograph: normal bone minerali-
zation; no fracture or focal bony lesion; joint spaces
appear preserved; no appreciable joint effusion.

Past Medical History

He is a healthy child. All immunizations are up to date
including the influenza vaccine this season. No prior hospi-
talizations or surgeries.
Family History
Paternal grandfather with hypertension. Maternal grand-
mother with type 2 diabetes mellitus. He lives with his
mother, father, and two younger brothers.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what is the differential diagno-
sis and biggest concern that would prompt further treat-
ment and investigation?
2 What additional imaging would be appropriate
and why?
3 Which antibiotics should be initiated?
4 What is the duration of therapy?

Rationale and Evidence-based Practice

Current Status
Explanation
On arrival to the emergency department (ED), he is slightly
Based on the data given, what is the differential
irritable but alert and cooperative. He is lying on the bed
diagnosis and biggest concern that would prompt
and is able to move all extremities, but with noticeable gri-
further treatment and investigation?
macing when asked to move his left lower extremity. He
reports significant pain with external and internal rotation Differential diagnoses for this patient includes osteomyeli-
of his left ankle and significant tenderness to the medial tis, septic arthritis, fracture, and toxic synovitis.

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

90 Cases in Pediatric Acute Care
Table 22.1 Complete blood count.
White blood cell count 13 000/mm3
Hemoglobin 11 g/dL
Hematocrit 34%
Platelet count 232 000/mm3
Toxic synovitis has a milder presentation than osteomy-
elitis or septic arthritis. Patients with toxic synovitis often
do not have concomitant fever. The white blood cell count
(WBC) and CRP are typically not elevated and patients
usually retain the ability to bear weight on the affected
extremity. Fracture has been excluded based on the radio-
graph findings. The most likely diagnoses for this child are
osteomyelitis and septic arthritis. Osteomyelitis and septic
arthritis may present alone or together. Both infections
present with pain to the affected joint and limitation of
mobility of the joint. Fever is also a common presenting
symptom. The WBC and CRP are both elevated. The
combination of these symptoms with the absence of
significant trauma, which might point to fracture, suggest
the diagnosis of osteomyelitis.
Osteomyelitis is caused most commonly by bacteria and
presents with inflammation, subsequently causing destruc-
tion of bone. The bones of the lower limbs are affected in up
to 90% of infections of children. The tibia is affected in a
large percentage of osteomyelitis involving the lower limbs.
Osteomyelitis can be contiguously or hematogenously
spread. Contiguous spread from infected tissues or open
wounds are more often polymicrobial. Hematogenously
spread osteomyelitis is typically caused by a single
organism.
The differentiation of osteomyelitis and septic arthritis
from toxic synovitis is important as treatment is signifi-
cantly different. Toxic synovitis is treated with rest and mild
analgesics. Osteomyelitis requires aggressive treatment
including antibiotics and surgical debridement. A further
complication of osteomyelitis is septic arthritis, which
can cause more prominent bone destruction and synovial
joint space compromise. Because bone destruction and
joint injury can occur quickly, treatment should begin
early. Septic arthritis can be a surgical emergency because
of the rapid destruction that can occur.
What additional imaging would be appropriate
and why?
Plain films of affected joints are important to obtain and
they can provide information about other possible causes
of symptoms, but changes in bone density that may occur
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with osteomyelitis can be difficult to detect in older chil-
dren. Because the bones of infants are less ossified, bone
density changes can be seen more easily. Plain films can
show areas of swelling, changes in translucent fat planes,
and in later stages may show the spread of edematous fluid.
However, bony destruction is usually not detectable for 2–3
weeks after the onset of symptoms in the child with
osteomyelitis.
MRI has become the imaging modality of choice, with a
90% sensitivity for detecting osteomyelitis. If the child can
be evaluated with MRI within hours of the suspected
diagnosis of osteomyelitis, it is reasonable to proceed first
with this modality. MRI can accurately demonstrate sub-
periosteal or soft tissue collections of pus that might
require surgical drainage without the use of ionizing
radiation.
Which antibiotics should be initiated?
Gram-positive bacteria are the most common pathogens
identified in osteomyelitis. Staphylococcus aureus and
Streptococcus pyogenes are the most common organisms.
Kingella kingae should be considered in younger chil-
dren under the age of 3 years. In unimmunized patients,
Haemophilus influenzae type B should also be consid-
ered. Empiric antibiotic selection should be targeted
toward treatment of S. aureus and S. pyogenes in most
cases. Antibiotic therapy can be tailored based on cul-
tures, sensitivities, and local area antibiograms. If the
S. aureus isolates are susceptible to methicillin, initial
therapy may consist of a pencillinase-resistant semisyn-
thetic penicillin such as nafcillin or oxacillin. In other
geographic areas where community-acquired methicillin-
resistant S. aureus (MRSA) is common, either vancomycin
or clindamycin should be included in the initial empiric
therapy.
What is the duration of therapy?
Most therapy plans start with intravenous antibiotics and
continue until the patient is afebrile for more than 48 hours,
is able to tolerate oral nutrition and medications, and has
decreased local signs and symptoms of infection and pain.
The laboratory values should also be normalized before
transitioning to oral antibiotics. The WBC should be nor-
malized and there should be a significant decrease in the
CRP. After these benchmarks have been met, antibiotic
therapy continues with oral medications for 4–6 weeks.
Normalization of the CRP can be the marker for discontin-
uation of therapy.
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Chapter 22 Osteomyelitis Case Study 91

Case Progression and Resolution

The child was admitted to the pediatric surgical floor for

additional evaluation with magnetic resonance imaging
(MRI), antibiotics, and surgical debridement of the left
ankle. MRI of the left ankle (Figure 22.1) exhibited a heter-
ogeneous marrow signal within the distal tibial metadia-
physis with associated subperiosteal collection measuring
approximately 0.6 cm in thickness. There was extensive
edema within the musculature predominantly involving
the posterior deep compartment. There was a focal area
in the flexor digitorum longus that demonstrated disorgan-
ized muscle fibers and a loculated collection suggestive of
osteomyelitis.
In the operating room, a biopsy of the tissue surrounding
the ankle was obtained and copious irrigation and debride-
ment of the area was completed.
After hospital discharge, patients should be followed up
at 1–2 week intervals to monitor continuing clinical
improvement and to monitor for complications with antibi- Figure 22.1 MRI of the left ankle.
otic therapy.

Further Reading
Batchelder, N. and Tsz-Yin, S. (2016). Transitioning
antimicrobials from intravenous to oral in pediatric acute
uncomplicated osteomyelitis. World Journal of Clinical
Pediatrics 5(3): 244–250.
Keren, R., Shah, S.S., and Srivastava, R. (2015). Comparative
effectiveness of intravenous vs oral antiobiotics for
postdischarge treatment of acute osteomyelitis in children.
JAMA Pediatrics 169(2): 120–128.
Krogstad, P. (2018). Osteomyelitis. In: Feigin and Cherry’s
Textbook of Pediatric Infectious Diseases, 8th edn (ed. J.D.
Cherry, G.J. Harrison, S.L. Kaplan, W.J. Steinbach, and P.J.
Hotez), 516–528. Philadelphia, PA: Elsevier.
Krogstad, P. (2018). Septic arthritis. In: Feigin and Cherry’s
Textbook of Pediatric Infectious Diseases, 8th edn (ed. J.D.
Cherry, G.J. Harrison, S.L. Kaplan, W.J. Steinbach, and P.J.
Hotez), 529–534. Philadelphia, PA: Elsevier.
Stone, B., Street, M., Leigh, W., and Crawford, H. (2016).
Pediatric tibial osteomyelitis. Journal of Pediatric
Orthopedics 36(5): 534–540.

23
A 2-Year-Old with Liver Transplant and Fever
Louise Flynn
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 2-year-old girl who received a live donor, left lateral seg-
ment, liver transplantation with a Roux anastomosis for
end-stage liver disease due to biliary atresia at 13 months
of age presents with fever.
History of Present Illness
This 2-year-old girl had fever for one day, which was treated
with acetaminophen at home. This morning she started
complaining of abdominal pain and parents had noted
decreased appetite and fatigue.
Past Medical History
The child was born full term by cesarean section for
failure to progress. On day 3 of life she was noted to be
jaundiced and was treated with phototherapy for 24 hours.
Bilirubin rebounded after discontinuation of photo-
therapy and she was seen by the hepatologist for hepato-
megaly, jaundice, direct hyperbilirubinemia, and elevated
gamma-glutamyltransferase (GGT). She was diagnosed
with biliary atresia at 5 weeks of age and a hepato-
portoenterostomy (Kasai) procedure was done. She was
treated for cholangitis with intravenous antibiotics at
4 months and 7 months of age and remained on prophylac-
tic-dose antibiotics until time of transplant. At 13 months of
age, she had a liver transplant from a living donor. Compli-
cations included a bile leak, managed with a peritoneal
drain for 2 months post transplant, and steroid-induced
hypertension, managed with amlodipine. No other medical
problems were identified to this age.
Past Surgical History
Surgical procedures for this child include a hepato-
portoenterostomy at 5 weeks of age, liver transplant at
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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93
13 months of age, and exploratory laparotomy and repair
of bile duct anastomosis 4 days after transplant.
Family History
The patient lives with her parents and 5-year-old sibling
and there is no family history of liver disease. Parents
and grandparents are all alive and healthy.
Current Status
The child arrives at the emergency department (ED) with
a 36-hour history of fever and abdominal discomfort.
There is no history of vomiting or diarrhea, appetite is
unchanged, and activity level has decreased with increase
in temperature. She has received acetaminophen every
4–6 hours, with the last dose 3 hours prior to ED visit.
Vital signs on arrival include temperature 38.7 C
(101.7 F), heart rate 138 bpm, respiratory rate 28 breaths
per minute, and blood pressure 98/63 mmHg.
Blood and urine cultures and chest radiograph obtained
were all normal. The results of the complete blood count
and hepatic panel are shown in Tables 23.1 and 23.2.
Questions
Answer the following questions using the details provided.
1 With elevated liver function studies, what is the differ-
ential diagnosis?
2 What radiologic studies should be considered?
Continuation of case: The patient was admitted to the
pediatric inpatient unit and intravenous antibiotics were
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94 Cases in Pediatric Acute Care

Table 23.1 Complete blood count.
White blood cell count 6000/mm3
Hemoglobin 10 g/dL
Hematocrit 32%
Platelet count 95 000/mm3
Table 23.2 Hepatic panel.
Total bilirubin 0.4 mg/dL
Direct bilirubin 0.1 mg/dL
Aspartate aminotransferase 386 U/L
Alanine aminotransferase 383 U/L
Albumin 3 mg/dL
started. At 24 hours after starting therapy, she is afebrile
with normal vital signs and the pain has resolved. Repeat
laboratory investigations showed improvement in aspartate
aminotransferase (249 U/L) and alanine aminotransferase
(330 U/L) but GGT (91 U/L) remained elevated. Immuno-
suppressive medication level was in therapeutic range.
Blood and urine cultures were negative. Abdominal ultra-
sound with vascular Doppler showed no bile duct dilatation
and patent abdominal vasculature with appropriate direc-
tion of flow. Magnetic resonance cholangiogram (MRCP)
showed dilatation of the proximal bile duct at the region
of the bile duct anastomosis to the Roux limb.
3 What further studies/procedures would you
recommend?
4 What are the primary concerns for a child post liver
transplant with symptoms of illness?
Rationale and Evidence-based Practice
Explanation
With elevated liver function studies, what is the
differential diagnosis?
The differential diagnoses for this child include acute
cellular rejection, cholangitis, biliary strictures, and other
infectious processes. Fever, elevated liver numbers, and
abdominal pain would support concern for cholangitis.
Biliary stricture would be high on the differential list due
to the history of bile leak and possible cholangitis. Rejection
must also be considered with any elevation in liver
enzymes, even with therapeutic levels of immunosuppres-
sive medication.
What radiologic studies should be considered?
An abdominal ultrasound with vascular Doppler study
should be considered. The ultrasound may show ductal dil-
atation, which would suggest a biliary stricture. The Dop-
pler study is diagnostic for patency and flow through the
hepatic artery and portal vein. A normal ultrasound does
not preclude further evaluation for biliary strictures. MRCP
is used to evaluate for biliary strictures.
What further studies/procedures would you
recommend?
Percutaneous transhepatic cholangiogram performed in
interventional radiology is used for both evaluation and
treatment of biliary stricture. Once the stricture is identified
by cholangiography, treatment with cholangioplasty (with
balloon dilatation) and stent placement is done. Strictures
are reevaluated every 6–12 weeks until resolved. For
patients who received a whole liver and had a duct-to-duct
anastomosis, endoscopic retrograde cholangiopancreato-
graphy (ERCP) can be performed. Surgery to revise anasto-
mosis may be required if cholangioplasty is unsuccessful.
What are the primary concerns for a child post liver
transplant with symptoms of illness?
Outcomes in liver transplantation have improved over
time. Long-term transplant follow-up is aimed at care
guided by the primary provider and gastroenterologist.
Long-term complications can affect 30–50% of liver trans-
plant recipients (Rawal and Yazigi, 2017). Preventive care
focuses on kidney and bone protection, immunosuppres-
sion minimization, vaccination, nutritional support, and
mental health assessment and support. Transplant-related
problems experienced by children post transplant include
graft rejection, vascular complications, biliary complica-
tions, and recurrent disease. They can also develop long-
term gastrointestinal problems such as feeding and motility
disorders, cardiovascular problems such as hypertension,
insulin resistance and obesity. Chronic mental health con-
cerns also exist, but children are also at a similar risk as
others for development of viral infections. Cautious and
comprehensive assessment is most important for positive
outcomes.
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Chapter 23 A 2-Year-Old with Liver Transplant and Fever 95

Reference Further Reading

Rawal, N. and Yazigi, N. (2017). Pediatric liver transplantation. Dunn, S.P. and Horslen, S. (ed.) (2018). Solid Organ
Pediatric Clinics of North America 64: 677–684. doi: 10.1016/ Transplantation in Infants and Children. Cham,
j.pcl.2017.02.003. Switzerland: Springer.

24
Respiratory Decompensation in Trached Infant
Jennifer Helman
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA
A 3-month-old female, admitted to the pediatric intensive
care unit (PICU) with complex congenital heart disease
and chronic respiratory failure, develops new-onset symp-
toms of respiratory distress.
History of Present Illness
She was admitted to the hospital following birth due to
complex congenital heart disease including dextrotranspo-
sition of the great arteries. Patient underwent arterial
switch procedure at day 3 of life and required venous–
arterial extracorporeal membrane oxygenation (VA-ECMO)
immediately following procedure for 6 days due to low
cardiac output. Patient has had persistent ventilator
requirement with inability to wean ventilatory support.
Discussion between primary team, pediatric otolaryngology
service, and family resulted in decision to proceed with tra-
cheostomy due to need for long-term mechanical ventila-
tion. Tracheostomy was completed 4 days ago by pediatric
otolaryngology, without complication.
Past Medical History
She was born at 37 weeks with prenatal diagnosis of dextro-
transposition of the great arteries to 30-year-old gravida 1
para 1 mother by cesarean section. Apgar scores were 6
and 9 following birth. She was initially placed on oxygen
per nasal cannula at birth that was transitioned to nasal
continuous positive airway pressure support at a few hours
of life due to increased work of breathing. She received
prostaglandin E infusion until arterial switch procedure
at day 3 of life. Patient was intubated by anesthesia with
3.0 cuffed oral endotracheal tube for arterial switch
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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97
operation and reported to be grade 1 view. Patient’s
postoperative course was complicated by need for
VA-ECMO, and sternal wound infection for which she is
receiving 6-week intravenous antibiotic course.
Past Surgical History
She had an arterial switch operation and VA-ECMO cannu-
lation at day 3 of life. She underwent ECMO decannulation
6 days later and had subsequent delayed sternal closure.
She had tracheostomy completed 4 days ago at 3 months
of age.
Family History
Her father has a history of asthma. Her mother’s family
history is negative.
Current Status
She is in the PICU and has been hemodynamically stable
following tracheostomy procedure. She is receiving seda-
tion infusions of midazolam, dexmedetomidine, and mor-
phine that are being administered through a peripherally
inserted central catheter. She is ordered to have bedrest
and limited movement due to risk for accidental decannu-
lation. She has a 3.5 neonatal cuffed tracheostomy tube in
place. Stay sutures are intact, labeled, and taped to the
chest (Figure 24.1). Nurse has been suctioning tracheost-
omy tube every 2 hours for a small amount of thin white
secretions. She is on the ventilator in pressure control/
assist control mode with rate 30 breaths per minute,

98 Cases in Pediatric Acute Care
Figure 24.1 Patient with stay sutures in place.
Figure 24.2 Chest radiography with tracheostomy tube in place.
positive end-expiratory pressure (PEEP) 10 cmH2O, peak
inspiratory pressure 32 cmH2O, and FiO2 0.3. She is breath-
ing comfortably with stable venous blood gases. Oxygen
saturations are currently 99% on the ventilator. Lung
sounds are slightly coarse bilaterally. Figure 24.2 shows
the most recent chest radiograph. She is receiving a milri-
none infusion as recent echocardiogram demonstrated
moderately diminished left ventricular function. Emer-
gency tracheostomy supply bag is at bedside including
same size and size smaller tracheostomy tubes. Pediatric
otolaryngology is planning to complete the first trach
change in 3 days.
You are called to the bedside by the nurse because of the
patient’s increased work of breathing and hypoxia, which
occurred acutely after she received a bath and the bedsheets
were changed. When you arrive at the bedside, she appears
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to have increased work of breathing with subcostal retrac-
tions and nasal flaring. She is also noted to have mottling of
her skin. Oxygen saturations are now 85% and the ventila-
tor alarm is sounding.
Questions
Answer the following questions using the details provided.
1 What emergency supplies should be immediately avail-
able for a patient status post tracheostomy?
2 What is the purpose of stay sutures in a postoperative
tracheostomy patient?
3 Based on this information, what are the possible differ-
ential diagnoses for sudden respiratory distress for this
patient?
4 What factors are important to assess during your phys-
ical examination for these potential differential
diagnoses?
Rationale and Evidence-based
Practice Explanation
What emergency supplies should be immediately
available for a patient status post tracheostomy?
Emergency supplies to replace the tracheostomy tube
should always be immediately available to tracheostomy
patients. This includes the same size tracheostomy tube
and the size smaller tracheostomy tube in case there is dif-
ficulty placing the same size tracheostomy tube. Other
needed supplies include trach ties, water-based lubricant,
scissors, hemostats, suction catheters, suction apparatus,
and bag valve mask. An appropriately sized oral endotra-
cheal tube should also be accessible.
What is the purpose of stay sutures in a
postoperative tracheostomy patient?
Stay suture placement is a surgical technique where non-
absorbable sutures are placed adjacent to the tracheal inci-
sion at the third and fourth cartilage rings, so that they
could be pulled to open the stoma and bring the trachea
closer to the skin if there was accidental dislodgement of
the tracheostomy tube. These are taped to the chest and
labeled “right” and “left” so that they do not inadvertently
get crossed if being used. These sutures are removed at the
time of the first trach change.

Based on this information, what are the possible
differential diagnoses for sudden respiratory
distress for this patient?
Possible major postoperative complications for pediatric
tracheostomy patients can include bleeding, occlusion of
tracheostomy tube most commonly by inspissated secre-
tions, and accidental decannulation. If a child with a tra-
cheostomy develops respiratory distress (typically acute in
nature), it is important to assess for these conditions.
Pneumothorax is also a risk factor with a tracheostomy
procedure. Respiratory and cardiac reasons, and mechan-
ical failure of the ventilator also need to be included as
part of the differential diagnosis of this patient.
What factors are important to assess during your
physical examination for these potential
differential diagnoses?
It is important to immediately assess the patient’s neck to
ensure that the tracheostomy tube is in place. Crepitus from
misdirected air into subcutaneous tissues can be ascer-
tained by simple palpation. Suctioning of the tracheostomy
tube should also be performed to evaluate for secretions in
the tube and easy passage of the suction catheter through
the tracheostomy tube. After these have been assessed, pro-
ceed with the remainder of the physical examination,
including assessment of the heart, auscultation of the chest
to ensure bilateral breath sounds, and checking vent tubing
from patient to machine.
Continuation of case: On physical examination, assess-
ment of the tracheostomy site shows that the tracheostomy
tube is decannulated from the trachea, though the tra-
cheostomy ties remain secured around the neck. The stoma
is slightly open and stay sutures are still intact. Patient
is continuing to have increased work of breathing,
with worsening subcostal retractions and cyanosis. Oxygen
saturations continue to decrease to 70%. The pediatric oto-
laryngology team is paged stat to the bedside. The nurse
removes the trach ties so that the neck is visible and there
is access to the stoma. The respiratory therapist begins bag-
mask ventilation of the patient, while the nurse covers the
stoma with gauze to make it occlusive. There is good chest
rise and oxygen saturations slowly increase to 84%.
Patient’s color is still slightly cyanotic, but slowly improv-
ing. You begin to prepare for replacing the tracheostomy
tube and the pediatric otolaryngology team arrives at the
bedside. They state that they will now be managing the air-
way for clarity to the providers in the room. They take the
same size trach tube and hemostats from the patient’s
emergency supply bag. The right and left stay sutures are
lifted up from the chest in a V formation to hold the edges
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Chapter 24 Respiratory Decompensation in Trached Infant 99
Figure 24.3 Image from tracheobronchoscopy: carina is
visualized with right and left mainstem bronchi.
of the stoma open. The same size trach tube is inserted with
minimal difficulty and the patient is placed back on the
ventilator. Trach ties are secured. Oxygen saturations are
now 93%. Bilateral breath sounds are confirmed. Pediatric
otolaryngology completes a bedside tracheobronchoscopy
examination after patient is placed on 100% oxygen and
confirms that the tracheostomy tube is appropriately posi-
tioned in the trachea (Figure 24.3). The patient’s color is
now pink and FiO2 has been quickly decreased to 50% with
oxygen saturations 98%.
5 What maneuvers could be used immediately to help
with ventilation of this patient?
6 What are the options for securing an airway in this
patient?
7 Why is it important to confirm that the tracheostomy
tube is in place following decannulation in an
un-established airway? What methods can be used to
confirm placement?
What maneuvers could be used immediately to
help with ventilation of this patient?
If patients with tracheostomy tube decannulation are not
demonstrating adequate respiratory drive, they can receive
bag-mask ventilation to help with oxygenation while
attempts are made to replace the trach tube. Patients with-
out upper airway issues that exclude them from being intu-
bated or bagged from above can receive bag-mask
ventilation with the tracheostomy stoma being covered
with gauze to make it occlusive. If the stoma is not covered,
the air will leak out and the patient cannot receive
appropriate ventilation. Certain upper airway anomalies
or surgical procedures (e.g. surgical history of

100 Cases in Pediatric Acute Care
laryngotracheal separation) do not allow patients to be
bagged from above, and would require bagging through
the trach stoma. This involves placing the mask over the
stoma with a tight seal and bagging the patient to achieve
adequate chest rise. If the patient is stable and has adequate
respiratory drive, bagging is not necessary while preparing
to replace the tracheostomy tube.
What are the options for securing an airway in this
patient?
Foreknowledge of the patient’s airway is important because
the team can then determine whether the patient can be
bagged from above in an emergency or whether the patient
needs to be bagged through the trach stoma. If the tra-
cheostomy tube cannot be replaced, the patient should be
orally intubated as long as there is no upper airway obstruc-
tion that would exclude this as an option.
Further Reading
D’Souza, J., Levi, J., Park, D., and Shah, U. (2016).
Complications following pediatric tracheotomy. JAMA
Otolaryngology Head and Neck Surgery 142(5): 484–488.
Kraft, S. and Schinder, J. (2015). Tracheotomy. In: Cummings
Otolaryngology Head and Neck Surgery, 6th edn
(ed. P. Flint), 95–103. Philadelphia, PA: Saunders.
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Why is it important to confirm that the
tracheostomy tube is in place following
decannulation in an un-established airway? What
methods can be used to confirm placement?
Appropriate placement of the tracheostomy tube is imper-
ative in order to achieve adequate ventilation and to have a
secure airway. False passage is a possible risk factor in pla-
cing a tracheostomy tube, especially in a stoma that is not
yet established. Physical examination findings confirming
that the tracheostomy tube is appropriately placed include
assessing for bilateral equal breath sounds and symmetric
chest rise with respirations. Presence of humidification of
the trach tube, and monitoring of end-tidal carbon dioxide
if available, can also help to demonstrate correct placement.
Tracheobronchoscopy allows direct visualization past the
distal end of the tracheostomy tube to confirm appropriate
positioning in the trachea by visualizing the wall of the tra-
chea, carina, and mainstem bronchi.
Mitchell, R., Hussey, H., Setzen, G., et al. (2013). Clinical
consensus statement: tracheostomy care. Otolaryngology
Head and Neck Surgery 148(1): 6–20.

25
Neonatal Hypoglycemia
Janice L. Wilson
University of Maryland School of Nursing, Baltimore, MD, USA
A full-term male infant is delivered by emergency cesarean
for fetal distress.
History of Present Illness
At 30 minutes of age, the infant is attempting to breastfeed.
He is tremulous and irritable. His heart rate is slightly ele-
vated from previously, but his color is pink and he is alert.
Past Medical History
The infant’s mother is a 35-year-old primipara with gesta-
tional diabetes and non-adherence to recommended
dietary management of glucose control. She has a history
of pre-pregnancy obesity, with a 20-kg weight gain during
this pregnancy. Fetal macrosomia was diagnosed in the
second trimester. She was noted to have an abnormal
glucose tolerance test at 28 weeks and declined the
recommended follow-up testing. Results of prenatal
laboratory investigations are shown in Table 25.1.
With advancing cervical dilation, a fetal heart rate drop to
60 bpm was noted, with no return to baseline. An emer-
gency cesarean section was performed. Apgar scores were
61 and 85. Resuscitative efforts consisted of bulb suctioning
and stimulation.
Birthweight was 4.375 kg (95th percentile), length 52 cm
(75th percentile), and head circumference 36 cm (50th
percentile).
Family History
The maternal grandmother has type 1 insulin-dependent
diabetes, and the paternal grandfather has type 2 diabetes.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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101
There is a history of morbid obesity on both sides of the
family.
Current Status
The infant is noted having difficulty latching while at the
breast and on physical examination he was jittery. Physical
examination reveals a macrosomic large-for-gestational age
male. Point-of-care glucose and serum glucose are obtained
(Table 25.2).
Questions
Answer the following questions using the details provided.
1 What concerning risk factors does this infant exhibit?
2 What signs and symptoms might be anticipated?
3 What do the laboratory results indicate?
4 Based on the information available, what is the most
likely diagnosis and what physiologic mechanisms
are responsible?
Continuation of case: Peripheral intravenous access was
established, a 2 mL/kg bolus of dextrose 10% in water was
given, and the infant was subsequently transferred to the
neonatal intensive care unit (NICU). Point-of-care glucose
testing on admission to the NICU was 50 mg/dL. An intra-
venous infusion of dextrose 10% in water was ordered, with
a glucose infusion rate of 8 mg/kg per min. Ad lib breast-
feeding was resumed at 6 hours of age when point-of-care
glucose glucose stabilized at 50–60 mg/dL. Over the next
3 days, despite successful breastfeeding, this newborn
was unable to wean below a 5 mg/kg per min glucose
infusion rate.
5 Has the differential diagnosis changed, and if so why?
6 What additional diagnostic testing might be warranted?
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102 Cases in Pediatric Acute Care

Table 25.1 Prenatal laboratory results. greater at lower glucose concentrations. Low point-of-care
glucose results need to be confirmed or verified by stat
Blood type and Rh A positive
plasma glucose sampling. There is no consensus regarding
Group B streptococci Negative an absolute laboratory value that defines hypoglycemia.
Hepatitis B surface antigen Negative This is due to the fact that the brain response to hypoglyce-
HIV Negative mia may vary with a range of glucose concentrations and it
Herpes simplex virus Unknown is difficult to identify a single glucose concentration that
Syphilis testing Negative can cause brain injury. Additionally, there are artifacts
Gonorrhea testing Negative
and inaccuracies in determining glucose that make it diffi-
cult to interpret plasma glucose levels. Despite the lack of
Chlamydia testing Negative
evidence, however, a plasma glucose level below 47 mg/
dL is often used to define hypoglycemia.

Table 25.2 Glucose test results.

Point-of-care glucose 25 mg/dL

Serum glucose 30 mg/dL
7 What are the potential long-term sequelae of neonatal
hypoglycemia?
Rationale and Evidence-based Practice
Explanation
What concerning risk factors does this infant
exhibit?
Risk factors specific for this child include large for gesta-
tional age, poor maternal glycemic control, fetal distress,
and familial history of diabetes. Other common risk fac-
tors for this disorder include small for gestational age,
intrauterine growth retardation, asphyxia, hypothermia,
sepsis, polycythemia, and prematurity. Less common
disorders associated with this condition include Beck-
with–Wiedemann syndrome, hypothyroidism, panhypopi-
tuitarism, growth hormone deficiency, cortisol deficiency,
and inborn errors of metabolism.
What signs and symptoms might be anticipated?
Symptoms can be subtle and may mimic other disease
states. Symptoms include jitteriness, tachycardia, pallor,
hypothermia, lethargy, irritability, poor feeding, cyanosis,
tachypnea, apnea, weak or high-pitched cry, poor tone,
and seizure-like behavior.
What do the laboratory results indicate?
It is critical to understand that point-of-care glucose analy-
zers can vary by as much as 10–20 mg/dL when compared
with plasma glucose levels. This variation may be even
Based on the information available, what is the
most likely diagnosis and what physiologic
mechanisms are responsible?
The most likely diagnosis for this infant is transient hyper-
insulinemic hypoglycemia or early transitional adaptive
hypoglycemia. Fetal insulin production increases with
exposure to high maternal glucose levels. The act of cut-
ting the umbilical cord at delivery will cause the new-
born’s glucose level to fall, while the insulin level
remains elevated. As the insulin levels gradually falls over
the first few days of life, the infant will remain at risk for
hypoglycemia.
Has the differential diagnosis changed, and if
so why?
High insulin levels, stimulated by poor maternal glucose
control, should gradually drop over the first 72 hours of life.
As insulin levels fall, glucose levels should begin to normal-
ize. Symptomatic hypoglycemia, the need for intravenous
dextrose to maintain normal plasma glucose levels, pre-
feeding plasma glucose levels below 50 mg/dL at 48 hours
of age, and pre-feeding plasma glucose levels below 60 mg/
dL after 48 hours of age require further evaluation for a
persistent hypoglycemia disorder or severe recurrent
hypoglycemia.
A “critical blood sample” should be obtained when the
plasma glucose falls below 40–50 mg/dL in infants who
continue to have persistent hypoglycemia. This critical
blood sample should include measurement of plasma glu-
cose, insulin, cortisol, growth hormone, β-hydroxybutyrate,
and free fatty acid levels (see Table 25.3). After the critical
sampling is completed and depending on institution proto-
col, a glucagon stimulation challenge may then be per-
formed. An increase in plasma glucose greater than
30 mg/dL after glucagon administration is an indication
of hyperinsulinism.
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Chapter 25 Neonatal Hypoglycemia 103

Table 25.3 Blood sampling rationale.

Critical blood sampling/testing Rationale

Plasma glucose Identification of hypoglycemia

Insulin Inappropriately high level compared to plasma glucose (hyperinsulinism)
Cortisol Evaluates function/integrity of hypothalamic–pituitary–adrenal axis (low cortisol levels
may indicate hypopituitarism)
β-Hydroxybutyrate Decreased levels may indicate excessive insulin action (even when insulin level is not
significantly elevated)
Free fatty acids Decreased levels may indicate excessive insulin action (even when insulin level is not
significantly elevated)
Growth hormone Evaluates function/integrity of hypothalamic–pituitary–adrenal axis (low growth
hormone levels may indicate hypopituitarism)
Glucagon stimulation challenge Increase in plasma glucose ≥30 mg/dL indicates hyperinsulinism
(administer 1 mg glucagon)

What additional diagnostic testing might be
warranted?
Additional testing may be needed to evaluate for defects in
carbohydrate metabolism, defects in amino acid metabo-
lism as well as other endocrine deficiencies. Genetic muta-
tions in genes responsible for insulin regulation can also
cause hyperinsulinemic hypoglycemia and testing can
identify specific genes of concern. Beckwith–Weidemann
syndrome is the most common syndrome associated with
hyperinsulinism.
What are the potential long-term sequelae of
neonatal hypoglycemia?
Glucose is the primary metabolic fuel for the brain and
drives hepatic glucose production, though the mechanisms
responsible are unknown. When hypoglycemia is severe or
prolonged, infants are at risk for significant irreversible
neurologic sequelae, including developmental delay, learn-
ing and behavior problems, hyperactivity, attention deficit
challenges, autism, microcephaly, and cortical blindness.

Further Reading
Adamkin, D.H. (2011). Clinical report: postnatal glucose
homeostasis in late pre-term and term infants. Pediatrics 127
(3): 575–579.
Blake, S.M., Jnah, A.J., and Adkins, D.W. (2019). The
metabolic and endocrine system. In: Fetal and Neonatal
Physiology for the Advanced Practice Nurse (ed. A.J. Jnah and
A.N. Trembath), 307–348. New York: Springer.
Burris, H.H. (2017). Hypoglycemia and hyperglycemia: In:
Cloherty and Stark’s Manual of Neonatal Care, 8th edn (ed.
E.C. Eichenwald, A.R. Hansen, C.R. Martin, and A.R. Stark),
312–325. Philadelphia, PA: Wolters Kluwer.
Devaskar, S.U. and Garg, M. (2015). Disorders of carbohydrate
metabolism in the neonate. In: Fanaroff and Martin’s
Neonatal–Perinatal Medicine, Diseases of the Fetus and
Infant, 10th edn (ed. R.J. Martin, A.A. Fanaroff, and M.C.
Walsh), 1434–1459. Philadelphia, PA: Elsevier.
Gorman, T. (2017). Neonatal effects of maternal diabetes. In:
Cloherty and Stark’s Manual of Neonatal Care, 8th edn (ed.
E.C. Eichenwald, A.R. Hansen, C.R. Martin, and A.R. Stark),
910–922. Philadelphia, PA: Wolters Kluwer.
Long, D. and Akhtar, Y. (2019). Hyperinsulinism. Pediatrics in
Review 40(4): 207–210.
Lord, K. and De Leon, D.D. (2018). Hyperinsulinism in the
neonate. Clinics in Perinatology 45(1): 61–74.
Perlman, J.M. and Volpe, J.J. (2019). Glucose. In: Volpe’s
Neurology of the Newborn, 6th edn (ed. J.J. Volpe, T.E. Inder,
B.T. Darras, et al.), 701–729. New York: Elsevier.
Thornton, P.S., Stanley, C.A., De Leon, D.D., et al. (2015).
Recommendations from the Pediatric Endocrine Society for
evaluation and management of persistent hypoglycemia in
neonates, infants, and children. Journal of Pediatrics 167(2):
238–245.

26
Chronic Otitis Media
Kimberly Young-Conner
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 3-year-old male is being followed by his primary care
provider for frequent episodes of otitis media.
History of Present Illness
This 3-year-old boy is being followed by the primary care
practice for recurrent otitis media (OM) after bilateral
tympanostomy tube (pressure-equalizing or PE tubes)
placement at 8 months of age. He has recently developed
cold symptoms and the parents are concerned that he
may have an ear infection.
Past Medical History
The child was born at 38 weeks’ gestational age by vaginal
delivery, with maternal prenatal care and no complications
at birth. He lives with mother, father, and one older sister.
He attends a home-based daycare during the school year,
when his mother works as a teacher. This patient is an over-
all healthy child whose growth and development have been
on target, although there was initial concern at 15 months
of age about his speech and language. He is currently in the
40% for weight and 50% for height with steady growth noted
on charts. He was diagnosed with a first episode of bilateral
OM at 6 months of age and was treated with amoxicillin. He
did not receive the full course of amoxicillin and 2 weeks
later presented with continued findings of OM, so was trea-
ted with cefdinir with resolution. Parents consulted an oto-
laryngologist at that time and were told to watch and see
what happens. One month later the child developed bilat-
eral OM and was treated with ceftriaxone intramuscularly
for 3 days. Parents again reached out to the otolaryngologist
who wanted to wait for full resolution of illness before
another office visit. Parents were not happy with this
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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105
decision, so sought the services of a general otolaryngolo-
gist who did schedule tube placement at that time. At
18 months of age, one of the PE tubes was displaced and
the child had another episode of OM. Returning to the oto-
laryngologist resulted in another surgical procedure for
adenoidectomy. The child has a history of chronic nasal
congestion and has been treated with albuterol twice for
wheezing.
Past Surgical History
This child had tympanostomy tubes placed at age 7 months,
under general anesthesia. Approximately one year later, at
age 18 months, he had an adenoidectomy and replacement
of one of the PE tubes.
Family History
No chronic illness has been identified in any family
members. Parents are both healthy. Older sibling is healthy,
but also had a history of OM with PE tubes placed at age
1 year. The family resides in a single-family home without
pets or smokers.
Current Status
In the primary care office today, this 3-year-old child is
noted to be happy, with clear speech and gross and fine
motor skills at age level. His physical examination is
normal, with one PE tube still in place to the left ear, but
the right one is missing.

106 Cases in Pediatric Acute Care
Questions
Answer the following questions using the details provided.
1 What concerns exist regarding recurrent OM diagnosed
in an infant or young child?
2 When is tympanostomy tube placement recommended?
What is the benefit?
3 What are the risks of the procedure?
4 What outcomes should be expected?
5 What further care or consults should this child receive?
Rationale and Evidence-based Practice
Explanation
What concerns exist regarding recurrent OM
diagnosed in an infant or young child?
Acute OM affects approximately 25% of children in the first
year of life and more than 50% of children by the age of
5 years. Factors associated with the development of OM
include attendance at daycare, bottle feeding versus breast-
feeding, atopy, and vaccine refusal, especially pneumococ-
cal and influenza vaccines. There are also other factors,
including anatomic problems such as cleft palate and
genetic conditions such as Down syndrome. The main con-
cern regarding recurrent or persistent OM is primarily
speech and language problems due to the conductive hear-
ing loss that occurs with constant or frequent episodes of
fluid in the eustachian tube, especially in young infants
and children. Other documented problems in children with
OM include pain, decreased appetite, sleep disturbances,
and behavioral problems. Perforation of the tympanic
membrane is another complication of OM, and can result
in conductive hearing loss if there is significant damage
and/or scarring of the tympanic membrane. Other pro-
blems include learning difficulties when hearing is
impaired, even temporarily. Another issue with recurrent
OM is frequent antibiotic use, which can result in allergic
reactions, development of resistant organisms, as well as
the rare potential for fungal infection. Development of
OM early in life also increases the risk of recurrent OM later
on in childhood.
When is tympanostomy tube placement
recommended? What is the benefit?
The American Academy of Pediatrics published clinical prac-
tice guidelines in 2013 that outline evidence-based recom-
mended care and consults for children with recurrent OM.
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The recommendation is for providers to offer tympanost-
omy tubes for children who have had three episodes of
OM in 6 months or four episodes in one year, with one
occurring in the previous 6 months (Lieberthal et al.,
2013). The benefits of PE tube placement include
decreased opportunity to develop OM, with venting of
the tympanic membrane allowing any fluid accumulation
to drain. Clearing fluid from the middle ear assists in
allowing a child to hear more consistently, and also allows
the process of language development.
What are the risks of the procedure?
Placement of PE or tympanostomy tubes is accomplished
by creating a small incision in the tympanic membrane
and placing a ventilation tube in that area. The procedure
is done under general anesthesia, which poses risks to any
child but especially to young infants. Complications of PE
tube placement include otorrhea, tympanosclerosis, and
permanent perforation. PE tubes will stay in place for var-
ied amounts of time, and shorter dwelling times may result
in replacement of the tube. Development of a cholestea-
toma and medial migration of the PE tube to the middle
ear space are two complications which are rare, but pose
more serious problems. Both of these conditions most often
require surgical intervention.
What outcomes should be expected?
Children who meet criteria for PE tube placement have
overall good outcomes following the procedure. Despite
some of these children requiring speech and language refer-
rals, it is noted that most children continue to do well with
improved hearing after tube placement. Quality of life was
evaluated in a study of 1208 children between the ages of 6
and 24 months of age, with higher quality of life noted for
those who had PE tubes placed as compared to those who
were referred and waiting for the procedure (Grindler
et al., 2014).
What further care or consults should this child
receive?
This child is doing well currently so should not need follow-
up by the otolaryngologist at this point, although if the PE
tube remains in place it will need to be removed. The time
frame for surgical removal is typically 3 years from place-
ment. This child was seen by the otolaryngologist too soon
during infancy, and had tubes placed prior to reaching the
recommended number of OM episodes, which did place the
child at higher risk of at least anesthesia complications.

References
Grindler, D.J., Blank, S.J., Schulz, K.A., Witsell, D.L., and Lieu,
J.E. (2014). Impact of otitis media severity on childrens’
quality of life. Pediatric Otolaryngology 151(2): 333–340. doi:
10.1177/0194599814525576.
Further Reading
Pichichero, M.E. (2018). Helping children with hearing loss
from otitis media with effusion. Lancet 392(10147): 533–534.
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Chapter 26 Chronic Otitis Media 107
Lieberthal, A.S., Carroll, A.E., Chonmaitree, T., et al. (2013).
The diagnosis and management of acute otitis media.
Pediatrics 131(3): e964–e999. doi: 10.1542/
peds.2012-3488.
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109

27

Complex Patient with Recurrent Lower Respiratory Tract Infection

Ann Marie Ramsey
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA

A 5-year-old female born with myelomeningocele at the

L3–6 level and Chiari malformation with shunted hydro-
• Flovent (fluticasone propionate 44 μg) two puffs
twice daily;
cephalus and acquired central hypoventilation requires tra-
cheotomy and ventilator support during all times of sleep.
• tobramycin 300 mg nebulizer mist treatment twice daily,
28 days on and 28 days off;
She is admitted to the pediatric intensive care unit (PICU)
with acute-on-chronic respiratory failure in the setting of
•• loratadine 5 mg daily;
ranitidine 84.5 mg twice daily.
an acute febrile illness.
Immunizations are up to date for age. She has had
the 2019 seasonal influenza vaccine. Received the poly-
History of Present Illness saccharide pneumococcal vaccine in 2016 (next dose
due 2021).
This patient was in her baseline state of health until 3 days The patient also has allergies to latex and levofloxacin
prior to admission when she developed oxygen desatura- (adverse reaction includes severe nausea and vomiting).
tions at night requiring 1–2 L of supplemental oxygen along
with usual ventilation. She also developed thick yellow tra-
cheostomy secretions and fever to 38.3 C (101 F) axillary. Family History
Mother contacted the ventilator team nurse practitioner
who instructed mother to begin inhaled tobramycin 300 Both parents healthy, no siblings. Father with seasonal
mg nebulized twice daily and increase albuterol 2.5 mg neb- allergies.
ulized treatments to every 4 hours followed by oscillatory
vest and cough-assist therapies. Mother was also instructed
to provide additional ventilator support during the day. The Social History
patient received her first dose of tobramycin that afternoon.
Despite this treatment, she continued to have fever to Lives with both parents in a single-family home. Attends
38.9 C (102 F) and developed spasmodic coughing with all-day school program in local elementary school 5 days
post-tussive emesis. Mother again contacted the ventilator weekly. Has a nurse in school.
team staff who recommended evaluation in the emergency
department (ED).
Current Status

Past Medical History On arrival at the ED the child has a temperature of 39 C

(102.2 F), spontaneous respiratory rate of 40 breaths per
The patient is taking the following medications at home: minute, heart rate of 140 bpm, and oxygen saturation of
86% on room air. She has intercostal and substernal retrac-

• albuterol 2.5 mg nebulizer mist treatment twice daily

when well, every 4 hours when sick;
tions and is placed on the Trilogy ventilator with home set-
tings: pressure control/assured volume average pressure

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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110 Cases in Pediatric Acute Care

Table 27.1 Complete blood count.

White blood cell count 1980/mm3

Hemoglobin 11.7 g/dL
Hematocrit 35.6%
Platelet count 248 000/mm3
Red blood cell count 4.29 × 106/mm3
Mean corpuscular volume 83.0 fL
Mean corpuscular hemoglobin 27.3 pg
Mean corpuscular hemoglobin 32.9 g/dL
concentration
Red cell distribution width 13.0%
Mean platelet volume 9.0 fL
Neutrophils 82.7%
Lymphocytes 7.5%
Monocytes 6.9%
Eosinophils 2.4%
Absolute neutrophil count 9.0%

Figure 27.1 Anteroposterior chest radiograph.

Table 27.2 Basic metabolic panel.

Sodium 148 mEq/L

Potassium 4.5 mEq/L
Chloride 101 mEq/L track with 2 L/min of supplemental oxygen. With this inter-
Carbon dioxide 29 mEq/L vention the retractions resolved, respiratory rate decreased
Urea nitrogen 22 mg/dL to 24 per minute, and oxygen saturation improved to 96%.
Creatinine 0.29 mg/dL She received acetaminophen 15 mg/kg and albuterol 2.5 mg
Glucose 70 mg/dL in line with the ventilator. An intravenous catheter was
placed and she received a 20 mL/kg normal saline fluid
Calcium 9.3 mg/dL
bolus. The results of the complete blood count, basic met-
Anion gap 9 mEq/L
abolic panel, and respiratory pathogen panel are shown in
Tables 27.1–27.3. The chest radiograph is shown in
Figure 27.1. Tracheotomy sputum was obtained and sent
Table 27.3 Respiratory pathogen panel by PCR. for culture.

Test name Result

Human metapneumovirus Detected Questions

Human rhinovirus/enterovirus Detected
ventilation of 12 breaths per minute, tidal volume 145 mL
(8 mL/kg), maximum inspiratory pressure (IPAPmax)
30 cmH2O, minimum inspiratory pressure (IPAPmin)
12 cmH2O, end-expiratory pressure (EPAP) 6 cmH2O,
inspiratory time 1.2, rise 3, flow trigger sensitivity auto
Answer the following questions using the details provided.
1 What is the differential diagnosis?
2 What is the pathophysiology associated with the diag-
nosis and how does it affect the patient’s respiratory
status?
3 What is the evidence-based treatment plan for this
patient?

Chapter 27 Complex Patient with Recurrent Lower Respiratory Tract Infection
Rational and Evidence-based Practice
Explanation
What is the differential diagnosis?
The primary cause for this patient’s symptoms is an acute
respiratory viral co-infection with human rhinovirus/
enterovirus (the PCR test cannot distinguish between
enterovirus and rhinovirus) and human metapneumo-
virus. Because of this infection, she demonstrated
acute airway inflammation as noted by symptoms of
cough and wheeze and peribronchial infiltrates on the
chest radiograph. She is also demonstrating hypersecre-
tion of mucus from the airway. Viral infection likely
contributes to overgrowth of existing colonizing
bacteria, triggering even further inflammation. The left
shift in the differential white count supports a bacterial
component.
The patient has additional risk factors to consider,
including the potential for aspiration due to her history
of vocal cord paralysis. There is also evidence of mild
dehydration as noted by the elevated sodium and blood
urea nitrogen on laboratory assessment. She has been una-
ble to tolerate tube feedings secondary to post-tussive
emesis.
The patient has multiple risk factors for severe compli-
cations from respiratory viral infections. The presence of a
foreign body in the airway (tracheostomy tube) increases
the risk of bacterial colonization and leads to the forma-
tion of biofilm (a film of bacteria that adheres to a surface)
which is difficult to eradicate. Children with tracheostomy
requiring ventilator support have been identified as hav-
ing higher risk for hospital readmission. Pseudomonas aer-
uginosa has been specifically linked to an increased risk of
readmission (McCaleb et al., 2016; Russell et al., 2017).
This patient has had multiple trach cultures positive for
this organism. The relationship between an acute viral
infection and bacterial overgrowth is not well understood.
There is speculation that increased vascular permeability
and secretions provide a nutritive media which promotes
bacterial organism proliferation. Viral infection may
depress the capacity of airway epithelial and immune cells
to clear bacteria. In practice, many experts note that chil-
dren with an acute viral infection as documented on viral
panel also demonstrate organism growth on tracheal cul-
ture. There continues to be debate on the importance of
treating these organisms as some assert these are simply
colonizing organisms. Other experts note patients often
demonstrate more timely improvement when treated with
antibiotics.
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111
What is the pathophysiology associated with the
diagnosis and how does it affect the patient’s
respiratory status?
The airway edema and swelling result in decreased airway
radius, and an associated increase in airway resistance,
therefore necessitating an increased work of breathing to
overcome the resistance. Also, there is an increase in atelec-
tasis due to airway inflammation and mucus plugging, and
this results in decreased lung compliance, forcing the
patient to generate higher negative pressures to move the
same volume of air.
What is the evidence-based treatment plan for this
patient?
The patient will require supportive care to allow her
immune system to respond to the viral pathogens, and this
includes respiratory support to reduce the work of breath-
ing and promote optimal ventilation and oxygenation. The
goal should be to acquire normal blood gases. A key factor
in children in acute respiratory failure secondary to viral
infection is airway inflammation leading to air trapping.
The patient will benefit from a 5-day course of methylpred-
nisolone 2 mg/kg daily. She should continue receiving albu-
terol 2.5 mg every 2–4 hours around the clock with chest
physiotherapy and suctioning.
The ventilator may require adjustments based on
blood gas analysis and patient status. The clinician
may be tempted to treat elevation of PCO2 by temporarily
increasing respiratory rate. This may be appropriate if
the child’s respiratory drive is depressed, but if airway
obstruction is severe, increasing the rate will decrease
the time available for exhalation and only make air trap-
ping worse. Increasing IPAP or tidal volume may also
worsen air trapping if there is not enough time for exha-
lation, leading to a condition called “auto-peep.”
Decreasing inspiratory time to allow more time for exha-
lation may help. As the child responds to anti-
inflammatory therapy and blood gases improve, she
may be slowly returned to home settings.
Nutrition and hydration are other goals which support
healing and patient improvement. As anti-inflammatory
agents take effect, the cough and post-tussive emesis should
improve. As symptoms improve, gastrostomy tube feedings
with an electrolyte solution running continuously may be
an initial method to begin feeding and, if tolerated, slowly
advance to a full maintenance volume, ultimately to her
home nutrition regimen. Intravenous fluids would then
be discontinued.

112 Cases in Pediatric Acute Care
Empiric antibiotic therapy with ceftazidime and gentami-
cin, along with inhaled tobramycin 300 mg twice daily,
should continue until final culture and sensitivities are
known. At this point in her admission, the patient is now
improving. Blood gases have normalized and she has
returned to home settings on room air. She is tolerating
1 hour off ventilator sprints and home tube feeding
regimen. Tracheotomy secretions are white but remain
thick and she continues to need tracheotomy suctioning
once or twice hourly.
Continuation of case: On day 3 of admission the culture
results are available (Table 27.4).
4 Based on the culture results, what antibiotics, if any,
would you prescribe for discharge and should the
inhaled tobramycin be continued?
Table 27.4 Sputum culture results.
Gram stain, respiratory
Abnormal: numerous polymorphonuclear leukocytes,
numerous Gram-negative rods
Respiratory culture/smear
Abnormal: Stenotrophomonas maltophilia complex,
Pseudomonas aeruginosa
Susceptibility
Stenotrophomonas maltophilia
Doxycycline: MIC ≤2 μg/mL
Levofloxacin: MIC 4 μg/mL (intermediate)
Minocycline: MIC ≤2 μg/mL (sensitive)
Trimethoprim/sulfamethoxazole: MIC ≤0.5 μg/mL (sensitive)
Pseudomonas aeruginosa
Amikacin: MIC 4 μg/mL (sensitive)
Aztreonam: MIC 8 μg/mL (sensitive)
Cefepime: MIC 4 μg/mL (sensitive)
Ciprofloxacin: MIC 0.5 μg/mL (sensitive)
Colistin: MIC 2 μg/mL (sensitive)
Gentamicin: MIC 2 μg/mL (sensitive)
Imipenem: MIC 4 μg/mL (intermediate)
Levofloxacin: MIC 2 μg/mL (sensitive)
Meropenem: MIC ≤1 μg/mL (sensitive)
Piperacillin/tazobactam: MIC 8 μg/mL (sensitive)
Tobramycin: MIC 8 μg/mL (resistant)
MIC, minimum inhibitory concentration.
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Based on the culture results, what antibiotics, if
any, would you prescribe for discharge and should
the inhaled tobramycin be continued?
The presence of numerous polymorphonuclear leukocytes
and two organisms in the culture is suggestive of a bacterial
infection in the lower airways. In order to treat both organ-
isms, this patient will benefit from a course of trimetho-
prim/sulfamethoxazole (Bactrim) which will treat the
Stenotrophomonas maltophilia infection. At a minimum,
she should continue the inhaled tobramycin to complete
the 28-day course. Some experts would endorse systemic
treatment of Pseudomonas by adding ciprofloxacin to the
antimicrobial regimen (Abu-Salah and Dhand, 2011).
The use of inhaled antibiotics for children with trache-
otomy and ventilator dependence with acute lower respi-
ratory bacterial infections is not well studied. There is an
emerging body of evidence studying their use in adults
requiring invasive ventilation in the critical care setting
(Abu-Salah and Dhand, 2011). The current opinion favors
inhaled antibiotics as an adjunct to systemic therapy.
The patient demonstrated pseudomonal resistance to
tobramycin in culture; however, it is important to recog-
nize that “resistance” is determined based on antibiotic
levels typically obtained during systemic administration.
During inhaled antibiotic therapy, concentrations of anti-
biotics in the lung are significantly higher than those
achieved during systemic administration, and therefore
Pseudomonas infection may be susceptible to inhaled
tobramycin by virtue of the greater concentration from
topical delivery. However, If the patient continues to dem-
onstrate recurrent lower respiratory bacterial infections
with tobramycin-resistant Pseudomonas, she may benefit
from transition to inhaled colistin (Vardakas et al., 2018)
or aztreonam.
Continuation of case: After receiving doses of ceftazidime
and gentamicin in the emergency room, the child was
admitted to the PICU on the ventilator (via tracheostomy)
with 2 L/min of supplemental oxygen, and normal saline
running at maintenance. She was placed nil by mouth
and the gastrostomy tube vented to gravity. There was
approximately 30 mL of light green mucoid drainage
in the gastrostomy tube drainage bag. Vital signs on admis-
sion as follows: temperature 37.5 C (99.5 F), heart rate
124 bpm, respiratory rate 24 per minute, blood pressure
96/66 mmHg, and oxygen saturation 95%. She continued
to have intermittent spasmodic coughing with retching
and emesis. On auscultation the breath sounds were coarse
with diffuse wheezing throughout. She required almost
constant tracheotomy suctioning for large amounts of thick
green/yellow secretions.

Chapter 27 Complex Patient with Recurrent Lower Respiratory Tract Infection
Case Resolution
The indications for discharge are as follows:
••
patient remains afebrile
patient tolerates oral feeds
References
Abu-Salah, T. and Dhand, R. (2011). Inhaled antibiotic therapy
for ventilator associated tracheobronchitis and ventilator
associated pneumonia: an update. Advances in Therapy 28
(9): 728–747. doi: 10.1007/s12325-011-0051-z.
McCaleb, R., Warren, R.H., Willis, D., et al. (2016). Description
of respiratory microbiology in children with long term
tracheostomies. Respiratory Care 61(4): 447–452.
Russell, C.J., Simon T.T., Mamey, M.R., Newth, C.J.L., and
Neely, M.N. (2017). Pseudomonas aeruginosa and post
Further Reading
Nackers, A., Ehlenbach, M., Kelly, M.M., et al. ((2019).
Encounters from device complications among children with
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113
• patient requires pulmonary toileting no more frequently
than every 4 hours
• patient back or close to home ventilator settings without
any extra oxygen requirement
• patient tolerating oral and inhaled antibiotics.
tracheotomy bacterial infection readmissions.
Pediatric Pulmonology 52(9): 1212–1218. doi: 10.1002/
ppul.23716.
Vardakas, K.Z., Voulgaris, G.L., Samonis, G., and Falagas, M.E.
(2018). Inhaled colistin monotherapy for respiratory tract
infections in adults without cystic fibrosis: a systemic
review and meta-analysis. International Journal of
Antimicrobial Agents 51(1): 1–9. doi: 10.1016/j.
ijantimicag.2017.05.016.
medical complexity. Hospital Pediatrics 9(1): 6–15. doi:
10.1542/hpeds.2018-0103.

28
Child with Racing Heart
Bradley Goettl
University of Texas Health, San Antonio, TX, USA
A previously healthy 5-year-old female was brought to the
emergency department (ED) for evaluation of irritability
and racing heart. The triage nurse noted a rapid heart rate
and the child was admitted to a monitored bed.
History of Present Illness
Caregivers reported that the child complained of a racing
heart after playing outside yesterday. Since then, the
patient has been irritable, anxious, and not eating well.
Fever, pain, vomiting, constipation, and diarrhea were all
denied. There were no reported sick exposures.
Past Medical History
She was born at 37 weeks’ gestation. She has had no prior
hospitalizations and has an established primary care pro-
vider. All vaccinations are up to date for age, including
influenza.
Past Surgical History
No previous surgeries.
Family History
Parents deny family history of heart disease, arrhythmias,
sudden cardiac death, or pulmonary diseases.
Social History
She lives with her parents. Has no siblings. Parents deny
tobacco or drug exposure.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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115
Current Status
On admission, she is awake, alert and anxious. Her respira-
tions are shallow with a respiratory rate of 28 breaths per
minute with mild subclavicular retractions. The lung
sounds are clear bilaterally. Her skin is warm but pale.
Her capillary refill time is 2 seconds with peripheral pulses
present and no peripheral edema. Her abdomen is soft,
non-distended, and without hepatosplenomegaly.
Vital signs are as follows: oral temperature 36.4 C
(97.6 F), heart rate 250 bpm, respiratory rate 28 breaths
per minute, blood pressure 104/62 mmHg, and oxygen sat-
uration (SpO2) 98% on room air.
Questions
Answer the following questions using the details provided.
1 What should be your initial approach to managing this
patient?
2 What initial diagnostic testing is needed for this
patient?
3 How would you interpret the electrocardiogram (EKG)
shown in Figure 28.1?
4 Which additional diagnostic studies would be appropri-
ate to order in the emergency department?
Rationale and Evidence-based
Practice Explanation
What should be your initial approach to managing
this patient?
After assuring adequate airway and breathing, oxygen
would be ordered if there was a concern for hypoxemia.
There are no signs of shock, but he is at risk of

116 Cases in Pediatric Acute Care
Figure 28.1 EKG 1.
decompensation due to significant tachycardia and requires
intravenous access. If it is not possible to achieve intrave-
nous access rapidly, intraosseous catheter placement would
be indicated. She is placed on a cardiac monitor to follow
her heart rate and rhythm.
What initial diagnostic testing is needed for
this patient?
An EKG is needed to determine if this is a narrow- or wide-
complex tachycardia.
How would you interpret the EKG shown
in Figure 28.1?
This EKG demonstrates narrow-complex tachycardia with
heart rate of 252 bpm.
Which additional diagnostic studies would be
appropriate to order in the emergency
department?
An EKG should be obtained with each rhythm change.
A metabolic panel should be obtained, including calcium,
magnesium, and potassium. Electrolyte imbalances and
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dehydration/hypovolemia can contribute to the develop-
ment of an arrhythmia. Severe anemia can cause tachycar-
dia, so a complete blood count should be obtained. A urine
drug screen will evaluate for toxicologic causes for tachycar-
dia. A thyroid-stimulating hormone (TSH) level can be
obtained. It is unlikely that the child will have thyrotoxico-
sis, but L-thyroxine ingestion can cause tachyarrhythmias.
A chest radiograph will evaluate for radiologic evidence of
acute cardiopulmonary disease and cardiomegaly.
A point-of-care ultrasound (POCUS) limited echocardio-
gram can quickly evaluate overall cardiac function and
quickly evaluate for pericardial effusion or right heart strain.
A formal echocardiogram will likely be obtained after
admission to evaluate cardiac function and wall motion
abnormalities and to rule out congenital heart defects.
Continuation of case: Diagnostic studies were initiated,
including chest radiograph, basic metabolic profile, com-
plete blood count, urine drug screen, cardiac ultrasound,
and 12-lead EKG. Results for basic metabolic profile and
complete blood count are shown in Tables 28.1 and 28.2.
The chest radiograph (Figure 28.2) shows the lung fields
free of infiltrate, edema and atelectasis. No significant pleu-
ral effusions were noted and there is no cardiomegaly.
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Chapter 28 Child with Racing Heart 117

Table 28.1 Basic metabolic profile. 6 What are the treatment options for this type of
tachyarrhythmia?
Sodium 141 mEq/L
7 What is unique about the EKG shown in Figure 28.3?
Potassium 4.2 mEq/L What is the relationship between this EKG and the
Chloride 109 mEq/L EKG in Figure 28.1?
Carbon dioxide 21 mEq/L
Blood urea nitrogen 8 mg/dL Based on the information provided, what is the
Creatinine 0.2 mg/dL
differential diagnosis and the most likely
diagnosis for this child?
Glucose 104 mg/dL
Magnesium 2.5 mg/dL The differential diagnosis includes, but is not limited to,
Thyroid stimulating hormone 0.625 mU/L palpitations, dysrhythmia including supraventricular tach-
ycardia (SVT) and Wolff–Parkinson–White syndrome,
Long–Ganong–Levine syndrome, electrolyte abnormality,
infection/sepsis, overdose/toxidrome, congenital malfor-
Table 28.2 Complete blood count.
mation, and cardiac/respiratory abnormalities.
White blood cell count 12 000/mm3
Wolf–Parkinson–White (WPW) syndrome is the most
common accessory pathway syndrome and the most likely
Hemoglobin 11 g/dL
diagnosis for this child. The diagnosis can be made from the
Hematocrit 32.7%
EKG. Often, WPW is found on routine EKGs and/or a
Platelet count 368 000/mm3 sports physical. For some patients, the initial presentation
could be for a symptomatic SVT or other tachyarrhythmia.

What are the treatment options for this type of

Figure 28.2 Chest radiograph.
Other results were as follows: (i) urine drug screen was
negative; (ii) point-of-care cardiac ultrasound showed nor-
mal cardiac function with no effusion or signs of right heart
strain; and (iii) 12-lead EKG indicated normal right ventric-
ular size and systolic function and normal left ventricular
size, wall thickness, and systolic function.
5 Based on the information provided, what is the differ-
ential diagnosis and the most likely diagnosis for
this child?
tachyarrhythmia?
This patient was in stable tachyarrhythmia due to his phys-
ical examination findings and vital signs. The following
should be considered as first-line therapies.
1 Vagal maneuvers: ice pack to eyes and forehead or ask-
ing the patient to blow through an occluded straw are
options for a child of this age.
2 Intravenous adenosine 0.1 mg/kg rapidly, followed
immediately by 10 mL normal saline flush. Prepare a
second dose of adenosine 0.2 mg/kg, double the initial
dose, if the first dose of adenosine is not successful in
converting rhythm.
3 If unable to obtain intravenous/intraosseous access,
or the rhythm is refractory to a second dose of adeno-
sine, perform synchronized cardioversion. Initial syn-
chronized cardioversion would be administered at
0.5–1 J/kg.
4 Pediatric cardiology and/or electrophysiology consult.
This patient’s rhythm did not convert with vagal maneu-
vers or adenosine and he was cardioverted with 1 J/kg.
After cardioversion, the EKG shown in Figure 28.3 was
obtained. Her heart rate dropped to 131 bpm. A pediatric
cardiology and electrophysiology consult should be
obtained. Initially, antiarrhythmics are prescribed to pre-
vent SVT. Common antiarrhythmics include, but are not
limited to, digoxin, beta-blockers, and amiodarone. Infants
with this diagnosis often outgrow the symptoms of the syn-
drome by 1 year of age. Older children should have limited

118 Cases in Pediatric Acute Care
Figure 28.3 EKG 2.
vigorous activity until cleared by cardiology. An electrophy-
siologic study and cardiac radiofrequency ablation is con-
sidered the gold standard for patients with recurrent
arrhythmia or those at high risk for sudden cardiac death.
What is unique about the EKG shown in
Figure 28.3? What is the relationship between this
EKG and the EKG in Figure 28.1?
This EKG demonstrated sinus tachycardia with a heart rate
of 131 bpm. A WPW pattern is recognized, including
Further Reading
American Heart Association. Web-based integrated guidelines
for cardiopulmonary and emergency cardiovascular care.
Part 12. Pediatric advanced life support. https://
eccguidelines.heart.org/index.php/circulation/cpr-ecc-
guidelines-2/part-12-pediatric-advanced-life-support/
Chu, P.Y., Hill, K.D., Clark, R.H., Smith, P.B., and Hornik, C.P.
(2015). Treatment of supraventricular tachycardia in
infants: analysis of a large multicenter database. Early
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(i) shortened PR interval of less than 120 ms; (ii) delta wave,
i.e. initial slurring of the QRS; (iii) QRS greater than 110 ms;
and (iv) ST-segment and T-wave changes indicating repo-
larization abnormalities.
Delta waves are diagnostic for WPW and represent early
ventricular depolarization via an accessory pathway. Atrio-
ventricular reentrant tachycardia is a common form of par-
oxysmal SVT for patients with WPW syndrome.
Human Development 91: 345–350. doi: 10.1016/j.
earlhumdev.2015.04.001.
Cohen, M.I., Triedman, J.K., Cannon, B.C., et al. (2012).
PACES/HRS expert consensus statement on the
management of the asymptomatic young patient with a
Wolff–Parkinson–White (WPW, ventricular preexcitation)
electrocardiographic pattern. Heart Rhythm 9(6): 1006–1024.
doi: 10.1016/j.hrthm.2012.03.050.
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119

29

Teen with Headache and Seizure

Valarie Eichler
Dallas Children’s Hospital, Dallas, TX, USA

A 15-year-old girl with recent past medical history of Past Surgical History
headaches, otherwise healthy, was admitted to the inten-
sive care unit with altered mental status and new-onset Tonsillectomy and adenoidectomy at age 3 years for recur-
seizures. rent streptococcal infections.

History of Present Illness
The adolescent reported intermittent headaches that have
been intensifying over the past 3 weeks prior to admission.
She was seen by her primary care provider and noted to
have a strong family history of migraine headaches. She
was prescribed ibuprofen and sumatriptan. She has used
sumatriptan twice in the past 2 weeks, most recently on
the day prior to admission. On the night prior to admis-
sion, she experienced nausea and emesis twice and pain
that was not relieved by ibuprofen or sumatriptan. She
was found by her sister at approximately 4:30 a.m. when
she screamed out and then began “shaking” and the emer-
gency medical service was called. On their arrival approx-
imately 10 minutes later, the adolescent was unresponsive
but breathing. En route to the emergency department
(ED), she began to have seizure activity again which was
initially focal on the left hand and arm, then generalized
to a tonic–clonic seizure. She was given one dose of rectal
diazepam for her seizure, became apneic and was intu-
bated for airway control.
Past Medical History
Born at term. She was discharged home with her mother on
day 2 of life. She is up to date on her immunizations for age,
including the influenza vaccine for the season. She has oth-
erwise been healthy. Last menses 3 weeks ago.
Family History
Positive history of maternal and paternal migraines.
Social History
Lives in a house in an urban city with parents and teenage
sister, no pets, no smokers, attends 10th grade and partici-
pates in competitive gymnastics and swim team. Family
denies drug, tobacco or alcohol use. She is not sexually
active and has not taken any contraceptives in the past.
Current Status
Five minutes after her arrival in the ED she begins having
tonic–clonic seizures and was given intravenous lorazepam
2 mg and an intravenous load of levetiracetam 60 mg/kg.
Vital signs are as follows: heart rate 77 bpm, respiratory
rate 16 breaths per minute (hand ventilating), blood pres-
sure 148/92 mmHg, oxygen saturation 98% with 100% bag
ventilation. Blood is obtained for arterial blood gas analysis,
complete blood count with differential, electrolytes, glucose,
coagulation panel (PT/PTT/INR), lactate, pregnancy test
(human chorionic gonadotropin), and blood culture. Urine
was collected for urinalysis and urine drug screen.
Results of the basic metabolic profile, complete blood
count and coagulation panel, and blood gas analysis are
shown in Tables 29.1–29.3. Test for β-human chorionic
gonadotropin was negative. Urine drug screen pending.

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© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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120 Cases in Pediatric Acute Care

Table 29.1 Basic metabolic profile. and is hemodynamically stable. It should be assumed that
the child has increased intracranial pressure (ICP), based
Sodium 141 mEq/L
on the rapid neurologic decline manifested by the seizure,
Potassium 4.2 mEq/L and should be treated with intravenous hypertonic (3%)
Chloride 102 mEq/L saline 5 mL/kg prior to obtaining additional neuroimaging.
Carbon dioxide 21 mEq/L Emergent treatment is the same as any other patient with
Blood urea nitrogen 11 mg/dL an acute neurologic decline and signs and symptoms of
Creatinine 0.5 mg/dL increased ICP: positioning, hyperosmolar therapy, mild
Glucose 147 mg/dL
hyperventilation, temperature control, seizure control,
sedation, analgesia, and pharmacologic paralysis if needed.
Lidocaine is also an important medication to use prior to
Table 29.2 Complete blood count. any noxious stimulus as it blunts the sympathetic response
so as not to increase the heart rate and blood pressure
White blood cell count 7200/mm3 acutely, as this increases cerebral blood flow/volume
Hemoglobin 9.3 g/dL thereby exacerbating ICP. She is given a fluid bolus of nor-
Hematocrit 28.5% mal saline 20 mL/kg and ceftriaxone at meningitic dosing.
Platelet count 99 000/mm3
Prothrombin time (PT) 15 s
Which imaging studies would be appropriate for
International normalized ratio (INR) 1.7 this patient?
Partial thromboplastin time (PTT) 37 s
A chest radiograph and head computed tomography (CT)
are obtained. A chest radiograph is indicated to evaluate
Table 29.3 Blood gas analysis. the position of the endotracheal tube and the presence of
underlying lung disease. The chest radiograph reveals clear
pH 7.37 lung fields and a correctly positioned endotracheal tube.
PaCO2 37 mmHg After initial stabilization has occurred, a non-contrasted
PaO2 109 mmHg CT scan of the brain should be the next study. CT scan is
Bicarbonate 20 mEq/L the initial diagnostic study for any acute change in neuro-
Base excess 1.9 logic status to evaluate for underlying hemorrhage, mass,
Lactate 1.9 mmol/L ventricle size, and presence of edema. The head CT
revealed a large right temporal–parietal intraparenchymal
hemorrhage that extended into the right lateral ventricle
Questions and fourth ventricle with a 4-mm shift to the left and efface-
ment of the basal cisterns (Figures 29.1 and 29.2). Neuro-
Answer the following questions using the details provided. surgery was consulted to evaluate for surgical intervention.

1 What are the immediate next steps in intervention for

this patient?
2 Which imaging studies would be appropriate for this
patient?
3 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this patient?
Rationale and Evidence-based Practice
Explanation
What are the immediate next steps in intervention
for this patient?
Initially, ensure the patient has a secure airway, adequate
oxygenation and ventilation via mechanical ventilation,
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for this
patient?
Differential diagnoses for this child include new-onset
seizure disorder, migraine headache, Moyamoya disease,
medial or posterior cerebral artery stroke, vein of Galen
malformation, cerebral aneurysm, intracranial hemor-
rhage related to blunt trauma, cavernous hemangioma,
subarachnoid hemorrhage, meningitis, hypertensive intra-
cranial hemorrhage, and hemorrhagic neoplasm.
The head CT scan revealed an intraparenchymal hemor-
rhage which allowed narrowing of the differential diagno-
sis. Based on the presenting symptoms, history of prior
unsuccessful treatment of migraine headache, and acute
neurologic decompensation, the most likely diagnosis for

Figure 29.1 CT head.
Figure 29.2 CT head: intraventricular extension of hemorrhage.
this teenager is an intracranial hemorrhage from a ruptured
arteriovenous malformation (AVM).
Continuation of case: After returning from the radiology
department, the patient is admitted to the intensive care
unit for further management. A ruptured AVM is consid-
ered a medical emergency secondary to the cerebral edema
and increased ICP associated with the hemorrhage. Neuro-
surgery reviewed the patient and neuroimaging to evaluate
for placement of an intracranial monitoring device. Options
included an external ventricular drain (EVD) or a fiberoptic
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Chapter 29 Teen with Headache and Seizure 121
monitor. Placement of an EVD would allow for not only
monitoring of ICP but also removal of cerebrospinal fluid
(CSF), which leads to a decrease in ICP as more room is cre-
ated with the fluid drainage. This may be performed at the
bedside. However, if the hematoma is causing mass effect
and a midline shift that may be exerting downward pres-
sure and which could lead to herniation of the brainstem
and brain death, the patient should be taken to the operat-
ing room for evacuation of the hematoma and possibly a
decompressive craniectomy to allow room for the brain
to swell during this acute phase.
4 How would you determine if the therapy was effective?
5 What other therapies would be recommended at
this time?
6 What additional imaging would be warranted?
7 What would be considered the next step in surgical
intervention?
How would you determine if the therapy
was effective?
Continued frequent neurologic monitoring of ICP, pupils,
and response to pain or other stimuli is paramount. The
goal is to prevent secondary injury and decrease the mor-
bidity associated with a ruptured AVM. If after all the inter-
ventions described the ICP fails to fall to an acceptable level
(<20 cmH2O), then the patient may be placed into a barbi-
turate coma to slow cerebral metabolism and allow the
brain time to cool down. Monitoring with a continuous
electroencephalogram (EEG) will allow the provider to
observe for burst suppression. The usual goal is one burst
per 10 seconds or five to six bursts per minute. An epilep-
tologist would be helpful in reading the EEG and reporting
results periodically to the provider. Of note, when using
barbiturates the patient may become hemodynamically
unstable and require fluid boluses and inotropic support.
This is usually managed with the use of an epinephrine
or norepinephrine infusion. Arterial and central lines are
needed to adequately monitor and provide medications to
the patient.
The overall goal is an ICP of less than 20 cmH2O. To
ensure this, frequent neurologic monitoring, physical
examinations, maintaining serum sodium goals of
150–155 mEq/L, and repeated head CT scans are needed
to assess the effectiveness of therapy. Monitoring of the
child’s ICP, arterial blood pressure, cerebral perfusion pres-
sure, PaCO2 and PaO2 are all components of the examination
needed to verify effectiveness of treatment. Maintaining
adequate sedation, analgesia, temperature control, and sei-
zure control will help ensure that ICP goals are met. The
goal is to maintain a cerebral perfusion pressure (CPP)

122 Cases in Pediatric Acute Care
adequate to perfuse the brain tissue. General CPP goals are
as follows: infants 40–50 mmHg, children 50–60 mmHg,
adolescents 60–65 mmHg.
What other therapies would be recommended at
this time?
If the ICP is refractory to medical management, the neuro-
surgeon may be asked to perform a decompressive craniect-
omy on the child. This is the surgical removal of the skull
over the affected region to allow more room for the brain to
swell during the acute phase, which is usually 72–96 hours.
After the acute phase has resolved and the child is stable
and ICP is no longer an issue, the surgeon will replace
the bone flap in a procedure called cranioplasty.
What additional imaging would be warranted?
After the patient is stable from a clinical standpoint, and
ideally after a period of “cooling off” that allows for reab-
sorption of the hematoma, the patient should be evaluated
with cerebral angiography (Figure 29.3). Considered the
gold standard, this study identifies the arteries involved
in the AVM, the nidus, and the large draining vessels. If
the angiogram is performed too early in the course, the
hematoma and cerebral edema surrounding the hematoma
may obscure or hide important vasculature.
What would be considered the next step in
surgical intervention?
Approach and successful excision of the AVM is depend-
ent on the location. If surgical clipping and obliteration
Figure 29.3 Cerebral angiogram reveals right middle cerebral artery AVM.
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is performed, intraoperative angiography or magnetic
resonance angiography would be obtained to evaluate
the removal of the AVM. If embolization is undertaken,
it is usually with a staged approach. Specialized glue,
beads or coils are used and deployed into the involved
vessel(s) to decrease the size of the nidus and ultimately
cease blood flow to the AVM. Embolization may be cur-
ative or may be performed prior to surgery to decrease
the size of the AVM to enhance successful removal. If
the AVM is located deep in the parenchyma, then a
Gamma knife (stereotactic radiosurgery) may be used
to focus high-energy beams of radiation to a specific tar-
geted area, ultimately reducing injury to the tissue near
the target.
An AVM is defined as a congenital intracranial mal-
formation distinguished by a persistently abnormal con-
nection between arteries and veins within the brain
without an interposed or developed capillary bed.
AVMs develop during the third week of gestation when
the capillary bed fails to develop between the arteries
and veins. They are seen in approximately 1% of the
pediatric population, with 1 in 100 000 children having
AVMs that are asymptomatic. The majority of AVMs
are supratentorial and within the parenchyma of the
brain. They are often associated with neurologic deficits
such as hemiparesis and seizures and, depending on the
location, vision and speech may be affected. A small
percentage of the time they are found in the brainstem,
thalamus, basal ganglia, and cerebellum. These smaller
group of AVMs are more detrimental because of their
location and often lead to catastrophic outcomes includ-
ing death.

Case Resolution
The child successfully recovered after surgical removal of
the AVM involving the right middle cerebral artery.
There was residual left-sided weakness but this was
improving with intense inpatient rehabilitation. After
the acute management has been completed, it is
Further Reading
Eichler, V. (2016). Neurologic disorders: arteriovenous
malformation. In: Lippincott Certification Review: Pediatric
Acute Care Nurse Practitioner (ed. A.M. Kline-Tilford and C.
Haut), 181–184. Philadelphia, PA: Wolters Kluwer.
Marcoux, K.K. (2012). Arteriovenous malformation. In:
Pediatric Acute Care: A Guide for Interprofessional Practice
(ed. K. Rueter-Rice and B. Bolick), 916–920. Burlington, MA:
Jones & Bartlett Learning.
Meling, T.R. and Patel, G. (2019). What is the best therapeutic
approach to a pediatric patient with a deep-seated brain
AVM? Neurosurgical Review 42(2): 409–416. doi: 10.1007/
s10143-019-01101-8.
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Chapter 29 Teen with Headache and Seizure 123
important to continue to support the patient throughout
the recovery and convalescent phase. This is accom-
plished through a multidisplinary approach with physi-
cal therapy, occupational therapy, child life specialists,
and physical medicine and rehabilitation services. When
medically stable the patient is transferred to an inpatient
rehabilitation facility.
O’Brien, N.F. (2012). Traumatic brain injury. In: Pediatric
Acute Care: A Guide for Interprofessional Practice (ed. K.
Rueter-Rice and B. Bolick), 1197–1203. Burlington, MA:
Jones & Bartlett Learning.
Ravindra, V.M., Bollo, R.J., Eli, I.M., et al. (2019). A study of
pediatric cerebral arteriovenous malformations: clinical
presentation, radiological features, and long-term functional
and educational outcomes with predictors of sustained
neurological deficits. Journal of Neurosurgery Pediatrics 24
(1): 1–8. doi: 10.3171/2019.2.PEDS18731.

30
A 4-Year-Old with Bloody Diarrhea
Tamara Hill
University of Maryland Medical Center, Baltimore, MD, USA
A 4-year-old female is brought to the emergency depart-
ment (ED) by her mother with a chief complaint of bloody
diarrhea and is later admitted to the pediatric intensive care
unit (PICU).
History of Present Illness
This 4-year-old child became ill after attending a birthday
party 7 days prior where she had consumed hot dogs and
hamburgers and went swimming in a nearby lake. She
has had no fever, ill contacts, or recent exposure to children
with diarrhea.
She is normally a very energetic and happy child but for a
week now she has been fussy and tired with abdomi-
nal pain.
Past Medical History
The child was born via uncomplicated spontaneous vaginal
delivery at term and went home with her mother. She is
current on all immunizations, including influenza vaccine.
She has no other medical history.
Past Surgical History
The child has not had any surgical procedures.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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125
Family History
Mother has a history of seizures and takes medication for
same. No other family history.
Current Status
The child is alert but fussy, appears pale, and is non-toxic.
Her conjunctiva are pale, pharynx and tonsils appear nor-
mal, and tympanic membranes intact. Her neck is supple
without adenopathy. Cardiac examination notes a regular
rhythm with tachycardia and a grade II/VI vibratory systolic
ejection murmur at the left sternal border without radiation.
No heaves, lifts, thrills, rubs, or gallops are present. Her lungs
are clear with good aeration and abdomen is flat, soft, and
non-tender, with the liver edge palpable 3 cm below the right
costal margin. The spleen is non-palpable. Her pulses and
perfusion are good. There is no edema, rash, or petechiae.
Vital signs are as follows: temperature 37.7 C (99.9 F),
heart rate 150 bpm, respiratory rate 28 breaths per minute,
blood pressure 100/45 mmHg, and oxygen saturation 100%
on room air. Weight is 17 kg (75th percentile).
A basic metabolic panel, complete blood count, and
urinalysis were obtained, with results as shown in
Tables 30.1–30.3. A blood peripheral smear shows schisto-
cytes, helmet cells, and polychromasia.
Questions
Answer the following questions using the details provided.
1 Based on data given, what are the differential diagnoses
and the most likely diagnosis for this child?
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126 Cases in Pediatric Acute Care

Table 30.1 Complete blood count. Table 30.3 Urinalysis.

White blood cell count 16 000/mm3 Color Clear yellow

Hemoglobin 6.9 g/dL pH 6.0
Hematocrit 22.4% Specific gravity 1.012
Platelet count 37 000/mm3 Protein 3+
Neutrophils 56% Nitrites Negative
Lymphocytes 27% Urobilirubin 1+
Monocytes 2% Blood 3+
Eosinophils 3% Leukocytes Negative
Bands 12% Ketones Negative
Glucose Negative
White blood cells 51%
Table 30.2 Complete metabolic profile and coagulation Red blood cells 21%
parameters.
Hyaline casts 3–5
Sodium 133 mEq/L Bacteria Occasional
Potassium 5.8 mEq/L Crystals None
Chloride 106 mEq/L
Carbon dioxide 13 mEq/L
Blood urea nitrogen 48 mg/dL
of cases. Bloody diarrhea and abdominal pain are usually
Creatinine 3.82 mg/dL manifestations of hemorrhagic enterocolitis caused by
Glucose 145 mg/dL Shiga toxin-producing bacteria, most commonly Escheri-
Calcium 7.8 mg/dL chia coli. Patients with Shiga toxin-associated HUS have
Albumin 3.0 mg/dL been described who do not have obvious diarrheal pro-
Total protein 6.6 g/dL dromes. On the other hand, patients with atypical HUS
Aspartate aminotransferase 28 U/L
commonly present with abdominal pain and diarrhea,
which may begin days to weeks before the patient seeks
Alanine aminotransferase 32 U/L
medical care, thus giving rise to the impression of diar-
Alkaline phosphatase 125 U/L
rhea-associated HUS. Diarrhea, with or without blood, is
Total bilirubin 0.2 mg/dL found in approximately 30% of patients with atypical
Phosphorus 7.1 mg/dL HUS at disease onset because of gut involvement. For
Lactate dehydrogenase 300 U/L this reason, categorization of HUS according to clinical
Prothrombin time 12.5 s presentation as diarrhea-positive or diarrhea-negative is
Partial thromboplastin time 41 s inappropriate.
International normalized ratio 0.9 Causes of HUS are typically related to exposure to con-
taminated food, especially undercooked beef, but can also
result from certain medications, including quinine, ciclos-
porin, tacrolimus, some drugs of abuse including cocaine,
Rationale and Evidence-based Practice
some chemotherapeutics, and antiplatelet agents.
Explanation If a patient presents with a diarrheal prodrome, Shiga
toxin-associated HUS can easily be differentiated by the
Based on data given, what are the differential
presence of Shiga toxin in the stools and/or serum antibo-
diagnoses and the most likely diagnosis for
dies against Shiga toxin. Another infectious form of HUS
this child?
occurs with T-antigen activation in association with pneu-
If a child presents with microangiopathic hemolytic ane- mococcal sepsis and this can also easily be differentiated
mia, thrombocytopenia, and renal failure preceded by from atypical HUS.
abdominal pain and diarrhea, the diagnosis can be made Other potential differential diagnoses for a child who pre-
for Shiga toxin-associated hemolytic uremic syndrome sents with bloody diarrhea include necrotizing enterocoli-
(HUS). This accounts for 90% of HUS cases in children, tis, colitis, and inflammatory bowel disease among
with Streptococcus pneumoniae responsible for at least 5% others. In this case, the child presents with very typical

symptoms and laboratory findings of hemolytic anemia,
thrombocytopenia, and renal failure.
Additional Questions and Evidence-
based Rationale
2 What is the best management for HUS?
3 How would you determine if the therapy was effective?
4 What other therapies would be recommended for HUS?
5 Would you obtain any other diagnostic studies?
What is the best management for HUS?
Therapy for HUS is supportive and includes volume reple-
tion, hypertension control, and managing complications of
renal insufficiency, including dialysis when warranted. Red
blood cell transfusions may be indicated. Platelet transfu-
sions should be avoided since they may contribute to the
thrombotic microangiopathy, and are indicated only by
active hemorrhage or prior to a procedure. Early hydration
during the diarrheal phase may lessen the severity of renal
insufficiency. Most children with Shiga toxin-associated
HUS survive the acute phase and recover normal renal
function.
Further Reading
Cody, E. and Dixon, B.P. (2019). Hemolytic uremic syndrome.
Pediatric Clinics of North America 66(1): 235–246.
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Chapter 30 A 4-Year-Old with Bloody Diarrhea 127
How would you determine if the therapy was
effective?
Electrolytes will normalize and the child will show
improved renal function along with normal blood pressure.
Early hydration during the diarrheal phase may lessen the
severity of renal insufficiency. Most children with Shiga
toxin-associated HUS recover and do not have chronic
symptoms or problems, although some may show evidence
of long-term renal complications and other morbidity.
What other therapies would be recommended
for HUS?
Patients who experience volume overload, metabolic acido-
sis, electrolyte abnormalities, uremia or oliguria may
require dialysis. Non-oliguric patients usually recover well,
but there still remains an increased risk for future renal
complications, including chronic kidney disease.
Would you obtain any other diagnostic studies?
Additional tests to include are stool culture for E. coli, stool
test for Shiga toxin, and liver enzymes. A renal ultrasound
may be helpful in determining category of acute kidney
injury.
Dixon, B. and Gruppo, R. (2018). Atypical hemolytic uremic
syndrome. Pediatric Clinics of North America 65(3): 509–525.
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129

31

Child with Persistent Fever

Misty Ellis
Norton Children’s Hospital, Louisville, KY, USA

A 5-month-old male is admitted with persistent fever for Past Surgical History
18 days.
No prior surgeries.

History of Present Illness

A 5-month-old male presents with persistent daily fever.
The fevers began 18 days ago, with temperature to 38.3 C
(101 F). He also developed an erythematous macular rash
on his torso, back, and neck with redness of feet and hands,
and had two episodes of emesis. On his second day of fever,
his temperature was 39.3 C (102.8 F) and he was taken to
the local emergency department (ED) where he was diag-
nosed with a viral infection and sent home. His mother
reports that the rash improved for a few days then returned
in conjunction with intermittent fevers, and eventually
completely resolved. On day 4 of illness, patient returned
for a follow-up visit with his primary care provider (PCP)
and again was diagnosed with a viral infection. Addition-
ally, he was prescribed eye drops for a non-exudative con-
junctivitis, which resolved after 5 days. His rash resolved,
but he continued to have daily fevers greater than 38 C
(100.4 F) (responsive to acetaminophen), and developed
cough, sneezing, and rhinorrhea. By day 9 of fevers, he
was diagnosed with respiratory syncytial virus (RSV). On
day 12 of fever, his upper respiratory infection symptoms
were improving, but due to persistent fevers he returned
to the PCP. The PCP transferred him to the ED for further
evaluation.
Past Medical History
He was born at 36 weeks via cesarean section and did not
require extended hospital stay following birth.
Family History
His father has type 1 diabetes and his mother is healthy.
His sister had a recent upper respiratory infection.
Current Status
In the ED, he was fussy with examination, but was consoled
by his mother. His examination was significant for temper-
ature of 38.5 C (101.3 F), cracked and peeling lips, breath
sounds clear to auscultation bilaterally with transmitted
upper airway sounds, but otherwise no other abnormal
examination findings.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses for this child?
2 What diagnostic tests would you obtain based on the
differential diagnoses?
Continuation of case: Diagnostic study results are shown
in Tables 31.1 and 31.2. Urinalysis showed sterile pyuria.
3 What is the most likely diagnosis?
4 What other diagnostic studies should be obtained?
5 Based on the diagnosis what is the treatment plan for
this patient?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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130 Cases in Pediatric Acute Care

Table 31.1 Complete metabolic profile. What is the most likely diagnosis?

Sodium 132 mEq/L The most likely diagnosis for this patient is Kawasaki
Potassium 4 mEq/L disease due to the patient’s clinical symptoms of fever,
Chloride 105 mEq/L
erythematous macular rash, redness of hands and feet,
non-exudative conjunctivitis, cracked/peeling lips, hypoal-
Carbon dioxide 22 mEq/L
buminemia, thrombocytopenia, elevated CRP, anemia, and
Blood urea nitrogen 14 mg/dL
sterile pyuria.
Creatinine 0.2 mg/dL Kawasaki disease is an acute self-limited vasculitis of
Glucose 98 mg/dL unknown etiology that occurs predominantly in infants
Albumin 2.4 g/dL and children. If not treated early with high-dose intrave-
Aspartate aminotransferase 153 U/L nous immunoglobulin, one in five children develop coro-
Alanine aminotransferase 200 U/L nary artery aneurysms. The clinical presentation of
Total bilirubin 0.5 mg/dL Kawasaki disease varies over time, with the clinical course
conventionally divided into three stages: acute, subacute,
Erythrocyte sedimentation rate 8.5 mm/hour
and convalescent phase. The acute stage begins with an
C-reactive protein 6.4 mg/dL
abrupt onset of fever and lasts approximately 7–14 days.
During this phase, the fever is typically high, usually ran-
ging from 39 to 40.5 C (101.2–104.9 F), and is unresponsive
Table 31.2 Complete blood count. to antibiotics or antipyretics. Additional classic clinical cri-
teria of Kawasaki disease include at least four of the five
White blood cell count 23 000/mm3 following features: extremity changes, polymorphous exan-
Hemoglobin 9.8 g/dL thema, conjunctival injection, oral/lip changes, and cervi-
Hematocrit 29.5% cal lymphadenopathy. Patients with Kawasaki disease
Platelet count 958 000/mm3 will usually have an elevated CRP and/or ESR. Kawasaki
Neutrophils 49%
disease is treated with immunoglobulin and aspirin with
the goal of preventing the development of coronary artery
Eosinophils 1%
aneurysms.
Monocytes 6%
Lymphocytes 44%
Bands 0% What other diagnostic studies should be obtained?
Other important diagnostic studies to include for this
patient would be an echocardiogram and an electrocardio-
Rationale and Evidence-based Practice gram (EKG). An echocardiogram is the primary imaging
Explanation modality for cardiac assessment during the acute phase
(Figures 31.1 and 31.2). An EKG may demonstrate sinus
Based on the data given, what are the differential node dysfunction with prolonged PR interval, and non-
diagnoses for this child? specific ST- and T-wave changes. Invasive angiography
(cardiac catheterization) can be of value for long-term fol-
Differential diagnoses for this child include RSV, adenovi- low-up of coronary artery aneurysm evaluation but is rarely
rus, enterovirus, parainfluenza virus, bacterial sepsis, utilized during the acute phase (Figure 31.3).
staphylococcal or streptococcal toxic shock syndrome,
and Kawasaki disease.
Based on the diagnosis what is the treatment plan
for this patient?
What diagnostic tests would you obtain based on
The goal of treatment is to promptly eradicate systemic and
the differential diagnoses?
tissue-level inflammation and to prevent development of
Diagnostic studies to include are complete blood count, aneurysms. Primary treatment during the acute phase is
complete metabolic panel, erythrocyte sedimentation rate high-dose intravenous immune globulin (IVIG) for
(ESR), C-reactive protein (CRP), urinalysis, urine culture, immune mediation. Aspirin is often used for antiplatelet
blood culture, and respiratory virus panel. effect to prevent the development of coronary aneurysm
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Chapter 31 Child with Persistent Fever 131

RCA

Aorta

Figure 31.1 Echocardiogram showing aorta and dilated right

coronary artery (RCA).

Figure 31.3 Cardiac catheterization showing left coronary artery

dilation.

Aorta
LCA
The following important points should be remembered.

• Infants less than 6 month of age are at significant risk of

aneurysm formation and require echocardiography every
few days until stable as well as frequently upon discharge.

• Patients with significant coronary artery aneurysm devel-

opment are at high risk for thrombosis, and therefore sys-
temic anticoagulation in addition to antiplatelet therapy
should be administered.

• Measles and varicella immunizations should be deferred

for 11 months after receiving IVIG administration.

Figure 31.2 Echocardiogram showing aorta and left coronary

artery (LCA).
• Reye syndrome has been reported in patients taking
high-dose aspirin for a prolonged period following Kawa-
saki disease. All children over 6 months of age and their
families should receive a seasonal influenza vaccine prior
from thrombus. Management is tailored based on repeat to discharge from the hospital. Inactivated vaccine
echocardiograms and measurement of coronary arteries. should be administered to children on aspirin therapy.

Further Reading
McCrindle, B., Rowley, A., Newburger, J., et al. (2017).
Diagnosis, treatment and long-term management of
Kawasaki disease. Circulation 135: e927–e999. doi: 10.1161/
cir.0000000000000484.
Newburger, J., Takahashi, M., and Burns, J. (2016). Kawasaki
disease. Journal of the American College of Cardiology 67:
1738–1749. doi: 10.1016/j.jacc.2015.12.073.

32
Quality Improvement Case Study
Peggy Dorr
University of Maryland Medical Center, Baltimore, MD, USA
An acute care nurse practitioner (ACNP) working in a pedi-
atric emergency department (ED) has noted a lack of under-
standing on how to correctly use a metered-dose inhaler
(MDI). Discussions with families indicate that most children,
regardless of age, and many parents are either not comfortable
using the inhaler or are demonstrating incorrect technique
with the device. There is also noted variance in discharge
teaching, possibly due to a recent influx of new graduate
nurses working on the unit. The nurse practitioner decides
it would be beneficial to evaluate this problem and potentially
develop a solution to benefit both staff and patients.
Understanding there are numerous stakeholders involved
in this issue, the nurse practitioner begins to develop a team
that can discuss this perceived issue and decide if interven-
tion is necessary. After a team is in place, an improvement
model can help provide guidance and structure as the prob-
lem is assessed and a plan is developed. There are numerous
models that can be utilized to guide this quality improve-
ment (QI) project.
Questions
Answer the following questions using the details provided.
1 Who should be included in this QI team and why?
2 Of the following, which process is best for this situation
and why?
a) Plan, Do, Study, Act (PDSA) cycle.
b) Root cause analysis (RCA).
c) Six Sigma (define, measure, analyze, improve and
control or DMAIC).
d) Failure mode and effects analysis (FMEA).
3 What would be an appropriate question to guide
analysis of the problem and devise a potential solution
given this case scenario?
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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133
4 What would be an appropriate goal(s) for this problem?
What are some guidelines to use when developing or
defining a goal?
5 What would be a way to filter literature articles to
ensure the retrieved data is accurate and applicable?
6 Besides developing the standardized education pro-
gram and a plan for implementation with the nursing
staff, what other factors must be discussed and
addressed?
7 Prior to implementation, is it necessary to submit your
project to your Institutional Review Board for approval?
8 Are there any final steps that could or need to be taken?
Rationale and Evidence-based Practice
Evaluation
Who should be included in this QI team and why?
The QI team should include the following key members.
• Nursing representative who would be instrumental in the
education process and best understands the needs of the
nursing group.
•
Respiratory therapists routinely work with these children
and parents and should contribute to and support the
unit’s choice of standardized education.
• Pharmacy team member represents the prescriptive
impacts, as orders for medication and medical equip-
ment, such as a spacer, would potentially be obtained
from the hospital pharmacy. Consistency in education
and any process impacts that occur from orders written
within the inpatient or outpatient settings need to be
addressed.
• Medical representative, in addition to the lead nurse
practitioner, should be invited to join this group. Physi-
cians and physician trainees should be aware of changes

134 Cases in Pediatric Acute Care
to discharge education, may contribute with recent
research or evidence-based medical practice, and may
contribute current American Academy of Pediatrics
(AAP) guidelines or recommendations.
• Nurse manager for the unit has financial and staffing
responsibilities. Nursing staff will be required to attend
educational sessions that may impact staffing numbers
or incur financial expenditures, which will impact the
unit budget.
A team working on a QI problem must comprise all key
stakeholders. A stakeholder is “anyone who has an interest
in a project or can influence its success or failure” (Silver
et al., 2016, p. 893). To ensure success, all stakeholders must
be identified, even those persons who can have a negative
influence on the outcome of the suggested project. Stake-
holder mapping can be helpful for identifying all potential
internal and external individuals and defining the relation-
ship they have to the problem being addressed (Swanson
and Pearlman, 2017, p. 113).
After all stakeholders have been identified, decisions
must be made regarding which of these to engage in the
project. Consideration must be given to motivations (e.g.
patient care, finances, process efficiency, and staff satisfac-
tion) or perspective of potential members. The team of sta-
keholders can be dynamic, based on the needs of the project
at a specific time.
In this setting, QI project groups are typically composed
of interdisciplinary team members. The team should be
small enough to promote efficiency and large enough to
provide expertise in all areas impacted by the project.
Members should be committed to review of the problem,
development of a solution, and the implementation. Care-
ful analysis of stakeholders is important to ensure accept-
ance of the suggested change or improvement, but
influence is not the only criterion to consider. The team
will require participants to take on many roles including
team lead, clinical lead, technical experts, and an execu-
tive sponsor (Silver et al., 2016, p. 894). Ensuring roles
such as these are filled will help balance the team and
encourage success.
Of the following, which process is best for this
situation and why?
Based on the identified issue, the Six Sigma process
(DMAIC) best fits this situation. Six Sigma methodologies
are used in a range of industries to improve process effi-
ciency and reduce defects. In healthcare, this means a
reduction in medical errors, an increase in customer satis-
faction, and a decrease in costs (https://www.purdue.edu/
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leansixsigmaonline/blog/healthcare-advancement-with-
lean-six-sigma/). The problem-solving methodology within
this principle is the DMAIC method, which focuses on
improving existing processes and if any deficiencies in these
processes are resolved, they will have the potential to
improve outcomes and are measurable (https://goleansix-
sigma.com/dmaic-five-basic-phases-of-lean-six-sigma).
The DMAIC mnemonic refers to specific steps defined
as follows.
Define: the problem is defined and focused. Problems
should be very specifically written to allow for better
analysis and resolution.
Measure: in the measure phase, the team needs to know
how the process operates and perform data collection
regarding best practices. This step includes the
performance of a gap analysis via a thorough literature
review.
Analyze: in this phase the team decides what are the actual
causes of the problem and these causes are validated
by data.
Improve: the goal is to determine a solution to the problem
at hand, select the best solution given the team’s input,
implement the solution, and collect data to confirm there
is measurable improvement.
Control: this phase is about sustainability. The implemen-
tation plan is reviewed and modified or refined as
necessary.
PDSA is a rapid and continuous process, more commonly
used for testing a potential change in a real work setting.
This method implements the change on a small scale, then
analyzes the results, refines the change, and re-implements
(https://innovations.ahrq.gov/qualitytools/plan-do-study-
act-pdsa-cycle), allowing for a rapid evaluation and change
process. While this method of implementing and evaluat-
ing change is routinely referenced for QI projects and will
be utilized during this project, the details provided in the
DMAIC methodology will provide concrete guidance for
this team through the entire process.
An RCA is a retrospective analysis of an untoward event
and an assessment of factors that led to the event and ave-
nues to avoid it in the future.
FMEA is a systematic prospective risk assessment to
anticipate vulnerabilities and potential adverse outcomes
of a process in order to identify the parts of that process that
most need change (http://www.ihi.org/resources/Pages/
Tools/FailureModesandEffectsAnalysisTool.aspx).
The first step in guiding an analysis of the problem and
devising a potential solution is to develop an answerable
evidence-based practice question.

What would be an appropriate question given this
case scenario?
A PICO format can help with question development.
P Patient, population or problem: consider attributes that
help narrow the focus.
I Intervention: this can be a treatment or clinical or
education intervention.
C Comparison with other interventions: will the intervention
be compared to another intervention? Not all questions
have comparisons.
O Outcomes: identify the outcomes of interest.
An appropriate PICO question for this project would be:
For pediatric patients, ages 5–18 years old and their
parents/guardians, does the use of standardized edu-
cation on the correct use of MDI device increases the
accuracy of its use and the comfort level of users with
the device?
The developed question will drive the strategies used to
search for evidence. Making the evidence-based practice
question as specific as possible helps to identify and narrow
search terms, focus the project, and communicate the target
population so the translation is appropriate (Dang and
Dearholt, 2017).
The problem of inconsistent MDI education is agreed
upon by the group. Now a decision must be made to deter-
mine as to what is a measurable goal that indicates educa-
tion has had an impact on the problem.
What would be an appropriate goal(s) for this
problem? What are some guidelines to use when
developing or defining a goal?
The acronym SMART can help when defining a goal.
SMART stands for specific, measurable, actionable, relevant,
and time bound. A SMART objective format provides a
structured approach, sets the stage for measuring perfor-
mance, succinctly communicates intended impact and
progress to stakeholders, and concretely describes how
goals will be met.
There may be several goals for this project.
1 After education is complete:
a) Nursing staff will feel confident providing the newly
standardized MDI education to patients of varying
ages and their families.
b) Documentation of MDI education in the electronic
medical record (EMR) will be greater than 90%
within 30 days of implementation.
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Chapter 32 Quality Improvement Case Study 135
c) Parents/guardians will immediately feel more confi-
dent in helping their child use the MDI.
d) Patients and parents will demonstrate correct use of
an MDI inhaler.
2 All nursing and respiratory staff of the pediatric unit
will complete the standardized MDI education within
1 month of roll-out.
These goals will help guide project design and the data
collection process. As the group decides what would be
an appropriate goal(s), they must also decide how to meas-
ure attainment of that goal. Does a tool already exist or does
one need to be developed? This will add time to project
development and implementation.
After the group agrees on the problem and the goal, a gap
analysis must be performed. This process informs the group
on the differences between the evidence-based standard of
care and their current practice. Key words are discussed
and all members perform a review of the literature. Numer-
ous articles are brought back to the next meeting for review.
What would be a way to filter literature articles to
ensure the retrieved data is accurate and
applicable?
The literature review demonstrates several research and QI
studies which have confirmed that standardized education
has a positive impact on accuracy of MDI use and improved
outcomes in asthma management. Determining a list of
guidelines for literature review can be accomplished by
using filters. For example, the following could be included:
only data from pediatric reviews or studies, English lan-
guage only, within 5 years of current date, only scientific
research from known journal/site (do not use Wikipedia,
but peer-reviewed journals), using well-known medical
subject headings (MeSH) as defined in PubMed or the
Cumulative Index to Nursing and Allied Health
Literature (CINAHL).
Besides developing the standardized education
program and a plan for implementation with the
nursing staff, what other factors must be discussed
and addressed?
Barriers to implementation
• Time for staff education: time away from clinical care,
cost of staff attendance.
• Staff members other than nursing that need to be aware
of new education format (e.g. respiratory therapy, medi-
cine, rehabilitative services, child life).
• Integration into EMR for documentation.

136 Cases in Pediatric Acute Care
• Recruitment of “champions” or key adopters to ensure
sustainability over time and act as a clinical resource
for staff with regard to process and content.
Measurement of outcomes
• Process measures: for example, is the education tool easy
to use, is documentation easy, is there a checklist to
ensure completeness?
• Outcome measures: for example, is there before and after
measurement of knowledge on use of MDI, is there
•
improvement after education, are patients tracked at
future hospitalizations or ED visits for sustained
•
performance?
• Patient and family satisfaction with the teaching method,
••
the perceived necessity of the education and the out-
comes as seen by the family are also important.
The team has developed a standardized education pro-
gram for patients and families as well as an educational
process for staff. They have also developed evaluation tools
to assess efficacy of the education for patients, families
and staff.
Prior to implementation, is it necessary to submit
your project to your Institutional Review Board for
approval?
The purpose of an Institutional Review Board (IRB) is to
protect the rights and welfare of human research subjects
who are involved in biomedical or behavioral research.
Table 32.1 Defining research versus quality improvement.
Human subject research
Purpose Designed to develop or contribute to generalizable knowledge
Starting point Knowledge seeking is independent of routine care and
intended to answer a question or test a hypothesis
Design Systematic, methodological approach
Benefits Might or might not benefit current subjects
Risks May put subjects at risk
IRB required Always required
Dissemination Expected to publish and present as an IRB-approved research
study
Adoption of Little urgency to disseminate results quickly
results
IRB, Institutional Review Board; PDSA, Plan, Do, Study, Act cycle.
Source: Adapted from Gregory (2015) and Merrill (2015) and (https://irb.research.chop.edu/quality-improvement-vs-research).
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To better understand if a project needs IRB approval,
research must be clearly defined in comparison to QI, as
both types of activities can be data driven and involve
human participants (Table 32.1). A group from the Has-
tings Center defined QI as “systematic, data-guided activ-
ities designed to bring about immediate improvements in
health delivery in particular settings” (Baily, 2016),
whereas research is a systematic investigation that includes
research development, testing and evaluation designed to
develop or contribute to generalizable knowledge.
IRB approval for QI activities is typically required if:
the project seeks to develop new knowledge or validate
new treatments;
a standard research methodology such as randomization
is used;
a protocol is rigid and fixed;
there is outside funding; and/or
• risks from participation are greater than minimal.
There may be institutional variability about submitting
QI process requests through the IRB but if there is a rapid
review process that assigns the project a title of “exempt
research” or “not human subjects” research, this is encour-
aged. When in doubt, contact your IRB staff or process a
submission.
Implementation is complete and the education proc-
ess has been ongoing for 3 months. There are several
unit-based champions who are available on various
shifts and the ACNP, nurse manager and nursing repre-
sentative have met monthly to review outcomes to date
Quality improvement
Apply known solutions to a focused problem or
process
Knowledge seeking is integral to ongoing
management system for delivering healthcare
Adaptive, iterative design; less rigorous, often
involves a PDSA
Directly benefits a process, system or program
Does not increase risk to patients with exception of
possible patient privacy or data
Typically not required but need to work through
process
Internal communication, may publish or present as
QI work
Results rapidly adopted into local care delivery

and discuss any issues that have come up. The process
seems to be well integrated into the unit’s clinical
practice.
Are there any final steps that could or need to
be taken?
• Modifications to the newly implemented plan based on
feedback or data results. The team needs to review all
results and determine the need for changes to process,
documentation or implementation based on those
results. Satisfaction data must be reviewed as well.
References
Baily, M.A. (2016). Quality improvement methods in health
care. Hastings Center Bioethics Briefings for Journalists,
Policymakers and Educators. https://www.
thehastingscenter.org/briefingbook/quality-improvement-
methods-in-health-care/ (accessed 11 June 2019).
Dang, D. and Dearholt, S.L. (2017). Johns Hopkins Nursing
Evidence-Based Practice: Model and Guidelines, 3rd edn.
Indianapolis, IN: Sigma Theta Tau International.
Gregory, K.E. (2015). Differentiating between research and
quality improvement. Journal of Perinatal and Neonatal
Nursing 29(2): 100–102.
Further Reading
Agency for Healthcare Research and Quality (2013). Plan-Do-
Study-Act (PDSA) Cycle. https://innovations.ahrq.gov/
qualitytools/plan-do-study-act-pdsa-cycle
Children’s Hospital of Philadelphia. Quality improvement vs
research. https://irb.research.chop.edu/quality-
improvement-vs-research (accessed 11 June 2019).
DMAIC: the 5 Phases of Lean Six Sigma. https://
goleansixsigma.com/dmaic-five-basic-phases-of-lean-six-
sigma (accessed 11 June 2019).
Institute for Healthcare Improvement (http://www.ihi.org).
Institute for Healthcare Improvement. Failure modes and
effects analysis (FMEA) tool. http://www.ihi.org/resources/
Pages/Tools/FailureModesandEffectsAnalysisTool.aspx
(accessed 11 June 2019).
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Chapter 32 Quality Improvement Case Study 137
• A review of recent ED admissions or readmissions for
asthma exacerbations compared to a time period prior
to implementation would be beneficial to evaluate
impact on patient care quality as well as financial savings
for family and institution.
• The team should look to disseminate the results of the
project within their institution as well as to a broader
audience through a manuscript or presentation at an
appropriate conference.
Merrill, K.C. (2015). Is this quality improvement or research?
American Nurse Today 10(4). https://www.
americannursetoday.com/quality-improvement-research/
(accessed 11 June 2019).
Silver, S.A., Harel, Z., McQuillan, R., et al. (2016). How
to begin a quality improvement project. Clinical
Journal of the American Society of Nephrology 11:
893–900.
Swanson, J.R. and Pearlman, S.A. (2017). Roadmap to a
successful quality improvement project. Journal of
Perinatology 37: 112–115.
Institute for Healthcare Improvement. Science of
improvement: how to improve. http://www.ihi.org/
resources/Pages/HowtoImprove/
ScienceofImprovementHowtoImprove.aspx
McQuillan, R.F., Silver, S.A., Harel, Z., et al. (2016). How to
measure and interpret quality improvement data. Clinical
Journal of the American Society of Nephrology 11: 908–914.
Purdue University Lean Six Sigma Online. Advances in
healthcare with Lean Six Sigma. https://www.purdue.edu/
leansixsigmaonline/blog/healthcare-advancement-with-
lean-six-sigma/ (accessed 11 June 2019).
White, K.M., Dudley-Brown, S., and Terhaar, M.F. (2016).
Translation of Evidence into Nursing and Health Care, 2nd
edn. New York: Springer Publishing Company.

33
Newborn Immediately Post Delivery
Heather Schniepp
University of Texas Health, San Antonio, TX, USA
A newborn infant is brought to the newborn nursery post
precipitate vaginal delivery 2 hours after mother’s arrival
to the hospital. Apgar scores were 6 at 1 minute and 8 at
5 minutes. There was no nuchal cord and no reports of res-
piratory distress or meconium.
History of Present Illness
The 17-year-old mother has received no medications during
delivery and has not been able to give an adequate health
history or pregnancy history. She had little prenatal care
and has not been seen in the obstetrics clinic since the
fourth month of her pregnancy where a note suggests a pos-
sible maternal diagnosis of lupus erythematosus. Mother
denied alcohol, drug, and tobacco use during pregnancy.
Current Status
The newborn’s initial vital signs are as follows: birthweight
3120 g, length 50 cm, head circumference 34.5 cm, heart
rate 138 bpm, blood pressure 72/34 mmHg, respiratory rate
52 breaths per minute, and temperature 37.1 C (98.8 F)
axillary.
Questions
Answer the following questions using the details provided.
1 After drying and warming this infant and ensuring sta-
bility, what diagnostic testing, in addition to normal
newborn laboratory evaluations and physical examina-
tion, is anticipated for this neonate on arrival in the
newborn nursery?
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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139
Rationale and Evidence-based
Practice Explanation
After drying and warming this infant and ensuring
stability, what diagnostic testing, in addition to
normal newborn laboratory evaluations and
physical examination, is anticipated for this
neonate on arrival in the newborn nursery?
A 12-lead EKG and echocardiogram are indicated as soon
as the neonate is stabilized. The EKG is required to evaluate
for heart block in infant of mother with possible lupus. The
infant will also need an echocardiogram to determine if
there are any structural heart defects. Neonates can also
be tested for antibodies associated with autoimmune syn-
dromes. Maternal laboratory testing can also be completed
for systemic lupus erythematosus (SLE), as some mothers
may be unaware that they have an autoimmune disease.
Of note, even though a mother may carry these autoantibo-
dies, only a small number of fetuses/neonates develop
heart block.
Because of lack of prenatal care and the mother being a
poor historian, the infant should have urine and meconium
toxicology screening to evaluate for drug use during preg-
nancy. There should be a low threshold to begin treatment
if you suspect neonatal infections. Close monitoring of the
infant’s vital signs including heart rate and temperature are
important as well.
Continuation of case: The results of the 12-lead electro-
cardiogram (EKG) are shown in Figure 33.1. Diagnostic
study results were as follows: urine and meconium toxicol-
ogy screens negative; echocardiogram showed normal
heart structure; and physical examination was within nor-
mal limits except capillary refill of 4 seconds to feet.
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
140 Cases in Pediatric Acute Care

Figure 33.1 12-Lead EKG.

2 What rhythm is noted on the infant’s EKG?

3 What treatment may be needed?
4 What anticipatory guidance should be provided to the
family?

What rhythm is noted on the infant’s EKG?

This EKG shows atrioventricular dissociation, probable
complete heart block. This is a 1-day-old infant with a post-
natal diagnosis of heart block.
Heart block is identified as first-, second-, or third-degree
heart block. First-degree heart block is defined by a PR
interval of greater than 0.2 seconds where a normal PR
interval is 0.12–0.2 seconds. Mobitz type I or Wenckebach
second-degree heart block occurs when the P wave
becomes blocked because of the prolonged PR interval.
Mobitz type II second-degree heart block occurs when there
is a sudden block of impulses from the atria to the ventricles
with no prolonged PR interval. Third-degree or complete
heart block occurs when there is no electrical coordination
between the atria and the ventricles.
Congenital atrioventricular block is very rare, occurring Figure 33.2 Chest radiograph with internal pacemaker in 6-day-
once in about 20 000 live births annually worldwide. The old neonate.

Figure 33.3 12-Lead EKG post pacemaker placement.
majority of cases are due to maternal transfer of autoantibo-
dies to the fetus causing an immune-mediated cardiac dis-
ease or neonatal lupus. Mothers may have antibodies due
to the following disorders: SLE, Sjögren syndrome, antipho-
spholipid syndrome, or other autoimmune syndrome. Other
causes can include structural heart defects, which cause dis-
turbance between the atria and ventricles, and, less com-
monly, maternal drug use or fetal viral infections.
What treatment may be needed?
Fetal echocardiography is currently the best diagnostic tool
to detect fetal heart block. If left untreated, the mortality
of fetal and neonatal heart block is between 14 and 34%.
Mortality increases in premature neonates, those with
fetal hydrops, and those with ventricular rates less than
55 bpm. Prenatal or transplacental options, including
maternal administration of dexamethasone or terbutaline,
are not used in all cases due to the side effects for mother
and fetus.
Postnatal cardiac pacing and pharmacologic therapy is
the mainstay of treatment in severe bradycardia and
hemodynamic insufficiency. The size of the neonate will
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Chapter 33 Newborn Immediately Post Delivery 141
determine if and when a permanent pacemaker system
can be placed. Temporary pacing can be completed
through transesophageal pacing or with temporary trans-
venous pacing wires placed surgically and connected to an
external pacemaker box. A permanent pacemaker is typi-
cally placed surgically in neonates and infants by a pedi-
atric cardiothoracic surgeon (Figure 33.2). Placement of
a pacemaker is in consultation with the electrophysiolo-
gist. Intravenous epinephrine, atropine, or dopamine
infusions may be used to improve chronotropy until a
pacemaker can be placed.
What anticipatory guidance should be provided to
the family?
This infant will require close follow-up with an electrophys-
iologist to monitor heart rhythm and pacemaker settings
and a pediatric rheumatologist for treatment and monitor-
ing of neonatal lupus.
The follow-up EKG (Figure 33.3) of the infant after pace-
maker placement demonstrated atrial-sensed ventricular-
paced rhythm. Pacemaker spikes are noted prior to each
QRS complex.

142 Cases in Pediatric Acute Care
Further Reading
Baruteau, A.-E., Pass, R.H., Thambo, J.-B., et al. (2016).
Congenital and childhood atrioventricular blocks:
pathophysiology and contemporary management. European
Journal of Pediatrics 175: 1235–1248. doi: 10.1007/s00431-
016-2748-0.
Brito-Zeron, P., Izmirly, P.M., Ramos-Casals, M., Buyon, J.P.,
and Khamashta, M.A. (2016). Autoimmune congenital heart
block: complex and unusual situations. Lupus 25: 116–128.
doi: 10.1177/0961203315624024.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Johnson, B. (2014). Overview of neonatal lupus. Journal of
Pediatric Health Care 28: 331–341. doi: 10.1016/j.
pedhc.2013.07.015.
Levesque, K., Morel, N., Maltret, A., et al. (2015). Description of
214 cases of autoimmune congenital heart block: results of
the French neonatal lupus syndrome. Autoimmunity
Reviews 14: 1154–1160. doi: 10.1016/j.autrev.2015.08.005

34
A 5-Year-Old with Poor Weight Gain and Dehydration
Kimberly Young-Conner
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 5-year-old female presents to the primary care office for a
routine well child appointment at 4 p.m. She is accompa-
nied by her maternal grandmother and grandfather who
are also her legal guardians. This is the first visit for the
child to see this pediatric provider who is not familiar with
the patient and family.
History of Present Illness
The patient is in kindergarten and was last seen in the
primary care office one year ago. She is scheduled today
for a routine health visit, but grandparents voice concern
over her slow weight gain. They feel that she just can’t gain
weight even though she “eats all the time.” Grandfather
states for a snack she might eat a frozen dinner and then
still eats a full cooked meal. Grandparents report a recent
diagnosis of strep throat, along with an asthma exacerba-
tion, which was treated in an urgent care center about
3 weeks ago. The child received a 10-day course of
amoxicillin, a 5-day course of prednisolone 2 mg/kg daily,
and has been using albuterol metered-dose inhaler (MDI)
two to three times per day up until this current week.
Past Medical History
The child was born by vaginal delivery to a mother who has
type 1 diabetes mellitus and received appropriate prenatal
care. The infant was large for gestational age, weighing 4.2
kg and did not initially pass her newborn hearing screen,
but did pass the second attempt. At age 8 months, the child
was admitted to the hospital for respiratory syncytial virus
bronchiolitis and has had several episodes of wheezing
since then, but none requiring hospitalization.
At age 2, this child had not met appropriate language
milestones and had another hearing evaluation, which
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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143
included an auditory brainstem response hearing test,
and was found to have bilateral sensorineural hearing loss.
She has had consistent follow-up with speech and language
services and now has a left cochlear implant.
The child has received all her required immunizations as
well as the influenza vaccine each year. Her height has been
consistently between the 25th and 40th percentiles, with
her weight between the 3rd and 6th percentiles. There is
no other medical history.
Past Surgical History
The child had a left cochlear implant at age 4, and is
followed by the local children’s hospital.
Family History
Mother of the child, who is not currently involved in her
care, has uncontrolled insulin-dependent diabetes. The
child has an older sibling aged 8 who also lives with mater-
nal grandparents and is reported to be healthy. Maternal
grandparents cannot provide information about the child’s
biological father or his family history. There is no known
family history of hearing concerns nor of juvenile diabetes.
Current Status
The child’s height and weight remain in the same percen-
tiles, with a weight gain of 1.4 kg in one year. Her heart rate
is 122 bpm and her blood pressure is 96/44 mmHg. The
child appears thin and very anxious, with dry lips and
dry mucous membranes. She has several enlarged cervical
lymph nodes on palpation of her neck and is noted to have a

144 Cases in Pediatric Acute Care
“sweet” odor to her breath. Other physical examination fea-
tures are within normal limits.
Questions
Answer the following questions using the details provided.
1 Despite the setting, the child is evaluated, what would
be the three top differential diagnoses?
2 What diagnostic testing should be considered?
Rationale and Evidence-based Practice
Explanation
Despite the setting, the child is evaluated. What
would be the three top differential diagnoses?
The three top differential diagnoses for this child include
failure to thrive, steroid-induced hyperglycemia, and type
1 or type 2 diabetes mellitus, the former being most likely
given the child’s age, history, and clinical presentation.
Since the child has not been weighed or measured in this
office in one year, it is difficult to know if she has sustained
weight loss or has historical slow weight gain, so failure to
thrive could be considered. Steroid-induced hyperglycemia
would not be likely in a healthy child 2 weeks after comple-
tion of dosing.
Diabetes mellitus typically presents with symptoms of
polyuria, polydipsia, and polyphagia with unexplained
weight loss. About 25% of patients will present with diabetic
ketoacidosis on initial diagnosis. Diabetes can be immune-
mediated and noted following a viral or bacterial illness. It
can also be genetic with risk of type 1 diabetes conferred by
two susceptible haplotypes, with a 5% lifetime risk for chil-
dren who have a first-degree relative with type 1 diabetes.
Children with cystic fibrosis have a higher incidence of dia-
betes mellitus, occurring in 20% of the patients in this
population.
What diagnostic testing should be considered?
Depending on what is available in the primary care office, it
is helpful to obtain at least a urinalysis and/or a fingerstick
blood glucose. The full laboratory work-up for a diagnosis
of diabetes mellitus would include:
•• fasting blood glucose;
basic metabolic panel to assess for dehydration, acidosis
and renal function;
• hemoglobin A1c, which indicates diabetes if higher
than 6.5%;
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• pancreatic islet autoantibodies and C-peptide levels,
which are useful in differentiating between type 1 and
type 2 diabetes mellitus;
• thyroid stimulating hormone, antithyroid peroxidase and
antithyroid antibodies;
• celiac antibody panel.
Continuation of case: Normal physical examination
results were reviewed with guardians. Based on the
growth chart and previous measurements, there was a
concern for poor weight gain along with increased appetite.
A glucometer was not available in the office, so a urinalysis
dipstick test was accomplished and this revealed a glucose
of over 500 mg/dL, no leukocytes or bacteria, and with a
trace of blood and ketones. The presence of glucosuria
and ketonuria along with physical findings and poor weight
gain or weight loss in a child do indicate the possibility of
diabetes, typically at this age type 1 or insulin-dependent
diabetes mellitus.
These results were shared with the child’s grandparents
along with the potential diagnosis which would be further
confirmed by blood testing and examination at the chil-
dren’s hospital. After consulting with pediatric endocrinol-
ogy, the child and her grandparents were referred to the
emergency department at the local children’s hospital, with
an explanation that she would most likely be admitted for
further evaluation, rehydration and education about the
disease.
3 As the primary care provider for this child, what are
important management skills to support the new diag-
nosis and continued management?
As the primary care provider for this child, what
are important management skills to support the
new diagnosis and continued management?
According to the American Diabetes Association, a diagno-
sis of diabetes must meet criteria that include symptoms of
polyuria, polydipsia or weight loss and a random blood glu-
cose of 200 mg/dL or greater. This patient’s final diagnosis
was type 1 diabetes mellitus. The child was admitted to the
hospital and started on a subcutaneous insulin regimen.
Over the course of the hospitalization, the family received
survival skill education, including carbohydrate counting
and treatment for hypoglycemia. They also received
instructions on insulin administration and monitoring of
ketones.
Follow-up with the primary care provider included edu-
cation on the honeymoon period, monitoring for ketones,
and sick day rules. The primary care provider should be
in contact with the endocrinology team for awareness of
laboratory results, insulin dosing and planned follow-up,
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Chapter 34 A 5-Year-Old with Poor Weight Gain and Dehydration 145

which would be every 3 months in the endocrinology clinic. learn carbohydrate counting. The school nurse was also
Discussion should focus on the need for healthy nutrition, contacted in order to be included in the child’s care and
proper hydration and exercise along with using labels to monitoring of blood sugars while in school.

Further Reading
Glassford, M.A. (2017). Recognizing type 1 diabetes mellitus complications. Best Practice and Research in Clinical
in children and adolescents. The Nurse Practitioner 42(6): Obstetrics and Gynecology 29(2): 256–269. doi: 10.1016/j.
16–22. doi: 10.1097/01.NPR.0000516121.77126.61. bpobgyn.2014.08.004.
Mitanchez, D., Yzydorczyk, C., Siddeek, B., et al. (2015). The
offspring of the diabetic mother: short- and long-term
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147

35

Toddler with Complex Chronic Condition

Jennifer Wright
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA

A 13-month-old male with a medical history significant for
preterm birth, hypoxic ischemic encephalopathy, cerebral
palsy, bronchopulmonary dysplasia, tracheostomy without
ventilator dependence, gastroesophageal reflux disease
(GERD) status post Nissen fundoplication, focal epilepsy
with tonic and myoclonic seizures, and infantile spasms.
He has presented to the hospital with increased seizures
and is admitted to the pediatric intensive care unit
(PICU) with respiratory failure in the setting of status
epilepticus. This is his third PICU admission this month.
Past Medical History
Preterm birth at 30 weeks’ gestation, hypoxic ischemic
encephalopathy, cerebral palsy, bronchopulmonary
dysplasia, tracheostomy without ventilator dependence,
GERD, focal epilepsy with tonic and myoclonic seizures,
and infantile spasms.
Past Surgical History

Circumcision and Nissen fundoplication with g-tube for

History of Present Illness
He was discharged from general care the evening prior to
admission. He had been hospitalized the previous 2 weeks
with status epilepticus and septic shock in the setting of
pneumonia. During that admission, clobazam was added
to his antiepileptic regimen, and his levetiracetam and phe-
nobarbital doses were increased. Vigabatrin was kept at the
same dose. Ketogenic diet ratio was increased to 4.5 from 4,
with a β-hydroxybutyrate goal of 4–6.5.
When the toddler arrived home after being discharged,
his mother noticed that he was breathing deeper and harder
without retractions or tachypnea. He was also having more
frequent nasal and tracheal secretions. The morning of
admission, he began having a seizure with shaking of all
extremities, consistent with prior semiology. After 10 min-
utes, he was given clonazepam rescue, but the seizure con-
tinued. After 20 minutes, he was given rectal diazepam, but
the seizure persisted so emergency medical services was
contacted. He was brought to the emergency department
and continued to seize. His glucose was within normal lim-
its at the time of transport.
GERD/feeding disorder at 2 months of age. A tracheostomy
for upper airway obstruction was placed at 5 months of
age. Bilateral submandibular gland excision for excess
secretions at 11 months of age.
Family History
Non-contributory.
Social History
Lives at home with mother, father, and 3-year-old brother.
Mother has a 15-year-old son who is living with his father.
She also has three daughters who live with their maternal
grandmother, who has custody. She states she has good
support from her mother. Has nursing care five nights
and three days per week due to complex care with
tracheostomy.

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

148 Cases in Pediatric Acute Care
Current Status
Vital signs on arrival notable for a temperature of 36.8 C
(98.2 F), heart rate 128 bpm, respiratory rate 29 breaths
per minute, blood pressure 82/58 mmHg, and oxygen
saturations 98% on humidified trach collar. He continued
to seize despite receiving lorazepam 1 mg, phenobarbital
20 mg/kg, and levetiracetam 20 mg/kg. His seizure
stopped after receiving fosphenytoin 20 mg/kg. Mechani-
cal ventilation was initiated through his tracheostomy.
He was given a fluid bolus and started on maintenance
intravenous fluids. A venous blood gas analysis and com-
plete blood count were largely unremarkable, with a white
cell count of 10 200/mm3. Comprehensive metabolic panel
was notable for alkaline phosphatase of 362 U/L (normal
range 44–147 U/L), procalcitonin 0.08 ng/L (normal range
0.1–0.49 ng/L), and electrolytes within normal limits.
A respiratory culture was sent from tracheal sections.
His chest radiograph was notable for interval increase in
perihilar and retrocardiac airspace opacities, most consist-
ent with atelectasis or edema. Pneumonia could not be
excluded so intravenous vancomycin and piperacillin/
tazobactam were initiated. He was admitted to the PICU
for further management.
Currently, the PICU and neurology teams are working on
antiepileptic drug adjustments and readdressing home res-
piratory support needs as he is requiring increased FiO2 and
flow during this stay. It is possible that he is chronically
under-ventilated, leading to progressive hypoxia that trig-
gers seizures.
Questions
Answer the following questions using the details provided.
1 As you learn more about this patient, you become con-
cerned about the overall trajectory of his care and
increasing medical complexity. In order to more clearly
address his treatment plan going forward, what addi-
tional information would be important to elicit from
his mother?
2 You learn that while his mother has interest in continu-
ing to pursue all treatments or interventions that may
help him, she has growing worries about his decline
in function and overall quality of life. How might you
further assess alignment of parental and treatment
team goals of care?
Continuation of case: He continues to have periods
of increased seizure activity despite optimization of
anti-seizure regimen. As the anti-seizure regimen has
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been adjusted, his awareness and interactivity
decline and he has increased dependence on ventilation
support. Attempts to reduce sedation have resulted in
increased seizures, neuro-agitation, and respiratory
decompensation. His mother has begun confiding in
his nurses that she is worried that he is suffering and
that it feels that we are no longer doing things “for”
him but rather “to” him.
3 It becomes clear that it is unlikely he will ever success-
fully wean from ventilator support and continuing the
ventilator long term is viewed as extraordinary mea-
sures and outside of his mother’s goals of care. In
addition, continued attempts to adjust medications
are leading to perceived and visible suffering. As
you work to transition the child to a comfort-focused
plan of care, how will you discuss this with his
mother?
4 What are the potential symptoms at end of life and how
will you prepare both the care team and his family?
Rationale and Evidence-based Practice
Explanation
In order to more clearly address his treatment plan
going forward, what additional information would
be important to elicit from his mother?
As his medical journey continues to unfold, it will be
increasingly important for those caring for him to begin
to elicit from his mother, who is his surrogate decision-
maker, her understanding of his current status along
with the trajectory of his illness. In addition, it will be
helpful to know what she is hoping for and what her big-
gest worries are for him. Asking open-ended questions
about understanding, hopes, and worries can assist the
provider in gaining insight into a parent’s perspective
that will create opportunities to clarify medical status,
share mutual concerns, and address goals of care going
forward. When asking about hopes and worries, it is
important to elicit parental perspectives on both the
immediate situation and expectations for the future.
Offering validation, where appropriate, builds trust
and alignment. For example, his mother may state “I
hope that he will be able to come off the ventilator
and not have any more seizures.” A provider response
that offers validation and moves to seeking alignment
in goals may be something like “We hope for that too,
but if that may not be possible, what else would be
important for him?”

How might you further assess alignment of
parental and treatment team goals of care?
Gaining insight into how this child’s daily life is interpreted
by his mother with respect to enjoyment and suffering,
along with her focus as his parent, can continue to elucidate
what goals are most consistent with her values as his sur-
rogate. Questions that may help further understanding
could include “Tell me what a good and bad day looks like
for him” or “How have things changed for him in the past
6 months” and “What is most important to you at this time
as his mother?” Being a “good parent” is often critical to
parental interpretation of current circumstances and can
change as the child’s complex chronic condition changes.
As you work to transition the child to a comfort-
focused plan of care, how will you discuss this with
his mother?
When you have reached a point in delivering medical care
where the perceived suffering outweighs the real or poten-
tial benefits of continuing aggressive intervention and the
likelihood of regaining acceptable neurologic and func-
tional baseline is low, it is important to compassionately
and directly address the status with surrogate decision-
makers. Often, as in this case, parents and medical teams
are separately wondering if “we are doing too much” long
before we directly address this together. For most parents,
using a model of shared decision-making allows them to
maintain their agency as “good parents” while offering
assurance and lessening of the burden of difficult decisions.
In this model, the medical provider identifies alignment of
medical and parental goals, offering recommendations for
next steps rather than asking a parent what they would like
to do. Statements such as “Given his current status and
what you have told me is most important for him, this is
what we should do next” can be utilized to move forward
to comfort focus without laying the full burden of this deci-
sion on the parent. When offering this form of communica-
tion of recommendation, it is crucial to allow the parent
time to consider what you have proposed and ask questions
or clarify to be sure you have accurately interpreted their
perspective. When moving forward with plans to de-
escalate medical interventions, the phrase “withdrawal of
Further Reading
de Vos, M.A., Bos, A.P., Plotz, F.B., et al. (2015). Talking with
parents about end-of-life decisions for their children.
Pediatrics 135(2): e465–e476.
Edmonds, B.T., Torke, A.M., Helft, P., and Wocial, L.D. (2015).
Doctor, what would you do? An ANSWER for patients
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Chapter 35 Toddler with Complex Chronic Condition 149
care” can be received negatively as though the child is no
longer going to receive medical attention. Clarifying with
parents that we will continue to aggressively care for their
child, but with the primary focus now on attending to their
comfort can ease parental distress and maintain a trusting
relationship.
What are the potential symptoms at end of life and
how will you prepare both the care team and his
family?
The most common symptoms at end of life are pain, secre-
tions, dyspnea, agitation, sometimes seizures, and nausea/
vomiting. It is important to anticipate the most likely
symptoms given a particular clinical condition and to be
prepared to respond effectively and efficiently. In circum-
stances similar to this child’s, when there is time for
thoughtful planning and transition in care, careful consid-
eration for the medical plan as well as logistical plan is key.
Time should be spent with mother offering anticipatory
guidance, opportunities for memory making, and bereave-
ment preparation. Taking care of required post-mortem
items such as discussion of autopsy and identification of
a funeral home in advance of the death can be helpful to
some families. Time should be spent with the medical team
in review of the plan for de-escalation and preparation for
possible symptoms. When planning for ventilator weaning
with compassionate extubation or removal of ventilator, it
is important to have a stepwise approach and acknowledge
that while death is expected relatively soon after cessation
of ventilation, this is not under our control and we will con-
tinue to follow the child’s lead, responding to symptoms of
distress or discomfort. Consideration for timing of cessation
of fluids or feeding should be taken to lessen the burden of
secretions and gastrointestinal distress as end of life
approaches. Allowing the family time after death to say
their goodbyes and prepare for separation is important.
For some families, having a familiar team member such
as the bedside nurse stay with, and even hold the child,
when the family leaves can lessen their sense of abandon-
ment. Asking parental preferences and continuing compas-
sionate care at each step along the way can mean the
difference in supporting a “good” death.
requesting advice about value-laden decisions. Pediatrics
136(4): 573–583.
Field, M. and Behrman, R. (ed.) (2003). When Children Die:
Improving Palliative and End-of-Life Care for Children and
their Families. Washington, DC: National Academies Press.

150 Cases in Pediatric Acute Care
Michelson, K.N., Patel, R., Haber-Baker, N., Emanuel, L., and
Frader, J. (2013). End-of-life decisions in the pediatric
intensive care unit: roles professionals play. Pediatric
Critical Care Medicine 14(1): e34–e44.
October, T.W., Fisher, K.R., and Feudtner, C. (2014). The
parent perspective: “Being a good parent” when making
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critical decisions in the PICU. Pediatric Critical Care
Medicine 15(4): 291–298.
University of Michigan Pediatric Palliative Care Team (2013).
Algorithms for end-of-life care in anticipated pediatric
deaths. https://open.umich.edu/sites/default/files/
downloads/code_cards_december_2013.pdf

36
Pre-adolescent with Knee Pain
Katelyn Hamann and Shannon Konieczki
Children’s Hospital of Michigan, Detroit, MI, USA
A 12-year-old African American female presents with new-
onset left knee pain.
History of Present Illness
She began complaining of left knee pain approximately
1 month ago without any prior injury, trauma or specific
event preceding the onset. Her pain is localized to the left
distal knee, described as constant aching, and causes a limp
with ambulation. Her pain was minimally relieved with
ibuprofen. She denies fever, numbness, paresthesias, pallor,
weight loss, or malaise.
Past Medical History
She was born full term with no complications and dis-
charged home with her mother. Her immunizations are
up to date for age including annual influenza vaccines.
She has never been hospitalized. She has no surgical history
and no known medication allergies.
Family History
Her maternal grandfather died of lung cancer; no other sig-
nificant family history.
Social History
She lives at home with her mother, aunt, and grandmother.
Attends 7th grade. She is not involved in any sports.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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151
Current Status
Her initial physical examination was within normal limits.
Her initial diagnostic evaluation included a radiograph of
the affected limb (Figures 36.1 and 36.2). A complete
blood count and complete metabolic panel (Table 36.1)
were also completed and the results are shown in
Tables 36.1 and 36.2.
The radiographs show a sclerotic lesion with periosteal
reaction in the left proximal tibia.
Questions
Answer the following questions using the details provided.
1 What are the differential diagnoses for this patient?
2 What diagnostic evaluation would you order for this
patient?
3 What is your next step in treating this patient?
Rationale and Evidence-based Practice
Explanation
What are the differential diagnoses for this
patient?
Differential diagnoses for this patient based on the initial
presentation would include septic arthritis, osteomyelitis,
osteosarcoma, Ewing sarcoma, fracture, sprain/strain,
Osgood–Schlatter disease, and osteochondritis dissecans.
Based on the imaging provided, the differential should also
include myositis ossificans, bone fibrosarcoma, and giant
cell bone tumor. Clues in the patient history can be used
to determine the most likely differential diagnosis. Absence
of a history of trauma makes fracture, sprain and strain less
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152 Cases in Pediatric Acute Care

Table 36.1 Basic metabolic profile.

Sodium 139 mEq/L

Potassium 4.3 mEq/L
Chloride 102 mEql/L
Carbon dioxide 27 mEq/L
Blood urea nitrogen 12 mg/dL
Creatinine 0.5 mg/dL
Glucose 101 mg/dL
Phosphorus 4.1 mg/dL
Anion gap 10 mEq/L
Alanine aminotransferase 23 U/L
Aspartate aminotransferase 19 U/L
Alkaline phosphatase 333 U/L
Lactate dehydrogenase 377 U/L
Uric acid 2.4 mg/dL

Table 36.2 Complete blood count.

White blood cell count 11 700/mm3

Hemoglobin 11.1 g/dL
Hematocrit 33.8%
Figure 36.1 Lateral radiograph of knee. Platelet count 365 000/mm3
Mean corpuscular volume (MCV) 69.2 fL
Neutrophils 73%
Eosinophils 1%
Monocytes 4%
Lymphocytes 3%
Bands 0%

sometimes fever which are not present in this child.

Absence of a prominent/edematous tibial tubercle makes
Osgood–Schlatter disease less likely. With these considera-
tions and the radiographic findings, malignancy as the eti-
ology of the pain moves to the top of the differential
diagnosis list.

What diagnostic evaluation would you order for

Figure 36.2 Anteroposterior radiograph of knee.
likely. Absence of fever moves osteomyelitis further down
the differential diagnosis list. Septic arthritis is typically
associated with extreme discomfort and is often accompa-
nied by an erythematous and edematous joint and
this patient?
A plain radiograph is the first step in diagnosis and was
completed on the child’s presentation. Radiographic dem-
onstration of sclerotic lesions indicates a thickening of
the bone that is typically associated with a slow-growing
process rather than acute changes. Sclerotic bone lesions
can be benign when asymptomatic, and are often found
as an incidental finding. Malignant bone disease is often

associated with pain that increases over time. Osteosar-
coma lesions can appear as lytic and/or sclerotic with an
internal or external osteoid matrix, which is associated with
soft tissue involvement. Periosteal reaction often occurs
with the classic “sunburst” pattern or with Codman’s trian-
gle. Codman’s triangle presents due to a break in the cortex
of the bone, causing a triangle of reactive bone to appear.
Skip lesions can also occur in patients with osteosarcoma;
these are osseous metastases that are non-contiguous. Fur-
ther imaging is required, based on the radiograph results. It
would be best to obtain magnetic resonance imaging (MRI)
of the lower extremity. MRI and computed tomography
(CT) are tools utilized to assess the extent of the tumor into
vessels, nerves and soft tissues, as well as identify the extent
of metastases.
MRI results include subperiosteal and soft tissue mass
located in the lateral tibial metaphysis with extension into
the lateral tibial epiphysis, posterior leg musculature and
neurovascular bundle (Figure 36.3).
According to the National Institutes of Health’s Sur-
veillance, Epidemiology and End Results Program for
the National Cancer Institute (2019), there will be an
estimated 3500 new cases of osteosarcoma diagnosed
in the United States in 2019. Although rare, osteosar-
coma is the most common bone tumor in childhood
and adolescence. It typically arises in the metaphysis
Figure 36.3 Initial MRI.
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Chapter 36 Pre-adolescent with Knee Pain 153
of long bones, with the most common locations being
the distal femur, proximal tibia, and humerus, consecu-
tively. Of children diagnosed with osteosarcoma, 20%
have metastatic disease, most commonly to the lung.
Age of occurrence is typically associated with puberty,
with highest incidence for males 15–19 years of age
and females 10–14 years of age. Certain genetic syn-
dromes are associated with osteosarcoma, including
bilateral retinoblastoma, Li–Fraumeni syndrome, Bloom
syndrome, Paget disease and Thomson syndrome. Chil-
dren with hereditary bilateral retinoblastoma are 500
times more likely to develop osteosarcoma than a child
without retinoblastoma. Children typically present with
pain as their chief complaint, which may or may not be
associated with a trauma in the recent months. Although
osteosarcoma is not caused by trauma, it is often discov-
ered following a trauma. Swelling does not immediately
follow the trauma, but often occurs over time. Pathologic
fractures are often found in conjunction with the solid
tumor. Associated laboratory findings include elevated
alkaline phosphatase, elevated lactate dehydrogenase,
mild anemia, or elevated erythrocyte sedimentation rate
(ESR). This child’s laboratory findings include elevations
in alkaline phosphatase and lactate dehydrogenase, and
mild anemia which support the diagnosis of osteosar-
coma. An ESR has not yet been obtained.
What is your next step in treating this patient?
CT of chest, abdomen and pelvis is needed to evaluate for
metastasis to other organs. Although CT exposes the
patient to higher doses of radiation, it is imperative to
obtain these scans so that the disease can be staged appro-
priately based on the presence of metastasis. CT showed left
lower lobe heterogeneous mass with ground glass opacities
scattered to bilateral lung fields, concerning for osteosar-
coma metastases. Tracheobronchial tree was patent. There
was no pleural effusion or pneumothorax (Figure 36.4).
Biopsy of the lesion by an orthopedic surgeon should be
performed to confirm the definitive diagnosis. Osteosar-
coma is histologically classified by the World Health Organ-
ization and includes parosteal (juxtacortical), low-grade
central (intramedullary), periosteal (juxtacortical chondro-
blastic), telangiectatic, small cell, high-grade surface, and
conventional (chondroblastic, fibroblastic or osteoblastic).
Parosteal and low-grade central are considered low-grade
subtypes. Periosteal is considered intermediate subtype.
Telangiectatic, small cell, high-grade surface, and conven-
tional are considered high grade. Conventional osteosar-
coma is the most common subtype.
Osteosarcoma treatment includes surgical resection of
the primary tumor. This may include an amputation or
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154 Cases in Pediatric Acute Care

Figure 36.4 CT of chest and abdomen.

a limb salvage, which would be determined by the Case Resolution

orthopedic surgeon. Neoadjuvant chemotherapy is the
standard of care, typically consisting of methotrexate,
doxorubicin, and cisplatin. Radiation is not utilized
as treatment because osteosarcoma is resistant to
radiation.
After biopsy confirming the diagnosis of osteosarcoma, she
underwent ampulation of the left lower extremity, followed
by chemotherapy with methotrexate, doxorubicin, and
cisplatin.

Reference
National Institutes of Health (2019). Cancer Stat Facts: Bone
and Joint Cancer. https://seer.cancer.gov/statfacts/html/
bones.html

Further reading
Dyson, K.A., Stover, B.D., Grippin, A., et al. (2019). Emerging
trends in immunotherapy for pediatric sarcomas. Journal of
Hematology Oncology 12: 1–10. doi: 10.1186/s13045-019-
0756-z.
Rana, K.A., Meyer, J., Ibrahim, S., Ralls, M., and Kent, P.M.
(2013). The role of imaging of malignant bone tumors in
children and young adults. Current Problems in Cancer 37:
181–191. doi: 10.1016/j.currproblcancer.2013.07.005.
Whelan, J.S. and Davis, L.E. (2018). Osteosarcoma,
chondrosarcoma, and chordoma. Journal of Clinical
Oncology 36(2): 188–193. doi: 10.1200/JCO.2017.75.1743.

37
Infant with Increased Fussiness and Decreased Oral Intake
Stephanie Haughey
Helen DeVos Children’s Hospital, Grand Rapids, MI, USA
An 11-month-old boy with no significant prior medical his-
tory presents to the emergency department (ED) with fever,
irritability, fatigue and vomiting.
History of Present Illness
This baby is a previously healthy 11-month-old boy present-
ing to the ED with a 4-hour history of irritability, fatigue,
poor oral intake, vomiting, decreased urine output, and
high fever. He was deteriorating quickly in the car to
the ED.
Past Medical History
He has had two episodes of otitis media, the last one a
month ago. He had a recent upper respiratory infection.
He is up to date on vaccinations. There are no developmen-
tal concerns. He eats formula and baby foods.
Past Surgical History
Circumcision as newborn without complication.
Family History
Healthy 7-year-old sister, father with type 2 diabetes,
mother with kidney stones.
Current Status
Infant is lethargic, difficult to arouse, and with pallor and
mottling. His pupils are equal and reactive, and the anterior
fontanelle is full. He is positive for opisthotonos, feels hot
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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155
centrally but cold peripherally with capillary refill of 5–6
seconds, and has dry mucous membranes. He
is tachypneic with abdominal muscle use but lungs
are clear and no retractions are present. Tachycardia
is present but normal S1 and S2 and no murmur. Abdomen
is soft and non-distended, with no hepatomegaly. Moans
with IV poke but otherwise non-interactive. Petechiae
noted across chest and abdomen. The infant weighs 10 kg.
Initial vital signs are as follows: temperature 40.1 C
(104.2 F) rectally, heart rate 189 bpm, blood pressure
72/34 mmHg, respiratory rate 45 breaths per minute, and
SpO2 88% on room air. Results for complete blood count
(CBC) and complete metabolic panel are shown in
Tables 37.1 and 37.2. Results for inflammatory markers
as follows: C-reactive protein 121.6 mmol/L, procalcitonin
>100.0 ng/mL.
Questions
Answer the following questions using the details provided.
1 What are the differential diagnoses for this child?
2 What is the most likely diagnosis for this child?
3 What are the immediate interventions in the emer-
gency department?
4 Are there any other studies you would obtain?
5 What are the next steps to help stabilize this patient?
Rationale and Evidence-based Practice
Explanation
What are the differential diagnoses for this child?
Differential diagnoses for this child should include causes of
septic shock, meningismus, and petechiae. This would
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156 Cases in Pediatric Acute Care

Table 37.1 Complete blood count. meningococcemia with meningitis and septic shock.
Meningococcemia is defined as bloodstream infection with
White blood cell count 23 080/mm3
the highly virulent Gram-negative bacterium Neisseria
Hemoglobin 7.6 g/dL meningitidis. It usually causes many of the signs and symp-
Hematocrit 22.9% toms seen with other types of bacteremia and bacterial
Platelet count 19 000/mm3 meningitis such as fever, nausea, vomiting, headache, neck
Neutrophils 51% stiffness, cold hands and feet, pallor and/or mottling,
Lymphocytes 2% decreased concentration, and myalgias. Its differentiating
Monocytes 1%
features include the severity of myalgias (particularly leg
pain), the sudden onset and rapid progression, a non-
Eosinophils 0%
specific rash rapidly progressing to a hemorrhagic rash,
Bands 37%
and toxin-induced profound capillary leak.
Meningococcemia caused by Neisseria meningitidis can
Table 37.2 Complete metabolic panel. cause fulminant disease or just remain harbored in the
nasal cavity. The difference in illness is related to the fact
Sodium 139 mEq/L that meningococcemia is widespread vascular injury char-
Potassium 4.1 mEq/L acterized by endothelial necrosis, intraluminal thrombosis,
Chloride 100 mEq/L and perivascular hemorrhage. Endotoxin, cytokines, and
Carbon dioxide 12 mEq/L
free radicals damage the vascular endothelium, producing
platelet deposition and vasculitis. Cytokine response will
Blood urea nitrogen 30 mg/dL
result in severe hypotension, reduced cardiac output, and
Creatinine 1.8 mg/dL
increased endothelial response. Intravascular infection
Glucose 56 mg/dL and intracranial bacterial growth and inflammation reflect
Calcium 8.5 mg/dL the pathology of the disease. Pathogens bind to the endo-
Albumin 3.5 mg/dL thelial cells, and microcolonies are formed on the apical
Total protein 6.0 g/dL portion of the endothelial base. These bacteria invade the
Aspartate aminotransferase 85 U/L subarachnoid space with resultant meningitis with multi-
Alanine aminotransferase 56 U/L ple organ failure, shock, and the possibility of death.
Patients with meningococcemia develop thrombosis and
Alkaline phosphatase 145 U/L
hemorrhage in the skin, which is often apparent on admis-
Total bilirubin 1.1 mg/dL
sion to the ED. Thrombosis of the glomerular capillaries
Magnesium 1.6 mEq/L may cause renal cortical necrosis. Meningococcemia is a med-
Phosphorus 4.0 mg/dL ical emergency requiring prompt recognition, therapy, and
hospitalization. Illness can range from transient fever and
bacteremia to fulminant disease with multisystem organ fail-
include Gram-negative bacteria such as Neisseria meningiti-
ure and death occurring within hours of the onset of illness.
dis, Haemophilus influenzae, Rickettsia species (which cause
Rocky Mountain spotted fever or typhus), and Ehrlichia
(which causes ehrlichiosis), and Gram-positive bacteria What are the immediate interventions in the
such as Staphylococcus aureus, Streptococcus pneumoniae, emergency department?
and group A Streptococcus. Other causes for petechiae
Address airway and breathing by placing the child on high-
and/or purpura, such as Henoch–Schönlein purpura,
flow nasal cannula with low threshold for initiating non-
drug-induced vasculitis, scurvy, immune thrombocytope-
invasive positive pressure support or intubation and
nia, and acute leukemia, should be considered, but are less
mechanical ventilation. Circulation is supported by giving
likely given the presentation of shock with fever. Hemolytic
fluid boluses 20 mL/kg.
uremic syndrome does not explain all the symptoms, but
Additional interventions include correction of hypogly-
would explain the anemia, thrombocytopenia, and kidney
cemia with dextrose bolus and management of other abnor-
injury and can rarely present with purpura.
mal laboratory results. A blood culture followed by
immediate administration of antibiotics is necessary to
What is the most likely diagnosis for this child?
treat potential pathogens. The antibiotic of choice for sus-
Given the sudden onset, rapid progression, and severity of pected meningococcemia is a third-generation cephalospo-
illness, the most likely diagnosis for this child is rin such as ceftriaxone or cefotaxime. Vancomycin should

also be administered to target cephalosporin-resistant
Gram-positive organisms. Antibiotics should be dosed for
meningitis and adjusted for renal dysfunction as indicated.
Antibiotic administration should not be delayed and
intraosseous or intramusclar routes should be used if intra-
venous access cannot be obtained swiftly. Rectal acetamin-
ophen can be administered to help reduce the fever and
therefore metabolic demand.
Are there any other studies you would obtain?
Given the hemorrhagic rash, full fontanelle, and altered
level of consciousness, a head computed tomography
(CT) scan should be done to assess for intracranial bleeding
and cerebral edema.
What are the next steps to help stabilize this
patient?
This patient is rapidly deteriorating and needs a central
venous line to administer vasopressors. The safest and most
appropriate approach is to intubate the patient and place
him on a mechanical ventilator. Not only will this allow
you to sedate him for the central line placement, but it will
reduce the metabolic demand due to breathing.
Continuation of case: With admission to the pediatric
intensive care unit (PICU) the infant now has subcostal
retractions, nasal flaring, and crackles on auscultation.
Capillary refill remains prolonged. Vital signs are currently
temperature 39.1 C (102.4 F) rectally, heart rate 175 bpm,
blood pressure 68/31 mmHg, respiratory rate 36 breaths per
minute, and SpO2 88% on 15 L/min high-flow nasal can-
nula with 100% oxygen. The patient has received ceftriax-
one and three boluses of normal saline 20 mL/kg;
dextrose 5% in 0.9% normal saline (40 mL/hour) and van-
comycin are currently infusing.
6 How would you interpret the chest radiograph and
how might this change your ventilation strategies?
7 Would a lumbar puncture be indicated and
appropriate?
8 What vasopressor would you choose and why?
9 How would you define his shock at this point?
10 What other studies could be obtained that might help
manage the patient’s shock?
11 How do you interpret this blood gas analysis and what
ventilator changes would you make?
12 What else can you do to improve his blood pressure?
13 What medication can you add to improve perfusion
once you have control of his blood pressure?
14 What explains his coagulopathy and how will you
treat it?
15 What is the prognosis for this child?
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Chapter 37 Infant with Increased Fussiness and Decreased Oral Intake 157
16 What other potential complications should you
anticipate?
17 Does anything need to be done for close contacts of
this patient?
How would you interpret the chest radiograph and
how might this change your ventilation
strategies?
The chest radiograph findings of diffuse bilateral infiltrates
together with his acute hypoxia are consistent with acute
respiratory distress syndrome (ARDS), a common manifes-
tation of sepsis that is caused by alveolar capillary leak.
Because the risk of further lung injury is high, a strategy
involving a higher ventilation rate and lower tidal volume
with tolerance of relative hypoxia and hypercapnia will
likely be warranted.
Would a lumbar puncture be indicated and
appropriate?
Although a lumbar puncture would be indicated given a
high suspicion for meningitis, this child is too unstable to
tolerate both the positioning and sedation for a lumbar
puncture. His petechiae are also a clue that he could be
at risk for spinal epidural hematoma and a positive blood
culture can also help to guide treatment. If available,
PCR assays can detect meningococcal DNA in cerebrospi-
nal fluid, plasma, and serum and results are less affected
by antibiotics. The main priorities for this child are to start
antibiotics, administer fluids, and transfer him to the PICU.
What vasopressor would you choose and why?
This child is in cold shock (cold extremities, delayed capil-
lary refill) so the most appropriate medication to start with
would likely be epinephrine, which provides inotropy,
chronotropy and, at larger doses, vasoconstriction. Norep-
inephrine would be more appropriate for a child with warm
shock (warm extremities, brisk capillary refill).
How would you define his shock at this point?
He has fluid-refractory and catecholamine-resistant shock.
What other studies could be obtained that might
help manage the patient’s shock?
A repeat blood gas analysis with lactate and electrolytes
including ionized calcium would help determine ventila-
tor changes, further fluid resuscitation, and whether
electrolyte replacements such as calcium, potassium or
magnesium are needed to support cardiac function.

158 Cases in Pediatric Acute Care
Myocardial suppression should be suspected with
meningococcemia, and could be evaluated with an
echocardiogram. A random cortisol level would screen
for adrenal insufficiency as is common with meningo-
coccemia due to adrenal infarction. A repeat CBC (for
platelet and hemoglobin levels) and set of coagulation
parameters including fibrinogen, prothrombin time
(PT), and partial thromboplastin time (PTT) would help
guide blood product replacement in the setting of his
purpura fulminans with active bleeding.
Continuation of case: Petechial rash on his abdomen and
chest has evolved into purpuric lesions extending down his
legs. Blood-tinged gastric drainage is present in nasogastric
tube. Vital signs include temperature 38.6 C (101.5 F) rec-
tally, heart rate 170 bpm, blood pressure 71/35 mmHg, res-
piratory rate 24 breaths per minute, and SpO2 90% on 80%
FiO2 via the ventilator. The child has been intubated for
approximately 1 hour with central venous and arterial lines
placed.
His chest radiograph is shown in Figure 37.1. His
ventilator settings on pressure-regulated volume control
with spontaneous intermittent mandatory ventilation
(PRVC-SIMV) include a tidal volume of 80 mL, rate of
24 breaths per minute, positive end-expiratory pressure
(PEEP) of 6 cmH2O, and pressure support of 10 cmH2O.
His peak inspiratory pressure is 33 cmH2O. He is receiving
an epinephrine infusion 0.14 μg/kg per min to support his
blood pressure. Norepinephrine is being sent from the
pharmacy for additional augmentation of blood pressure.
An arterial line has been placed in his right femoral artery
after failed attempts at both radial arteries, and a nurse
technician is still maintaining pressure on his left wrist
because it has not stopped bleeding. Bruising is noted
where his blood pressure cuff has been.
Figure 37.1 Chest radiograph.
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Table 37.3 Arterial blood gas analysis 1 hour after intubation.
pH 7.19
PaCO2 59 mmHg
PaO2 68 mmHg
Bicarbonate 20 mEq/L
Base excess –6.3
Lactate 4.2 mmol/L
Table 37.4 Additional laboratory studies.
Cortisol 1.2 μg/dL
Fibrinogen 175 mg/dL
Prothrombin time (PT) 21.4 s
INR 2.3
Partial thromboplastin time (PTT) 39 s
Results of the blood gas analysis, cortisol level, and coag-
ulation panel are shown in Tables 37.3 and 37.4.
How do you interpret this blood gas analysis and
what ventilator changes would you make?
He has a mixed respiratory and metabolic acidosis. Now
that he is sedated and muscle relaxed, he is unable to com-
pensate for his metabolic acidosis by increasing his own res-
piratory rate. Administration of sodium bicarbonate is not
indicated except in cases of severe acidosis. The metabolic
acidosis will continue to improve with fluid resuscitation
and lactate clearance. Based on his chest radiograph, he
is developing ARDS, and his peak inspiratory pressures
are high. A lower tidal volume balanced with a faster rate
for ventilation would be targeted. A pressure control (PC)
mode of ventilation will allow tighter control of his peak
pressures to limit barotrauma. He is also hypoxic which
may respond to increases in PEEP. In ARDS, PEEP can
improve oxygenation by maintaining lung volume during
exhalation. For example, you could change his ventilator
settings to PC-SIMV rate of 30 breaths per minute, peak
inspiratory pressure 30 cmH2O, PEEP 9 cmH2O, and pres-
sure support 10 cmH2O and repeat a blood gas in 30–60
minutes.
What else can you do to improve his blood
pressure?
Based on the cortisol level, hydrocortisone at stress dosing
is initiated. This therapy may be initiated prior to receiving
the laboratory confirmation due to the severity of illness.
Transfusion of PRBCs could improve his blood pressure

as well as improve oxygen delivery to the tissues. Replete
calcium, magnesium, and potassium as indicated to sup-
port cardiac function.
What medication can you add to improve
perfusion once you have control of his blood
pressure?
Once blood pressure is better controlled, milrinone can be
added to help improve cardiac function and improve
perfusion.
What explains his coagulopathy and how will you
treat it?
Meningococcemia is known for causing disseminated intra-
vascular coagulation (DIC) due to factor loss from capillary
leak, endothelial activation, and clotting factor consump-
tion. The purpuric rash this causes in meningococcemia
is known as purpura fulminans, which is a coagulopathy
similar to DIC. The primary treatment for DIC is treating
the underlying infection. Therapy aimed at treating the
DIC specifically should be directed toward clinical stability
rather than correcting laboratory values. In the context of
active bleeding, vitamin K and blood products such as pla-
telets, fresh frozen plasma, cryoprecipitate, and PRBCs can
be used.
What is the prognosis for this child?
The current mortality rates for meningococcal meningitis
are approximately 10–15%. Of the 90% of patients who do
Reference
Wick, J.M., Krajbich, I., Kelly, S., and Dewees, T. (2013).
Meningococcemia: the pediatric orthopedic sequelae. AORN
Journal 97(5): 559–578. doi: 10.1016/j.aorn.2013.03.005.
Further Reading
Killien, E.Y. and Watson, R.S. (2017). Early death from
pediatric severe sepsis: still a problem and a mandate for
clinical research. Pediatric Critical Care Medicine 18(9):
891–892. doi: 10.1097/PCC.0000000000001239.
Medeiros, I., Melo, A.R., Baptista, V., and Ribeiro, A. (2018).
Meningococcemia: rare but life-threatening. BMJ Case
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Chapter 37 Infant with Increased Fussiness and Decreased Oral Intake 159
survive, 20% have permanent disabilities (Wick et al.,
2013). Therefore, despite its relative rarity compared to
the past, it remains important to recognize meningococce-
mia and act promptly. The most important intervention is
to reduce the risk of death by early administration of appro-
priate antibiotics. Antibiotic administration should not be
delayed and intraosseous or intramusclar routes should
be used if intravenous access cannot be obtained swiftly.
What other potential complications should you
anticipate?
Potential short-term complications include purpura fulmi-
nans leading to deep necrosis of digits or limbs, renal fail-
ure, ARDS, nosocomial pneumonia due to superinfection,
neurologic complications including coma, diabetes insipi-
dus, stroke and seizures, and immunologic complications
such as arthritis, cutaneous vasculitis, iritis, and pericardi-
tis. Long-term complications include hearing loss, scarring,
and orthopedic sequelae. Amputations are not uncommon,
and orthopedics should be consulted early to monitor extre-
mities (Wick et al., 2013).
Does anything need to be done for close contacts
of this patient?
Close contacts should be given post-exposure antibiotic
prophylaxis with a course of rifampin, ciprofloxacin, or cef-
triaxone, ideally within 24 hours of patient diagnosis. Refer
to the Centers for Disease Control for the most recent
recommendations.
Reports 2018: bcr-2018-226914. doi: 10.1136/bcr-2018-
226914.
Olbrich, K.J., Muller, D., Schumacher, S., et al. (2018).
Systematic review of invasive meningococcal disease:
sequelae and quality of life impact on patients and their
caregivers. Infectious Disease Therapeutics 7: 421–438. doi:
10.1007/s40121-018-0213-2.

38
Infant with Prolonged Jaundice
Aimee Bucci1,2
1
Phoenix Children’s Hospital, Phoenix, AZ, USA
2
Arizona State University, Edson College of Nursing and Health Innovation, Phoenix, AZ, USA
A 22-day-old female is transferred from the acute care
pediatric unit to the pediatric intensive care unit (PICU)
with chief complaint of fatigue, dehydration, and jaundice.
History of Present Illness
This infant has been overall healthy, but parents describe
her as yellow appearing and state that her eyes have
become more yellow over the last week. Parents also report
that her stools have become very pale and her urine is a
dark color. She has had decreased appetite over the last sev-
eral days and has appeared more tired. They deny any
fevers.
Past Medical History
This baby was born full term to a gravida 1, para 1 mother
who is breastfeeding. Mother’s pregnancy was uncompli-
cated except for a maternal fever at delivery so the infant
was treated with antibiotics for 48 hours and discharged at
3 days of life with negative cultures. She received vitamin
K and the first dose of hepatitis B vaccine while in the hos-
pital. Newborn metabolic screens revealed no abnormalities.
Past Surgical History
None.
Family History
No reports of liver disease, hemolytic conditions, vascular
conditions, or cardiac disease in the family.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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161
Current Status
The infant appears dehydrated and lethargic. She does not
appear to have dysmorphic features. She has jaundice with
scleral icterus present. Her abdomen is mildly distended
and she has evidence of hepatomegaly with the liver border
noted to be 2 cm below the right costal margin. Urine is
dark amber in color. Her heart rate is 165 bpm, blood pres-
sure is 72/35 mmHg, respiratory rate is 45 breaths per
minute, and oxygen saturations 95% on room air.
A complete metabolic panel was obtained with results
shown in Table 38.1.
Questions
Answer the following questions using the details provided.
1 Based on the history and examination findings, what
differential diagnoses should be considered for this
patient?
2 What additional laboratory evaluation is indicated for
this infant?
3 What diagnostic studies should be considered?
4 What other consultants should be considered in this
infant’s care?
Rationale and Evidence-based Practice
Explanation
Based on the history and examination findings,
what differential diagnoses should be considered
for this patient?
Differential diagnoses for this infant should include
diseases that cause physiologic infant cholestasis
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162 Cases in Pediatric Acute Care

Table 38.1 Complete metabolic profile. past 2–3 weeks of age have conjugated/direct bilirubin
levels measured. Basic additional studies to assess
Sodium 135 mEq/L
the synthetic function of the liver should include
Potassium 3.9 mEq/L alanine aminotransferase (ALT), aspartate aminotrans-
Chloride 101 mEq/L ferase (AST), alkaline phosphate (AP), gamma-
Carbon dioxide 18 mEq/L glutamyltranspeptidase (GGT), prothrombin time (PT),
Blood urea nitrogen 25 mg/dL international normalized ratio (INR), albumin, glucose,
Creatinine 0.7 mg/dL ammonia, complete blood count, urinalysis, and bacte-
Glucose 78 mg/dL
rial cultures of blood and urine if clinically indicated
(Fawaz et al., 2017, pp. 160–161). More advanced testing
Calcium 9.1 mg/dL
may be initiated by a hepatologist to evaluate for other
Albumin 3.0 mg/dL
causes of cholestatic jaundice.
Total protein 5.4 g/dL
Aspartate aminotransferase 147 U/L
Alanine aminotransferase 186 U/L What diagnostic studies should be considered?
Alkaline phosphatase 84 U/L
An abdominal radiograph can be obtained to assess the
Total bilirubin 18.6 mg/dL
abdominal distension. Fasting abdominal ultrasound is a
Direct bilirubin 4.6 mg/dL
non-invasive and easy study to obtain and can help to
identify visible obstructions of the biliary tree, the pres-
including biliary causes such as obstruction of large extra- ence of a choledochal cyst, and evidence of advanced liver
hepatic or small intrahepatic bile ducts or hepatocellular disease (Fawaz et al., 2017, p. 161). Hepatobiliary scintig-
etiology including defects in membrane transport, embryo- raphy can be used to confirm biliary tract patency but is
genesis, or metabolic dysfunction (Fawaz et al., 2017, not commonly used because of limitations in differentiat-
p. 155). Common conditions can include biliary atresia, ing idiopathic hepatitis from biliary atresia (Fawaz et al.,
common duct gallstones, choledochal cysts, inborn errors 2017, p. 161). Endoscopic retrograde cholangiopancreato-
of metabolism, Alagille syndrome, and various infections graphy (ERCP) can be used to detect the patency of the
(Fawaz et al., 2017, pp. 155, 163–165). extrahepatic biliary tree but can be technically challenging
The most likely diagnosis for this infant given the age, in small infants and requires specialized equipment that
persistent jaundice, and hyperbilirubinemia is biliary atre- some centers may not have available (Fawaz et al., 2017,
sia. Biliary atresia is a rare condition that affects the func- pp. 161–162; Verkade et al., 2016, p. 632). Liver biopsy
tion or the anatomy along the canalicular-bile tract remains the most widely used diagnostic tool, yielding
(Verkade et al., 2016, p. 631). Biliary atresia is the most com- a 90–95% accuracy for biliary atresia (Fawaz et al.,
mon cause of persistent cholestatic jaundice, with an inci- 2017, p. 162).
dence of 1 in 10 000–19 000 (Verkade et al., 2016, p. 631).
The common laboratory finding in infants with biliary atre-
sia is conjugated or direct hyperbilirubinemia. Infants with What other consultants should be considered in
benign or physiologic jaundice will present with an uncon- this infant’s care?
jugated or indirect hyperbilirubinemia (Verkade et al., Pediatric gastroenterologists or hepatologists should be
2016, p. 631). Examination findings of poor oral intake, consulted for any infant with direct hyperbilirubinemia
fatigue, abdominal distension, persistent jaundice, hepato- and presence of liver dysfunction. Pediatric surgeons
megaly, acholic stools, and dark urine should alert practi- should be consulted for the potential for a Kasai portoenter-
tioners to evaluation for liver disease. ostomy procedure to provide a temporary fix for biliary
atresia. A pediatric neurologist can be considered if the
What additional laboratory evaluation is indicated alterations in mental status are clinically significant.
for this infant?
A pediatric hematologist could be considered if the hyper-
The American Academy of Pediatrics (2004, p. 300) bilirubinemia is caused from unconjugated or indirect
recommends that all infants with jaundice persisting causes.

References
American Academy of Pediatrics Subcommittee on
Hyperbilirubinemia (2004). Management of
hyperbilirubinemia in the newborn infant 35 or more weeks
of gestation. Pediatrics 14: 297–316.
Fawaz, R., Baumann, U., Ekong, U., et al. (2017). Guideline for
the evaluation of cholestatic jaundice in infants: joint
recommendations of the North American Society for
Pediatric Gastroenterology, Hepatology, and Nutrition and
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Chapter 38 Infant with Prolonged Jaundice 163
the European Society for Pediatric Gastroenterology,
Hepatology and Nutrition. Journal of Pediatric
Gastroenterology and Nutrition 64(1): 154–168. doi: 10.1097/
MPG.0000000000001334.
Verkade, H.J., Bezerra, J.A., Davenport, M., et al. (2016).
Biliary atresia and other cholestatic childhood diseases:
advances and future challenges. Journal of Hepatology 65:
631–642.

39
Child with Rash and Drooling
Mark Weber
Children’s Hospital of Philadelphia, Philadelphia, PA, USA
A 32-month-old male with no significant past medical
history presents to the emergency department (ED) with
a 2-day history of a rash and respiratory concerns.
History of Present Illness
Two days prior to presentation, the child developed lip
swelling after eating a watermelon. He was given acetamin-
ophen, Benadryl and ibuprofen. His lips became more blis-
tered and he developed a fever of 38.5 C (101.3 F). He
developed a red macular rash on lips, hands including
palms, feet and trunk. He was seen at a local ED and
was diagnosed with hand, foot and mouth disease. He
was discharged to home following this visit.
Two days later his rash and blistering progressed and his
fever persisted. He had poor oral intake secondary to pain
and he complained of pain with urination. His mother
brought him back to the ED for further evaluation. While
in the ED, he did not have frank hematuria, melena/
hematochezia, hemoptysis or hematemesis. There was no
respiratory distress, although his mother did note hoarse-
ness and increasing drooling on the day of admission. He
did not have recent upper respiratory infection (URI)
symptoms, though he has been around other children with
URI symptoms. He has not taken any medications other
than acetaminophen, Benadryl and ibuprofen recently.
He does not take any medications on a daily basis.
Past Medical History
His history was only significant for a wheeze in the past but
has not required the use of his albuterol inhaler for several
months.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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165
Past Surgical History
No prior surgeries.
Family History
His mother has a family history of childhood asthma. There
is no other significant family history.
Current Status
His physical examination reveals a preference to keep his
eyes closed. His conjunctiva are injected bilaterally. His
lips and oral mucosa show significant erythema and des-
quamation. He is drooling with a hoarse voice and inter-
mittent coughing. The rest of his body has several red/
purple macules and plaques as well as vesicles across
the face, trunk and extremities, including palms and soles.
He has several bullae on his trunk and extremities
(Figure 39.1) covering about 35% of his body surface
area (BSA).
On arrival in the pediatric intensive care unit (PICU), he
continues with a hoarse voice and drooling. His initial vital
signs are temperature 40.5 C (104.9 F), heart rate 144 bpm,
blood pressure 115/56 mmHg, and oxygen saturation 98%
on room air. His respiratory examination shows a respira-
tory rate of 44 breaths per minute with clear breath sounds
bilaterally and no increased work of breathing. His weight
is 14.5 kg. He has received a normal saline fluid bolus and
one dose of oxacillin and clindamycin and initial laboratory
evaluation is obtained (Table 39.1).
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166 Cases in Pediatric Acute Care

Rationale and Evidence-based Practice

Explanation

What considerations are needed for his disposition

considering the dermatologic findings involving
approximately 35% of his BSA?
With the large BSA involvement, he would be best served in
a facility that has experience in managing burn patients. He
will require intensive wound management requiring an
expert multidisciplinary approach.

What are the differential diagnoses and the

leading diagnosis?
Figure 39.1 Plaques and bullae on back during the day of There are several diagnoses on the differential, most nota-
admission. Source: Private collection.
bly Stevens–Johnson syndrome and toxic epidermal necro-
lysis (SJS/TEN). Early in his disease course, his hands and
the soles of his feet were suspicious for hand, foot and
mouth disease. The areas of early erythema on the hands
Table 39.1 Basic metabolic profile.
and feet and his fever could also be concerning for Kawa-
Sodium 144 mEq/L saki disease. With the blisters and erythematous skin the
diagnosis of staphylococcal scalded skin syndrome should
Potassium 4.1 mEq/L
also be considered. The progressive nature of the skin
Chloride 114 mEq/L
lesions and the transition to blisters makes SJS/TEN more
Carbon dioxide 25 mEq/L likely. Both SJS and TEN will have mucosal involvement in
Blood urea nitrogen 18 mg/dL 90% of cases. The distinguishing feature between the two is
Creatinine 0.4 mg/dL the percentage of BSA involved. SJS has skin detachment
Glucose 178 mg/dL that is usually less than 10% BSA. If there is 10–30% BSA
Albumin 2.6 g/dL of skin involvement, the disease is referred to as SJS/TEN
overlap. The diagnosis of TEN is made when there is over
30% BSA skin involvement.
SJS and TEN are severe cutaneous adverse reactions.
Questions Most commonly, SJS/TEN is triggered by medications
and is associated with relatively high morbidity and mortal-
Answer the following questions using the details ity. Medications that have been associated with SJS/TEN
provided. include phenobarbital, carbamazepine (Tegretol), non-
1 What considerations are needed for his disposition con- steroidal anti-inflammatory medications, allopurinol, and
sidering the dermatologic findings involving approxi- fluconazole. Typically, medications with longer half-lives
mately 35% of his BSA? result in more serious disease. Therapy for SJS/TEN
2 What are the differential diagnoses and the leading includes removing the inciting agent (e.g. medication),
diagnosis? proper fluid and electrolyte balance, maintenance of aseptic
3 What is the most concerning feature of this examina- environment, dressing changes/wound debridement, and
tion that needs to be addressed in the PICU? exquisite eye care with referral to ophthalmologist as indi-
4 What subspecialties should be consulted in his care and cated by individual patient. Systemic steroids are indicated
what treatments do you anticipate they would in the treatment of SJS/TEN, often in conjunction with
recommend? other therapies including ciclosporin, tacrolimus, IVIG,
5 What other concerns would you need to anticipate and cyclophosphamide. Other strategies include plasma-
when managing his fluids and nutrition? pheresis and biologic agents (e.g. anti-tumor necrosis factor

Figure 39.2 Progression of epidermal destruction on back
13 days after admission. Source: Private collection.
alpha medications), but there remain controversies with
regard to all recommended therapeutics.
What is the most concerning feature of this
examination that needs to be addressed in
the PICU?
While in the ED and PICU, he was noted to have drooling
and a hoarse voice, which are features that indicate concern
about his ability to protect his airway and the possibility of
edema and sloughing that could obstruct his upper airway.
His aiway was secured via nasotracheal intubation per-
formed by anesthesia with fiberoptic guidance. The naso-
tracheal tube was chosen as it provides more stability for
securement and provides an increased level of comfort with
potential prolonged intubation.
What subspecialties should be consulted on his
care and what treatments do you anticipate they
would recommend?
The etiology of the disease was never determined for the
child, but a consult with dermatology indicated, first, a
Further Reading
Barron, S.J., Del Vecchio, M.T., and Aronoff, S.C. (2015).
Intravenous immunoglobulin in the treatment of Stevens–
Johnson syndrome and toxic epidermal necrolysis: a meta-
analysis with meta-regression of observational studies.
International Journal of Dermatology 54(1): 108–115.
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Chapter 39 Child with Rash and Drooling 167
recommendation to not give acetaminophen or ibuprofen
in the future as these medications may have been implicated
in the diagnosis. Dermatology also recommended treating
him with intravenous gamma globulin (IVIG) 1 g/kg
daily for 3 days as well as methylprednisolone 2 mg/kg daily
for 3 days. The use of IVIG can block the keratinocyte death
receptor Fas, therefore limiting the destruction of the
epidermis. It was also recommended to keep blisters intact
if possible (Figure 39.2).
Ophthalmology was consulted to assist in the manage-
ment of his conjunctivitis. The initial recommendation
was for erythromycin eye ointment every 2 hours alternat-
ing with Lacri-Lube every 2 hours. Ten days after admission
he began to develop epithelial injury to his cornea and he
was taken to the operating room for an amniotic membrane
transplantation (AMT) over his corneas bilaterally. An
AMT can help repair a damaged cornea by acting as a scaf-
fold that promotes cell migration and epithelial regenera-
tion on the cornea.
What other concerns would you need to anticipate
when managing his fluids and nutrition?
On presentation, he required fluid resuscitation related to
2-day history of poor oral intake and alterations in his skin
barrier. Once his initial deficit was replete, he was placed
on a maintenance intravenous fluid rate. Patients with
SJS/TEN may not require as much fluid as those with
burns; however, they have increased insensible losses
and higher fluid requirements than individuals with intact
skin. In addition to fluid loss due to disruption in epithelial
skin cells, he is also at significant risk for infection due to
loss of this barrier. He was transitioned to early enteral
feeds via a nasogastric tube to help optimize his nutrition
for wound healing; a high-protein diet is needed to promote
wound healing.
Gupta, L.K., Martin, A.M., Agarwal, N., et al. (2016).
Guidelines for the management of Stevens–Johnson
syndrome/toxic epidermal necrolysis: an Indian perspective.
Indian Journal of Dermatology, Venereology and Leprology 82
(6): 603–625.

168 Cases in Pediatric Acute Care
Kumar, R., Das, A., and Das, S. (2018). Management of
Stevens–Johnson syndrome/toxic epidermal necrolysis:
looking beyond the guidelines. Indian Journal of
Dermatology 63(2): 117–124.
Liu, W,. Nie, X., and Zhang, L. (2016). A retrospective analysis
of Stevens–Johnson syndrome/toxic epidermal necrolysis
treated with corticosteroids. International Journal of
Dermatology 55: 1408–1413.
Schwartz, M.A., Mcdonough, P.H., and Lee, B.W. (2013).
Toxic epidermal necrolysis: Part II. Prognosis, sequelae,
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diagnosis, differential diagnosis, prevention and
treatment. Journal of the American Academy of
Dermatology 69(2): 187.e1–187.e16. doi: 10.1016/j.
jaad.2013.05.002.
Sharma, N., Thenarasun, S.A., Kaur, M., et al. (2016). Adjuvant
role of amniotic membrane transplantation in acute ocular
Stevens–Johnson syndrome: a randomized control trial.
Ophthalmology 123(3): 484–491.

40
Child with Fever and Sore Throat
Stephanie Robell1 and Danielle Van Damme2
1
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA
2
Norton Children’s Hospital, University of Louisville, Louisville, KY, USA
A 4-year-old male presents to the emergency department
(ED) with fever, right-sided neck swelling, and sore throat.
History of Present Illness
He has a 1-week history of headache and sore throat. He
was evaluated by his primary care provider (PCP) at the
time and diagnosed with viral upper respiratory infection
(URI) and pharyngitis. This morning, he woke up with a
fever, right-sided neck swelling, difficulty swallowing,
and pain with neck movement. His mother took him back
to the PCP, where an influenza swab and streptococcus
throat culture were obtained. The PCP then referred the
child to the ED for further evaluation.
Past Medical History
The patient was born full term via spontaneous vaginal
delivery with no complications. Immunizations are up to
date for age, with exception of influenza vaccine this year.
There have been no recent sick contacts.
Past Surgical History
No prior surgeries.
Family History
No pertinent family history.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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169
Social History
Lives at home with both parents and one sibling. He is not
exposed to smoke and attends school and daycare.
Current Status
On arrival to the ED, he appears moderately ill but non-
toxic. On examination, he has an erythematous right tym-
panic membrane with effusion and his oropharynx is ery-
thematous with midline uvula. His right neck is swollen
with an 8 × 5 cm mass that is tender and without fluctu-
ance. He has decreased range of motion of his neck and
mild trismus. Vital signs include temperature of 39.1 C
(102.3 F), heart rate 118 bpm, blood pressure 96/65 mmHg,
respiratory rate 18 breaths per minute, and SpO2 98% in
room air.
Questions
Answer the following questions using the details provided.
1 Based on the clinical data, what are the differential
diagnoses and what is the leading diagnosis consistent
with the presenting symptoms and physical examina-
tion findings?
2 Based on your differential diagnoses, what diagnostic
studies are warranted?
3 What treatment would be recommended?
4 What are the management priorities?
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170 Cases in Pediatric Acute Care

Rationale and Evidence-based Practice

Explanation

Based on the clinical data, what are the

differential diagnoses and what is the leading
diagnosis consistent with the presenting
symptoms and physical examination findings?
Differential diagnoses for this child include retropharyngeal
abscess (RPA), peritonsillar abscess, bacterial tracheitis, epi-
glottitis, viral and bacterial pharyngitis, Lemierre syndrome,
meningitis, and tonsillitis. The leading diagnosis is RPA due
to the patient’s age and presenting symptoms of fever, right-
sided neck swelling, difficulty swallowing, pain with neck
movement, and sore throat. He also has a history of viral
URI and pharyngitis. Peritonsillar abscess does have a sim- Figure 40.1 CT image: retropharyngeal opacity on lateral neck/
ilar presentation to RPA, including sore throat, fever, dys- cervical spine view.
phagia, odynophagia, voice change, drooling, and trismus.
However, in peritonsillar abscess there is uvular deviation
toward the contralateral side, ipsilateral tonsillar bulging
and/or submandibular lymphadenopathy, and neck mass.
RPA is a suppurative deep neck infection that occurs in
the area extending from the base of the skull to the posterior
mediastinum between the posterior pharyngeal wall and
prevertebral fascia. It typically occurs following a URI of
one or more of these areas disseminating through the cap-
illary lymphatic drainage pathways, leading to suppuration
of the retropharyngeal lymph nodes and abscess formation.
RPA is frequently seen in children under 5 years of age and
more often in the winter to spring seasons.

Based on your differential diagnoses, what

diagnostic studies are warranted?
Based on the differential diagnoses, it is reasonable to ini-
Figure 40.2 CT image: worsening fluid collection/abscess on
tially obtain a lateral soft tissue radiograph of the neck; lateral neck/cervical spine view.
however, there is a high false-positive rate due to variability
in the technique of obtaining X-ray images. Therefore, com-
puted tomography (CT) with contrast is the imaging modal-
ity of choice as it can differentiate phlegmon and cellulitis
from abscess (Figures 40.1–40.3). CT scan is also helpful for
surgical planning. Findings of gas or air–fluid levels in the
soft tissue and widening of the prevertebral space beyond
5–7 mm at the level of the second cervical vertebra or
greater than 14 mm at the level of the sixth cervical vertebra
are consistent with a diagnosis of RPA.
Laboratory studies to consider include a throat culture to
evaluate for specific bacterial organisms to guide antimicro-
bial therapy. Complete blood count with differential will
typically demonstrate leukocytosis and possibly thrombo-
cytosis as an inflammatory response. C-reactive protein
may be elevated due to inflammation from a serious bacte-
rial infection. The results from these laboratory studies are Figure 40.3 CT image: demonstrating retropharyngeal fluid
shown in Tables 40.1 and 40.2. collection.
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Chapter 40 Child with Fever and Sore Throat 171

Table 40.1 Basic metabolic profile. most common pathogens. Occasionally, Haemophilus spe-
cies, Neisseria species, and Gram-negative enteric microbes
Sodium 137 mEq/L
are isolated. Antibiotic selections to eradicate these types of
Potassium 4.0 mEq/L organisms include beta-lactamase penicillins, cephalospor-
Chloride 107 mEql/L ins, clindamycin, and metronidazole. Vancomycin can be
Carbon dioxide 23 mEq/L considered if there is high suspicion for methicillin-resistant
Blood urea nitrogen 6 mg/dL Staphylococcus aureus (MRSA). Consideration should also
Creatinine 0.3 mg/dL be given to the patient’s ability to swallow, severity of illness,
Glucose 114 mg/dL
and respiratory distress when deciding between intravenous
and oral medication therapy. If there is no response to anti-
Total protein 5.8 g/dL
biotic therapy after 24–48 hours or there is airway compro-
Albumin 2.9 g/dL
mise, then surgical incision and drainage is usually
Calcium 9.0 mg/dL indicated. Steroids may be considered in patients exhibiting
Total bilirubin 0.3 mg/dL severe torticollis, stridor or odynophagia, with symptoms
Alanine aminotransferase 18 U/L typically demonstrated to improve 24 hours after use. Ibu-
Aspartate aminotransferase 23 U/L profen and acetaminophen can also be beneficial in treating
Alkaline phosphatase 130 U/L symptoms of fever, pain, and swelling.
C-reactive protein 7.9 mg/dL
What are the management priorities?
Management of a child with RPA should focus on mainte-
Table 40.2 Complete blood count.
nance and stabilization of the airway, including assessment
White blood cell count 28 200/mm3 of respiratory rate and effort, oxygenation, presence of stri-
Hemoglobin 13 g/dL dor, aeration in lung fields, ability to speak, position of com-
fort, and level of consciousness. It is important to allow the
Hematocrit 38%
patient to assume a position of comfort and to avoid agita-
Platelet count 399 000/mm3
tion in an effort to decrease the risk of airway compromise.
Neutrophils 85.9% Care should be taken during oral examination of these chil-
Eosinophils 2% dren to avoid rupturing the abscess. Supplemental humidi-
Monocytes 7% fied oxygen, nasal trumpet, or positive pressure ventilation
Lymphocytes 5.2% may be beneficial for moderate airway obstruction and
Bands 0% endotracheal intubation or tracheostomy may be indicated
for severe cases. Endotracheal intubation should be per-
formed by skilled providers with extensive training in air-
way management, and an otolaryngologist should be
What treatment would be recommended?
consulted urgently for a potential surgical airway and/or
First-line treatment for RPA, in a clinically stable patient, is operative drainage. A patient will require initial evaluation
antibiotic therapy as approximately 60% of infections will in the ED and close monitoring in a pediatric intensive care
resolve with medical management. Empiric antibiotic unit or acute care unit depending on the severity of illness,
agents should be selected to predominantly cover Gram- due to the risk of airway obstruction requiring an emergent
positive and anaerobic organisms. Streptococcal species, intervention until antibiotic therapy has treated the abscess
staphylococcal species, and respiratory anaerobes are the or surgical drainage is deemed necessary.

Further Reading
Bochner, R.E., Gangar, M., and Belamarich, P.F. (2017). A
clinical approach to tonsillitis, tonsillar hypertrophy, and
peritonsillar and retropharyngeal abscesses. Pediatrics in
Review 38(2): 81–92.
Jain, A., Singh, I., Meher, R., et al. (2018). Deep neck space
abscesses in children below 5 years of age and their
complications. International Journal of Pediatric
Otorhinolaryngology 109: 40–43.
Klein, M.R. (2019). Infections of the oropharynx. Emergency
Medicine Clinics of North America 37: 69–80.
Woods, C.R., Cash, E.D., Smith, A.M., et al. (2016).
Retropharyngeal and parapharyngeal abscesses among
children and adolescents in the United States: epidemiology
and management trends, 2003–2012. Journal of the Pediatric
Infectious Disease Society 5(3): 259–268.
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173

41

Teenager with Sickle Cell Disease and Fever

Shannon Konieczki and Katelyn Hamann
Children’s Hospital of Michigan, Detroit, MI, USA

A 17-year-old male with sickle cell anemia presented to the he was diagnosed with mononucleosis. He has mild
emergency department (ED) after developing a fever of intermittent asthma for which he takes albuterol as needed.
40 C (104 F) at home.

Past Surgical History

History of Present Illness No prior surgeries.

On presentation, he reports that he felt warm when he

woke up today, and also developed a cough. He took ibu-
profen after taking his temperature, but his fever persisted
and he began to feel dizzy. Given his history of sickle cell
anemia, he immediately presented to the ED for evaluation.
He reports that no one else living in his home is sick. He
attends high school, but had no knowledge of any sick con-
tacts. He has no recent travel history. He has been able to
attend school/participate in activities without difficulty. He
has not been more tired than normal, and denies night
sweats, rashes, bleeding or bruising. He has been eating
well, with no recent dietary change, and bowel movements
are normal. He had no persistent pain or joint swelling. Of
note, he was diagnosed with mononucleosis one month
prior and since that time has experienced a 2.3-kg weight
loss (3% weight loss from baseline).
Family History
Both parents have sickle cell trait; no one else in the family
has sickle cell anemia. His maternal grandmother has
hypertension and diabetes mellitus. No one in the family
has a history of cancer, autoimmune disease, bleeding, or
clotting disease.
Current Status
In the ED, a fever of 39.3 C (102.7 F) is noted. He has a mild
cough and chest pain. He also has a headache and feels dizzy.
His blood pressure is 109/55 mmHg, heart rate 120 bpm,
respiratory rate 20 breaths per minute, and oxygen satura-
tion 96% on room air. Bilateral lung sounds are clear and
equal and he has a comfortable respiratory effort.

Past Medical History

He was born full-term without complications. Sickle cell

anemia was diagnosed on newborn screen, and he has
had appropriate follow-up appointments since birth. He
has a history of acute chest syndrome that required a blood
exchange transfusion when he was 4 years old. His baseline
hemoglobin ranges from 7 to 9 g/dL. His last admission to
the hospital was 1 month prior with symptoms of fever, and
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses for this patient?
2 What initial diagnostic testing is indicated?
3 What therapy would you initiate?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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174 Cases in Pediatric Acute Care

Table 41.1 Basic metabolic profile.

Sodium 141 mEq/L

Potassium 4.1 mEq/L
Chloride 102 mEq/L
Carbon dioxide 24 mEq/L
Blood urea nitrogen 7 mg/dL
Creatinine 0.7 mg/dL
Glucose 100 mg/dL

Table 41.2 Complete blood count.

White blood cell count 25 000/mm3

Hemoglobin 7 g/dL
Hematocrit 20.2%
Platelet count 234 000/mm3
Neutrophils 88%
Eosinophils 0%
Monocytes 2%
Figure 41.1 Chest radiograph showing bilateral lower lobe
Lymphocytes 7% pneumonia.
Bands 3%

Continuation of case: The results of the initial laboratory Table 41.3 CMV viral load.
investigations become available (Tables 41.1 and 41.2).
Day 2 of admission 353 853
Chest X-ray is obtained and noted to be unremarkable.
Day 4 of admission 485 082
On day 3 of admission, his fevers persist and he reports
chest tightness. His pulse-oximeter reading drops to 88% Day 7 of admission 15 862
and he requires 2 L/min of oxygen via nasal cannula. On
auscultation, his chest sounds are diminished bilaterally.
No crackles are heard. A chest radiograph is obtained
Table 41.4 Complete blood count day 7 of admission.
and is significant for bilateral lower lobe pneumonia
(Figure 41.1). Polymerase chain reaction (PCR) for cyto- White blood cell count 27 000/mm3
megalovirus (CMV) and Epstein–Barr virus (EBV) returns
Hemoglobin 6.9 g/dL
positive but blood culture remains negative.
Hematocrit 19.8%
Infectious disease service is consulted, and ganciclovir is
Platelet count 158 000/mm3
started for suspected CMV pneumonia. Azithromycin is
added for possible concurrent mycoplasma pneumonia, Neutrophils 68%
and cefepime is continued. The CMV viral load decreases Eosinophils 3%
appropriately (Table 41.3), but fevers are persistent. On Monocytes 5%
day 7 after admission, another complete blood count is Lymphocytes 8%
obtained (Table 41.4). Bands 4%

Rationale and Evidence-based Practice

Explanation
Based on the data given, what are the differential
diagnoses for this patient?
Differential diagnoses for acute fever, dizziness, chest pain
and weight loss can be separated into infectious and non-
infectious etiologies. Infectious etiologies include viral
and bacterial causes of upper respiratory tract infection
and pneumonia, as well as reactivation of EBV, blood-
stream infection, and sexually transmitted infections
(STIs). Non-infectious causes include autoinflammatory
disorders, immune-mediated disease, neoplasia, and

hypersensitivity disorders. Specifically, acute chest syn-
drome must be considered in the setting of sickle cell dis-
ease with chest pain.
What initial diagnostic testing is indicated?
Initial diagnostic testing should include a basic metabolic
profile to evaluate for electrolyte derangements, dehydra-
tion and renal function, and a complete blood count is
needed to evaluate for infection. C-reactive protein can also
be obtained as a marker of inflammation/infection. A blood
culture is needed to evaluate for bloodstream infection.
Because of the new cough, a chest radiograph is needed
to evaluate for a pulmonary process. Because of the recent
history of mononucleosis, PCR for CMV and EBV is also
obtained.
What therapy would you initiate?
Given his history of sickle cell anemia, he is started on
intravenous 0.9% saline solution for rehydration and a
dose of ibuprofen is administered for his fever. He is trans-
ferred to the hematology department from the ED for con-
tinued evaluation of his fever and monitoring. He
continues to have fevers up to 40.4 C (104.7 F) despite
receiving ibuprofen and acetaminophen (alternating)
every 6 hours. He is started on intravenous cefepime for
broad-spectrum bacterial coverage while cultures are
pending, and he is monitored for signs of acute chest syn-
drome. He is continued on intravenous 0.9% saline at
maintenance fluid rate.
4 Because of the persistent fevers, what further questions
would you ask the patient?
5 What other tests would be appropriate?
Because of the persistent fevers, what further
questions would you ask the patient?
According to the history of the present illness, the patient
had not recently traveled, had no family history risk fac-
tors for immune-mediated disease, and did not show
any symptoms of a neoplasm such as bone pain or night
sweats. He is also older than the typical patient with an
autoinflammatory disease. It would be appropriate to
ask questions about drug use and about sexual history
to evaluate for risk of STI.
What other tests would be appropriate?
Testing should be done for HIV and other STIs such as gon-
orrhea, syphilis, chlamydia, and hepatitis. According to the
Centers for Disease Control and Prevention (CDC), patients
should be notified that HIV screening will be done, but no
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Chapter 41 Teenager with Sickle Cell Disease and Fever 175
Table 41.5 HIV testing.
HIV antigen/antibody combined Reactive
HIV-1 antibody Geenius Positive
written consent is required. The general consent for medi-
cal care required on admission is sufficient consent for test-
ing. Patients may decline testing, referred to as “opt-out
screening,” and should be documented in the medical
record.
Continuation of case: On further questioning, the patient
discloses to family and the medical team that he has been
sexually active and that he engaged in receptive sex with
one male partner 2 months previously. He was unaware
of his partner’s HIV status. Laboratory work to evaluate
HIV status is obtained (Table 41.5).
6 What are the symptoms of acute HIV and what testing
should be done to diagnose it?
7 What is the treatment of acute HIV?
What are the symptoms of acute HIV and what
testing should be done to diagnose it?
Acute infection of HIV is the period between viral transmis-
sion and the emergence of HIV-specific antibodies, gener-
ally accompanied by a burst of viremia. Symptoms of acute
HIV infection are non-specific and include fever, rash,
malaise, myalgia, and headache. Acute HIV infection is
often described as mononucleosis or influenza-like. Usually
symptoms develop 1–4 weeks after transmission, and per-
sist for 2–4 weeks. Physical examination can be notable
for hepatosplenomegaly. Presentation may consist of new
opportunistic infections, indicating a compromised
immune system.
According to CDC, initial testing for HIV should be done
via the HIV-1/HIV-2 antigen/antibody immunoassay (a
fourth-generation HIV test). This test detects HIV antibo-
dies as well as the HIV p24 antigen. Prior testing using
the third-generation HIV tests only detected HIV-specific
antibodies, missing the earliest pre-antibody phase, and
may result in false negatives. If the first HIV antigen/anti-
body combined test is “reactive,” it is considered positive
for HIV. Next, testing should be performed to differentiate
HIV-1 and HIV-2 antibodies. The confirmatory test, as used
in this case, reports the HIV-1 antibody as “positive” con-
firming HIV-1.
New rapid HIV tests are HIV antibody-based tests (RNA
viral load) that measure the level of HIV nucleic acid using
quantitative PCR testing, branched DNA, or nucleic acid
amplification assays, and can show positive 11 days from

176 Cases in Pediatric Acute Care
time of infection. To confirm diagnosis, viral load testing
should be done with ELISA or Western blot test again
within 4–6 weeks.
Additional laboratory tests should be done, including
CD4 and CD8 lymphocyte counts to monitor immune sup-
pression, hepatitis B and C, and tuberculosis.
What is the treatment of acute HIV?
Initial management of acute HIV includes psychosocial
evaluation and stabilization including a domestic violence
screen, substance abuse screen, and referral to counseling
and support services. Providers should educate patients
about their infectiousness and how to prevent risk in
others. Advances in antiretroviral therapy (ART) have
transformed HIV into a chronic and treatable disease, but
many patients are still being diagnosed at a late stage. Pro-
viders must be trained to recognize indicators of infection
and subsequent testing for HIV must be promoted. Late
presentation of HIV is associated with increased morbidity
Further Reading
Centers for Disease Control and Prevention (2014). Laboratory
Testing for the Diagnosis of HIV Infection: Updated
Recommendations. Atlanta, GA: CDC. doi: 10.15620/
cdc.23447.
Rutstein, S., Ananworanich, J., Fidler, S., et al. (2017). Clinical
and public health implications of acute and early HIV
detection and treatment: a scoping view. Journal of the
International AIDS Society 20: 21579. doi: 10.7448/
IAS.20.1.21579.
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and mortality. Infected individuals should be identified
early so they can benefit from early ART, as well as redu-
cing transmission to others.
Case Resolution
The patient improved and was discharged from the hospital
on day 14. He has since followed up at an HIV clinic. There,
he is under the care of a multidisciplinary team including a
pediatrician, a nurse practitioner, several nurses, a social
worker, and a psychologist. He was started on bictegravir,
emtricitabine and tenofovir alafenamide (Biktarvy), and
continued on valganciclovir to complete his CMV treat-
ment. He was also started on levofloxacin for prophylactic
coverage of bacterial infections. He was given education on
HIV, strategies of medication adherence, counseling on
safe sex and risk reduction, and was screened for depres-
sion/anxiety.
Tominski, D., Katchanov, J., Driesch, D., et al. (2016). The late-
presenting HIV-infected patient 30 years after the
introduction of HIV testing: spectrum of opportunistic
diseases and missed opportunities for early diagnosis. HIV
Medicine 18: 125–132.
Torreggiani, S., Filocamo, G., and Esposito, S. (2016).
Recurrent fever in children. International Journal of
Molecular Science 17(4): 448. doi: 10.3390/ijms17040448.

42
Ethical Case Study
Jamie Tumulty
University of Maryland Children’s Hospital, University of Maryland Medical Center, Baltimore, MD, USA
A 15-year-old male with cerebral palsy, hypotonia, develop-
mental delay, asthma, chronic lung disease, gastroesopha-
geal reflux disease, and epilepsy was admitted to the
pediatric intensive care unit (PICU) with hypoxic
respiratory failure secondary to pneumonia with pleural
effusion, and septic shock.
This ethical case study will address issues faced by the
acute care pediatric provider to include effective communi-
cation for obtaining informed consent, addressing decision-
making conflict, and when to initiate palliative care consul-
tation or an ethical consult.
History of Present Illness
The patient is a medically complex child with cerebral
palsy, hypotonia, developmental delay, asthma, chronic
lung disease, gastroesophageal reflux disease, and epi-
lepsy who presented with fever and acute onset of respi-
ratory distress. Two days prior to presentation he
developed increased secretions and was febrile to
38.9 C (102 F). On the day of presentation, his family
noted labored breathing and he became less interactive,
which prompted the visit to the emergency depart-
ment (ED).
Past Medical and Surgical History
The teen is wheelchair dependent, with problems already
described. At baseline, he is non-verbal but alert, interac-
tive, smiles and laughs. He was intubated once for status
asthmaticus.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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177
Family History
The child is adopted. His biological mother had a rare
X-linked genetic disorder causing severe intellectual
disability and hypotonia. She is now deceased.
Social History
The patient has been in medical foster care since age 2 years
and was recently adopted by the foster parents. His adop-
tive parents have a biological son who is a pediatric ED phy-
sician in another state, and two other children aged 7 and
8 years for whom they provide medical foster care. Parents
provide all care for their son without home nursing.
Current Status
In the ED, the teen requires aggressive respiratory treatment
but is ultimately intubated and mechanically ventilated. He
is transferred to the pediatric intensive care unit (PICU)
where a chest tube is placed to evacuate a pleural effusion,
noted on the admission chest radiograph. This child was
intubated with attempts to extubate twice through his PICU
stay. As a result of the two failed extubation attempts, his
underlying muscle weakness and restrictive lung disease,
the PICU team felt a tracheostomy may be of benefit.
Questions
Answer the following questions using the details provided.
1 Has the PICU team given this teen significant opportu-
nity to recover from his illness and adequate chance for
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178 Cases in Pediatric Acute Care

successful extubation before suggesting tracheostomy? Rationale and Evidence-based Practice

If so, when is the appropriate time to perform
tracheostomy?
2 How does the team gain consensus with the family on
plan of care?
3 As a family–provider communication opportunity,
what are the essential components of the family
meeting?
Explanation
Has the PICU team given this teen significant
opportunity to recover from his illness and
adequate chance for successful extubation before
suggesting tracheostomy? If so, when is the
appropriate time to perform tracheostomy?

Continuation of case: A family meeting was scheduled
and included the attending physician, acute care nurse
practitioner (ACNP), nurse, social worker, child life spe-
cialist, and the chaplain. The family members included
the adoptive mother, father, and physician brother who
attended via video conferencing. Although a family meet-
ing may have been conducted earlier in this child’s admis-
sion, this family meeting was timely as it occurred within
24 hours of a crucial decision point. Table 42.1 describes
this patient’s family meeting.
There is no consensus about the appropriate time to con-
sider performing a tracheostomy for pediatric patients. Sev-
eral retrospective studies have shown that tracheostomy
performed before 14 days is associated with shorter hospital
length of stay and fewer complications than tracheostomy
performed after 14 days (Holloway et al., 2015). In this case,
there were two failed extubations, a 22-day PICU length of
stay, and an expected prolonged hospitalization even if a
tracheostomy is performed. The patient’s parents stated
they were not interested in pursuing a tracheostomy.

4 What successful processes are utilized in the family

How does the team gain consensus with the family
meeting?
on plan of care?
5 When should the palliative care team be consulted?
6 Based on this case, when would an ethics committee Families consistently rate communication as one of the
consult be warranted? most important skills of clinicians, often as important as

Table 42.1 Script of family meeting.

Participant Script

Attending physician
• Introduces health team members

• Identifies the problem: patient has had failed attempts at intubation, would benefit from tracheostomy, but
family members are not in agreement

• Inquires about family goals for patient, asking about child’s life at home

• Child was adopted at age 2 and has always been cared for at home by family members

•
Parents
He has been happy and interactive and it is difficult to see him so ill, especially with breathing issues

Attending physician • Offers options:

– Wean ventilator and add non-invasive technology

••
– Tracheostomy
Educates about the purpose of tracheostomy
Asks what questions the family has

Father • Expresses interest in trying gradual weaning approach, and offers that the tracheostomy and ventilator at
home are overwhelming and is concerned about the effect on family life and other children

Attending physician • Adds information about the possibility of home nursing and child life therapist to support the other children
if tracheostomy is decided

Parents • Share that child has been deteriorating slowly and they would like to inquire about hospice and
palliative care

Attending physician • Offers to organize a meeting with the palliative care team and closes the meeting by asking if there are other
questions
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Chapter 42 Ethical Case Study 179

Table 42.2 VALUE acronym. should elicit the patient and family’s preferred method of
decision-making and tailor their own role to match the
V Value family statements
family’s preference.
A Acknowledge family emotions
L Listen to the family
U Understand the patient as a person
As a family–provider communication opportunity,
E Elicit family questions what are the essential components of the family
Source: Data from Curtis (2008). meeting?
There are several platforms for family communication
including participation in daily ICU rounds, daily updates,
clinical skills, especially during critical illness. Parents
electronic patient portals, and interdisciplinary family
want honest, clear, and complete information in order to
meetings (Seaman et al., 2017). Teleconferencing and video
participate in the decision-making for the care of their chil-
conferencing offer the added benefit of allowing ICU provi-
dren even if it is distressing. Seaman et al. (2017) identified
ders to communicate with physically distanced family
five goals of clinician–family communication, including
members, other family support individuals such as faith
establishing a trusting relationship, providing emotional
leaders, family friends, and other members of the health-
support, helping families understand the prognosis and
care team (De Havenon et al., 2015). Ultimately, the ICU
treatment options, allowing clinicians to understand the
family meeting is an essential means of communication
patient as a person, and creating conditions for careful
about patient status and subsequent decision-making about
deliberation about difficult decisions. Table 42.2 shows
goals of care.
an acronym for communication that can be helpful for
Although the evidence to support the benefits of family
interaction with families.
meetings is substantial, they often fail to occur in a timely
Shared decision-making is a central component of
manner. Some studies have shown these benefits from fam-
patient-centered care in the intensive care unit (ICU)
ily meeting that occur 3–6 days after admission, but the
(Kon et al., 2016). The Informed Medical Decisions Foun-
ACCM recommends that multidisciplinary family meetings
dation, American College of Critical Care Medicine
occur within 24–48 hours of ICU admission and are sched-
(ACCM), and American Thoracic Society have defined
uled at regular intervals. Family meetings should be timely,
shared decision-making as a collaborative process that
occur in a setting that is quiet with adequate seating, and
allows patients, or their surrogates, and providers to make
include appropriate interprofessional team members.
healthcare decisions together, based on the best scientific
There are many barriers to family meetings, including
evidence available and the patient’s values, goals, and
burdens of schedule coordination, lack of provider availa-
preferences.
bility, and physical distance of pertinent family partici-
The standard approach to shared decision-making
pants. Other barriers to family meetings include
occurs when:
inadequate communication skill training of providers, cul-

• providers share information with families about the

patient’s medical condition, prognosis, and therapeutic
options along with the associated risks of each option,
including the option of palliative care;
tural barriers, language, and emotional stress for providers
(and families). Table 42.3 shows strategies to overcome
some of these barriers. The ACCM Guideline for Family
Conferences advocates for multiple strategies to improve

• the family shares with the provider the patient’s values,

goals and preferences;
the effectiveness of family conferences (Kon et al., 2016).
The family meeting structure allows for other team mem-

• the provider and family deliberate together to determine

the best treatment option for the patient and the patient
is included if developmentally appropriate and clini-
cally able;
bers to listen, clarify issues as appropriate, and affirm deci-
sions. A scribe documents the discussion and may use a
template to transfer information to the chart.

• once global goals and preferences are determined, the

provider is responsible for making routine decisions
What successful processes are utilized in the
family meeting?
(e.g. frequency of vital signs and laboratory data);

• the provider explains the care provided and rationale in

language that is understandable to the patient and family
• The family meeting is planned to include all identified
family and healthcare team members.
and elicits their preferences in the decision-making proc-
ess (Kon et al., 2016; Davidson et al., 2017). The clinician
• A venue for inclusion of distant family members is pro-
vided by video conferencing.
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180 Cases in Pediatric Acute Care

Table 42.3 Goals of family communication.

Goal Skills Strategies

Establish a trusting relationship Patient and sensitive listening VALUE

Provide emotional support
Help families understand
diagnosis, prognosis, and
treatment options
Allow clinicians to understand
the patient as a person
Create conditions for careful
deliberation about difficult
decisions
Source: Seaman et al. (2017).
Providing consistent message
about patient’s condition and
plan of care
Addressing emotions (e.g. anger,
grief, guilt)
If necessary, preparing patient
and family for death
Recognizing cultural and
language barriers
Tailoring explanation of complex
physiology and technology to
family’s level of knowledge
Eliciting information about
values, goals, and treatment
preferences
Attending to family dynamics
Conflict resolution
Assisting in decisions about life
sustaining therapies
Ascertain family’s communication preferences
Manage specialists’ input (may require a preliminary
multidisciplinary meeting)
VALUE
Consider consults to pastoral care, child life specialist
Include interpreter if language barrier exists or
requested by family
Avoid medical jargon, use simple lay terms
Tailor language to the comprehension level of the
family
Use written or electronic supplementary materials
(e.g. pamphlets, diagrams)
Develop awareness of own bias to remain open to
patient and family preferences that differ from
your own
VALUE
Use shared decision-making
Dedicated multidisciplinary family meetings
Ethics consultation
Palliative care consultation
Facilitate restful sleep for families (e.g. private room,
noise reduction strategies)
Consider involving the child in decisions if
developmentally appropriate and clinically able

• The physician uses a strategy to seek information from

the family about their child, acknowledge their emotions,
(2016), children with complex medical problems are surviv-
ing longer due to new and improved treatments, especially
listen to their concerns, and offer options. among preterm infants and neonates with congenital disor-

• The meeting is held in a quiet place, gaining family per-

ception by asking for their input, inviting them to share
their knowledge, and providing knowledge about care
options.
ders. It is currently estimated that there are approximately
400 000 children living with life-threatening or chronic
complex conditions, with about 17 million adults serving
as caregivers to these children. Despite some concern about

• Shared decision-making was facilitated by providing

information about care choices, eliciting family prefer-
ences, and deliberating to reach a decision.
When should the palliative care team be
consulted?
Palliative care strives to manage pain, incorporate psycho-
social and spiritual care, identify values of patients and
their loved ones, and resolve conflicts in cases in which
the patient is terminally ill (Celie and Prager, 2016).
According to the Center to Advance Palliative Care
consulting too soon, it is recommended to introduce the
concept of palliative care early in the life of a child who
has a life-limiting condition (Center to Advance Palliative
Care, 2016). The American Academy of Pediatrics recom-
mends initiating a palliative care consult when a diagnosis
is made and integrating interventions, both curative and
comfort measures, throughout the course of illness to sup-
port quality of life. Unfortunately, only about half of all
children’s hospitals have formal palliative care programs,
so much more work needs to be done to improve access
for children and families.

Based on this case, when would an ethics
committee consult be warranted?
Ethics consultation is a commonly accepted procedure
for facilitating communication, resolving conflict, and
easing moral distress in healthcare. It should be pur-
sued when there is disagreement among team members
or between the medical team and family. Some situa-
tions that may warrant an ethics consult include family
preference to withdraw life-sustaining therapy when
the medical team believes continuing therapy is in
the best interests of the child; family request for inter-
ventions the medical team believes are futile; and fam-
ily desire to perform rituals that violate state health
and safety codes (Kon et al., 2016). The ethics commit-
tee is advisory only and consults can come from indi-
vidual healthcare providers, a healthcare team or
family members. The consult will provide recommen-
dations based on the posed question. Studies have
shown that ethics consults decrease ICU length of stay
and life-sustaining therapies for patients who poten-
tially will not survive to discharge. These consults
may reduce prolonged suffering for patients and
families.
Ethicists use four main ethical principles to guide recom-
mendations for complex cases.
References
Celie, K. and Prager, K. (2016). Health care ethics consultation
in the United States. AMA Journal of Ethics 18(5): 475–478.
doi: 10.1001/journalofethics.2017.18.5.fred1-1605.
Center to Advance Palliative Care (2016). Pediatric palliative
care field guide. Available at https://www.capc.org/toolkits/
designing-a-pediatric-palliative-care-program/
Curtis, J.R. (2008). 34th Donald F. Egan Scientific Memorial
Lecture: Caring for patients with critical illness and their
families: the value of the integrated clinical team.
Respiratory Care 53(4): 480–487.
Davidson, J., Aslakson, R., Long, A., et al. (2017). Guidelines
for family centered care in the neonatal, pediatric, and adult
ICU. Critical Care Medicine 45(1): 103–128. doi: 10.1097/
CCM.0000000000002169
De Havenon, A., Petersen, C., Tanana, M., et al. (2015). A pilot
study of audiovisual family meetings in the intensive care
unit: a randomized controlled trial. Journal of Critical Care
30(5): 881–883. doi: 10.1016/j.jcrc.2015.05.027.
Further Reading
Moon, M. and American Academy of Pediatrics Committee on
Bioethics (2019). Institutional Ethics Committees Policy
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Chapter 42 Ethical Case Study 181
•• Beneficence: the therapy must help the patient.
Non-maleficence: the therapy must not harm the patient.
•• Justice: therapies must be applied fairly.
Autonomy: the patient must be free to decide.
In this case, ethical questions may include the following.
• Would a tracheostomy be of benefit or could it
cause harm?
•• Is it fair that a child receive a tracheostomy?
Given that tracheostomy is a standard therapy, can par-
ents opt not to pursue tracheostomy?
In this case, the ethical considerations were not chal-
lenged. The values and judgments of parents should gener-
ally be honored in difficult ethical matters, although
providers can seek to override parental wishes when the par-
ent’s request is not in the best interest of the child. However,
some of these decisions are state regulated (Weise et al.,
2017). Some ethicists believe the decision should be based
on the best interests of the child only, while others consider
the best interests of the family. In this patient case, either
decision was supported by the medical team. When making
this type of decision, though, families want reassurance that
they will not be abandoned by the healthcare team if they
choose a palliative care strategy or do not resuscitate.
Holloway, A.J., Spaeder, M.C., and Basu, S. (2015). Association
of timing of tracheostomy on clinical outcomes in PICU
patients. Pediatric Critical Care Medicine 16(3): e52–e58. doi:
10.1097/PCC.0000000000000336.
Kon, A., Davidson, J., Morrison, W., et al. (2016). Shared
decision making in ICUs: an American College of Critical
Care Medicine and American Thoracic Society Policy
Statement. Critical Care Medicine 44(1): 188–201. doi:
10.1097/CCM.0000000000001396.
Seaman, J., Arnold, R., Scheunemann, L.P., and White, D.B.
(2017). An integrated framework for effective and efficient
communication with families in the adult intensive care
unit. Annals of the American Thoracic Society 14(6): 1015–
1020. doi: 10.1513/AnnalsATS.201612-965OI.
Weise, K.L., Okun, A.L., Carter, B.S., Christian, C.W. and the
Committees on Bioethics, Section on Hospice and Palliative
Medicine and Committee on Child Abuse and Neglect
(2017). Guidance on forgoing life-sustaining treatment.
Pediatrics 140(3): e20171905; doi: 10.1542/peds.2017-1905
Statement. Pediatrics 143(5): e20190659. doi: 10/1542/
peds.2019-0659.

43
Infant with Neurologic Changes and Respiratory Failure
Blythe Pollack and Stacey Sears
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA
The patient is a 5-month-old male, with no significant med-
ical history, admitted to the pediatric intensive care unit
(PICU) with neurologic changes and acute respiratory
failure.
History of Present Illness
The infant was in his usual state of health until 1 day prior
to his emergency department (ED) visit and subsequent
admission. His parents brought him in with concerns for
one day of fever over 38.9 C (102 F), bilious non-bloody
emesis, generalized abdominal pain, and decreased oral
intake. Ibuprofen was administered for fever relief, but
the fever returned despite multiple doses. Primarily
breast-fed, but showed no interest in feeding during the
hours preceding visit. He was reported to have three wet
diapers during the day. Parents deny constipation or
diarrhea.
Past Medical History
He was born term at 39 2/7 weeks with a non-complicated
vaginal delivery. He did not require airway interventions at
birth, tolerated breastfeeding, and was discharged home on
day of life 2. His immunizations are up to date. Parents deny
recent travel.
Past Surgical History
No past surgical history.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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183
Family History
No pertinent family medical history. There are two older
siblings in the home, who are currently well, but occasion-
ally have intermittent viral illnesses.
Current Status
On examination, he appears ill with dry cracked lips,
sunken eyes, and flat anterior fontanelle. Extremities are
cool to the touch, with warm head and trunk. He has
decreased bowel sounds with abdominal distension. Nurs-
ing staff have witnessed an episode of emesis while breast-
feeding, but he has easy work of breathing, without
adventitious sounds. Vital signs on arrival to the ED are
temperature 38.6 C (101.5 F), heart rate 138 bpm, blood
pressure 124/59 mmHg, respiratory rate 34 breaths per
minute, and oxygen saturation 100% on room air.
With concern for an abdominal obstruction and fever in
an infant, an abdominal radiograph, urinalysis, and a res-
piratory viral nasal swab are completed. Because of signs
of dehydration, a peripheral intravenous catheter is placed,
blood work obtained, and rehydration therapy started fol-
lowing an initial 40 mL/kg of normal saline. While in the
ED, he shows positional preference with his head, and
no longer turns it to the right (with negative meningeal
signs). He has undergone computed tomography (CT) of
the neck without pertinent findings. He continues to have
fever and blood cultures are obtained and a single dose of
ceftriaxone administered. An abdominal ultrasound and
upper gastrointestinal investigation are completed for fur-
ther evaluation of his emesis. He is admitted to the general
care ward. Results of basic metabolic profile, complete
blood count and urinalysis are shown in Tables 43.1–43.3.
Other initial results are as follows.
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184 Cases in Pediatric Acute Care

Table 43.1 Basic metabolic profile. colon. There is minimal small bowel gas. No pneumoper-
itoneum is seen.
Sodium
Potassium
136 mEq/L
5.1 mEq/L • Abdominal ultrasound: non-visualization of the appen-
dix; however, no free fluid seen in the right lower quad-
Chloride 103 mEq/L rant and no visible inflammatory changes.
Carbon dioxide
Blood urea nitrogen
23 mEq/L
7 mg/dL
• Upper gastrointestinal examination: negative, no evi-
dence of malrotation.
Creatinine 0.2 mg/dL
Glucose 118 mg/dL Questions
Calcium 10.2 mg/dL
Albumin 4.4 g/dL
Answer the following questions using the details provided.
1 Based on the information given, what is the differential
diagnosis?
2 What are meningeal signs?
Table 43.2 Complete blood count.

White blood cell count 15 000/mm3

Hemoglobin 9.9 g/dL Rationale and Evidence-based Practice
Hematocrit 30.8% Explanation
Platelet count 528 000/mm3
Neutrophils 66% Based on the information given, what is the
Eosinophils 1% differential diagnosis?
Monocytes 11% This case was well differentiated in the ED. The differential
Lymphocytes 21% diagnosis includes viral illness, bronchiolitis, dehydration,
Bands 0% constipation, bowel obstruction, malrotation, testicular tor-
sion, pyloric stenosis, meningitis, and peritonsillar and ret-
ropharyngeal abscess.
While in the ED, respiratory and urine samples were sent
Table 43.3 Urinalysis. for viral and bacterial cultures for evaluation of infection.
A chest radiograph was completed to evaluate for infil-
Color Normal
trates. Meningitis was also considered; however, the clini-
Appearance Cloudy
cal examination was not consistent, as he had no nuchal
Specific gravity 1.025 rigidity, was consolable by parents, the fontanelle was flat,
pH 5.5 and there was no evidence of lethargy. However, there was
Leukocyte esterase Negative positional head preference and possible neck stiffness so a
Nitrate Negative CT of the neck was completed to evaluate for retropharyn-
Protein <30 mg/dL geal and peritonsillar abscess. The abdomen was consid-
Glucose Normal ered a concerning source, and therefore appendicitis,
malrotation, pyloric stenosis, and testicular torsion were
Ketones <5 mg/dL
evaluated with abdominal ultrasound, radiography, and
clinical examination.

••
Respiratory panel: rhinovirus/enterovirus positive.
Neck CT: no findings consistent with tonsillar, peritonsil-
lar or retropharyngeal abscess. Mild prominence of Wal-
deyer’s ring lymphoid tissue without significant
associated nasopharyngeal or oropharyngeal airway nar-
rowing. Presumed reactive lymph nodes identified
throughout the upper neck.
When the differential is wide, supportive care and fre-
quent reassessment is optimal. It was noted at the time of
presentation that he was dehydrated. He had continued
emesis and poor oral intake, likely in the setting of a viral
illness. Dehydration is a commonly treated symptom in the
pediatric population. Pediatric patients have significant
evaporative losses due to the high ratio of surface area to

•
weight. He was given a 40 mL/kg bolus in the addition to
Abdominal radiograph: normal, non-obstructive bowel maintenance intravenous fluids to expand plasma volume
gas pattern. Moderate gas is present within normal-caliber while preventing circulatory collapse.
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Chapter 43 Infant with Neurologic Changes and Respiratory Failure 185

What are meningeal signs? setting of acute neurologic decline, his trachea is orally
intubated for airway protection. He is transported to the
Meningeal signs are clinical findings which help the prac-
PICU for further management of his acute neurologic
titioner assess for meningeal inflammation as a sign of
decline with hypoxic respiratory failure.
meningitis. Meningeal clinical signs include neck pain/
Focused neurologic examination produces the following
stiffness, presence of Kernig and/or Brudzinski sign, and
results. His eyes are closed and he does not respond to voice
tripod phenomenon. The Kernig sign evaluates flexion con-
or tactile stimuli other than with triple flection to noxious
tracture in the knees, with inability to flex further than
stimuli in the right lower extremity. His pupils are 1 mm,
135 . To perform the Kernig maneuver, the patient is placed
round and minimally responsive to light. Oculocephalic
in the supine position with hips flexed. Examiner will
reflex not clearly present. He has symmetric facial move-
slowly extend one knee at a time. If the patient experiences
ment with a midline tongue. His four limbs are flaccid. He
pain or spasms with the popliteal angle less than 135 , it is
has no response to noxious stimuli in bilateral upper extre-
considered positive for meningeal irritation. When per-
mities or left lower extremity. He has a 1+ deep tendon reflex
forming the Brudzinski test, the examiner places one hand
(DTR) at the bilateral brachioradialis. Trace DTR at the
on the patient’s chest and the other behind the head while
patella and achilles bilaterally. No clonus. His toes are mute
the patient is in the supine position. When passive flexion
on the left, downgoing on the right with plantar stimulation.
of the neck is performed, there is an involuntary flexion of
the hips and knees. The tripod sign is a different from the 3 What diagnostic studies could be performed to help nar-
tripod position clinicians may associate with asthma. When row the differential diagnosis and determine plan
the clinician observes the patient attempting to sit up, they of care?
will use the support of their hands behind them. This posi- 4 What is the indication for tracheal intubation?
tion allows the weight to be taken off the spine, and prevent 5 What is the triple flexion reflex?
flexion. This maneuver can only be completed in patients
Continuation of case: After 24 hours in the PICU, he
who are old enough to support themselves upright.
develops decreased lung compliance and worsening respi-
There are situations when these maneuvers will not be
ratory examination, likely due to aspiration pneumonia.
present, or are not clinically capable of being performed.
The infant is placed on minimal conventional ventilator
In these situations, clinical findings for meningitis in
settings with a dexmedetomidine infusion for sedation
infants would include lethargy, a full fontanelle, marked
and analgesia. Antibiotic coverage is broadened to cover
irritability with a variable fever, poor feeding, jaundice
for sepsis, aspiration pneumonia, and viral encephalitis.
and decrease in muscle tone. In older children, reported
A CT scan of the head (Figures 43.1–43.3) and lumbar
findings can include photophobia, frontal headaches,
puncture (Table 43.4) are completed. CT evaluation of
myalgia, anorexia, ataxia, and confusion. If the older child
the head is unremarkable, with no convincing evidence
exhibits an increase in intracranial pressure, bradycardia,
of parenchymal or extraparenchymal hemorrhage noted.
hypertension and altered mental status may also be
Focal area of increased attenuation seen over the left pari-
present.
etal convexity appears to be artifactual.
Continuation of case: During the first day of his inpatient
stay, he continues to be febrile and tachycardic, responsive
to antipyretics. He continues to favor his left side and does
not rotate his head to the right. He is irritable with poor oral
intake, but is soothed when held by parents. During his
afternoon feed, he starts to cough which is followed by a
large emesis. An hour following the emesis, the nurse is
called to the room to find patient with retractions, tachyp-
nea, and grunting. A provider is called to bedside, pulse
oximetry placed, and suction attempted. Oxygen saturation
readings are in the 70s, respiratory rate in the 70s, and he is
pale and mottled with weak thready pulses. The rapid
response team is called to evaluate the patient.
The rapid response team arrive and initiate bag mask
ventilation. The infant is not fighting against the positive
pressure. A peripheral intravenous line is placed and he
does not withdraw to the pain when it is inserted. In the Figure 43.1 CT scan of brain on admission to PICU.
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186 Cases in Pediatric Acute Care

Table 43.4 Lumbar puncture.

Appearance Clear
Color Colorless
Xanthochromia Absent
Red blood cell count 9/mm3
Leukocytes 108/mm3
Protein 21 mg/dL
Glucose 101 mg/dL
Herpes simplex virus Negative

consistent with mild encephalopathy. No patterns with spe-

cific correlation with seizure were present, and no focal
abnormalities were found.
Magnetic resonance imaging (MRI) of the brain is
completed due to progression of neurologic symptoms.
Findings on MRI suggest rhombencephalitis (likely
viral) due to bilateral symmetric signal abnormalities
involving the brainstem and extending to the cervical
cord. Additionally, there is high suspicion for acute flac-
Figure 43.2 CT scan of brain on admission to PICU. cid myelitis due to his MRI and clinical findings (see
Figures 43.4 and 43.5).

What other diagnostic studies could be performed

to help differentiate and determine plan of care?
At this point in the case, there are only a few diagnostic
studies he has yet to complete. He will require a chest radi-
ograph to evaluate for an aspiration pneumonia in the set-
ting of his acute decline during a choking episode. A CT of

Figure 43.3 CT Scan of brain on admission to PICU.

An electroencephalogram (EEG) is placed for continued

evaluation of brain activity. This 2-day EEG recording
showed an abnormal awake and asleep pattern. The diffuse
background slowing and disorganization was initially con-
sistent with moderate encephalopathy, but improved to Figure 43.4 MRI and magnetic resonance venogram (MRV) of
demonstrate state cycling and sleep architecture, more brain and spine: completed day 2 of admission while in PICU.

Figure 43.5 MRI/MRV of brain and spine: completed day 2 of
admission while in PICU.
the head and a lumbar puncture are indicated in the setting
of acute neurologic decline. However, prior to the lumbar
puncture, a CT scan assists in determining acute risk for
herniation. A lumbar puncture can be performed to evalu-
ate the cerebrospinal fluid (CSF) for infections, including,
but not limited to, herpes simplex virus, arbovirus, rabies,
West Nile virus, varicella zoster, and cytomegalovirus. Pleo-
cytosis is supportive but not necessarily a diagnostic crite-
rion for encephalitis, as it is only 50% sensitive. CSF glucose
less than 40 mg/dL and protein above 100–500 mg/dL is
indicative of bacterial meningitis. Predominant lympho-
cytes in the CSF are indicative of tuberculous and viral
meningitis, while predominant neutrophils are more indic-
ative of bacterial meningitis.
What is the indication for tracheal intubation?
The deteriorating neurologic examination is the indication
for endotracheal intubation. Pediatric patients can quickly
become unable to protect their airway due to loss of pharyn-
geal reflexes, significantly increasing the risk of aspiration.
A poor respiratory effort can lead to hypoventilation, hyper-
carbia, hypoxia, and further progress to a respiratory arrest.
What is the triple flexion reflex?
Triple flexion is a spasm in which the thigh flexes towards
the pelvis, the calf to the thigh, and the foot to the calf. This
movement is a spinal reflex, which may be present as a
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Chapter 43 Infant with Neurologic Changes and Respiratory Failure 187
response without brain death. When triple flexion is noted
on examination, further imaging with flow studies, func-
tional imaging or electrophysiologic examinations are
indicated.
6 What is IVIG? Why would you use it?
7 What is rhombencephalitis? What is the most com-
mon cause?
8 What is acute flaccid myelitis?
9 What is the difference between rhombencephalitis and
encephalitis?
What is IVIG? Why would you use it?
IVIG is a blood product, collected from 1000–1500 donors.
The mechanism of action is not well understood, and is
dose dependent. Dosing is based on treatment recommen-
dations and can be adjusted based on clinical outcome. In
this case, the dose was 2 g/kg over 2 days (high dose). Mon-
itoring immunoglobulins (IgG, IgM, IgE) can help deter-
mine appropriate timing of additional dosing of IVIG.
IVIG can be used in a wide range of neurologic, immuno-
logic, and hematologic conditions. It was initially studied in
the 1980s for treatment of immune thrombocytopenia. Later
research was expanded to autoimmune and inflammatory
conditions. IVIG can reduce the number of circulating neu-
trophils and leukocytes, and generally protect against inflam-
matory tissue damage. Literature is available describing
dosing, outcomes, and overall benefit with use of IVIG and
encephalitis, but large-scale studies have not been completed.
What is rhombencephalitis? What is the most
common cause?
Rhombencephalitis is inflammation of the hindbrain
(brainstem and cerebellum). The terms “brainstem enceph-
alitis” and “rhombencephalitis” are synonymous. Causes
include infectious, autoimmune etiology, or paraneoplastic
syndromes. The most common infectious agents are Liste-
ria, enterovirus 71, and herpes simplex virus. Behçet’s dis-
ease, systemic lupus erythematosus, and relapsing
polychondritis have also been documented to cause rhom-
bencephalitis. MRI is the gold standard for evaluation. The
most notable abnormal hyperintense areas on MRI that
indicate viral encephalitis are seen in T2-weighted
imaging and fluid-attenuated inversion recovery pulse
image (FLAIR) sequences. Some studies suggest using
diffusion-weighted imaging during MRI as it could be used
as a predictor of outcome. A CT is often done early in the
course of illness to evaluate the etiology of mental status
changes, but it often cannot capture changes associated
with rhomboencephalitis.
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188 Cases in Pediatric Acute Care

What is acute flaccid myelitis?

Acute flaccid myelitis is a condition of the nervous system
that primarily affects the gray matter, leading to weakness
of the muscle and reflexes. Symptoms are consistent with
sudden weakness of the limbs, difficulty moving eyes or eye-
lids, difficulty swallowing or speaking, and a facial droop. It
has historically been associated with poliovirus, but now is
seen in association with non-polioviruses such as enterovi-
rus 68 and 71, coxsackievirus, adenovirus, and West Nile
virus. Supportive care is the best treatment, with aggressive
physical and occupational therapy to maximize function.
Most children with acute flaccid myelitis display residual
motor deficits 12 months following initial diagnosis.

What is the difference between rhombencephalitis

and encephalitis?
Figure 43.7 MRI of brain and spine 10 days following prior study.
Common terms used for encephalitis are not interchange-
able. Encephalitis is defined as altered level of conscious-
ness lasting for 24 hours or more, plus two or more of
the following: fever (temperature >38 C), seizures (gener-
alized or partial) or abnormality on EEG, pleocytosis (white
blood cells in CSF ≥5/mm3), neuroimaging consistent with
encephalitis, or detection of autoantibodies in CSF
(Figure 43.6). When the criteria for encephalitis are not
met due to lack of inflammatory criteria (e.g. absence of
fever, CSF pleocytosis, and/or imaging changes) and with
altered mental status lasting more than 24 hours, it is
defined as encephalopathy. Both encephalitis and

Brain Parenchymal
Neurologic Dysfunction + Inflammation

Encephalopathy:
altered mental
status >24 h
Major
• EEG c/w + • Fever
Encephalitis
Minor Figure 43.8 MRI of brain and spine 10 days following prior study.
2: possible • CSF pleocytosis
• Seizures
≥3: probable
• Neuroimaging c/w
• Focal neurologic brain inflammation
findings encephalopathy include all structures of the brain paren-
chyma, while rhombencephalitis is specific inflammation
of the hindbrain structures only.

Encephalitis
Case Resolution
Figure 43.6 Overview of diagnostic criteria for encephalitis.
Source: Messacar, K., Fischer, M., Dominguez, S., Tyler, K.L., and
Abzug, M.J. (2018). Encephalitis in US children. Infectious Disease
High-dose steroids and intravenous immunoglobulin
Clinics of North America 32(1): 145–162. Reproduced with (IVIG) are administered without improvement. He receives
permission of Elsevier. multiple rounds of antibiotics. Twenty days after initial

admission, subtype of enterovirus resulted as D71. Addi-
tionally, the pulmonary and stable ventilator teams are con-
sulted due to prolonged intubation course and poor
respiratory drive. Additional neuroimaging with repeat
MRI of the brain is completed due to lack of clinical
improvement despite aggressive care and interventions
(see Figures 43.7 and 43.8). In comparison with the previ-
ous brain MRI, there is significant improvement in the dif-
fusion signal involving the brainstem, median thalami, and
upper cervical spinal cord.
Further Reading
Campos, L., Trindade, R., Faistauer, Â., et al. (2016).
Rhombencephalitis: pictorial essay. Radiologia Brasileira
49(5): 329–336. doi: 10.1590/0100-3984.2015.0189.
Centers for Disease Control and Prevention (2019). Acute
flaccid myelitis. http://www.cdc.gov/features/acute-flaccid-
myelitis/ (accessed 31 March 2019).
Gogou, M., Papadopoulou-Alataki, E., Spilioti, M., Alataki, S.,
and Evangeliou, A. (2017). Clinical applications of
intravenous immunoglobulins in child neurology. Current
Pharmaceutical Biotechnology 18(8): 628–637. doi: 10.2174/
1389201018666170915123700
Graeter, S., Simon, H., and von Gunten, S. (2019). Granulocyte
death mediated by specific antibodies in intravenous
immunoglobulin (IVIG). Pharmacological Research 2019:
104168. doi: 10.1016/j.phrs.2019.02.007.
Hopkins, S. (2017). Acute flaccid myelitis: etiologic challenges,
diagnostic and management considerations. Current
Treatment Options in Neurology 19(12): 48. doi: 10.1007/
s11940-017-0480-3.
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Chapter 43 Infant with Neurologic Changes and Respiratory Failure 189
The patient did not regain neurologic function following
his course in the PICU. After multiple attempts to wean
ventilatory support, he underwent a tracheostomy place-
ment on day 27 of admission. He required a ventilator for
respiratory support, and a nasogastric feeding tube for
nutrition. He regained only small movements of his fingers
and toes on the right side of his body, and no spontaneous
large motor movements. He could not track past midline,
and still favored head position to the left. He continued
with physical therapy to facilitate maximal recovery.
Iro, M., Martin, N., Absoud, M., and Pollard, A.J. (2017).
Intravenous immunoglobulin for the treatment of childhood
encephalitis. Cochrane Database of Systematic Reviews (10):
CD011367. doi: 10.1002/14651858.
Messacar, K., Fischer, M., Dominguez, S., Tyler, K.L., and
Abzug, M.J. (2018). Encephalitis in US children. Infectious
Disease Clinics of North America 32(1): 145–162. doi:
10.1016/j.idc.2017.10.007.
Reuter-Rice, K. and Bolick, B. (2012). Pediatric Acute Care:
A Guide to Interprofessional Practice. Burlington, MA:
Jones & Bartlett Learning LLC.
Shaffner, D. and Nichols, D. (2016). Rogers’ Textbook of
Pediatric Intensive Care, 5th edn. Philadelphia, PA: Wolters
Kluwer.
Venkatesan, A. and Geocadin, R. (2014). Diagnosis and
management of acute encephalitis: a practical approach.
Neurology: Clinical Practice 4(3): 206–215. doi: 10.1212/
CPJ.0000000000000036.

44
Acute Hyperglycemia
Jeanne Little
Rush University College of Nursing, Chicago, IL, USA
An 8-year-old female with no significant past medical
history presents to the emergency department (ED) with
lethargy, new abdominal pain with nausea and vomiting.
History of Present Illness
This 8-year-old female presented to her primary care pro-
vider (PCP) 1 day ago with nausea and vomiting, without
diarrhea for 3 days. She has had a 1.8 kg weight loss since
her last well child visit, which was 2 months ago. General-
ized abdominal discomfort started today so she was brought
to the ED. The child’s appetite has been worse over the past
month, and she admits to drinking more fluids. Her abdom-
inal pain is a constant dull ache and nothing makes it worse
or better. The child reports using the bathroom more at night
and she wet the bed last week so sleep has been disrupted.
There are no known sick contacts and the patient is attend-
ing some summer day camps where she felt fatigued.
Past Medical History
The child was born full-term and went home with mother.
She has had all recommended immunizations including the
annual influenza vaccine. She has no previous hospitaliza-
tions or surgeries, does not take any medications, and has
no known allergies. Her stature and weight are both in the
50% band for age and gender. Her current weight is 22 kg,
height 119 cm, and body mass index (BMI) 15.5.
Family History
Mother has a history of hypothyroidism, but it is well
controlled. Her father is on medication for hypertension.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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191
A 5-year-old brother is healthy. Paternal grandmother
has adult-onset diabetes.
Current Status
On arrival to the ED, the child appears dehydrated on
examination with dry buccal mucosa and lips, capillary
refill of 3 seconds, tenting skin turgor, and slightly dimin-
ished pedal and radial pulses, with cool extremities. She is
drowsy but opens her eyes when spoken to, and has a Glas-
gow Coma Scale score of 14. Vital signs are as follows: tem-
perature 37.1 C (98.7 F), heart rate 125 bpm, blood
pressure 112/65 mmHg, respiratory rate 26 breaths per
minute, oxygen saturation 96% on room air, and general-
ized abdominal pain 4/10. She has no retractions or signs
of increased work of breathing. No localized abdominal
tenderness during palpation. A peripheral intravenous
catheter is inserted. A point-of-care test (POCT) glucose
is 482 mg/dL. Serum basic metabolic profile (BMP), venous
blood gas analysis, complete blood count, and urine
ketones are also obtained (see Tables 44.1–44.4).
Questions
Answer the following questions using the details provided.
1 Based on the data, what are the differential diagnoses
and the most likely diagnosis for this child?
2 What is the best way to treat dehydration, acidosis, and
hyperglycemia and how would these things be ordered?
3 Which monitoring parameters and laboratory studies
would you follow, how often, and why?
Continuation of case: The patient is admitted to the
pediatric intensive care unit (PICU) and receives an initial
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192 Cases in Pediatric Acute Care

Table 44.1 Basic metabolic panel. 4 If the patient develops a headache during the treatment,
what is the most important treatment?
Sodium 134 mEq/L
5 What indicators are used to transfer from intravenous
Potassium 3.7 mEq/L insulin to subcutaneous administration?
Chloride 101 mEq/L
Carbon dioxide 12 mEq/L
Blood urea nitrogen 24 mg/dL
Creatinine 0.9 mg/dL
Rationale and Evidence-based Practice
Glucose >511 mg/dL
Explanation
Calcium 9.0 mg/dL
– –
Based on the data, what are the differential
Anion gap (Na + K ) – (Cl + HCO3 )
+ +
25 mEq/L diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include insulin-
Table 44.2 Venous blood gas analysis.
dependent diabetes mellitus (IDDM or type 1 diabetes) with
pH 7.18 diabetic ketoacidosis (DKA), type 2 diabetes under extreme
PCO2 35 mmHg
stress (infection, trauma), hyperglycemic hyperosmolar
state, and other causes of high anion gap acidosis such as
PO2 38 mmHg
ingestion of salicylate, methanol, and ethylene glycol. Gas-
Bicarbonate 13 mEq/L
troenteritis, which can precipitate DKA, may also be
Base excess –8 considered.
The most likely diagnosis for this child is new-onset
IDDM with DKA. This child presents with classic symp-
Table 44.3 Complete blood count. toms of polyuria, nocturia, enuresis, polydipsia, and weight
loss. Abdominal discomfort and vomiting are also common.
White blood cell count 14 000/mm3 IDDM results from immune-mediated pancreatic beta-cell
Hemoglobin 14 g/dL destruction leading to an absolute deficiency in insulin
Hematocrit 45% secretion. Insulin is required in the metabolism of glucose
Platelet count 232 000/mm3 for energy. Without insulin, glucose levels rise and the body
shifts to a catabolic state by breaking down glycogen stores,
using triglycerides from adipose tissues, and using amino
Table 44.4 Urinalysis. acids from muscle for energy. The cause of IDDM is
thought to be multifactorial, but the exact roles of genetic
Nitrites Negative susceptibility, environmental factors, and the immune sys-
Blood Negative tem are unclear. Without prompt diagnosis, IDDM results
Ketones Moderate in weight loss, persistent hyperglycemia, dehydration from
Leukocytes Negative
osmotic diuresis/glycosuria, ketone body production, and
acidosis. The biochemical criteria for the diagnosis of
Glucose 3+
DKA are (i) hyperglycemia (blood glucose >200 mg/dL);
Pregnancy test Negative
(ii) venous pH <7.3 or serum bicarbonate <15 mmol/L;
and (iii) ketonemia or ketonuria.

fluid bolus of 0.9% normal saline 20 mL/kg over 1 hour.
Replacement fluids of 0.9% normal saline with 20 mmol/L
potassium acetate and 20 mmol/L potassium phosphate are
initiated to account for dehydration (10%) and daily main-
tenance fluids. One hour after fluid replacement therapy
has started a continuous intravenous insulin infusion is
started at 0.1 unit/kg per hour. A second peripheral intra-
venous line is placed. After 1 hour on continuous insulin, a
repeat POCT glucose is 390 mg/dL.
What is the best way to treat dehydration, acidosis,
and hyperglycemia and how would these things be
ordered?
Goals of therapy for IDDM with DKA are to improve cir-
culatory volume and tissue perfusion, correct electrolyte
derangements, reverse acidosis and ketosis, gradually
reduce serum glucose and osmolality to near normal,
and to prevent and monitor for DKA complications.
Calculations for fluid deficit can be completed with

knowledge of previous weight or using the percentage of
dehydration along with the child’s weight. A fluid deficit
of 10–15% of body weight is usually present in patients
with DKA and should be replaced along with mainte-
nance intravenous fluids over 24–48 hours (subtract
fluid boluses). Volume expansion begins with isotonic
fluids 20 mL/kg. Normal saline or 0.45% fluids with
potassium (with chloride, acetate, or phosphate) are
preferred for maintenance fluids. There are varied
recommendations in the literature for replacement intra-
venous fluids, but the overall goal is to provide glucose
and electrolytes, especially to replace potassium which
moves intracellularly as insulin treatment is initiated.
Similarly, phosphorus also moves intracellularly with
insulin treatment, but there is not as much concern as
replacement of potassium. Using fluids with higher
concentrations of sodium chloride, as normal saline,
can assist in preventing cerebral edema. A 3% saline
can also be used in the treatment of DKA when a patient
has neurologic complications such as seizures, related to
cerebral edema. A continuous intravenous infusion of
insulin 0.1 unit/kg per hour should be started as soon
as possible along with intravenous fluids. Fluids and
insulin reverse acidosis by allowing metabolism of
ketoacids, although replacement of insulin is most
important. Bicarbonate administration is not routinely
recommended except in cases of extreme hyperkalemia
or pH less than 6.85. A large systematic review indicated
that sodium bicarbonate use in patients with DKA
increases the risk of cerebral edema and prolongs acido-
sis and overall treatment (Chua et al., 2011).
An aggressive decrease in plasma glucose, greater than
90 mg/dL per hour, can lead to hypoglycemia and rapid
osmolality changes. When serum glucose is below
300 mg/dL or blood glucose falls more than 90 mg/dL
per hour, dextrose (5–12.5%) is added to fluids. Insulin rates
may be decreased to 0.05 unit/kg per hour to prevent rapid
glucose decline, but treatment relies on insulin, so this
should not be a first choice.
Which monitoring parameters and laboratory
studies would you follow, how often, and why?
Vital signs and neurologic checks should be monitored
hourly as well as POCT glucose. It is recommended that
serum electrolytes, or BMP and venous blood gases are
checked every 2 hours. A POCT glucose and if available a
blood β-hydroxybutyrate (BOHB) concentration should
be measured immediately; a BOHB level of 3 mmol/L or
more indicates serum ketosis. Frequent neurologic checks
are necessary to evaluate for signs of cerebral edema. Tach-
ycardia usually resolves with rehydration.
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Chapter 44 Acute Hyperglycemia 193
Initial serum sodium may appear low because hypergly-
cemia causes osmosis of water into the extracellular space
leading to dilutional hyponatremia. Corrected sodium
values can be calculated to estimate the expected
sodium if hyperglycemia was absent. After insulin and
fluids are administered, the serum sodium should gradu-
ally increase. Total body potassium deficits are often pres-
ent from acidosis, increased plasma osmolality, and
vomiting. The first potassium level may appear normal,
but insulin administration and acidosis correction moves
potassium intracellularly, resulting in decreased serum
potassium. Blood urea nitrogen may appear high from
dehydration. Hypophosphatemia, hypomagnesemia, and
hypocalcemia are also complications of DKA and should
be monitored closely. Hemoglobin A1c levels are usually
obtained to provide information regarding the duration
of hyperglycemia.
If the patient develops a headache during the
treatment, what is the most important treatment?
A new headache during treatment or a worsening headache
is concerning for cerebral edema. Other findings include
inappropriate slowing of heart rate or rising blood pressure,
vomiting recurrence, and change in mental status (irritabil-
ity, increased drowsiness). If cerebral edema is suspected,
immediately administer 3% saline, or intravenous mannitol
0.5–1 g/kg over 10–15 minutes. Prevent hypotension to
maintain cerebral perfusion pressure, but avoid excessive
fluid administration that might lead to worsening cerebral
edema.
What indicators are used to transfer from
intravenous insulin to subcutaneous
administration?
Transitioning to subcutaneous insulin occurs when the
metabolic acidosis has improved with evidence of a normal-
ized anion gap. Venous pH (>7.3), normal anion gap, bicar-
bonate (>15 mmol/L), and BOHB (<1 mmol/L) are used to
determine DKA resolution. Ketonuria often continues for
several hours after blood ketone levels have normalized
so is not a good indicator of DKA recovery. The ideal time
to convert to subcutaneous insulin is just before a meal.
A dose of basal subcutaneous insulin (long- or intermedi-
ate-acting) can be administered hours before intravenous
insulin is stopped or at the time the patient typically
receives it. Rapid-acting insulin, given for meal coverage,
should be administered 15–30 minutes before stopping
the insulin infusion and just before eating. Endocrinology
specialists can help guide this conversion.

194 Cases in Pediatric Acute Care
Reference
Chua, H.R., Schneider, A., and Bellomo, R. (2011).
Bicarbonate in diabetic ketoacidosis: a systematic review.
Further Reading
Mayer-Davis, E., Kahkoska, A., Jefferies, C., et al. (2018).
ISPAD Clinical Practice Consensus Guidelines 2018:
definition, epidemiology, and classification of diabetes in
children and adolescents. Pediatric Diabetes 19(Suppl 27):
7–19. doi: 10.1111/pedi.12773.
Von Oettingen, J., Rhodes, E.T., and Wolfsdorf, J. (2018).
Resolution of ketoacidosis in children with new onset
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Annals of Intensive Care 1(1): 23. doi: 10.1186/2110-
5820-1-23.
diabetes: evaluation of various definitions. Diabetes Research
and Clinical Practice 135: 76–84. doi: 10.1016/j.
diabres.2017.09.011.
Wolfsdorf, J., Glaser, N., Agus, M., et al. (2018). ISPAD Clinical
Practice Consensus Guidelines 2018: diabetic ketoacidosis
and the hyperglycemic hyperosmolar state. Pediatric
Diabetes 19(Suppl 27): 155–176. doi: 10.1111/pedi.12701.

45
Infant with Progressive Lethargy and Poor Feeding
Kathleen P. Thompson1,2
1
Baylor College of Medicine, Houston, TX, USA
2
Pediatric Critical Care Medicine, Texas Children’s Hospital, Houston, TX, USA
A 4-month-old male presents to the emergency department
(ED) with progressive lethargy and poor feeding over the
past several days.
History of Present Illness
The parents report that over the last 2 days, they have had
to wake him from sleep so that he will eat. This morning, he
would not wake up and they noted that his breathing was
very shallow. He has also had rhinorrhea and cough at
night over the past week. His parents report approximately
one to two episodes of emesis each day for the past month.
There are no known sick contacts.
Past Medical History
The infant was born full-term and was initially healthy. He
has been noted to have poor weight gain over the last
month. At approximately 3 months of age, he transitioned
from expressed breast milk (EBM) to formula. Prior to the
start of formula, he would feed every 2–3 hours with
approximately 113–170 g of EBM. Since transitioning to for-
mula, he has been feeding approximately every 4–6 hours,
taking about 113 g per feed. He does not have any known
allergies and is not on any medications. He immunizations
are up to date.
Past Surgical History
Circumcision on second day of life without complications.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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195
Family History
Father has hypertension that is well controlled with medi-
cation. One male paternal cousin died unexpectedly at
6 months of age of an unknown cause approximately two
decades ago. No other significant family medical history.
Current Status
He is sleeping during the examination, although he easily
awakens and cries in response to tactile stimulation. His
vital signs are temperature 36.4 C (97.5 F), heart rate 180
bpm, blood pressure 90/50 mmHg, and respiratory rate
50 breaths per minute. His weight is 4.5 kg. Other examina-
tion findings are significant for a full fontanelle with dry
mucous membranes, and clear rhinorrhea. He is tachyp-
neic, but has comfortable work of breathing. He is tachycar-
dic with 2+ pulses and capillary refill time of 2–3 seconds.
No organomegaly. He moves all extremities spontaneously
and falls asleep easily at the end of the examination. Initial
laboratory studies are obtained in the ED. Results of basic
metabolic profile and arterial blood gas analysis are shown
in Tables 45.1 and 45.2. Complete blood count and chest
radiograph are unremarkable.
While in the ED, the patient develops apnea and requires
intubation and mechanical ventilation. Transport to a ter-
tiary children’s hospital is arranged and he is accepted by
the pediatric intensive care unit (PICU) for presumed men-
ingitis. While in the ED, he receives fluid resuscitation with
60 mL/kg of 0.9% normal saline for tachycardia and
dehydration. Blood, urine, and cerebrospinal fluid (CSF)
cultures are obtained and empiric broad-spectrum antimi-
crobials comprising ceftriaxone 50 mg/kg every 12 hours
and vancomycin 15 mg/kg every 6 hours are initiated.
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196 Cases in Pediatric Acute Care

Table 45.1 Basic metabolic profile. Table 45.4 Complete blood count and coagulation studies
in PICU.
Sodium 138 mEq/L
Potassium 4.0 mEq/L White blood cell count 12 100/mm3

Chloride 108 mEq/L Hemoglobin 9.1 g/dL

Carbon dioxide 20 mEq/L Hematocrit 28%

Glucose 115 mg/dL Platelet count 175 000/mm3

Differential Pending
Prothrombin time 13.0 s
Table 45.2 Arterial blood gas analysis. Partial thromboplastin time 34.5 s
International normalized ratio 1.3
pH 7.56
PaCO2 22 mmHg
PaO2 90 mmHg Table 45.5 Arterial blood gas analysis in PICU.
Bicarbonate 20 mmol/L
Lactate 2.7 mmol/L pH 7.40
PaCO2 35 mmHg
Pao2 93 mmHg
Bicarbonate 21 mmol/L
Table 45.3 Complete metabolic profile in PICU.
Lactate 1.5 mmol/L
Sodium 140 mEq/L
Potassium 4.3 mEq/L
Chloride 108 mEq/L
Table 45.6 Urinalysis in PICU.
Carbon dioxide 22 mEq/L
Blood urea nitrogen 15 mg/dL Color Yellow
Creatinine 0.5 mg/dL Clarity Clear
Glucose 115 mg/dL pH 5.0
Calcium 8.5 mg/dL Specific gravity 1.015
Magnesium 2.0 mg/dL Glucose Negative
Phosphorus 4.5 mg/dL Ketones Negative
Alanine aminotransferase 55 U/L Nitrites Negative
Aspartate aminotransferase 60 U/L Leukocyte esterase Negative
Alkaline phosphatase 160 U/L Bilirubin Negative
Gamma-glutamyltransferase 50 U/L
Albumin 3.0 g/dL
Conjugated bilirubin <0.35 mg/dL Repeat laboratory results in the PICU are shown in
Unconjugated bilirubin <1.0 mg/dL Tables 45.3–45.6. CSF analysis demonstrates no cells, and
normal protein and glucose. Remaining CSF Gram stain
Ammonia 500 μg/dL
and culture are pending.

In transport, he is placed on intravenous fluids comprising

20 mEq/L KCl in 10% dextrose and 0.9% normal saline at
1600 mL/m2 daily.
On arrival at the PICU, he has a full bulging fontanelle
with minimal movement of his extremities with tactile
stimulation. Sedation is not required for his invasive proce-
dures or diagnostic studies. Non-contrast computed tomog-
raphy (CT) of the brain is obtained after intubation at the
referring hospital and was unremarkable.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what is the differential
diagnosis?
2 Describe the pathophysiology that causes acute crises
secondary to inborn error of metabolism (IEM) to pres-
ent later than the newborn period.

3 What are some common presenting signs and symp-
toms of IEM listed by system?
4 Describe your diagnostic evaluation of a patient with
suspicion of IEM. What is your initial approach? What
additional studies may be warranted as you continue to
suspect this diagnosis?
5 What are some causes of anion gap metabolic acidosis?
How is it calculated?
6 Based on the initial laboratory screening, what three
major diagnostic categories may be identified in chil-
dren presenting with acute encephalopathy or acute
metabolic emergencies due to an IEM?
7 What is your initial management of the patient with
suspected acute metabolic encephalopathy due to
an IEM?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what is the differential
diagnosis?
A number of diagnoses may be contributing to this patient’s
presentation and acute deterioration. Some differential
diagnoses to consider include sepsis, central nervous sys-
tem infection, acute liver failure, toxic ingestion, IEM with
hyperammonemia, or child maltreatment.
Although it is difficult to differentiate between these
diagnoses, it is important to include IEM on the differential
diagnosis any critically ill neonate or child with abnormal
unexplained laboratory results and unknown etiology of ill-
ness. The clinical suspicion for a possible IEM should be
heightened if there is a family history of death during
infancy, as noted in this case.
Describe the pathophysiology that causes acute
crises secondary to IEM to present later than the
newborn period
Children with inherited metabolic disorders almost always
appear normal at birth. This is because the small metabolic
intermediate that is responsible for the disorder is easily fil-
tered by the placenta and the maternal metabolism acts as
an effective dialyzer of fetal metabolic byproducts.
A metabolic crisis occurs when there is a build-up of toxic
metabolites. An acute metabolic decompensation is usually
triggered by factors that cause increased catabolism or
increased consumption of protein. The duration of the
symptom-free period and timing of presentation may be
anywhere from hours to months, or possibly even years.
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Chapter 45 Infant with Progressive Lethargy and Poor Feeding 197
What are some common presenting signs and
symptoms of IEM listed by system?
•
Constitutional: dysmorphic features; failure to thrive;
unusual odor of urine, breath, cerumen, or other
body fluid.
•
Respiratory: tachypnea without pulmonary pathol-
ogy, apnea.
•
Cardiac: congestive heart failure, cardiomegaly, ischemic
heart disease.
•
Neurologic: hypotonia, lethargy, encephalopathy, sei-
zures, inconsolability, change in mental status, unex-
plained developmental delay or regression of
developmental milestones.
•
Gastrointestinal: jaundice, diarrhea, vomiting, abdomi-
nal pain.
Acute presentation with multisystem involvement is typical
and strongly suggestive of a possible IEM. The onset and
progression of symptomology and severity of presentation
are all important clues that point to an acute metabolic
and encephalopathic IEM.
Describe your diagnostic evaluation of a patient
with suspicion of IEM. What is your initial
approach? What additional studies may be
warranted as you continue to suspect this
diagnosis?
The initial evaluation of a child with suspected IEM should
include laboratory evaluation of glucose, serum electro-
lytes, complete blood count with differential, lactate, pH,
ammonia, liver function, clotting studies, venous blood
gas, and urinalysis. As these patients often present with
vague symptoms that may be consistent with overwhelm-
ing illness, an infectious work-up should also be completed
in a timely manner. The infectious evaluation is also impor-
tant given increased risk for infection in an IEM and the
potential of infection to cause increased catabolism and
emergent presentation.
If the initial screening reveals an elevated ammonia,
anion gap metabolic acidosis, or hypoglycemia, consulta-
tion with genetics is warranted and further confirmatory
studies should be sent with their collaboration. In some
cases, initial screening results are unremarkable due to
the interventions that are performed during resuscitation.
In these cases, additional laboratory studies may be war-
ranted to further evaluate the possible diagnosis of IEM.
Finally, IEM should continue to be evaluated in the setting
of a patient who fails to respond to standard therapy. Addi-
tional diagnostic studies may include plasma and urine
amino acids, urine organic acids, lactate, pyruvate, plasma
acylcarnitine profile, and carnitine analysis.

198 Cases in Pediatric Acute Care
What are some causes of anion gap metabolic
acidosis? How is it calculated?
In the setting of metabolic acidosis, the anion gap should be
calculated as follows:
Anion gap = Na + + K + – Cl – + HCO3 –
Normal anion gap is 8–16 mEq/L. There are several etiol-
ogies that may cause an anion gap metabolic acidosis. An
acronym to aid in the evaluation of anion gap acidosis is
MUDPILES: methanol, uremia, diabetic ketoacidosis, par-
aldehyde, intoxication, lactic acidosis, ethylene glycol, and
salicylates.
Based on the initial laboratory screening, what
three major diagnostic categories may be
identified in children presenting with acute
encephalopathy or acute metabolic emergencies
due to an IEM?
Three major diagnostic categories include hyperammone-
mia, anion gap metabolic acidosis, and hypoglycemia.
One of the most important laboratory findings when an
IEM is suspected is hyperammonemia; levels of ammonia
greater than 200 μmol/L should heighten concern for an
IEM. The second most important laboratory feature is met-
abolic acidosis with an increased anion gap; this is a prom-
inent finding in glycogen storage diseases, pyruvate
metabolism defects, and organic acid disorders, among
others. The presence of hypoglycemia can assist in differen-
tiation of metabolic disorders, especially when considered
with other findings such as hepatomegaly, acidosis, and
the presence or absence of ketosis.
What is your initial management of the patient
with suspected acute metabolic encephalopathy
due to an IEM?
Patients with an IEM require immediate management in an
effort to prevent further deterioration and reduce neurologic
Further Reading
MacNeill, E.C. and Walker, C.P. (2018). Inborn errors of
metabolism in the emergency department (undiagnosed and
management of the known). Emergency Medicine Clinics 36
(2): 369–385.
Rezvani, I. and Rezvani, G.A. (2016). An approach to inborn
errors of metabolism. In: Nelson’s Textbook of Pediatrics,
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
sequalae. Life-saving treatment is often initiated prior to con-
firmation of the diagnosis, and largely consists of supportive
care standard in the PICU. Ventilatory support should be
initiated and managed according to the etiology of the
respiratory failure; current recommendations for acute
respiratory distress syndrome (ARDS), cerebral edema,
and other causes should be considered. Fluid resuscitation
with 0.9% sodium chloride should be administered to main-
tain adequate perfusion and circulation; because of the risk
of increased lactic acidosis, lactated Ringer’s solution should
be avoided, and hypotonic fluids are discouraged due to the
risk of cerebral edema. Management of cerebral edema and
neuroprotective measures should follow evidence-based
guidelines.
Hypoglycemia should be corrected immediately. If possi-
ble, it is preferable to obtain blood samples prior to initia-
tion of therapy. Acidosis may be corrected with careful
administration of bicarbonate, but this must be done cau-
tiously to avoid the risk of fluid shifts and cerebral edema.
Hyperammonemia may be addressed with emergent initia-
tion of hemodialysis. Additional interventions to reduce
ammonia in the absence of metabolic acidosis may include
intravenous dextrose and lipid emulsion to provide protein-
free energy, arginine hydrochloride with sodium phenyla-
cetate, and sodium benzoate. Antimicrobial therapy should
be initiated due to the potential for concurrent serious
infection.
Protein intake should be discontinued immediately if a
urea cycle defect or protein intolerance is suspected in
the setting of organic academia. Catabolism can be pre-
vented through liberal administration of intravenous dex-
trose, sometimes requiring concurrent administration of
insulin. Intravenous intralipid may also be administered
to meet caloric requirements during physiologic stress.
With the support of genetics consultation, cofactors may
be initiated while awaiting confirmation of a diagnosis.
This presumptive cocktail may include vitamin B12, thia-
mine, biotin, riboflavin, folic acid, and carnitine.
20th edn (ed. R.M. Kliegman, B.F. Stanton, J.W. St Geme,
N.F. Schor and R.E. Behrman), 634–636. Philadelphia, PA:
Elsevier.
Rice, G.M. and Steiner, R.D. (2016). Inborn errors of
metabolism (metabolic disorders). Pediatrics in Review 37(1):
3–15.

46
Neonatal Tachypnea
Janet Dutcher
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A newborn male is admitted to the neonatal intensive care
unit (NICU) at 2 hours of life for respiratory distress.
History of Present Illness
Baby was born at 39 weeks’ gestation to a 30-year-old grav-
ida 2 woman via planned, repeat cesarean section. The
delivery was uneventful, and the infant cried immediately
after birth. He received no advanced resuscitation and was
assigned Apgar scores of 8 and 9 at 1 and 5 minutes of life,
respectively. The newborn was noted to have intermittent
grunting in the delivery room. Pulse oximetry was
90–92%, so he went to recovery with his mother where
he continued to receive routine care and monitoring. How-
ever, at approximately 2 hours of life the pediatric team was
called to evaluate for signs of increasing respiratory
distress, including tachypnea (respiratory rate 84 breaths
per minute), retractions, and supplemental oxygen require-
ment to maintain oxygen saturations (SpO2) greater than
90%. At that time the newborn was moved to the NICU
for evaluation and admission.
Past Medical History
Prenatal laboratory results are as follows: blood type A+,
antibody negative, rubella immune, syphilis negative, hep-
atitis B negative, HIV negative, gonorrhea negative, chla-
mydia negative, group B β-hemolytic Streptococcus (GBS)
untested. Glucose tolerance test was abnormal at 1 hour,
but normal at 3 hours. Mother has a history of previous
cesarean section and asthma with occasional albuterol neb-
ulizer use; however, the antenatal period during this preg-
nancy was otherwise uncomplicated. There was no
apparent exposure to tobacco, alcohol, or drugs during
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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199
pregnancy. Fetal ultrasonography at 20 weeks revealed
normal anatomy.
Family History
Four-year-old sister has asthma. There is no family history
of genetic or cardiac disorders. Both parents are healthy.
Current Status
On admission to the NICU the newborn is placed on an
open radiant warmer and attached to a cardiopulmonary
monitor and pulse oximeter. Pertinent findings on physical
examination include mildly diminished breath sounds
bilaterally, mild intermittent subcostal retractions, tachyp-
nea, no murmur, and mildly decreased tone. Temperature,
heart rate, and four-extremity blood pressures are all nor-
mal for gestational age. He is treated with humidified
high-flow nasal cannula at 2 L/min FiO2 30%, targeting oxy-
gen saturation of 94–96%. At 4 hours of life, pre-ductal
(right hand) and post-ductal (left foot) were 96% and
92%, respectively.
An arterial blood gas analysis, complete blood count
(CBC), blood culture, and blood glucose are completed
via radial arterial stick. Empirical antibiotics are ordered
including ampicillin 100 mg/kg per dose every 12 hours
and gentamicin 4 mg/kg per dose every 24 hours.
A supine anteroposterior chest radiograph is obtained with
available reading reporting increased lung volumes with
flat diaphragm, increased vascular markings (perihilar
streaking), and fluid in the horizontal fissure.
Results for CBC and arterial blood gas analysis are shown
in Tables 46.1 and 46.2 and the chest radiograph in
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200 Cases in Pediatric Acute Care

Table 46.1 Complete blood count. 2 What results would you expect from a hyper-oxygen test
with this disorder? Would you consider doing any other
White blood cell count 9800/mm3
screening tests or diagnostics on the newborn? If yes,
Hemoglobin 17.9 g/dL what would you include?
Hematocrit 49.5% 3 What would be your management goals for the care of
Platelet count 241 000/mm3 this infant?
Neutrophils 56% 4 Are antibiotics indicated for this patient?
Lymphocytes 33% 5 Which of the following strategies, if any, have been
Monocytes 9%
shown to help in the management of the patient’s diag-
nosis: diuretic therapy, fluid restriction, and/or inhaled
salbutamol therapy?
6 While obtaining consent for NICU admission and
Table 46.2 Arterial blood gas analysis. updating the family on the newborn’s status and plan
of care, the mother wants to know what “causes” the
pH 7.30
condition and how long you expect it to last. How
PCO2 38 mmHg
would you respond?
PaO2 75 mmHg
Bicarbonate 22 mEq/L
Base excess –5 Rationale and Evidence-based
Practice Explanation
Based on the data provided, what is the most likely
diagnosis and differential diagnosis for this
newborn? What are the perinatal risk factors for
this diagnosis?
After ruling out all other pathologic causes of respiratory
distress, transient tachypnea of the newborn (TTN) would
be the most likely diagnosis for this infant. Since confirm-
atory diagnosis of TTN comes after symptoms are no longer
present, other conditions presenting with respiratory
distress in a term newborn shortly after birth would
include:

•• respiratory distress syndrome (RDS)

early-onset sepsis

•• congenital pneumonia
meconium aspiration syndrome (MAS)

Figure 46.1 Chest radiograph. Source: Courtesy of

•• congenital heart disease (CHD)
persistent pulmonary hypertension of the new-
born (PPHN)

D. Dirnberger, MD.
Figure 46.1. Other results show blood glucose 59 mg/dL,
while blood culture is pending.
Questions
Answer the following questions using the details provided.
1 Based on the data provided, what is the most likely diag-
nosis and differential diagnosis for this newborn? What
are the perinatal risk factors for this diagnosis?
••
congenital metabolic acidosis
pneumothorax.
TTN is the result of residual fluid in a newborn’s lungs and
is the most common cause of respiratory distress in the
immediate newborn period. The maternal and birth history,
clinical presentation, physical examination, and chest radi-
ograph findings for the newborn support the diagnosis of
TTN. Delivery by cesarean section, male sex, and family his-
tory of asthma are perinatal risk factors for TTN. The deliv-
ery was uncomplicated with no noted intrauterine distress
or perinatal asphyxia, clear amniotic fluid, and blood gas

analysis with no evidence of significant acidosis (which is
more common in conditions such as MAS, RDS, and PPHN).
Since the patient was delivered close to term, he was also at
less risk for RDS and congenital metabolic disease.
Infants with TTN may have cyanosis with a supplemental
oxygen requirement, but they usually do not require FiO2
greater than 0.4. They generally have blood gases that do
not reflect carbon dioxide retention, and rarely require
advanced respiratory support (i.e. intubation and mechan-
ical ventilation); such intervention is more common with
other causes of neonatal respiratory distress such as RDS,
MAS, and PPHN.
While neonates with CHD can present with cyanosis and
tachypnea, there is no family history of CHD and there were
no other clinical symptoms (i.e. murmur, decreased or absent
pulses, poor perfusion, active precordium, acidosis) to suggest
a diagnosis of CHD. In addition, the baby’s oxygen saturation
response to the administration of oxygen and the pre-ductal
and post-ductal SpO2 values less than 10% decrease the likeli-
hood of CHD or PPHN as a contributing diagnosis.
The infant’s chest radiograph showed the classic patho-
physiologic findings of TTN, including normal to slightly
hyperinflated lung volumes, mildly hazy diffuse alveolar
appearance, and central radiating linear opacities demon-
strating excess fluid being carried out via the pulmonary
vasculature. The early radiographs in TTN can appear sim-
ilar to other neonatal respiratory disorders, particularly
congenital pneumonia, which typically takes longer to
clear. Radiographically, RDS will typically show hypoinfla-
tion and a ground-glass appearance, as opposed to the
hyperinflation generally noted with TTN. The patient’s
chest X-ray also excluded pneumothorax as a diagnosis.
Despite an unknown maternal GBS status at birth, rup-
ture of membranes was at the time of delivery and there
were no other perinatal risk factors for infection, including
prolonged rupture of membranes, complicated labor, and
chorioamnionitis (e.g. maternal fever, elevated maternal
white cell count, purulent amniotic fluid, uterine tender-
ness, and fetal tachycardia). Likewise, the infant’s CBC
showed normal immature to total neutrophil, white cell
and platelet counts. The baby had no symptoms such as
lethargy, hypothermia, apnea, and respiratory failure that
may accompany neonatal infection.
What results would you expect from a
hyper-oxygen test with this disorder? Would you
consider doing any other screening tests or
diagnostics? If yes, what would you include?
Hyperoxia test is performed by measuring the arterial blood
gases of the patient while he or she breathes room air, then
remeasuring the blood gases after the patient has breathed
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Chapter 46 Neonatal Tachypnea 201
100% oxygen for 10 minutes. If the cause of the cyanosis is
primarily pulmonary in nature, the PaO2 will increase to
greater than 150 mmHg after 10 minutes on 100% oxygen.
This will not occur in cardiac disease of a shunt physiology
(right-to-left shunt).
Hyperoxia test could be useful for differentiating between
pulmonary and cardiac causes of cyanosis, and in the case
of TTN would show improvement in oxygen saturation
with the administration of 100% oxygen.
A C-reactive protein may be helpful if there is a concern
for bacterial illness. An echocardiogram would confirm or
exclude CHD or PPHN. Repeat chest X-ray may be useful if
clinical history suggests pneumonia, respiratory status wor-
sens, or for questionable diagnoses.
What would be your management goals
for the care of this infant?
Since TTN is a self-limited disorder, the goal of therapy is
supportive and includes the following:
• Respiratory support, including supplemental oxygen via
hood, nasal cannula, or continuous positive airway pres-
sure (CPAP), to provide adequate (>90%) arterial oxygen
saturation.
• Maintaining a neutral thermal environment (keeping
patient normothermic).
• Fluid and nutrition support with intravenous fluids and/
or gavage feedings until the respiratory rate is low
enough to allow oral feedings.
• Family support.
It is important to monitor for signs of respiratory fatigue
or clinical deterioration, which may suggest an alternative
diagnosis. Infants with TTN independent of other comor-
bidities rarely have oxygen requirements greater than
40% and invasive mechanical ventilation is usually not
required.
Are antibiotics indicated for this patient?
TTN alone is not an indication for antibiotic therapy. How-
ever, owing to the difficulty of distinguishing TTN from
congenital pneumonia at presentation, many newborns
with TTN receive empiric antibiotics (i.e. ampicillin, which
covers GBS and Listeria, in combination with gentamicin
for Gram-negative coverage) for a minimum of 48 hours
pending blood culture results.
There is evidence to suggest that empiric antibiotics may
be avoidable with the use of strict criteria for TTN and dil-
igent patient monitoring (Salama et al., 2013). However,
these authors recommend further studies employing rando-
mized controlled trials to confirm the feasibility and safety
of this approach.

202 Cases in Pediatric Acute Care
Which of the following strategies, if any, have
been shown to help in the management of the
patient’s diagnosis: diuretic therapy, fluid
restriction, and/or inhaled salbutamol therapy?
A Cochrane review finds no current evidence to support
diuretic therapy in the management of TTN. There are
limited data showing that infants with severe TTN might
benefit from fluid restriction, and there is some support
for the use of salbutamol therapy with TTN. However,
current data remain inconclusive with a need for larger-
scale studies. Therefore, none of these therapies is currently
recommended as standard therapy.
While obtaining consent for NICU admission and
updating the family on the newborn’s status and
plan of care, the mother wants to know what
“causes” the condition and how long you expect it
to last. How would you respond?
TTN typically carries a good prognosis, and symptoms
generally resolve within 24–48 hours and rarely go beyond
72 hours.
Reference
Salama, H., Abughalwa, M., Taha, S., Sharaf, N., and Mansour,
A. (2013). Transient tachypnea of the newborn: is empiric
Further Reading
Hagen, E., Chu, A., and Lew, C. (2017). Transient tachypnea of
the newborn. NeoReviews 18(3): e141–e149.
Kassab, M., Khriesat, W.M., and Anabrees, J. (2015). Diuretics
for transient tachypnoea of the newborn. Cochrane
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Case Resolution
Over the course of several hours, the neonate was weaned
off oxygen and the nasal cannula discontinue by 12 hours of
life. He became stable in room air, with oxygen saturations
greater than 95%, and tachypnea resolved by 32 hours of
life. The antibiotics (ampicillin and gentamicin) that had
been started due to clinical symptoms and oxygen require-
ment were discontinued with the discovery of negative
blood cultures at 48 hours.
Breastfeeding was initiated at 36 hours of life, when
respiratory rate was less than 65 breaths per minute
and intravenous fluids were weaned as he tolerated
breastfeeding and was feeding adequately. His clinical
state continued to remain stable, and he was transferred
to couplet care with his mother at approximately
48 hours of life.
antimicrobial therapy needed? Journal of Neonatal Perinatal
Medicine 6(3): 237–241. doi: 10.3233/NPM-1367012.
Database of Systematic Reviews (11): CD003064. doi:
10.1002/14651858.CD003064.pub3.

47
Diplopia and Esotropia
Lindsay Chapman
St. Jude Children’s Research Hospital, Memphis, TN, USA
An 8-year-old male with no significant past medical history
is evaluated for new complaints of diplopia and esotropia
following a 2-day history of low-grade fever and upper res-
piratory infection.
History of Present Illness
An 8-year-old right-handed male was in his normal state of
health until 2.5 weeks prior to presentation. Upon return-
ing from a vacation 2.5 weeks ago, he reported diplopia and
stated he did not feel well. He developed a fever of 38.8 C
(101.8 F) that persisted for approximately 2 days that was
associated with upper respiratory symptoms and mild
headache. He received acetaminophen and ibuprofen dur-
ing this time.
Parents noted left esotropia after resolution of illness, and
the child told his teacher about his diplopia. He presented
to his primary care provider and his evaluation was positive
for sinus drainage; he was prescribed cefdinir for sinusitis.
An ophthalmologic evaluation confirmed the presence of
left eye esotropia with normal visual acuity. As part of
the evaluation for esotropia, magnetic resonance imaging
(MRI) of the brain was performed, which demonstrated
the presence of an expansile cystic and solid lesion in the
right side of the pons measuring 2.4 × 3.4 × 2.7 cm with
nodular enhancement. He was then referred for neurosur-
gical evaluation.
Past Medical History
No significant past medical history. He is up to date on his
immunizations, including annual influenza vaccine. He is
not on any medication and does not have any food or
environmental allergies.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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203
He has achieved his developmental milestones appropri-
ately. He ambulated at 12 months. He was a toe walker so
had physical therapy starting at age 4 years and had casts
applied to both of his feet. No other developmental
concerns.
Past Surgical History
No past surgeries.
Family History
His maternal great grandmother was a previous smoker
and was diagnosed with lung cancer in her seventies.
Another maternal great grandmother was diagnosed with
stomach cancer in her late eighties. No family history of
childhood cancer, neurologic disorders, or ophthalmologic
diseases.
Current Status
He is admitted to the pediatric intensive care unit (PICU).
On physical examination, his vital signs are unremarka-
ble: temperature 37.2 C (99.0 F), heart rate 83 bpm, respi-
ratory rate 20 breaths per minute, blood pressure 115/
56 mmHg, and oxygen saturation 100% on room air. He
is a well-developed, well-nourished male who is active
and cooperative. His neurologic examination is remarka-
ble for an adduction deficit in the right eye and in the left
eye. He has horizontal nystagmus on lateral gazes bilater-
ally, and esotropia. His strength and reflexes were intact.
The remainder of the examination is unremarkable.
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204 Cases in Pediatric Acute Care

Table 47.1 Basic metabolic profile. 4 What is the best imaging modality to identify lesions in
the posterior fossa?
Sodium 141 mEq/L
Potassium 4.1 mEq/L
Chloride 103 mEq/L
Rationale and Evidence-based Practice
Carbon dioxide 27 mEq/L
Explanation
Blood urea nitrogen 19 mg/dL
Creatinine 0.3 mg/dL
Based on these data, what are the differential
Glucose 74 mg/dL diagnoses for this child?
Central nervous system (CNS) tumors are the most com-
mon solid tumor of childhood, and most of these tumors
Table 47.2 Complete blood count. occur in the infratentorium. The differential diagnosis of
infratentorial tumors may include medulloblastoma, epen-
White blood cell count 7200/mm3 dymoma, atypical teratoid rhabdoid tumor, pilocytic astro-
Hemoglobin 12.6 g/dL cytoma, and brainstem glioma that includes the diffuse
Hematocrit 37.1% midline glioma H3 K27M-mutant. Brainstem gliomas
Platelet count 316 000/mm3 account for 15–20% of all childhood CNS tumors, and dif-
Neutrophils 43.3% fuse midline gliomas may account for up to 85% of these
Eosinophils 1.4% tumors. Diffuse midline glioma with histone H3 K27M
mutation is a new diagnostic entity, previously known as
Monocytes 8.4%
a diffuse intrinsic pontine glioma. This new classification
Lymphocytes 46.1%
is not limited to the pons but may be found in supratentor-
Bands 0%
ial midline structures of the brain.
Diffuse midline gliomas are high-grade tumors classified
as WHO grade IV, and typically children have quick onset
of symptoms. The most common cranial nerve deficit asso-
A basic metabolic profile and complete blood count are
ciated with this type of tumor is an abducens nerve palsy,
obtained on arrival at the PICU (results shown in
the second most common being facial nerve palsy. Abdu-
Tables 47.1 and 47.2). He receives one dose of intravenous
cens nerve palsy is dysfunction of cranial nerve VI which
dexamethasone 4 mg followed by dexamethasone 2 mg
is responsible for causing contraction of the lateral rectus
every 8 hours and famotidine 10 mg by mouth twice daily
muscle that results in abduction of the eye. Therefore, a
for gastrointestinal protection. He undergoes repeated
patient with an abducens nerve palsy will have a difficult
sedated imaging the following day at which time the previ-
time turning the eye laterally, and this may result in an
ous findings are verified (see Figures 47.1 and 47.2).
esotropia. A facial nerve palsy is dysfunction of cranial
The brain MRI demonstrates a heterogeneous mass cen-
nerve VII that innervates the facial muscles. On examina-
tered in the pons measuring 2.4 × 3.4 × 2.7 cm with two
tion, a patient with a facial nerve palsy will exhibit facial
dominant components. Associated mass effect effaces the
weakness (e.g. drooping of the face that may involve a
prepontine cistern and partially effaces the fourth ventricle.
decrease in the prominence of the nasolabial fold, an ina-
There is no hydrocephalus. The spine MRI demonstrates no
bility to close the eyelid completely and an inability to
evidence of disease.
raise the eyebrows).

Why was he started on dexamethasone?

Questions
Answer the following questions using the details provided.
1 Based on these data, what are the differential diagnoses
for this child?
2 Why was he started on dexamethasone?
3 What are the signs and symptoms of increased intracra-
nial pressure?
Patients with a diagnosis of H3 K27M diffuse midline glio-
mas may exhibit a variety of neurologic deficits that include
cranial nerve deficits, hemiparesis, and ataxia in addition to
signs and symptoms of increased intracranial pressure
(ICP), hallmark features that include headache, nausea,
emesis, and lethargy. Dexamethasone is administered on
diagnosis to reduce peritumoral edema and associated neu-
rologic deficits. Symptoms may improve with use of

Figure 47.1 Brain MRI.
Figure 47.2 Brain MRI.
dexamethasone until the tumor responds to irradiation or
other indicated therapy.
What are signs and symptoms of increased
intracranial pressure?
Increased ICP is an increase in pressure in the CNS that
may occur due to cranial trauma, tumor, hypoxic and ische-
mic states, encephalitis, meningitis, or cerebral hemor-
rhage and infarction. The clinical manifestations of
increased ICP in children and adults may include head-
ache, nausea, projectile emesis, lethargy, vision changes,
papilledema, and ocular palsies. Clinical manifestations
in infants with increased ICP may include a bulging fonta-
nelle and increased head circumference, as their cranial
sutures may not have fused. It is imperative to recognize
and treat increased ICP in a timely manner. If not treated
appropriately, cerebral damage or death may occur. Cush-
ing’s triad, an ominous sign indicative of impending brain-
stem herniation, is characterized by irregular respirations,
bradycardia, and hypertension. Non-contrast computed
tomography (CT) can be used to diagnose cerebral edema,
evaluate ventricle size (e.g. presence of hydrocephalus as a
component of increased ICP), evaluate mass lesions,
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Chapter 47 Diplopia and Esotropia 205
determine presence of hemorrhage, and evaluate gray/
white matter.
What is the best imaging modality to identify
lesions in the posterior fossa?
MRI provides slice images of the brain and better resolution
of different structures of the brain than head CT.
A radiologist can see structures in the posterior fossa and
cervicomedullary junction much better than with CT, as
MRI provides views in three planes without interfering
bony artifact.
Continuation of case: The patient undergoes a right sub-
occipital transcerebellar burr hole and stereotactic brain-
stem biopsy that he tolerated well; he was discharged on
postoperative day 2. After surgery, his examination was sig-
nificant for a right facial palsy, dysconjugate gaze, left lower
extremity weakness, and unsteady gait that was improving.
His fatigue was improving daily. Pathology revealed a dif-
fuse midline glioma, H3 K27M-mutant, World Health
Organization (WHO) grade IV. He and his parents met with
the oncologist and discussed diagnosis, prognosis, and
treatment options including irradiation and chemotherapy
on clinical trials.

206 Cases in Pediatric Acute Care
5 Based on this additional information, what is your man-
agement plan?
Based on this additional information, what is your
management plan?
Diffuse midline gliomas are difficult to treat and are the
most challenging of all brainstem gliomas due to location
and their diffuse infiltrative nature. Children with this diag-
nosis have a poor prognosis. Median survival is approxi-
mately 10 months, and the 2-year survival rate is less
than 10%. Radiation therapy is the gold standard of treat-
ment, but provides only temporary relief of symptoms
and is primarily palliative in nature. The patient typically
receives irradiation daily for about 6 weeks. This is not a
curative treatment, but the hope is that with irradiation
the child’s neurologic symptoms may improve for a short
time. Studies have shown that children who receive con-
ventional chemotherapy do not have better outcomes than
those who receive irradiation. More recently, some children
with this diagnosis are undergoing biopsies of their tumor
in order to obtain tissue confirmation of the diagnosis and
understand the biological characteristics of these tumors.
Further Reading
American Academy of Pediatrics Section on Hospice and
Palliative Medicine and Committee on Hospital Care
(2013). Pediatric palliative care and hospice care
commitments, guidelines and recommendations. Pediatrics
132(5): 966–972.
Beltran, C., Sharm, S., and Merchant, T. (2011). Role of
adaptive radiation therapy for pediatric patients with diffuse
pontine glioma. Journal of Applied Clinical Medical Physics
12(2): 1–11.
D’Arco, F., Khan, F., Mankad, K., et al. (2018). Differential
diagnosis of posterior fossa tumours in children: new
insights. Pediatric Radiology 48: 1955–1963.
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Molecular analysis of the biopsy sample also helps detect
the presence of genetic alterations in the tumor and direct
the use of molecularly targeted therapies. Several such
agents are currently undergoing clinical trials. However,
biopsy does not come without risk. Irradiation continues
to be the gold standard of therapy, and there are research
efforts to develop and explore targeted therapies to over-
come the identified mutations gathered through tissue
biopsies in the hope of generating effective treatments for
this type of tumor. Based on the life-threatening nature
of this tumor, consultation to palliative care services or inte-
gration of palliative care concepts on diagnosis is also
recommended.
Case Resolution
The patient and his parents made the decision to enroll him
on a clinical trial that incorporated radiation therapy fol-
lowed by use of a PI3 kinase/mTOR inhibitor. He demon-
strated transient symptom relief and is engaged in palliative
care services.
Gwak, H.S. and Park, H.J. (2017). Developing chemotherapy
for diffuse pontine intrinsic gliomas (DIPG). Critical Reviews
in Oncology/Hematology 120: 111–119.
Hargrave, D., Bartels, U., and Bouffet, E. (2006). Diffuse
brainstem glioma in children: critical review of clinical
trials. Lancet Oncology 7(3): 241–248.
Ropper, A.H., Samuels, M.A., Klein, J.P., and Prasad, S. (2019).
Adams and Victor’s Principles of Neurology, 11th edn. New
York: McGraw Hill.
Weaver, M.S., Heinze, K.E., Kelly, K.P., et al. (2015). Palliative
care as a standard of care in pediatric oncology. Pediatric
Blood and Cancer 62(Suppl 5): S829–S833.

48
Acetaminophen Ingestion
Aaron Carpenter
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 19-month-old female presents to the emergency
department (ED) for parental concern of acetaminophen
overdose.
History of Present Illness
The patient’s mother reports that this 19-month-old child
developed cough, runny nose, and fever 2 days prior to pres-
entation with a maximum temperature at home of 39.1 C
(102.3 F). The 5-year-old sibling developed similar symp-
toms 4 days prior. A new bottle of acetaminophen liquid
(160 mg per 5 mL) was purchased by the mother and two
doses had been given from the new bottle the previous
day, one dose for this patient and one dose for her older
brother. Two hours prior to arrival in the ED, the patient’s
mother took the acetaminophen bottle out of the cabinet to
administer a dose and, while distracted by the sibling, the
19-month-old child took the bottle and carried it to another
room. The mother found the young child with the bottle
opened and more than half empty with no evidence of spill-
age. The mother then called the child’s pediatric nurse
practitioner at the primary care provider who recom-
mended calling the poison control center, and referred
her to the ED.
Past Medical History
The patient was born at 39 weeks’ gestation via spontane-
ous vaginal delivery with no complications. Mother experi-
enced positive prenatal care and pregnancy was
unremarkable. The child has met her developmental mile-
stones and has been growing well, with weight gain
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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207
tracking at the 50th to 60th percentile and height gain at
the 35th to 40th percentile. She attends daycare and has
had two episodes of wheezing associated with upper respi-
ratory virus infection for which she was prescribed albu-
terol as needed. She does not take any regularly
prescribed medications. Her height and weight on the
day of presentation to the ED are 82 cm and 11 kg (body
mass index 16.4).
Current Status
The child appears well, sitting on her mother’s lap, smiling,
playful and interactive; she is appropriately fearful of
the examiner. Temperature is 37.7 C (99.9 F), heart rate
108 bpm, respiratory rate 28 breaths per minute, non-
labored blood pressure 88/52 mmHg, and oxygen satura-
tion 99% on room air.
Questions
Answer the following questions using the details provided.
1 Based on the history, would you be concerned with a
potentially toxic dose of acetaminophen?
2 What data would you need to determine the appropri-
ate intervention, if any?
3 What pharmacologic interventions are available for
acute acetaminophen toxicity?
4 What are the contraindications for the use of NAC?
5 How should the patient be monitored during the course
of treatment?

208 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
Based on the history, would you be concerned with
a potentially toxic dose of acetaminophen?
Determining concern for acetaminophen toxicity requires
identifying, as accurately as possible, the total dose ingested
and the timing of the dose. Fortunately, in this case the par-
ent brought the bottle of acetaminophen to the ED. After
subtracting the volume of doses given the previous day
and measuring the amount remaining in the bottle, it
was estimated that the patient had ingested a total of
60 mL of acetaminophen suspension (160 mg per 5 mL)
2 hours prior to evaluation in the ED. The total acetamin-
ophen dose ingestion was 1920 mg or 174.5 mg/kg. A dose
of 150 mg/kg or more is concerning for potential toxicity
(Ogilvie et al., 2012).
What data would you need to determine
the appropriate intervention, if any?
The total dose, time since ingestion, and the Rumack–
Matthew nomogram (Figure 48.1) are used in the setting
of an acetaminophen overdose to direct treatment of the
patient. The Rumack–Matthew nomogram was devised in
1975 and has been used to determine acetaminophen tox-
icity based on timing of ingestion and serum acetamino-
phen level. Consultation with the poison control center
also assists in management and monitoring. Serum aceta-
minophen levels are obtained no sooner than 4 hours after
ingestion to assess for peak serum level. Determining that
the accidental ingestion occurred 2 hours prior to arrival in
the ED is important in patient management as it informs
the decision to administer pharmacologic agents to
reduce the risk of liver toxicity. Additionally, liver enzyme
testing aids in the determination of damage to liver from
toxic metabolites.
The toddler’s serum acetaminophen level drawn 4 hours
after ingestion was 187 mg/L, a level that fits within the rec-
ommendation for treatment with N-acetylcysteine (NAC).
Liver enzymes were all within normal range and the patient
remained alert, active and playful during the course of her
ED stay.
Managing acetaminophen poisoning has been a
long-term concern for healthcare providers due to rapid
liver involvement and the potential for liver failure.
Figure 48.2 shows the normal metabolism of acetamino-
phen and the safe excretion of a toxic metabolite, while
Figure 48.3 shows the pathways of acetaminophen toxicity
that result in the development of liver disease or failure.
The challenge remains in identifying potential for liver
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failure early in the ingestion phase and which therapies
are most effective.
Chiew et al. (2018) published a systematic review of
11 randomized controlled trials assessing interventions
for acetaminophen overdose in approximately 700 people.
Although the evidence was classified as low or very low,
the authors concluded that activated charcoal seemed to
be the best agent for preventing the absorption of
acetaminophen. NAC has been shown, in observational
studies, to reduce morbidity and mortality from liver
toxicity.
What pharmacologic interventions are available
for acute acetaminophen toxicity?
When administered in therapeutic doses, acetaminophen is
metabolized in the liver, primarily through glucuronidation
and sulfation. A toxic metabolite, N-acetyl-p-benzoquinone
imine (NAPQI), is conjugated by glutathione into mercap-
turic acid, which can be safely excreted through the kid-
neys. In the setting of an overdose, glutathione stores can
be overwhelmed leading to an increase in the toxic metab-
olite, which can lead to liver cell death.
Activated charcoal, which may reduce the absorption of
acetaminophen, can be given if ingestion occurs within
4 hours of presentation and the dose is within the potential
for toxicity (150–200 mg/kg). NAC can replenish glutathi-
one stores, thereby allowing for increased conjugation of
the toxic metabolite NAPQI into mercapturic acid, which
is then safely excreted through the kidneys (Ogilvie et al.,
2012). Given the relative accuracy of total dose ingested
by this patient, activated charcoal and NAC were started
3 hours after ingestion. NAC can be administered orally
on a 72-hour regimen or intravenously on a 21-hour regi-
men. The oral form is not well tolerated in children due
to its odor and may cause vomiting.
What are the contraindications for the use of NAC?
As with most pharmacologic agents, hypersensitivity to
NAC is a contraindication. Caution is recommended in
patients with a history of bronchospasm or asthma as these
patients may be at increased risk for hypersensitivity reac-
tion (Lexicomp, 2019).
Treatment duration for acetaminophen toxicity is
dependent on the patient’s course and timing of initiation
of NAC from ingestion. In this case, the serum acetamino-
phen level was zero and alanine aminotransferase
remained within normal limits at 20 hours of treatment,
allowing for discontinuation of NAC at 21 hours of treat-
ment and discharge of the patient.
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Chapter 48 Acetaminophen Ingestion 209

Single Acute Acetaminophen Overdose Nomogram

μg/mL
500
400

300

200

150
Rumack-Matthew Line

100
90
ACETAMINOPHEN PLASMA CONCENTRATION

80
70
60
50
40

30

20

Treatment Line

10
9
8
7
6
5
4

Treatment should be administered

2
if level is above solid line

4 8 12 16 20 24 28 32 36

HOURS POSTINGESTION

Nomogram: acetaminophen plasma concentration vs time after
acetaminophen ingestion (adapted with permission from Rumack
and Mathew, Pediatrics 1975:55-871-876). The nomogram has
been developed to estimate the probability of whether a plasma
acetaminophen concentration in relation to the interval postinges-
tion will result in hepatotoxicity and, therefore, whether acetylcys-
teine therapy should be administered.
CAUTIONS FOR USE OF THIS CHART:
1. Time coordinates refer to time postingestion.
2. Graph relates only to plasma concentrations following a single,
acute overdose ingestion.
3. The Treatment Line is plotted 25% below the Rumack-Matthew
Line to allow for potential errors in plasma acetaminophen
assays and estimated time from ingestion of an overdose
(Rumack et al, Arch latern Med. 1981:141(suppl):380-385).

Figure 48.1 Rumack–Matthew nomogram. Source: Merlin Cyrstal, https://upload.wikimedia.org/wikipedia/commons/9/9b/

Rumack_Matthew_nomogram_with_treatment_%28study%29_line.pdf. Licensed under CC BY SA 3.0.
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
210 Cases in Pediatric Acute Care

Acetaminophen
Acetaminophen Liver Liver
Metabolism with
Metabolism
Toxicity

CYP450 2E1 CYP450 2E1

Glucuronidation Sulfation Glucuronidation Sulfation
oxidation oxidation
8
%

Forms a reactive metabolite Major pathways Increased production of

N-acetyl-p-benzoquine (NAPQI) become saturated N-acetyl-p-benzoquine (NAPQI)
90% with drug
Conjugated and
excreted through
kidney
Toxic metabolite reacts with When glutathione is
sulfhydryl groups in depleted, NAPQI
glutathione to harmless accumulates in hepatocytes
metabolites, then excreted resulting in hepatic damage

Other names: N-acetyl-p-aminophenol, APAP, paracetamol, PARA

High oral bioavailability: reaches peak concentration in 90 minutes Figure 48.3 Metabolism of acetaminophen toxicity.
Toxic dose can cause liver damage: 120–150 mg/kg in a single dose can cause
hepatotoxicity
Liver, kidneys and intestine are organs implicated in metabolism
Maximum pediatric dosing per day: 50–75 mg/kg
Half-life increases from 1.5–2.5 hours to 4–8 hours with toxic doses
admission with frequent monitoring of liver function. Edu-
Ingestion or overdose: glucuronidation and sulfation hepatic pathways are saturated cation on the importance of safe storage of medications is
with drug, causing more tylenol to be metabolized by CYP450 system
N-Acetylcysteine is the antidote, by replacing glutathione to metabolize drug another essential piece of patient and family management,
with referral to social work sometimes necessary.
Figure 48.2 Metabolism of acetaminophen.

How should the patient be monitored during the

course of treatment?
Patients who present with liver toxicity or fulminant liver
failure require expert consultation and intensive care unit

References
Chiew, A.L., Gluud, C., Brok, J., and Buckley, N.A. (2018).
Interventions for paracetamol (acetaminophen)
overdose. Cochrane Database of Systematic
Reviews (2): CD003328. doi: 10.1002/14651858.
CD003328.pub3.
Lexicomp: Alfred I. duPont Hospital for Children formulary
(2019). Wolters Kluwer Clinical Drug information, Inc.
Ogilvie, J.D., Rieder, M.J., and Lim, R. (2012). Acetaminophen
overdose in children. Canadian Medical Association Journal
184(13): 1492–1496. doi: 10.1503/cmaj.111338.

49
A 3-Year-Old with Sudden Onset of Breathing Difficulty
Judy Verger
University of Iowa School of Nursing, Iowa City, IA, USA
A 3-year-old female with Tetralogy of Fallot (TOF) is being
admitted to the pediatric intensive care unit (PICU) for
abdominal pain. The nurse practitioner has been tasked
to administer moderate sedation for abdominal computed
tomography (CT) prior to transfer to PICU.
History of Present Illness
Patient is a 3-year-old female with history of CHARGE syn-
drome who is to undergo an abdominal CT with and with-
out contrast under moderate sedation. Topical lidocaine
cream was used when gaining peripheral intravenous
access, and midazolam 0.05 mg/kg and fentanyl 0.5 mcg/kg
were administered at the beginning of the procedure. The
patient begins to have difficulty breathing in the middle
of the procedure, during contrast infusion.
Past Medical History
The child has CHARGE syndrome with the following asso-
ciated anomalies: TOF, tracheomalacia with gastroesopha-
geal reflux disease, ocular coloboma, deafness, and
developmental delay. She also has failure to thrive and
asthma.
She has no known drug allergies. Her immunizations are
up to date as reported by her parents. Home medications
include aldactone, digoxin, ranitidine, and albuterol.
She was nil by mouth at the time of the event, but is oth-
erwise fed using a nasogastric tube with homemade
blended food.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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211
Family History/Social History
She has no significant family medical history. She was
transferred from Qatar to the United States for further
care. She lives with both parents and her younger
brother in Qatar. Her father is the only one who speaks
English.
Current Status
Vital signs are as follows: temperature 36.8 C (98.2 F),
heart rate 167 bpm, blood pressure 52/28 mmHg, respira-
tory rate 56 breaths per minute, and oxygen saturation
82% on room air. Weight is 12.5 kg.
The child appears flushed and anxious and is thrashing
and attempting to sit up.
• Head, eyes, ears, nose and throat: malformed protruding
pinna, wide-set ears, perioral edema, erythematous
mouth, swollen lips, very hoarse/raspy voice. Coloboma
of right eye noted. Right-sided facial palsy.
•
Respiratory: retractions, poor air entry bilaterally with
inspiratory and expiratory wheeze.
•
Cardiovascular: tachycardic, first and second heart
sounds heard, harsh systolic murmur.
••
Extremities: cool, normal musculoskeletal examination.
Abdomen: soft and round, positive bowel sounds.
•
Skin: large hives over face, neck, chest, abdomen
and back.
•
Neurologic: pupils 5 mm equal and react to light
briskly.
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212 Cases in Pediatric Acute Care

Questions What is the differential diagnosis of the patient’s

acute deterioration during the CT scan, and what is
Answer the following questions using the details provided. the most likely diagnosis?

1 What are the features and genetic mutations associated Because of this patient’s complex history, there are many
with CHARGE syndrome? potential differential diagnoses:
2 What is the differential diagnosis of the patient’s acute
deterioration during the CT scan, and what is the most •• anaphylactic shock
anxiety/panic attack

3
likely diagnosis?
What is the pathophysiology of the most likely •• TET spell
pulmonary embolism

4
diagnosis?
What are the initial steps in treating this patient for
anaphylaxis?
Rationale and Evidence-based Practice
Explanation
What are the features and genetic mutations
associated with CHARGE syndrome?
CHARGE syndrome is an autosomal dominant genetic dis-
order typically caused by pathogenic variants of the CHD7
gene. CHARGE syndrome comprises the following (Kahl
and Hughes, 2018, p. 496):
C Coloboma
H Heart anomalies
A Atresia choanae
R Retardation of growth and development
G Genitourinary anomalies
E Ear anomalies (including deafness and vestibular
disorders).
These features are present in various combinations and to
varying degrees. No single feature is universally present.
Clinical diagnosis of CHARGE syndrome requires the
presence of at least four major features or three major fea-
tures plus three minor features. Major features include:
•• airway obstruction due to tracheomalacia
acute asthmatic attack.
Most likely diagnosis is anaphylactic shock, due to the
sudden onset of breathing difficulty, rash (hives), hypoten-
sion, and hypoxia with associated biphasic wheeze after
administration of intravenous contrast. Intravenous CT
contrast is known to be associated with hypersensitivity/
anaphylactic reactions, with an incidence of 0.001–0.004%
(Kim, M.H. et al., 2012), and can be fatal (Kim, S.M.
et al., 2016).
Anxiety of panic attacks may be associated with wheeze
and occasionally erythematous rash, but not hypotension.
Asthma may also be associated with wheezing, but rarely
hypotension. Obstruction from tracheomalacia is typically
associated with stridor, but no hypotension or sudden onset
of rash. Pulmonary embolism, and TET spells are typically
associated with hypotension, but not rash and wheezing.
What is the pathophysiology of the most likely
diagnosis?
Anaphylaxis is a serious allergic or hypersensitivity reac-
tion mediated by IgE. It is rapid in onset and usually life-
threatening (Castells, 2017). A previously sensitized
B lymphocyte produces IgE against a specific antigen.
The IgE resides on the mast cells and basophils. Mast cell
and basophil degranulation occurs when a specific antigen,

••
or one similar to it, binds to the IgE receptor on the mast
choanal atresia or stenosis cell and basophil, leading to release of histamine.
cranial nerve anomalies

••
The clinical presentations seen in anaphylaxis are due to
eye coloboma activation of multiple histamine receptors. Histamine H1
auditory/auricular anomalies. and H2 receptor activity result in the following.
Minor features include:
• Respiratory: bronchial smooth muscle constriction and

••
increased mucus viscosity result from H1 receptor activ-
cleft lip or palate
ity. H2 activity causes increased mucus production.

••
tracheoesophageal fistula
congenital heart defects
genitourinary anomalies
• Cardiovascular: H1 and H2 receptor stimulation results in
increased vascular permeability resulting in decreased

••
preload and hypotension, and vasodilation leading to
developmental delay
flushing, reflex tachycardia, and headache.
distinctive facial dysmorphology (asymmetrical square
face, malar flattening, unilateral facial nerve paralysis, H1 and H3 receptor activity is associated with cutaneous
micrognathia, low-set cupped ears). itch and nasal congestion.

1
Anaphylaxis can therefore be defined three ways.
Sudden onset of an illness that involves the skin and/
or mucous membranes with associated sudden respi-
ratory symptoms, or hypotension/signs of end-organ
dysfunction.
2 Sudden occurrence of two or more of the following after
exposure to a likely allergen:
•• alterations of the skin/mucous membrane
respiratory signs/symptoms
••gastrointestinal symptoms
hypotension/signs of end-organ dysfunction.
3 Hypotension after exposure to known allergen (Simons
et al., 2015, p. 10).
What are the initial steps in treating this patient
for anaphylaxis?
••
Immediately stop administration of the contrast.
••
Remove child from scanner.
Assess airway, breathing and circulation (ABC).
Call for assistance from radiology personnel and/or
•
resuscitation team.
Provide supplemental oxygen (preferably high flow) via
facemask.
• Administer epinephrine with EpiPen autoinjector intra-
muscularly (or epinephrine 1 in 1000 0.01 mg/kg, up to
0.5 mg) in the mid-outer thigh. If no response, repeat
injection every 5 minutes. Epinephrine administration
addresses both airway obstruction and hypotension by
decreasing bronchospasm and increasing vascular
resistance.
References
Castells, M.J. (2017). When to order contrast-enhanced CT.
Journal of Allergy and Clinical Immunology 140: 321–333.
Kahl, L. and Hughes, H. (2018). The John’s Hopkins Hospital
Harriet Lane Handbook, 21st edn. Philadelphia, PA: Mosby
Elsevier.
Kim, M.H., Park, C.H., Kim, D.I., et al. (2012). Surveillance of
contrast-media-induced hypersensitivity reactions using
signals from an electronic medical recording system. Annals
of Allergy, Asthma and Immunology 108: 167–171.
Kim, S.M., Ko, B.S., Kim, J.Y., et al. (2016). Clinical factors for
developing shock in radiocontrast media induced
anaphylaxis. Shock 45: 315–319.
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Chapter 49 A 3-Year-Old with Sudden Onset of Breathing Difficulty 213
• Place patient supine and administer 20 mL/kg of isotonic
fluid bolus, and elevate legs at 30 above the head to help
maximize preload (unless prominent upper airway swell-
ing or compromise, in which case elevating legs may be
counterproductive) (Lieberman et al., 2015; Restauri
et al., 2016; Castells, 2017; Hughes and Kahl,
2018, pp. 4–5).
•
Consider administering histamine H1 (and H2) receptor
antagonist (Lieberman et al., 2015; Simons et al., 2015,
•
p. 10; Castells, 2017; Hughes and Kahl, 2018, pp. 4–5).
Consider albuterol or racemic epinephrine for wheezing
and bronchospasm.
• Start intravenous steroids (methylprednisolone 2 mg/kg):
has no immediate effect but can prevent late or biphasic
reactions.
If symptoms persist or there is impending respiratory
arrest, carry out the following.
• Administer 10–20 mL/kg of intravenous fluids (normal
saline, Ringer’s lactate) followed by continuous infusion
of intravenous fluids.
•• Start continuous intravenous epinephrine.
Position the airway to facilitate aeration and provide bag-
mask ventilation. Use nasopharyngeal airway to aid in
•
keeping airway patent.
Prepare for intubation. Severe anaphylaxis can cause
angioedema, obstructing the airway (Lieberman et al.,
2015; Castells, 2017; Hughes and Kahl, 2018, p. 87).
The most experienced practitioners should perform intu-
bation, as airway narrowing may make intubation
difficult.
Lieberman, P., Nicklas, R.A., Randolph C., et al. (2015).
Anaphylaxis: a practice parameter update 2015. Annals of
Allery, Asthma and Immunology 115: 341–384.
Restauri, N., Lio, E., Glueck, D., et al. (2016). Best practice for
safe and effective administration of epinephrine for the
treatment of anaphylaxis in the radiology department.
Journal of the American College of Radiology 13(3): 303–306.
doi: 10.1016/j.jacr.2015.08.018.
Simons, F.E., Ebisawa, M., Sanchez-Borges, M., et al. (2015).
2015 update of the evidence base: World Allergy Organization
anaphylaxis guidelines. World Allergy Organization Journal
8(1): 32. doi: 10.1186/s40413-015-0080-1.

50
A 2-Month-Old with Acute Abdomen
Christyne Kyper
Alaska, Native Tribal Health Consortium, Anchorage, AK, USA
A 2-month-old male infant, born at term, presents with
vomiting and decreased oral intake for 1 week. He is being
evaluated for an acute abdomen, with laboratory analysis
and radiologic procedure. He will go to the operating room
emergently once the work-up is completed.
History of Present Illness
This 2-month-old infant was healthy until 1 week prior to
admission when he developed persistent non-bilious vom-
iting. He was seen in the emergency department where an
abdominal radiograph and abdominal ultrasound were
negative. An upper gastrointestinal examination was con-
cerning for intestinal obstruction. After consultation with
the pediatric surgery team, he was taken to the operat-
ing room.
Past Medical History
The male infant was born at 38 weeks’ gestation by
spontaneous vaginal delivery to a mother with gesta-
tional diabetes. Other than being large for gestational
age, there were no other complications at birth. The
infant has reported colic and is a “spitter” according
to mother, but has been gaining weight and has regular
bowel movements. The infant recently had his 2-month
well child visit, and received second hepatitis B, rotavi-
rus, diphtheria, tetanus and pertussis (DTaP), Haemo-
philus influenzae, and first pneumococcal vaccines at
that time.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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215
Past Surgical History
Circumcision during birth hospitalization.
Family History
Mother has gestational diabetes and history of asthma as a
child. Father is not involved with infant care, but also had
asthma as a child. The maternal grandmother is deceased
from lung cancer, maternal grandfather has hypertension,
and paternal grandparents are alive and healthy.
Questions
Answer the following questions using the details provided.
1 What differential diagnoses would you consider and
what would be the top diagnosis on the list?
2 What diagnostic study is most accurate when evaluat-
ing for anatomic abnormalities?
3 What additional information should be obtained before
taking the infant to the operating room?
4 What is involved in the process of an upper gastrointes-
tinal examination and what findings would indicate
malrotation?
5 What is the pathophysiology of intestinal malrotation?
Continuaton of case: This infant is diagnosed with malro-
tation and plans are being made to take him to the operat-
ing room for surgical intervention.
6 What is a Ladd procedure?

216 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
What differential diagnoses would you consider
and what would be the top diagnosis on the list?
Differential diagnoses for this infant include gastroesopha-
geal reflux disease (GERD), pyloric stenosis, intussuscep-
tion, malrotation (with or without volvulus), duodenal
obstruction, and gastroenteritis.
Gastroenteritis in an otherwise well appearing infant
without diarrhea is unlikely and there is no history of expo-
sure to illness. GERD is a possibility, but given the acute
nature of the vomiting, it is important to evaluate for other
diagnoses first.
Pyloric stenosis, intussusception, duodenal obstruction,
and malrotation are all anatomic abnormalities determined
by radiographic studies. An infant with a bowel obstruction
would typically present with bilious emesis. Patients pre-
senting with an acute abdomen should have a plain abdom-
inal radiograph to evaluate for the possibility of an
intestinal perforation, which is a life-threatening surgical
emergency. Laboratory studies can assist in the identifica-
tion of a bowel perforation. A simple metabolic panel can
indicate the presence of acidosis, which is typically present
with a perforation.
What diagnostic study is most accurate when
evaluating for anatomic abnormalities?
Ultrasound is a reliable method for diagnosis of pyloric ste-
nosis, but not for anatomic abnormalities. Upper gastroin-
testinal investigation is considered the gold standard for
diagnosing these conditions.
What additional information should be obtained
before taking the infant to the operating room?
An infant who has been vomiting is at risk for dehydration
and electrolyte abnormalities, which should both be cor-
rected prior to going to the operating room. Most parents
and nurses have difficulty quantifying the volume of eme-
sis, which makes the accuracy of this assessment question-
able. Hydration status can be assessed clinically by
checking the infant’s weight, condition of fontanelle, skin
turgor, and mucous membranes, and by asking how many
wet diapers the infant has had in the last 24 hours. Evalu-
ation of electrolytes will also provide an indication of
hydration status.
Plain radiographs are most often obtained first with a gas-
trointestinal complaint or problem. An abdominal X-ray
taken in the left-lateral decubitus position can assist in
the diagnosis of intestinal perforation. Suggestion of an
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obstruction and/or constipation are two other problems
that can be identified using plain abdominal films. Findings
that are diagnostic of a bowel perforation are a nasogastric
or orogastric tube that extends into an abnormally posi-
tioned duodenum and the “double-bubble” sign, which is
present when partial obstruction of the duodenum causes
distension of the stomach and first part of the duodenum.
What is involved in the process of an upper
gastrointestinal examination and what findings
would indicate malrotation?
An upper gastrointestinal contrast study is the best exami-
nation for visualizing the duodenum in infants and chil-
dren. Sensitivity for this study is 96% for malrotation. An
upper gastrointestinal examination is performed under
fluoroscopy by a radiologist. The infant is given a bottle
with contrast to drink and then a series of images are taken
as the contrast passes through the small bowel. Radio-
graphic signs of malrotation include a clearly misplaced
duodenum with the ligament of Treitz on the right side
of the abdomen, a duodenum with a “corkscrew” appear-
ance, or duodenal obstruction.
What is the pathophysiology of intestinal
malrotation?
Rotational abnormalities occur as a result of an arrest of
normal rotation of the embryonic gut. Malrotation is often
associated with other abnormalities (duodenal atresia,
imperforate anus, Meckel diverticulum, biliary atresia,
and cardiac anomalies are among some of the associated
findings), but can also occur in otherwise healthy infants
and children. Over half of children with intestinal malrota-
tion will present by 1 year of age. Primary clinical findings
in infants are vomiting (93%), typically bilious but can be
non-bilious, abdominal distention and tenderness (which
is difficult to assess in infants), and hemodynamic instabil-
ity from hypovolemia and/or septic shock if there is a per-
foration. Less common presentations are failure to thrive,
malabsorption, solid food intolerance, chronic diarrhea,
pancreatitis, biliary obstruction, gastrointestinal motility
disorder, and chylous ascites. Malrotation can lead to intes-
tinal volvulus, a life threatening condition that can result in
bowel ischemia, necrosis, sepsis and death, so it is
extremely important to begin work-up quickly, diagnose
the problem and take the infant to the operating room.
What is a Ladd procedure?
The Ladd procedure, first described by William Ladd, is
used to correct malrotation and midgut volvulus. At the
time of surgery, if a volvulus is visualized, it is untwisted

or detorsed in a clockwise fashion. If present, division of
the Ladd bands, which are the peritoneal bands crossing
from the right paracolic gutter over the abdomen, is per-
formed along with widening of the base of the mesentery
by dividing any adhesions between the duodenum and
cecum. In a neonate, a tube is passed through the duode-
num to evaluate for associated duodenal obstruction. Via-
ble bowel is placed in a position of non-rotation, small
bowel on the right, colon on the left, and adhesions are
created to hold the intestines in place, making subsequent
volvulus less likely; an incidental appendectomy is also
Further Reading
Graziano, K., Islam, S., Dasgupta, R., et al. (2015).
Asymptomatic malrotation: diagnosis and surgical
management. Journal of Pediatric Surgery 50(10):
1783–1790. doi: 10.1016/j.jpedsurg.2015.06.019
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Chapter 50 A 2-Month-Old with Acute Abdomen 217
performed. In the absence of volvulus or bowel ischemia,
this procedure can be done laparoscopically. Complica-
tions are rare from a Ladd procedure if it is an isolated
malrotation. If it is associated with a volvulus, bowel re-
section could be necessary and this can result in short
bowel syndrome.
Preoperative management includes cardiopulmonary
and circulatory resuscitation if necessary, placement of a
gastric decompression tube, and administration of broad-
spectrum antibiotics to treat bowel flora which may have
spilled into the peritoneal cavity.
Hasosah, M.Y., Meer, W.I., and Alsaleem, K. (2016). Intestinal
volvulus: a life-threatening disease. Journal of Clinical and
Diagnostic Research 10(3): SJ01.
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219

51

Henoch–Schönlein Purpura Nephritis

Tamara Hill
University of Maryland Medical Center, Baltimore, MD, USA

A 16-year-old female presents with fever, gross hematuria Family History

and sore throat for 5 days.
The girl’s mother has had hypertension since age 40 and
maternal grandfather has also been treated for the same.
Father of child is healthy.
History of Present Illness

This 16-year-old female was treated for strep throat with

oral penicillin by her primary care provider. After a day,
Current Status
she developed an erythematous non-pruritic rash which
On physical examination, there is pharyngeal erythema,
progressed proximally from both feet to thighs and upper
petechiae on the soft palate, cervical lymphadenopathy,
extremities including palms and soles. Later, her feet
and a nodular, non-tender, non-blanching purpuric rash
became swollen with moderately intense (7/10) burning
involving both upper and lower extremities with non-
pain, aggravated by ambulation. Two days later, the patient
pitting pedal edema. There is no truncal involvement.
developed vomiting and abdominal pain involving the right
A comprehensive metabolic panel, complete blood count
and left upper quadrant that was constant, colicky in
and urinalysis are obtained and the results are shown in
nature, 8/10 in intensity, and aggravated with meals and
Tables 51.1–51.3. Results of additional investigations are
bloody stools.
as follows.

•• C-reactive protein (CRP): 57.5 mg/L

Prothrombin time: 18.0 seconds
Past Medical History
•• International normalized ratio: 4
Complement: C3 125 mg/dL; C4 11 mg/dL
The patient was born by spontaneous vaginal delivery at
term and went home with her mother. She is current on •• Antistreptolysin O titer: 823 IU
Anti-nuclear antibody: negative
all immunizations and has received annual influenza
immunization. She has a history of mild intermittent • Stool for occult blood: positive.

asthma.

Questions
Past Surgical History
Answer the following questions using the details provided.
This teen had a gastrojejunostomy following a remote 1 Based on the data given, what is the differential diagno-
motor vehicle accident 2 years ago. sis and the most likely diagnosis for this child?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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220 Cases in Pediatric Acute Care

Table 51.1 Complete blood count. 2 What is the best initial management for Henoch–
Schönlein purpura?
White blood cell count 23 000/mm3
3 How would you determine if the therapy was effective?
Hemoglobin 10.8 g/dL 4 What other therapies would be recommended for
Hematocrit 32% Henoch–Schönlein purpura?
Platelet count 693 000/mm3 5 Would you obtain any other diagnostic studies?

Table 51.2 Complete metabolic profile.

Rationale and Evidence-based Practice
Explanation
Sodium 138 mEq/L
Potassium 4.6 mEq/L Based on the data given, what is the differential
Chloride 101 mEq/L diagnosis and the most likely diagnosis for
this child?
Carbon dioxide 22 mEq/L
Blood urea nitrogen 24 mg/dL IgA vasculitis (IgAV), also called Henoch–Schönlein
Creatinine 2.4 mg/dL purpura (HSP), is a systemic vasculitis with a prominent
Glucose 88 mg/dL cutaneous component. HSP is an acute, systemic, immune
complex-mediated leukocytoclastic vasculitis. It is charac-
Calcium 7.8 mg/dL
terized by a clinical triad of palpable purpura (without
Albumin 2.9 mg/dL
thrombocytopenia), abdominal pain, and arthritis. Glomer-
Total protein 4.9 g/dL
ulonephritis and gastrointestinal bleeding are common
Aspartate aminotransferase 24 U/L complications. In patients with HSP, IgA immune
Alanine aminotransferase 24 U/L complexes are deposited in small vessels, which causes
Alkaline phosphatase 60 U/L petechiae and palpable purpura. When immune complexes
Total bilirubin 0.5 mg/dL occur in small vessels of the intestinal wall, gastrointestinal
Magnesium 1.6 mEq/L hemorrhage may develop. If the immune complexes affect
Phosphorus 4.2 mg/dL
the renal mesangium, it may produce mild proliferative to
severe crescentic glomerulonephritis.
IgAV (HSP) is characterized by the tissue deposition of
IgA-containing immune complexes. The histologic findings
in the kidney are identical to those in IgA nephropathy,
Table 51.3 Urinalysis. suggesting that the two disorders may have a similar path-
ogenesis. Likewise, the finding of high circulating levels of
Color Dark yellow galactose-deficient IgA1 in patients with both IgA nephro-
pH 6.3 pathy and IgAV (HSP) suggest a common underlying path-
Specific gravity 1.008 ogenetic mechanism. Exposure to an antigen from an
Protein 3+ infection, medication, or other environmental factor may
Nitrites Negative trigger antibody and immune complex formation.
Urobilirubin 1+
Blood 3+ What is the best initial management
Leukocytes Negative for Henoch–Schönlein purpura?
Ketones Negative
No specific therapy for HSP exists. The main goal of treat-
Glucose Negative
ment is to relieve symptoms such as joint pain, abdominal
White blood cell count 26 per high power field pain, and swelling. People with kidney involvement may
Red blood cell count 12 per high power field receive treatment aimed at preventing long-term kidney
Hyaline casts None disease.
Bacteria Occasional Treatment is rarely required for the rash. Joint pain is
Crystals none often treated with non-steroidal anti-inflammatory medica-
tions such as aspirin or ibuprofen. Corticosteroids may be

more effective in treating joint pain. Corticosteroids are also
used to treat abdominal pain.
Though rare, surgery may be needed to treat intussuscep-
tion or to determine the cause of swollen testicles, if present
in a male.
How would you determine if the therapy was
effective?
There will be resolution of symptoms and a decrease in
erythrocyte sedimentation rate (ESR), CRP and IgA levels.
What other therapies would be recommended
for Henoch–Schönlein purpura?
HSP that affects the kidneys may be treated with cortico-
steroid and immunosuppressive medications. Immunosup-
pressive medications prevent the body from making
antibodies. Patients with severe acute kidney failure are
treated with high-dose corticosteroids and the immunosup-
pressive cyclophosphamide (Cytoxan).
Further Reading
Dillon, M.J. and Ozen, S. (2016). A new international
classification of childhood vasculitis. Pediatric Nephrology 21
(9): 1219–1222.
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Chapter 51 Henoch–Schönlein Purpura Nephritis 221
Patients may require antihypertensives to treat hyperten-
sion, including angiotensin-converting enzyme (ACE) inhi-
bitors and angiotensin receptor blockers (ARBs). These
medications have proven effective in slowing the progres-
sion of kidney disease. Many people require two or more
medications to control their blood pressure. In addition
to an ACE inhibitor or an ARB, diuretics may also be
warranted. Beta-blockers, calcium channel blockers, and
other blood pressure medications may also be needed.
Blood and urine tests are used to check the kidney func-
tion of patients with HSP for at least 6 months after the
main symptoms disappear.
Would you obtain any other diagnostic studies?
An abdominal or renal ultrasound may be required to rule
out other causes of abdominal pain and to check for possi-
ble complications such as a bowel obstruction. A renal
biopsy may be useful in distinguishing between HSP and
IgA nephropathy.
Pohl, M. (2015). Henoch–Schönlein purpura nephritis.
Pediatric Nephrology 30(2): 245–252. doi: 10.1007/s00467-
014-2815-6

52
Toddler with Tachypnea and Cool Extremities
Cathy Woodward
Long School of Medicine, University of Texas Health San Antonio, San Antonio, TX, USA
A 3-year-old previously healthy toddler presented to the
emergency department (ED). Caregivers report cool
extremities, rapid breathing for past 3 days, and he is
refusing to eat today.
History of Present Illness
This 3-year-old child developed symptoms 3 days ago when
caregivers noticed the toddler breathing fast and not as
active as normal. Over the past 3 days he has been sleeping
more, is irritable and only taking small amounts from his
sippy cup. It is reported that the patient had a “cold” about
2 weeks ago with fever, cough and congestion which lasted
approximately 7 days. The family feels the toddler has not
returned to baseline activity since that illness.
Past Medical History
He was born at term and went home with his mother. All
required vaccines along with annual influenza vaccines are
up to date. Weight and height are at the 80th and 85th per-
centiles for his age and gender. His does not take any reg-
ular medications. No previous surgeries.
Current Status
On arrival at the ED, the toddler responds by crying and
weakly pulling away. His initial respiratory rate is
48 breaths per minute with mild subclavicular retractions,
and coarse crackles bilaterally. No wheezing is noted. His
extremities are cool to the touch, with 1+ pulses in all
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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223
extremities. His abdomen is soft and his liver is palpated
2 cm below the right costophrenic angle. Vital signs include
axillary temperature 37 C (98.6 F), heart rate 150 bpm, reg-
ular rhythm, blood pressure 72/48 mmHg, and oxygen
saturations 92% on room air.
A complete metabolic panel, complete blood count, and
capillary blood gas analysis are obtained and the results are
shown in Tables 52.1–52.3. Additional investigations to
help differentiate an infectious or inflammatory cause for
illness include blood and urine cultures, respiratory viral
polymerase chain reaction (PCR) nasal swab, cardiac tropo-
nins, C-reactive protein, and erythrocyte sedimentation
rate (ESR), with results as follows.
• Serum cardiac troponin I: 8.0 ng/mL (normal <2.0
ng/mL).
•• C-reactive protein: 25 mg/L (normal 0–10 mg/L).
ESR: 35 mm/hour (normal 0–10 mm/hour).
• Respiratory viral PCR nasal swab: positive for
adenovirus.
Questions
Answer the following questions using the details provided.
1 Which other diagnostic studies would be appropriate
to order?
2 Based on the data given, what is the differential diagno-
sis and the most likely diagnosis for this child?
3 What is the best way to improve this child’s work of
breathing?
4 What medications might improve his condition in
the ED?
5 What other therapies or medications would be appro-
priate on admission to the pediatric intensive care unit?
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224 Cases in Pediatric Acute Care

Table 52.1 Basic metabolic profile. Based on the data given, what is the differential
diagnosis and the most likely diagnosis for
Sodium 141 mEq/L this child?
Potassium 3.8 mEq/L
The differential includes myocarditis, community-acquired
Chloride 101 mEq/L
pneumonia, acute respiratory distress syndrome, cardiomy-
Carbon dioxide 15 mEq/L
opathy, and sepsis.
Blood urea nitrogen 24 mg/dL The most likely diagnosis for this child is myocarditis sec-
Creatinine 0.6 mg/dL ondary to recent viral respiratory illness. Myocarditis is an
Glucose 86 mg/dL inflammatory disease of the myocardial muscle, and in the
United States the most common cause of myocarditis is
viral infection. Some viruses associated with myocarditis
are adenovirus, enterovirus, parvovirus, human herpesvi-
Table 52.2 Complete blood count.
rus, hepatitis C, Epstein–Barr virus (EBV), and cytomegalo-
White blood cell count 10 000/mm3 virus (CMV). In other countries, and less commonly in the
Hemoglobin 13 g/dL United States, the causes include autoimmune disease,
Chagas disease, diphtheria, hypersensitivity reactions,
Hematocrit 40%
and toxins. The clinical presentation of myocarditis in chil-
Platelet count 346 000/mm3
dren is varied, from children presenting with relatively mild
Neutrophils 79% symptoms to those in cardiogenic shock with dysrhyth-
Eosinophils 6% mias. A mild early case of myocarditis may be missed as
Monocytes 6% the symptoms mimic respiratory illnesses.
Lymphocytes 9%
Bands 0% What is the best way to improve this child’s work
of breathing?
Assistive support is indicated. A non-invasive strategy pro-
Table 52.3 Capillary blood gas analysis. vides an ideal stepwise approach. He is placed on 6 L/min of
50% oxygen via high-flow nasal cannula. Oxygen satura-
pH 7.30 tions improve to 96% and work of breathing is improved,
PCO2 44 mmHg with a respiratory rate of 30 breaths per minute, and min-
PaO2 60 mmHg imal subclavicular retractions and bilateral crackles.
Bicarbonate 16 mEq/L
Base excess –5 What medications might improve his condition
in the ED?
Lactate 2.5 mmol/L
Cardiac failure is suspected so an intravenous dose of furo-
semide 1 mg/kg is given to reduce intravascular volume
load and workload on the heart. A single intravenous dose
Rationale and Evidence-based Practice of ceftriaxone is administered, while awaiting culture
Explanation results, before the child is transferred to the pediatric inten-
sive care unit.
Which other diagnostic studies would
be appropriate to order? What other therapies or medications would be
appropriate on admission to the pediatric
Cardiac troponins and echocardiogram are ordered
intensive care unit?
because his non-febrile respiratory findings and liver
enlargement suggests a cardiac cause for his failure. On admission to the pediatric intensive care unit, his heart
A chest radiograph report demonstrates mild pulmonary rate is 150 bpm, blood pressure 65/35 mmHg, and oxygen
edema. An echocardiogram demonstrates reduced left ven- saturation 94% on 6 L/min of 50% oxygen via high-flow
tricular ejection, mild left ventricular dilation, and reduced nasal cannula. Decreasing the workload on the heart begins
septal wall function. An electrocardiogram (EKG) reveals with rest, oxygen therapy and inotropes to improve diastolic
low-voltage QRS complexes and mild non-specific ST–T filling, systolic function and cardiac output. A 5 mL/kg
wave abnormalities. bolus of normal saline is administered over several

minutes. A milrinone infusion is started at 0.3 μg/kg per
min and an epinephrine infusion at 0.03 μg/kg per min.
The child is placed nil by mouth and intravenous fluids
are started at 100 mL/kg daily. A central line is placed to pro-
vide venous samples for laboratory blood draws and to
administer medications. A cardiac magnetic resonance study
is ordered.
The gold standard diagnostic test for myocarditis is endo-
myocardial tissue biopsy; however, obtaining the sample is
not without risk and can be delayed if the child improves on
medical therapies. A recent study has demonstrated the
possible value of cardiac magnetic resonance to help in
the determination of myocarditis in children with signs
and symptoms of cardiac infarction (i.e. chest pain, EKG
changes and increased cardiac biomarkers). This diagnostic
test could be considered in order to avoid endomyocardial
biopsy.
Oxygen will allow the child to maintain normal oxygen
saturations. This child was hypotensive perhaps due
partially to poor oral intake for the past few days leading
to dehydration, but also due to poor cardiac output associ-
ated with myocardial dysfunction. A volume bolus of 5 mL/
kg can be administered while closely monitoring effect as
the chest radiograph and respiratory assessment were
concerning for pulmonary congestion due to poor cardiac
Further Reading
Butts, R., Boyle, G., Deshpande, S., et al. (2017). Characteristics
of clinically diagnosed pediatric myocarditis in a
contemporary multi-center cohort. Pediatric Cardiology 38:
1175–1182.
Canter, C. and Simpson, K. (2014). Diagnosis and treatment of
myocarditis in children in the current era. Circulation 129:
115–128.
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Chapter 52 Toddler with Tachypnea and Cool Extremities 225
function. Milrinone, a phosphodiesterase 3 inhibitor, is
an effective medication for improving cardiac filling during
diastole, improving systolic function and reducing after-
load. Epinephrine can be used as an adjunct to milrinone
to improve inotropy and systolic function. If this medical
management is started early it may be possible to avoid
intubation. If he was experiencing worsening cardiac fail-
ure, cardiac dysrhythmias or worsening respiratory failure,
his myocardial workload could be reduced by administra-
tion of sedation, muscle relaxation and intubation. On
occasion, children with severe heart failure myocarditis
who show no sign of improvement may require heart
transplantation.
Case Resolution
Over the next few hours, the child’s condition stabilizes. His
metabolic acidosis improves and the epinephrine and
oxygen are weaned. The toddler remains extubated and
on milrinone infusion for several days as myocardial
function improves, as demonstrated by echocardiogram,
improved blood gases, and improvement in clinical signs
and symptoms.
Martinez-Villar, M., Gran, F., Sabate-Rotes, A., et al. (2018).
Acute myocarditis with infarct-like presentation in a
pediatric population: Role of cardiovascular magnetic
resonance. Pediatric Cardiology 39(1): 51–56.

53
Teen with Intractable Headache
Tara Pezzuto
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 13-year-old girl diagnosed at age 11 years with intermit-
tent migraine headaches at a frequency of four to five times
per month presents to the emergency department (ED)
with an intractable constant headache for 1 week, diplopia
intermittently for 3 days, and nausea.
History of Present Illness
She has a history of anxiety, migraine with aura, acne, and
exercise-induced asthma. She is not taking any prevention
medications for migraine, is receiving counseling for anxi-
ety but is not on any anxiolytics. She uses her albuterol
inhaler as needed for her asthma. Three weeks ago she
was started on minocycline for her acne. She has been
doing well with her headache hygiene: drinking ade-
quately, sleeping adequately, eating well and three meals
a day, limiting screen time, and exercising routinely. She
has tried ibuprofen 400 mg every 8 hours for 1 week with
no relief. While she reports her typical migraine features
of frontal head pain, photophobia, phonophobia, nausea
and vertigo, she is now also complaining of seeing double
and is bumping into walls when she attempts to walk.
She has no fever or history of trauma. She has no history
of recent acute illness. She has been going through a signif-
icant growth spurt and parents report an increase in appe-
tite. She has gained 4.5–6.8 kg in the last 4–6 months.
Past Medical History
She was full-term to a 36-year-old gravida 3, para 2, miscar-
riage 1 mother via cesarean section due to fetal stress and
prolonged labor. Her birthweight was 3.09 kg and her
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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227
feeding, vision and hearing screening were normal, with
no congenital anomalies, no cardiac concerns, and no infec-
tion or fever. She achieved developmental milestones on
time and is doing very well on the honor roll at school.
She is overweight with a body mass index (BMI) of 29.47;
she is in the 95th percentile for weight and the 27th percen-
tile for height.
Past Surgical History
No overnight hospitalization or any surgeries.
Family History
No history of epilepsy, cerebral palsy, muscular dystrophy,
neurofibromatosis, tuberous sclerosis, autism, schizophre-
nia, Tourette syndrome, multiple sclerosis, or developmen-
tal delay. Positive family history for migraine in mother and
maternal grandmother, kidney stones in paternal uncle,
hypertension on paternal side of family, high cholesterol
on maternal side of family, and stroke in maternal grand-
mother at age 65 years and in maternal aunt at 40 years.
There is celiac disease noted in another maternal aunt
and fibromyalgia in paternal aunt. Anxiety and depression
are common on paternal side of the family and a maternal
cousin was diagnosed with bipolar disorder.
Current Status
The patient is currently complaining of head pain (7/10 on
numeric scale) with photophobia and phonophobia. She is
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228 Cases in Pediatric Acute Care

nauseated and is having trouble walking because of diplo-

pia. Her examination includes appropriate vital signs for
her age. She is awake, alert and oriented, answering ques-
tions appropriately. Her pupils were equal, round and reac-
tive to light. Her fundoscopic examination suggests cloudy
disk margins and her finger-to-nose exercise is inaccurate.
She moves all extremities with full range of motion; she has
normal muscle bulk, strength and tone. Deep tendon
reflexes are +3 in all extremities. She has difficulty with
tandem walking and Romberg test. She follows a five-step
command but struggles with visual acuity. She is resting in
an emergency room with the lights off. An intravenous
catheter has been placed and normal saline is infusing at
maintenance rates, she was given ondansetron 4 mg intra-
venously, and the neurology team has been consulted.
Neurology suggests an ophthalmologic consult with vis-
ual acuity testing, dilated fundoscopic examination, and
optic nerve imaging. Bilateral papilledema is noted on
examination, while visual acuity testing indicates visual
field cuts in bilateral peripheral vision. Magnetic resonance
imaging (MRI) of the brain with and without contrast as
Figure 53.1 MRI of brain.
well as magnetic resonance venography (MRV) is ordered.
The imaging suggests a normally structured brain
except for enlargement of the optic nerves suggesting Questions
papilledema. Neurology reviews the images and suggests
a lumbar puncture for opening pressure, and cerebrospinal Answer the following questions using the details provided.
fluid (CSF) for protein, glucose, white blood cell count, red
blood cell count, viral panel, Lyme titer and an extra vial on 1 Based on the data, what are the differential diagnoses
hold for possible further testing. and the most likely diagnosis for this child?
Results of CSF analysis are shown in Table 53.1 and MRI 2 What is the best way to treat this problem and how
brain scan in Figure 53.1. Analysis of MRI scan found intra- would you order this?
cranial hypertension, narrowed anterior superior sagittal 3 What other therapies would be recommended at
sinus and straight sinus, and patent deep and superficial this time?
cerebral venous drainage without evidence of current
thrombosis, with normal intracranial magnetic resonance
angiography.
Rationale and Evidence-based Practice
Other results showed opening pressure of 46 cmH2O and
viral panel negative.
Explanation

Based on the data, what are the differential

diagnoses and most likely diagnosis for this child?

Table 53.1 Cerebrospinal fluid analysis. The differential diagnoses include migraine with aura,
pseudotumor cerebri syndrome (PTCS), Lyme meningitis,
Reference range Result viral encephalitis, venous sinus thrombosis, and tumor.
Lyme meningitis, viral encephalitis, tumor, and venous
Color Colorless sinus thrombosis were excluded based on the results of lab-
Clarity Unknown Clear oratory testing, diagnostic testing, and focused clinical
RBC manual count <100/mm 3
5/mm3 examination. Lyme meningitis would have been suspected
WBC manual count 0–5/mm 3
1/mm3 if there was presence of an erythema migrans rash, head-
Glucose 40–75 mg/dL 53 mg/dL
ache lasting more than 7 days, CSF pleocytosis, or positive
Lyme titer. The presentation for viral encephalitis would
Protein 15–45 mg/dL 32 mg/dL
typically include other symptoms, including fever, ataxia,

change in mental status, and possibly seizures. Also, she
does not have MRI findings indicative of venous sinus
thrombosis or tumor. She may have secondary migraine
with aura; however, the primary cause for increased head-
ache frequency is PTCS.
She is diagnosed with PTCS, which is caused by the rapid
overproduction of CSF. Visual loss is a potential complica-
tion of this syndrome. The goal of treatment is preservation
of vision and alleviation of symptoms. Visual field testing,
dilated fundoscopic examination, and imaging of optic
disks are necessary for proper diagnosis and should be
included initially and at follow-up examinations every
2–6 weeks. Pseudotumor cerebri most often occurs in
women of childbearing years who have had significant
weight gain. When the BMI is over 40, the severity of papil-
ledema and visual loss rises significantly. The diagnosis of
PTCS is made with the assistance of the modified Dandy
criteria. These criteria include papilledema (and other signs
of increased intraranial pressure), a normal neurologic
examination (except possible cranial nerve VI palsy), nor-
mal neurologic imaging and CSF composition, and an
opening lumbar puncture pressure greater than 28 cmH2O
for children.
What is the best way to treat this problem and how
would you order this?
Lumbar puncture is not only a diagnostic measure for PTCS
but also a treatment for it. Normal opening pressure for an
adult patient would be below 25 cmH2O; however, some
sedative agents can increase opening pressure and over-
weight patients can have a slightly elevated opening pres-
sure by virtue of being overweight. In this case, the
minocycline was stopped because PTCS can be a side effect
of this medication. Initial treatment strategies should
include weight reduction and a salt-restricted diet. In many
instances, acetazolamide is used as the first-line pharmaco-
logic therapy to reduce the risk of recurrence of PTCS.
A slow titration of acetazolamide is recommended to
reduce side effects. Alternates to acetazolamide include a
trial of topiramate or a loop diuretic.
Further Reading
Bell, S. (2016). Idiopathic intracranial hypertension
(pseudotumor cerebri). Journal of Neuroscience Nursing 48
(6): 303–310.
Friedman, D., Grant, T.L., and Digre, K.B. (2013). Revised
diagnostic criteria for pseudotumor cerebri syndrome in
adults and children. Neurology 81: 1159–1165.
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Chapter 53 Teen with Intractable Headache 229
What other therapies would be recommended at
this time?
Reoccurrence of PTCS is commonly associated with weight
gain so many patients are referred to a nutritionist, a psy-
chologist and possibly a bariatric specialist for assistance.
Studies have indicated that even a 5–10% weight loss signif-
icantly improved PTCS symptoms. Often children have
other coexisting health problems that will need to be
assessed and treated, including obstructive sleep apnea,
anemia, hypertriglyceridemia, and fatty liver disease. In
advanced cases, neurosurgical consult will be needed for
possible ventricular shunt, stent or decompression.
Low-pressure headache is often a side effect of a lumbar
puncture and can be avoided by lying flat for a period of
time and transitioning to increased activity slowly. Low-
pressure headaches are positional headaches that are
decreased by lying flat but which are increased by sitting
up or standing and can happen after a lumbar puncture.
Case Resolution
The patient was admitted to the hospital overnight while
she recovered from the lumbar puncture, making sure
she was lying flat for at least 4 hours and transitioning to
sitting and standing positions slowly after this. Her head-
ache resolved after the lumbar puncture. She was dis-
charged to home the next day and started on
acetazolamide 250 mg twice daily for 1 week and then
increased to 500 mg twice daily for 4 months. Her minocy-
cline was stopped and she was encouraged to increase activ-
ity and healthy food choices. The family was requested to
monitor for side effects and monitor papilledema with
ophthalmologic examination every 2 weeks to 2 months
until resolved. If any symptoms occurred again (e.g. blurry
vision, intractable headache or any other neurologic focal
symptoms) they were advised to contact ophthalmology
and neurology. Neurology will follow up monthly to mon-
itor the acetazolamide treatment and for any signs of
increased pressure.
Sheldon, C.A., Paley, G.L., Beres, S.J., and McCormack, S.E.
(2017). Pediatric pseudotumor cerebri syndrome: diagnosis,
classification and underlying pathophysiology. Seminars in
Pediatric Neurology 24(2): 110–115.

54
Seizures in School-age Child
Ann Marie Felauer
University of Maryland School of Nursing, Baltimore, MD, USA
An 8-year-old female, weighing 35 kg, with a past history of
febrile seizures and new diagnosis of epilepsy is admitted to
the intermediate care unit for status epilepticus.
History of Present Illness
Two days prior to arrival this child developed nausea and
had two episodes of non-bloody non-bilious emesis. She
denies any diarrhea. Her brother and father had similar
symptoms last week so her parents felt that she had gastro-
enteritis. She continued on her levetiracetam maintenance
seizure medication and over the last 24 hours was able to
keep some clear liquids down. There were no reports of
fevers and she denies chills or rigors. She reported feeling
tired and that she did not want to play on her computer.
On the day of presentation the patient was watching televi-
sion when it was noted that her head arched back and she
stiffened and began full body shaking. Rectal diazepam
(Diastat) 10.5 mg was administered by her parents when
the activity continued for several minutes, and they called
911. When emergency medical services (EMS) arrived the
activity was continuing and lorazepam 2 mg was given
intravenously with eventual cessation of seizure activity.
EMS transported her to the local children’s hospital emer-
gency department (ED) for further care and evaluation. In
the ED, the child was post-ictal, but was able to maintain
her own airway.
Past Medical History
This patient was born at 38 weeks of gestation via normal
spontaneous vaginal delivery and was discharged home
with her mother. She has had all required vaccines along
with annual influenza vaccines. Her weight has been at
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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231
52% for gender and age since age 3, and her current body
mass index (BMI) is 19.3. She had her first simple febrile
seizure at 18 months. She continued to have febrile seizures
intermittently with illness, a total of nine by age 6 years.
When she was 7 years old she presented to her local com-
munity ED after a brief seizure lasting less than 2 minutes
at home. This seizure was generalized tonic–clonic and was
not associated with fever or illness at the time. She returned
to her baseline neurologic status when she was seen in the
ED and her family was told to follow up with her primary
care provider (PCP) and neurologist. Subsequent visits with
her PCP and neurology resulted in a diagnosis of seizure
disorder and she was started on levetiracetam. No other
medical history. Recent visit to PCP for complaints of
growing pains.
Past Surgical History
No previous history of surgeries.
Family History
The girl’s mother has had hypothyroidism since adoles-
cence, her father has had type 1 diabetes mellitus since
diagnosis at age 10, and she has a brother who was diag-
nosed as type 1 diabetes mellitus at age 5.
Current Status
In the ED, the child is postictal, slow to arouse, but main-
taining patent airway. Vital signs as follows: temperature
38.1 C (100.6 F) axillary, heart rate 112 bpm, blood
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232 Cases in Pediatric Acute Care

Table 54.1 Complete blood count. 3 Is hypoparathyroidism a common finding in children?

4 What therapies are used to treat hypocalcemia and
White blood cell count 9000/mm3
hypoparathyroidism?
Hemoglobin 12 g/dL
Hematocrit 36%
Platelet count 190 000/mm3 Rationale and Evidence-based Practice
Neutrophils 66% Explanation
Lymphocytes 22%
Monocytes 1% Are any other diagnostic studies recommended?
Eosinophils 10% Further studies to help determine the diagnosis include
measurement of serum vitamin D, 25-hydroxyvitamin
D and 1,25-dihydroxyvitamin D, as well as serum parathy-
Table 54.2 Basic metabolic profile. roid hormone (PTH), magnesium, and phosphorus levels. It
is also important to check levels of levetiracetam; if low,
Sodium 141 mEq/L
this could have been the reason for the seizure, but a low
Potassium 3.8 mEq/L calcium can also contribute to seizure activity.
Chloride 101 mEq/L There is a relationship between vitamin D and calcium.
Carbon dioxide 24 mEq/L Vitamin D is essential for utilization and absorption of dietary
Blood urea nitrogen 12 mg/dL calcium. In a vitamin D-deficient state, the amount of calcium
Creatinine 0.6 mg/dL absorbed is decreased, resulting in the increased production
Glucose 133 mg/dL and secretion of PTH. Tubular reabsorption of calcium in
the kidneys is decreased when PTH is low and increased with
Calcium 7.3 mg/dL
higher circulation of PTH. In a vitamin D-deficient state,
Ionized calcium 3.8 mg/dL
inadequate amounts of 1,25-dihydroxyvitamin D are pro-
duced, resulting in calcium production and utilization from
bone, which has other consequences. Another effect of higher
pressure 95/66 mmHg, and oxygen saturation 97% on 1 L/ levels of PTH is phosphaturia, resulting in hypophosphate-
min oxygen by nasal cannula. mia, where low levels of PTH will result in hyperphosphate-
Physical examination reveals the following. mia. Low calcium levels, associated with low PTH, also result

•• General: sleepy, slow to arouse, no acute distress.

Respiratory: clear breath sounds throughout, symmetric
in low magnesium levels.

chest rise, no wheezing. Based on the data given, what are the differential

• Cardiovascular: heart with regular rate/rhythm, no mur-

murs noted.
diagnoses and the most likely diagnosis for
this child?

•• Abdomen: soft, non-tender, bowel sounds hyperactive.

Musculoskeletal: full movement, normal strength
Differential diagnoses for this child include hypocalcemia
due to hypoparathyroidism, hyperparathyroidism, or vita-
and tone.

•
Neurologic: waking slowly, Glasgow Coma Scale (GCS)
score 12 at this time, no focal deficits.
A complete blood count, basic metabolic profile and
ionized calcium were obtained and the results are shown
in Tables 54.1 and 54.2.
Questions
Answer the following questions using the details provided.
1 Are any other diagnostic studies recommended?
2 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
min D deficiency. The most likely diagnosis for this child
is hypocalcemia due to hypoparathyroidism. Both parents
have endocrine disorders, so this could be a familial risk
factor. Hypocalcemia can be seen as a consequence of
severe or chronic vitamin D deficiency, but low serum cal-
cium is not usually associated with mild vitamin
D deficiency. A low calcium level resulting from hypopar-
athyroidism can also lead to magnesium deficiency, so fur-
ther studies are needed to document the diagnosis.
Is hypoparathyroidism a common finding in
children?
Hypoparathyroidism is a rare disease in any age group, but
especially so in children. It is characterized by low levels of
PTH and low levels of calcium with high circulating levels

of phosphorus. In children, hypoparathyroidism can result
from an autoimmune syndrome, can be genetically
acquired, or can be transient, most commonly seen in the
newborn period. Hypoparathyroidism has been associated
with DiGeorge syndrome or 22q11 chromosome microdele-
tion, can be X-linked recessive or familial or be associated
with other genetic syndromes. Hypoparathyroidism can
result from neck surgery, with damage to the parathyroid
or thyroid gland or surgical removal of the parathyroid
gland. Symptoms of hypoparathyroidism are the same as
those associated with hypocalcemia, including muscle
spasm, muscle aches, seizures, and syncope.
The pathophysiology of hypoparathyroidism is complex.
Simply, PTH increases serum calcium and decreases serum
phosphorus. PTH acts on both bone and kidneys to increase
serum concentrations of 1,25-dihydroxyvitamin D, which
indirectly stimulates calcium and phosphorus reabsorp-
tion. Hypothyroidism results in loss of both direct and indi-
rect effects of PTH, with effects on bone and kidney and
reabsorption of calcium and phosphorus, resulting in low
calcium and high phosphorus levels.
Further Reading
Hassan-Smith, Z. and Gittoes, N. (2017). Hypocalcaemia.
Medicine 55(9): 555–558.
Liu, M.J., Li, J.W., Shi, X.Y., Hu, L.Y., and Zou, L.P. (2017).
Epileptic seizure, as the first symptom of
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Chapter 54 Seizures in School-age Child 233
What therapies are used to treat hypocalcemia and
hypoparathyroidism?
A patient who presents with seizures as a result of hypocal-
cemia requires treatment with intravenous calcium, recom-
mended as calcium gluconate, with continuous cardiac
monitoring for arrhythmias in the intensive care unit. Once
calcium levels are near normal (at least 7.5 mg/dL), oral cal-
cium can be administered along with calcitriol, the active
form of 1,25-dihydroxyvitamin D, but the patient should
continue to be monitored for 24 hours to ensure that the
calcium is absorbed. Endocrinology referral and continued
care is also recommended.
Continuation of case: This 8-year-old girl is given cal-
cium gluconate intravenously (1.86 mg elemental cal-
cium per kilogram) over 10 minutes with a goal to
correct serum calcium to above 7.5 mg/dL. She is then
transferred to the pediatric intensive care unit (PICU)
and started on supplemental calcium with close monitor-
ing of her electrocardiogram and frequent monitoring of
her calcium levels.
hypoparathyroidism in children, does not require
antiepileptic drugs. Childs Nervous System 33(2): 297–305.
Winer, K.K. (2019). Advances in the treatment of
hypoparathyroidism with PTH 1–34. Bone 120: 535–541.
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235

55

An 18-Year-Old with Upper Respiratory Symptoms, Headache and Fever

Misty Evans
Vanderbilt College of Nursing, Nashville, TN, USA

An 18-year-old male presents with upper respiratory tract Family History

infection (URI) symptoms, nausea, headache, and neck
stiffness. Parents are alive and healthy with no contributory medical
history. Father of the teen does have hypertension, treated
with medication, and mother recently had a cold.
History of Present Illness

He reports having a sore throat and mild nasal congestion

over the past week. Today, he developed a severe headache,
Current Status
“the worst he had ever had.” Approximately 2 hours prior
Vital signs are as follows: temperature 37 C (98.6 F), heart
to admission, he developed severe headache, neck pain,
rate 120 bpm, respiratory rate 22 breaths per minute, and
and photophobia. He presents to the emergency depart-
blood pressure 89/36 mmHg. He appears clinically ill but
ment for further evaluation. He is a freshman college stu-
fully oriented. Nuchal rigidity was present on neurologic
dent who lives in the dormitory on campus. He has no
examination (positive Brudzinski and Kernig signs).
known sick contacts.
The results of basic metabolic profile, complete blood
count and coagulation profile are shown in Tables 55.1
and 55.2.
Past Medical History

The patient has no recent history of tick bites, viral illness,

or recent travel. No significant medical history. He reports
that he believes all immunizations are up to date; how-
ever, he does recall that he recently received his first
meningococcal vaccine and still needs to receive the sec-
ond dose.
Past Surgical History
The teen had a circumcision at birth, but no other surgical
procedures.
Questions
Answer the following questions using the details provided.
1 What are the significant abnormalities noted on physi-
cal examination and laboratory results?
2 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this teenager?
3 Are other diagnostic studies needed?
4 What is the best initial therapy for this patient?
5 What other therapies would be recommended at
this time?

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236 Cases in Pediatric Acute Care

Table 55.1 Basic metabolic profile. rigidity is the inability to place the chin to the chest and is
due to inflammation of the meninges. Abnormal laboratory
Sodium 138 mEq/L
results include normocytic anemia, thrombocytopenia,
Potassium 4 mEq/L prolonged prothrombin time (PT) and partial thromboplas-
Chloride 102 mEq/L tin time (PTT), elevated D-dimer, decreased fibrinogen,
Carbon dioxide 22 mEq/L elevated creatinine, hypocalcemia, hypomagnesemia, hypo-
Blood urea nitrogen 28 mg/dL phosphatemia, and elevation in transaminases. Thrombocy-
Creatinine 1.7 mg/dL topenia, prolonged PT and PTT, elevated D-dimer, and
Glucose 81 mg/dL
decreased fibrinogen are indicative of disseminated intra-
vascular coagulation (DIC) secondary to sepsis.
Calcium 7.8 mg/dL
Magnesium 1.2 mEq/L
Phosphorus 1.4 mg/dL Based on the data given, what are the differential
diagnoses and the most likely diagnosis for this
Aspartate transaminase 51 U/L
teenager?
Alanine transaminase 48 U/L
Alkaline phophatase 124 U/L The differential diagnoses include bacterial meningitis,
Gamma-glutamyltransferase 45 U/L viral meningitis, encephalitis, meningococcemia, focal
infections such as brain abscess, brain tumor, and infec-
Creatine kinase 181 U/L
tious thrombophlebitis. Because of immunization against
Lactate dehydrogenase 850 U/L
meningitis, the incidence of bacterial meningitis has
decreased, but it does still affect adults between the ages
Table 55.2 Complete blood count and coagulation profile. of 18 and 49. There are four main processes as the infection
invades the body: colonization, invasion in the blood-
White blood cell count 5500/mm3 stream, survival in the bloodstream, and then passage into
Hemoglobin 11.5 g/dL the subarachnoid space. The gold standard for diagnosing
Hematocrit 33% meningitis is evaluation of cerebrospinal fluid (CSF).
Platelet count 90 000/mm3 Table 55.3 includes normal results as compared to results
Prothrombin time (PT) 13.4 s
with bacterial and viral meningitis.
Fever, headache, and nuchal rigidity are typical
Partial thromboplastin time (PTT) 40 s
symptoms which occur with meningitis along with the
International normalized ratio 1.8
possibility of mental status changes. Infection can start
D-dimer 23 ng/mL with a fever and headache in a previously healthy individ-
Fibrinogen 100 mg/dL ual. Streptococcus pneumoniae, Neisseria meningitidis, and
Haemophilus influenzae type B are the most common
etiologies beyond the newborn period. Teenagers are
Rationale and Evidence-based Practice
specifically susceptible to Neisseria meningitidis and
Explanation Streptococcus pneumoniae. College campuses have
reported outbreaks of meningococcal disease caused by
What are the significant abnormalities noted on
N. meningitidis, which is also the organism found in bac-
physical examination and laboratory results?
terial meningitis. Profound alterations in consciousness
The physical examination reveals that this patient has are less common in viral meningitis than in bacterial men-
tachycardia, hypotension, and nuchal rigidity. Nuchal ingitis. Meningitis differs from other brain infections such

Table 55.3 Cerebrospinal fluid: typical results.

Opening WBC Predominant Protein

Appearance pressure (cmH2O) (/mm3) cell type (g/L) Glucose (mg/dL)

Normal Clear 10–20 <5 NA <0.4 2.6–4.5

Bacterial Cloudy, turbid, Raised >100 Lymphocytes Raised, Low
purulent >1.0
Viral Clear Normal or mildly Raised, Lymphocytes Mildly Normal or slightly
increased <1000 raised low

Chapter 55 An 18-Year-Old with Upper Respiratory Symptoms, Headache and Fever
as encephalitis, focal infections, and infectious thrombo-
phlebitis, which generally result in focal neurologic defi-
cits. The patient in this case had symptoms consistent
with meningitis (nuchal rigidity and headache) in addition
to evidence of infection resulting in mild DIC. The most
likely diagnosis for this patient is N. meningitidis bactere-
mia and meningitis. Neisseria meningitidis (meningococcal
disease) is the leading cause of bacterial meningitis in chil-
dren and young adults in the United States, with an over-
all mortality rate of 10–25% related to the urgency of
treatment. Meningitis is often associated with striking
morbidity including hearing loss, cognitive dysfunction,
visual impairment, educational difficulties, developmental
delays, motor nerve deficits, seizure disorders, and behav-
ioral problems.
Teenagers living in crowded conditions such as dormi-
tories and barracks are at high risk of infection. The
highest incidence of disease occurs during the winter
months when respiratory viral illnesses are at their peak.
Meningococci can colonize the upper respiratory tract of
healthy individuals, and infection occurs by either direct
contact or by respiratory droplets. Immunization with
two vaccines at least 6 months apart offers immunity
to meningitis type A and a newer vaccine has been devel-
oped to protect against meningitis caused by serotype B.
The pathophysiology of meningitis includes entry of the
bacteria typically through the respiratory system; the
bacteria often colonize the nasopharyngeal cavity and
then progress to the bloodstream. The infection crosses
the blood–brain barrier and proliferates in the CSF,
resulting in meningeal inflammation and increased
intracranial pressure. Much of the damage caused by
meningitis is believed to be caused by cytokines released
within the CSF as the host mounts an inflammatory
response.
Are other diagnostic studies needed?
Additional diagnostic studies include a blood culture to iso-
late the infectious pathogen, a chest radiograph to evaluate
for underlying lung pathology, and echocardiogram to eval-
uate underlying function. Continuous respiratory and
hemodynamic monitoring should be initiated.
Computed tomography (CT) of the brain is often used in
patients with neurologic changes or decreased level of con-
sciousness who are diagnosed with meningitis. It is fre-
quently recommended when there are broad or uncertain
differential diagnoses. Although a lumbar puncture is
standard of care for the evaluation and diagnosis of menin-
gitis and to assess intracranial pressure, this patient pre-
sents with significant coagulopathy and may be at risk of
bleeding if a this procedure is pursued. Polymerase chain
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237
reaction (PCR) assays can detect meningococcal DNA in
CSF, plasma, and serum. Sensitivity and specificity are over
90%. PCR testing is more sensitive than blood cultures and a
confirmatory diagnosis can be made in 4–8 hours. In this
case, PCR testing for meningococcal disease should be sent
on plasma or serum.
Serial laboratory monitoring will be needed in the acute
phase of illness. Blood glucose should be monitored closely
due to alterations during stress.
What is the best initial therapy for this patient?
Continuous respiratory and hemodynamic monitoring
should be initiated. Antibiotics including cefotaxime, cef-
triaxone and penicillin are the preferred medications to
treat meningitis, with penicillin as first-line therapy.
The New England Journal of Medicine has published a
study which recommends the use of high-dose dexametha-
sone in cases of suspected bacterial meningitis before (ide-
ally within 4 hours), and no longer than 12 hours following,
the first dose of parenteral antibiotics and which should
continue for 2–4 days (de Gans and van de Beek, 2002).
An elevated serum creatinine indicates acute kidney
injury in the presence of infection and septic shock. Phys-
ical examination also reveals hypotension and tachycardia.
Aggressive fluid replacement and vasopressor support is
indicated for sepsis/shock. Laboratory findings suggest
DIC in the setting of sepsis. PT, PTT and fibrinogen should
be monitored serially. The coagulopathies require correc-
tion with fresh frozen plasma and cryoprecipitate.
What other therapies would be recommended
at this time?
Vascular collapse and shock are frequent early manifesta-
tions, predominantly caused by the bacterial toxin. Cardiol-
ogy involvement is essential due to evidence of myocardial
dysfunction, which may be seen in sepsis. Correction of
electrolyte imbalance, volume resuscitation, and inotropic
support may improve cardiac function. The clinical mani-
festations of meningitis may vary in severity, ranging from
fever and bacteremia to fulminant disease with death
occurring within hours of the onset of symptoms. Early
treatment and aggressive supportive care is critical in the
treatment of patients with meningococcal disease.
Continuation of case: The patient is admitted to the pedi-
atric intensive care unit. On admission he was treated
empirically for suspected meningitis with ceftriaxone ini-
tially, then with penicillin. An echocardiogram revealed
mild left ventricular enlargement with decreased function,
and ejection fraction of 40% (normal 50–70%). A chest radi-
ograph demonstrates developing pulmonary edema.
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238 Cases in Pediatric Acute Care

Reference
de Gans, J. and van de Beek, D. (2002). Dexamethasone Journal of Medicine 347: 1549–1556. doi: 10.1056/
in adults with bacterial meningitis. New England NEJMoa021334.

Further Reading
Mcgill, F., Heyderman, R.S., Panagiotou, S., et al. (2016). Nadel, S. (2016). Treatment of meningococcal disease. Journal
Acute bacterial meningitis in adults. Lancet 366: of Adolescent Health 59(2 Suppl): S21–S28. doi: 10.1016/j.
3036–3047. jadohealth.2016.04.013.

56
Testicular Torsion
Amanda Mandel
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 13-year-old male presents to the emergency department
(ED) with acute left testicular pain at 4 a.m.
History of Present Illness
On arrival to the ED, the patient and his parents advise that
he awoke at 2 a.m. with acute, sudden left testicular pain.
The pain was severe enough to wake him from sleep and
contributed to vomiting. He also reports that the left side
of his scrotum is red, swollen and painful to touch. He finds
it difficult to walk. His parents called his primary care pro-
vider’s after-hours number and they directed him immedi-
ately to the ED. He denies dysuria and any trauma to the
scrotum, and is not sexually active. He has never had this
pain before.
Past Medical History
The teen is an otherwise healthy male, born full term, and
has received vaccines as required. He has no other signifi-
cant medical health problems.
Past Surgical History
He was circumcised as a newborn without complications.
Family History
No significant family history. Parents are both alive,
healthy and deny any family history of bleeding or anesthe-
sia problems.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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239
Current Status
The patient presents to the triage nurse in acute distress. He
is having difficulty walking and is hunched over and unable
to stand up straight. His vital signs include temperature of
36.7 C (98 F), heart rate 110 bpm, blood pressure 100/
70 mmHg, and respiratory rate 20 breaths per minute.
His weight is 48 kg. Urine sample is taken and results of uri-
nalysis are shown in Table 56.1.
The triage nurse immediately notifies the ED attend-
ing physician of the teen’s symptoms and he is immedi-
ately brought back to a room and examined. Most noted
on physical examination is his pain level and difficulty
ambulating. Heart and lungs are normal. Abdominal
examination indicates normal bowel sounds, soft to pal-
pation, non-tender, without rebound or pain at McBur-
ney point. The genitourinary examination is significant
for left scrotal swelling, with significant erythema,
exquisite tenderness to palpation, and testis difficult to
palpate due to pain but appears descended and has more
of a horizontal lie with absent cremasteric reflex. The
right scrotum appears normal, with testis descended
and non-tender to palpation (see Figure 56.1). Tanner
scoring is level II.
On completion of the physical examination, the ED pro-
vider promptly calls radiology for an immediate ultrasound
of the scrotum with Doppler and calls urology for an urgent
consult. Results of the scrotal ultrasound with Doppler are
as follows.
• Left testis: enlarged; measures 4.9 × 2.8 × 3.2 cm with a
calculated volume of 31.2 mL.
• Right testis: normal in size and echotexture with no focal
abnormality; measures 4.4 × 2.2 × 2.4 cm with a calcu-
lated volume of 16.5 mL.
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240 Cases in Pediatric Acute Care

Table 56.1 Urinalysis.

Color Clear, yellow

Specific gravity 1.030
pH 7.0
Nitrite Negative
Ketones Negative
Urobilirubin Negative
Leukocytes Negative
Protein Negative
Glucose Negative
Blood Negative

Figure 56.2 Intraoperative testicular torsion. Source: Courtesy of

T. Ernesto Figueroa, MD, Nemours Alfred I. duPont Hospital for
Children, Wilmington, Delaware.

the testis appeared quite dusky but improved after the tor-
sion was reduced. The torsion was reduced and the testis
was fixated. The right testis was also fixated. At the end
of the case, the left testis looked to be perfusing well.

Questions

Answer the following questions using the details provided.

1 Based on the data given, what are the differential diag-
noses and most likely diagnosis for this child?
2 Why was the child instructed to go directly and urgently
to the ED for assessment?
3 What additional diagnostic testing would be
considered?
4 When would urology be consulted?
5 What are the key educational points we need to discuss
with families regarding testicular torsion?
6 What is the long-term prognosis of boys with testicular
torsion?
Figure 56.1 External scrotal examination. Source: Courtesy of
T. Ernesto Figueroa, MD, Nemours Alfred I. duPont Hospital for
Children, Wilmington, Delaware.
Rationale and Evidence-based Practice

•
Normal blood flow and normal testicle on the right. Left
testis enlarged with no Doppler blood flow (venous or
arterial) to the testis.
The urology team was present during the ultrasound and
examined the patient and he was taken emergently to the
operating room for scrotal exploration. Surgery was started
at 5 a.m., 3 hours after the onset of symptoms. The left tes-
ticle was found to have a 360 torsion (Figure 56.2). Initially
Explanation
Based on the data given, what are the differential
diagnoses and most likely diagnosis for this child?
A patient who presents with unilateral testicular pain, usu-
ally with nausea and vomiting, should be managed imme-
diately, with clinical suspicion for acute testicular torsion
requiring immediate surgical exploration. If imaging is
not available, it is unnecessary to wait for an ultrasound

before calling surgery. The use of bedside ultrasound in the
ED is helpful with diagnosis but requires a skilled provider
to obtain images and assess results. Testicular torsion can
occur at any age, but is more common in adolescent males.
Differential diagnoses for this child include testicular tor-
sion, torsion of the appendix testis, and epididymitis. The
most likely diagnosis is testicular torsion where the testis
twists on itself. Testicular torsion presents as acute sudden
onset of significant testis pain. It will often wake a child up
from sleep and can cause vomiting. Torsion of the appendix
testis can also cause significant scrotal swelling, redness
and pain. Epididymitis would be unusual in a child who
is not sexually active, but can affect any age boy and
involves inflammation of the epididymis and causes pain
and inflammation of the testicles. It is always important
to rule out testicular torsion prior to accepting the diagnosis
of epididymitis, which is usually bacterial in origin and
treated with antibiotics.
Why was the child instructed to go directly and
urgently to the ED for assessment?
Time is of the essence in boys with testicular torsion as
there is no blood supply to the testis. The longer the testis
is not perfused, the less chance there is of being able to sur-
gically restore circulation. Any male patient who has testis
pain needs emergent evaluation.
What additional diagnostic testing would be
considered?
Scrotal ultrasound with Doppler is the primary diagnostic
test to evaluate a patient with testis pain. It is needed to
assess the blood flow to the testis and helps differentiate
torsion from a torsion of the appendix testis (which is
self-limiting and will resolve on its own). A urinalysis
Further Reading
Bowlin, P.R., Gatti, J.M., and Murphy, P. (2017). Pediatric
testicular torsion. Surgical Clinics of North America 97:
161–172.
Lee, L.K., Monuteaux, M.C., Hudgins, J.D., et al. (2018).
Variation in the evaluation of testicular conditions
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Chapter 56 Testicular Torsion 241
should also be done to rule out an infectious cause for
the pain.
When would urology be consulted?
In patients with high suspicion of testicular torsion, urology
should be called immediately after assessment in the ED.
This will help expedite emergent surgery if it is warranted.
What are the key educational points we need to
discuss with families regarding testicular torsion?
It is very important that boys and their families be educated
on testicular torsion during well child examinations. Four
out of ten young men who have testicular torsion will lose a
testicle, often due to a delay in evaluation. It is important to
stress to families that prompt evaluation is needed when-
ever a boy has complaints of scrotal pain. Boys should be
encouraged to tell their parents immediately if they ever
have testis pain. It is also important to perform a scrotal
examination on any boy who is complaining of abdominal
pain. Boys may not always endorse scrotal pain and an
examination is vital to make sure testicular pathology is
not missed.
What is the long-term prognosis of boys with
testicular torsion?
Patients who have had testicular torsion will need contin-
ued urologic follow-up after their surgery. The viability of
the affected testis long term will depend on the duration
of ischemia. There is a risk of testicular atrophy in patients
with testicular torsion that has been surgically corrected.
The affected testis is not always salvageable in the operating
room and an orchiectomy sometimes has to be performed.
Patients with solitary testis must use a protective scrotal cup
during sports.
across United States pediatric emergency
departments. American Journal of Emergency Medicine
36(2): 208–212.
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243

57

Teen with Fever and Rash

Christine Renke1 and Elizabeth Hoppe2
1
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA
2
Ann and Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

A previously healthy 16-year-old female presents to the Family History

emergency department (ED) with a 2-week history of tactile
fever and rash with progression to abdominal pain and
emesis yesterday.
History of Present Illness
She was adopted at 3 weeks of age and therefore the family
history is limited. Father with hypertension and high cho-
lesterol. Paternal grandfather with heart disease. No known
history of cancer. Mother’s medical history unknown.

She was in her previous state of health until approximately

Social History
2 weeks ago, when she developed a fever followed by a rash.
She was taking intermittent doses of acetaminophen with
Lives at home with adoptive parents and adopted sister. She
some relief. She describes her rash as limited to her nose
attends school and is in the 10th grade. Denies smoking,
and cheeks. It was dry and red in appearance. She has been
alcohol, or illicit drug use. Reports sexual activity with
applying lotion without change in the rash. She also reports
one partner.
puffy eyes and believes this may be related to her rash. She
denies any new lotions, shampoos or soaps, or laundry
detergents.
The day prior to presentation she reports non-bloody,
Current Status
non-bilious emesis, and was only able to keep some fluids
On arrival to the ED, she is non-toxic in appearance. Her
down. She denies diarrhea and reports a normal stooling
initial vital signs are as follows: temperature 38.3 C
pattern. She reports that she has been voiding less than nor-
(100.9 F) orally, heart rate 118 bpm, blood pressure
mal and her urine is darker than normal. She denies dysu-
140/88 mmHg, oxygen saturation 99% on room air, and res-
ria. No sick contacts and reports overall low energy
piratory rate 14 breaths per minute. She has generalized,
throughout the course of her symptoms.
non-focal tenderness and does not have hepatosplenome-
galy. You note a red rash localized to her face and extending
Past Medical History from cheek to cheek across the bridge of her nose with
nasolabial sparing. She has mild periorbital edema.
She has exercise-induced asthma and eczema, and takes Otherwise, her examination is unremarkable.
Her initial laboratory values are shown in
albuterol as needed for cough and wheezing. No history
Tables 57.1–57.3.
of hospitalizations.

Past Surgical History Questions

No surgical history. Answer the following questions using the details provided.

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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244 Cases in Pediatric Acute Care

Table 57.1 Basic metabolic profile. 3 Based on the initial laboratory evaluation, clinical
examination, and vital signs, what findings are
Sodium 135 mEq/L
significant?
Potassium 4.2 mEq/L 4 What specialty service(s) should be consulted to be
Chloride 104 mEq/L involved in her care?
Carbon dioxide 20 mEq/L
Continuation of case: She is admitted to the general pedi-
Blood urea nitrogen 20 mg/dL
atric floor due to her ongoing emesis and inability to remain
Creatinine 1.7 mg/dL hydrated with oral intake. It is noted that her blood pres-
Glucose 109 mg/dL sure remains elevated, with systolic blood pressure sus-
Alkaline phosphatase 92 U/L tained at 130 mmHg. The rest of her examination
Aspartate aminotransferase 26 U/L remains stable. The rheumatology evaluation reveals ele-
Alanine aminotransferase 34 U/L vated erythrocyte sedimentation rate (ESR), positive anti-
Albumin 2.5 g/dL nuclear antibody (ANA), positive anti-double-stranded
DNA, positive anti-Smith, positive anti-phospholipid anti-
Total protein 6.7 g/dL
body, direct Coombs positive, and low C3 and C4. Labora-
C-reactive protein 0.4 mg/dL
tory results ultimately diagnose her with systemic lupus
erythematosus (SLE) with suspected lupus nephritis.

Table 57.2 Complete blood count. 5 Are any other diagnostic studies warranted?
6 What interventions would you implement?
White blood cell count 3800/mm3
Hemoglobin 10.2 g/dL
Hematocrit 30.6%
Platelet count 164 000/mm3
Rationale and Evidence-based Practice
Neutrophils 69%
Explanation
Eosinophils 4%
What initial differential diagnoses should be
Monocytes 7%
considered?
Lymphocytes 9%
Given the non-specific presentation, differential diagnoses
Bands 0%
are broad and include viral gastroenteritis with dehydra-
Monocytes 6%
tion, nephrotic syndrome, pyelonephritis, urinary tract
infection, contact dermatitis, and autoimmune disorders.

Table 57.3 Urinalysis. What initial diagnostics test should be obtained?

Color Straw The initial differential diagnoses are broad, and therefore
pH 6.2 your initial diagnostic testing is also broad. You should
obtain a urinalysis, given hypertension and concern for uri-
Specific gravity 1.025
nary tract infection (UTI) or nephrotic syndrome, as well as
Clarity Cloudy
a complete metabolic panel to assess blood urea nitrogen
Glucose Negative
and creatinine as markers of renal function and hydration
Ketones Trace status. Finally, a complete blood count with differential can
Leukocytes Trace quickly help to assess for signs of infection, hematologic
Nitrites Negative disease, or abnormalities indicative of chronic disease.
Occult blood Moderate
Protein Large Based on the initial laboratory evaluation, clinical
examination, and vital signs, what findings are
significant?
1 What initial differential diagnoses should be The abnormal laboratory values of concern include leuko-
considered? penia with lymphocytopenia, mild anemia, elevated creat-
2 What initial diagnostics test should be obtained? inine, hypoalbuminemia, proteinuria, and hematuria.
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Chapter 57 Teen with Fever and Rash 245

Table 57.4 Revised criteria for classification of SLE.

Criterion Definition

Hematologic Anemia defined as >2SD below the mean for age and sex
Leukopenia: white blood cell count <4000/mm3
Thrombocytopenia: platelet count <100 000/mm3
Lymphopenia <1500/mm3
Hemolysis
Mucocutaneous Oral or nasopharyngeal ulceration, usually painless, observed by provider
Non-scarring alopecia
Subacute cutaneous or discoid lupus
Malar rash or maculopapular rash
Musculoskeletal Non-erosive arthritis or synovitis involving two or more peripheral joints, characterized by tenderness,
swelling, or effusion
Pleural or pericardial Imaging evidence of pleural effusion, or
effusion Pericarditis: documented by EKG or imaging evidence of pericardial effusion
Renal disorder Persistent proteinuria >0.5 g/day or
Cellular casts: may be red cell, hemoglobin, granular, tubular, or mixed
Class II, III, IV or V lupus nephritis on renal biopsy
Neurologic disorder Seizures: in the absence of offending drugs or known metabolic derangements, or
Psychosis: in the absence of offending drugs or known metabolic derangements
Delirium
Fever Temperature >38.3 C
Immunologic disorders Anti-DNA: antibody to native DNA in abnormal titer, or
Anti-Smith (Sm): presence of antibody to Sm nuclear antigen, or
Positive findings of anti-phospholipid antibodies on:

••
an abnormal serum level of IgG or IgM anti-cardiolipin antibodies

•
a positive test result for lupus anticoagulant using a standard method
or a false-positive test for at least 6 months confirmed by Treponema pallidum immobilization or
fluorescent treponemal antibody absorption test
Anti-nuclear antibodies ANA titer ≥1 : 80 or an equivalent positive test at least once
Complement Low C3 or C4 or low C3 and C4

SD, standard deviation.

The sustained hypertension and fever are abnormal vital
signs. On examination, her malar rash and periorbital
edema are also significant. The laboratory findings suggest
that the most likely diagnosis is an autoimmune disorder
with renal disease.
What specialty service(s) should be consulted to
be involved in her care?
When autoimmune disorders are suspected, the rheumatol-
ogy service should be consulted. Begin an autoimmune
evaluation and obtain ESR and ANA. Additional rheuma-
tologic tests may include anti-double-stranded DNA, anti-
Smith, anti-phospholipid antibody, direct Coombs, and
complement with C3 and C4. With the concern for renal
disease coupled with hypertension, the nephrology team
should be consulted.
The diagnosis of SLE is based on clinical manifestations
and laboratory testing. Diagnosis of SLE is defined by meet-
ing four of eleven criteria based on the American College of
Rheumatology 1997 Revised Criteria for Classifications of
SLE (Table 57.4).
This patient had the following criteria for diagnosis of
SLE: malar rash, persistent proteinuria, leukopenia, lym-
phopenia, thrombocytopenia, positive anti-double-
stranded DNA, and positive ANA test. She also presented
with evidence of lupus nephritis, including hematuria, pro-
teinuria, hypertension, and acute kidney injury.
Are any other diagnostic studies warranted?
Hypocomplementemia adds to the suspicion for lupus
nephritis, so renal biopsy is the gold standard for diagnosis,
classifying degree of nephritis (I–VI), and guiding therapy.
What interventions would you implement?
Renal involvement with SLE occurs in approximately 75%
of children, usually within first two years of disease. Spe-
cific treatment is formulated for each patient based on
organ involvement. Sunscreen is advised for all SLE

246 Cases in Pediatric Acute Care
patients to prevent exacerbation and non-steroidal anti-
inflammatory drugs (NSAIDs) help with fevers and arthri-
tis. With renal involvement, glucocorticoids are the first-
line therapy. Multiple side effects, including hypertension,
hyperglycemia, and immune suppression, have led to
improved steroid-dosing techniques such as pulse doses
or every other day doses. Immunosuppressive therapy
including cyclophosphamide and mycophenolate mofetil
Further Reading
Aringer, M., Costenbader, K., and Daikh, D. (2019). 2019
European League Against Rheumatism/American College
of Rheumatology classification criteria for systemic lupus
erythematosus. Arthritis and Rheumatology 71(9): 1400–
1412. doi: 10.1002/art.40930.
Petri, M., Orbai, A.-M., Alarcón, G.S., et al. (2012). Derivation
and validation of the Systemic Lupus International
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
(MMF) is second-line therapy (class III–V) and allows
hopeful reduction in steroid resistance. Class VI SLE
nephritis may require dialysis and renal transplant. Current
research is being conducted regarding benefits of autolo-
gous stem cell transplant for refractory SLE. Treatment of
hypertension secondary to SLE nephritis and steroid use
includes low salt diet and antihypertensives.
Collaborating Clinics classification criteria for systemic
lupus erythematosus. Arthritis and Rheumatism 64(8): 2677–
2686. doi: 10.1002/art.34473.
Wenderfer, S.E. and Eldin, K.W. (2019). Lupus nephritis.
Pediatric Clinics of North America 66(1): 87–99. doi: 10.1016/
j.pcl.2018.08.007.

58
Preterm Infant Unable to Wean from Mechanical Ventilation
Michelle Dokas
Children’s Hospital of Michigan, Wayne State University, Detroit, MI, USA
This is a 10-day-old infant born at 26 weeks’ gestation to a
gravida 1 para 0 mother after spontaneous rupture of mem-
branes. Birthweight was 620 g, current weight is 635 g.
History of Present Illness
The infant is intubated in an Isolette, is hemodynamically
stable, and has a right arm peripherally inserted central
catheter in place through which she is receiving total par-
enteral nutrition.
Past Medical History
Apgar scores were 3 and 7 at 1 and 5 minutes, respectively.
Amniotic fluid was noted to be clear on delivery. The infant
was intubated at birth. The mother’s membranes had
ruptured 14 hours before birth. Blood and respiratory
cultures sent at birth were negative. Mother had received
betamethasone prior to delivery.
Family History
Mother is 28 years old, married and received prenatal care.
She denies drug or alcohol use during pregnancy. She
smokes one and a half packs of cigarettes per day, and con-
tinued through pregnancy. No sexually transmitted dis-
eases noted, and no significant past medical history.
Current Status
The infant was intubated on the following ventilator settings:
FiO2 0.45, rate 50 breaths per minute, pressure control
10 cmH2O, pressure support 8 cmH2O, PEEP 4 cmH2O.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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247
Oxygen saturations were 92%. Most recent vital signs are
temperature 37.5 C (99.5 F), heart rate 182 bpm, respiratory
rate 74 breaths per minute, and blood pressure 72/28 mmHg.
Efforts were made to wean the ventilator towards extubation
with recurrent development of respiratory acidosis, tachyp-
nea, and increased work of breathing. Most recent laboratory
results are shown in Tables 58.1–58.3 and most recent chest
radiograph in Figure 58.1.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What additional testing would you order?
3 What are the treatment options for this diagnosis?
4 How would you determine if the therapy was effective?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
Differential diagnoses include viral pneumonia, broncho-
pulmonary dysplasia (BPD), PDA, congenital heart disease,
and meconium aspiration.
The most likely diagnosis for this neonate is PDA. This
infant is very premature. By day of life 7, 65% of neonates
born at 25–28 weeks’ gestation have failure of the PDA to
close. In addition, her PDA is hemodynamically significant
based on her widened pulse pressure, elevated creatinine,
and inability to wean off the ventilator.
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248 Cases in Pediatric Acute Care

Table 58.1 Basic metabolic profile. PDA occurs when the ductus arteriosus present in the
fetal circulation fails to close 48–72 hours after birth. The
Sodium 138 mEq/L
persistent blood flow from the aorta to the pulmonary
Potassium 4.1 mEq/L artery consists of a left-to-right shunt through the duct. This
Chloride 112 mEq/L leads to pulmonary over-circulation as the pulmonary vas-
Carbon dioxide 28 mEq/L cular pressures drop after birth, and an essential “flooding”
Blood urea nitrogen 22 mg/dL of the lungs. This increased flow to the lungs leads to a
Creatinine 1.1 mg/dL “steal” from the systemic circulation causing decreased
Glucose 78 mg/dL
blood flow to the mesentery and renal systems. This is man-
ifested by either low systolic and diastolic blood pressures
or adequate systolic pressure with low diastolic pressure
(widened pulse pressure). Complications from this left-
to-right shunting include necrotizing enterocolitis (NEC),
Table 58.2 Complete blood count. renal failure, pulmonary hemorrhage, IVH, and BPD.
White blood cell count 18 000/mm3
What additional testing would you order?
Hemoglobin 12 g/dL
Hematocrit 32.2% An echocardiogram to confirm the presence of the PDA, the
Platelet count 221 000/mm 3 size of the shunt (e.g. communication), and the direction of
blood flow between the vessels is required. The PDA can
only be ligated if the flow in the shunt is left to right, indi-
cating excessive blood flow from the systemic system to the
lungs. Right-to-left shunting of blood flow indicates high
Table 58.3 Arterial blood gas analysis. pulmonary pressures (greater than systemic) and is a con-
traindication to ligation. A respiratory viral panel to rule
pH 7.28 out infection can also be considered, although this diagno-
PaCO2 59 mmHg sis is unlikely given the absence of temperature instability.
PaO2 86 mmHg
Bicarbonate 29 mEq/L What are the treatment options for this diagnosis?
Lactate 1.6 mmol/L Pharmacologic therapy comprising cyclooxygenase inhibi-
Oxygen saturation 92% tors is the first-line intervention for PDA closure. Indo-
methacin and ibuprofen are the most studied agents.
However, indomethacin has been associated with NEC,
intestinal perforation, renal impairment, and platelet dys-
function. Other studies have investigated the use of ibupro-
fen and, more recently, acetaminophen. Ibuprofen also has
nephrotoxic effects and has been associated with pulmo-
nary hypertension, hyperbilirubinemia, and retinopathy
of prematurity. Surgical intervention with ductus ligation
can be used as primary intervention or as a secondary inter-
vention after failure of pharmacologic therapy. It provides
rapid and complete ductal closure, and is done at the bed-
side at most institutions in the United States. It usually
requires marked escalation of intensive care support in
the immediate postoperative period. Long-term complica-
tions of surgical intervention include left vocal cord paresis,
left hemidiaphragm paresis, chylothorax, and increased
incidence of BPD. An additional option for some infants
is transcatheter closure. This therapy is performed in the
cardiac catheterization laboratory and negates the need
for surgical closure. Venous access is established and an
Figure 58.1 Chest radiograph. occlusion device is placed directly in the PDA. Typically,
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Chapter 58 Preterm Infant Unable to Wean from Mechanical Ventilation 249

this is done in adults, children, and infants over 6 months of

age; however, research continues on using this method in
very low birthweight infants. Another management is
non-intervention in very low birthweight infants with
hemodynamically significant PDA since these babies may
have less incidence of BPD compared to infants undergoing
medical or surgical closure. Spontaneous closure rates are
low, and some infants require mechanical closure at later
ages. The incidence of NEC, IVH, and mortality is not sig-
nificantly different from that in earlier intervention. Very
limited evidence exists that the consequences of ductal
dependency over time can be ignored. When watchful wait-
ing is selected, careful fluid management and judicious use
of diuretics is needed.

How would you determine if the therapy was

effective?
Clinical signs of PDA closure include an increase in dias- Figure 58.2 Post-surgical chest radiograph.
tolic pressure, resulting in a narrower pulse pressure and
improvement in blood pressure. These changes can be
noted immediately after the surgical clip is applied. These
left-to-right shunting. Her follow-up creatinine level was
changes are also noted with successful medical treatment.
1.3 mg/dL and she continued with widened pulse pressure.
A gradual improvement in renal function is also noted after
Pediatric cardiovascular surgery was consulted and surgical
PDA closure because of improved systemic blood flow and
ligation was performed at the bedside. Her blood pressure
less pulmonary blood flow. Ventilator settings may be
immediately after the surgical clip was applied to the PDA
weaned as the left-to-right shunting is eliminated and pul-
was 80/38 mmHg with oxygen saturations of 95%. The
monary over-circulation is reduced. The chest radiograph
infant required increased ventilator settings the first night
after removal of the left-to-right shunt also demonstrated
after surgery but was subsequently weaned and extubated
improvement (Figure 58.2).
to non-invasive positive pressure ventilation 1 week post-
Continuation of case: The infant was given one dose of operatively. Her respiratory support was gradually weaned,
indomethacin. A repeat head ultrasound revealed a grade feedings were initiated and successfully advanced, she
II intraventricular hemorrhage (IVH), and repeat echocar- gained weight nicely, and was discharged home in stable
diogram revealed large patent ductus arteriosus (PDA) with condition by her due date.

Further Reading
Benitz, W.E. (2016). Patent ductus arteriosus in preterm
infants. Pediatrics 137(1): e20153730. doi: 10.1542/
peds.2015-3730.
Mitra, S., Florez, I., Tamayo, M., et al. (2016). Effectiveness and
safety of treatments used for the management of patent
ductus arteriosus (PDA) in preterm infants: a protocol for a
systematic review and network meta-analysis. BMJ Open 6
(7): e011271. doi: 10.1136/bmjopen-2016-011271.
Morville, P., Douchin, S., Bouvaist, H., and Dauphin, C. (2018).
Transcatheter occlusion of the patent ductus arteriosus in
premature infants weighing less than 1200g. Archives of
Disease in Childhood, Fetal and Neonatal Edition 103: F198–
F201. doi: 10.1136/archdischild-2016-312582.
Prescott, S. and Keim-Malpass, J. (2017). Patent ductus
arteriosus in the preterm infant. Advances in Neonatal Care
17(1): 10–18.
Sung, S., Chang, Y., Chun, J., et al. (2016). Mandatory closure
versus nonintervention for patent ductus arteriosus in very
preterm infants. Journal of Pediatrics 177: 66–71.

59
Toddler with Acute Respiratory Failure
Ahmed Aly and Bradley Tilford
Children’s Hospital of Michigan, Detroit, MI, USA
Patient is a 22-month-old boy with a history of reactive air-
way disease. He presents with acute respiratory failure with
hypoxia and hypercarbia requiring intubation and mechan-
ical ventilation.
History of Present Illness
Patient is a 22-month-old boy who developed low-grade
fever, occasional sneezing, and cough 2 days prior to admis-
sion. His mother has been giving him albuterol treatments
twice daily. Mother reports that early this evening after din-
ner, the family sat down on the couch for movie night with
popcorn and pretzels. After a short time, the patient lost
interest in the movie and began playing with the family
dog. He abruptly began gasping for air while climbing over
the dog onto the coffee table. Mother reports she responded
quickly by hitting the patient on the back. He began to
cough, but continued to have labored breathing, became
very agitated, and his lips turned blue. The patient went
limp while mother attempted to give him another albuterol
treatment. He was unresponsive to voice and his eyes rolled
into the back of his head. Mother immediately called the
emergency medical service (EMS) who arrived in approxi-
mately 10 minutes. EMS found him to be cyanotic and
hypoxic, with gasping respirations and a bounding pulse
on arrival to the scene. They provided 100% oxygen via
non-rebreather mask and transported him to the nearest
hospital.
Past Medical History
Admitted to the pediatric intensive care unit (PICU) at
13 months of age for viral pneumonia and wheezing,
requiring non-invasive positive-pressure ventilation. He
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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251
was administered continuous albuterol and steroids during
that admission.
Immunizations are up to date for age including annual
influenza vaccine. He is taking albuterol as needed and
montelukast daily. He has no known drug allergies.
Past Surgical History
None.
Social History
Lives with mother, maternal grandmother, and older sister.
No smoke exposure at home. Two dogs at home.
Current Status
On arrival in the emergency department (ED), the patient is
hypoxic with an oxygen saturation of 65% on 100% oxygen
via non-rebreather mask. He has poor respiratory effort and
a capillary blood gas analysis demonstrated pH of 7.1 and
PCO2 of 67 mmHg. The child is immediately intubated. Fol-
lowing intubation, a basic metabolic panel, complete blood
count, and portable chest X-ray are obtained. The results of
the laboratory investigations are shown in Tables 59.1 and
59.2 and the chest radiograph in Figure 59.1.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
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252 Cases in Pediatric Acute Care

Table 59.1 Basic metabolic profile. Initial ventilator settings of synchronized intermittent
mandatory ventilation (SIMV) pressure control mode, rate
Sodium 138 mEq/L
34 breaths per minute, pressure control 19 cmH2O, pres-
Potassium 3.5 mEq/L sure support 10 cmH2O, PEEP 5 cmH2O, inspiratory time
Chloride 105 mEq/L 0.7 seconds, FiO2 1.0. Initial vital signs as follows: heart rate
Carbon dioxide 20 mEq/L 135 bpm, respiratory rate 34 breaths per minute, blood pres-
Blood urea nitrogen 4 mg/dL sure 108/60 mmHg, and SpO2 100% on mechanical
Creatinine 0.32 mg/dL ventilation.
Glucose 140 mg/dL
His physical examination is significant for normal right-
sided breath sounds, but diminished breath sound on the
left, with mild expiratory wheezing. He is started on nebu-
lized continuous albuterol at 10 mg/hour and methylpred-
Table 59.2 Complete blood count.
nisolone 2 mg/kg daily. Ceftriaxone is started for suspected
White blood cell count 20 600/mm3 acute bacterial pneumonia. Because of the unusual findings
Hemoglobin 10.9 g/dL on lung examination and chest radiograph, otolaryngology
Hematocrit 33.9% (ENT) was consulted. A bedside flexible bronchoscopy
demonstrated complete occlusion of the left mainstem
Platelet count 393 000/mm3
bronchus with secretions and a visible, circular foreign
Neutrophils 27%
body. Child was subsequently taken for rigid bronchoscopy
Monocytes 7% and removal of foreign body. The foreign body was found to
Lymphocytes 63% be a popcorn kernel. It was successfully removed from left
mainstem bronchus on the first attempt. The child was
brought back to the PICU intubated. Albuterol, methypred-
nisolone, and ceftriaxone were stopped. He received dexa-
methasone for airway edema. He was successfully weaned
from mechanical ventilation and extubated 24 hours
following foreign body removal. He was discharged home
the following day in stable condition.
5 Would you use flexible versus rigid bronchoscopy in
evaluating a child with suspected foreign-body
aspiration?
6 Would you obtain other diagnostic studies?
7 Would you continue steroids and antibiotic treatment
following foreign body removal?

Rationale and Evidence-based Practice

Explanation

Figure 59.1 Portable chest radiograph of foreign body.
2 What is the pathophysiology associated with this
diagnosis?
3 What are the most common presenting signs and symp-
toms of this diagnosis?
4 What are the radiologic features of this diagnosis?
Continuation of case: Child is admitted to the pediatric
intensive care unit and placed on mechanical ventilation.
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for the cause of acute respiratory fail-
ure in this child include status asthmaticus, pneumonia,
and foreign body aspiration.
The most likely diagnosis for this child is foreign body
aspiration due to the sudden presentation of cough,
hypoxia, and gasping for air following eating popcorn.
The abnormalities localized to the left lung field on physical

examination and chest X-ray are consistent with this diag-
nosis. In asthma, there is often a history of recurrent symp-
toms with good response to bronchodilators and a positive
family history. Wheezing is typically audible in all lung
fields. Patients with pneumonia may have a history of fever
and respiratory distress, but no history of choking or acute-
onset symptoms. Physical examination and chest radio-
graph findings may be similar to foreign body aspiration.
Pneumonia may occur as a complication of foreign body
inhalation. Foreign body aspiration into the airway is a
common and dramatic pediatric emergency. It is most com-
mon in children aged 6 months to 5 years of age, predom-
inantly in children under 2 years of age. Immaturity of the
swallowing mechanism, small caliber of the upper airways,
curiosity of young children that is expressed by putting a
new object into the mouth, and an immature mastication
system are all factors in the occurrence of foreign body
aspiration.
What is the pathophysiology associated with this
diagnosis?
The pathophysiology depends on the type of foreign body
and location in the airway. The object itself may cause air-
way obstruction. A foreign body causing total obstruction
of the trachea/larynx may cause respiratory failure, car-
diac arrest, and death within minutes. Acute obstruction
at the level of the carina or mainstem bronchus may cause
lung collapse and hypoxemia. Pooled secretions caused by
partial obstruction may progress to acute bacterial pneu-
monia. Vegetable matter may swell over hours or days
leading to worsened airway obstruction. Organic foreign
bodies may produce inflammation and edema. A sharp
foreign body may lead to erosion, as is the case with pins.
What are the most common presenting signs
and symptoms of this diagnosis?
A clear history of aspiration (choking event) is the most fre-
quent presenting symptom. Complete obstruction can pres-
ent with:
• severe respiratory distress, inability to speak or cough,
cyanosis, altered mental status;
• universal choking sign.
Partial obstruction can present with:
• cough, tachypnea, stridor, focal/generalized mono-
phonic wheezing or decreased air entry, regional varia-
tion in aeration;
• “classic triad” of wheeze, cough, diminished breath
sounds.
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Chapter 59 Toddler with Acute Respiratory Failure 253
What are the radiologic features of this diagnosis?
The most common feature is lung atelectasis. Other signs
include air trapping, lung consolidation, and shifting of
the mediastinum. A radiopaque foreign body is only visible
in 20% of cases.
Would you use flexible versus rigid bronchoscopy
in evaluating a child with suspected foreign-body
aspiration?
The tracheobronchial tree should be examined in all cases
with a moderate or high suspicion of foreign body aspira-
tion, typically using rigid bronchoscopy so that the object
can be safely removed. Flexible rather than rigid bronchos-
copy may be used for diagnostic purposes in cases in which
the diagnosis is unclear, or if the foreign body is known but
the location of the object is unclear. In this case, most cen-
ters also arrange to have rigid bronchoscopy available on
standby because this is the preferred approach for foreign
body removal.
Would you obtain other diagnostic studies?
Foreign body aspiration is a medical emergency. Securing
the airway is the top priority. Proximally located foreign
bodies may be removed during direct laryngoscopy. Neck
and chest radiographs (both inspiratory and expiratory
films) and urgent ENT consult should be obtained once
the airway is secured. Fluoroscopy and computed tompgra-
phy (CT) do not improve diagnostic accuracy compared to
history, physical examination, and chest radiograph. CT
scan can be useful for diagnosing radiolucent foreign
bodies.
If foreign body aspiration is excluded from the differen-
tial diagnosis, then continued evaluation for viral or bacte-
rial illness is warranted. With the 2-day history of viral
prodrome in our patient scenario, that evaluation may
include viral nasal swabs/washes. Blood culture and
C-reative protein may be obtained to evaluate for bacterial
illness. Blood gas analysis may be required in patients
with respiratory distress.
Would you continue steroids and antibiotic
treatment following foreign body removal?
No medications are routinely needed. If significant swelling
is observed in the airway or if granulation tissue is present,
a corticosteroid (e.g. prednisolone, dexamethasone) may be
administered. Unless airway secretions are infected, anti-
biotics are not necessary.

254 Cases in Pediatric Acute Care
Further Reading
Boufersaoui, A., Smati, L., Benhalla, K.N., et al. (2013). Foreign
body aspiration in children: experience from 2624 patients.
International Journal of Pediatric Otorhinolaryngology 77
(10): 1683–1688. doi: 10.1016/j.ijporl.2013.07.026.
Committee on Injury, Violence, and Poison Prevention (2010).
Prevention of choking among children. Pediatrics 125(3):
601–607. doi: 10.1542/peds.2009-2862.
Donato, L., Hong Tran, T.M., Ghori, U.K., and Musani, A.L.
(2017). Pediatric interventional pulmonology. Clinics in
Chest Medicine 39(1): 229–238. doi: 10.1016/j.
ccm.2017.11.017.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Lowe, D.A., Vasquez, R., and Maniaci, V. (2015). Foreign body
aspiration in children. Clinical Pediatric Emergency Medicine
16(3): 140–148.
Shlizerman, L., Mazzawi, S., Rakover, Y., and Ashkenazi, D.
(2010). Foreign body aspiration in children: the effects of
delayed diagnosis. American Journal of Otolaryngology 31
(5): 320–324. doi: 10.1016/j.amjoto.2009.03.007.
Yang, C., Hua, R., Xu, K., et al. (2015). The role of 3D computed
tomography (CT) imaging in the diagnosis of foreign body
aspiration in children. European Review for Medical and
Pharmacological Sciences 19(2): 265–273.
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
255

60

Adolescent with Head Injury

Jennette Firlein
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

The patient is a 16-year-old male hockey player who pre- He has allergies to amoxicillin, cefdinir (Omnicef ) and
sents to the emergency department (ED) with a head injury. lactose.

History of Present Illness Past Surgical History

He was playing ice hockey 4 hours ago when he was Colonoscopy and biopsy 2 years ago. Circumcision at birth
elbowed in the right jaw and hit the left side of his head with revision (ureteral meatus opening) at 1 year of age.
on the boards at the hockey arena. He was wearing a helmet
at the time of injury, and there was no loss of consciousness
(LOC). Initial symptoms included dizziness and loss of bal-
Family History
ance. He was removed from the ice a few minutes later as
dizziness persisted. After the game, he experienced epi-
Mother with scoliosis and constipation, maternal grand-
staxis briefly. He experienced some numbness in his hands
mother with IBS, sister with gastroesophageal reflux, father
and feet that lasted about 20 minutes while transported to
with migraines, and brother with bipolar disorder.
the ED, but this resolved prior to arrival.

Social History
Past Medical History
Attends 11th grade, is an A/B student, and participates in
A concussion 2 years prior required 3 months to recover.
ice hockey year-round. No developmental delays, learning
Also has a history of irritable bowel syndrome (IBS), growth
disabilities, anxiety, depression, sleeping difficulties,
delay issues, pediatric migraine (none since 6 years of age),
speech therapy, or attention deficit disorder. Lives with
and asthma.
his mother, father, brother, and sister.
Current medications include:

•• amitriptyline (Elavil) 10 mg daily in the evening;

cyproheptadine (Periactin) 4 mg daily in the evening; Current Status
• tretinoin 0.05% cream applied to affected area daily at
bedtime; Review of systems is positive for epistaxis, dizziness, photo-

•• anastrozole 1-mg tablet daily;

albuterol (ProAir) 90 μg per two puffs as needed every
phobia, visual disturbance (peripheral blurred vision), nau-
sea, neck pain, headache (mainly in the frontal region), and
4 hours for wheezing; decreased concentration. He also complains of right clavi-

•• Lactaid 1-mg tablet as needed;

hyoscyamine 0.125-mg tablet as needed for cramping or
cle pain but has full range of motion in his right arm. He
received acetaminophen for his headache and used ice
diarrhea. for his sore neck during transport to the hospital. Glascow

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

256 Cases in Pediatric Acute Care
Figure 60.1 C-spine three-view radiograph.
Coma Scale (GCS) score is 15 on arrival. He is placed in a
cervical collar with complaints of neck pain and cervical
spine (C-spine) tenderness. Ibuprofen 600 mg oral tablet
and a C-spine three-view radiograph are ordered on his
arrival.
Vital signs are temperature 36.9 C (98.4 F), heart rate
89 bpm, respiratory rate 16 breaths per minute, and blood
pressure 113/60 mmHg. His weight is 56.7 kg.
The C-spine radiograph (Figure 60.1) showed that the
lateral distance between the C1 arch and the dens is larger
on the left than on the right, appreciated on the odontoid
(open mouth) view. The radiologist recommends further
evaluation with computed tomography (CT) examination.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
2
noses and the most likely diagnosis for this child?
Are any additional diagnostic studies indicated at
this time?
Continuation of case: The patient continued with nausea
and pain. He was given acetaminophen 975 mg oral tablet
and ondansetron (Zofran) 4 mg oral tablet. The trauma
team is consulted and evaluates the patient due to neck film
results. Trauma team recommends neurosurgery consult.
Neurosurgery reviews the case, stating the C-spine is most
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likely secondary to rotation of the C-spine during the radi-
ographic evaluation. They recommend follow-up with neu-
rosurgery as an outpatient. An hour later, the patient begins
to complain of increased right-sided facial pain despite ace-
taminophen and ibuprofen. He is also reporting increasing
head pressure focal to the left temporal region. One hour
later, he is in tears with increased left facial pain. There
are no new neurologic deficits noted.
The patient is experiencing persistent and worsening
headache and jaw pain. Neuroimaging is ordered due to
increasing intensity of neurologic symptoms despite appro-
priate management. Additionally, further imaging of the
cervical spine was recommended from X-ray results. Fur-
ther imaging included CT of head, facial bones, and cervical
spine, with results as follows.
• CT head/brain without contrast and three-dimensional
reconstruction: no acute intracranial abnormality, no
visualized calvarial fracture.
• CT cervical spine without contrast but with three-
dimensional rendering: redemonstration of minimal
asymmetry between the lateral masses of C1 and the dens
as described of unclear clinical significance. This finding
may be within normal limits and may be positional. Cor-
relate with physical examination and point tenderness. If
there is continued clinical concern for ligamentous
injury, MRI may be obtained. No evidence of fracture,
jumped facets, or rotatory subluxation.

• CT facial bones/mandible without contract but with
three-dimensional rendering: normal non-contrast CT
of the facial bones.
Neurosurgery reviews the CT, stating results were sec-
ondary to a neck spasm and rotation of C1 on the dens, with
no further evaluation warranted. The case is discussed with
the trauma team; given intractable pain and nausea, they
recommend admission for an overnight stay. At this time,
the patient receives 1000 mL of 0.9% saline and intermittent
intravenous dosing of ketorolac.
The patient is admitted and monitored overnight with
neurologic assessments and pain management every
4 hours. On arrival to the unit, he reports 5/10 pain and
nausea which decreases to 0/10 shortly thereafter. The
patient sleeps comfortably most of the morning, alert and
oriented when awake, and reports dizziness with getting
out of bed. The nurse also notes difficulties holding steady
gaze and some photophobia. Neuro checks are within nor-
mal limits. He is evaluated by rehabilitation medicine and
neurosurgery and diagnosed with concussion/mild TBI.
The patient has an abnormal vestibular ocular motor screen
on examination, and poor balance on single foot stance.
The patient tolerates lunch and vital signs remain stable.
Concussion education, discharge instructions, and a school
note are provided and reviewed with the family. The patient
is discharged home with the family.
3 What pain management strategies are used for
concussion?
4 What is the initial acute brain rest management for
concussion?
5 What recommendations for follow-up after discharge
would be made based on the diagnosis of concussion?
6 When would the patient be able to return to normal
activities after receiving a diagnosis of concussion?
7 What recommendations would be made for return to
school and sport after discharge?
8 What typical initial home medication management is
recommended after concussion?
9 What are the red flags for families to recognize and
would warrant return for evaluation?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
The differential diagnoses for this child are head injury,
concussion/mild traumatic brain injury (TBI), TBI, primary
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Chapter 60 Adolescent with Head Injury 257
headache, cervicalgia, cervical spine injury, and facial frac-
ture. The most likely diagnosis is mild TBI/concussion with
concomitant cervicalgia and/or cervical spine injury. The
symptom report is consistent with concussion or mild
TBI, so this case should not be classified as head injury only.
There was a report of trauma that resulted in rapid acceler-
ation of the brain through a hit to the face and a direct hit of
his head to the boards of the hockey arena. Symptoms sug-
gesting concussion are typically non-specific and can
include headache, fatigue, dizziness, nausea, self-limiting
vomiting, unsteadiness, feeling mentally slowed/foggy, dif-
ficulties remembering, trouble with concentration, distur-
bance of sleep, and emotional changes. This patient was
reporting headache, dizziness, nausea, blurred vision,
and difficulties concentrating. Because of clinical examina-
tion and low suspicion for intracranial injury (ICI), based
on Pediatric Emergency Care Applied Research Network
(PECARN) assessment, his diagnosis is less likely a TBI.
A tool that can be helpful in assessing athletes for concus-
sion on the sideline or on the field is the SCAT5 (Sport Con-
cussion Assessment Tool). This resource provides guidance
and immediate assessment, clinical follow-up, and recom-
mendations for returning to school and play after a
mild TBI.
Primary headache could be considered early in the differ-
ential diagnosis as headache can be a non-specific symptom
caused by several other etiologies, including cervicogenic,
migraine, tension, and/or temporomandibular joint injury.
At this time in assessment, there was known cervicalgia
and jaw pain related to the injury, which may be contribut-
ing to the patient’s report of headache. However, primary
headache can be excluded as there was a reported blow
to the face, as well as a direct hit to the head, suggesting that
the symptom is likely secondary to concussion.
With complaints of neck pain, C-spine tenderness, and
positive X-ray imaging findings, the differential should
include cervicalgia and C-spine injury. Cervicalgia is a term
for neck pain and stiffness often referred to as “whiplash.”
Are any additional diagnostic studies indicated at
this time?
According to the Centers for Disease Control (CDC), provi-
ders should not routinely obtain a head CT to diagnose mild
TBI in children. Recommendations suggest that use of vali-
dated clinical decision rules that assess multiple risk factors
are more effective in identifying children at low risk for ICI,
negating the use of CT, or those with higher risks, warrant-
ing a head CT. PECARN guidelines are an example of these
recommendations which assess a multitude of factors that
can influence the associated risk. Following the PECARN
guidelines, this patient is 5 hours from initial injury with
no focal neurologic signs, GCS score of 15, no vomiting,

258 Cases in Pediatric Acute Care
no LOC, mild mechanism of injury, no amnesia, and no
non-frontal scalp hematoma. While magnetic resonance
imaging (MRI) has increased the sensitivity in identifying
structural abnormalities compared to CT, it should not be
routinely used in the acute evaluation of suspected or
diagnosed mild TBI. MRI can often require sedation in
the pediatric population, and is more expensive than CT.
Skull X-ray should not be used in diagnosing pediatric mild
TBI. It is not the ideal test to diagnose a skull fracture with
ICI, specifically because it has a 63% sensitivity for diagnos-
ing a single skull fracture in children, cannot detect ICI,
and utilizes radiation.
There is currently no laboratory test that clearly identifies
concussion/mild TBI. Studies using advanced neuroimag-
ing tools, such as functional MRI and diffusion tensor ima-
ging, are being performed in an attempt to identify
potential brain biomarkers found in serum and genetic
tests. However, there is insufficient evidence to obtain
serum markers outside of a research setting for the sole pur-
pose of diagnosing a child with mild TBI.
What pain management strategies are used for
concussion?
Acutely, acetaminophen in doses of 15 mg/kg and ibupro-
fen in doses of 7.5–10 mg/kg are recommended. The patient
received 975 mg of oral acetaminophen, 4 mg of oral ondan-
setron for nausea, and 600 mg of oral ibuprofen. The patient
did not experience relief, reports an increase in headache,
and has a history of migraine. Patients with a history of
migraine may develop a migraine that is triggered by a head
injury. The physician has already identified red flags and
ordered imaging to exclude intracranial pathology. Treat-
ment should begin with a silent dark environment and
encouragement of sleep or rest. Next steps include intrave-
nous rehydration and medication. After a failed trial of oral
non-steroidal anti-inflammatory drugs (NSAIDs), intrave-
nous NSAIDs are a reasonable choice for migraine treat-
ment in an adolescent. Rehydration can specifically
prevent possible acute renal failure caused by NSAIDs such
as intravenous ketorolac. One study recommends using
ketorolac starting with 0.5 mg/kg to a maximum of
30 mg per dose. Ketorolac proved effective in half of the
patients within 1 hour of administration. Side effects are
not common with these medications and are therefore con-
sidered a first-line option. Other options include neurolep-
tics/antiemetics, which can have side effects such as
restlessness in the legs or body, or uncontrollable muscle
movements such as tics and tremors. Sumatriptan spray
is effective and can also be considered for the acute treat-
ment of migraine. There are inadequate data to make a
judgment on use of subcutaneous sumatriptan.
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Sumatriptan can produce pain/swelling at the injection
site, and other side effects can include redness in the face
and neck, burning feeling, feelings of tightness, and
drowsiness.
What is the initial acute brain rest management for
concussion?
Recommendation for initial management in mild TBI
includes brain rest for a brief period (24–48 hours) followed
by slow gradual return to normal school and daily activities.
Healthcare professionals should counsel the patient to limit
physical and cognitive activity during the first several days
after mild TBI and resume gradual return of activity with-
out exacerbating symptoms. Close monitoring of symptoms
during this time is recommended. During brain rest,
screens should be significantly limited, especially if they
trigger symptoms for the patient.
What recommendations for follow-up after
discharge would be made based on the diagnosis
of concussion?
The patient should follow up with their primary care pro-
vider 1–2 days after discharge from the ED. Patients should
not return to sport or gym/recess until cleared by a pro-
vider. CDC guidelines recommend referral to a specialist
if symptoms do not resolve after 3–5 days.
When would the patient be able to return to
normal activities after receiving a diagnosis of
concussion?
Healthcare providers should counsel families that 70–80%
of children with mild TBI do not show lasting symptoms
1–3 months after injury. Providers should also advise that
post injury, there is a lower threshold or vulnerability for
re-injury. Patients should return to activities as tolerated,
as long as they do not trigger symptoms. Patients will be
allowed to return to full activity after they have returned
to their premorbid performance and have remained symp-
tom-free at rest and with increasing levels of physical
activity.
What recommendations would be made for return
to school and sport after discharge?
Return to school after mild TBI needs to be individualized
and monitored closely by a collaborating team, while meet-
ing the academic needs of the child without significant
symptom exacerbation. Return to school should be gradual,
with accommodations to ease burden of return. Students
should be functioning at full days of school, symptom-free,

before they begin the step-by-step process for returning to
sports and physical activities.
What typical initial home medication
management is recommended after concussion?
Medication management comprising non-opioid analgesics
such as acetaminophen and ibuprofen is most effective in
treating headache in the home environment. Opioids are
not recommended as therapy for headache. Patients need
to be counseled about analgesic overuse (more than
15 times in a month) and possibility of rebound headaches.
Further Reading
Echemendia, R.J., Meeuwisse, W., McCrory, P., et al. (2017).
The Sport Concussion Assessment Tool 5th Edition
(SCAT5): Background and rationale. British Journal of
Sports Medicine 51: 848–850.
Lumba-Brown, A., Yeates, K.O., Sarmiento, K., et al. (2018).
Centers for Disease Control and Prevention guideline on the
diagnosis and management of mild traumatic brain injury
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Chapter 60 Adolescent with Head Injury 259
What are the red flags for families to recognize
and would warrant return for evaluation?
Families should be counseled about red flags that warrant
return for evaluation. These include drowsiness or inabil-
ity to rouse, unequal pupil sizes, headache that gets worse
and does not go away, slurred speech, weakness, numb-
ness, decreased coordination, repeated vomiting, convul-
sions, seizures, unusual behavior, confusion, agitation,
and LOC. For infants and toddlers, red flags would include
food refusal, inconsolability or any of the symptoms
listed above.
among children. JAMA Pediatrics 172(11): e182853. doi:
10.1001/jamapediatrics.2018.2847.
McCory, P., Messuwisse, W.H., Dvorak, J., et al. (2018).
Consensus statement on concussion in sport: the 5th
international conference on concussion in sport held in
Berlin, October 2016. British Journal of Sports Medicine 51
(11): 838–847. doi: 10.1136/bjsports-2017-097699.

61
Newborn with Ambiguous Genitalia
Barbara Wise
University of Maryland School of Nursing, Baltimore, MD, USA
An infant girl, born at 38 weeks’ gestation via normal
spontaneous vaginal delivery to a gravida 1, para 1, 27-
year-old mother, is now 24 hours old. She was admitted
to the neonatal intensive care unit (NICU) for evaluation
and management of ambiguous genitalia and
hypoglycemia.
History of Present Illness
This 24-hour-old infant weighed 2560 g at birth. At delivery
Apgar scores were 91 and 95, the infant only requiring suc-
tioning after delivery. Rupture of membranes occurred
1 hour prior to delivery.
Past Medical History
Maternal history includes prenatal care beginning at
10 weeks’ gestation, maternal blood type O positive, nega-
tive for group B Streptococcus, and negative for HIV and
sexually transmitted infections. No prenatal medications
except prenatal vitamins.
Neonatal history includes vitamin K 1 mg intramuscu-
larly once after birth to prevent vitamin K deficiency bleed-
ing, hepatitis B vaccine, and erythromycin ophthalmic
ointment 1 cm applied to both eyes after birth. Infant is
breastfeeding every 2–3 hours, has voided multiple times,
and passed a meconium stool. The infant was jittery at
2 hours, so a point-of-care blood glucose test was obtained
and found to be 50 mg/dL, with the need for continued
monitoring. Plan for first metabolic screening to be
obtained.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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261
Family History
Mother is 27 years old and has a history of asthma, with no
recent admissions or urgent care visits. Father is 30 years
old and in good health. Parents are married and both are
employed. This is a first child for both parents. There is
no previous history of endocrine disorders in the family,
infertility or consanquinity. However, a second cousin
has a son diagnosed with hypospadias and cryptorchidism.
Current Status
On admission to the NICU the physical examination
revealed atypical genitalia with clitoromegaly and posterior
labial fusion. Vital signs are temperature 36.5 C (97.7 F),
heart rate 172 bpm, respiratory rate 36 breaths per minute,
and blood pressure 60/30 mmHg. Length is 48 cm and head
circumference is 35 cm. The infant has mild jaundice to the
face, increasing irritability, and vomited twice after the
initial breastfeeding. The remainder of the physical
examination is unremarkable.
Results of complete blood count, basic metabolic profile
and steroid profile are shown in Tables 61.1–61.3.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What diagnostic studies should be obtained to confirm
the diagnosis?
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262 Cases in Pediatric Acute Care

Table 61.1 Complete blood count. Rationale and Evidence-based Practice

White blood cell count 11 500/mm3
Explanation
Hemoglobin 16.2 g/dL
Based on the data given, what are the differential
Hematocrit 45.9%
diagnoses and the most likely diagnosis for
Platelet count 205 000/mm3 this child?
Differential diagnoses for this newborn include congenital
adrenal hyperplasia, primary adrenal insufficiency, and
Table 61.2 Basic metabolic profile. ovotesticular disorder of sexual development.
The most likely diagnosis for this child is congenital adre-
Sodium 131 mEq/L nal hyperplasia (CAH, of which the most common cause is
Potassium 4.7 mEq/L 21-hydroxylase deficiency) based on the infant’s presenting
Chloride 99 mEq/L symptoms of ambiguous genitalia and laboratory studies
Carbon dioxide 25 mEq/L that indicate low sodium and chloride. It is important to dif-
Blood urea nitrogen 10 mg/dL ferentiate 21-hydroxylase deficiency from other less com-
Creatinine 0.2 mg/dL mon forms of CAH, including 11β-hydroxylase
deficiency, which occurs in 5–10% of children with CAH
Glucose 42 mg/dL
(presenting symptoms include virilization and hyperten-
Calcium 8.5 mg/dL
sion), and 3β-hydroxysteroid dehydrogenase deficiency, a
rare form that occurs in less than 1% of children diagnosed
with CAH (presents with salt wasting in males and females,
Table 61.3 Steroid profile.
but minimal virilization of the external genitalia).
The parents of this infant did not undergo genetic testing,
Test Normal values Results on day of life 6
as there were no prenatal concerns. For future pregnancies,
chorionic villous testing may be performed during the first
17-OHP <40 ng/dL 2400 ng/dL trimester to identify a mutation in the allele CYP21A2 that
ACTHa 0.7 pg/mL >9 pg/mL results in deficiency of 21-hydroxylase, the enzyme that is
Testosterone 20–62.8 ng/dL 1740 ng/dL necessary to convert 17-hydroxyprogesterone (17-OHP) to
11-deoxycortisol.
Androstenedione 20–300 ng/dL 1020 ng/dL
Cortisol 5–25 g/dL 11.3 g/dL
a
Pre-cortisol level of 0.9 g/dL and post-cortisol level of 5.9 g/dL. What diagnostic studies should be obtained
to confirm the diagnosis?
Newborn metabolic screening is available in all 50 states in
3 What therapies should be initiated for the neonate and the United States and is an appropriate tier one diagnostic
how would you determine if the therapy was effective? study for the diagnosis and management of CAH and
4 Would other studies need to be obtained? should be obtained at 48–72 hours of life. Newborn screen-
ing tests include an immunoassay to measure 17-OHP.
Continuation of case: The child was discharged home at
Exact cutoff values vary by laboratory and assay available
1 week of life after completing genetic testing, and the fam-
but are usually set low, resulting in a 1% positive rate of
ily provided with discharge instructions about administra-
detection. Approximately 1 in 100 neonates with a positive
tion of mineralocorticoid and glucocorticoid replacements
screening test will be diagnosed with CAH. Although 17-
and sodium chloride supplementation. At 3 weeks of age
OHP levels are initially elevated in most newborns and fall
the child was seen in the emergency department with a
rapidly in the first days of life, 17-OHP levels continue to
24-hour history of vomiting. The infant was tolerating
rise in children with CAH. Premature, sick, or stressed
breast milk feeds every 3 hours and had been tolerating
infants typically have higher levels of 17-OHP than term
the oral hydrocortisone.
infants and generate many false positives unless higher cut-
5 What other therapies would be recommended at offs are used. Antenatal corticosteroids used to stimulate
this time and are there other laboratory studies lung maturation in premature infants may reduce
indicated? 17-OHP levels. Premature infants should be retested after

several days of life related to inconsistent effects of antena-
tal steroids. Obtaining a blood sample prior to 48 hours may
cause a false-positive result.
What therapies should be initiated for the neonate
and how would you determine if the therapy was
effective?
The goal of therapy in the treatment of CAH is to decrease
excessive androgen secretion and prevent adrenal crises
and virilization. All patients diagnosed with classic CAH
should be treated with a combination of glucocorticoids
(hydrocortisone 20 mg/m2 daily) and mineralocorticoids
(fludrocortisone 0.2 mg/day) and 1–2 g daily of sodium
chloride supplements divided and administered with feeds
in the newborn period and early infancy. During the neo-
natal and early infancy periods, a higher dose of mineralo-
corticoids may be required because of immature kidney
function that contributes to poor water and sodium regula-
tion. Both prednisolone and dexamethasone should be
avoided to prevent growth suppression in children. Hydro-
cortisone solution is not adequate for the control of CAH in
children because of the uneven distribution of drug in a liq-
uid formulation. The glucocorticoid is usually administered
three times daily, with the highest dose (two-thirds total
dosing) administered in the morning to mimic the circa-
dian rhythm of cortisol secretion. During periods of illness
(gastroenteritis or fever associated with an ear infection) or
surgical procedures, the family should understand that
trauma/stress dosing (doubling or tripling) of the oral
steroid is necessary. Families should have an emergency
hydrocortisone intramuscular injection available for use
if their child is unable to take oral medications. Careful
monitoring for poor feeding, vomiting, lethargy, and sep-
sis-like symptoms is necessary to prevent life-threatening
hypovolemic shock. Adequate management of CAH is indi-
cated by normal linear growth, achievement of develop-
mental milestones, and prevention of salt wasting crisis.
Infants less than 6 months of age should be evaluated every
Further Reading
El-Maouche, D., Arlt, W., and Merke, D. (2017). Congenital
adrenal hyperplasia. Lancet 390(10108): 2194–2210. doi:
10.1016/S0140-6736(17)31431-9.
Levy-Shraga, Y. and Pinhas-Hamiel, O. (2016). High 17-
hydroxyprogesterone level in newborn screening test for
congenital adrenal hyperplasia. BMJ Case Reports 2016:
bcr2015213939. doi: 10.1136/bcr-2015-213939.
Speiser, P.W., Aziz, R., Baskin, L.S., et al. (2010). Congenital
adrenal hyperplasia due to steroid 21-hydroxylase
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Chapter 61 Newborn with Ambiguous Genitalia 263
6 weeks and then every 3 months until transitioning to
adult care to prevent intercurrent illnesses and support
growth.
Psychological support at the time of diagnosis is essential
to aid parents in decision-making, especially in children
with significant virilization of the external genitalia. The
multidisciplinary team, including endocrinology, urology,
gynecology, genetic counselor, psychologist, social worker,
and primary care providers, coordinate the clinical care
with patient/family preferences.
Would other studies need to be obtained?
This infant should have a metabolic panel obtained every
other day to monitor for hyponatremia and hyperkalemia
while starting sodium supplementation, mineralocorticoid
and glucocorticoids, with consideration of close monitoring
after discharge. A rapid fluorescence in situ hybridization
(FISH) test for the sex-determining region on the
Y chromosome can be obtained. The karyotype results in
this case revealed 46XX female. A pelvic ultrasound was
scheduled to evaluate internal reproductive organs, and
findings indicated a normal uterus and enlarged adrenal
glands, which exhibited a cerebriform pattern characteris-
tic of CAH in neonates.
What other therapies would be recommended at
this time and are there other laboratory studies
indicated?
The emergency department physician consulted with the
endocrinologist who recommended increasing the oral
hydrocortisone to 30 mg/m2 divided into four doses every
6 hours. A basic metabolic panel was obtained and vital
signs monitored with attention to the child’s blood pres-
sure. At discharge, parental education was reviewed regard-
ing adrenal insufficiency and the need for hydrocortisone
stress dosing in the future for intercurrent illnesses.
deficiency: an Endocrine Society clinical practice guideline.
Journal of Clinical Endocrinology and Metabolism 95(9):
4133–4160.
Webb, E.A. and Krone, N. (2015). Current and novel
approaches to children and young people with congenital
adrenal hyperplasia and adrenal insufficiency. Best Practice
and Research in Clinical Endocrinology and Metabolism 29:
449–468.

62
Anemia in a Toddler
Dyane Bunnell
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 20-month-old infant was referred for management of
abnormal laboratory values.
History of Present Illness
This 20-month-old infant was investigated at the Women
Infants and Children (WIC) office where she was found
to have a hemoglobin level of 4.8 g/dL. She was referred
to an outside hospital emergency department (ED) where
her hemoglobin level was confirmed to be 5.2 g/dL. Her
white blood cell (WBC) count was 117 000/mm3 and plate-
let count was 24 000/mm3. A basic metabolic profile (BMP)
was within normal limits at that time. She was started on
maintenance intravenous fluids during transport from
the outside hospital. Her mother also reported that she
has been pale and has bruised easily in the past month.
No recent history of fever. Her weight has remained the
same over the last 3 months. She does not take any medica-
tions at baseline.
Past Medical History
She was born full term with a birthweight of 3.005 kg after
an uncomplicated pregnancy and delivery. Her weight is at
the 26th percentile for gender and age and her body mass
index (BMI) is at the 16.3th percentile for gender and
age. Immunizations are up to date, including annual influ-
enza vaccination.
Past Surgical History
No prior surgical history.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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265
Family History
No siblings. No significant family history reported.
Social History
She lives with her mother, maternal grandmother, and 23-
year-old maternal uncle. Mother is in a relationship with
her father, but they are not married and he does not live
in the home.
Current Status
She is alert and playful with pale conjunctiva. She has hepa-
tosplenomegaly and bruising along her distal extremities.
The rest of her examination is unremarkable. A complete
blood count and BMP are obtained, with results as noted
in Tables 62.1 and 62.2. The chest radiograph is shown in
Figure 62.1.
On the chest radiograph the lungs are hypoventilated and
clear. There is no effusion of pneumothorax. The cardiothy-
mic silhouette is normal and the aortic arch is on the left.
There is no mediastinal adenopathy. The spleen appears
enlarged and the bowel pattern is unremarkable.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What emergencies may be associated with this
diagnosis?
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266 Cases in Pediatric Acute Care

Table 62.1 Basic metabolic profile. Rationale and Evidence-based Practice

Sodium 142 mEq/L
Explanation
Potassium 4.1 mEq/L
Based on the data given, what are the differential
Chloride 109 mEq/L
diagnoses and the most likely diagnosis for
Carbon dioxide 20 mEq/L this child?
Blood urea nitrogen 11 mg/dL
Differential diagnoses for this patient include infection,
Creatinine 0.3 mg/dL
bone marrow failure (aplastic anemia), and leukemia.
Glucose 94 mg/dL
New-onset leukemia is the most likely diagnosis due to
the presentation of bruising, pallor, hepatosplenomegaly,
and abnormal laboratory findings demonstrating anemia,
Table 62.2 Complete blood count.
thrombocytopenia and leukocytosis with evidence of blasts.
White blood cell count 142 200/mm3 Viral infection/bone marrow suppression is unlikely with-
Hemoglobin 4.8 g/dL
out focal signs of infection. Bone marrow failure such as
aplastic anemia is less likely given the presence of leukocy-
Hematocrit 13%
tosis rather than leukopenia.
Platelet count 26 000/mm3
Neutrophils 3%
What emergencies may be associated
Monocytes 2% with this diagnosis?
Lymphocytes 24%
Hyperleukocytosis is an oncologic emergency requiring
Blasts 68%
prompt recognition and intervention. Hyperleukocytosis
presents in up to 20% of children diagnosed with leukemia.
It is defined as a WBC count greater than 100 000/mm3 and
has been associated with early morbidity and mortality. The
elevated WBC count and presence of leukemic blasts that
do not deform easily may lead to leukostasis and occlusion
of small vessels. Leukostasis may present as intracranial
hemorrhage, stroke, acute kidney injury, or acute respira-
tory distress syndrome. Patients at highest risk for compli-
cations from hyperleukocytosis include those with acute
lymphoblastic leukemia (ALL) and acute myeloid leukemia
(AML), especially the monocytic (M4) and promyelocytic
(M5) subtypes. Patients with AML are more likely to be
symptomatic and suffer more complications from hyperleu-
kocytosis than those with ALL, even at WBC counts that
are considerably lower than those with ALL.
A secondary concern in the setting of hyperleukocytosis
is tumor lysis syndrome (TLS). TLS is an oncologic emer-
gency that is caused by tumor cell lysis with the release
of intracellular components including potassium, phos-
phate, and nucleic acids into the circulation. One or more
of these molecular abnormalities may lead to acute kidney
injury and cardiac arrhythmias.

Figure 62.1 Chest radiograph.

3 What are the goals of management and initial
interventions?
4 How would you determine if the interventions are
effective?
5 Which additional evaluations may be necessary?
What are the goals of management and initial
interventions?
Oxygenation and circulation need to be optimized. Intra-
venous hydration will be administered to promote circu-
lation. Interventions to reduce the WBC count including
chemotherapy and hydroxyurea may be administered.

Once the immediate threat of leukostasis secondary to
hyperleukocytosis is decreased, it is important to con-
servatively transfuse packed red blood cells (5 mL/kg
over 3 hours) to promote oxygenation; if the patient is
hemodynamically unstable, slow transfusion may be
needed prior to administration of cytoreductive therapy.
Ongoing assessment of pulse oximetry and physical
examination including neurologic status should be
monitored.
Anticipation of TLS is also important in this case. Fre-
quent laboratory evaluation, aggressive hydration, close
monitoring of renal function, and consideration of allopu-
rinol to prevent hyperuricemia or rasburicase to treat
hyperuricemia is necessary.
How would you determine if the interventions
are effective?
The effectiveness of initial interventions can be determined
by normalization of laboratory values, adequate fluid/elec-
trolyte balance, normal renal function, and the absence of
secondary complications. Frequent physical examinations
with attention to cardiac, respiratory, and central nervous
system function are important.
Which additional evaluations may be necessary?
Evaluation for an infectious etiology is also warranted.
Bone marrow failure and leukemia may be associated with
bacterial or viral illnesses. A close evaluation of the com-
plete blood count with differential and fever history/fever
Further Reading
Creutzig, U., Rossig, C., Dworzak, M., et al. (2016). Exchange
transfusion and leukapheresis in pediatric patients with
AML with high risk of early death by bleeding and
leukostasis. Pediatric Blood and Cancer 63(4): 640–645.
Kar, A. and Hijiya, N. (2018). Diagnosis and initial
management of pediatric acute leukemia in the emergency
department setting. Clinical Pediatric Emergency Medicine
19(2): 135–144.
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Chapter 62 Anemia in a Toddler 267
curve can assist in determining the need for antibiotics. If
febrile, obtain blood cultures and initiate broad-spectrum
antibiotics with pseudomonal coverage.
Continue close assessment of pulmonary signs and symp-
toms, such as dyspnea and hypoxia. Evaluation may
include chest radiograph and computed tomography (CT)
as needed to evaluate for interstitial or alveolar infiltrates
due to leukostasis. Changes in neurologic function (e.g.
visual changes, headache, dizziness, tinnitus, unsteady gait,
confusion, and somnolence) may indicate intracranial
hemorrhage. Continuous monitoring of the electrocardio-
gram is important given the risk of arrhythmias associated
with electrolyte derangements.
Close laboratory monitoring for disseminated intravascu-
lar coagulation, including prothrombin time (PT), partial
thromboplastin time (PTT), fibrinogen, fibrin split
products, and D-dimer, which may worsen following initi-
ation of chemotherapy. Consult pediatric hematology/
oncology to consider leukapheresis or exchange transfu-
sion, as there is conflicting evidence regarding the utility
and possible complications of these interventions. To eval-
uate the leukemia diagnosis, a bone marrow aspirate,
biopsy, and lumbar puncture should be obtained. Empiric
intrathecal chemotherapy will be administered during that
procedure. The marrow and cerebrospinal fluid will be
evaluated to further describe the disease, identify prognos-
tic factors, and guide therapy. After the results of diagnostic
studies are final, the initiation of chemotherapy should
commence, with vigilance in the prevention and manage-
ment of TLS.
Korkman, S. (2018). The management of hyperleukocytosis in
2017: do we still need leukapheresis? Transfusion and
Apheresis Science 57(1): 4–7.
Nguyen, R., Jeha, S., Zhou, Y., et al. (2016). The role of
leukapheresis in the current management of
hyperleukocytosis in newly diagnosed childhood acute
lymphoblastic leukemia. Pediatric Blood and Cancer 63(9):
1546–1551.

63
A 2-Year-Old with Fever and Cough
Jessica Strohm Farber
Children’s Hospital of Philadelphia, Philadelphia, PA, USA
A 2-year-old female with a history of prematurity presents
to the emergency department (ED) with cough, fever, and
increased work of breathing.
History of Present Illness
This 2-year-old female developed an upper respiratory
infection including low-grade fever, congestion, rhinor-
rhea, and cough that improved by day 4 of illness. On
day 6, she developed fever to 40 C (104 F), worsened pro-
ductive cough, malaise, and decreased appetite. On day 7 of
illness, she was evaluated by the pediatric provider for con-
tinued fever and productive cough who noted focal crackles
on examination. The child had post-tussive emesis,
decreased urine output, malaise, and poor appetite. She
was referred to the ED for further evaluation.
Past Medical and Surgical History
The patient was born at 29 weeks’ gestation, was intubated
at birth and remained on mechanical ventilation for
approximately 1 week, then transitioned to CPAP (contin-
uous positive airway pressure) for approximately 2 weeks.
She spent a total of 8 weeks in the neonatal intensive care
unit and has not had any major illnesses, surgical proce-
dures or hospitalizations since discharge. Immunizations
are current and the child does not have any known allergies
to any food or medications.
Family History
Both parents and grandparents are alive and healthy. There
is no family history of asthma or food allergies. The patient
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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269
has a 4-year-old sibling who has also recently had a cold but
is otherwise healthy.
Current Status
On arrival to the ED, the child is in moderate respiratory
distress with nasal flaring, intermittent grunting, and mod-
erate retractions. She is hypoxic with an oxygen saturation
of 89% in room air. Respiratory rate is 48 breaths per
minute. Evaluation of breath sounds indicates dense
crackles in the left lower lobe with no wheezing and no
other adventitious sounds.
The child has a temperature of 38.9 C (102 F) by oral
route, with a heart rate of 155 bpm. She is well perfused
but slightly cool peripherally. Capillary refill is 2–3 seconds.
Abdomen is soft, non-tender, and non-distended without
focal findings. She is neurologically appropriate for age
and situation.
Current treatment includes oxygen via nasal cannula.
A peripheral intravenous line is inserted, and blood work
and a chest radiograph are obtained. The patient remains
tachypneic with increased work of breathing and fever.
Results of complete blood count and basic metabolic pro-
file are shown in Tables 63.1 and 63.2. Anteroposterior and
lateral chest radiographs are shown in Figures 63.1
and 63.2.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
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270 Cases in Pediatric Acute Care

Table 63.1 Complete blood count.

White blood cell count 22 000/mm3

Hemoglobin 13 g/dL
Hematocrit 39%
Platelet count 435 000/mm3
Neutrophils 77%
Lymphocytes 8%
Monocytes 10%
Eosinophils 5%

Table 63.2 Basic metabolic profile.

Sodium 145 mEq/L

Potassium 4 mEq/L
Chloride 103 mEq/L
Carbon dioxide 19 mEq/L
Blood urea nitrogen 18 mg/dL
Creatinine 0.7 mg/dL
Glucose 85 mg/dL Figure 63.2 Lateral chest radiograph. Source: Courtesy of Cheryl
Bartke.
Calcium 9 mg/dL

Rationale and Evidence-based Practice

Explanation

Based on the data given, what are the differential

Figure 63.1 Anteroposterior chest radiograph. Source: Courtesy
of Cheryl Bartke.
2 What therapy may improve this child’s work of
breathing?
3 What additional studies may be indicated?
4 What antibiotic therapy is appropriate?
diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include a respiratory
viral illness such as bronchiolitis or viral pneumonia, ate-
lectasis, bacterial pneumonia, atypical pneumonia, pleural
effusion, aspiration, and status asthmaticus. The most
likely diagnosis for this child is a community-acquired bac-
terial pneumonia. The history of viral symptoms with a
period of improvement then worsening with fever and pro-
ductive cough provides the explanation for the develop-
ment of pneumonia. The respiratory rate and retractions
are physical examination findings that are considered reli-
able in the diagnosis of community-acquired pneumonia
(CAP) (Florin et al., 2017). Focal crackles on examination,
new fever after a period of defervescence, and hypoxia add
to the likelihood of pneumonia as a diagnosis. Without
audible wheezing and the presence of high fever, bronchi-
olitis, status asthmaticus and atelectasis can be ruled out as
possible differentials. The chest radiograph is suggestive of
left lower lobe infiltrate, with volume loss on the left side of
the chest. These findings, together with the clinical picture,
make the diagnosis of a bacterial pneumonia most likely.

This child also has a history of prematurity, and there may
be a component of chronic lung disease. However, there is
no evidence of chronic lung disease on the radiograph.
There is always a possibility for the presence of a pleural
effusion in a child with pneumonia, but no indication of
this is noted on the chest radiograph.
What therapy may improve this child’s work
of breathing?
In the presence of hypoxia, tachypnea, and increased work
of breathing, non-invasive positive pressure ventilation
(NIPPV) would be indicated to include supplemental oxy-
gen. NIPPV therapy such as CPAP or bilevel positive airway
pressure (BiPAP) suggests the need for intensive care unit
(ICU) admission or admission to a unit that is able to per-
form continuous cardiorespiratory monitoring (Bradley
et al., 2011). The need for NIPPV also indicates respiratory
failure or impending failure, so admission to the pediatric
intensive care unit (PICU) is definitely warranted for
this child.
What additional studies may be indicated?
A blood culture should be obtained in febrile children who
require hospitalization for CAP that is complicated or asso-
ciated with moderate to severe disease. A blood culture
should be strongly considered in children with comorbid-
ities, those who require ICU hospitalization, and those with
complicated pneumonia such as the presence of a pleural
effusion (Neuman et al., 2017). Testing for influenza and
other viruses can also be accomplished, but antimicrobial
therapy should not be withheld in the presence of markers
of bacterial infection such as radiologic evidence, clinical
evidence, or history concerning for a bacterial co-infection.
If radiologic findings or history are more suggestive of atyp-
ical pneumonia, then testing for Mycoplasma pneumoniae
should be considered as this may impact antibiotic selec-
tion (Bradley et al., 2011).
What antibiotic therapy is appropriate?
Knowledge of immunization status is important in the diag-
nosis and treatment of CAP, as missing vaccinations may
assist the provider in determining likely organisms to con-
sider for antimicrobial therapy. The most likely causes of
pneumonia in children between birth and age 2 include
primarily viral organisms, along with Streptococcus
pneumoniae, Mycoplasma pneumoniae and Chlamydia
pneumoniae. This child failed outpatient antibiotic therapy,
meets criteria for moderate to severe disease and is not yet
fully immunized, so a third-generation cephalosporin
administered parenterally (such as ceftriaxone) is indicated
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Chapter 63 A 2-Year-Old with Fever and Cough 271
for the treatment of both Gram-positive and Gram-negative
organisms. Vancomycin or clindamycin may be
considered as an additional antimicrobial for patients with
evidence or concern for Staphylococcus aureus infection. If
the clinical history and findings are more suggestive of
atypical pneumonia, then a macrolide such as
azithromycin can be administered (Bradley et al., 2011).
In this clinical case, ceftriaxone should be initiated for treat-
ment of left lower lobe pneumonia, with clindamycin or
vancomycin strongly considered given the presence of
moderate to severe pneumonia. A positive blood culture
with antibiotic sensitivities can also assist in guiding
treatment.
Continuation of case: The 2-year-old child is admitted to
the PICU and placed initially on non-invasive ventilation
with CPAP, (positive end-expiratory pressure) of 8 cmH2O
for hypoxia and labored breathing, then escalated to BiPAP
with settings of inspiratory positive airway pressure
+16 cmH2O and expiratory positive airway pressure
+6 cmH2O with 30% set oxygen. Ceftriaxone and vancomy-
cin are chosen for broad-coverage antibacterial therapy,
with the awareness that monitoring for therapeutic and
toxic vancomycin levels is necessary.
With BiPAP, the patient’s respiratory rate slows to
30 breaths per minute, but continues with mild subcostal
retractions. She is weaned to 21% oxygen, but remains on
BiPAP settings for work of breathing.
Additional Questions and
Evidence-based Rationale
5 What other therapies would be recommended at
this time?
6 What are indicators of clinical improvement?
What other therapies would be recommended
at this time?
Given the presence of fever, tachycardia, and history of
emesis, volume expansion with crystalloid boluses should
be considered, as a 20 mL/kg volume. Maintenance intra-
venous fluids should be initiated. The child should remain
nothing by mouth (NPO) if there is a concern for progres-
sion to respiratory failure. Airway clearance therapies could
also be considered for secretion mobilization. Additional
imaging, such as chest ultrasound, to evaluate for the pres-
ence of parapneumonic effusion or empyema should be
considered as their presence my require surgical interven-
tion (Bradley et al., 2011).
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272 Cases in Pediatric Acute Care

What are indicators of clinical improvement? of breathing and need for respiratory support should
improve. Heart rate should also normalize, and the fever
Clinical improvement would be evaluated by serial physical
curve should decrease. Repeat chest radiograph could be
examinations considering respiratory rate, work of breath-
considered if there is failure of clinical improvement.
ing, and oxygen requirement. As the child improves, work

References
Bradley, J.S., Byington, C.L., Shah, S.S., et al. (2011). The
management of community-acquired pneumonia in infants
and children older than 3 months of age: Clinical practice
guidelines by the Pediatric Infectious Diseases Society and
the Infectious Diseases Society of America. Clinical
Infectious Diseases 53(7): e25–e76. doi: 10.1093/cid/cir531.
Florin, T.A., Ambroggio, L., Brokamp, C., et al. (2017).
Reliability of examination findings in suspected community-
acquired pneumonia. Pediatrics 140(3): e20170310. doi:
10.1542/peds.2017-0310.
Neuman, M.I., Hall, M., Lipsett, S.C., et al. (2017). Utility of
blood culture among children hospitalized with community-
acquired pneumonia. Pediatrics 140(3): e20171013. doi:
10.1542/peds.2017-1013.

Further Reading
Dean, P. and Florin, T.A. (2018). Factors associated with Shan, W., Shi, T., Chen, K., et al. (2019). Risk factors for severe
pneumonia severity in children: a systematic review. community-acquired pneumonia among children
Journal of the Pediatric Infectious Diseases Society 7(4): hospitalized with CAP younger than 5 years of age. Pediatric
323–334. Infectious Disease Journal 38(3): 224–229.
Messinger, A.I., Kupfer, O., Hurst, A., and Parker, S. (2017).
Management of pediatric community acquired bacterial
pneumonia. Pediatrics in Review 38(9): 394–409.

64
Research Review
Lauren R. Sorce1,2
1
Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA
2
Northwestern University Feinberg School of Medicine, Chicago, IL, USA
The acute care provider in a busy pediatric intensive care
unit (PICU) has been asked to participate in a multiprofes-
sional clinical research study on biobanking. After meeting
with the research team, she has been identified as an inte-
gral part in the study through contributions to the develop-
ment and implementation of the clinical study. However,
she has not participated in clinical research at this level
and is uncertain of what is required for her participation
and does not really know where to start.
Questions
Answer the following questions using the details provided:
1 Who could help the provider learn about the research
process?
2 Where can the healthcare provider seek information
regarding education on the protection of human
subjects?
3 What education is required for the protection of human
subjects?
4 When are consents and assents required in the research
process?
5 How is child assent different from informed consent?
6 What responsibilities do those who participate in data
collection for a research study have?
Rationale and Evidence-based Practice
Explanation
Who could help the provider learn about the
research process?
• Members of the biobanking research team can provide
insight into why this work is important and how and
why her contributions will strengthen it.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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273
• Research nurses are formally or informally educated on
the conduct of research and can provide useful informa-
tion on study implementation.
• Nurse scientists are researchers who design and imple-
ment independent research and can facilitate learning
for those who wish to be part of data collection for a
research study.
• Clinical research coordinators are team members who
facilitate research in many hospitals by managing the
research studies for principal investigators.
Participating in the research process can be an over-
whelming task regardless of one’s role. At the graduate level,
formal classroom education on research is variable depend-
ing on the program of study. While many nurse practitioner
programs have transitioned to the Doctor of Nursing Practice
(DNP) degree, the focus of these programs is evidence-based
practice, not conducting research. Master’s degree programs
do include varying elements of the research process, but
unless the provider has a Doctor of Philosophy (PhD) degree,
formal education in research is usually limited. Yet, many
healthcare providers, including nurse practitioners, learn
about research on the job and have performed independent
research and participated in the research of others.
Where can the healthcare provider seek
information regarding education on the protection
of human subjects?
Critical to the performance of research is the protection of
human subjects. As such, education prior to the participa-
tion in a research study is required.
•
The Department of Health and Human Services website
contains information on federal policy for the protection
of human subjects called the Common Rule. More
specifically, the Office of Human Research Protections

274 Cases in Pediatric Acute Care
(https://www.hhs.gov/ohrp/) is responsible for protect-
ing the rights of human subjects and provides regulatory
oversight regarding the performance of behavioral and
biomedical research that is federally funded.
• The local institutional review board (IRB) is responsible
for approval of research specifically ensuring the protec-
tion of human subjects. Although some research
approved through the IRB may not be federally funded,
investigators must comply with federal regulations.
What education is required for the protection
of human subjects?
The primary program which outlines information on the
protection of human subjects for those conducting or par-
ticipating in research activities is called the Collaborative
Institutional Training Initiative (CITI) program. Individual
organizations such as hospitals and universities can use the
CITI training programs and/or outline their own specific
content.
• The CITI program (https://about.citiprogram.org/en/
homepage/) provides education on research ethics and
compliance. CITI training is required by nearly all IRBs
to ensure the researcher(s) are educated in the protection
of human subjects. Therefore, each healthcare or educa-
tional institution has access to this education. Renewal of
CITI training is required every 2 years.
• Good clinical practice (GCP) training is also provided by
the CITI program as a separate educational requirement
prior to engagement in research. This training is geared
towards the ethical conduct of clinical research (as
opposed to research in the laboratory) and scientific stan-
dards for study design, implementation and dissemina-
tion. The courses are devised based on the type of
research (e.g. investigational drugs, medical devices, clin-
ical research). Renewal of GCP training may vary by
institution, but many institutions allow 3 years until
expiration.
In addition to these educational requirements for clinical
research, institutions may have individualized require-
ments. Pediatric clinical research can be particularly com-
plicated as children are the research subjects yet the legal
authorized guardian, in many cases the parent(s), provides
consent. Although the rule for child assent may be different
state to state or institution to institution, child assent is nec-
essary for participation in research.
When are consents and assents required in the
research process?
Assent differs from consent in that assent is obtained from
someone (usually a minor) who is not legally able to
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provide consent. To obtain assent, one must provide the
information in an understandable way to the minor.
How is child assent different from informed
consent?
Recently, Tait and Geisser (2017) recommended the follow-
ing operational definition of assent given the overall lack of
consensus:
Children who lack the legal authority to provide
informed consent per state laws should provide their
assent to participate in a research study unless they
either lack the cognitive ability, their clinical condi-
tion precludes their ability to communicate a choice,
or the research holds out the prospect of direct ben-
efit that is only available in the context of the
research. Assent is an interactive process between
a researcher and child participant involving disclo-
sure of cognitively and emotionally appropriate
information regarding, at minimum, why the child
is being asked to participate, a description of the pro-
cedures and how the child might experience them,
and an understanding that participation in the study
is voluntary. Children should understand that they
can decline participation or withdraw from the study
at any time. Assent requires that the child explicitly
affirms his or her agreement to participate in a man-
ner that reflects their age-appropriate understanding
and that is free of undue influence or coercion. In the
absence of an explicit agreement, mere failure of the
child to object cannot be construed as assent.
Obtaining consent and/or assent in research is required
in any study where there is a posed risk to participants, such
as an interventional drug trial or when personal health
information will be used in research. Most institutions
require assent for children beginning at the age of 7 in addi-
tion to informed consent obtained from their parents or
guardian. Most institutions also offer templates or recom-
mendations for creating assent and consent forms, which
include required elements. The IRB often requires that con-
sent and assent forms be submitted with a research package
to be approved with the methodology of the study.
Another issue associated with pediatric research is tran-
sition of the subject to adulthood at 18 years of age. Any
subject participating in research who turns 18 years old
during the research study must provide consent for ongoing
participation. This becomes particularly problematic if the
subject cannot be located. If consent cannot be obtained,
the subject’s participation ceases.
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Chapter 64 Research Review 275

Relevant to this study on biobanking, the subject must be What responsibilities do those who participate in
consented upon turning 18 years of age, otherwise the sam- data collection for a research study have?
ples must be destroyed if they are linked to identifiable data.
Those healthcare providers who want to participate in
If the samples are not linked to identifiable data (described
research also need awareness of how to safeguard and pro-
by the Health Insurance Portability and Accountability Act;
tect the information related to a human subject. In most
https://www.hhs.gov/hipaa/for-professionals/privacy/
cases, this involves keeping the data locked or double
laws-regulations/index.html), the samples may continue to
locked, within a password-protected computer, or in a
be used. In a recent study on biobanking in the pediatric
locked desk drawer. It is the responsibility of the research
critical care setting, adolescents wanted to have a say in
team to maintain confidentiality of study participants at
the use of their samples (Paquette et al., 2018). If partici-
all times.
pants in any research study do not agree to all of the stipu-
lations outlined in the consent/assent process, and agree
with a witnessed signature, the participant cannot be a part
of the study.

References
Paquette, E.D., Derrington, S.F., Shukla, A., et al. (2018). Tait, A.R. and Geisser, M.E. (2017). Development of a
Biobanking in the pediatric critical care setting: adolescent/ consensus operational definition of child assent for research.
young adult perspectives. Journal of Empirical Research on BMC Medical Ethics 18: 41. doi: 10.1186/s12910-017-0199-4.
Human Research Ethics 13: 391–401. doi: 10.1177/
1556264618782231.

Further Reading
Collaborative Institutional Training Initiative (CITI) (2019).
https://about.citiprogram.org/en/homepage/ (accessed
19 June 2019).
United States Department of Health and Human Services.
Health information privacy. https://www.hhs.gov/hipaa/
index.html (accessed 19 June 2019).
United States Department of Health and Human Services.
Health information privacy: summary of the HIPPAA
Privacy Rule (2013). https://www.hhs.gov/hipaa/for-
professionals/privacy/laws-regulations/index.html
(accessed 19 June 2019).
United States Department of Health and Human Services,
Office for Human Research Protection (2019). https://www.
hhs.gov/ohrp/ (accessed 19 June 2019).

65
Toddler with Fever and Redness Behind Ear
Amanda Lewis Weir
Norton Children’s Hospital, Louisville, KY, USA
A 2-year-old boy presents to pediatric emergency depart-
ment (ED) with a chief complaint of “fussiness and redness
behind ear.”
History of Present Illness
He was seen last night in the same ED with a 1-week history
of runny nose, cough, and congestion and 2 days of fever up
to 40.5 C (105 F). On examination, he was found to have a
bilateral acute otitis media (AOM) and was discharged
home with amoxicillin 90 mg/kg daily for 10 days. On
the morning of presentation, the patient woke up again
with fever of 40.5 C and tugging on the right ear. On further
investigation, the mother noted that he had redness behind
his right ear and it appeared to be “sticking out” further
than his left ear. He has had decreased oral intake, but
has been urinating normally.
Past Medical History
He was born full term at 39 weeks and was discharged
home with his mother. He has a history of mild persistent
asthma, seasonal allergies, and recurrent otitis media. He
has been evaluated by pediatric infectious disease with con-
cerns for frequent infections. An evaluation for primary
immune deficiency was negative. He has no previous hos-
pitalizations. All immunizations are up to date, including
influenza.
His current chronic medications are albuterol 2.5 mg
nebulized every 4 hours as needed for wheezing, montelu-
kast 4 mg every evening, and cetirizine 5 mg every evening.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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277
Past Surgical History
He had myringotomy and placement of bilateral tympa-
nostomy tubes at 10 months of age, which fell out at approx-
imately 18 months of age.
Family History
The mother denies any pertinent maternal medical history.
The patient’s sibling also has a history of asthma. The
mother is unsure of medical history on the father’s side
of the family.
Social History
He lives at home with single mother and one older sibling.
His mother is an emergency medical technician. He attends
daycare. The mother denies any sick contacts at home but is
unsure of sick contacts at daycare. There is no tobacco expo-
sure in the home.
Current Status
On examination, he appears an ill toddler, lying in his
mother’s arms. He becomes appropriately agitated by the
examination. He has clear nasal drainage and occasional
congested cough. On otoscopic examination, both right
and left tympanic membranes are red and bulging with
purulence. Both external ear canals are normal without
redness and swelling. The right pinna is displaced upward
and outward and there is development of postauricular
swelling, redness, and tenderness. There is no obvious
abscess or fluid accumulation noted behind the right ear.
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278 Cases in Pediatric Acute Care

He does have mild end-expiratory wheezing on examina-
tion but has no retractions or tachypnea. His neck is with-
out signs of meningismus. His vital signs are within normal
range for his age, except for a temperature of
39.1 C (102.3 F).
Questions
Answer the following questions using the details provided.
1 Based on the history and examination what are the dif-
ferential diagnoses?
2 What is the likely diagnosis?
3 What diagnostics are helpful in making the diagnosis?
Rationale and Evidence-based Practice
Explanation
concern for sepsis, a blood culture should be obtained prior
to the initiation of antibiotics.
Acute mastoiditis can be diagnosed and treated without
radiographic evidence. However, if clinical examination
alone is unable to determine the severity of the infection
or if there is evidence of subperiosteal abscess or intracra-
nial involvement, imaging can be obtained. The two pri-
mary imaging modalities used with diagnosis of acute
mastoiditis are computed tomography (CT) with contrast
and magnetic resonance imaging (MRI) with gadolinium.
A CT with contrast is helpful in determining bony changes,
while MRI is used when there are clinical concerns for
intracranial involvement.
Continuation of case: A basic metabolic profile, CBC, and
CRP are obtained and the results are shown in Tables 65.1
and 65.2. CT with intravenous contrast is also obtained and
is significant for opacification of the mastoid air cells on the
right side (left side of Figure 65.1).

Based on the history and examination what are Additional Questions and Evidence-
the differential diagnoses? based Rationale
Differential diagnoses for this patient include mastoiditis,
parotitis, postauricular lymphadenitis, periauricular cellu- 4 What are the common pathogens of this diagnosis?
litis with or without otitis externa, periauricular inflamma- 5 What is the treatment for a patient with this diagnosis?
tion secondary to insect bite or trauma, mumps, or tumor.
Table 65.1 Basic metabolic profile.
What is the likely diagnosis?
Sodium 135 mEq/L
The likely diagnosis for this patient given concurrent AOM Potassium 4.6 mEq/L
with postauricular redness, edema, and tenderness and Chloride 102 mEq/L
upward and outward displacement of the left pinna is acute Carbon dioxide 30 mEq/L
mastoiditis. Acute mastoiditis is the most common compli-
Blood urea nitrogen 12 mg/dL
cation of AOM in children and affects 1.2–6 per 100 000 chil-
Creatinine 0.3 mg/dL
dren per year. It develops concurrently with AOM when
purulent material is unable to drain and blocks the commu- Glucose 94 mg/dL
nication between the mastoid cavity and the tympanic cav-
ity. As this purulence builds within the mastoid cells it
causes ongoing inflammation and breakdown of the mas- Table 65.2 Complete blood count.
toid bone, leading to acute mastoiditis. Often, patients will
develop a periosteal abscess secondary to the infection White blood cell count 35 000/mm3
and fluctuance behind the ear can be noted on examination. Hemoglobin 13 g/dL
Hematocrit 42%
What diagnostics are helpful in making the Platelet count 256 000/mm3
diagnosis? Neutrophils 72%
Both a complete blood count (CBC) and C-reactive protein Eosinophils 1%
(CRP) are non-specific for acute mastoiditis; however, an Monocytes 4%
increased CRP and elevated white blood cell count with left Lymphocytes 1%
shift on differential is often noted at the time of diagnosis. Basophils 1%
CBC and CRP can be helpful in distinguishing the severity Bands 11%
of acute mastoiditis. In those patients with a clinical

Figure 65.1 CT scan. Source: Courtesy of Dr. Karen Moeller.
What are the common pathogens of this
diagnosis?
The most common pathogens to consider when treating
acute mastoiditis include Streptococcus pneumoniae, Pseu-
domonas aeruginosa, Streptococcus pyogenes, Haemophilus
influenzae, and Staphylcoccus aureus. Less common patho-
gens, but still isolated in some cases, are Escherichia coli,
Acinetobacter, Enterococcus, Serratia, Klebsiella oxytoca,
and Moraxella catarrhalis.
What is the treatment for a patient with this
diagnosis?
An evaluation by an otolaryngologist or ear, nose and
throat (ENT) specialist is needed. While some studies show
Further Reading
Alkhateeb, A., Morin, F., Aziz, H., et al. (2017). Outpatient
management of pediatric acute mastoiditis. International
Journal of Pediatric Otorhinolaryngology 102: 98–102.
Bilavsky, E., Yarden- Bilvasky, H., Samra, Z., Amir, J., and
Nussinovitch, M. (2009). Clinical, laboratory and
microbiological differences between children with simple or
complicated mastoiditis. International Journal of Pediatric
Otorhinolaryngology 73: 1270–1273.
Chesney, J., Black, A., and Chou, D. (2014). What is the best
practice for acute mastoiditis in children? Laryngscope 124:
1057–1059.
Garcia, C., Salguerio, A.B., Luis, C., Correia, P., and Brito, M.J.
(2017). Acute mastoiditis in children: middle ear cultures
may help in reducing use of broad spectrum antibiotics.
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Chapter 65 Toddler with Fever and Redness Behind Ear 279
it is reasonable for patients who are well appearing
and without evidence of abscess to be discharged
home with outpatient management, most patients present-
ing with acute mastoiditis have already failed outpatient
measures for AOM and will need admission to the hospital
for parenteral treatment. With increasing prevalence of
Staphylococcus aureus, initial treatment should include
an anti-staphylococcal agent until culture and sensitivities
have been obtained. Initial antibiotic choices vary between
centers and regional antibiograms, but common initial
therapy often includes empiric amoxicillin/clavulanic acid
or a second- or third-generation cephalosporin such as
cefuroxime or ceftriaxone with or without the addition of
clindamycin. For patients with complicated acute mastoid-
itis, clinical judgment can be used in adding Gram-negative
coverage for those with high risk for Pseudomonas aerugi-
nosa and in the addition of antibiotic coverage for methicil-
lin-resistant Staphylococcus aureus. If it is possible to obtain
cultures from placement of tympanostomy tube, abscess
drainage, or mastoidectomy, antibiotics should be tailored
to susceptibilities.
Some patients with acute mastoiditis will require surgical
intervention. Often treatment involves myringotomy for
drainage of the middle ear with or without tympanostomy
tube placement for ongoing drainage. Patients with ima-
ging demonstrating a subperiosteal abscess will need inci-
sion and drainage by a trained provider. Some patients
may require mastoidectomy if treatment with myringot-
omy and intravenous antibiotics fails. Recent case reviews
show that older children have a longer duration of treat-
ment and are much more likely to need mastoidectomy
than younger children. This is likely because older children
take longer to present with clinical signs of acute
mastoiditis.
International Journal of Pediatric Otorhinolaryngology
92: 32–37.
Luntz, M., Brodsky, A., Nusem, S., et al. (2001). Acute
mastoiditis: the antibiotic era. A multicenter study.
International Journal of Pediatric Otorhinolaryngology
57: 1–9.
Marom, T., Roth, Y., Boaz, M., et al. (2016). Acute mastoiditis
in children: necessity and timing of imaging. Pediatric
Infectious Disease Journal 35: 30–34.
Mierzwinkski, J., Tyra, J., Haber, K., et al. (2018). Pediatric
recurrent acute mastoiditis: risk factors and insights into
pathogenesis. International Journal of Pediatric
Otorhinolaryngology 111: 142–148.
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281

66

A 5-Year-Old with Right-Sided Weakness

Suzette Meinke1 and Jessica Spruit2
1
Children’s Hospital of Michigan, Detroit, MI, USA
2
Wayne State University College of Nursing, Detroit, MI, USA

A 5-year-old female with sickle cell anemia (homozygous
SS type) presents to the emergency department (ED) with
right-sided weakness.
History of Present Illness
Mother reports that last evening she noticed the child was
“walking funny” but thought that she was just “off
balance,” until this morning when she noticed that the
child could not sit up by herself. There were no complaints
of pain or fevers. Mother denied any associated upper res-
piratory symptoms or any traumatic events. Mother reports
that the child was recently seen in the hospital for a history
of headache and right hand pain during which time a frac-
ture was excluded and they were sent home.
has no asthma-related hospitalizations and uses albuterol
metered-dose inhaler as needed for asthma symptoms.
Mostly recently, she presented to the ED with complaints
of headache and right hand pain. She reported waking
up with a mild headache and attending school when she
injured her hand on the bathroom stall. She had a radio-
graph of the right hand that did not demonstrate a fracture.
She was given intravenous ketorolac and experienced reso-
lution of pain. She remained stable overnight and was dis-
charged home the following day.
Past Surgical History
She has no prior surgical history.

Past Medical History
She was born in Florida as a full-term infant by cesarean
section. She spent no additional time at the hospital after
birth. She is up to date on her vaccines including Pneumo-
vax and her annual influenza vaccine. She has always been
considered a smaller than average child (weight percentiles
range from 1 to 10%) yet her body mass index (BMI) on
presentation was 15%. She has been admitted to the hospi-
tal for fever and pain related to her diagnosis of sickle cell
disease. Her first transcranial Doppler (TCD) assessment at
age 2 was normal. A repeat study was abnormal 1 year later,
although the subsequent study was normal. She was due for
an additional study at age 4 years but that was not com-
pleted. She was diagnosed with cough variant asthma at
age 3 years after a 1-month history of cough that was worse
at night and which did not respond to antihistamines. She
Family History
Her parents both have the sickle cell trait. Her brother is
4 years old and does not have sickle cell disease or trait.
Social History
Born in Florida and moved to Michigan at age 2 years with
her mother and brother. She is currently in kindergarten
and developing appropriately for her age.
Current Status
On arrival to the ED, she has a right gaze deviation and
right arm and leg weakness. Her vital signs are temperature
36.7 C (98.1 F), heart rate 117 bpm, respiratory rate

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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282 Cases in Pediatric Acute Care

24 breaths per minute, blood pressure 99/67 mmHg, and

oxygen saturation 98% on room air. She is alert, orientated,
and following commands. Deep tendon reflexes are brisk
on the right and normal on the left. Sensation is intact
through all extremities. Cough, gag, and corneal reflexes
are present. She has the ability to wiggle her fingers and
some of her toes but there is minimal large muscle move-
ment on the right side.

Questions

Answer the following questions using the details provided.

1 Based on the data given, what are the differential diag-
noses for this child and the most likely diagnosis?

Rationale and Evidence-based Practice

Explanation

Based on the data given, what are the differential Figure 66.1 Brain MRI. Source: Courtesy of Valarie Eichler.
diagnoses for this child and the most likely
diagnosis?
Trans-ischemic attack, complicated migraine, cerebrovas-
Table 66.1 Basic metabolic profile.
cular accident, and conversion disorder should all be con-
sidered when evaluating this patient. Based on the history Sodium 136 mEq/L
and presentation, the most likely diagnosis for this child is a Potassium 4.8 mEq/L
cerebrovascular accident (CVA).
Chloride 98 mEq/L
Continuation of case: The child was sent immediately for Carbon dioxide 24 mEq/L
magnetic resonance imaging (MRI)/magnetic resonance Blood urea nitrogen 14 mg/dL
venography (MRV)/magnetic resonance angiogram (MRA) Creatinine 0.13 mg/dL
of the brain that demonstrated an ischemic stroke in the left Glucose 74 mg/dL
temporal and parietal regions. She had asymmetrical nar-
rowing of the distal right internal carotid artery and a beaded
appearance of the middle cerebral arteries bilaterally with
possible collaterals (Figure 66.1). The pediatric intensive care Table 66.2 Complete blood count.
unit (PICU) and neurology teams were consulted and she
was transferred to PICU for close monitoring. Complete White blood cell count 20 100/mm3
blood count, comprehensive metabolic panel, and coagula- Hemoglobin 7.5 g/dL
tion studies were obtained (Tables 66.1 and 66.2). Hematocrit 21.6%
Her initial orders on arrival to the PICU included the
Platelet count 415 000/mm3
following:
Reticulocytes 26.5%

•• maintaining pulse oximetry above 92%;

permissive hypertension;
Prothrombin time (PT) 12.2 s

••
International normalized ratio 1.13
continuous cardiorespiratory monitoring; Partial thromboplastin time (PTT) <21 s
echocardiogram;

• normal saline intravenous fluids at 1.5 times
maintenance;
•• monitoring sodium and glucose every 4 hours;
preventing hypoglycemia/hyperglycemia and hyponatre-
mia/hypernatremia;
• monitoring for syndrome of inappropriate antidiuretic
hormone secretion (SIADH);
•• nothing to eat or drink by mouth;
occupational therapy consult for swallow study
evaluation;
•• partial exchange transfusion;
neuro checks every 1 hour, lay flat for 48 hours.
Additional Questions and
Evidence-based Rationale
2 What diagnostic screening is used to identify a child
who may be at increased risk for a stroke?
3 When a CVA is suspected in a child with sickle cell ane-
mia, what evaluation and immediate interventions
should be initiated?
4 What are the long-term treatment options for children
with overt strokes to help prevent subsequent strokes?
What diagnostic screening is used to identify a
child who may be at increased risk for a stroke?
Children with sickle cell anemia should be screened with
TCD imaging annually between 2 and 16 years of age.
TCD imaging detects increased velocities in the large intra-
cranial blood vessels, which is secondary to stenosis and
predictive of increased stroke risk. The results of TCD
can guide management; patients with abnormally elevated
velocity (called conditional or elevated depending on the
flow per second) may require chronic transfusions to pre-
vent primary stroke. If prevention is not provided, approx-
imately 10% of children with sickle cell anemia will
experience an overt stroke and an additional 20–35% will
have silent cerebral infarcts. Asymptomatic children do
not require screening with MRI or computed tomogra-
phy (CT).
The goal of chronic transfusion therapy is to reduce the
risk of primary stroke by maintaining the hemoglobin
S level less than 30%. Because of the risks associated with
chronic transfusions, a multicenter trial was conducted to
evaluate hydroxycarbamide in children with abnormal
TCD velocities and increased risk for stroke. The study con-
cluded that following 1 year of transfusion therapy and in
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Chapter 66 A 5-Year-Old with Right-Sided Weakness 283
the absence of severe vasculopathy, hydrocycarbamide may
be utilized rather than chronic transfusions to maintain
TCD velocities and prevent primary stroke.
When a CVA is suspected in a child with sickle cell
anemia, what evaluation and immediate
interventions should be initiated?
Children who have symptoms of a stroke should be evalu-
ated immediately by pediatric neurology and undergo diag-
nostic imaging, including a CT followed by MRI/MRA. The
stroke team should be activated, if in a center with this
availability. Once neuroimaging confirms an ischemic
stroke, initial therapy should be administered in consulta-
tion with a sickle cell expert. Intravenous fluids should be
initiated and an exchange transfusion should be performed.
The goal of the exchange transfusion is to decrease the
hemoglobin S level to below 30%. If it is not possible to per-
form an exchange transfusion, a standard packed red blood
cell transfusion may be administered, with caution to keep
the hemoglobin less than 13 g/dL to prevent further risk of
increased viscosity/sludging.
What are the long-term treatment options for
children with overt strokes to help prevent
subsequent strokes?
Children with sickle cell anemia who have a history of overt
strokes are treated with chronic transfusion therapy, which
is thought to decrease the risk of stroke by decreasing cere-
bral blood flow and oxygen extraction fraction. Chronic
transfusion therapy usually includes monthly simple or
exchange transfusions to maintain the hemoglobin
S level below 30%. Because of the risks of chronic transfu-
sions, ongoing monitoring including evaluation of liver
function, serum ferritin, infectious disease screening for
hepatitis B, hepatitis C and HIV, and evaluation of iron
overload should be performed according to periodic evalu-
ation recommendations.
Case Resolution
She spent several weeks in the PICU recovering from her
stroke. She had a repeat MRI 1.5 weeks after the CVA
and a head CT 3 weeks from the CVA. Figures 66.2
and 66.3 demonstrate the progression of the CVA. She
was transferred to inpatient rehabilitation for fur-
ther care.

284 Cases in Pediatric Acute Care
Figure 66.2 Brain MRI 1.5 weeks after CVA. Source: Courtesy of
Valarie Eichler.
Further Reading
Guilliams K.P., Fields, M.E., Ragan, D.K., et al. (2018). Red cell
exchange transfusions lower cerebral blood flow and oxygen
extraction fraction in pediatric sickle cell anemia. Blood 131
(9): 1012–1021.
National Heart, Lung, and Blood Institute (2014). Evidence-
based Management of Sickle Cell Disease: Expert Panel
Report. Available at https://www.nhlbi.nih.gov/sites/
default/files/media/docs/sickle-cell-disease-report%
20020816_0.pdf
Simon, E., Long, B., and Koyfman, A. (2016). Emergency
medicine management of sickle cell disease complications:
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Figure 66.3 Head CT 3 weeks after CVA. Source: Courtesy of
Valarie Eichler.
an evidence-based update. Journal of Emergency Medicine 51
(4): 370–381.
Ware, R.E., Davis, B.R., Schultz, W.H., et al. (2016).
Hydroxycarbamide versus chronic transfusion for
maintenance of transcranial Doppler flow velocities in
children with sickle cell anaemia, TCD With
Transfusion Changing to Hydroxyurea (TWiTCH): a
multi-centre, open-label, phase 3 non-inferiority trial.
Lancet 387: 661–670.

67
Postoperative Heart Block
Cathy Woodward
Long School of Medicine, University of Texas Health, San Antonio, TX, USA
A 10-month-old female is admitted to the cardiac intensive
care unit after complete repair of atrioventricular septal
defect (AVSD).
History of Present Illness
She is a 38-week gestation infant born to a 33-year-old
mother. At birth the diagnosis of trisomy 21 was confirmed
by chromosome microarray and a diagnosis of AVSD was
made. The infant went home with mother and struggled
with feeding, weight gain, and worsening heart failure
despite medical management.
Past Medical History
She has received all age-appropriate immunizations,
including influenza and palivizumab. Her birthweight
was 3.1 kg and current weight is 7.5 kg. She was bottle-
fed with 24 calories/ounce Similac Sensitive, and has just
started to take cereal. Current medications include multivi-
tamin daily, furosemide 3 mg orally twice daily, and digoxin
35 μg orally twice daily.
Current Status
Intraoperative report of brief third-degree heart block when
coming off cardiopulmonary bypass and atrial/ventricular
temporary pacing wires were placed and are securely
attached to a pulse generator that was not turned on imme-
diately postoperatively. She remained intubated on dexme-
detomidine drip 0.5 μg/kg per hour and milrinone 0.5 μg/kg
per min postoperatively.
Current vital signs are heart rate 130 bpm in sinus
rhythm, blood pressure 80/50 mmHg, and oxygen
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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285
saturations 100% on 50% oxygen through mechanical ven-
tilation. Approximately 6 hours postoperatively, her RN
calls you into the room because she has noticed that the
rhythm has changed. Vital signs have changed and are
now heart rate 60 bpm, blood pressure 55/30 mmHg, and
oxygen saturations 92% on the same mechanical ventilation
settings as previously postoperatively.
The electrocardiogram (EKG) rhythm strip is shown in
Figure 67.1.
Questions
Answer the following questions using the details provided.
1 What is your interpretation of the rhythm strip?
2 What are your first interventions?
Continuation of case: The cardiologist and surgeon are
notified of the change in the patient’s status. After these
changes, the rhythm shown in Figure 67.2 was noted.
A pulse is present with each contraction and vital signs
are improving.
Rationale and Evidence-based Practice
Explanation
What is your interpretation of the rhythm strip?
This strip demonstrates atrioventricular discordance or
third-degree atrioventricular block. The ventricular
response is only 60 bpm.
What are your first interventions?
Immediate treatment with basic life support is necessary,
including compressions and manual ventilations while

286 Cases in Pediatric Acute Care
Figure 67.1 Rhythm strip.
Figure 67.2 Rhythm strip with ventricular pacing.
the pulse generator is prepared. Since there is atrial electri-
cal activity which is being blocked and a slow ventricular
response, an emergent setting on the pulse generator (e.g.
pacemaker) would be ventricular pacing at a rate of
100–130 bpm based on vital signs and perfusion.
Additional Information
Atrioventricular block is a risk factor for children under-
going cardiac surgery. The surgeries most commonly asso-
ciated with heart block requiring permanent pacemaker
placement are the arterial or atrial switch and repair of
tricuspid or mitral valve. In one multicenter study, approx-
imately 4.1% of pediatric cardiac surgery patients developed
second- or third-degree heart block after surgery. In this
case, the patient underwent a repair of AVSD which can
involve the atrioventricular node conducting system. Often,
the heart block occurs immediately postoperatively due to
direct injury or to edema and inflammation around the
Further Reading
Liberman, L., Silver, E.S., Chai, P.J., and Anderson, B.R.
(2015). Incidence and characteristics of heart block after
heart surgery for pediatric patients: a multicenter study.
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surgical area. Since this infant had sinus rhythm on emer-
ging from surgery followed by development of atrioventric-
ular block, the prognosis was encouraging for return of
spontaneous conduction as the edema and inflammation
resolved. Temporary pacing is the best option and most cen-
ters place pacing wires in the operating room for high-risk
procedures. If pacing wires are not immediately available,
transcutaneous pacing could be attempted while making
surgical plans for placement of pacing wires. The decision
to place a permanent pacemaker is usually made 7–14 days
after surgery if the blocked rhythm continues.
Case Resolution
There was a return of conduction 3 days after surgery and
the pacing wires were removed on postoperative day 5 and
the infant continued to recover uneventfully from the sur-
gical procedure.
Journal of Thoracic and Cardiovascular Surgery 152(1):
197–202.
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287

68

Recognizing Human Trafficking

Christine DiPaolo
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

A 15-year-old adolescent girl was recently placed in foster
care when her mother trafficked her for sex in order to
pay the rent. She was last seen in this practice when she
was 13 years old for irregular periods.
History of Present Illness
This teen presents to the adolescent practice with
her foster mother for sexually transmitted infection
(STI) screening and birth control counseling. She
endorses vaginal discharge and odor. The patient was
recently placed in foster care after she disclosed to a
friend that for the past year she was having sexual inter-
course with her mother’s boyfriend and a couple of his
male friends in exchange for rent money. The teen’s
friend shared this with her mother who contacted the
police, resulting in a referral to family social services
and the teen being removed from her home. She was
not evaluated in the emergency room since the abuse
had occurred over 72 hours previous to family services
involvement.
The teen has not had contact with her mother up to this
point. She admits to smoking marijuana and taking “pills”
sometimes. She was getting marijuana and other medicines
from her mother’s boyfriend, and is not sure what the pills
were but thinks Xanax. The patient states, “It made it easier
to be with them.” She reports the last time she had sex was
2 weeks ago and “they never used condoms. They didn’t
care if I got pregnant. Sometimes they would laugh at the
possibility of my getting pregnant.” She reports being a
virgin prior to having sex with her mother’s friends. She
reports it was mostly vaginal sex and sometimes oral.
Denies anal sex.
Past Medical History
The patient was born at term and has had no previous sig-
nificant medical history. Immunizations current except
human papillomavirus (HPV) has not initiated. Has never
been hospitalized.
She experienced initial menses at 12 years old. Periods
are monthly, lasting 5 days. She uses four tampons a day
and they are not soaked. She has cramps but does not need
medication. Last menstrual period was 2 days ago.
Past Surgical History
No previous surgical procedures.
Family History
Mother has addiction and was diagnosed with bipolar dis-
order. Father’s history is unknown, as is history of paternal
grandmother and paternal grandfather. Sister aged 4 years
is healthy. Maternal grandmother with depression, mater-
nal grandfather has history of addiction
Social History
Currently lives with foster mother, foster father, 14-year-
old foster sister, and her 4-year-old biological sister. Prior
to foster placement she was living in a two-bedroom apart-
ment with mother, mother’s boyfriend, and her 4-year-old
biological sister. She denies being physically abused, except
for forced intercourse and other sexual activities. The
patient admits to smoking marijuana almost daily which
she receives from mother’s boyfriend. Has not smoked

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

288 Cases in Pediatric Acute Care
for the past 2 weeks since being in foster care. Feels she does
not miss it. She also takes “pills” that she thinks are Xanax
“once in a while.” Last used 2 months ago. She denies
alcohol or any other drug use, also denies juuling or vaping.
The teen does report having a boyfriend, who attends her
school, for the past 3 months. Feels they have a good rela-
tionship and is having consensual sex with him. Is not using
condoms consistently in this relationship.
The teen is in 10th grade. She used to be a B student but
now gets Cs and Ds. Her favorite subject is science. She likes
to dance and listen to music.
Current Status
The only positive in the review of systems is genitourinary/
gynecology, with lower persistent abdominal pain with vag-
inal discharge. The physical examination is basically nor-
mal except for the pelvic examination, which indicates
pale thick discharge noted at the cervical os, positive for
cervical motion tenderness.
Diagnostic testing for STI is positive for Chlamydia, and
the patient is treated for pelvic inflammatory disease with
Doxycycline 100 mg orally twice daily for 14 days and intra-
muscular ceftriaxone 250 mg. Blood test for HIV and rapid
plasma reagin (RPR) test for syphilis are completed and
prove negative.
The patient is counseled about birth control and Nexpla-
non placed per patient decision.
Questions
Answer the following questions using the details provided.
1 Where does human trafficking occur and what different
types exist?
2 What signs or complaints are common when children
who are being trafficked come for healthcare?
3 What screening can be done in any environment to
identify children who are being trafficked?
Rationale and Evidence-based Practice
Explanation
Where does human trafficking occur and what
different types exist?
It is important for providers in any environment to under-
stand human trafficking and its implications for children
and teens. These patients can present to any healthcare
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institution, including acute care settings. Human traffick-
ing is defined by the United States Victims of Trafficking
and Violence Protection Act as the “recruitment, harbor-
ing, transportation, provision or obtaining of a person for
labor or services through the use of force, fraud or coercion”
(US Trafficking in Persons Report, 2019). Commercial sex-
ual exploitation of children, otherwise known as child sex
trafficking, is a major public health issue in the United
States and globally. Children can be sexually trafficked over
international borders, within the United States, a state or a
single neighborhood, or in this case a specific home.
Domestic minor sex trafficking (DMST) refers to those chil-
dren or teens who are US citizens under the age of 18 who
are trafficked within the borders of the United States.
It is difficult to document the prevalence of DMST as
there are many variables, especially related to reporting,
but one source conservatively estimates that 200 000 US
minors are involved (Goldberg and Moore, 2018). Risk
factors for involvement include history of child abuse
or neglect, family dysfunction or conflict, homelessness,
foster care, LGBTQ, legal system involvement, peer pres-
sure, social isolation, gang involvement, and under-
resourced schools, neighborhoods and communities
and overall lack of resources. Knowledge deficit or lack
of awareness of commercial sexual exploitation is
another factor. In this case, the patient had a relationship
with the traffickers and the parent and children were at
risk with a lack of financial resources to survive. Traffick-
ers can have a relationship with the victim or can be
strangers. In addition, victims of human trafficking are
often forced to use illicit drugs, such as the marijuana
and Xanax given to this teen, which may in turn result
in an addiction problem for the teen. The use of drugs
can be coercive on the side of the trafficker and/or a cop-
ing mechanism for the child.
Healthcare providers need to be aware and sensitive to
the possibility of human trafficking, especially when chil-
dren present to a non-familiar clinic, practice, urgent care
or emergency department with concerns of drug or alcohol
abuse, STIs, suicidal ideation or physical trauma. Youth
may be fearful of their exploiters, distrust the clinician,
or have an interest in protecting their traffickers, so will
not describe their experiences or the real reasons for their
complaints. Providers are often not trained nor equipped to
acknowledge these victims.
What signs or complaints are common when
children who are being trafficked come for
healthcare?
Signs of involvement in DMST include the presence of
tattoos, evidence of physical abuse or neglect such as

malnutrition or wound infections, multiple or recurrent
STIs, multiple sexual partners, inappropriate clothing or
expensive items such as shoes or purses, and cutting or other
self-harm behaviors. Psychological or mental health signs
can include depression, withdrawal, fear, distrust and indi-
cations of illicit drug or alcohol use. In addition, there are
social signs such as controlling behavior on the part of the
person accompanying the patient, history of running away
from home, or involvement with child protection services.
What screening can be done in any environment to
identify children who are being trafficked?
Interviewing the child away from the adult who accompa-
nies the patient is important; asking the adult for identity
References
Goldberg, A. and Moore, J. (2018). Domestic minor sex
trafficking. Child and Adolescent Psychiatric Clinics of North
America 27(1): 77–92. doi: 10.1016/j.chc.2017.08.008
Further Reading
Moore, J.L., Kaplan, D.M., and Barron, C.E. (2017). Sex
trafficking of minors. Pediatric Clinics of North America 64
(2): 413–421. doi: 10.1016/j.pcl.2016.11.013
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Chapter 68 Recognizing Human Trafficking 289
and confirming this identity is also important, although this
may be difficult in a “walk-in” setting, where this type of
questioning may cause the patient to leave without treat-
ment. The child should also be asked in confidence about
where they are living, if they have food, shelter and cloth-
ing, attending school and other activities, forced or coerced
sexual experiences, having sex with someone other than the
trafficker, or having sex in exchange for food, clothing, shel-
ter or other physical items. These are just some of the
recommended interview questions that can assist in expos-
ing the trafficking.
A most important part of addressing human trafficking is
awareness and prevention. Whether in well care visits or in
any healthcare setting it is most appropriate to discuss this
problem, especially with high-risk youth.
US Trafficking in Persons Report, June 2019. https://www.
state.gov/wp-content/uploads/2019/06/2019-Trafficking-in-
Persons-Report.pdf
Reap, V.J. (2019). Sex trafficking: a concept analysis for health
care providers. Advanced Emergency Nursing Journal 41(2):
183–188. doi: 10.1097/TME.0000000000000236

69
A Toddler With Stridor
Mari Welch
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 21-month-old male is admitted to the pediatric intensive
care unit (PICU) with a 1-day history of increased work of
breathing and harsh cough.
History of Present Illness
This patient is a 21-month-old male with no significant past
medical history who was diagnosed with acute otitis media
(AOM) and started on a 10-day course of amoxicillin 4 days
prior to admission in the PICU. He initially had fever,
which resolved after antibiotics were initiated. He was
noted to have developed rhinorrhea, significant barky
cough, and hoarseness 2 days prior to the day of admission.
He also had decreased oral intake.
The toddler was with grandparents on the day of presen-
tation when they noticed that his cough was worsening,
with increasing work of breathing and wheezing. He had
significantly decreased oral intake and number of wet dia-
pers. These concerns prompted the grandparents to bring
him to the emergency department (ED).
The patient’s mother reports that the 11-month-old
brother was diagnosed with croup 6 days ago.
Past Medical History
This child was born at 38 week’s gestation after a normal
pregnancy and normal perinatal and early neonatal periods
and was discharged home from the newborn nursery on
day 1 of life. All required immunizations are up to date
including annual influenza vaccine.
His present weight and height are at the 11th percentile
for age, and he has remained on the same growth curve
since 6 months of age.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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291
Past Surgical History
None.
Family History
Non-contributory. Parents are both healthy, and there is a
younger sibling.
Current Status
On arrival at an outlying hospital ED, the patient is noted to
have inspiratory and expiratory stridor. His initial respira-
tory rate is 50 breaths per minute with oxygen saturation of
88% and moderate supraclavicular and subcostal retrac-
tions. He is given dexamethasone (Decadron) and three
0.5-mL doses of racemic epinephrine 2.25%, and is started
on 100% oxygen via high-flow nasal cannula (HFNC) at 8 L/
min, with minimal improvement in work of breathing. As
he continues to exhibit increased work of breathing, he is
placed on continuous positive airway pressure (CPAP) sup-
port. Despite ongoing administration of racemic epineph-
rine and CPAP support, his work of breathing continues
to worsen, with persistent oxygen desaturation and worsen-
ing retractions. A venous blood gas (VBG) analysis demon-
strates respiratory acidosis, pH of 6.94, PCO2 100 mmHg,
and bicarbonate 25 mg/dL. He is emergently intubated
for hypercarbic respiratory failure and placed on the venti-
lator with settings of FiO2 0.4, positive end-expiratory pres-
sure (PEEP) 5 cmH2O, tidal volume 60 mL, inspiratory time
0.6 seconds, and rate 30/min. A chest X-ray demonstrates
diffuse hazy bilateral airspace opacity without evidence
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292 Cases in Pediatric Acute Care

Table 69.1 Venous blood gas analysis before and after Rationale and Evidence-based Practice
intubation.
Explanation
Before intubation After intubation
Based on the data given, what are the differential
pH 6.94 7.15 diagnoses and the most likely diagnosis for
PCO2 (mmHg) 100 82
this child?
PO2 (mmHg) 80 73 The differential diagnoses for this child include the
– following.
HCO3 (mmol/L) 25.6 28.6

• Croup (laryngotracheobronchitis): associated with viral

illness.
Table 69.2 Complete blood count.

White blood cell count 11 460/mm3

• Acute epiglottitis (rare in setting of Haemophilus influen-
zae vaccination): patients have toxic appearance, sitting
upright and drooling.
Hemoglobin
Hematocrit
11.4 g/dL
33.5% • Peritonsillar abscess: patients sit upright, drooling, with
extended neck, neck swelling and muffled voice.
Platelet count 292 000/mm3
• Retropharyngeal abscess: similar in presentation to peri-
tonsillar abscess, with neck stiffness, barky cough is not
present.

Table 69.3 Basic metabolic panel.

• Foreign body aspiration: often a history of acute choking,
coughing, and focal wheeze.

Sodium 136 mEq/L • Anaphylaxis: acute presentation without cold symptom

prodrome.
Potassium 3.7 mEq/L
Chloride 102 mEq/L Croup is the most likely diagnosis due to the presenting
Carbon dioxide 29 mEq/L
symptoms of hoarseness, barky cough, and stridor, after a
few days of coryza and viral prodrome.
Blood urea nitrogen 9 mg/dL
Creatinine 0.3 mg/dL
What is the pathophysiology of the diagnosis?
Glucose 270 mg/dL
Calcium 8.8 mg/dL
A viral infection causes inflammation of the larynx, tra-
chea, bronchi, and occasionally bronchioles, and lung
parenchyma. This inflammation leads to narrowing of
the laryngeal area and tracheal and bronchial lumens, caus-
of pneumothorax or pleural effusion. Respiratory viral ing obstruction that is pronounced in the subglottic region.
panel and repeat VBG analysis, complete blood count, basic Obstruction increases the work of breathing, occasionally
metabolic panel and blood culture are all obtained and the resulting in hypercapnia and respiratory failure. Atelectasis
results are shown in Tables 69.1–69.3. may occur concurrently if the bronchioles become
obstructed. Croup is most common in children aged
6 months to 3 years. Symptoms of croup are almost always
preceded by a viral prodrome.
Questions
What are the indications for admission?
Answer the following questions using the details provided.
Only 8–15% of patients with croup require hospitalization,
1 Based on the data given, what are the differential diag- of which 1% are admitted to the PICU (Rosychuk
noses and the most likely diagnosis for this child? et al., 2010).
2 What is the pathophysiology of the diagnosis? Severe croup leads to poor air entry, significant stridor at
3 What are the indications for admission? rest, altered consciousness, impending respiratory failure,
4 What are the recommended therapies and their persistent or deteriorating respiratory distress after treatment
mechanisms of action? with nebulized epinephrine and corticosteroids, “toxic”
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Chapter 69 A Toddler With Stridor 293

appearance or clinical picture suggesting serious secondary children may require fluid boluses and maintenance
bacterial infection, need for supplemental oxygen, and severe intravenous fluid administration.
dehydration or intolerance of adequate oral intake.
The indications for PICU admission include:
• Intubation: rarely indicated, with less than 3% of croup
admissions to the PICU requiring intubation. Intubation

•
should be performed by a highly skilled practitioner
respiratory failure with imminent respiratory arrest, (ENT, anesthesiology), because airway edema may make
requiring endotracheal intubation;

•
intubation difficult. The median duration for intubation
persistent severe symptoms requiring frequent nebulized is 60 hours (Tyler et al., 2017).

•
epinephrine dosing;
underlying conditions placing the child at high risk for
progressive respiratory failure (e.g. neuromuscular dis-
ease or bronchopulmonary dysplasia) (Petrocheilou
et al., 2014).
What are the recommended therapies and their
mechanisms of action?
• Diagnostic testing: a neck radiograph is often considered
for children with croup. However, croup is a clinical diag-
nosis and an X-ray is not required nor is there always an
evident classic “steeple” sign.
Continuation of case: The patient’s respiratory viral panel
is positive for coronavirus OC43. He patient is admitted to
the PICU and placed on conventional ventilator, with set-
tings of PEEP 5 cmH2O, tidal volume 6 mL/kg (60 mL), rate

•• Supportive care: humidified air, antipyretics.

Glucocorticoids: the mainstay of treatment for croup.
35/min, and FiO2 0.3. He is started on dexamethasone
(Decadron) 0.6 mg/kg every 6 hours. A bronchoalveolar
lavage is obtained and sent for evaluation. Subsequent
Glucocorticoids decrease the severity of croup symptoms
VBG analyses are normal.
in 2–24 hours, reducing hospital length of stay and rate of
On day 2 of admission, the toddler is weaned to CPAP and
return visits (Gates et al., 2018). The mechanism of action
extubated to helium/oxygen mixture (Heliox 70/30) combi-
involves reduction of inflammation by suppression of
nation via HFNC on day 3 of admission. He completes four
neutrophil migration, decreased production of inflam-
doses of Decadron and receives another four doses for per-
matory mediators, and reversal of capillary permeability.
sistent stridor post extubation. A nasoduodenal tube is
Dosing in infants and children is 0.6 mg/kg, routinely
placed on PICU day 3 prior for nutrition.
used once, but in certain situations where the patient
The child is weaned to room air on day 5 of admission,
exhibits recurrent stridor it has been repeated.

• Racemic epinephrine: nebulized racemic epinephrine

2.25% should be considered for hypoxia or severe croup.
and at this time he is breathing at a rate of 36/min with
minimal intermittent inspiratory stridor. He is transferred
to the regular inpatient care area, but restarted on Heliox
The adrenergic effects induce vasoconstriction, leading to
for worsening stridor and hypoxia, receiving two further
decreased subglottic edema. Onset is less than 10 minutes
doses of racemic epinephrine and is therefore transferred
with duration of 2–3 hours; patient therefore requires
back to the PICU.
observation for 3–4 hours for recurrence of stridor before
discharge.

• Heliox: There is evidence to suggest that Heliox therapy,

which comprises 70% helium and 30% oxygen, may be
Additional Question and Evidence-
based Rationale
used for moderate to severe distress or illness not respon-
sive or refractory to racemic epinephrine, and before the How would you determine if the patient is ready
full effects of glucocorticoids are established (Moraa for discharge?
et al., 2013). It works by establishing laminar gas flow
Typically, croup is self-limited and once the acute period
in edematous airways, thereby decreasing the effect of
has resolved it is unlikely for severe symptoms to recur.
increased airway resistance and decreases the respiratory
Indications for discharge include:
muscle work of breathing. If oxygen needs are more than

•
30%, then Heliox may not be effective.
Hydration: an assessment of the child’s hydration status
•• no stridor at rest;
normal pulse oximetry in room air;
is also necessary. Children with respiratory distress have
insensible fluid loss requirements. Also, children who are
•• good air exchange;
normal color;
ill may have had difficulty drinking adequate volumes of
fluids preceding the hospitalization. These infants and
•• normal level of consciousness;
demonstrated ability to tolerate fluids by mouth.
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294 Cases in Pediatric Acute Care

References
Gates, A., Gates, M., Vandermeer, B., et al. (2018).
Glucocorticoids for croup in children. Cochrane Database of
Systematic Reviews (8): CD001955.
Moraa, I., Sturman N., McGuire T., and van Driel, M.L. (2013).
Heliox for croup in children. Cochrane Database of
Systematic Reviews (12): CD006822.
Petrocheilou, A., Tanou, K., Kalampouka, E., et al. (2014). Viral
croup: diagnosis and a treatment algorithm. Pediatric
Pulmonology 49: 421–429.
Rosychuk, R.J., Klassen, T.P., Metes, D., et al. (2010). Croup
presentations to emergency departments in Alberta,
Canada: a large population-based study. Pediatric
Pulmonology 45(1): 83–91.
Tyler, A., McLeod, L., Beaty, B., et al. (2017). Variation in
inpatient croup management and outcomes. Pediatrics 139
(4): pii: e20163582. doi: 10.1542/peds.2016-3582.

Further Reading
Cherry, J.D. (2008). Clinical practice. Croup. New England
Journal of Medicine 358(4): 384–391.
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295

70

Delirium in a 9-Year-Old Boy

Heather Herrera
Children’s Hospital of San Antonio, San Antonio, TX, USA

The patient is a 9-year-old male with sepsis, ventilated with
sedation and analgesia, who has developed delirium.
History of Present Illness
This 9-year-old boy presented to the pediatric intensive care
unit (PICU) 5 days ago with septic shock secondary to
pneumonia. He became hypoxic with subsequent respira-
tory failure unresponsive to non-invasive ventilation, so
was intubated and placed on a ventilator. The patient then
developed fluid-refractory shock and required vasopressor
support. After 48 hours, his respiratory status began to
improve along with his blood pressure, and vasopressor
support was weaned off. He remained on a ventilator for
a total of 6 days, with sedation and pain management
achieved with morphine and midazolam continuous infu-
sions. As his status has improved, sedation has been
weaned to allow him to be more awake yet comfortable.
Past Medical History
Family History
The patient lives with the paternal grandmother, as neither
parent is involved in his care. Grandmother has hyperten-
sion and chronic lung disease. The father has drug depend-
ency and is in and out of rehabilitation and jail. The
grandmother does not know where the mother of the child
is and does not know her health history.
Current Status
Over the last 2 days, the patient has been noted to be awake
more at night than during the day. Today, his behavior has
ranged from being intermittently quiet and non-interactive
to agitated, crying, and fearful of a “man” he is seeing in the
corner of his room. He will occasionally correctly state his
name and where he is, but then reverts back to being con-
fused and unable to identify family members or his current
location. After assessing this boy, the bedside provider
orders delirium scoring using the Cornell assessment of
pediatric delirium.

This patient was born at term via spontaneous vaginal

delivery and discharged home with his mother. He has
received required immunizations along with a flu vaccine
this season. His weight has been steady at 75% for gender
and age and his current body mass index (BMI) is 22.5.
He has had no previous hospitalizations.
Past Surgical History
This child had tympanostomy tubes placed at 15 months of
age for frequent episodes of otitis media; no complications
during the procedure or postoperatively.
Questions
Answer the following questions using the details provided.
1 What is the pathophysiology of delirium and what fac-
tors can predispose a patient to develop this problem?
2 What factors contribute to the development of
delirium?
3 What are the different types of delirium and what are
the available screening tools?
4 What are the best ways to optimize therapy: environ-
mental or pharmacologic?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

296 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
What is the pathophysiology of delirium and what
factors can predispose a patient to develop this
problem?
Delirium is best described as an acute brain dysfunction or
a disruption in cerebral homeostasis with significant effects
on patient outcomes, medical costs, morbidity, and mortal-
ity. Although delirium is multifactorial, some medications,
such as benzodiazepines, glucocorticoids, anesthetics,
diuretics, opiates, and antihistamines, are thought to poten-
tiate its development (Patel et al., 2017). Children under the
age of 2 years and those with developmental delay, high
severity of illness, low albumin, mechanical ventilation,
and preexisting medical condition(s) are predisposed to
the development of delirium (Patel et al., 2017). There
are three hypotheses for the development of pediatric delir-
ium as discussed by Patel et al. (2017); neuro-inflammatory,
neurotransmitter, and oxidative stress. According to the
neuro-inflammatory hypothesis, systemic inflammation,
such as that seen in critical illness, “leads to either compro-
mise in the integrity of the blood–brain barrier or de novo
production of inflammatory products within the brain.”
The neurotransmitter hypothesis relates to use of medica-
tions that change neurotransmitter function and the oxida-
tive stress hypothesis “suggests that reduced oxygen
delivery in critical illness, coupled with increased cerebral
metabolism, leads to the production of reactive oxygen spe-
cies that cause global CNS dysfunction.” It is important to
note that hypoxia has been clearly associated with delirium
development.
What factors contribute to the development
of delirium?
Factors contributing to the development of pediatric delir-
ium in critically ill children can be separated into three cate-
gories: predisposing, precipitating, and perpetuating (Malas
et al., 2017). Predisposing risk factors cannot be modified
and include age less than 2, developmental delay, high
severity of illness, low albumin, genetic susceptibility,
mechanical ventilation, and preexisting medical conditions.
Precipitating factors are treatment related and can poten-
tially be modified for the patient, such as anticholinergic
medications, benzodiazepines, cardiac bypass surgery,
immobilization, prolonged length of stay in the intensive
care unit (ICU), and restraints. Perpetuating factors are
found in the patient’s care environment, including lack of
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mobility, disruption of sleep–wake cycle, and lack of
familiar environment and supports (Malas et al., 2017).
Sedatives may also contribute to the development of
delirium in critically ill pediatric patients. A possible
replacement therapy is the use of dexmetomidine which
has been noted to prevent and/or treat delirium; however,
it is not currently FDA approved for the treatment of pedi-
atric delirium. Identification and treatment of iatrogenic
withdrawal syndrome (IWS) is also very important. Once
IWS has been managed, if the patient continues to experi-
ence symptoms of delirium, environmental modifications
may be needed as increasing sedative medications may
actually end up prolonging delirium (Patel et al., 2017).
What are the different types of delirium and what
are the available screening tools?
The Diagnostic and Statistical Manual of Mental Disorders
recognizes three types of delirium: hypoactive, hyperactive,
and mixed type (Patel et al., 2017). Typical symptoms of
hypoactive delirium include lethargy, inattentiveness,
and decreased responsiveness. Hyperactive delirium is
characterized by agitation, restlessness, hypervigilance,
and combative behavior. Mixed-type delirium is character-
ized by a combination of both hypoactive and hyperactive
symptoms. Hypoactive delirium has been associated with
poor prognosis and occurs at the greatest frequency. Emo-
tional lability as well as visual and auditory hallucinations
can also be experienced by pediatric patients with delirium
along with disordered sleep. Delirium most often occurs
early in the ICU course of children, with median time of
onset 1–3 days and typically lasting about 2 days. About
one-third of pediatric patients who develop delirium early
in their hospital course will experience recurrent episodes
during their ICU stay. Children with mild to severe delir-
ium can experience longer time on mechanical ventilation,
longer hospital length of stay, longer time to emerge from
sedation, longer time to extubation, and higher resource
utilization (Patel et al., 2017). Delirium is often a temporary
condition, although it has been strongly linked with poor
outcomes, including increase in mortality and enduring
cognitive impairment in survivors (Patel et al., 2017). In
2013, the Society of Critical Care Medicine (SCCM)
released guidelines recommending that screening for delir-
ium become a standard of care in adults, but there are not
guidelines specific to pediatrics at this time.
Current assessment of delirium includes two versions of
validated pediatric delirium screens: the Pediatric Confu-
sion Assessment Method for the ICU with two versions,
the pCAM-ICU for children older than age 5 years and

the psCAM-ICU for those aged 6 months to 5 years. The
other option is the CAPD, which is an observation screen
useful for patients aged 0–21; it can provide a picture of a
pediatric patient over a period of time and has been vali-
dated in both developmentally normal and developmen-
tally delayed children (Patel et al., 2017). Prior to
assessing a child for delirium, their level of consciousness
should be assessed using a tool such as the RASS (Smith
et al., 2013). Routine monitoring of critically ill children
should occur at least once per shift. The challenge is provid-
ing the most minimal sedation required to keep patients
comfortable, especially those requiring mechanical ventila-
tion or other invasive treatments, along with assessment
and treatment for delirium.
What are the best ways to optimize therapy:
environmental or pharmacologic?
When delirium is diagnosed in a critically ill child, the key
to effective treatment is to identify the underlying cause
(Patel et al., 2017). Delirium is most often caused by a com-
bination of factors, including the ICU environment, iatro-
genic effects of treatment, and an effect of the underlying
illness. Assessment for infection, treating hypoxemia, opti-
mizing pain management, and correcting any metabolic
abnormalities are some recommendations. Iatrogenic
factors should be determined, followed by environmental
modifications to help improve symptoms; alternatively,
pharmacologic treatment may be needed.
Modifications of the child’s environment can help in
decreasing the risk of delirium as well as aiding in alleviat-
ing symptoms. This can include adjusting the room temper-
ature and lighting to reflect day and night time, reducing
noise, and decreasing the use of physical restraints (when
safe). Objects and individuals familiar to the child should
be close by. Consistent caregivers along with stable daily
routines can also reduce the risk of confusion and help reg-
ulate the sleep–wake cycle. Reorientation strategies have
been shown to produce a statistically significant decrease
in symptoms. It is important to address both sensory and
communication limitations when able. Early mobilization
References
Children’s Hospital of Philadelphia (2019). CICU/PCU/PICU
Clinical pathway for delirium. http://www.chop.edu/
clinical-pathway/picu-pcu-delirium-clinical-pathway
Malas, N., Brahmbhatt, K., McDermott, C., et al. (2017).
Pediatric delirium: evaluation, management, and special
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Chapter 70 Delirium in a 9-Year-Old Boy 297
can reduce delirium risk and improve time to improvement
(Malas et al., 2017).
If physical and environmental adjustments have not been
successful, a trial of pharmacologic therapy can begin.
Atypical antipsychotics are recommended because of their
precognitive effects and favorable side-effect profile (Patel
et al., 2017), but this use is off-label at this time. Haloperidol
has been used clinically for many years to manage delirium
symptoms, but also comes with side effects (Turkel, 2017).
When managing pediatric delirium with atypical antipsy-
chotics, quetiapine, risperidone, and olanzapine are cur-
rently the usual first choices. As mentioned previously,
dexmetomidine can also be used. The ability to discontinue
pharmacologic therapy should be discussed no later than
5–7 days after initiation (Children’s Hospital of Philadel-
phia, 2019).
Prevention of delirium should be the first goal of ICU
care, followed by astute evaluation. The risk of developing
delirium in the ICU environment is high, so early evalua-
tion and intervention can assist in management. With
improved awareness of pediatric delirium and its adverse
effects, implementation of improved sedation techniques,
early mobilization, and involvement of family members
in daily care, delirium may be able to be prevented in
critically ill children.
Case Resolution
The patient scored 17 on the delirium scale, which indicates
the presence of delirium. His nurses scheduled a daily rou-
tine and family brought some familiar objects from home.
Nursing was also encouraged to cluster care at night for
fewer disturbances. The child initially had improvement
in his symptoms, but he continued to have moments of
confusion and hallucinations. He was thus started on an
antipsychotic medication to help allay delirium symptoms.
Within a week of starting the medication, his symptoms
completely resolved and the patient returned to his neuro-
logic baseline.
considerations. Current Psychiatry Reports 19(9): 65.
doi: 10.1007/s11920-017-0817-3.
Patel, A.K., Bell, M.J., and Traube, C. (2017). Delirium in
pediatric critical care. Pediatric Clinics of North America
64(5): 1117–1132. doi: 10.1016/j.pcl.2017.06.009.
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
298 Cases in Pediatric Acute Care

Smith, H.A.B., Brink, E., Fuchs, D.C., Wesley, E., and
Pandharipande, P.P. (2013). Pediatric delirium monitoring
and management in the pediatric intensive care unit.
Pediatric Clinics of North America 60(3): 741–760. doi:
10.1016/j.pcl.2013.02.010.
Turkel, S.B. (2017). Pediatric delirium: recognition,
management, and outcome. Current Psychiatry Reports 19
(12):101. doi: 10.1007/s11920-017-0851-1.

Further Reading
Children’s Hospital of Philadelphia (2019). Clinical team to
bedside to assess patient: BRAIN MAPS. http://www.chop.
edu/clinical-pathway/picu-pcu-delirium-brain-maps

71
Feeding Challenge in a Child with Spinal Muscular Atrophy
Nicole Fragale
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 16-month-old child with spinal muscular atrophy (SMA)
type 1, diagnosed as an infant, is admitted to the pediatric
intensive care unit (PICU) with severe malnutrition and
aspiration pneumonia leading to acute hypercarbic respira-
tory failure.
History of Present Illness
The child was in her usual state of health until 2 days ago
when she began to have increased work of breathing and
post-tussive emesis. Parents report that she pulled out
her nasogastric feeding tube 2 days prior to admission.
The tube was not replaced by the family, and instead the
child was fed orally until she was brought into the hospital.
Past Medical History
This child was diagnosed with SMA type 1 during infancy.
At 10 months of age, a bedside feeding evaluation by a
speech language pathologist indicated that the infant had
discoordination with suck and swallow. A modified barium
swallow study indicated that the patient had evidence of
aspiration, and nil by mouth status was recommended.
As a result, a nasogastric tube was placed and the patient
was sent home with tube feedings to meet 100% of her
nutritional requirements. This child also has a night-time
bilevel positive airway pressure (BiPAP) requirement.
Between the two admissions, she was lost to follow-up
and there is concern for medical neglect.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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299
Current Status
She is hypoglycemic on admission and requires
Vapotherm® (high-flow nasal cannula therapy), progres-
sing to BiPAP due to respiratory distress. She is treated with
Unasyn (ampicillin sulbactam) due to a left-sided pneumo-
nia, seen as a white-out on X-ray. Her admission weight
(6.4 kg, z-score –3.78) is noted as a 17% reduction in body
weight since last seen in the hospital 6 months prior.
Her admission length (71 cm) is below the 1st percentile
(z-score –5). Her weight/length 6 months prior was following
the 75th percentile (z-score 0.65) and on admission to the hos-
pital is now less than the 1st percentile (z-score –3). She meets
criteria for severe malnutrition (see Table 71.1 for indicators
of the diagnosis of malnutrition).
The results of the basic metabolic panel are shown in
Table 71.2.
Questions
Answer the following questions using the details provided.
1 Based on the background information and the labora-
tory results on admission, what is your assessment of
this child’s fluid and nutritional status?
2 How should enteral nutrition be resumed in this patient
and what parameters will be monitored?
3 What are the calorie and protein goals for this patient?
4 How will you determine if the nutrition being provided
is adequate and what feeding protocol should be recom-
mended for discharge and home care?
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300 Cases in Pediatric Acute Care

Table 71.1 Primary malnutrition indicators when two or more data points available.

Mild malnutrition Moderate malnutrition Severe malnutrition

Weight gain velocity Less than 75% of the norm for Less than 50% of the norm for Less than 25% of the norm for
(<2 years of age) expected weight gain expected weight gain expected weight gain
Weight loss (2–20 years of 5% usual body weight 7.5% usual body weight 10% usual body weight
age)
Deceleration in weight for Decline of 1 z-score Decline of 2 z-score Decline of 3 z-score
length/height z-score
Inadequate nutrient intake 51–75% estimated energy/ 26–50% estimated energy/ ≤25% estimated energy/protein
protein need protein need need
Source: Becker et al. (2014). Reproduced with permission of Elsevier.

Table 71.2 Basic metabolic panel. neuromotor control can be affected, including feeding and
breathing. Children with SMA can have feeding problems,
Sodium 145 mEq/L dysphagia or gastrointestinal dysfunction, and because of
Potassium 3.9 mEq/L muscle laxity and overall neuromuscular weakness, it is
Chloride 115 mEq/L not uncommon for these children to gain unnecessary
Carbon dioxide 14 mEq/L weight. Consequently, they need to be monitored very care-
Blood urea nitrogen 18 mg/dL
fully and nutrition needs to include vitamin D, calcium and
phosphorus for bone health.
Creatinine <0.1 mg/dL
This child has a history of poor feeding abilities and aspi-
Glucose 53 mg/dL
ration on modified barium swallow. She presents to the
Calcium 8.5 mg/dL hospital after having been without her nasogastric feeding
Magnesium 1.7 mEq/L tube for 2 days (by parent report). Her elevated sodium,
Phosphorus 3.0 mg/dL chloride, and blood urea nitrogen indicate she is dehy-
drated, which is likely due to inability to consume adequate
fluids by mouth. The significant decrease in weight on
admission and the stunted length indicate that this child
Rationale and Evidence-based Practice is chronically malnourished and she has likely not received
Explanation adequate nutrition in at least 3 months.
Because this 16-month-old child with type 1 SMA is sus-
Based on the background information and the pected to have been without adequate nutrition for several
laboratory results on admission, what is your months, she is at risk for refeeding syndrome. Refeeding
assessment of this child’s fluid and nutritional
syndrome presents as metabolic derangements that occur
status?
as a result of the rapid shift from fat and protein catabolism
This child has a history of SMA type 1 which is considered a (which occur with starvation) to carbohydrate metabolism,
severe progressive neuromuscular illness. SMA is due to a resulting in an increase in circulating insulin levels. When
defect in the survival motor neuron 1 (SMN1) gene and is insulin levels increase in the body, there is rapid uptake of
present in types 1 to 4. SMA is characterized by degenera- glucose, potassium, phosphorus and magnesium into the
tion of the motor neurons in the spinal cord, resulting in cells, resulting in decreased serum concentrations. Fluid
progressive muscle atrophy, weakness, and paralysis. Typ- retention is common with these shifts and can result in
ically, infants with SMA type 1 present with hypotonia acute heart failure as a result of increased cardiac demand
early in life along with weakness of the legs as compared with accompanying acute respiratory distress.
to the arms, and weakness of the intercostal muscles result-
ing in ineffective or paradoxical breathing patterns. SMA
How should enteral nutrition be resumed in this
type 1 is typically diagnosed within the first 6 months of life
patient and what parameters will be monitored?
and is the most severe form, often resulting in death at a
very early age. Diagnosis is accomplished by clinical find- In order to avoid refeeding syndrome, electrolytes should
ings, genetic testing, electromyography, and creatine be normalized prior to starting enteral feeds, which should
kinase levels, which may be normal, mildly elevated, or sig- then be initiated at 50–75% of intended goal and advanced
nificantly elevated. Any physiologic function that requires by only 10–20% daily. Electrolytes should continue to be

monitored two to four times daily, and repleted as needed.
Phosphorus (250 mg daily) and thiamine supplementation
(100 mg three times daily) is indicated when initiating feeds
in order to aid carbohydrate metabolism. Strict monitoring
of fluid status is required and fluid restriction may be nec-
essary if edema is noted. Refeeding syndrome can occur up
to 2 weeks after initiation of enteral feeds.
What are the calorie and protein goals for this
patient?
On admission, the patient has a post-pyloric nasoduodenal
tube placed. The team would like feeding recommenda-
tions. Prior to determining calorie goals for the patient,
an assessment of the diet followed prior to admission is
indicated. In this situation, the parent reports a tube feed-
ing regimen that mimics what the patient received in the
hospital 6 months prior, in addition to taking bottles by
mouth. If the patient had been receiving the reported reg-
imen, she would have been receiving 135 cal/kg per day.
A typical 16-month-old child would require 100 cal/kg
daily. A malnourished child requires more for catch-up
growth (100–120 cal/kg daily). In order to avoid refeeding
syndrome, the following recommendation is given for start-
ing continuous feedings.
•
On initiating enteral feeds, use Nutren Junior started at
15 mL/hour (50% goal). Advance by 5 mL/hour every
day until goal of 30 mL/hour is reached (will take 3 days).
•
Using weight of 6.4 kg, regimen at goal will provide 720 mL
(112 mL/kg), 720 cal (112 cal/kg), 22 g protein (3.3 g/kg).
Reference
Becker, P.N., Carney, L.N., Corkins, M.R., et al. (2014).
Concensus Statement of the Academy of Nutrition and
Dietitics/American Society for Parenteral and Enteral
Nutrition: indicators recommended for the identification
Further Reading
Beer, S., Bunting, D., Canada, N., et al. (2016). Pediatric
Nutrition Reference Guide, 11th edn. Houston, TX: Texas
Children’s Hospital.
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Chapter 71 Feeding Challenge in a Child with Spinal Muscular Atrophy 301
How will you determine if the nutrition being
provided is adequate and what feeding protocol
should be recommended for discharge and
home care?
It is most important to include the interprofessional
team to assist in making sure that this child comes back
for follow-up. Engaging the social worker, case man-
ager, and neurology service can assist in beginning this
process. The registered dietitian is also extremely
important in evaluating nutritional status through mon-
itoring weekly weight trends and mid-upper arm cir-
cumferences. Normal weight gain for a 16-month-old
child ranges from 5 to 9 g/day. When catch-up growth
is indicated, daily weight gain goals should be two to
three times this amount. Increases in the percentile
and z-score of patient length should also be seen
over time.
Ideally, this patient will have a surgically placed gastric
feeding tube. If this is not possible, the nasoduodenal tube
should be moved to become a nasogastric tube so that the
patient can be given bolus feeds. This will mimic normal
physiologic eating. Gastroenterology service can assist in
supporting the patient and insuring that the gastric tube
is placed in a good position, along with education for the
family on how to manage, maintain and use for feeding.
Frequent follow-up with an outpatient dietitian is recom-
mended so that adjustments can be made as the patient
continues to grow.
and documentation of pediatric malnutrition
(undernutrition). Journal of the Academy of Nutrition and
Dietetics 114(12): 1988–2000.

72
A 5-Year-Old with Osteomyelitis, Electrolyte Disturbance and Headaches
Kimberly L. DiMaria
Children’s Hospital Colorado, Aurora, CO, USA
A 5-year-old previously healthy male is admitted with oste-
omyelitis. He has been hospitalized for 3 days and his
course is now complicated by electrolyte abnormalities
and associated mental status changes.
History of Present Illness
A 5-year-old boy with osteomyelitis of the right ankle is
admitted for parenteral antibiotics and peripherally
inserted central catheter line placement. He developed
severe diarrhea 2 days after initiation of antimicrobial ther-
apy. Today, he is lethargic and complaining of a headache.
He has had decreased urine output since yesterday. The
rapid response team is activated.
Past Medical History
He was born full term after an uncomplicated pregnancy
and delivery. He had one episode of croup at 6 months of
age that required hospitalization and steroids. He has sea-
sonal allergies for which he takes a daily cetirizine. No
known drug allergies.
Past Surgical History
No previous surgeries.
Family History
Non-contributory. He lives with both parents and three
older siblings, two dogs and a parakeet. Is in kindergarten.
No recent travel.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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303
Current Status
He is a well-developed, well-nourished, pale and tired-
appearing school-age child lying in bed watching a movie.
His vital signs are heart rate 136 bpm, respiratory rate
30 breaths per minute, blood pressure 86/42 mmHg, and
oxygen saturation 94% on room air. He is 18 kg on admis-
sion; there is no subsequent weight documented.
His examination is notable for the following. He has dry
mucous membranes, clear breath sounds with easy work of
breathing, sinus tachycardia, 2+ pulses throughout, and
capillary refill time of 4 seconds with warm/dry extremities.
He has a non-tender abdomen with hyperactive bowel
sounds and no organomegaly. He is awake, but lethargic
and irritable. It takes several attempts for him to look at
the healthcare provider, respond to questions, and follow
simple commands. He tells the healthcare provider that
his “head hurts.” His cranial nerves II–XII are grossly
intact.
At the time of his rapid response team call, his mother
shares with the team that the child had diarrhea almost
every hour last night. She is not sure if he had urine output
with any of his frequent trips to the bathroom.
He is transferred to the pediatric intensive care unit
(PICU) for further evaluation and management. He is
placed on the cardiorespiratory monitor and a basic meta-
bolic profile is obtained. The result of his laboratory work is
shown in Table 72.1.
Questions
Answer the following questions using the details provided.
1 Based on the information provided, what is the most
likely diagnosis for this patient?

304 Cases in Pediatric Acute Care
Table 72.1 Basic metabolic profile.
Sodium 160 mEq/L
Potassium 4.5 mEq/L
Chloride 125 mEq/L
Carbon dioxide 16 mEq/L
Blood urea nitrogen 26 mg/dL
Creatinine 1.6 mg/dL
Glucose 102 mg/dL
2 Is transfer to the PICU indicated?
3 What are the initial steps of management?
4 How will you correct the electrolyte derangement?
5 What complications will you monitor for?
Rationale and Evidence-based Practice
Explanation
Based on the information provided, what is the
most likely diagnosis for this patient?
This patient has hypernatremia. Supported by his diarrheal
losses, physical examination and vital signs, he is also dehy-
drated. He has hypernatremic dehydration. Hypernatremia
is defined as serum sodium greater than 150 mEq/L and
hypernatremic dehydration signifies a greater water loss
in relation to total body sodium. There are various ways
that a patient can develop hypernatremic dehydration.
The patient in this case study developed hypernatremic
dehydration from fluid losses. All gastrointestinal losses
(emesis or diarrhea) are hypotonic because they have a
sodium concentration less than the plasma sodium concen-
tration. An excessive loss of hypotonic fluid (emesis or diar-
rhea) resulting in an increase in output can lead to
hypernatremia. This type of electrolyte disturbance typi-
cally occurs gradually, which explains why the patient is
less symptomatic than if the hypernatremic state had
occurred abruptly.
Severe hypernatremic dehydration can cause a spectrum
of neurologic symptoms, including altered mental status,
cerebral hemorrhage, seizures, and encephalopathy. The
mechanism of intracranial hemorrhage is explained by
tearing of the cerebral blood vessels (bridging veins) as
the dehydrated brain tissue shrinks.
Is transfer to the PICU indicated?
Yes, this patient is experiencing hypernatremic dehydra-
tion with associated mental status changes. He will require
close monitoring of his neurologic status and laboratory
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values during rehydration and sodium correction. The
morbidity and mortality associated with hypernatremia is
typically not from the disorder itself, but from inappropri-
ate management (e.g. excessively rapid correction). He will
require slow rehydration with frequent monitoring of
serum sodium levels because a rapid decrease in serum
sodium can result in cerebral edema, seizures, or osmotic
demyelination syndrome.
What are the initial steps of management?
Throughout the management of this patient, it is essential
to ensure slow and careful rehydration. Rapid decrease in
serum sodium can result in severe neurologic complica-
tions. The initial step in management is to begin careful
fluid resuscitation to restore the intravascular volume. Iso-
tonic fluid must be used for fluid resuscitation, using small
aliquots (10–20 mL/kg) unless the patient has evidence of
hypovolemic shock. Normal saline has a sodium concentra-
tion of 154 mEq/L and is thus technically a hypotonic fluid
in the presence of a serum sodium of 160 mEq/L in this
hypernatremic child. Therefore, if the child remains hemo-
dynamically stable, only a single, small, fluid bolus of nor-
mal saline should be administered prior to rechecking the
serum sodium.
How will you correct the electrolyte
derangement?
The goal is to decrease his serum sodium by 10–15 mEq/L
per day, which is roughly 0.5 mEq/L every hour. To achieve
this slow decrease in serum sodium, begin by calculating
the free water deficit using the following formula:
Free water FW deficit = 0 6 ×
weight kg × current serum sodium 140 – 1
This child’s free water deficit, if targeting a serum sodium
of 145 mEq/L (rather than 140 mEq/L in the equation
above) is 1.1 L.
FW deficit = 0 6 × 18 kg × 160 145 – 1
= 1 117 L rounded down to 1 1 L
This amount of fluid (1.1 L or 1100 mL) will be adminis-
tered over 48 hours at a rate of 23 mL/hour. It is important
to note that the rehydration fluid (the FW deficit calculated
rate) will be administered in combination with mainte-
nance fluids. For the patient, this would be a total fluid rate
of 79 mL/hour (23 mL/hour of rehydration fluid plus
56 mL/hour of maintenance fluids). Once the rate of intra-
venous fluid therapy has been determined, the next step is
to select the type of fluid to be administered. Dextrose 5%
with 0.2% normal saline is the typical intravenous fluid

Chapter 72 A 5-Year-Old with Osteomyelitis, Electrolyte Disturbance and Headaches
of choice for mild to moderate dehydration, but the sodium
concentration may need to be increased depending on the
serum sodium value and rate of decrease in serum sodium.
The serum sodium should be evaluated 2 hours after the
initiation of intravenous therapy, and then every 2–4 hours
thereafter. If the serum sodium is decreasing by more than
0.5 mEq/L per hour, increase the sodium concentration in
the intravenous fluids.
Further Reading
Adtogue, H.J. and Madias, N.E. (2000). Hypernatremia. New
England Journal of Medicine 342(20):
1493–1499.
Liamis, G., Filippatos, T.D., and Elisaf, M.S. (2016).
Evaluation and treatment of hypernatremia: a
practical guide for physicians. Postgraduate Medicine
128(3): 299–306. doi: 10.1080/00325481.2016.
1147322.
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305
What complications will you monitor for?
Seizures, rapid decrease in sodium, signs of cerebral edema
(pupillary changes, mental status changes and late findings
of Cushing’s triad), and cerebral sinus venous thrombosis
should be be monitored for. The speed of sodium reduction
is the key determinant as to whether a patient develops
complications from electrolyte shifts.
Powers, K.S. (2015). Dehydration: isonatremic, hyponatremic,
and hypernatremic recognition and management. Pediatrics
in Review 36(7): 274–285. doi: 10.1542/pir.36-7-274.
Santilanes, G. and Rose, E. (2018). Evaluation and
management of dehydration in children. Emergency
Medicine Clinics of North America 36(2): 259–273.
Schwaderer, A.S. and Schwartz, G.J. (2005). Treating
hypernatremic dehydration. Pediatrics in Review 26(4): 148–150.

73
Child with Scald Burn Injury
Amarilis Martin and Bradley Tilford
Children’s Hospital of Michigan, Detroit, MI, USA
Patient is a 4-year-old non-verbal male with Down syn-
drome, developmental delay, and a history of repaired com-
plete atrioventricular canal (CAVC) defect and patent
ductus arteriosus (PDA) at 5 months of age. He is admitted
to the burn unit with an 8-day history of scald burns to
the feet.
History of Present Illness
This 4-year-old child suffered 4.5% total body surface area
scald burns to the feet 8 days ago, leaving a circumferential
mark at the ankles. According to the mother, she placed the
child in the bath tub, turned on the water, and momentarily
left the room. She returned when she heard him scream. He
quickly developed blisters to both feet. Mother did not seek
medical attention and managed the burns at home with
topical lidocaine cream and dry gauze dressings. Over the
week following the burns, the wounds worsened in appear-
ance. On the night of admission, he developed poor oral
intake, nausea, and bilious emesis for which she brought
him to an emergency department. He was then transferred
to a tertiary care center as a direct admission to the burn
service. There is no history of fever, voiding or stooling
concerns, or change in behavior. Mother is unsure if he
is in pain because he is developmentally delayed and
non-verbal.
Past Medical History
He was born at term and went home with his mother after a
2-day stay in the newborn nursery for jaundice and hypo-
glycemia. His cardiac defects were discovered after birth
due to feeding difficulty and failure to thrive. After repair
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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307
of his cardiac defects, he has had no other hospitalizations.
He takes oral ranitidine for acid reflux and nebulized albu-
terol as needed for intermittent asthma. He is followed
outpatient for his developmental and speech delays. Immu-
nizations are up to date for age, including the annual influ-
enza vaccine. He has a weight at the 75th percentile for
gender and age based on the Down syndrome growth curve
and a current body mass index (BMI) slightly greater than
the 95th percentile for gender and age.
Past Surgical History
Repair of CAVC defect and PDA ligation at 5 months of age.
Family History
Mother has type 2 diabetes mellitus. Father’s medical his-
tory is unknown.
Social History
Patient lives with his mother and his mother’s boyfriend.
His father is not involved in his care. He has no siblings.
Current Status
In the burn unit, he is found to have scald burns to both feet
with a circumferential mark at the ankles, a right buttock
wound with healthy granulation tissue and sloughing of
skin along the gluteal fold, and a right knee wound with
associated scale along the edges. His skin is dry. His vital
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308 Cases in Pediatric Acute Care

Table 73.1 Complete metabolic profile. Table 73.3 Venous blood gas analysis.

Sodium 147 mEq/L pH 7.01

Potassium 5.0 mEq/L PCO2 60 mmHg
Chloride 107 mEq/L PaO2 15 mmHg
Carbon dioxide 20 mEq/L Bicarbonate 14 mEq/L
Blood urea nitrogen 56 mg/dL Base excess –17
Creatinine 2.41 mg/dL SvO2 8%
Alanine aminotransferase 794 U/L Lactate 12.7 mmol/L
Aspartate aminotransferase 554 U/L Sodium 145 mmol/L
Lipase 137 U/L Chloride 115 mmol/L
Potassium 7.9 mmol/L
Ionized calcium 1.39 mmol/L

Table 73.2 Complete blood count and coagulation studies.

White blood cell count 14 900/mm3

1 Based on the history and presented data, what are the
Hemoglobin 12.8 g/dL differential diagnoses and the most likely diagnosis
Hematocrit 41.5% for this child?
Platelet count 136 000/mm3 2 What, if anything, is concerning about his complete
Neutrophils 75% metabolic panel?
Eosinophils 0% 3 Would you continue the current maintenance IVF at
Monocytes 8% this time?
Lymphocytes 12% Continuation of case: Patient has bilious emesis following
Bands 4% completion of his burn dressings and IV ondansetron is
Prothrombin time (PT) 17.0 s given. An hour later, during morning rounds by the burn
Partial thromboplastin time (PTT) 25.6 s service, the patient is found unresponsive and pulseless
and a code blue is called. His electrocardiogram (EKG)
International normalized ratio 1.67
leads and pulse oximeter had become disconnected when
he vomited and were not replaced. Cardiopulmonary resus-
citation (CPR) is started. He is intubated and bilious secre-
signs on admission are temperature 36.2 C (97.2 F), heart tions are noted in the endotracheal tube. Intraosseous
rate 153 bpm, respiratory rate 32 breaths per minute, blood access is obtained. A venous blood gas analysis obtained
pressure 80/43 mmHg, and oxygen saturation 93% on room during the resuscitation demonstrates the results shown
air. A basic metabolic panel, complete blood count with dif- in Table 73.3.
ferential, liver function tests, lipase, coagulation panel, and
4 In addition to following the Pediatric Advanced Life
type and screen are obtained. He is kept nil by mouth and is
Support (PALS) guidelines for cardiac arrest, what is
given maintenance intravenous fluids (IVF) consisting of
the best way to manage the patient’s condition?
5% dextrose in 0.9% normal saline containing 20 mEq/L
5 Would you obtain any other diagnostic studies?
of potassium chloride (KCl). Over the next 2 hours,
6 How would you determine if the therapy was effective?
his wounds are cleaned and dressed with silver sulfadiazine
(Silvadene) and sterile gauze. He is given intravenous (IV) Continuation of case: Code doses of IV epinephrine, IV
diphenhydramine and a small dose of IV morphine for pain calcium chloride 20 mg/kg per dose, and IV sodium bicar-
associated with cleaning and dressing the burns. His bonate 1 mEq/kg per dose are administered during the
laboratory results are presented in Tables 73.1 and 73.2. resuscitation. A blood culture is obtained and he is started
on broad-spectrum antibiotics. The echocardiogram does
not show a pericardial effusion and he continues to have
bilateral breath sounds without hypoxemia. He is started
Questions on an epinephrine infusion. While CPR is ongoing, the
patient is emergently transferred to the pediatric intensive
Answer the following questions using the details provided. care unit (PICU), where he undergoes extracorporeal

Figure 73.1 Sine wave pattern seen on EKG tracing of patient with severe hyperkalemia.
cardiopulmonary resuscitation (ECPR) with veno-arterial
extracorporeal membrane oxygenation (VA-ECMO). He
• Non-accidental trauma: scald burns to the feet with clear
is given additional IV doses of calcium chloride, sodium
bicarbonate, and insulin with 25% dextrose for the hyperka-
lemia. Despite these treatments, his potassium level
increases to 10.8 mEq/L, and his cardiac rhythm changes
• Ingestion of unknown drug causing anion-gap metabolic
from pulseless electrical activity to wide complex tachycar-
dia (sinusoidal waveform pattern) for which he is defibril-
lated four times (Figure 73.1). He is also given amiodarone
and lidocaine for the ventricular tachycardia.
7 What other therapies would be recommended at
this time?
Rationale and Evidence-based Practice
Explanation
Based on the history and presented data, what are
the differential diagnoses and the most likely
diagnosis for this child?
The child has multiple vital sign and laboratory
derangements: tachycardia, tachypnea, leukocytosis with
granulocytosis (75% neutrophils, 4% bands), mild thrombo-
cytopenia, acute kidney injury (AKI) with hyperkalemia
and uremia, pancreatic injury, and liver dysfunction with
coagulopathy concerning for disseminated intravascular
coagulation. Looking closer at the electrolytes, he has an
anion-gap metabolic acidosis.
Because of the multiple organ systems involved, the dif-
ferential diagnoses for this child include the following.
• Severe sepsis with multiorgan dysfunction syndrome
(MODS). Possible causes of sepsis include:
• Skin: 8-day history of inappropriately treated scald
burns to the feet, untreated wounds to right buttocks
••
and right knee.
• Gut: gastroenteritis (nausea, emesis), bowel obstruction
with bacterial translocation (nausea, bilious emesis).
• Pancreas: pancreatitis (nausea, emesis, elevated
lipase).
• Other unknown viral or bacterial etiology.
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Chapter 73 Child with Scald Burn Injury 309
demarcation, right buttock wound with skin sloughing,
possible head trauma (nausea, emesis), possible abdom-
inal trauma (nausea, emesis, liver and pancreatic injury).
acidosis and subsequent multiorgan dysfunction. This is
less likely given the history but should always be consid-
ered, especially in a developmentally delayed non-verbal
child.
Sepsis is defined as the presence of the systemic inflam-
matory response syndrome (SIRS) with a known or sus-
pected infection. SIRS is the presence of at least two of
the following: altered temperature, heart rate, respiratory
rate, or leukocyte count; however, either an altered temper-
ature or an altered leukocyte count should be present for
the patient to have SIRS. In this case, the child has tachy-
cardia, tachypnea, and granulocytosis (SIRS); there is con-
cern for an infection given his history of inappropriately
treated burns (sepsis); and he has signs of MODS.
What, if anything, is concerning about his basic
metabolic panel?
He has hyperkalemia due to sepsis, MODS, and AKI. He is
at high risk for worsening hyperkalemia if these conditions
are not managed promptly and appropriately. Patients with
sepsis, MODS, and burns have a high risk for developing
hyperkalemia due to potassium spillage from the damaged
cells into the extracellular space. AKI also increases the risk
for hyperkalemia due to a decreased ability to excrete potas-
sium through the urine, especially if the patient is oliguric
or anuric.
Hyperkalemia is defined as an elevation of serum potas-
sium levels. The normal range of potassium varies with age:
preterm 3.0–6.0 mEq/L
newborn 3.7–5.9 mEq/L
••
infant 4.1–5.3 mEq/L
child 3.4-4.7 mEq/L
•
adult 3.5-5.1 mEq/L.

310 Cases in Pediatric Acute Care
Hyperkalemia is frequently seen in patients with renal
disease, excess exogenous potassium administration, criti-
cal illness (e.g. sepsis, septic shock, MODS), or in patients
taking a medication that raises the serum potassium level
(e.g. angiotensin-converting enzyme inhibitors, spironolac-
tone). Hyperkalemia alters the cardiomyocyte action
potential, leading to cardiac arrhythmias. Even though
electrocardiogram (EKG) changes do not correlate with
the severity of hyperkalemia, there is usually a progression
of EKG findings as the potassium level rises. Peaked
T waves and PR prolongation are initially seen, followed
by widened QRS and P-wave loss. Eventually, the widened
QRS complexes fuse with the wide and tall ST–T segments,
forming a sine-wave pattern (Figure 73.2) that can lead to
ventricular fibrillation if not promptly treated. Even though
severe hyperkalemia can be life-threatening, the type of
EKG alteration is more predictive of life-threatening hyper-
kalemia than the level itself. In contrast to peaked T waves,
QRS widening heralds a life-threatening hyperkalemia that
should be emergently treated.
Would you continue the current maintenance IVF
at this time?
With AKI and sepsis, the patient’s hyperkalemia could
worsen, especially if he is given potassium-containing IVF.
His urine output should be monitored closely and the IVF
rate should be decreased or stopped if he becomes oliguric
or anuric to prevent fluid overload. If the decision is made
to continue IVF administration, KCl should be removed.
In addition to following the PALS guidelines for
cardiac arrest, what is the best way to manage the
patient’s condition?
The patient had multiple potential causes of cardiac arrest:
multiorgan failure associated with septic shock, hyperkale-
mia-induced arrhythmia with subsequent cardiogenic shock,
and aspiration of emesis. His hyperkalemia may be exacer-
bated by the administration of potassium-containing IVF in
the setting of AKI and ongoing cellular injury from MODS.
Figure 73.2 Peaked T waves, widened QRS complex, and loss of P waves seen on EKG.
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In addition to following the PALS guidelines for manage-
ment of septic shock and cardiopulmonary arrest, the child
should be aggressively treated for hyperkalemia, a part of
the H’s of the reversible causes of cardiac arrest described
in the PALS guidelines. All patients with hyperkalemia
should have any potassium supplementation discontinued
and should have an EKG to evaluate for cardiac arrhyth-
mias. Emergent management of severe hyperkalemia
requires stabilization of the cardiomyocyte membrane
potential with IV calcium (calcium chloride is preferred
over calcium gluconate, although either can be used).
Potassium is shifted intracellularly with IV sodium bicarbo-
nate, nebulized β-adrenergic agonists such as albuterol, and
IV insulin (bolus is recommended over other routes of
administration due to faster onset of action). Insulin should
always be administered with dextrose to avoid causing
hypoglycemia. Other therapies to remove potassium from
the body include administration of diuretics that increase
urinary potassium excretion (e.g. furosemide); cation
exchange resins such as enteral sodium polystyrene sulfo-
nate (Kayexalate), which bind potassium in the large intes-
tine in exchange for sodium; and hemodialysis, which
directly removes potassium from the blood. The effect of
diuretics and cation exchange resins on serum potassium
takes time, and these medications are not effective in
patients with oliguria/anuria or bowel pathology. They
should not be used independently for emergent manage-
ment of hyperkalemia. Hemodialysis is an effective way
to quickly lower the serum potassium level and is used in
patients with acute, persistent, or life-threatening
hyperkalemia.
If a patient remains in cardiac arrest despite standard
resuscitation measures, ECPR can be instituted in some
centers. ECPR is early placement of a child with cardiac
arrest on extracorporeal life support such as extracorporeal
membrane oxygenation (ECMO). ECMO provides cardiac
and/or respiratory support in patients refractory to conven-
tional therapy, but with a potentially reversible cause of ill-
ness, such as hyperkalemia-induced arrhythmia or septic
shock. Despite its life-saving potential, there is considerable
morbidity and mortality associated with ECMO.
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Chapter 73 Child with Scald Burn Injury 311

Would you obtain any other diagnostic studies? arrhythmias have subsided or have been controlled.
Frequent physical examinations and assessments of the
A bacterial blood culture should be obtained as soon as
vital signs are imperative to determine the patient’s hemo-
possible. Broad-spectrum antibiotics should be started
dynamic status. Evaluating the child’s temperature, respi-
immediately in patients with suspected sepsis, even if
ratory rate, oxygen saturation, pulse, heart rate, blood
blood culture is delayed. If clinically indicated, other spe-
pressure, capillary refill time, urine output, and fluid status
cific microorganisms (bacteria, viral, fungal) can be iden-
will give evidence of the child’s response to therapy. Blood
tified via culture, polymerase chain reaction (PCR),
gas analysis may help assess adequacy of therapy and
antigen, or antibody detection from sources such as respi-
whether the patient’s shock is improving.
ratory secretions, blood, urine, stool, vesicles, or cerebro-
spinal fluid if feasible. Antivirals or antifungals should be
started as soon as possible if there is high suspicion for What other therapies would be recommended at
such microorganisms, even if obtaining diagnostic studies this time?
is delayed.
The child should be started on hemodialysis as soon as pos-
In addition to assessing and managing infection (sepsis,
sible. Dialysis decreases serum potassium levels relatively
septic shock), other causes of cardiac arrest should be eval-
quickly and is indicated in acute, persistent, or life-
uated and treated if present. Reversible causes of cardiac
threatening hyperkalemia. Hemofiltration and hemodialy-
arrest include the H’s and T’s described in the PALS guide-
sis can be performed through the ECMO circuit. Otherwise,
lines. Hypovolemia can be reversed with IVF boluses; vas-
a hemodialysis catheter is required.
oactive medications can help with contractility and
Once the child stabilizes, further imaging may be
vasoconstriction to further improve cardiac output.
obtained to assess for head trauma, abdominal trauma,
Hypoxia can be treated with supplemental oxygen and
and fractures. If there is suspicion of non-accidental trauma
assessed via pulse oximetry or blood gas testing. A blood
or neglect, social work should be consulted and a referral
gas can also help assess the severity of respiratory and met-
made to child protective services. Given the delay in seek-
abolic acidosis. A point-of-care blood sugar check can
ing medical care for severe burns, a child protective services
determine if hypoglycemia is present, which should be
referral is appropriate in the case presented.
promptly treated with IV dextrose. The patient should
Application of topical antimicrobials such as silver
be warmed to normal body temperature if a temperature
sulfadiazine (Silvadene) and daily dressing changes to
check demonstrates hypothermia. Auscultating for breath
his wounds are also important to prevent infection and
sounds bilaterally and, if in doubt, performing needle
other wound complications. However, they are not a sub-
decompression can be used to evaluate for and treat a ten-
stitute for systemic antibiotics and these should be contin-
sion pneumothorax. A targeted bedside echocardiogram
ued and tailored to the organism isolated from culture
can be done to assess for cardiac tamponade. Blind peri-
results.
cardiocentesis is rarely performed, but may be considered
with high suspicion of cardiac tamponade. Blood and
urine testing can be sent to assess for toxins. Pulmonary
embolism or myocardial ischemia, although rare in this Case Resolution
child’s age group, can be suggested via EKG and
confirmed via chest computed tomography or cardiac He is started on an insulin and dextrose infusion and con-
catheterization. Arrhythmias can be assessed with EKG tinuous renal replacement therapy via the ECMO circuit.
or telemetry and treated with antiarrhythmics. Amiodar- As his potassium level decreases, his EKG findings change
one or lidocaine is recommended for unstable patients from sinusoidal wave appearance to atrioventricular block,
with ventricular fibrillation or pulseless ventricular during which time he has return of spontaneous circula-
tachycardia. tion. His EKG eventually changes to sinus rhythm as the
potassium level completely normalizes. The patient is con-
tinued on broad-spectrum antibiotics until his blood cul-
How would you determine if the therapy was
ture isolates Klebsiella pneumoniae, for which he finishes
effective?
a full course of IV cefepime. He is successfully decannu-
Frequent repeated evaluations of both serum potassium lated from ECMO 4 days following the cardiac arrest, but
(via serial blood gases or serum electrolyte panel) and continues to require mechanical ventilation and renal
telemetry, defibrillator monitor, or EKG are needed to replacement therapy in the pediatric intensive care unit
trend the potassium level and to assess whether the for several weeks.

312 Cases in Pediatric Acute Care
Further Reading
Arnholt, A.M., Duval-Arnould, J.M., McNamara, L.M., et al.
(2015). Comparatively evaluating medication preparation
sequences for treatment of hyperkalemia in pediatric
cardiac arrest: a prospective, randomized, simulation-based
study. Pediatric Critical Care Medicine 16: e224–e230.
Duff, J.P., Topjian, A., Berg, M.D., et al. (2018). 2018 American
Heart Association focused update on pediatric advanced life
support: an update to the American Heart Association
guidelines for cardiopulmonary resuscitation and
emergency cardiovascular care. Circulation 138: e731–e739.
Durfey, N., Lehnhof, B., and Bergeson, A. (2017). Severe
hyperkalemia: can the electrocardiogram risk stratify for
short-term adverse events? West Journal of Emergency
Medicine 18(5): 963–971.
Hughes, H.K. and Kahl, L.K. (ed.) (2018). The Johns Hopkins
Hospital The Harriet Lane Handbook, 21st edn. Philadelphia,
PA: Elsevier.
Kleinman, M.E., Chameides, L., Scheznayder, S.M., et al.
(2010). Part 14: Pediatric Advanced Life Support: 2010
American Heart Association guidelines for
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
cardiopulmonary resuscitation and emergency
cardiovascular care. Circulation 122(Suppl 3): S876–S908.
Lee, J. and Moffett, B.S. (2016). Treatment of pediatric
hyperkalemia with sodium polystyrene sulfonate. Pediatric
Nephrology 31: 2113–2117.
Littmann, L. and Gibbs, M.A. (2018). Electrocardiographic
manifestations of severe hyperkalemia. Journal of
Electrocardiology 51: 814–817.
Petrov, D.B. (2012). An electrocardiographic sine wave in
hyperkalemia. New England Journal of Medicine 366
(19): 1824.
Prusakowski, M.K. and Chen, A.P. (2017). Pediatric sepsis.
Emergency Medicine Clinics of North America 35: 123–138.
Tijssen, J.A. and Filler, G. (2017). When CRRT on ECMO is not
enough for potassium clearance: a case report. Canadian
Journal of Kidney Health and Disease 4: 2054358117722559.
doi: 10.1177/2054358117722559.
Wooten, J.M., Kupferman, F.E., and Kupferman, J.C. (2019). A
brief review of the pharmacology of hyperkalemia: causes
and treatment. Southern Medical Journal 112(4): 228–233.

74
Parvovirus Requiring Blood Transfusion
Ashley Thibodeau
Seattle Children’s Hospital, Seattle, WA, USA
A 2-year-old male presents to urgent care with viral symp-
toms, lethargy, and pale appearance.
History of Present Illness
Last evening, this 2-year-old male presented to urgent care
with viral symptoms, lethargy, and pale appearance. He has
a recent history of parvovirus, diagnosed by his primary
care provider (PCP) 5 days prior to presentation. He has
been acting tired and looking pale, so his mother brought
him to urgent care. A complete blood count (CBC) was
drawn and demonstrated a hemoglobin of 6.5 g/dL and
hematocrit of 18%. Previous iron deficiency anemia screen-
ings at 12 and 19 months by his PCP had been normal. The
child eats a regular diet and normal amount of milk for his
age. He was admitted to an acute care unit for slow blood
transfusion for symptomatic relief of anemia. Following
transfusion therapy, intravenous immunoglobulin admin-
istration was planned to treat red cell aplasia caused by
parvovirus.
Past Medical History
No significant past medical or surgical history. Normal
term delivery with no pregnancy complications. He was
discharged from the hospital on day of life 2 with mother.
No medication or food allergies. The child is taking vitamin
D and children’s multivitamin.
Family History
No significant history. No history of anemia, no blood dis-
orders or clotting disorders.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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313
Social History
He lives at home with mother, father, and 5-year-old sister.
No pets. No daycare.
Current Status
The child is admitted to the acute care service and you are the
provider covering his management. You receive a call from
the acute care nurse who reports that the child is itching
his arms and squirming around in the bed. You meet the
child, his mother, and the nurse at the bedside and they report
the itching began approximately 5 minutes ago. The red blood
cell transfusion has been infusing for 45 minutes. The child
had no prior rashes, skin infections, or history of eczema.
The vital signs at the time of your assessment (45 minutes
into transfusion) are temperature 37.0 C (98.6 F), heart rate
102 bpm, respiratory rate 34 breaths per minute, blood pres-
sure 96/52 mmHg, and oxygen saturation 100% on room air.
The vital signs at the start of packed red blood cell infu-
sion were temperature 36.5 C (97.7 F), heart rate 98 bpm,
respiratory rate 32 breaths per minute, blood pressure
92/50 mmHg, and oxygen saturation 100% on room air.
An urgent complete blood count is ordered with the
results shown in Table 74.1.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for the acute change
in this child?
2 What is your immediate response for this patient?
Describe your assessment of each body system.
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314 Cases in Pediatric Acute Care

Table 74.1 Complete blood count and reticulocyte count. reaction, febrile non-hemolytic reaction, allergic reaction,
anaphylactoid/anaphylactic reaction, and transfusion-
White blood cell count 9000/mm3 Normal
related acute lung injury. Non-immunologic complica-
Hemoglobin 7 g/dL Low tions include transmission of infection including
Hematocrit 19.8% Low cytomegalovirus and bacterial sepsis, transfusion-
Mean corpuscular volume 78 fL Normal associated circulatory overload, hypothermia, and other
Mean corpuscular hemoglobin 26 pg Normal metabolic derangements. The most common type of
Mean corpuscular hemoglobin 14.0% Normal transfusion reaction during red blood cell or platelet
concentration transfusion is an allergic reaction. There are additional,
Red blood cell count 4.1 × 106/ Normal less-common potential transfusion reactions that should
mm3 be considered when caring for a patient receiving a blood
Red cell distribution width 13.0% Normal product. In order of prevalence they are: febrile non-
Platelet count 250 000/ Normal hemolytic reaction, circulatory volume overload, acute
mm3 hemolysis, hypotensive reaction, bacterial contamination,
Reticulocyte count 2.2% High sepsis, acute lung injury, and air embolism.
Corrected reticulocyte index 3.6% Elevated
What is your immediate response for this patient?
Describe your assessment of each body system
Continuation of case: The child appears calm, pale, and is It is essential to verify that the blood transfusion has been
itching his arms. He is interactive with his mother, has no stopped and 0.9% saline is infusing intravenously. The
change in voice quality, and denies pain or stomach ache. patient should immediately be assessed for evidence of air-
His face is symmetric and pale. His oropharynx is pink way, breathing, or circulatory compromise. As you evaluate
without exudate or edema with tonsils 1+ bilaterally, ton- the patient, it is important to obtain any history of previous
gue protruded midline. His breath sounds are clear bilater- allergic reactions or exposures, any complaints of pain,
ally with no cough, wheeze, crackles, or increased work unusual sensation in the throat, change in voice, or gastro-
of breathing. He has a normal cardiac rate and rhythm intestinal symptoms including nausea and stomach ache.
at apex, with warm and well-perfused extremities with Vital signs should be obtained.
2-second capillary refill. His abdomen is protuberant, soft, Your evaluation by system should include the following.

•
non-tender, with normoactive bowel sounds. He has three
General: mental status, ability to respond to questions,
urticarial lesions on his neck and scratches on his arms
anxiety, perception of impending doom.

from itching. No other skin changes on lower extremities
or trunk are noted.
3 Based on the additional information, what differential
diagnoses will you continue to consider?
4 What pharmacologic interventions may be necessary
for this patient?
5 Would you resume the blood transfusion?
•
Head, eyes, ear, nose and throat: facial color, nasal con-
gestion, patency of the throat, mucosal edema.
• Cardiovascular: chest pain, central and peripheral
perfusion.
•• Lungs: breath sounds, work of breathing.
Integument: presence of urticarial or other rash,
skin color.

It is necessary to verify the label on the blood transfusion

bag and also notify the blood bank of a suspected transfu-
Rationale and Evidence-based Practice sion reaction. Additional laboratory studies may be
Explanation required as directed by the blood bank.

Based on the data given, what are the differential
diagnoses and the most likely diagnosis for the
acute change in this child?
The onset of the child’s symptoms suggest that he is experi-
encing a reaction to the blood transfusion. Blood transfusion
reactions are divided into immediate immunologic compli-
cations and non-immunologic complications. Immediate
immunologic complications include hemolytic transfusion
Based on the additional information, what
differential diagnoses will you continue to
consider?
The child is not having respiratory symptoms, such as tach-
ypnea, hypoxemia, bronchospasm, or dyspnea, so acute
lung injury and circulatory volume overload are less likely
at this time. The most common signs/symptoms of a trans-
fusion reaction are fever, chills, pruritis, and urticaria. Any

increase in temperature with chills, rigors, hypotension,
nausea or vomiting should lead you to consider a hemolytic
reaction or reaction due to bacterial contamination. Since
he has not had an increase in temperature of 1 C or more
and did not have a temperature above 38.0 C, febrile trans-
fusion reaction and possible sepsis could be excluded from
the differential diagnosis. However, new signs could still
arise and transfusion reactions can develop up to 24 hours
following the transfusion. One should always be aware of
the potential for a more severe reaction to develop and
for the signs which indicate a severe transfusion reaction.
Other findings of more severe transfusion reactions include
chills, rigors, nausea, vomiting, hypotension, respiratory
distress, hemoglobinuria, loss of consciousness, back pain,
jaundice, abnormal bleeding, and oliguria. This child was
experiencing an allergic blood transfusion that was limited
to urticaria, though he required ongoing evaluation for
more extensive signs and symptoms. The CBC obtained
during the transfusion demonstrated a slight rise in the
hemoglobin and hematocrit, appropriate for the volume
of red cells that had been administered at the time the sam-
ple was drawn.
What pharmacologic interventions may be
necessary for this patient?
An H1 antihistamine is recommended to treat the symptom
of hives and itching. The pediatric dosing recommendation
is 1–2 mg/kg. If the reaction progressed and was consistent
with anaphylaxis, additional intervention would be
required according to Pediatric Advanced Life Support.
Signs of anaphylaxis include bronchospasm, respiratory
Further Reading
American Academy of Blood Banks (2017). Circular of
information for the use of human blood and blood
components. https://www.aabb.org/tm/coi/Documents/
coi1017.pdf
American Academy of Pediatrics and American Heart
Association (2016). Pediatric Advanced Life Support. Dallas,
TX: American Heart Association.
Delaney, M., Wendel, S., Bercovitz, R.S., et al. (2016).
Transfusion reactions: prevention, diagnosis, and treatment.
Lancet 388: 2825–2836.
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Chapter 74 Parvovirus Requiring Blood Transfusion 315
distress, hypotension, and gastrointestinal upset. The
first-line intervention for anaphylaxis is intramuscular epi-
nephrine (1:1000) at a dose of 0.01 mg/kg, with a maximum
dose 0.3 mg. In addition to the H1 antihistamine, an H2
antihistamine such as ranitidine may also be prescribed
for cutaneous manifestations. Corticosteroids may also be
indicated. A normal saline bolus of 20 mL/kg may be admi-
nistered for hemodynamic instability. In the setting of
wheezing, albuterol, a β2-adrenergic receptor agonist bron-
chodilator, may also be administered via inhaler or
nebulizer.
Would you resume the blood transfusion?
If the patient’s only symptom of blood product transfusion
reaction was urticaria that resolved after diphenhydramine
administration, the transfusion with the same unit can be
restarted at a slower rate with close observation. If addi-
tional signs of allergic reaction or new signs of fever or other
type of reaction occur, the unit should be returned to the
blood bank accompanied by appropriate transfusion reac-
tion laboratory sampling.
Case Resolution
This child tolerated resumption of the packed cell transfu-
sion at a slower rate. The urticarial rash resolved and the
follow-up CBC demonstrated an appropriate rise in hemo-
globin and hematocrit.
Lexicomp Online. Diphenhydramine. Updated 8 June 2019;
accessed June 10 2019.
Oakley, F.D., Woods, M., Arnold, S., et al. (2015) Transfusion
reactions in pediatric compared with adult patients.
Transfusion 55(3): 563–570.

75
A Teen With High Fever, Rash and Lethargy
Jessica Strohm Farber
Children’s Hospital of Philadelphia, Philadelphia, PA, USA
A 16-year-old, previously healthy adolescent female pre-
sents to the emergency department (ED) with fever, weak-
ness, malaise, lethargy, and rash.
History of Present Illness
This 16-year-old became ill 2 days ago with fatigue,
headache, and abdominal pain. Her mother administered
ibuprofen for pain, increased fluids, and encouraged rest
believing the teen had a viral illness. Last night, she
developed fever to 40.1 C (104.1 F), chills, vomiting, and
weakness. She was not tolerating oral intake and remained
febrile this morning with lethargy. She developed a rash
this afternoon and has not voided in 18 hours so was
brought to the ED.
Past Medical History
The patient was born at term and was a healthy infant. All
vaccines are up to date except for influenza vaccine that she
refused this year. Her weight is at the 50th percentile and
height at 60th percentile for gender and age. She has never
been hospitalized, but has had two prior ED visits in the last
5 years for sports-related injuries.
Past Surgical History
The teen had myringotomy tubes placed for recurrent otitis
media in early childhood.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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317
Family History
The patient’s mother has a history of migraine headaches.
Her father has hypertension that developed in adulthood
and had childhood asthma. She has one sibling (10-year-
old female) who is healthy.
Current Status
On arrival to the ED, the patient is febrile to 39.4 C
(102.9 F) orally, pale, toxic appearing, tachypneic with a
respiratory rate of 28 breaths per minute, has sinus tachy-
cardia with a heart rate of 145 bpm, and blood pressure of
78/42 mmHg. She is oriented but lethargic and weak with
cool hands and feet and peripheral capillary refill of 3–4
seconds. Peripheral pulses are somewhat weak, but femoral
and jugular pulses are strong. She has a generalized
erythematous macular rash (Figures 75.1 and 75.2) and
oropharyngeal, tongue (Figure 75.3), and conjunctival
hyperemia. Her abdomen is soft without focal tenderness.
Bowel sounds are hypoactive.
Investigations including a blood culture, complete blood
count, and basic metabolic panel are obtained with results
as noted in Tables 75.1 and 75.2.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and what is the most likely diagnosis for this
adolescent?
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318 Cases in Pediatric Acute Care

Figure 75.1 Erythematous macular rash. Source: Courtesy of The

Centers for Disease Control and Prevention (CDC).

Figure 75.3 Oropharyngeal findings. Source: Courtesy of The

Centers for Disease Control and Prevention (CDC).

Table 75.1 Basic metabolic profile.

Sodium 136 mEq/L

Potassium 4.2 mEq/L
Chloride 101 mEq/L
Carbon dioxide 17 mEq/L
Blood urea nitrogen 28 mg/dL
Creatinine 2.2 mg/dL
Glucose 164 mg/dL
Calcium 7.8 mg/dL

Table 75.2 Complete blood count.

Figure 75.2 Erythematous macular rash. Source: Courtesy of The
White blood cell count 28 000/mm3
Centers for Disease Control and Prevention (CDC).
Hemoglobin 10.2 g/dL
Hematocrit 30.6%
2 What other historical information would be Platelet count 72 000/mm3
beneficial? Neutrophils 80%
3 What additional laboratory studies would be helpful to Lymphocytes 13%
understand the severity of the disease process and help Monocytes 5%
guide therapy? Eosinophils 2%
4 What are initial treatment priorities?

Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and what is the most likely diagnosis for
this adolescent?
Differential diagnoses for this patient include septic shock,
toxic shock syndrome, acute abdominal process, Kawasaki
disease shock syndrome, viral syndrome, and pneumonia.
The most likely diagnosis is toxic shock syndrome (TSS).
Symptoms in TSS are associated with both the infectious
process and toxin secretion and include fever, rash, and
hypotension. Other symptoms are those associated with
serious acute bacterial infection, such as general malaise,
weakness, and chills. Headache, sore throat, and gastroin-
testinal symptoms such as emesis, nausea, diarrhea, and
abdominal pain may also occur (Gottlieb et al., 2018).
The clinical case definition of Staphylococcus aureus TSS
includes initial clinical findings of fever, a rash that is
typically diffuse macular erythroderma, hypotension, and
multisystem involvement (Kimberlin et al., 2018). The child
in this case has evidence of multisystem involvement
including emesis, elevated serum creatinine, and hemato-
logic effects along with fever, rash, and hypotension, thus
meeting this case definition.
What other historical information would be
beneficial?
TSS may be caused by strains of group A Streptococcus or,
more commonly, toxin-producing strains of Staphylococcus
aureus. TSS is typically subdivided into two categories:
menstrual TSS and non-menstrual TSS (LeRiche et al.,
2012). While the majority of menstrual toxic shock cases
are associated with tampon use, TSS can be related to
bacterial infections such as pneumonia, pharyngitis, soft
tissue infections, or viral infections including influenza
(Gottlieb et al., 2018). Eliciting the history for tampon
use in females may reveal contributory factors.
What additional laboratory studies would be
helpful to understand the severity of the disease
process and help guide therapy?
Given the presenting symptom of tachypnea, a chest radi-
ograph may be obtained. However, tachycardia, tachypnea,
and hypotension are likely clinical manifestations of septic
shock. Decreased oral intake may indicate a component of
hypovolemic shock.
Additional laboratory evaluation could include creatine
phosphokinase with muscular complaints, hepatic function
tests, and additional cultures to identify fever source
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Chapter 75 A Teen With High Fever, Rash and Lethargy 319
(Wilkins et al., 2017). A serum lactate to evaluate for evi-
dence of metabolic acidosis and shock should be obtained,
and a blood gas can also support diagnosis.
Serial laboratory studies should be obtained to evaluate
the severity of shock and organ dysfunction (such as blood
gas and lactate) as well as to evaluate for disseminated
intravascular coagulation, anemia, thrombocytopenia,
and electrolyte abnormalities.
Lastly, the identification of infection source and any inter-
ventions such as surgical debridement or others as necessary
to remove the infection source are indicated (Wilkins et al.,
2017). Cerebrospinal fluid infectious studies should be con-
sidered based on presenting symptoms (LeRiche et al., 2012).
In this case, a history of tampon use is the likely etiology of
the TSS in this adolescent female.
What are initial treatment priorities?
Fluid resuscitation, hemodynamic stabilization, and rever-
sal of shock are a significant priority. Following current
guidelines for the timely recognition and management of
shock and monitoring for reversal of shock to ensure ade-
quate end-organ perfusion is vital. Monitoring capillary
refill and pulses, urine output, mental status, and estab-
lished blood pressure targets are recommended. Treatment
priorities include the identification and control of fever
source, appropriate antimicrobial therapy, as well as possi-
ble surgical evaluation for instances of soft tissue involve-
ment. As with any case of septic shock, the timely
administration of appropriate broad-spectrum antibiotics
is imperative. In suspected TSS, coverage for group
A Streptococcus, Staphylococcus aureus species, as well as
methicillin-resistant Staphylococcus aureus (MRSA) is indi-
cated (Wilkins et al., 2017).
Continuation of case: The patient receives a total of
60 mL/kg crystalloid fluids followed by an epinephrine infu-
sion at 0.05 μg/kg per min for hemodynamic support. She
receives vancomycin and ceftriaxone for presumed sepsis
and is admitted to the pediatric intensive care unit (PICU).
Her blood pressure and work of breathing improve after fluid
resuscitation and initiation of the epinephrine infusion. The
blood culture is found to be positive for Staphylococcus
aureus at 6 hours. Infectious disease specialty is consulted.
Additional Questions and Evidence-
based Rationale
5 What antibiotics are appropriate at this time?
6 How would you determine if the interventions were
effective?

320 Cases in Pediatric Acute Care
What antibiotics are appropriate at this time?
The rapid administration of broad-spectrum antibiotics is
indicated, including coverage for MRSA. This patient has
received ceftriaxone and vancomycin. However, clinda-
mycin is indicated as well in the treatment of TSS, largely
to inhibit toxin production but also to potentiate phagocy-
tosis and other beneficial effects (Wilkins et al., 2017).
Other antimicrobials may be used instead of clindamycin
to reduce toxin release including erythromycin,
fluoroquinolones, linezolid, or rifampin (Gottlieb
et al., 2017).
References
Gottlieb, M., Long, B., and Koyfman, A. (2018). The evaluation
and management of toxic shock syndrome in the emergency
department: a review of the literature. Journal of Emergency
Medicine 54(6): 807–814. doi: 10.1016/j.
jemermed.2017.12.048.
Kimberlin, D.W., Brady, M.T., Jackson, M.A., and Long, S.S.
(ed.) (2018) Red Book 2018: Report of the Committee on
Infectious Diseases, 31st edn. Itasca, IL: American Academy
of Pediatrics.
Further Reading
Centers for Disease Control and Prevention (CDC) (2011).
Toxic shock syndrome (other than streptococcal) (TSS) 2011
case definition. Available at https://wwwn.cdc.gov/nndss/
conditions/toxic-shock-syndrome-other-than-
streptococcal/case-definition/2011/
Davis, A.L., Carcillo, J.A., Aneja, R.K., et al. (2017). American
College of Critical Care Medicine clinical practice
parameters for hemodynamic support of pediatric and
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How would you determine if the interventions
were effective?
Appropriate shock resuscitation should result in improved
hemodynamics with resolution of hypotension and tachy-
cardia. Metabolic acidosis and any elevation in serum lac-
tate should also resolve with adequate resuscitation. The
fever curve should improve as well, and the patient should
become afebrile by approximately day 4–5 of illness.
Gastrointestinal symptoms such as abdominal pain and
diarrhea may persist for 7–10 days. Skin changes may prog-
ress or develop into desquamation at approximately day
7–10, then begin to resolve (LeRiche et al., 2012).
LeRiche, T., Black, A., and Fleming, N.A. (2012). Toxic shock
syndrome of a probable gynecologic source in an adolescent:
a case report and review of the literature. Journal of Pediatric
and Adolescent Gynecology 25(6): e133–e137. doi: 10.1016/j.
jpag.2012.08.011.
Wilkins, A.L., Steer, A.C., Smeesters, P.R., and Curtis, N.
(2017). Toxic shock syndrome: the seven Rs of management
and treatment. Journal of Infection 74(Suppl 1): S147–S152.
doi: 10.1016/S0163-4453(17)30206-2.
neonatal septic shock. Critical Care Medicine 45: 1061–1093.
doi: 10.1097/CCM.0000000000002425.
Lin, Y., Cheng, M., Lo, M., and Chien, S. (2015). Early
differentiation of Kawasaki disease shock syndrome and
toxic shock syndrome in a pediatric intensive care unit.
Pediatric Infectious Disease Journal 34(11): 1163–1167. doi:
10.1097/INF.0000000000000852.

76
A 9-Year-Old With Scoliosis
Alicia McCarthy
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 9-year-old female presents to the outpatient orthopedic
clinic for evaluation of scoliosis.
History of Present Illness
This 9-year-old girl was seen recently by the primary care
provider (PCP) for annual well child check. PCP noticed left
thoracic prominence on Adams forward bend test. There-
fore, she was referred to orthopedics for further evaluation.
Her family had not previously noticed asymmetry of her
back and the patient denies having pain.
Past Medical History
The patient was born at 36 weeks’ gestation, mother with
premature rupture of membranes, delivered vaginally, with
no neonatal complications. Development included rolling
over at 3 months and walking at 14 months. She has had
all required immunizations and received annual influenza
vaccine. She has never been hospitalized, but did require
physical therapy for an ankle sprain 2 years ago. There
are no chronic health conditions. Her current weight is
30 kg, height 115 cm.
Past Surgical History
No previous surgical procedures.
Family History
Negative for scoliosis or other orthopedic issues. No neuro-
muscular conditions in the family.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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321
Social History
The patient lives at home with her parents and brother and
is in the 4th grade. She is a competitive gymnast, with
practice and competitions taking up 15 hours per week.
Current Status
This patient is an alert and cooperative female who is in no
acute distress, and with age-appropriate affect and mood.
She has no dysmorphic features and walks and changes
positions independently. Neck range of motion (ROM)
shows loss of 10 of left lateral rotation compared to right.
Also loss of 20 of left head tilt compared to right. No
increased work of breathing. Standing evaluation reveals
her head is level over the pelvis, left shoulder higher than
right. No trunk shift. Iliac crest height is symmetric. No
pain on palpation of the spine, with forward bending or
hyperextension. Adams forward best test reveals left
thoracic prominence. Skin shows no café au lait spots,
hemangiomas, hairy patches, or skin dimpling. Bilateral
upper extremities are grossly symmetrical with normal
alignment and full ROM. Bilateral lower extremities show
left calf circumference is 1.5 cm smaller than right, left leg
also 1 cm shorter than right, with bilaterally normal
alignment and full ROM.
Questions
Answer the following question using the details provided.
1 Based on history and physical examination, what are
the differential diagnoses for this child?

322 Cases in Pediatric Acute Care
Figure 76.1 Scoliosis radiographs.
Continuation of case: Further examination of the upper
extremities reveals 5/5 strength in all major muscle groups.
Sensation intact to light touch bilaterally. Patellar deep ten-
don reflexes are 3+ on left, 1+ on right. Achilles deep ten-
don reflexes are 3+ on left, 1+ on right. Abdominal reflexes
are asymmetric. Babinski negative bilaterally. Ankle clonus
of three beats on left, zero beats on right. The patient walks
with a heel–toe gait and bears equal weight through both
lower extremities. She can walk on her heels and toes
independently with normal strength and coordination.
Patient was sent to radiology for erect posteroanterior/
lateral scoliosis radiographs (entire spine on single cassette)
(Figure 76.1).
2 Based on these additional findings, what are the next
steps in management?
3 What time frame would be appropriate to obtain
additional imaging?
Rationale and Evidence-based Practice
Explanation
Based on history and physical examination, what
are the differential diagnoses for this child?
The differential diagnoses for this child include idiopathic
scoliosis or secondary scoliosis. Scoliosis is defined as a lat-
eral curve in the spine of 10 or more. Idiopathic scoliosis
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refers to patients for whom there is no specific cause; how-
ever, there is a genetic component. Secondary scoliosis
refers to patients who have another diagnosis that is caus-
ing the scoliosis. Given this child’s age, unusual curve pat-
tern, and abnormalities seen on physical and neurologic
examination, further work-up is necessary to determine a
possible secondary cause.
Based on these additional findings, what are the
next steps in management?
After initial spine radiographs, this patient should receive
a full spine (cervical, thoracic and lumbar) MRI to inves-
tigate any intraspinal pathology that may be contributing
to her curve. Most common findings include Chiari
I malformation, syrinx and/or tethered cord. Approxi-
mately 20% of patients with scoliosis under the age of
10 have one of these findings. The pathophysiology of
Chiari I malformation is poorly understood, although
some have suggested that the formation of a syrinx, a
fluid-filled cavity located within the spinal cord or in
the brainstem, may cause anterior horn cell dysfunction,
resulting in scoliosis (Kelly et al., 2015). If imaging is pos-
itive, referral to neurosurgery would be appropriate. If
imaging is negative, referral to neurology for evaluation
of other etiologies would be the next step.
For idiopathic scoliosis, treatment with a trunk brace is
recommended if a curve reaches 25 while the child has
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Chapter 76 A 9-Year-Old With Scoliosis 323

growth remaining and surgical intervention may be neces- What time frame would be appropriate to obtain
sary if the scoliosis progresses to the severe range, additional imaging?
usually 50 . For secondary scoliosis, treatment recommen-
The patient has abnormal reflexes and calf size, but
dations depend on the etiology and the location of the
strength, sensation and gait are unaffected by the scoliosis.
curve. If the child has a Chiari I malformation, syrinx
Therefore, MRI should be obtained urgently, but not emer-
and/or tethered cord, surgical correction may result in
gently. Waiting up to several weeks, as long as the patient
improvement or lack of progression of the scoliosis. At
has no change in neurologic status, would be appropriate.
minimum, this patient would require ongoing monitoring
of her spine to evaluate for any changes to the curve.

Reference
Kelly, M.P., Guillaume, J.T., and Lenke, L.G. (2015). Spinal Neurosurgical Clinics of North America 26(4): 579–585. doi:
deformity associated with Chiari malformation. 10.1016/j.nec.2015.06.005.

Further Reading
Chotai, S., Basem, J., Gannon, S., et al. (2018). Effect of neural axis anomalies in idiopathic scoliosis: a systematic
posterior fossa decompression for Chiari malformation I on review. Spine Journal 18(7): 1261–1271. doi: 10.1016/j.
scoliosis. Pediatric Neurosurgery 53(2): 108–115. doi: spinee.2018.02.013.
10.1159/000485254.
Heemskerk, J.L., Kruyt, M.C., Colo, D., Castelein, R.M., and
Kempen, D.H.R. (2018). Prevalence and risk factors for
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325

77

An Unresponsive Infant
Charlene Pringle
University of Florida, Gainesville, FL, USA

A 9-month-old infant boy with no significant past medical Past Medical History
history was admitted to the pediatric intensive care unit
(PICU) after cardiopulmonary arrest at home. The baby was born at term and went home with his
mother. He has had all required vaccines up to 6 months
and his height and weight recorded on a growth chart
noted weight in the 16th percentile, length in the 8th
History of Present Illness percentile, weight for length in the 50th percentile, and
head circumference well above the 99th percentile.
This 9-month-old baby who was born at term is being
Newborn metabolic screening was reported as normal.
transferred from a referring hospital following cardiopul-
His parents state they discussed the large head with the
monary arrest with roughly 1 hour without cardiac or
primary care provider at a well child visit early in life.
respiratory function prior to return of spontaneous circu-
He was also spitting up after every meal. They never
lation (ROSC). Parents report that he has been “sick” and
noticed any abnormal movements and do not report any
has seen his primary care provider several times since
known trauma. The infant is home with parents, does
birth for respiratory illnesses and vomiting, but has never
not attend daycare, and there are no other caregivers.
required hospital admission. He has not been febrile
Social services have been involved with this family and
recently. The father of the infant reports that he put the
there is currently an open case for another child in the
baby in his bouncy seat to sleep. He heard him coughing,
home per the emergency department (ED) social worker.
and went to check on him. After finding him limp and not
breathing, he attempted stimulation without success.
Emergency medical service (EMS) was called and arrived
5–10 minutes after the call. No cardiopulmonary resusci-
tation was performed while awaiting EMS. On arrival of Past Surgical History
EMS, baby was noted to be in full arrest. Compressions
and one dose of epinephrine was given en route to the hos- The infant had a circumcision at age 1 day of life, with no
pital. Compressions continued with four additional doses bleeding or complications.
of epinephrine administered at referring hospital with
ROSC roughly 1 hour after initial call. The first blood
gas obtained indicated a pH of 6.8. The patient was intu-
bated and transferred for further care. Initial arterial blood Family History
gas analysis on arrival of transport team confirmed severe
metabolic acidosis with pH 6.7 and lactate 11.7 mmol/L. Parents are both healthy, but the father is being treated
The infant received two 20 mL/kg isotonic fluid boluses with methadone for substance use. There is a 3-year-old sib-
and was started on an epinephrine infusion for ling who has been treated in the ED several times for viral
hypotension. illnesses.

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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326 Cases in Pediatric Acute Care

Current Status Table 77.3 Arterial blood gas analysis.

pH 7.1
The baby is intubated and mechanically ventilated, receiv-
PCO2 58 mmHg
ing epinephrine infusion at 0.1 μg/kg per min. His exami-
nation indicates macrocephaly and a bulging and tense PO2 234 mmHg
anterior fontanelle. Pupils are unequal in size, with reactiv- Bicarbonate 15 mEq/L
ity as follows; left pupil mid-position fixed and 7 mm; right Base excess +3
pupil 2–3 mm and sluggishly reactive to light. Corneal
reflexes are absent. Heart sounds are normal, with strong
peripheral pulses and tachycardia. There is clear nasal dis-
Table 77.4 Coagulation studies.
charge, and lungs are clear bilaterally to auscultation. The
infant is breathing over ventilator breaths with retractions Prothrombin time (PT) 14.8 s
with some agonal breaths. Abdomen is soft and non- Partial thromboplastin time (PTT) 46 s
distended. No response to deep pain, nor gag reflex noted
International normalized ratio 2.0
with suctioning. Skin is mottled with prolonged capillary
Fibrinogen 185 mg/dL
refill time, cyanosis to feet. No bruising or other skin lesions

Table 77.1 Complete metabolic panel.

Sodium 140 mEq/L

Potassium 5.1 mEq/L
Chloride 109 mEq/L
Carbon dioxide 10 mEq/L
Blood urea nitrogen 14 mg/dL
Creatinine 0.32 mg/dL
Glucose 100 mg/dL
Calcium 8.1 mg/dL
Albumin 2.8 mg/dL
Total protein 5.4 g/dL
Aspartate aminotransferase 230 U/L
Alanine aminotransferase 568 U/L
Alkaline phosphatase 156 U/L
Total bilirubin 0.3 mg/dL

Table 77.2 Complete blood count and inflammatory markers.

White blood cell count 14 000/mm3

Hemoglobin 8 g/dL Figure 77.1 Non-contrast CT brain, image 1.

Hematocrit 25.6%
observed. A complete metabolic panel, complete blood
Platelet count 340 000/mm3 count, coagulation studies, arterial blood gas analysis,
Neutrophils 51% and inflammatory markers were obtained, with results as
Lymphocytes 45% shown in Tables 77.1–77.4. A chest radiograph is unre-
Monocytes 1% markable and shows endotracheal tube position 1 cm above
Eosinophils 3% the carina. Non-contrast computed tomography (CT) of the
Erythrocyte sedimentation rate 42 mm/hour head reveals large bilateral acute-on-chronic subdural
hematomas, as well as early diffuse cerebral edema
C-reactive protein 304 mg/L
(Figures 77.1–77.3).

Figure 77.2 Non-contrast CT brain, image 2.
Figure 77.3 Non-contrast CT brain, image 3.
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Chapter 77 An Unresponsive Infant 327
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What other studies are indicated to confirm your
diagnosis?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
There could be many potential diagnoses for this child, but
the primary differential diagnoses include abusive head
trauma, accidental injury, sudden infant death syndrome,
underlying bleeding disorder, and toxic ingestion. After
resuscitation, determining the cause of unresponsiveness
is the next challenge. The results of the CT scan obviously
offer information, but the eye examination is a quicker eval-
uation and can be telling. The history does not suggest an
accidental injury nor an ingestion, but if the infant is crawl-
ing he may easily find something on the floor to ingest.
The most likely diagnosis for this infant is abusive head
trauma. The acute-on-chronic nature of the subdural hema-
tomas noted on CT scan demonstrate repeat bleeding events.
The denial of traumatic injury as well as the severity of injury
limits the likelihood of accidental injury. An underlying
bleeding disorder is extremely rare, but should be considered
if a pertinent patient or family history is obtained.
What other studies are indicated to confirm your
diagnosis?
An additional diagnosis for this infant is hypoxic ischemic
encephalopathy due to his prolonged cardiopulmonary
arrest. The early global cerebral edema on the initial head
CT is characteristic of hypoxic ischemic encephalopathy
and the elevated lactic acid, liver enzymes and metabolic
acidosis are all evidence of global ischemic insult. Addi-
tional studies should also include a skeletal survey and
indirect ophthalmoscopy evaluation to determine addi-
tional injury. If the child is sufficiently stable, brain mag-
netic resonance imaging (MRI) can be more precise than
CT, and there is no exposure to radiation. Serum
amylase/lipase, liver enzymes and urinalysis should be
obtained to screen for abdominal trauma.

328 Cases in Pediatric Acute Care
Figure 77.4 Retinal hemorrhage.
Retinal hemorrhages are found in 65–85% of patients
with abusive head trauma (Figure 77.4). It is hypothesized
that the eye is also subjected to the acceleration/decelera-
tion forces that the brain is subjected to during abusive
traumatic injury. These hemorrhages are typically bilateral,
multilayered, and extend to the periphery. Rib fractures are
commonly encountered when the mechanism of injury is
shaking; other bony fractures can also be associated.
Continuation of case: The infant is admitted to the PICU
and standard care for hypoxic ischemic encephalopathy
and traumatic brain injury is instituted. Pediatric neurosur-
gery is consulted, emergently performs a subdural tap
through the fontanelle and drains 100 mL of blood, after
which the pupils transiently become equal in size at
3 mm and sluggishly reactive bilaterally. Despite standard
supportive care including seizure prophylaxis, head eleva-
tion and midline, normoxia, euglycemia, normothermia,
normoventilation and adequate blood pressure, the infant
progresses to brain death on day 4 of hospitalization.
Additional Questions and Evidence-
based Rationale
3 What does supportive care for hypoxic ischemic
encephalopathy and traumatic brain injury include?
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Table 77.5 Brain Trauma Foundation: pediatric traumatic brain
injury guidelines.
Monitoring
Intracranial pressure (ICP) monitoring
Advanced neuromonitoring
Specific guidelines for CT imaging
Thresholds
Treatment of ICP to <20 mmHg
Treatment for cerebral perfusion pressure target between 40 and
50 mmHg
Treatment
Hyperosmolar therapy: hypertonic saline, initial 3%, specific use
for 23.4%
Specific uses for analgesics, sedatives and neuromuscular
blockade to avoid brain hypoperfusion
Seizure prophylaxis
Normoventilation
Normothermia, hypothermia to control increasing ICP
Cerebrospinal fluid drainage
Use of barbiturates for hemodynamically stable patients with
refractory intracranial hypertension
Decompressive craniotomy for neurologic deterioration,
increased ICP not responsive to medical therapy, or herniation
Initiation of early enteral feedings for nutrition
Use of corticosteroids is not recommended
Source: Kochanek et al. (2019).
4 What determines effective therapy?
5 What are some risk factors for non-accidental trauma?
What does supportive care for hypoxic ischemic
encephalopathy and traumatic brain injury
include?
The ultimate goal in treating traumatic brain injury is to
reduce secondary brain injury by limiting cerebral ischemia
and optimizing cerebral blood flow. The Brain Trauma
Foundation has recently published the third edition of evi-
dence-based guidelines for management of traumatic brain
injury in children, which includes the recommendations
shown in Table 77.5.
What determines effective therapy?
In the case of hypoxic ischemic encephalopathy and abu-
sive head trauma, the only mechanisms available are sup-
portive care and surgical intervention if indicated. In this
case, drainage of the subdural hematoma proved to be a

temporizing measure. Overall supportive care includes sei-
zure prophylaxis and maintenance of normonatremia,
euglycemia, normothermia, normoventilation, and normo-
tension. The head should be elevated and kept in the mid-
line to optimize cerebral venous return. Avoidance of
hypoxia is paramount. Avoiding hyponatremia lessens risk
of increase in cerebral edema. Avoiding hypoglycemia or
hyperglycemia optimizes cerebral metabolic demand.
Avoiding hypoventilation or hyperventilation optimizes
cerebral blood flow. Ensuring normotension is important
for maintaining cerebral perfusion pressure. While thera-
peutic hypothermia has proven successful in other popula-
tions, there is not sufficient evidence in children to
recommend this therapy as routine at this time. Fever
should be avoided to optimize cerebral metabolic demand.
Frequent, repeated physical examinations including
focused neurologic assessment are needed to determine
the effectiveness of therapy or progression to brain death.
Reference
Kochanek, P., Tasker, R.C., Carney, N., et al. (2019) Guidelines
for the management of pediatric severe traumatic brain
injury, third edition: update of the Brain Trauma
Further Reading
Armijo, V. (2016). Child abuse and neglect. In: Comprehensive
Critical Care: Pediatric (ed. J. Stockwell and M. Kutko),
763–774. Mount Prospect, IL: Society for Critical Care
Medicine.
Fink, E., Clark, R., Bayir, H., et al. (2017). Hypoxic-ischemic
encephalopathy: pathobiology and therapy of the post-
resuscitation syndrome in children. In: Pediatric Critical
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Chapter 77 An Unresponsive Infant 329
Continuous electroencephalography may be useful in
ensuring seizure control.
What are some risk factors for non-accidental
trauma?
Identified risk factors for non-accidental traumatic brain
injury fit into two categories: household risk factors and
risk factors in the child. Household risk factors include sub-
stance abuse, mental illness, domestic violence, single par-
enting, poverty, increased family size, and social isolation.
While traditionally it was thought that non-related male
caregivers were typical perpetrators, recent demographic
evidence demonstrates that the majority of perpetrators
are the parents of the child. Risk factors in the child include
younger age (<3 years), prematurity, low birthweight,
chronic or recurrent illness, and either a developmental
or physical disability.
Foundation guidelines, executive Summary. Neurosurgery
84(6): 1169–1178. doi: 10.1093/neuros/nyz051.
Care, 5th edn (ed. B.P. Fuhrman and J.J. Zimmerman), 929–
951. Philadelphia, PA: Elsevier.
O’Lynnger, T.M., Shannon, C.N., and Le, T.M. (2016).
Standardizing ICU management of pediatric traumatic
brain injury is associated with improved outcomes at
discharge. Journal of Neurosurgery Pediatrics 17: 19–26. doi:
10.3171/2015.5.PEDS1544.
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331

78

A Toddler With Abdominal Pain, Diarrhea and Lethargy

Jessica Pech
Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, USA

An 18-month-old male presents to the emergency depart- circumference. Developmental milestones reached: rolled
ment (ED) with irritability, suspected abdominal pain, over at 5 months, crawled at 9 months, walked at
and lethargy. 13 months, and is saying about six words at present.

History of Present Illness Past Surgical History

An afebrile 18-month-old male presents to the ED with irri- Uncomplicated circumcision the first day of life.
tability, non-bloody diarrhea, and lethargy for the past
24 hours. His mother describes the pain as intermittent,
as he plays in between. Each episode is lasting about
10–15 minutes followed by a period of fatigue. He has
Family History
had decreased oral intake and increased lethargy over the
Mother has migraine headaches, father with elevated
past 12 hours and had one episode of non-bilious emesis
cholesterol, maternal grandmother with a history of breast
this morning and his last wet diaper was 12 hours ago.
cancer, treated and in remission, and maternal grandfather
A week ago, the child had symptoms of an upper
has hypertension, type 2 diabetes mellitus, coronary artery
respiratory infection and a low-grade fever but these symp-
disease, s/p coronary stent placement.
toms have resolved. His 4-year-old sister is currently sick
with symptoms of a cold. The child attends daycare and
there has been no recent travel.
Social History

He lives with his parents and 4-year-old sister (healthy),

Past Medical History
The patient was born full term via uncomplicated vaginal
delivery. Mother had prenatal care starting at 10 weeks’
gestation and her laboratory testing was negative for
sexually transmitted infection, HIV, rubella and group
B Streptococcus. The baby required phototherapy for hyper-
bilirubinemia and was discharged home on the third day of
life. He has received required immunizations up to current
age, including MMR (measles, mumps, rubella) and first
varicella and first and second influenza vaccinations.
Growth and development has been as targeted. He is at
the 50% for weight, 60% for height, and 85% for head
and attends daycare.
Current Status
Initial vital signs in the ED are temperature 38.5 C
(101.3 F), heart rate 124 bpm, respiratory rate 28 breaths
per minute, and blood pressure 96/48 mmHg. The child
is awake and alert and appears comfortable. The only
abnormal physical finding is mild diffuse abdominal
tenderness with palpation. Laboratory results are shown
in Tables 78.1 and 78.2. Abdominal X-ray showed no

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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332 Cases in Pediatric Acute Care

Table 78.1 Basic metabolic panel. Meckel’s diverticulum. Reviewing the primary symptoms
of each of these problems would not support a diagnosis
Sodium 140 mEq/L
for this child. Gastroenteritis would entail more persistent
Potassium 3.6 mEq/L vomiting and diarrhea, malrotation with or without volvu-
Chloride 95 mEq/L lus would most likely present with bilious emesis, Meck-
Carbon dioxide 20.5 mEq/L el’s diverticulum would present with rectal bleeding, and
Blood urea nitrogen 12 mg/dL appendicitis is very unusual in this age child.
Creatinine 0.5 mg/dL
Glucose 82 mg/dL
Calcium 9.8 mg/dL
Additional Questions and Evidence-
based Rationale
Table 78.2 Complete blood count. 2 Describe the incidence, etiology and common risk fac-
tors associated with intussusception.
White blood cell count 13 500/mm3
3 What are the most common presenting symptoms asso-
Hemoglobin 11.8 g/dL ciated with intussusception?
Hematocrit 33% 4 What is the preferred method of radiologic imaging
Platelet count 306 000/mm3 used to diagnose intussusception and what other meth-
Neutrophils 78% ods of imaging may be considered?
Lymphocytes 12% 5 What is the preferred method of non-operative manage-
Bands 2% ment of intussusception?
6 What symptoms and risk factors are associated with
Eosinophils 2%
recurrent intussusception after enema reduction?
7 When is surgical intervention indicated for
intussusception?
obstruction and moderate stool burden. Abdominal ultra-
sound is shown in Figure 78.1.

Describe the incidence, etiology and common risk

Question
Answer the following question using the details provided.
1 Based on the available data, what are the possible differ-
ential diagnoses and the most likely diagnosis for
this child?
Rationale and Evidence-based Practice
Explanation
Based on the available data, what are the possible
differential diagnoses and the most likely
diagnosis for this child?
With regard to the presentation of lethargy, decreased
appetite, and colicky intermittent pain, the most likely
diagnosis for this child is intussusception. In many cases,
there is blood in the stool, noted as “currant jelly” in
appearance. Differential diagnoses include, but are not
limited to, viral gastroenteritis, malrotation, mesenteric
lymphadenitis, intussusception, acute appendicitis, or
factors associated with intussusception
Intussusception occurs in an estimated 3.4 in 10 000 chil-
dren each year and is most prevalent in children less than
3 years of age. Approximately 90% of intussusception cases
are classified as ileocolic, involving both the ileum and
colon at the region of the ileocecal valve. The remainder
of intussusception cases are isolated to the small bowel or
colon. An estimated 90% of pediatric intussusception cases
are idiopathic and attributed to a recent respiratory or gas-
trointestinal viral infection with no identifiable or patholo-
gic lead point (Gluckman et al., 2017). Viral infections such
as adenovirus, norovirus, and rotavirus have been linked to
intussusception as a result of hypertrophic lymphatic tissue
within the terminal ileum, known as Peyer’s patches, that
acts as a lead point (Gluckman et al., 2017). Other cases of
intussusception, occurring in children over the age of
5 years, can result from other pathologic problems includ-
ing intestinal duplication cysts, polyps, Meckel’s diverticu-
lum, and lymphoma (Gluckman et al., 2017). Further risk
factors include a history of cystic fibrosis complicated by
distal intestinal obstruction syndrome, the presence of prior
abdominal surgical adhesions, or Henoch–Schönlein
purpura.

Figure 78.1 Abdominal ultrasound: target sign noted superior to umbilicus, no lead point or free fluid.
What are the most common presenting symptoms
associated with intussusception?
The presenting symptoms of intussusception may be non-
specific or associated with the classic triad of symptoms that
includes vomiting, colicky abdominal pain, and hemato-
chezia or “currant jelly” stools (Gluckman et al., 2017).
A typical pain episode includes intermittent colicky abdom-
inal pain with legs drawn followed by periods of fatigue or
lethargy. Additional symptoms may include fever, diarrhea,
or anorexia, with the presence of “currant jelly” stools often
a late or unnoticed sign of intussusception.
What is the preferred method of radiologic
imaging used to diagnose intussusception and
what other methods of imaging may be
considered?
Abdominal ultrasound is currently the standard of care for
diagnosis of intussusception. The presence of intussuscep-
tion on ultrasound may be described as a “bull’s eye” or
“target” sign demonstrating the affected area of telescoped
bowel (see Figure 78.1). Additional methods of imaging
include abdominal radiography or computed tomography
(CT) (Smith and Fox, 2016). The presence of a “crescent”
sign on abdominal radiograph may be indicative of intus-
susception, although the sensitivity for accurate diagnosis
of intussusception is only 62–86% (Gluckman et al.,
2017). CT scan may be indicated with concern for bowel
obstruction, perforation, or in cases of recurrent intussus-
ception, but CT imaging poses risk of radiation exposure
and should not be routinely utilized.
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Chapter 78 A Toddler With Abdominal Pain, Diarrhea and Lethargy 333
What is the preferred method of non-operative
management of intussusception?
The presence of suspected or confirmed intussusception
requires urgent evaluation and treatment. Initial manage-
ment includes intravenous fluid resuscitation, serial
abdominal examinations, review of diagnostic imaging,
and pediatric surgery consultation to evaluate for enema
reduction versus surgical intervention. Enema reduction
is the standard of care for non-operative management of
uncomplicated intussusception, with a success rate of
80–95%. Enema reduction should be avoided in patients
with peritonitis, bowel obstruction, or bowel perforation.
Successful enema reduction is performed by radiology
using instillation of air, saline or water-soluble contrast
per rectum in an attempt to increase intraluminal bowel
pressure (Gluckman et al., 2017). There are data suggesting
that air enema reduction is more beneficial than contrast
enema reduction due to speed, safety and lower perforation
rates when compared with liquid enema reduction.
What symptoms and risk factors are associated
with recurrent intussusception after enema
reduction?
After successful enema reduction, the child will be
admitted to the hospital for observation or monitored in
the ED for evidence of recurrent intussusception prior to
discharge. Despite successful enema reduction, the pres-
ence of abdominal pain, vomiting, hematochezia, or diet
intolerance may indicate recurrence and require repeat
imaging or enema reduction (Smith and Fox, 2016). The
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334 Cases in Pediatric Acute Care

recurrence rate after successful enema reduction is approx-
imately 5–10% at 24 hours, but decreasing by 48 hours. Risk
factors include children over 2 years of age, duration of
symptoms greater than 48 hours, rectal bleeding, or the
presence of a palpable mass or pathologic lead point (Xie
et al., 2018).
When is surgical intervention indicated for
intussusception?
Surgical intervention may be indicated with failed attempts
at enema reduction or with the presence of a pathologic
lead point, acute peritonitis, or bowel perforation. In
uncomplicated cases of intussusception. surgical or manual
reduction is performed by a minimally invasive or laparo-
scopic approach. The presence of bowel necrosis, perfora-
tion, or a pathologic lead point may indicate the need for
an exploratory laparoscopy or laparotomy with excision
of mass and possible bowel resection.

References
Gluckman, S., Karpelowsky, J., Webster, A., and McGee, R.G. Xie, X., Wu, Y., Wang, Q., Zhao, Y., and Xiang, B. (2018). Risk
(2017). Management of intussusception in children (review). factors for recurrence of Intussusception in pediatric
Cochrane Database of Systematic Reviews (6): CD006476. doi: patients: a retrospective study. Journal of Pediatric Surgery
10.1002/14651858.CD006476.pub3 53(11): 2307–2311. doi: 10.1016/j.jpedsurg.2018.03.023.
Smith, J. and Fox, S. (2016). Pediatric abdominal pain: an
emergency medicine perspective. Emergency Medical Clinics of
North America 34: 341–361. doi: 10.1016/j.emc.2015.12.010.

Further Reading
Mallicote, M., Isani, M., Roberts, A., et al. (2017). Hospital
admission unnecessary for successful uncomplicated
radiographic reduction of pediatric intussusception.
American Journal of Surgery 214(6): 1203–1207. doi:
10.1016/j.amjsurg.2017.08.040.
Wong, C., Jin, S., Chen, J., et al. (2016). Predictors for bowel
resection and the presence of a pathological lead point for
operated childhood intussusception: a multi-center study.
Journal of Pediatric Surgery 51(12): 1998–2000.

79
Sore Throat and Shortness of Breath
Amy Manion
Rush University College of Nursing, Chicago, IL, USA
An 11-year-old, well-nourished boy presents to the emer-
gency department (ED) with sore throat and shortness of
breath.
History of Present Illness
This 11-year-old boy became ill 3 days ago when he woke
up with sore throat and fever of 38.3 C (101 F). His mother
brought him to see his primary care provider on his first day
of illness. A rapid group A Streptococcus (GAS) test was
positive in the office. He was diagnosed with GAS pharyn-
gitis and started on a 10-day course of 800 mg amoxicillin
(400 mg/5 mL) orally twice daily. He has not had a fever
since starting the antibiotics. On day 3 of illness, he was
sleeping for approximately 2 hours when he awoke sud-
denly and started gasping for air and coughing, at which
time his mother decided to bring him to the emergency
department (ED) for further evaluation.
His mother reports that he is often tired during the day
and falls asleep at school. He has had prominent night-time
snoring for the past 3 years and often complains of head-
aches. Over the last three nights the snoring has gotten lou-
der. He was recently seen by otolaryngology (ENT) and
diagnosed with enlarged tonsils and adenoids. He is sched-
uled for polysomnography next week.
Past Medical History
Born at 38 weeks. He was small for gestational age (SGA)
and weighed 2041 g. He spent 7 days in the neonatal inten-
sive care unit (NICU) because of SGA concerns and tachyp-
nea. He required oxygen support by nasal cannula for
2 days and then was weaned off oxygen by day 3 of life.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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335
He was discharged home at 1 week of age weighing 2268 g.
He is up to date with all immunizations including his
annual influenza vaccine. His weight has been above the
99th percentile for gender and age since age 6. Currently
he weighs 68.18 kg and his height is 145 cm. His body mass
index (BMI) is 32.5, which places him in the 99th percentile
for his age and gender. He has a history of nocturnal enu-
resis and takes desmopressin 0.4 mg 1 hour before bedtime.
He is followed by a pediatric urologist. His renal ultrasound
is normal. No history of urinary reflux or urinary tract
infections. He was diagnosed with attention deficit/hyper-
activity disorder (ADHD) 2 years ago by his primary care
provider. He takes methylphenidate extended-release
18 mg tablets once daily. No history of asthma. No prior
hospitalizations.
Past Surgical History
He had tympanostomy tubes placed at 28 months of age for
frequent otitis media with effusion.
Family History
His father has type 2 diabetes. His paternal grandfather also
has type 2 diabetes and had a stroke at 55 years of age. He
does not have any siblings. Both of his parents are obese.
Current Status
On examination in the ED, he has moderate inspiratory
stridor while awake with an oxygen saturation of 96% on
room air. His respiratory rate is 22 breaths per minute with

336 Cases in Pediatric Acute Care
moderate work of breathing. His lungs are clear, but with
referred upper airway noise to auscultation. Mouth and
throat examination reveal enlarged tonsils, grade 3+ with
erythema. No head or neck lymphadenopathy is noted.
Question
Answer the following question using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include obstructive
sleep apnea, GAS pharyngitis, viral infection, foreign body
aspiration, allergic reaction, asthma, epiglottitis, peritonsil-
lar abscess, retropharyngeal abscess, and panic attack.
On further evaluation, the patient has normal complete
blood count results and lack of fever, moving epiglottitis,
peritonsillar abscess, and retropharyngeal abscess lower
on the differential diagnosis. The recent acute episode of
streptococcal pharyngitis is being adequately treated with
the amoxicillin, but may be associated with residual tonsil-
lar edema. Panic attacks caused by stress and anxiety can
occur in the school-aged child. Symptoms seen with panic
attacks such as shortness of breath and hyperventilation
can present acutely. Although this child awoke suddenly
with shortness of breath and gasping for air, his symptoms
did improve with racemic epinephrine, which is used to
treat upper airway edema. A diagnosis of asthma exacerba-
tion and/or allergic reaction is less likely due to normal
chest radiograph and lack of expiratory wheezing, forced
exhalation pattern, hives, or facial swelling.
The most likely diagnosis for this child is OSA exacer-
bated by streptococcal pharyngitis infection. This child is
at risk for OSA due to his history of enlarged adenoids
and tonsils and obesity (BMI >95th percentile), further
exacerbated by an infection.
Continuation of case: He is treated with nebulized race-
mic epinephrine 2.25% solution, 0.5 mL diluted in 3 mL
normal saline (0.05 mL/kg per dose; maximum 0.5 mL).
In addition, he receives oral dexamethasone 12 mg (0.6
mg/kg; maximum 12 mg). He is placed on continuous pulse
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Table 79.1 Complete blood count.
White blood cell count 8000/mm3
Hemoglobin 13 g/dL
Hematocrit 39%
Platelet count 310 000/mm3
oximetry. After completion of the nebulized racemic epi-
nephrine, he is resting comfortably with no audible stridor
with 98% oxygen saturation on room air.
After 2 hours of observation in the ED, he develops mod-
erately audible stridor and his oxygen saturation decreases
to 92% on room air. He is given a second dose of nebulized
racemic epinephrine and started on 30% humidified oxygen
by facemask. On humidified oxygen, his oxygen saturation
quickly increases to 98%. A chest radiograph is obtained
and is normal. A complete blood count is obtained and
the results are shown in Table 79.1.
He is admitted to the pediatric general care floor for fur-
ther monitoring and management due to his continued
need for humidified oxygen support, risk for rebound effect
after racemic epinephrine administration, and history of
enlarged adenoids. His oxygen saturation is 100% on
oxygen 5 L/min by facemask rebreather. When he falls
asleep his oxygen saturation decreases to 90% and he is
noted to be snoring loudly, with increased respiratory effort
and respiratory rate of 20 breaths per minute. The amoxi-
cillin treatment is continued to complete the 10-day course.
Additional Questions and
Evidence-based Rationale
2 What symptoms does this patient have that would sug-
gest obstructive sleep apnea (OSA) as a comorbidity?
3 Would you obtain any other diagnostic studies?
4 What birth defects or genetic disorders are linked
to OSA?
5 What medical therapy would you recommend for this
patient?
6 What surgical therapy would be beneficial for this
patient after resolution of his infectious symptoms?
What symptoms does this patient have that would
suggest OSA as a comorbidity?
Obesity can contribute to OSA due to the excess soft tissue
in the neck area, which obstructs the airway when the
patient’s muscles relax during sleep. This child is in the
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Chapter 79 Sore Throat and Shortness of Breath 337

99th percentile for BMI which puts him at risk for OSA.
Furthermore, he has been diagnosed by ENT with enlarged
tonsils and adenoids. Enlarged tonsils and adenoids can
occlude the airway during sleep and are a major contributor
to OSA in children. Symptoms of headache, hyperactivity
and nocturnal enuresis are often seen in children with
OSA. Headaches can be caused by short episodes of hypoxia
that frequently occur during the night due to temporary air-
way obstruction and apnea. This chronic lack of oxygen
during the night causes restless sleep and the child wakes
up tired despite having “slept” a full night. During the
day, the child is tired which can cause them to be irritable
and/or have difficulty concentrating. This chronic lack of
restful sleep can cause a patient with OSA to be diagnosed
with ADHD. The ADHD symptoms will often resolve once
the OSA is treated. Nocturnal enuresis is another comorbid-
ity associated with OSA that will resolve once the OSA is
properly treated.

Would you obtain any other diagnostic studies?

No further testing is warranted at this time due to acute ill-
ness. Although a physical examination is important for
diagnosis, large tonsils and adenoids do not directly indi-
cate the presence of OSA. Nocturnal polysomnography
(PSG) should be completed as scheduled on discharge in
order to confirm suspected diagnosis of OSA. Nocturnal
PSG (sleep study) is the gold standard for identification
of OSA in children (see Figure 79.1). The PSG diagnostic
criteria for OSA differ for adults versus children. The
PSG uses the apnea–hypopnea index to measures the num-
ber of apneas or hypopneas per hour and this determines
OSA severity: mild, more than one but less than five events
per hour; moderate, more than five events per hour; and
severe, more than 10 events per hour.
What birth defects or genetic disorders are linked
to OSA?
Children with Pierre Robin syndrome are at higher risk for
OSA due to micrognathia (small lower jaw) which causes a
crowded tongue. Children with Down syndrome are also at
increased risk of OSA due to their associated enlarged
tongue, adenoids and tonsils and decreased muscle tone.
What medical therapy would you recommend for
this patient?
Positive airway pressure is a non-surgical option for man-
agement of OSA in children. Both continuous positive air-
way pressure (CPAP) and bilevel positive airway pressure
(BiPAP) are the types of therapy often used during the night
to prevent airway obstruction and decrease work of
Figure 79.1 Patient undergoing polysomnography.
breathing so the patient can have a more normal sleep cycle
and wake up less tired. CPAP delivers positive pressure
throughout the entire respiratory cycle, whereas BiPAP
delivers more positive pressure during inspiration than
expiration. Additionally, positive-pressure ventilation
reduces the episodes of hypopnea and hypoxia, and this
reduces the risk of right heart failure. For children with
severe OSA, supplemental oxygen may also be necessary.
For treatment of mild OSA, intranasal corticosteroids are
an option to decrease inflammation in the airway especially
in children with seasonal allergies
What surgical therapy would be beneficial for this
patient once his infectious symptoms resolve?
For adenotonsillar hypertrophy, an adenotonsillectomy or
adenoidectomy is considered first-line treatment for chil-
dren with moderate to severe OSA. PSG should be repeated
if the patient is having persistent symptoms postopera-
tively. CPAP can be continued for persistent OSA despite
adenotonsillectomy intervention. Enuresis symptoms will
often improve or resolve after surgical intervention.

338 Cases in Pediatric Acute Care
Further Reading
Ahn, Y.M. (2010). Treatment of obstructive sleep apnea in
children. Korean Journal of Pediatrics 53(10): 872–879. doi:
10.3345/kjp.2010.53.10.872.
Alexopoulos, E., Malakasioti, G., Varlami, V., et al. (2014).
Nocturnal enuresis is associated with moderate-to-severe
obstructive sleep apnea in children with snoring. Pediatric
Research 76(6): 555–559.
Alsubie, H.S. and BaHammam, A.S. (2017). Obstructive sleep
apnoea: children are not little adults. Paediatric Respiratory
Reviews 21: 72–79. doi: 10.1016/j.prrv.2016.02.003.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Barone, J.B., Hanson, C., DaJusta, D.G., et al. (2009). Nocturnal
enuresis and overweight are associated with obstructive
sleep apnea. Pediatrics 124(1): e53–e59. doi: 10.1542/
peds.2008-2805
Marcus, C.L., Brooks, L.J., Ward, S.D., et al. (2012). Diagnosis
and management of childhood obstructive sleep apnea
syndrome. Pediatrics 130(3): e714–e755.

80
Oral Presentation and Communication
Jill Siegrist Thomas
University of Maryland Children’s Hospital, University of Maryland Medical Center, Baltimore, MD, USA
Effective and comprehensive communication is an essen-
tial skill of every healthcare provider for optimizing patient
safety and quality patient-centered care. This case presen-
tation will follow the same patient from the inpatient unit,
to the operating room, and to the pediatric intensive care
unit (PICU) to highlight various types of oral presentations
that may be required. The case progression will focus on
key information to present/handoff to other medical provi-
ders to prevent communication gaps potentially leading to
medication or other therapy errors and adverse events.
Case Presentation
A 4-month-old male with trisomy 21 and atrioventricular
septal defect (AVSD) is admitted to the inpatient pediatric
unit due to congestive heart failure.
History of Present Illness
The patient was doing well at home until 1 week prior to
admission when he developed tachypnea at rest with
decreased oral intake and slowed weight gain. His parents
report he sweats and takes frequent breaks during oral feed-
ing with worsening of respiratory effort, but does not
exhibit cyanosis. He consumes approximately 57–113 g
(2–4 ounces) of breast milk by bottle every 4 hours. He is
admitted from cardiology clinic for medical management
of congestive heart failure.
Past Medical History
Baby was born at term and discharged to home with his
mother. His weight has tracked along the 25th percentile
on the trisomy 21 growth curve for gender and age.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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339
Vaccines are up to date for age and he has no known aller-
gies, and is not taking any prescribed or over-the-counter
medications. No recent fever, illness or sick contacts. No
rhinorrhea or congestion.
Past Surgical History
None. Elective repair of his AVSD is scheduled to occur in
1 month.
Family History
Parents and grandparents are reported to be healthy. There
is no familial history of congenital heart disease, trisomy 21,
or sudden or unexplained death.
Current Status
Vital signs are as follows: weight 5.2 kg, afebrile, heart rate
112 bpm, tachypnea with respiratory rate 72 breaths per
minute and mild subcostal retractions, blood pressure
74/48 mmHg, and oxygen saturation 77% on room air.
He has crackles bilaterally upon auscultation. A grade II/VI
holosystolic murmur is auscultated at the left lower sternal
border. Liver is palpable 2 cm below the right costal margin
and he is warm and well perfused. A peripheral intravenous
line has been placed and he receives a one-time intravenous
dose of furosemide 1 mg/kg and is ordered furosemide
1 mg/kg per dose daily PO and enalapril 0.1 mg/kg per dose
PO every 12 hours. A chest radiograph is significant for
mild bilateral pulmonary edema.

340 Cases in Pediatric Acute Care
Questions
Answer the following questions using the details provided.
1 What are the key components of an oral presentation
for a new admission?
2 Describe a comprehensive oral presentation for this
patient.
Continuation of case: The same patient has now under-
gone elective surgery, and is being admitted to the PICU
from the operating room.
3 What information is important to obtain during patient
handoff from the operating room to the PICU?
4 What barriers impact ideal communication systems
during patient handoff?
Continuation of case: Consider the next four questions
and then continue to read to determine the answers and
view examples of the handoff processes.
5 How can a structured or standardized handoff tool
improve communication and patient care?
6 How do shift-to-shift handoffs differ from oral presenta-
tions during multidisciplinary rounds?
7 What are the key components of a handoff
communication?
8 How do oral presentations during multidisciplinary
rounds differ from the presentation of a newly admitted
patient?
Rationale and Evidence-based Practice
Explanation
Oral presentation of a new admission closely follows the
written documentation of the history and physical exami-
nation and note performed on admission. Organization of
the data strives to set the context for the listener, presenting
the information in a concise, logical and story-like fashion.
This formatting facilitates processing of information and
ultimately leads listeners to derive similar diagnostic
conclusions.
What are the key components of an oral
presentation for a new admission?
Standard framework for oral presentation for a new
admission
A) Chief complaint and identifying information
B) History of present illness
C) Pertinent past medical history (pertinent positive and
negative information only)
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•• Birth history
Past medical and surgical history
•• Allergies and medications
Diet
•• Well child, developmental history and immunizations
Family history
•• Social history and determinants of health
Review of systems
D) Vital statistics and physical examination: targeted phys-
ical examination highlighting pertinent positive, nega-
tive or abnormal findings
E) Laboratory and diagnostic data
F) Assessment statement: one-sentence summary that
includes the patient’s age, sex, pertinent past medical
history, active issues and trajectory of illness
G) Differential diagnosis list
H) Plan: can be presented in a system or problem-based
format.
Describe a comprehensive oral presentation for
this patient
W.D. is a 4-month-old male with trisomy 21 and AVSD pre-
senting with tachypnea and poor weight gain.
History of present illness/pertinent past medical history
He was born full term, is bottle fed, and has been doing well
at home with fair weight gain. One week prior to admission
he developed tachypnea at rest with decreased oral intake
and slowed weight gain. His parents report he sweats, takes
frequent breaks, during oral feeding with worsening respi-
ratory effort. His oral intake has recently decreased, cur-
rently consuming 2–4 ounces of breast milk by bottle
every 4 hours. No concerns of cyanosis. He was admitted
from cardiology clinic due to concern for congestive heart
failure and optimization of medication management. He
takes no medications and is scheduled to have a complete
repair of his AVSD in 1 month. Of note, W.D. has had no
recent fever, illness, sick contacts, congestion or rhinor-
rhea. Prior to this admission he was growing well, trending
in the 25% percentile on his adjusted growth curve.
Vital signs/physical examination
Upon admission, his vital signs were significant for tachyp-
nea to the 70s in room air and oxygen saturation of 77%.
On examination he has crackles bilaterally. He has a
grade II/VI holosystolic murmur. He is warm and well
perfused. His liver is palpable 2 cm below the right costal
margin. Chest X-ray is significant for mild bilateral
pulmonary edema.

Assessment statement
W.D. is a 4-month-old male with trisomy 21 and AVSD
admitted with respiratory insufficiency, feeding difficulties
and poor weight gain due to congestive heart failure.
Differential diagnosis
The differential diagnosis list for this acute presentation
includes congestive heart failure, viral respiratory illness
and anemia. He has most likely developed congestive heart
failure due to his known structural heart disease. Fatigue
and associated feeding difficulties are likely due to insuffi-
cient cardiac output to meet his higher metabolic demands
while feeding. A viral infectious etiology for his tachypnea
and feeding difficulties is less likely since he is afebrile, and
has no sick contacts, rhinorrhea or congestion. Given his
history, anemia is also a less likely cause of congestive heart
failure in W.D.
Management plan by system
Cardiovascular: Administer a single dosage of IV furose-
mide 1 mg/kg due to his tachypnea and chest X-ray findings
of pulmonary edema. Will initiate furosemide 1 mg/kg per
dose daily PO and enalapril 0.1 mg/kg per dose PO every
12 hours. W.D. is scheduled for AVSD repair in 1 month.
I will notify surgery of his admission and discuss further
management with the cardiologist.
Respiratory: Monitor tachypnea. Initiate oxygen support
and/or consider high-flow nasal cannula (HFNC) if respira-
tory insufficiency worsens. Goal oxygen saturation is >80%.
Fluids/electrolytes/nutrition: Continue bottle feeds of
breast milk and monitor for increased work of breathing
with feeds. Send a basic metabolic panel to evaluate for
electrolyte anomalies.
Hematology: Send a complete blood count (CBC) to eval-
uate for anemia.
Infectious disease: He has no signs or symptoms of an
infectious etiology so I will not send a respiratory viral
panel at this time.
Social: I have updated W.D.’s family on his overall status
and plan of care.
In addition to using a standard framework for oral pre-
sentations, the use of a semi-structured rounding tool
and daily goal sheets can facilitate communication and
exchange of information between providers, patients and
families during multidisciplinary rounds and ensure team
member awareness of goals of care (Ganesan et al., 2017).
Consistent utilization of a safety checklist is associated with
decreased invasive device usage and patient care costs
(Tarrago et al., 2014).
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Chapter 80 Oral Presentation and Communication 341
What information is important to obtain during
patient handoff from the operating room to
the PICU?
Multidisciplinary handoff includes the cardiac surgeon,
anesthesiologist, PICU attending, advanced practice provider
(APP), respiratory therapist, and nurse at patient bedside.
Institutional factors and use of a structured handoff tool
will dictate the content of information included during
patient handoff from the operating room. The content
needs to include information from the sender (surgical
team representative and anesthesiologist) to the ICU team
needed to provide safe, complete and continuous care.
Key information includes the following (Chatterjee
et al., 2019).
1) Patient characteristics
a) Name, medical record number or date of birth
b) Age, weight, and sex
c) Preoperative diagnoses
2) Surgical sign-out
a) Operative procedure performed
b) Surgical findings and intraoperative complications
c) Cardiopulmonary bypass, aortic cross clamp, and cir-
culatory arrest times as applicable
d) Bleeding, rhythm or bypass separation issues
e) Presence of drains or pacing wires
f) Special instructions, surgical concerns, and antici-
pated adverse events
3) Anesthesia sign-out
a) Pertinent past medical history, preoperative medica-
tions, and allergies
b) Ease of laryngoscopy and airway issues
c) Ventilator and oxygenation issues with current ven-
tilator settings
d) Administration of fluid and blood products
e) Medications administered: includes vasopressors,
inotropes, analgesics, sedation, muscle relaxants
and antibiotics
f) Recent laboratory data prior to ICU arrival (hemoglo-
bin and ABG)
g) Desired hemodynamic goals
h) Special instructions, concerns or anticipated con-
cerns with extubation.
What barriers impact ideal communication
systems during patient handoff?
Handoff communication is an integral high-risk compo-
nent of patient care associated with an increased risk of
clinical instability and sentinel events (Ong and Coiera,

342 Cases in Pediatric Acute Care
2011). Failure to communicate or receive the important
information is multifactorial in nature. Disorganization
and miscommunication of information occurs due to
informal or unstructured sign-out processes and inconsist-
ent, incomplete or inaccurate transfer of information
between service lines. Human factors, including lack of
team focus, parallel conversations and number of indivi-
duals present during transfer of care, also contribute to
information miscommunication (Vergales et al., 2015;
Chatterjee et al., 2019).
How can a structured or standardized handoff tool
improve communication and patient care?
Effective handoff processes enhance interdisciplinary
team communication and patient safety. Utilization of for-
malized handoff processes such as standardized proce-
dures, scripts and checklists can improve transmission
of key data (Dixon et al., 2015). Use of a dedicated hand-
over process during transfer of care from the operating
room to the PICU after cardiothoracic surgery is also
associated with improved handover completeness, few
early postoperative events, improved compliances with
process measures, and provider satisfaction (Chatterjee
et al., 2019).
The following is an example of a PICU sign-out presen-
tation of a new postoperative patient.
W.D. is a 5-month-old male with trisomy 21, AVSD and
medically controlled heart failure who is admitted follow-
ing single patch AVSD repair with bovine pericardium.
Cardiovascular: There were no intraoperative complica-
tions. His cardiopulmonary bypass time was 132 minutes
and he had a cross clamp time of 56 minutes. Before coming
off cardiopulmonary bypass the patient underwent modi-
fied ultrafiltration for 100 mL. He arrived to PICU on epi-
nephrine and milrinone infusions. Since admission he
developed junctional ectopic tachycardia (JET) which
resolved with controlled normothermia, sedation and dis-
continued inotropic support. He is now in normal sinus
rhythm with a heart rate of approximately 120 bpm. Blood
pressures are 75/48 to 95/55 mmHg and renal near-infrared
spectroscopy (NIRS) are in the 60s. Examination is signifi-
cant for a grade II/VI mid-frequency systolic murmur at the
left lower sternal border. No gallop or rub. He is warm and
well perfused on a milrinone infusion of 0.5 μg/kg per min.
Lines include a right internal jugular central venous line, a
right radial arterial line, a left hand peripheral IV, and atrial
and ventricular pacing wires. The lactate is 1.1. Postopera-
tive transesophageal echocardiogram shows trivial residual
ventricular septal defect with left-to-right ventricular
shunt. No residual atrial septal defect. Normal biventricular
function.
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Respiratory: Remains intubated and mechanically venti-
lated on pressure regulated volume control. ABG 7.41/38/
194/24/1/98% on 40% FiO2. Chest X-ray shows mild pulmo-
nary edema without presence of pleural effusions. The
endotracheal tube and all lines are in good position.
Neurology: Pain is well controlled with scheduled aceta-
minophen and fentanyl PRNs for moderate pain. He is
sedated with dexmedetomidine at 1 μg/kg per hour.
Infectious disease: Receiving cefazolin for postoperative
prophylaxis.
Fluids/electrolytes/nutrition: Receiving D5 ½NS at 75%
maintenance. Electrolytes have been stable. Last electrolyte
panel is sodium 146, potassium 3.9, chloride 111, bicarbo-
nate 23, BUN 8, Creatinine 0.18, glucose 195, calcium
8.7, ionized calcium 1.31, magnesium 2.1 and phosphorus
4.9. No electrolyte supplementation given.
Hematology: After cardiopulmonary bypass he received
packed red blood cells (PRBCs), cell saver, platelets and
fresh frozen plasma. His hemoglobin and hematocrit are
11.9 and 35. Platelet count 142. PT 18.4, INR 1.4, PTT 38,
and fibrinogen 210. He has not required any blood products
since admission to the PICU. He has three chest tubes
draining serosanguineous fluid and output is approxi-
mately 1 mL/kg per hour.
Social: Parents are at bedside and updated on plan of care.
Overnight tasks: Monitor for dysrhythmias and bleeding.
Initiate diuresis with intermittent furosemide and chloro-
thiazide. Goal extubation is tomorrow morning – wean
the ventilator, attempt a pressure support trial and check
for an airway leak overnight. Lab schedule is ABGs and
electrolyte panel every 6 hours, CBC and coagulation stud-
ies every 12 hours. He is ordered for a CXR in the morning.
How do shift-to-shift handoffs differ from oral
presentations during multidisciplinary rounds?
Communication during a shift-to-shift handoff is brief and
concise, reporting only the necessary information needed to
assume care for the patient. Length of presentation will
vary based on the complexity of the patient. Structured
handoff communication tools assist in ensuring necessary
information is both given and received by healthcare
providers.
What are the key components of a handoff
communication?
Patient handoffs are high-risk times associated with
adverse and sentinel events. Use of the I-PASS Handoff Pro-
gram has been shown to facilitate efficient transition of
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Chapter 80 Oral Presentation and Communication 343

Table 80.1 IPASS format example.

I: Illness W.D. is a critically ill infant s/p cardiothoracic surgery. He is a full code
severity
P: Patient W.D. is a 4-month-old male with trisomy 21, AVSD, and medically controlled heart failure who was admitted
summary today following single-patch AVSD repair with bovine pericardium. There were no intraoperative complications
or surgical concerns. He remains intubated and sedated with adequate hemodynamics. After returning from the
OR he had JET which resolved with controlled normothermia, sedation and discontinuation of inotropic support
Pertinent review by systems:
CV: He is now in normal sinus rhythm after controlling his JET. His vital signs are stable with renal NIRS in the
60s. He is warm and well perfused on a milrinone infusion of 0.5 μg/kg per min. He has atrial and ventricular
pacing wires
RESP: He is mechanically ventilated without issues
FEN/GIGU: He is NPO on intravenous fluids. He has no electrolyte abnormalities
ID: He is receiving cefazolin for postoperative prophylaxis
HEME: His hemoglobin and hematocrit are 11.9 and 35.2. His chest tube output is approximately 1 mL/kg per hour
NEURO: He is receiving dexmedetomidine at 1 μg/kg per hour. His pain is well controlled with scheduled
acetaminophen and PRN fentanyl
A: Action Continue to assess for hemodynamic instability, dysrhythmias, and bleeding. Initiate diuresis with furosemide and
items chlorothiazide this evening if he is hemodynamically stable. Goal extubation is tomorrow morning – wean the
ventilator, attempt a pressure support trial and check for an airway leak overnight
S: Situation If he develops JET again, consider over-drive pacing. Please start dexamethasone to reduce airway swelling if he
awareness does not have a leak around the endotracheal tube
and
contingency
planning
S: Synthesis Okay, thanks. It sounds like he is doing well despite the JET. Overnight tasks will include monitoring for
by receiver dysrhythmia and bleeding, monitoring for hemodynamic stability, initiating diuresis with furosemide and
chlorothiazide and preparing W.D. for extubation in the morning. I don’t have any additional questions

AVSD, atrioventricular septal defect; OR, operating room; JET, junctional ectopic tachycardia; NIRS, near infrared spectroscopy.
Source: Data from Starmer et al. (2014).

information and decrease medical errors and adverse
events (Starmer et al., 2014). The cornerstone mnemonic
for the key elements of the handoff process are I, illness
severity; P, patient summary; A, action items; S, situation
awareness and contingency planning; and S, synthesis by
receiver. Table 80.1 presents the IPASS format for shift-
D) System or problem-based presentation of medications
with interpretation of laboratory and diagnostic data
E) Assessment statement: one-sentence summary which
includes the patient’s age, sex, pertinent past medical
history, active issues and trajectory of illness
F) Plan

to-shift handoff between W.D.’s providers.
How do oral presentations during multidisciplinary
rounds differ from the presentation of a newly admitted
patient?
Oral presentations during daily multidisciplinary rounds
concisely review the data from the previous 24 hours and
do not contain the depth of information presented during
an oral presentation of a patient who was recently admitted.
The standard framework for oral presentation during
multidisciplinary rounds is as follows.
A) Introductory statement: one-sentence summary which
includes the patient’s age, sex, pertinent past medical
history, reason for admission and active diagnosis
B) Pertinent overnight events and current active issues
C) Vital statistics and physical examination: targeted
physical examination highlighting pertinent positive,
negative or abnormal findings
••
Communicate overarching goals as well as daily plan
Can be presented in a systems or problem-based
format.
The following example includes an assessment and man-
agement plan for postoperative day 1 (POD #1) presented in
multidisciplinary rounds.
Assessment statement: W.D. is a 5 month old male with
trisomy 21 and AVSD now POD #1 s/p repair via a single
patch technique with resolved JET and adequate hemody-
namics who is being prepared for extubation.
Overnight events: W.D. remained intubated, sedated and
ventilated overnight. His cardiac output remained ade-
quate and he has had no further episodes of JET. He
is warm and well perfused on a milrinone infusion of
0.5 μg/kg per hour. His chest tube output decreased to
0.5 mL/kg per hour and diuresis was initiated with

344 Cases in Pediatric Acute Care
intermittent furosemide and chlorothiazide. The ventila-
tor has been weaned overnight and he has successfully
completed a pressure support trial.
Plan: Our major goals for the day will be to focus on extu-
bation and diuresis:
Cardiovascular: Continue milrinone at the current dose
through extubation for afterload reduction. Continue furo-
semide 1 mg/kg per dose every 6 hours and chlorothiazide
2.5 mg/kg every 6 hours for diuresis. His goal fluid balance
for the day is negative 100 mL. Monitor chest tube output.
Monitor cardiac rhythm and cardiovascular endpoints
including vital sign trends, perfusion, NIRS trends, urine
output and neurologic status.
Respiratory: Extubate to 2 L of oxygen via nasal can-
nula, with goal oxygen saturation of >92%. Obtain an
ABG 30 minutes after extubation. Include physical ther-
apy to help patient out of bed to a chair or held by care-
giver two to three times today for pulmonary toilet and to
References
Chatterjee, S., Shake, J.G., Arora, R.C., et al. (2019). Handoffs
from the operating room to the intensive care unit after
cardiothoracic surgery: From the Society of Thoracic
Surgeons workforce on critical care. Annals of Thoracic
Surgery 107(2): 619–630. doi: 10.1016/j.
athoracsur.2018.11.010.
Dixon, J.L., Stagg, H.W., Wehbe-Janek, H., et al. (2015). A
standard handoff improves cardiac surgical patient transfer:
operating room to intensive care unit. Journal for Healthcare
Quality 37(1): 22–32. doi: 10.1097/01.
JHQ.0000460123.91061.b3.
Ganesan, R., Rajakumar, P., Fogg, L., Silvestri, J., and Kane, J.
M. (2017). WE CARE 4 KIDS: use of a rounding tool in the
pediatric intensive care unit. Pediatric Quality and Safety 6
(2): e044. doi: 10.1097/pq9.0000000000000044.
Ong, M.S. and Coiera, E. (2011). A systematic review of failures
in handoff communication during intrahospital transfers.
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promote chest tube drainage. Obtain a chest X-ray in the
morning to evaluate lung fields, chest tube placement and
for the presence of pleural or pericardial effusions.
Fluids/electrolytes/nutrition: Continue IVF. May start
oral feeds of expressed breast milk 4–6 hours after extuba-
tion. Send a basic metabolic panel to evaluate for electrolyte
anomalies every 12 hours.
Neurology: Discontinue dexmedetomidine infusion and
PRN fentanyl now. Postoperative pain regimen is acetamin-
ophen 15 mg/kg PO every 6 hour and ketorolac 0.5 mg/kg
per dose every 6 hours.
Hematology: Send a CBC daily to evaluate for anemia or
elevation in white blood cell count and coagulation studies
daily while the chest tubes are in place.
Infectious disease: Continue cefazolin for 48 hours for
postoperative prophylaxis.
Social: W.D.’s parents remain at his bedside and updated
frequently.
Joint Commission Journal on Quality and Patient Safety 37
(6): 274–284. doi: 10.1016/S1553-7250(11)37035-3.
Starmer, A.J., Spector, N.D., Srivastava, R., et al. (2014).
Changes in medical errors after implementation of a handoff
program. New England Journal of Medicine 371(19): 1803–
1812. doi: 10.1056/NEJMsa1405556.
Tarrago, R., Nowak, J.E., Leonard, C.S., and Payne, N.R.
(2014). Reductions in invasive devise use and care costs after
institution of a daily safety checklist in a pediatric critical
care unit. Joint Commission Journal on Quality and
Patient Safety 40(6): 270–278. doi: 10.1016/S1553-7250(14)
40036-9.
Vergales, J., Addison, N., Vendittelli, A., et al. (2015). Face-to-
face handoff: improving transfer to the pediatric
intensive care unit after cardiac surgery. American Journal
of Medical Quality 30(2): 119–125. doi: 10.1177/
1062860613518419.

81
A 7-Year-Old with Migratory Joint Pain
Christin Diller1,2
1
Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA
2
Vanderbilt University College of Nursing, Nashville, TN, USA
A 7-year-old male presents to the emergency department
(ED) with new-onset joint pain.
History of Present Illness
Mother reports that he has been complaining of migratory
pain in knees, elbows, and ankles over last 2 days. She
reports he is a generally well and active child who is now
complaining of frequent pain, is more irritable, and is less
cooperative and today appeared to be short of breath when
playing after school. No fever in last 24 hours, unsure if any
fever in the last 7 days.
Past Medical History
No known drug allergies. No previous hospitalizations. No
previous surgeries. Up to date on immunizations. Has not
visited primary care provider in past 6 months. Mother states
he did stay home from school approximately 2 weeks ago for
2 days with fever and sore throat, but recovered quickly.
Family History
No family history of heart disease, cancer, or unexpected
death. Maternal grandmother has type 2 diabetes.
Current Status
On arrival to ED, vital signs are noted to be temperature
38.3 C (100.9 F) orally, heart rate 132 bpm, respiratory rate
38 breaths per minute, blood pressure 110/75 mmHg, and
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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345
pulse oximetry 89% on room air. On examination he is
awake, alert and oriented. He has coarse crackles through-
out all lung fields and is slightly tachypneic with mild
retractions. Normal S1 and S2 with a regurgitant systolic
murmur and gallop. He has warm hands and feet with cap-
illary refill of 2 seconds and 2+ peripheral pulses. Liver
2 cm below right costal margin. No abdominal tenderness
appreciated and active bowel sounds. He is placed on
oxygen 2 L/min via nasal cannula with improvement in
oxygen saturations to 93%. During examination, no com-
plaints of joint pain. A chest radiograph, complete blood
count, and basic metabolic profile are ordered and the
results are shown in Tables 81.1 and 81.2. The chest radio-
graph shows evidence of pulmonary edema.
Questions
Answer the following questions using the details provided.
1 What are the differential diagnoses?
2 What additional laboratory studies would be useful in
making a diagnosis?
3 What additional studies would you order?
Rationale and Evidence-based Practice
Explanation
What are the differential diagnoses?
Differential diagnoses for this patient would include myo-
carditis, endocarditis, and rheumatic heart disease based on
history and physical examination findings of gallop, tachy-
cardia, crackles, and new-onset murmur, which suggest a
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346 Cases in Pediatric Acute Care

Table 81.1 Basic metabolic profile.
Sodium 139 mEq/L
Potassium 3.9 mEq/L
Chloride 101 mEql/L
Carbon dioxide 24 mEq/L
Blood urea nitrogen 25 mg/dL
Creatinine 0.9 mg/dL
Glucose 115 mg/dL
Table 81.2 Complete blood count.
White blood cell count 18 000/mm3
Hemoglobin 9 g/dL
Hematocrit 28%
Platelets 105 000/mm3
cardiac etiology. Differential diagnoses for the migrating
joint pain include septic arthritis, Lyme disease, autoim-
mune connective tissue disease, and leukemia/lymphoma.
Bacterial and viral etiologies should also be considered with
increased work of breathing and mild elevation in
temperature.
What additional laboratory studies would be
useful in making a diagnosis?
Anti-streptolysin titer can confirm the previous presence of
a group A β-hemolytic Streptococcus infection. In addition,
acute-phase reactants should be ordered and trended if ele-
vated. Generally, erythrocyte sedimentation rate (ESR) and
C-reactive protein are elevated in cases of rheumatic fever.
Blood cultures should be evaluated for primary bacterial
infection and if positive can guide antimicrobial therapy.
Viral studies can be used to evaluate for viral infection
and liver function tests can be useful to evaluate for end-
organ damage. A urinalysis is helpful to evaluate for strep-
tococcal glomerulonephritis.
What additional studies would you order?
An electrocardiograph (EKG) should be completed to look
for signs of cardiac strain, ST-segment changes, and other
arrhythmia or other cardiac irritability. A prolonged PR
interval is one of the minor Jones criteria for diagnosis of
rheumatic heart disease. Echocardiogram will confirm
the presence of intracardiac changes consistent with rheu-
matic fever, including mitral valve and/or aortic valve
changes, vegetation, and ventricular dysfunction. Most
changes involve the mitral valve.
Continuation of case: Patient is transferred to the pediat-
ric intensive care unit (PICU) for further monitoring. After
transfer, he continues to have increased work of breathing
with desaturations and was placed on bilevel positive air-
way pressure (BiPAP) with improvement in oxygen satura-
tions and work of breathing. An echocardiogram shows
moderate to severe mitral regurgitation with abnormal
coaptation of the mitral valve leaflets, and mild to moderate
aortic valve regurgitation. Left ventricular function is mod-
erately diminished while right ventricular function is nor-
mal. His EKG is significant for sinus tachycardia with
prolonged PR interval.
Additional Questions and Evidence-
based Rationale
4 What is the most likely diagnosis?
5 What additional therapies would you initiate?
What is the most likely diagnosis?
Rheumatic heart disease with carditis is the most likely
diagnosis. Rheumatic fever is an inflammatory illness,
likely with an autoimmune cause, that can occur after
group A β-hemolytic streptococcal pharyngitis. Rheumatic
heart disease can be diagnosed using the Jones criteria.
Diagnosis is confirmed with presence of two major criteria
or one major and two minor criteria (Table 81.3). Rheu-
matic carditis does not appear in all rheumatic fever
patients but if present presents with varying severity. This
carditis can cause permanent changes in the endocardium.
Early signs of carditis present as new-onset regurgitant
murmur and tachycardia progressing to signs and symp-
toms of congestive heart failure.
To meet criteria for rheumatic heart disease, the patient
must show evidence of the presence of preceding group
A streptoccocal infection (e.g. positive throat culture for
group A β-hemolytic streptococci or positive/rising ASO
titer). The updated Jones criteria (Gewitz et al., 2015) iden-
tified low-, medium- and high-risk populations. Low-risk
populations are identified as those in which acute rheumatic
fever occurs in two or less cases per 100 000 school-age chil-
dren or an all-age rheumatic heart disease prevalence of
≤1 per 1000 population per year. Medium- to high-risk

Table 81.3 Jones criteria.a
Low risk
Minor criteria Polyarthralgia
Fever ≥38.5 C (101.3 F)
ESR >60 mm/h in the first hour and/or CRP >3 mg/dL
Prolonged PR interval (for patient age)
Major criteria Carditis (clinical or subclinical)
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodules
a
Must meet either two major criteria or one major and two minor criteria.
ESR, erythrocyte sedimentation rate; CRP, C-reactive protein.
Source: Gewitz et al. (2015). Reproduced with permission of Wolters Kluwer Health, Inc.
populations are those that do not meet the criteria for low
risk (Table 81.3).
What additional therapies would you initiate?
Treatment of carditis should be managed with conven-
tional heart failure therapy (e.g. with diuretic and fluid
management) depending on severity of carditis. Bedrest
and restricted activity is generally advised, though there
is lack of recent data to support this practice. Initiation of
anti-inflammatory medications is indicated when evidence
Reference
Gewitz, M., Baltimore, R., Tani, L., et al. (2015). Revision of the
Jones criteria for the diagnosis of acute rheumatic fever in
the era of Doppler echocardiography: a scientific statement
Further Reading
Allen, H.D., Driscoll, D.J., Shaddy, R.E., and Feltes, T.F. (ed.)
(2013). Moss and Adams’ Heart Disease in Infants, Children,
and Adolescents, 8th edn. Philadelphia, PA: Lippincott
Williams & Wilkins.
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Chapter 81 A 7-Year-Old with Migratory Joint Pain 347
High risk
≥38.0 C (100.4 F)
Monoarthralgia
Prolonged PR interval
ESR >30 mm/h in the first hour and/or CRP >3 mg/dL
Carditis (clinical or subclinical)
Monoarthritis, polyarthritis, and/or polyarthralgia
Chorea
Erythema marginatum
Subcutaneous nodules
of carditis is present, in the form of aspirin for mild cases
and prednisone or methylprednisolone for severe cases.
Anti-inflammatory medications will also improve the joint
pain associated with polyarthritis. Antibiotic therapy
should be initiated in the form of penicillin or, if allergy,
erythromycin to eradicate group A Streptococcus. After
initial therapy, these patients will need continued
endocarditis prophylaxis on ongoing evaluation of cardiac
function. Household contacts should be tested for group
A Streptococcus if symptomatic and treated for positive
results.
from the American Heart Association. Circulation 131:
1806–1818.
Reuter-Rice, K. and Bolick, B. (2012) Pediatric Acute Care:
A Guide for Interprofessional Practice. Burlington, MA:
Jones & Bartlett Learning.
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349

82

Adolescent with Murmur and Chest Flutter

Natalie Hecht
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA

An adolescent presents to a pediatric cardiology clinic after Current Status

referral from his primary care provider (PCP) for a murmur
and palpitations. Vital signs are as follows: heart rate 92 bpm, blood pressure
90/52 mmHg, respiratory rate 20 breaths per minute, and
oxygen saturation of 99% on room air.
He is an alert adolescent in no acute distress. Normal S1,
History of Present Illness
normally split S2. III/VI systolic ejection murmur at left
mid-sternal border. The point of maximal impulse was at
The adolescent was noted to have a new murmur on a well-
the left anterior axillary line. Clear breath sounds noted
child examination. He also complained that his heart was
bilaterally. Mild shortness of breath after walking from
“flipping around in my chest” a couple of times a day,
the waiting room to examination room. His abdomen
which has been going on for several months. When this
was soft and his liver border was palpable 2 cm below
happens, it lasts a few seconds, and is accompanied by dif-
the right costal margin.
ficulty breathing. The adolescent complained of shortness
of breath when climbing stairs and exercising, as well as
fatigue requiring daily naps after school. He denied
chest pain. Questions

Answer the following questions using the details provided.

Past Medical History 1 What are the differential diagnoses for this child?
2 Which diagnostic tests are indicated?
Frequent otitis media as an infant. Obesity. His immuniza-
tions are up to date.

Rationale and Evidence-based Practice

Explanation
Past Surgical History
What are the differential diagnoses for this child?
Tympanostomy tubes placed at 18 months of age.
The differential diagnoses include hypertrophic cardiomy-
opathy (HCM), dilated cardiomyopathy, restrictive cardio-
myopathy, arrhythmias, structural heart disease,
Family History mediastinal mass, and aortic stenosis.

Father has atrial fibrillation and hyperlipidemia. His

Which diagnostic tests are indicated?
mother has irritable bowel syndrome. His younger sister
has mild intermittent asthma. His mother’s cousin died Indicated diagnostic testing for this adolescent includes an
suddenly at age 30 of unknown cause. electrocardiogram (EKG) to evaluate for arrhythmias, an
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

350 Cases in Pediatric Acute Care
echocardiogram to evaluate heart structure and function,
and a chest radiograph to evaluate lung fields and
heart size.
Continuation of case: The results of the diagnostic studies
are as follows. EKG showed ST–T changes, deep Q waves,
missing R waves, and left ventricular (LV) hypertrophy.
Echocardiogram demonstrated moderate LV hypertrophy,
moderate to severe mid-ventricular and outflow LV
obstruction, moderate mitral regurgitation, and asymmet-
ric septal hypertrophy of interventricular septum, with sep-
tal wall thicker than posterior LV wall. The chest
radiograph showed lung fields clear bilaterally with prom-
inent cardiomegaly.
Additional Questions and Evidence-
based Rationale
3 What is the most likely diagnosis?
4 Which therapies should be used to medically manage
this patient?
5 What anticipatory guidance should you provide to the
patient?
What is the most likely diagnosis?
HCM is the most likely diagnosis, given the classic echocar-
diogram results, characteristic EKG and murmur, and his-
tory of shortness of breath with activity. Given the level of
obstruction to LV outflow, HCM can be further classified as
obstructive. HCM is characterized by a hypertrophied left
ventricle and decreased ventricular cavity with impaired
ventricular filling. A thickened septal wall beyond two to
three times the standard deviation for age is clinically sig-
nificant for HCM. HCM is associated with symptomatic
heart failure and is the most common cause of cardiac
death in teenagers due to fatal arrhythmias.
Which therapies should be used to medically
manage this patient?
This adolescent was started on labetalol 20 mg three times
daily. Pharmacologic therapy with beta-blockers or calcium
channel blockers helps to relax the hypertrophied myocar-
dial tissue and reduces the heart rate to improve filling and
ejection time, thereby improving cardiac function. A Holter
monitor was ordered to further evaluate for arrhythmia as
an outpatient. He was instructed to follow up with pediatric
cardiology in 1 month. Hospital admission and automatic
implantable cardioverter defibrillator (AICD) were not
indicated as his heart failure symptoms were mild and he
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did not have a significant arrhythmia on EKG. These thera-
pies may be indicated in the future if his status deteriorates.
HCM has a strong genetic component. The patient under-
went cardiomyopathy genetic testing, which is indicated in
all patients with HCM. If this screening is positive, all at-
risk and first-degree relatives should have this testing per-
formed as well. If the patient’s testing is negative, all at-risk
and first-degree relatives should undergo cardiology eva-
luations with an EKG and echocardiogram to evaluate
for HCM. Guidelines are needed to standardize use of AICD
in pediatric patients. Indications for AICD placement are
increased risk of sudden death, including ventricular
arrhythmias, syncope, sudden death in a first-degree rela-
tive, and/or massive hypertrophy. Based on his presenta-
tion, this patient does not qualify, though his outpatient
Holter results may prompt further intervention.
Treatment for HCM is dependent on severity of symp-
toms and presence of LV outflow obstruction. Patients with
non-obstructive HCM may not require pharmacologic
treatment. Given his diagnosis of obstructive HCM and
symptomatic presentation, this adolescent will likely bene-
fit from a beta-adrenergic blocking agent (e.g. labetalol).
Beta-blockers are recommended for patients who have
LV outflow tract obstruction or who are symptomatic with
angina or dyspnea. Beta-blockers decrease heart rate and
prolong diastole, thereby promoting more LV filling and
reducing LV outflow obstruction. Beta-blockers are also
beneficial due to their antiarrhythmic properties.
For patients who do not tolerate or respond to beta-
blockers, calcium channel blockers (CCBs) may be consid-
ered. Similar to beta-blockers, CCBs are negative inotropes
and reduce heart rate. CCBs are thought to improve dias-
tolic performance by decreasing myocardial ischemia.
However, CCBs should be used with caution, as a decrease
in blood pressure may worsen outflow tract obstruction,
leading to pulmonary edema. Of note, verapamil has been
linked to increased risk of death in pediatric patients with
advanced LV hypertrophy.
Diuretics should be carefully considered. Patients with
HCM need adequate preload to allow for sufficient ventric-
ular filling. Thus, diuretics are generally contraindicated, as
they may worsen symptoms. However, patients with con-
gestive symptoms may benefit from careful diuretic usage.
Diuretics were not indicated in this patient since he did not
demonstrate congestive symptoms.
What anticipatory guidance should you provide to
the patient?
Strenuous exercise, such as basketball, sprinting, and
soccer, increases the risk of sudden cardiac death and is
not recommended for patients with HCM. However,

lower-intensity exercises, such as golf, walking, biking, and
bowling, are likely safe for patients with HCM. Exercise
recommendations should be individualized for each
patient.
Patients with obstructive HCM may require a myectomy.
This is a surgical reduction of the ventricular septum,
which decreases the LV outflow tract obstruction. Surgical
Further Reading
Gersh, B., Maron, B., Bonow, R., et al. (2011). 2011 ACCF/AHA
guideline for the diagnosis and treatment of hypertrophic
cardiomyopathy. Circulation 124: e783–e831. doi: 10.1161/
CIR.0b013e318223e2bd.
Lee, T., Hsu, D., Kantor, P., et al. (2017). Pediatric
cardiomyopathies. Circulation Research 121: 855–873. doi:
10.1161/CIRCRESAHA.116.309386.
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Chapter 82 Adolescent with Murmur and Chest Flutter 351
myectomy is typically recommended for patients who are
refractory to oral medication therapy, have worsening
mitral regurgitation, or a LV outflow gradient greater than
60 or 70 mmHg. A myectomy was not indicated for this
patient based on his presentation, but he should have care-
ful outpatient follow-up with echocardiograms to identify
any changes.
Park, M.K. (2014). Park’s Pediatric Cardiology for Practitioners.
Philadelphia, PA: Elsevier.
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353

83

Outpatient Management of Asthma

Jolene Dickerson
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

A 7-year-old boy with a history of mild persistent asthma occasions for asthma exacerbations. His past medical his-
diagnosed at age 6 presents to his primary care office with tory is significant for seasonal allergies, eczema, failure to
wheezing and acute respiratory distress. thrive, and autism.

History of Present Illness Past Surgical History

This 7-year-old boy became ill 2 days ago with cough and This child has not had any surgical procedures.
wheezing. He has been treated with albuterol via nebulizer
and/or metered-dose inhaler every 4 hours since the onset
of symptoms yesterday with no improvement. His last albu- Family History
terol treatment was given 3 hours ago. He had problems
with cough and wheezing in school, so was taken to the pri- The patient’s mother and maternal grandfather both have
mary care provider to be seen urgently. There is no history asthma. His mother also has a history of seasonal allergies.
of fever, no recent changes in the weather, nor does the No other family history of asthma, allergies, eczema, or
patient have cold symptoms. He has not had any recent lung disease.
environmental exposures such as cigarette smoke. There
are no known food or drug allergies, but he does have sea-
sonal environmental allergies and today he was playing
outside when his symptoms began. His appetite is also
Current Status
decreased.
On arrival at the primary care office, the patient is wheez-
ing throughout all lung fields and has decreased breath
sounds with poor air exchange. He is hypoxic, with an
Past Medical History oxygen saturation of 88–90% on room air. His initial respi-
ratory rate is 34 breaths per minute with severe intercostal,
This boy was born at term and went home with his mother. suprasternal, and supraclavicular retractions. He is sitting
He is up to date on his routine immunizations, but did not up on the examination table with forward posturing.
have his annual influenza vaccine this year. His weight is
less than the first percentile for his age and gender and
his current body mass index (BMI) is 11.5, which is also less
than the first percentile for age and gender. At age 6 years,
Questions
the patient was diagnosed with asthma after experiencing
Answer the following questions using the details provided.
recurrent episodes of wheezing, treated in outpatient care.
He has no history of hospitalizations for asthma, although 1 Based on the data given, what are the differential diag-
he has been to the emergency department (ED) on multiple noses and the most likely diagnosis for this child?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

354 Cases in Pediatric Acute Care
2 What is the best way to increase this patient’s
oxygenation?
3 What medications are recommended to be given in the
outpatient areas?
4 What are the next steps in managing this case? How
would you provide a formal sign-out to the ED using
the SBAR format?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include severe asthma
exacerbation associated with viral illness, exposure to aller-
gens, exercise, or other inciting factors. Other potential
diagnoses would include foreign body aspiration and pneu-
monia. It would be unlikely that the boy had a viral illness
or pneumonia, as he has not had any other symptoms
except for wheezing and cough and these symptoms were
only present for 2 days.
A severe asthma exacerbation is the most likely cause of
the child’s symptoms, related to the history and findings of
difficulty breathing, wheezing, cough, and hypoxia. This
child has also reported inciting factors of exercise and envi-
ronmental allergy exposure that may have triggered the
exacerbation (Link, 2014).
Moderate to severe asthma exacerbations are typically trig-
gered acute episodes of respiratory distress, cough, and
wheezing in a patient with a history of asthma. This patient
requires prompt treatment with beta-agonists, systemic cor-
ticosteroids, and oxygen. Close monitoring for clinical dete-
rioration and ongoing clinical assessments are important. In
the primary care office, initial therapies can be provided,
especially if there will be a wait for emergency medical serv-
ice (EMS) to arrive. However, without monitoring equip-
ment, it is important to observe and assess the patient
frequently until appropriate monitoring is available.
What is the best way to increase this patient’s
oxygenation?
Oxygen therapy is recommended for children with asthma
who cannot maintain oxygenation above 92% on room air.
For patients who are spontaneously breathing, oxygenation
often begins with delivery via a facemask or nasal cannula.
If the patient fails to respond to interventions and requires
additional oxygen support, non-invasive positive pressure
ventilation may be beneficial. This may provide relief for
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fatigued muscles while also preventing airway collapse
and supporting oxygenation.
What medications are recommended to be given in
the outpatient areas?
The pathogenesis of asthma involves underlying inflamma-
tion, which is variable and based on the individual patient
and the severity of disease. In an acute exacerbation,
bronchoconstriction occurs with exposure to a trigger or ill-
ness resulting in airway obstruction. Inhaled short-acting
beta-agonists (SABAs) such as albuterol and levalbuterol
combined with ipratropium bromide are first-line therapy
for an acute outpatient asthma exacerbation. SABAs have
a rapid onset of action (within 15 minutes), and because
they are bronchodilators, airflow is improved by relieving
smooth muscle constriction and relaxing the airway. Ipra-
tropium bromide (Atrovent) also provides bronchodilation
through smooth muscle relaxation in combination with
albuterol (Link, 2014). These therapies should be adminis-
tered up to three times back-to-back or at 20–30 minute
intervals. Continuous albuterol therapy may be needed if
there is no significant improvement in the patient’s respira-
tory status. Because of the risk for side effects from SABAs
(hypertension, tachycardia, or tremors), patients must be
continuously monitored in an acute care setting.
Systemic corticosteroids decrease airway inflammation
in patients who experience acute asthma exacerbation.
Their onset of action is within 2–6 hours after administra-
tion. It is best to administer systemic corticosteroids as soon
as possible after arrival in the office or ED. Early adminis-
tration has been shown to reduce hospital admission rates
for asthma exacerbations. Prednisone or prednisolone are
typically first-line steroid treatments, but there is limited
documentation of the benefits of using dexamethasone
due to its longer-lasting effect.
What are the next steps in managing this case?
How would you provide a formal sign-out to the
ED using the SBAR format?
This patient continues to be in respiratory distress and he
will therefore require immediate transport to the nearest
ED for continued support and additional intervention. The
local EMS should be notified to respond to the pediatric
office, as opposed to asking family to drive him to the ED.
It is then important for the provider to notify the ED and pro-
vide a sign-out regarding the patient. SBAR (situation, back-
ground, assessment, recommendation) is a standardized
method of providing comprehensive information using a
mnemonic to assist in remembering important information
to provide between healthcare providers in patient handoffs.
Failed communication has been identified as a primary
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Chapter 83 Outpatient Management of Asthma 355

Table 83.1 Example of SBAR handoff.

Explanation of section Handoff information to relay

S Current status and situation
of the patient
B Background information to
include the history of the
present illness, allergies,
current medications
A Assessment of the patient,
with potential diagnoses
R Recommendation for care
This is the primary care office on ABC street. We are sending a child who is experiencing status
asthmaticus by EMS to the ED. He needs continued oxygen and potentially hospital admission.
This is a 7-year-old male who presented to our office today in status asthmaticus with oxygen
saturation of 88% on room air, requiring oxygen and continuing to wheeze despite receiving
albuterol nebs and dexamethasone. Vital signs are temperature 99, HR 136, RR 38, BP 92/46, oxygen
saturation is 94% on 15 L/min oxygen. Weight 19 kg, height 114 cm
This boy was diagnosed with mild, intermittent asthma a year ago and has had two ED visits since
then, but is currently only taking albuterol nebs PRN for wheezing. He began with a cough
and wheezing 2 days ago without any associated illness or fever. Wheezing worsened in school
today and his mother brought him to us this afternoon. His other history includes a diagnosis
of failure to thrive and seasonal allergies. He has no known food or drug allergies and albuterol is
his only current medication
I think this child is in status asthmaticus and needs more aggressive treatment
I do not think this child needs any diagnostic testing, unless he needs to be NPO for a long period
of time, develops fever or becomes more tachypneic, indicating the possibility of pneumonia

reason for sentinel and adverse events as patients are cared
for across healthcare settings. The Joint Commission and the
Agency for Healthcare Research and Quality, among other
organizations, recommend using a standardized approach
when communicating patient information. Using the SBAR
approach, the handoff would result in a phone call to the ED
in order to speak with a pediatric provider. See Table 83.1 for
an example of this call in SBAR format.
Continuation of case: The boy is treated with oxygen via
facemask, and administered oral dexamethasone 0.6 mg/kg
and a nebulized treatment of ipratropium bromide 250 μg
and albuterol 2.5 mg per 3 mL. Oxygen is administered at
10 L/min initially, but requires an increase to 15 L/min
to maintain his oxygen saturations above 96%. Because of
the level of hypoxia, respiratory distress, and oxygen
requirement of this patient, staff calls 911 for EMS transport
to the nearest ED. Two additional doses of albuterol are
administered at continuous intervals while awaiting the
arrival of EMS.
Additional Questions and Evidence-
based Rationale
5 What other therapies would be initiated in the ED?
6 Should diagnostic studies be obtained for this patient in
the ED?
What other therapies would be initiated in the ED?
Continuous albuterol therapy should be considered if
the patient has limited response to the initial three
back-to-back treatments. Intravenous magnesium sulfate
(25–75 mg/kg, maximum 2.5 g administered over 20 min-
utes) has been shown to be beneficial in children with
severe asthma exacerbation and is considered for those
who have failed traditional first-line therapies of albu-
terol and glucocorticoids. It is part of asthma pathways
in the ED. It is thought that magnesium relaxes smooth
muscle by enhancing calcium uptake in the sarcoplasmic
reticulum and/or as a calcium antagonist, but the exact
mechanism of action is not known. Another benefit of
magnesium sulfate in asthma is that it reduces release
of histamine from mast cells and may act as an anticho-
linergic agent. Advanced oxygenation therapy such as
non-invasive positive pressure ventilation may be used
if the patient has poor response to other interventions
and remains hypoxic. Subcutaneous or intramuscular
epinephrine or terbutaline (systemic beta-agonists) may
be better than inhaled therapies in children who have
poor inspiratory flow, are anxious or uncooperative, or
who have poor response to initial inhaled therapies. It
is also important to assess hydration and, if needed,
administer intravenous fluid boluses and maintenance
fluid. Children with asthma can become dehydrated
quickly due to poor oral intake, insensible fluid losses,
and increased metabolic demand.
Should diagnostic studies be obtained for this
patient in the ED?
Arterial blood gases are not as useful in the child who is
breathing spontaneously without severe distress and are
painful to obtain, which could worsen respiratory distress.

356 Cases in Pediatric Acute Care
With rapid deterioration, a blood gas can assist in making a
decision for intubation and ventilation. Chest radiographs
only need to be considered with focal findings of crackles or
decreased breath sounds, along with fever and/or severe
disease. Chest radiographs can help to rule out pneumonia,
atelectasis, air leak, and foreign body in certain severe cases
that do not respond to conventional therapies. Overall,
there is evidence supporting the view that radiographs
and laboratory studies are not indicated if the diagnosis
is isolated as an asthma exacerbation without other con-
founding factors.
Reference
Link, H. (2014). Pediatric asthma in a nutshell. Pediatrics in
Review 35(7): 287–298. doi: 10.1542/pir.35-7-287.
Further Reading
Anderson, W.C., Gleason, M., Miyazawa, N., and Szefler, S.J.
(2017). Approaching current and new drug therapies for
pediatric asthma. Pediatric Clinics of North America 64(6):
1197–1207. doi: 10.1016/j.pcl.2017.08.001.
Campbell, D. and Dontie, K. (2019) Implementing bedside
handoff in the emergency department: a practice
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As the primary care provider for this child, it is important
to stay in contact with the ED and inpatient unit if the child
is admitted. With this type of exacerbation and/or hospital-
ization, prevention of future acute illness is the goal. Since
this boy was not previously taking daily medications, the
National Institutes of Health Asthma Guidelines recom-
mend starting a daily medication such as an inhaled steroid.
The patient should also return to his primary care provider
within 1–3 days following discharge. Monitoring in the office
on a regular basis is also recommended to monitor symp-
toms and need for escalation in maintenance therapies.
improvement project. Journal of Emergency Nursing 45(2):
149–154.
Schwarz, E.S. and Cohn, B.G. (2015). Is dexamethasone as
effective as prednisone or prednisolone in the management
of pediatric asthma. Annals of Emergency Medicine 65(1):
81–82. doi: 10.1016/j.annemergmed.2014.05.023.
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357

84

A Newborn with Abnormal Movements

Maura Bernardo
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

A 12-day-old female presents to the neurology outpatient
clinic for evaluation of tremors, with a concern that the
child is having seizures. The infant had been evaluated in
the pediatric office and again in the emergency department
(ED) as parents were very worried about this infant’s
movement.
had hearing screen and cardiac testing which were both
normal. She has been seen twice by the primary care pro-
vider and has an umbilical hernia which will be followed
with the possibility of surgery in the future. She is taking
ranitidine for hiccups after feeding.

History of Present Illness
Since birth, this infant has been having jerking/tremulous
episodes of both lower and upper extremities that occur
during sleep only. Initially, she was doing these jerks at
least once a day but for the past 3 days parents report only
having seen one episode. They deny any breathing disorder,
color changing, or vomiting after feedings. No facial droop
or weakness after episodes has been noted. Parents are able
to move her out of the jerking episodes and deny lethargy or
sleeping after episodes. Her eyes remain closed during epi-
sodes as she is asleep for each one. Parents state she does
hiccup a lot. This is their fourth child; no other child has
had these types of episodes and there have been no recent
illnesses, such as cold symptoms or fever.
Past Medical History
Family History
Parents and siblings of this infant are healthy. There is no
family history of seizures, cerebral palsy, multiple sclerosis,
autism, mental retardation, developmental delay, stroke, or
deep vein thrombosis in the family. There are no reported
genetic disorders in the family. Maternal grandmother has
rheumatoid arthritis and there are headaches and prostate
cancer reported on the maternal side. Paternal family his-
tory is positive for hypertension in the great grandfather.
Current Status
The infant is alert, awake, and has no birthmarks or other
abnormal pigmentation. Overall physical examination is
within normal limits. The neurologic examination is as
follows.

The infant was born at 39 weeks’ gestation to a gravida 4,

• Cranial nerve (CN) II: pupils equal, round and reactive to
light and accommodation.
para 4, 35-year-old female with prenatal care beginning
early in pregnancy. She was born by planned cesarean
• CN III, IV, and VI: all extraocular movements intact, no
nystagmus noted.
section after an uneventful pregnancy. No complications
after birth. The infant was discharged home with mother
•• CN V: facial sensation normal and symmetrical.
CN VII: facial contours and strength symmetrical.
at 3 days of age. Blood glucose at birth was normal and
the first metabolic screen was normal, second is pending.
•• CN X: good cry.
CN XII: tongue protrusion midline, no fascicula-
Infant received hepatitis B vaccine in the nursery and tions noted.

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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358 Cases in Pediatric Acute Care

Figure 84.1 Electroencephalogram (EEG) is normal.

• Motor examination: normal strength, muscle mass, and

tone in all extremities.
Rationale and Evidence-based Practice

•
Explanation
Reflexes: brisk, 2+ bilaterally, (+) plantar flexor
bilaterally. Based on the information given, what are the
differential diagnoses and which one is the most
Other results as follows.
likely for this infant?

• Skin: no birthmarks or other abnormal pigmentation,

no edema.
Differential diagnoses for this infant include seizure

•
disorder, metabolic disorder (inborn error of metabolism),
Head: atraumatic/normocephalic, normal hair newborn tremor, benign neonatal sleep myoclonus,
distribution.

•
opsoclonus–myoclonus disorder, benign familial neonatal
Extremities: no deformity or asymmetry, normal strength seizures, movement disorder, infantile spasms, and neona-
and tone bilateral, full range of motion

•
Back: no scoliosis, full range of motion, no tufts
The infant was evaluated with a short electroencephalo-
gram (EEG) with results as shown in Figure 84.1.
Questions
Answer the following questions using the details provided.
1 Based on the information given, what are the differen-
tial diagnoses and which one is the most likely for this
infant?
2 What other tests should be considered/ordered?
3 What treatment/management is warranted for this
infant?
tal abstinence syndrome. Differentiating between these dis-
orders can be difficult at the age of 2 weeks, so careful
consideration and understanding of each diagnosis is nec-
essary. The metabolic disorder is least likely as the first
screen was determined to be normal, but a second screen
is still pending. Seizure disorder or benign familial neonatal
seizures would also be less likely related to the presentation
and normal EEG.
The most likely diagnosis would be benign neonatal sleep
myoclonus due to the history, symptoms, and normal EEG
result. Sleep myoclonus is an underdiagnosed disorder of
sleep that occurs from birth to age 3 and consists of rhyth-
mic jerking when drowsy or asleep. It occurs particularly
frequently during quiet sleep but may occur in any sleep
state. The jerking movements can be focal, multifocal,
marching, or generalized, involving both upper and lower

limbs. Movements are usually symmetric and occur bilater-
ally and can range in severity from mild to severe, which
can make diagnosis difficult. These jerks stop when the
child is woken up and the patient maintains a normal
EEG and neurologic examination. These movements are
also not usually associated with any other more ominous
seizure feature such as crying, change of color, change in
breathing pattern, facial movements, or eye movements.
Some familial cases have been reported.
What other tests should be considered/ordered?
Other testing that should be done includes serum electro-
lytes, including calcium, magnesium and phosphorus to
determine normalcy. A metabolic work-up should also be
Further Reading
Afawi, Z., Bassan, H., Heron, S., et al. (2012). Benign neonatal
sleep myoclonus: an autosomal dominant form not allelic to
KCNQ2 or KCNQ3. Journal of Child Neurology 27(10):
1260–1263.
Fernandez-Alvarez, E. (2018). Transient benign paroxysmal
movement disorders in infancy. European Journal of
Paediatric Neurology 22(2): 230–237.
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Chapter 84 A Newborn with Abnormal Movements 359
ordered, with ammonia, serum amino acids and urine
organic acids and vitamin B12 levels. If the EEG was abnor-
mal, then sedated magnetic resonance imaging (MRI)
would be indicated.
What treatment/management is warranted for
this infant?
Sleep myoclonus does not require medication management
but should be followed to ensure it is not increasing in fre-
quency or occurring during wakefulness. Developmental
milestones should also be assessed as a plateau or regres-
sion would suggest a more malignant disorder or process.
No medication is necessary.
Goraya, J.S. (2015). Acute movement disorders in children:
experience from a developing country. Journal of Child
Neurology 30(4): 406–411.

85
Postoperative Infant with Tachycardia
Cathy Woodward
Long School of Medicine, University of Texas Health, San Antonio, TX, USA
An 8-month-old infant is admitted to the intensive care unit
after complete repair of tetralogy of Fallot (TOF).
History of Present Illness
The infant was diagnosed prenatally with TOF and has
been monitored closely since birth. Increasing cyanotic
spells, failure to thrive, and worsening right heart failure
led to a decision to proceed with surgery at this time.
Past Medical History
Born at term and has received all vaccines as well as influ-
enza and palivizumab. Birthweight was 3.2 kg and current
weight is 6 kg. She has been breast-fed primarily with occa-
sional small amounts of rice cereal and fruits. Home med-
ications include vitamin D supplements every day and oral
propranolol 5 mg three times daily.
Family History
No family history of congenital heart disease, arrhythmias,
or sudden cardiac death. She has a 3-year-old sibling who is
healthy.
Current Status
The infant is admitted to the intensive care unit immedi-
ately after complete repair of TOF (i.e. closure of ventricu-
lar septal defect, narrowing of the pulmonary valve and
right ventricular outflow tract is augmented by a combina-
tion of resection of obstructive muscle tissue in the right
ventricle and enlargement with a patch). The operative
course was uneventful. She is intubated and is on milrinone
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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361
and epinephrine infusions. Temporary transcutaneous
atrial and ventricular pacing wires are wrapped and secure
on chest. Initial postoperative laboratory results are shown
in Tables 85.1 and 85.2.
Vital signs are as follows: temperature 37.3 C (99.2 F)
rectal, heart rate 144 bpm in sinus rhythm, blood pressure
90/45 mmHg, and oxygen saturation 94% on FiO2 0.6.
Approximately 1 hour after admission, the registered nurse
calls you to the bedside because the heart rate is now 188 bpm.
Other vital signs include a temperature of 38 C (100.4 F),
blood pressure 68/35 mmHg, and oxygen saturation 92% on
FiO2 0.6 via ventilator circuit. An electrocardiogram (EKG)
is recorded and the results are shown in Figure 85.1.
Questions
Answer the following questions using the details provided.
1 What is your interpretation of the rhythm strip shown
in Figure 85.1?
2 What would be your initial interventions and what
additional studies would you order?
3 What additional interventions and medications can be
considered if the rhythm does not convert to sinus
rhythm spontaneously?
Rationale and Evidence-based Practice
Explanation
What is your interpretation of the rhythm strip
shown in Figure 85.1
This infant is tachycardic with a regular rate. Differential
diagnoses prior to detailed review of the rhythm include
sinus tachycardia, atrial tachycardia, and junctional ectopic
tachycardia.
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362 Cases in Pediatric Acute Care

What would be your initial interventions and what intravenous piggy back (IVPB) over 60 minutes, which
additional studies would you order? can be repeated up to maximum total dose of 15 mg/kg.
Reducing the inotropic epinephrine infusion support may
A 10 mL/kg bolus of normal saline is administered with no
be considered as it can contribute to tachycardia and
change in rhythm but the blood pressure increases to
arrhythmias. Consider using pacing wires for overdrive
74/40 mmHg. The magnesium level was low immediately
pacing.
postoperatively (0.8 mEq/L) and a dose of magnesium is
JET is a serious and potentially life-threatening rhythm
administered. A 12-lead EKG is ordered with findings
in the postoperative period after surgery to repair TOF. It
of narrow-complex tachycardia, inverted P waves, and
can be the result of edema or injury to the tissue surround-
ventricular–atrial dissociation These findings are consist-
ing the electrical conducting atrioventricular (AV) node
ent with junctional ectopic tachycardia (JET).
and occurs in up to 10% of children after cardiac surgery.
Risk factors include surgeries occurring near the AV node
What additional interventions and medications
such as TOF and atrioventricular septal defect repair. Elec-
can be considered if the rhythm does not convert
trolyte imbalance, particularly low magnesium levels, can
to sinus rhythm spontaneously?
precipitate JET in the postoperative period as can low intra-
Decreasing body temperature should be treated with aceta- vascular volume, fever, or high inotropic support. Success-
minophen and cooling blankets to maintain body temper- ful treatment of JET is difficult and several steps may be
ature at 35.5 C (96 F). Administer amiodarone 5 mg/kg needed, including antiarrhythmics, pacing, cooling, and
electrolyte replacement in addition to maintaining normal
Table 85.1 Blood gas analysis. intravascular volume. The use of amiodarone is considered
by some to be a first-line therapy, and when considered it
pH 7.36
can be done concomitantly with other cooling and replace-
PaCO2 48 mmHg
ment strategies. The rhythm, if a result of edema or inflam-
Pao2 130 mmHg
mation, usually converts 24–48 hours after surgery.
Bicarbonate 24 mEq/L
Base excess –1
Lactate 1.2 mmol/L Further Reading
Ismail, M.F., Arafat, A.A., Hamouda, T.E., et al. (2018).
Table 85.2 Pertinent laboratory findings. Junctional ectopic tachycardia following tetralogy of Fallot
repair in children under two years. Journal of Cardiothoracic
Sodium 140 mEq/L
Surgery 13: 60. doi: 10.1186/s13019-018-0749-y.
Potassium 3.6 mEq/L Kovacikova, L., Hakacova N., Dobos, D., Skrak, P., and
Magnesium 0.8 mEq/L Zahorec, M. (2009). Amiodarone as a first-line therapy for
Hematocrit 33% postoperative junctional ectopic tachycardia. Annals of
Thoracic Surgery 88(2): 616–622.

Figure 85.1 EKG rhythm strip.


86
Brain Death
Maria C. Woosley
Long School of Medicine, University of Texas Health, San Antonio, TX, USA
A 3-month-old infant is admitted with respiratory failure,
traumatic brain injury, and concern for child abuse.
History of Present Illness
The patient is a 3-month-old male who was taken to an out-
side facility emergency department (ED) by his parents after
he was found unresponsive in the crib. The child was trans-
ported by private vehicle approximately 15 minutes to the
hospital, during which time he remained unresponsive.
On arrival to the ED, cardiopulmonary resuscitation (CPR)
was initiated, the patient was intubated, and CPR was con-
tinued for approximately 6 minutes until return of spontane-
ous circulation was attained. He had a head computed
tomography (CT) completed which indicated a possible right
subdural hemorrhage, slit ventricles, and near complete
effacement of the basal cisterns. He was transferred by
helicopter to the pediatric level I trauma center for
continued care.
Past Medical and Surgical History
The patient’s medical history was unobtainable due to lack
of family presence on arrival to the trauma center.
Current Status
On initial assessment at the trauma center, the patient has a
Glasgow Coma Scale (GCS) score of 3T (intubated), fixed
and dilated pupils, and no cough or gag reflexes. He has
a repeat head CT and a chest, abdomen and pelvic CT. Ini-
tial laboratory studies are obtained, and the patient is then
transferred to the pediatric intensive care unit (PICU). Vital
signs in PICU are as follows: temperature 33.9 C (93.0 F),
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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363
heart rate 114 bpm, respiratory rate 30 breaths per minute,
which was the set ventilator rate, blood pressure BP
73/41 mmHg, and oxygen saturation (SpO2) 100%, with
ventilator oxygen set at 100%. Laboratory studies included
a complete blood count, coagulation panel, and a compre-
hensive metabolic profile. The results are basically within
normal limits, except for an elevated glucose (182 mg/dL)
and potassium (5.8 mEq/L), and carbon dioxide level of
12 mg/dL. The results of arterial blood gas analysis are
shown in Table 86.1.
The results of the head CT show diffuse hypoattenuation
throughout the brain parenchyma with sulcal effacement
and slit-like lateral ventricles and near-complete efface-
ment of the basal cisterns. These findings are consistent
with brain edema likely from hypoxic brain injury. No
intracranial bleed was appreciated.
The chest and abdominal CT results show:
••
grade I right mid pole renal laceration;
displaced posterior left 10th rib fracture;
•
non-displaced fracture involving the medial third of the
left clavicle;
• thin periostial reaction about the bilateral humeri seen
best on the coronal reformats, which suggests healing
of prior trauma that may be non-accidental;
•
transverse fracture through the right hallux proximal
phalanx;
• bilateral lung consolidations, likely due to aspiration and
atelectasis.
Questions
Answer the following questions using the details provided.
1 Based on the data presented, what are the differential
diagnoses and what is the most likely diagnosis for this
patient?

364 Cases in Pediatric Acute Care
Table 86.1 Arterial blood gas analysis.
pH 7.28
PCO2 28 mmHg
PaO2 307 mmHg
Bicarbonate 14 mEq/L
Base deficit –8
2 What are the important considerations when determin-
ing whether a brain death examination is warranted?
3 What are the relevant clinical findings and laboratory
and imaging results, presented or missing in reported
data, that help determine the possible outcomes and
plans for this patient?
Continuation of case: The infant continued on mechani-
cal ventilation with no sedation required. A blood gas anal-
ysis indicated hypocapnia, so the ventilator rate was
decreased. Neurologic examinations remained the same,
with no responses in pupillary reaction, cough or gag reflex.
4 What disciplines should be included in the care of this
patient?
Rationale and Evidence-based Practice
Explanation
Based on the data presented, what are the
differential diagnoses and what is the most likely
diagnosis for this patient?
The differential diagnoses for this patient are non-
accidental trauma to the brain, osteogenesis imperfecta,
ingestion of a toxic substance, bleeding disorder, or cardiac
failure leading to respiratory failure. The most likely diag-
nosis for this patient is traumatic brain injury with acute
respiratory failure. The diagnosis of brain death is also
appropriate in this case because of the apparent lack of
brainstem function and reflexes.
This patient’s lack of brainstem reflexes is an important
clinical finding that helps determine the likely outcome,
along with the period of unresponsiveness and consistency
of the poor neurologic examinations. Relevant imaging
results in this case are the findings on the head CT. The
patient’s CT performed at the outside hospital revealed a
subdural hemorrhage and subsequently showed hypoxic
ischemic encephalopathy. Both of these injuries are com-
mon results of traumatic brain injury that can lead to brain
death. The near effacement of the basal cisterns also sig-
nifies pending brain herniation. These findings are poor
prognostic indicators for this patient.
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The pathology involved with this type of brain injury is
easily explained. The intracranial vault is composed of a
fixed volume of brain, cerebrospinal fluid, and blood. The
Monro–Kellie doctrine states that an increase of one of
these fixed volumes must be offset by a decrease in another
(Saceleanu et al., 2018, p. 48). Secondary cerebral lesions
such as subdural hematomas or other intracranial hemor-
rhages can cause cerebral edema. A small increase in cere-
bral edema or intracranial volume can cause a serious
increase in intracranial pressure which can compromise
cerebral perfusion, resulting in brain ischemia, cerebral
edema, brain herniation, and death (Saceleanu et al., 2018).
What are the important considerations when
determining whether a brain death examination is
warranted?
Brain death is a clinical diagnosis and is defined as the irre-
versible loss of all brain functions including the brainstem.
Coma, apnea, and the absence of brainstem reflexes are
essential findings in brain death. A task force consisting
of representatives including the American Academy of
Pediatrics, the Society of Critical Care Medicine, and the
Child Neurology Society established guidelines for deter-
mining brain death in children initially in 1987, and these
were revised in 2011 (Nakagawa et al., 2011) and reaffirmed
in 2015. In summary, to determine brain death in children:
after hypotension, hypothermia and metabolic distur-
bances are treated and corrected and current medications
that can interfere with neurologic examination are discon-
tinued and cleared, two examinations including apnea test-
ing with each examination are completed. Prerequisite
conditions should be met; an etiology of the coma should
be known, and potentially reversible causes of coma should
be excluded.
•• Systolic blood pressure should be normalized for age.
Core body temperature should be >35 C.
• Sedative and narcotic elimination should be considered,
but there is no definite recommendation for the number
of half-lives that must pass, as in the adult criteria.
• The PaCO2 must be at least 60 mmHg and must be
20 mmHg greater than baseline for an apnea test to be
considered positive.
• Ancillary testing may be done if there is uncertainty
about the results of the examintion or if medications
may affect evaluation of the patient (Nelson and
Lewis, 2017).
The brain death examintion is a clinical investigation of
the brainstem reflexes. It includes pupillary response to
light; gag, cough, sucking and rooting reflexes; corneal
reflexes; oculovestibular and oculocephalic reflexes; and
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Chapter 86 Brain Death 365

movement of bulbar musculature including facial and oro- antiepileptics, and neuromuscular blockers can all affect
pharyngeal muscles. The pupillary response to light tests the neurologic examination. Knowledge of administration
cranial nerves II and III. When the pupils are not respon- of any of these and other medications is imperative in order
sive to light, it confirms that these nerves are not intact. to determine whether the medication is affecting the
Cranial nerves III, IV, VI, and VIII are responsible for ocu- patient’s clinical examination. The original mechanism of
lar movements and are tested by performing oculovestibu- injury is also unknown.
lar testing or what is commonly called “cold caloric”
testing. If no eye movement is noted after ice water is What are the relevant clinical findings and
injected into each ear 5 minutes apart, this indicates a lack laboratory and imaging results, presented or
of response. Oculocephalic (doll’s eyes) testing is performed missing in reported data, that help determine the
by briskly rotating the head horizontally and vertically and possible outcomes and plans for this patient?
if no eye movement is noted it confirms lack of response.
The patient’s temperature is a vital component when deter-
Corneal reflex is controlled by cranial nerves V and VII,
mining plans for this patient and must be normalized to
and involves the lack of eyelid movement when the cornea
complete the brain death testing. Hypothermia may alter
is touched with a cotton swab. Cranial nerves IX and X are
the metabolism and clearance of medications that can
responsible for the cough and gag reflexes, and they are
interfere with brain death testing, and it is a known cause
absent when a patient is brain dead (Nelson and
of depression of central nervous system functions
Lewis, 2017).
(Nakagawa et al., 2011). In pediatrics, a core body temper-
The apnea test is also part of the brain death examination.
ature of greater than 35 C (95 F) should be achieved when
The main objective of the apnea test is to prove that there is
performing a brain death examination (Nelson and
absence of respiratory system reflexes in the brainstem,
Lewis, 2017).
indicating that there is no physiologic stimulation to
Electrolyte abnormalities can alter a patient’s physical
breathe. There must be documentation of a rise in PaCO2
ability, and can be the cause of major organ failure, so cor-
of 20 mmHg above the baseline value, and the reading must
rection of the abnormal value is imperative to optimize the
be more than 60 mmHg with absence of respiratory effort
patient’s outcome or contribute to the decision to declare
during the test (Nelson and Lewis, 2017).
brain death. Reversible electrolyte disturbances such as
Two examinations must be completed separated by an
severe electrolyte imbalances, hyperglycemia or hypoglyce-
observation period, and must be performed by different
mia, severe pH disturbances, and hepatic or renal dysfunc-
attending physicians (although the apnea test may be per-
tion can all lead to coma in the pediatric patient.
formed by the same physician). The recommended observa-
Sometimes the ancillary tests are not congruent with the
tion period is 24 hours for neonates (37 weeks’ gestation to
clinical examination. For example, a patient with normal
term infants 30 days of age) and 12 hours for infants and
cranial nerve ability may have an isoelectric EEG in the set-
children (>30 days to 18 years) (Nakagawa et al., 2011).
ting of hypothermia or metabolic encephalopathy; another
There are no data available to document brain death in
example is an EEG that shows electrical activity, but this
infants less than 37 weeks’ gestation. Teens may also fall into
may possibly be due to electrical artifact in an intensive care
either pediatric or adult guidelines, based on where they are
environment (pumps, ventilators) (Lewis et al., 2017). It is
cared for. If in the PICU, the child is determined to be brain
important to recognize that there are limitations and poten-
dead based on pediatric guidelines. Ancillary tests are used
tial incongruences with ancillary tests. In pediatric brain
in addition to clinical brain death examinations; for infants
death, ancillary tests are done if there is uncertainty about
and children, they include the nuclear blood flow or perfu-
brain death examination results or if medication effects
sion scan, and electroencephalogram (EEG) only. Reduction
interfere with evaluation of the patient (Nelson and Lewis,
of the observation periods is acceptable using an accepted
2017). They may also be utilized if components of the exam-
ancillary study, but when an ancillary study is used to
ination or apnea testing cannot be completed safely, or to
decrease the observation interval, two examinations and
reduce the observation period between the two examina-
two apnea tests are recommended. One examination and
tions (Nakagawa et al., 2011).
an apnea test should be completed before the ancillary
study, and the second examination and an apnea test should
What disciplines should be included in the care of
be completed after the ancillary study The conditions above
this patient?
must be persistent when examination is repeated.
Missing or unclear information relevant to this patient’s A multidisciplinary approach is important because of
current status and potential outcome is the use of any nar- the mechanism of injury and the poor prognosis of this
cotic or brain-altering medication. Sedatives, analgesics, patient. The medical team, neurosurgery, trauma

366 Cases in Pediatric Acute Care
surgery, and pediatric critical care providers will all be
involved in the care of this patient. The bedside pediatric
nurses are important contributors to discussions invol-
ving the potential brain death examinations because of
their proximity to the bedside and the patient’s family.
Palliative care and social work are also an important
resource for the patient’s family as well as with the
staff’s emotional needs. Organ donation teams may also
be involved, along with the palliative care team because
they can help with supporting the bereaved family mem-
bers if they decide to donate their child’s organs. The
involvement of the palliative care team, in collaboration
References
Basak, R., Louis, S., Shin, N., et al. (2018). Organ donation in
pediatric patients with severe anoxic brain injury. Indian
Journal of Palliative Care 24: 362–364.
Lewis, A., Adams, N., Chopra, A., et al. (2017). Use of ancillary
test when determining brain death in pediatric patients in
the United States. Journal of Child Neurology 32(12):
975–980.
Nakagawa, T.A., Ashwal, S., Mathur, M., et al. (2011). Clinical
Report: Guidelines for the determination of brain death in
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with the medical providers, is important as literature
indicates that this aspect of care provision greatly
impacts the decision for parents to agree to organ dona-
tion (Basak et al., 2018).
For this particular case, the child abuse specialists should
also be involved due to the unknown cause of injury and
obvious non-accidental head injury. Child life specialists
can assist with grief and bereavement support, as well as
provide handprints, molds, and other special gifts for the
family. Pastoral services are also an important addition to
the team as they can provide spiritual guidance to the fam-
ily and care providers.
infants and children: An update of the 1987 task force
recommendations. Pediatrics 128: e720–e740. doi: 10.1542/
peds.2011-1511.
Nelson, A. and Lewis, A. (2017). Determining brain death:
basic approach and controversial issues. American Journal
of Critical Care 26(6): 496–500. doi: 10.4037/ajcc2017540.
Saceleanu, C., Cindea, C., and Saceleanu, A. (2018). Pediatric
traumatic brain injury: pathophysiology, treatment and case
reports. Acta Medica Transilvanica 23(3): 48–51.
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367

87

Postoperative Spinal Fusion with Complications

Jaime Panton1 and Melissa C. Varrone2,3
1
Columbia University School of Nursing, New York, NY, USA
2
Morgan Stanley Children’s Hospital of New York, New York, NY, USA
3
New York Presbyterian Hospital, Columbia University Irving Medical Center, New York, NY, USA

A 14-year-old female who weighs 50 kg was diagnosed with
adolescent idiopathic scoliosis and is being admitted to the
pediatric intensive care unit (PICU) after undergoing pos-
terior spinal fusion.
History of Present Illness
The adolescent was diagnosed with idiopathic scoliosis at
12 years of age. Bracing was attempted but due to her rap-
idly progressing spinal curve, the orthopedic team elected
to perform a posterior spinal fusion, from T2 to L4. The
patient was in her usual state of good health when she pre-
sented to the operating room today. She had an easy mask
induction with oxygen and sevoflurane. She was given a
dose of rocuronium, fentanyl, and ondansetron and was
intubated with a 7.5 cuffed endotracheal tube. End-tidal
CO2 recording was obtained and maintained throughout
the procedure. Bilateral breath sounds were auscultated.
Once asleep she had two peripheral intravenous catheters
placed in her left and right hand as well as a right radial
20-gauge arterial line placed. Anesthesia was maintained
throughout the case with sevoflurane, air, and intermittent
doses of fentanyl and rocuronium. She was given cefazolin
1 g at 11:40 at the start of the case. Vital signs remained
within accepted parameters and she tolerated the proce-
dure well. At the conclusion of the case, she was given
glycopyrrolate and neostigmine and was reversed and
extubated without difficulty. An autologous blood recovery
system was used. Urine output was 120 mL. She received
2000 mL of crystalloid fluids and was then transferred to
PICU in satisfactory condition.
Per the orthopedic surgery team report, the surgery was
successful. There was no documented loss of motor or
sensory function during the procedure.
Past Medical History
This adolescent female was diagnosed with adolescent idi-
opathic scoliosis at age 12 during a routine sports physical.
She has had a rapidly progressive spinal curve that contin-
ued despite bracing, requiring surgical intervention. She
had recurrent acute otitis media during early childhood
but otherwise has had no significant past medical history.
Past Surgical History
Bilateral myringotomy tubes placed at 18 months of age.
She is currently status post posterior spinal fusion (postop-
erative day 1).
Family History
Mother has hypertension and paternal grandfather has
hypercholesterolemia.
Current Status
Postoperative day 1 vital signs as follows: heart rate 78–95
bpm, arterial blood pressure 100/52 to 118/71 mmHg, res-
piratory rate 14–26 breaths per minute, and pulse oximetry
with oxygen saturations of 95–98% on room air. Her pain is
rated 1–2/10 on a hydromorphone patient-controlled
analgesia pump.
The 24-hour fluid intake and output totals are shown in
Table 87.1. She is currently on maintenance intravenous
fluid of 5% dextrose with 0.9% normal saline infusing at

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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368 Cases in Pediatric Acute Care

Table 87.1 Fluid intake and output totals (mL). Continuation of case: The PICU team is concerned about
the oliguria and orders the following studies:

••
Intake Intravenous fluids 3160 Total 3310
Oral 0 urine specific gravity 1.025
Intravenous medications 150
urine osmolarity 300 mosmol/kg
Output Urine output 480
Drains 100
Operating room blood loss 120
Total 700
•• urine sodium 30 mEq/L
serum osmolarity 267 mosmol/L.
Net +2610 3 Based on this additional information, what is the most
likely diagnosis?
4 What is the appropriate management for this case?
Table 87.2 Complete blood count.

White blood cell count 8000/mm3

Hemoglobin 9 g/dL
Rationale and Evidence-based Practice
Hematocrit 27%
Explanation
Platelet count 188 000/mm3
Based on the information given, what are the most
Neutrophils 69%
likely differential diagnoses?
Lymphocytes 19%
Monocytes 1%
Hyponatremia is one of the most common electrolyte dis-
turbances seen in critically ill children. The most common
Eosinophils 1%
causes of severe hyponatremia are syndrome of inappropri-
ate antidiuretic hormone (SIADH) and cerebral–renal salt
wasting, although iatrogenic causes should be considered
Table 87.3 Basic metabolic profile.
as well, such as the administration of excessive free water
Sodium 129 mEq/L via either intravenous or enteral route. The differential
Potassium 4.4 mEq/L for hyponatremia can be narrowed down by evaluating
the relationship of total body water to sodium levels.
Chloride 98 mEq/L
Hypovolemic hyponatremia is seen in patients who have
Carbon dioxide 23 mEq/L
losses with diuretic use or significant gastrointestinal losses
Blood urea nitrogen 8 mg/dL
of sodium and water, which in turn leads to osmotic anti-
Creatinine 8 mg/dL diuretic hormone (ADH) control resulting in the body
Glucose 122 mg/dL retaining water and increasing the degree of hyponatremia.
Calcium 8.5 mg/dL Other types of decreased total body sodium are secondary to
extrarenal losses, such as with burns and rhabdomyolysis
or through renal losses, as seen in patients with sodium-
90 mL/hour with only sips of water and two episodes of losing renal disease or in patients receiving thiazide-type
non-bloody non-bilious emesis. Urine output is 0.4 mL/kg diuretics. Adrenal insufficiency is another cause and is usu-
per hour with the oliguria occurring over the last 4 hours. ally associated with hyperkalemia. Euvolemic hyponatre-
The physical examination is unremarkable; her surgical site mia is most often seen with excessive water intake, or
(T2–L4) is covered with a surgical dressing that has slight associated with some drugs that potentiate decreased water
shadowing noted, but the dressing is otherwise intact. Two excretion, or with SIADH. The overproduction of ADH acts
drains (one shallow, one deep) are in place, with 100 mL on the collecting ducts of the kidneys resulting in inappro-
of serous drainage output for 24 hours. Complete blood priate retention of water. Excessive ADH may be the result
count and basic metabolic profile are obtained and the of changes in autonomic signals stemming from factors
results are shown in Tables 87.2 and 87.3. common in postoperative patients, such as pain, stress,
hypoxia, and volume depletion. Congestive heart failure,
nephrotic syndrome, or renal failure are common causes
Questions
for hypervolemic hyponatremia. The stimulation of aortic
and carotid vasoreceptors and receptors within the heart
Answer the following questions using the details provided.
may increase sympathetic activity and stimulate the
1 Based on the information given, what are the most renin–angiotensin–aldosterone axis, in turn increasing
likely differential diagnoses? renal vascular resistance resulting in decreased urinary
2 What additional laboratory testing may be helpful? sodium excretion and increased retention of water.

What additional laboratory testing may be
helpful?
Assuming that preoperative laboratory work was obtained
and the initial basic metabolic panel was unremarkable,
urine studies and serum osmolarity should be obtained.
The hyponatremia found in SIADH is accompanied by
low serum osmolarity as well as increased urine specific
gravity, urine osmolarity, and urine sodium.
Based on this additional information, what is the
most likely diagnosis?
Based on the laboratory findings and clinical picture, this
patient is diagnosed with SIADH related to surgical poste-
rior spinal fusion.
What is the appropriate management for
this case?
Sudden and severe hyponatremia can be life-threatening
and requires very careful management to prevent cerebral
Further Reading
Ball, S.G. and Iqbal, Z. (2015). Diagnosis and treatment of
hyponatremia. Best Practice and Research in Clinical
Endocrinology and Metabolism 30: 161–173. doi: 10.1016/j.
beem.2015.12.001.
Fuhrman, B. and Zimmerman, J. (2011). Pediatric Critical
Care, 4th edn. Philadelphia, PA: Elsevier Saunders.
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Chapter 87 Postoperative Spinal Fusion with Complications 369
swelling/edema and, in worst-case scenarios, herniation of
the brainstem. Low serum sodium causes water to shift
from extracellular fluid to intracellular fluid, resulting in
cellular edema.
The goal is to correct and normalize sodium levels.
Sodium levels should be increased by no more than
0.5 mEq/L per hour in patients with chronic hyponatremia,
and no faster than 1 mEq/L per hour in patients with acute
hyponatremia. More rapid corrections are generally better
tolerated if the hyponatremia has developed within the last
4 hours.
The use of isotonic intravenous fluids is always indicated
while avoiding hypotonic solutions. Patients who exhibit
symptoms of increased intracranial pressure or seizures
due to hyponatremia should be given a bolus of 3% hyper-
tonic saline to correct serum sodium to at least 125 mEq/L.
SIADH due to spinal surgery is generally self-limiting
and resolves quickly. However, care should be taken to pre-
vent sequelae from the dilutional hyponatremia and poten-
tial fluid overload.
Jones, D. (2018). Syndrome of inappropriate secretion of
antidiuretic hormone and hyponatremia. Pediatrics in
Review 39: 27–35. doi: 10.1542/pir.2016-0165.

88
Orthopnea in a 10-Year-Old
Max Pizzo
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA
A 10-year-old female presents to her primary care provider
(PCP) with complaints of worsening orthopnea for 1 week,
stating she has required additional pillows to sleep
comfortably.
History of Present Illness
Over the preceding month she has noted an intermittent
non-productive cough and exertional dyspnea. In addition,
she has complained of abdominal pain and nausea, and has
noted a 2.7 kg weight loss. The abdominal pain and nausea
with vomiting have become worse over the past 5 days.
Past Medical History
History of reactive airway disease. Her current medications
include budesonide 0.5 mg daily via nebulization and albu-
terol 0.63 mg every 4–6 hours as needed for cough/wheez-
ing via nebulization. She has noted no improvement in
cough, dyspnea, and orthopnea with inhalation treatments.
Currently up-to-date immunizations for age excluding the
seasonal influenza vaccination.
Past Surgical History
No past surgical history.
Family History
Father, brother, maternal grandmother, maternal aunt,
and maternal uncle are positive for a history of kidney
stones. Brother is also positive for a history of ulcerative
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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371
colitis. No known family history of cancer or
immunodeficiency.
Current Status
Patient denies fever and night sweats. Clinical examination
is notable for cervical lymphadenopathy. Her PCP orders
an initial complete blood count (Table 88.1) with differen-
tial, peripheral smear, and a chest radiograph. The chest
radiograph is notable for a widened mediastinum, so a
follow-up computed tomography (CT) scan of the chest is
urgently scheduled. A referral is placed for follow-up with
pediatric hematology and oncology.
Chest CT results are reviewed by pediatric hematology/
oncology. Imaging is concerning for a large anterior medi-
astinal mass, mild tracheal narrowing, a moderate pericar-
dial effusion, and a moderate right pleural effusion
(Figures 88.1 and 88.2). The family is contacted and direc-
ted to immediately present to the emergency department
(ED) for evaluation and admission.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential
diagnoses?
2 What laboratory work or tests would be helpful in
determining a diagnosis?
Continuation of case: On arrival at the ED, vital signs are
as follows: temperature 36.9 C (98.4 F), heart rate 120
bpm, respiratory rate 22 breaths per minute, blood pres-
sure 124/75 mmHg, and oxygen saturation 98% in room
air. Complete blood count with differential, peripheral
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372 Cases in Pediatric Acute Care

Table 88.1 Initial complete blood count. Table 88.2 Complete metabolic profile.

White blood cell count 10 600/mm3 Sodium 140 mEq/L

Hemoglobin 9.2 g/dL Potassium 3.7 mEq/L
Hematocrit 27.6% Chloride 102 mEq/L
Platelet count 103 000/mm3 Carbon dioxide 19 mEq/L
Atypical lymphocytes 48% Blood urea nitrogen 17 mg/dL
Creatinine 1.19 mg/dL
Glucose 73 mg/dL
Calcium 8.3 mg/dL
Phosphorus 5.5 mg/dL
Lactate dehydrogenase 650 U/L
Uric acid 24.5 mg/dL

Table 88.3 Complete blood count.

White blood cell count 5500/mm3

Hemoglobin 12.1 g/dL
Hematocrit 33.6%
Figure 88.1 Chest CT scan.
Platelet count 65 000/mm3
Neutrophils 5%
Eosinophils 0%
Monocytes 2%
Lymphocytes 6%
Bands 0%
Blasts 86%

She is transferred to the pediatric intensive care unit

Figure 88.2 Chest CT scan.
blood smear and flow cytometry, comprehensive meta-
bolic panel and phosphorus, lactate dehydrogenase
(LDH), and uric acid are obtained and the results are
shown in Tables 88.2 and 88.3. Peripheral smear and flow
cytometry are consistent with T-cell acute lymphoblastic
leukemia (ALL).
An echocardiogram is obtained with results significant
for a large global pericardial effusion with right atrial col-
lapse and intermittent right ventricle collapse. Pediatric
cardiology is consulted. She is taken for placement of a per-
icardial drain, left upper extremity peripherally inserted
central catheter, lumbar puncture, and bone marrow
biopsy. Midazolam, ketamine, and fentanyl are used for
sedation and analgesia during the procedure. Cytarabine
is administered intrathecally during the lumbar puncture.
(PICU) on completion of the procedures.
3 What are the complications associated with this
diagnosis?
4 What is the complication associated with a pericardial
effusion?
5 What is the concern, if any, with the use of sedation in a
patient with this diagnosis?
Continuation of case: Intravenous fluid at twice the cal-
culated maintenance rate is continued on admission to
the PICU. Rasburicase is administered to treat a uric
acid level greater than 8 mg/dL and prevention of tumor
lysis syndrome. Renal function panel, LDH, and uric
acid continue to be assessed every 6 hours. Fluid that
has been removed via the pericardial drain is replaced
1:1 with intravenous fluid for volume repletion. Cefazo-
lin is initiated as prophylaxis while the pericardial drain
remains in place.
6 What are the complications this patient might face
given the current data provided?

Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses?
Of the data provided, the most concerning symptom is wor-
sening orthopnea over the past week. The differential diag-
nosis should include reactive airway disease, infection,
gastroesophageal reflux disease, and anterior mediastinal
mass. The most common anterior mediastinal masses
include Hodgkin lymphoma, non-Hodgkin lymphoma,
thymomas, and teratomas. Kikuchi–Fujimoto disease is a
benign self-limiting histiocytic necrotizing lymphadenitis
that can result in an anterior mediastinal mass. Orthopnea
can also be the result of a pleural or pericardial effusion.
What laboratory work or tests would be helpful in
determining a diagnosis?
A complete blood count with differential and peripheral
smear would be helpful in determining if there is malig-
nancy. A chest radiograph would be beneficial in evalua-
tion for an anterior mediastinal mass or pleural effusion.
Pericardial effusions can be appreciated with an
echocardiogram.
What are the complications associated with this
diagnosis?
Several cardiorespiratory complications are associated with
an anterior mediastinal mass, many of which are specifi-
cally related to the position of the mass and the airways
and vasculature that are obstructed. Hypoxemia can result
from compression of airways. Pulmonary edema and
decreased pulmonary perfusion can result from com-
pressed pulmonary vasculature. Hypoventilation may
result from air trapping and elevated carbon dioxide due
to airway collapse.
Superior vena cava syndrome, a consequence of superior
vena cava compression, is another complication of an anterior
mediastinal mass. Compression of the superior vena cava
leads to venous congestion and edema of superior tissues.
Superior mediastinal syndrome describes superior vena cava
syndrome with accompanying compression of the trachea.
What is the complication associated with a
pericardial effusion?
The complication associated with a pericardial effusion is
cardiac tamponade. Fluid collection in the pericardium
results in increased intrapericardial pressure that can even-
tually exceed transmural myocardial pressure. Compres-
sion of the myocardium results in decreased diastolic
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Chapter 88 Orthopnea in a 10-Year-Old 373
filling and precipitously to decreased left ventricular
output. Hemodynamic instability is the end result.
The definitive treatment for cardiac tamponade requires
decompression of the pericardium.
What is the concern, if any, with the use of
sedation in a patient with this diagnosis?
Sedation should be used with caution in those patients with
an anterior mediastinal mass. These patients are at risk for
cardiopulmonary collapse when sedated; patients with
orthopnea have an increased risk of airway collapse in
the setting of anesthesia. The smooth muscle in airways
and vasculature that maintain patency are relaxed with
sedation, allowing for compression, or further compres-
sion, by the anterior mediastinal mass. Negative pressure
created by spontaneous inspiration is compromised with
sedation, leading to an increase in intrathoracic pressure
that results in greater potential for obstruction from an
anterior mediastinal mass.
In addition, the positioning of a patient with an anterior
mediastinal mass during a procedure can impact ventilation
and perfusion. Supine positioning increases the collapse cre-
ated by the anterior mediastinal mass due to the effect of
gravity, worsening ventilation and perfusion mismatch.
The use of sedation resulting in the need for positive-
pressure ventilation is also not an ideal situation in a patient
with an anterior mediastinal mass. The point of obstruction
may be beyond the reach of an endotracheal tube, limiting
the ability to stent open the airways. Positive-pressure venti-
lation can increase intrathoracic pressure. As mentioned
already, an increase in intrathoracic pressure can result in
greater potential for obstruction from an anterior mediasti-
nal mass. Emergent intervention with steroid or radiation
therapy may be required to treat the tumor and maintain
a patent airway, as the most common tumors causing this
presentation are responsive to steroids as first-line therapy
and radiation as a second-line intervention.
What are the complications this patient might face
given the current data provided?
The diagnosis of T-cell ALL with a mediastinal mass and
hyperuricemia places this patient at high risk for the devel-
opment of tumor lysis syndrome. Tumor lysis syndrome is
the result of cell breakdown and the release of intracellular
potassium, phosphate, and nucleic acid into the blood-
stream. It is defined by two or more metabolic abnormal-
ities (hyperuricemia, hyperkalemia, hyperphosphatemia,
and hypocalcemia) 3 days before or up to 7 days after the
initiation of therapy. Tumor lysis syndrome can result in
acute kidney injury due to hyperphosphatemia and hyper-
uricemia. Hydration at generally two times maintenance is
the initial treatment for tumor lysis syndrome and the

374 Cases in Pediatric Acute Care
resultant acute kidney injury. Further treatment directed at
specific metabolic abnormalities may be necessitated.
Worsening of the moderate right pleural effusion
requires frequent assessment. This patient is receiving a
significant amount of fluid and is at risk for further fluid
accumulation. The pericardial drain will prevent reaccu-
mulation in the pericardium.
Further Reading
Acker, S.N., Linton, J., Tan, G.M., et al. (2015). A
multidisciplinary approach to the management of anterior
mediastinal masses in children. Journal of Pediatric Surgery
50: 875–878.
Henry, M. and Sung, L. (2015). Supportive care in pediatric
oncology. Pediatric Clinics of North America 62: 27–46.
Kang, H.M., Kim, J.Y., Choi, E.H., et al. (2018). Clinical
characteristics of severe histiocytic necrotizing
lymphadenitis (Kikuchi–Fujimoto disease) in children.
Journal of Pediatrics 171: 208–212. doi: 10.1016/j.
jpeds.2015.12.064.
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Case Resolution
The patient responded well to induction therapy and was
able to transfer to the hematology/oncology ward after
her metabolic derangements were stabilized and her peri-
cardial effusion resolved and the pericardial drain was
removed.
Mejia, R., Rodriguez, N.J., Cortes, J.A., et al. (2016). Oncologic
emergencies and complications. In: Rodgers’ Textbook of
Pediatric Intensive Care (ed. D.G. Nichols and D.H.
Shaffner), 1872–1893. Philadelphia, PA: Lippincott
Williams & Wilkins.
Prusakowski, M.K. and Cannone, D. (2017). Pediatric
oncologic emergencies. Hematology Oncology Clinics of
North America 31: 959–980.
Stephanos, K. and Picard, L. (2018). Pediatric oncologic
emergencies. Emergency Medicine Clinics of North America
36(3): 527–535. doi: 10.1016/j.emc.2018.04.007.

89
Complication of Traumatic Brain Injury
Christine Schindler1,2
1
Marquette University College of Nursing, Milwaukee, WI, USA
2
Medical College of WI/Children’s Hospital of WI, Milwaukee, WI, USA
A previously healthy 14-year-old boy is admitted to the
pediatric intensive care unit (PICU) with severe traumatic
brain injury (TBI).
History of Present Illness
The teen was an unrestrained passenger in a high-speed
motor vehicle collision 3 days ago. He presented to the chil-
dren’s hospital trauma center with a Glasgow Coma Scale
(GCS) score of 7 and initial head computed tomography
(CT) demonstrated evidence of diffuse axonal injury with
cerebral edema and slit-like ventricles (Figure 89.1). He
was emergently intubated, and the severe TBI protocol
initiated.
Past Medical History
The patient was born at term and discharged home with his
mother at 2 days of life. He has no medical problems and
has received all required vaccines along with annual influ-
enza immunization. His weight is at 40% for gender and age
and current body mass index (BMI) is 19.5. He has been
well throughout his life with the only notable injury a
non-comminuted right radial fracture at 5-year-old.
Past Surgical History
No surgical history, except for circumcision at birth.
Family History
The patient’s father has hypertension and type 2 diabetes
and his mother has Crohn’s disease. The patient has a
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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375
12-year-old sister with well-controlled atopic dermatitis
and asthma and a healthy 10-year-old brother.
Current Status
The patient is being cared for in the PICU for manage-
ment of severe TBI. He is currently intubated and
mechanically ventilated with tight carbon dioxide con-
trol, is deeply sedated, and on seizure prophylaxis with
levetiracetam. He has an extraventricular drain in place
monitoring intracranial pressures ranging from 15 to
18 mmHg. Norepinephrine is infusing at 0.05 μg/kg
per min to maintain cerebral perfusion pressure of
65 mmHg. The teen has a urinary drainage catheter in
place and on day 3 post injury he develops an acute
increase in urine output. For the last 3 hours, urine out-
put has been 7 mL/kg per hour.
The results of a basic metabolic profile are shown in
Table 89.1. The results of serum and urine osmolality are
as follows:
••
serum osmolality 332 mosmol/kg
urine osmolality 190 mosmol/kg
•
urine specific gravity 1.005
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 Why are children with severe traumatic brain injury at
risk for diabetes insipidus?
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376 Cases in Pediatric Acute Care

Rationale and Evidence-based Practice

Explanation

Based on the data given, what are the differential

diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include syndrome of
inappropriate antidiuretic hormone secretion (SIADH),
cerebral salt wasting (CSW), nephrogenic diabetes insipi-
dus, and central diabetes insipidus.
The most likely diagnosis for this child is central
diabetes insipidus. Diabetes insipidus is a disorder resulting
in decreased secretion (central/neurogenic) or action
(nephrogenic) of antidiuretic hormone (ADH). ADH acts
on specific vasopressin receptors and its main physiologic
actions are to increase water absorption in the distal neph-
ron and promote the expression of specific water channel
proteins on the luminal surface of the collecting duct. Dia-
betes insipidus manifests with loss of large volumes (>5
mL/kg per hour) of dilute urine (specific gravity <1.010)
Figure 89.1 Head CT. in the presence of high plasma osmolality (>295 mos-
mol/kg). Diabetes insipidus is distinguishable with polyuria
and polydipsia in uncontrolled diabetes mellitus or other
forms of solute diuresis by the absence of glucosuria and
Table 89.1 Basic metabolic profile.
a relatively normal rate of total urinary solute excretion.
Sodium 158 mEq/L
Potassium 2.9 mEq/L
Chloride 102 mEq/L Why are children with severe traumatic brain
Carbon dioxide 22 meq/L
injury at risk for diabetes insipidus?
Blood urea nitrogen 28 mg/dL Neuroendocrine dysfunction can be a consequence of TBI
Creatinine 0.8 mg/dL and is a result of both anterior and posterior pituitary insuf-
Glucose 110 mg/dL ficiency. TBI can directly and indirectly lead to damage of
Calcium 8.2 mg/dL
the ADH-producing neurons, their axons, or the posterior
pituitary resulting in post-traumatic diabetes insipidus.
The pituitary gland and stalk are at particular risk of direct
injury in high-speed acceleration–deceleration shear-type
injuries, because the stalk is surrounded anatomically by
Continuation of case: This 14-year-old is diagnosed with
the rigid diaphragma sellae and the gland is encased within
post-traumatic diabetes insipidus. His central venous pres-
the rigid bony sella. The rotational velocity of the head may
sure (CVP) decreases from 7 to 4 mmHg. In children with
result in stretching or tearing of small vessels or neuronal
altered consciousness, hypotonic polyuria can rapidly lead
structures in the hypothalamic–pituitary unit. Indirect
to hypovolemia and hypernatremia. Rapid and accurate
injury to the hypothalamic–pituitary unit can result from
assessment of the patient’s volume status is a crucial com-
small vessel damage, inflammatory edema, and hypoxia.
ponent of clinical management. Hypovolemia is associated
The incidence of acute post-traumatic hypopituitarism of
with adverse outcomes in the child with severe TBI, and
any anterior pituitary hormonal axis is 50–76%. The inci-
therefore initial therapy includes rapid volume expansion
dence of posterior pituitary dysfunction (diabetes insipidus)
with 20 mL/kg of 0.9% normal saline administered over
in the acute state following moderate or severe TBI is
10 minutes.
approximately 20% and permanent diabetes insipidus
3 What additional therapies would be recommended at develops in approximately 7% of patients.
this time? It is often difficult to remember differences between
4 What determines if the therapy was effective? SIADH, CSW and diabetes insipidus (see Table 89.2).
5 Should any other diagnostic studies be obtained? SIADH occurs for a variety of reasons, including TBI,

Table 89.2 Characteristics of diabetes insipidus, SIADH and CSW.
Diabetes insipidus
Serum sodium High
Serum osmolarity (mosmol/kg) >295
Urine sodium Low
Urine osmolarity Low
Urine specific gravity <1.010
Urine output ≥4 mL/hour
meningitis, and space-occupying brain lesions. SIADH
occurs as a result of excessive release of ADH from the pos-
terior pituitary gland and causes findings of hyponatremia
and decreased urine output. CSW is also related to an intra-
cranial disease process, but the pathophysiology is not as
well understood. CSW results in renal loss of sodium with
hyponatremia and volume depletion. Diabetes insipidus is
a deficiency in ADH, resulting in polyuria with low specific
gravity. Diabetes insipidus can be either central or nephro-
genic; the nephrogenic form can be either congenital or
acquired.
What additional therapies would be recommended
at this time?
Ongoing fluid replacement should be guided by constant
clinical monitoring and CVP measurements to avoid under-
hydration leading to hypovolemia and potentially
decreased cerebral perfusion pressure, as well as overhydra-
tion which can exacerbate cerebral edema, increase intra-
cranial pressure, or precipitate pulmonary edema.
Additionally, hormonal replacement with desmopressin
(exogenous ADH) is indicated. 1-Deamino-8-D-arginine
vasopressin (DDAVP), or desmopressin, is a synthetic ana-
log of vasopressin with minimal pressor effects and is the
drug of choice for the management of diabetes insipidus.
Desmopressin can be administered orally, intranasally,
sublingually, or parenterally. In the intensive care setting,
the parenteral desmopressin formulation is preferred.
There is significant individual variability in the duration
and magnitude of effect. The dose of desmopressin should
be titrated to normalize urine output and provide the
required rate of plasma sodium reduction to avoid risks
of overtreatment. Additional doses of desmopressin should
Further Reading
Capatina, C., Paluzzi, A., Mitchell, R., et al. (2015).
Diabetes insipidus after traumatic brain injury. Journal
of Clinical Medicine 4: 1448–1462. doi: 10.3390/
jcm4071448.
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Chapter 89 Complication of Traumatic Brain Injury 377
SIADH CSW
Low Low
<280 <280
High High
High High
>1.020 >1.010
≤1 mL/hour 2–3 mL/kg per hour
be administered for evidence of persistent posterior pitui-
tary dysfunction with derangements in fluid balance,
serum osmolality, and serum sodium.
What determines if the therapy was effective?
Frequent electrolyte laboratory evaluation is indicated as
caution must be taken to avoid rapid correction of serum
sodium. Cerebral tissue is very sensitive to quick osmotic
changes, which can lead to worsening cerebral edema.
Clinicians should target a serum sodium correction rate
of no more than 0.5 mmol/hour or 10–12 mmol/L over
24 hours. Clinicians should titrate therapy for a urine out-
put of approximately 1 mL/kg per hour and urine specific
gravity of 1.010–1.030.
Should any other diagnostic studies be obtained?
Pituitary dysfunction following TBI can be significant and
the incidence of anterior pituitary dysfunction after TBI is
around 30% in the acute time frame and remains about 20%
at 1 year post injury. Consensus guidelines recommend
screening all patients with moderate to severe TBI for pitu-
itary axis dysfunction. Basal hormone testing should be
completed on any hospitalized patient with hypotension
or hyponatremia and testing at 3 and 12 months regardless
of severity of TBI should be performed. Basal testing
includes morning cortisol, free T3, free T4, thyroid-
stimulating hormone (TSH), insulin-like growth factor
(IGF)-1, follicle-stimulating hormone (FSH), luteinizing
hormone (LH), testosterone (males), estradiol (females),
prolactin, and a 24-hour urine collection for urinary free
cortisol. Patients with polyuria should also have serum
sodium and serum osmolality checked.
Idowu, O.E., Obafunwa, J.O., and Soyemi, S.O. (2017).
Pituitary gland trauma in fatal nonsurgical closed traumatic
brain injury. Brain Injury 31(3): 359–362. doi: 10.1080/
02699052.2016.1257823.

378 Cases in Pediatric Acute Care
Lal, R.A. and Hoffman, A.R. (2019). Pituitary response to
traumatic brain injury. In: Pituitary Disorders of Childhood
(ed. B. Kohn). Cham, Switzerland: Humana Press.
Robertson, G.L. (2016). Diabetes insipidus: differential
diagnosis and management. Best Practice and Research in
Clinical Endocrinology and Metabolism 30: 2015–218. doi:
10.1016/j.beem.2016.02.007.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Scranton, R.A. and Baskin, D.S. (2015). Impaired pituitary axes
following traumatic brain injury. Journal of Clinical
Medicine 4: 1463–1479. doi: 10.3390/jcm4071463.
Silva, P.P.B., Bhatnagar, S., Herman, S.D., et al. (2015).
Predictors of hypopituitarism in patients with traumatic
brain injury. Journal of Neurotrauma 32: 1789–1795. doi:
10.1089/neu.2015.3998.

90
Newborn with Hypothermia
Marshay James1,2
1
Vanderbilt University School of Nursing, Nashville, TN, USA
2
Department of Critical Care Medicine, St. Jude Children’s Research Hospital, Memphis, TN, USA
A 9-day-old female arrived at her local community emer-
gency department (ED) with lethargy and dehydration.
The infant was fussy the past few days, would not take a
bottle, and this morning was difficult to wake. The infant
is under custody of the grandmother who is unsure of
her perinatal course.
History of Present Illness
This 9-day-old infant became ill-appearing within the last
24 hours, was initially irritable and poorly consoled, pro-
gressing to poor oral intake, and inciting concern for the
caregiver. Temperature was not taken at home. There is
no report of cough, runny nose, difficulty breathing, wheez-
ing or other respiratory symptoms. Grandmother feels the
infant looks pale. The infant is having soft bowel move-
ments and no excess spitting.
Past Medical History
Previous hospital records reveal that this infant was born at
36.4 weeks’ gestation. At birth, she was appropriate for ges-
tational age, in the 40th percentile for weight and length,
with birthweight of 2.86 kg. She was delivered via uncom-
plicated vaginal delivery but admitted to the neonatal
intensive care unit because of the mother’s known sub-
stance abuse and concern for neonatal abstinence syn-
drome. She has had first hepatitis B vaccine, vitamin
K injection, and first metabolic screening completed.
Family History
This infant was discharged home to the maternal grand-
mother; mother incarcerated; father’s history is unknown
and he is not involved with the mother or infant.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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379
Current Status
On arrival to the ED, the infant is extremely lethargic,
with pediatric Glasgow Coma Scale (GCS) score of 6
(Table 90.1). Her initial vital signs include temperature
35.1 C (95.2 F), heart rate 68 bpm, respiratory rate
14 breaths per minute which are very shallow, blood
pressure 65/32 mmHg, and oxygen saturation with use
of non-rebreather mask 88%. The infant is intubated
and mechanically ventilated due to mental status and
poor respiratory effort. Complete blood count (CBC),
basic metabolic panel (BMP), C-reactive protein (CRP),
cerebrospinal fluid (CSF) studies including cell count,
Gram stain, and blood, urine and CSF cultures are
obtained, with herpes simplex virus (HSV) PCR. Results
for CBC, BMP and CSF analysis are shown in
Tables 90.2–90.4. CRP level is 234 mg/dL and initial lac-
tate level 7.5 mmol/L.
Head computed tomography (CT) is obtained prior to
lumbar puncture to assess the presence of increased intra-
cranial pressure. A chest radiograph demonstrates endotra-
cheal tube just proximal to the carina and no acute
intrapulmonary findings.
Questions
Answer the following questions using the details provided.
1 Based on the data, what are the differential diagnoses
and the most likely diagnosis for this child?
2 Given the most likely diagnosis, what is the initial phar-
macologic therapy?
3 What medications to treat herpes simplex virus would
be indicated?
4 What additional diagnostic studies would be warranted
at this time?
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380 Cases in Pediatric Acute Care

Table 90.1 Pediatric Glasgow Coma Scale (GCS) score. she had ampicillin and cefotaxime administered in the ED
and was given a 10 mL/kg fluid bolus.
Best eye opening No response, score 1
Best verbal response Moans to pain, score 2
Best motor response Abnormal flexion to pain, score 3
Total 6/15 Rationale and Evidence-based Practice
Explanation

Based on the data, what are the differential

Table 90.2 Complete blood count.
diagnoses and the most likely diagnosis for
White blood cell count 2500/mm3 this child?
Hemoglobin 11.0 g/dL Differential diagnoses for this child include non-accidental
Hematocrit 33.6% trauma, inborn errors of metabolism, and infectious etiol-
Platelet count 85 000/mm3 ogy including but not limited to sepsis, bacteremia and
Neutrophils 36% meningitis. The most likely diagnosis for this child is neo-
natal sepsis due to the child’s history and presenting
Lymphocytes 42%
symptoms of hypothermia, hypoglycemia, irritability pro-
Monocytes 13%
gressing to lethargy, decreased oral intake, and initial lab-
Eosinophils 1% oratory findings. Meningitis is unlikely with normal CSF
results, but cultures are still pending. This child also has
an unclear perinatal history and the maternal health status
Table 90.3 Basic metabolic panel. at the time of birth is unknown, so maternal infection may
be a possibility and typical neonatal organisms include
Sodium 145 mEq/L group B Streptococcus, Escherichia coli, Streptococcus pneu-
Potassium 3.9 mEq/L moniae, and Listeria monocytogenes. In viral pathogenesis,
Chloride 115 mEq/L herpes simplex virus (HSV)-1 and HSV-2 can cause neona-
Carbon dioxide 12 mEq/L tal meningitis, although HSV-2 accounts for 70% of cases.
Blood urea nitrogen 18 mg/dL
Late-onset neonatal sepsis or bacteremia occurs in infants
greater than 72 hours old and can still reflect bacterial organ-
Creatinine <0.1 mg/dL
isms present in early-onset neonatal infection. Organisms
Glucose 53 mg/dL
can be transferred through an ascending pathway from
Calcium 8.5 mg/dL mother to infant or through ruptured membranes. Neonatal
sepsis is associated with high risk of morbidity and mortality.
The risk of sepsis increases in a neonate whose mother had
fever, lack of prenatal care, premature labor, premature rup-
Table 90.4 CSF initial results. ture of membranes, group B Streptococcus colonization, uri-
nary tract infection, and others.
Appearance Clear
Sepsis is invasion by microbial pathogens, characterized
Opening pressure 15 cmH2O
by a proinflammatory response that can lead to severe sep-
White cell count 4500/mm3 sis and septic shock. In neonates, cytokines are regulators
Predominant cell type Lymphocytes of sepsis. Interleukin (IL)-6 is a cytokine which is evident
Protein 62 mg/dL in the neonatal population, and shows early response to
Glucose 46 mg/dL infection, preceding the increase in CRP. IL-6 acts as a sig-
Red cell count 0 nal in the activation of T cells, and induces the secretion of
antibodies by B cells and the differentiation of T cells. It sti-
mulates the release of other cytokines, increasing several
hours before the increase in CRP. These regulators
Continuation of case: This 9-day-old infant is transferred have high sensitivity and are capable of effective defense
to the pediatric intensive care unit (PICU) and placed on against pathogens; however, cytokines can dysregulate
minimal ventilatory support. After cultures were obtained, the immune response and ultimately promote tissue

damage. There is a fine line here, between repair and
improvement and further demise within the infectious
cascade.
Given the most likely diagnosis, what is the initial
pharmacologic therapy?
Neonates admitted from the community are at lower risk
for infection caused by multidrug-resistant pathogens than
infants who have been hospitalized since birth. The combi-
nation of ampicillin plus gentamicin or ampicillin plus a
third-generation cephalosporin (e.g. cefotaxime) are regi-
mens for empiric treatment of sepsis without an apparent
focus of infection, as in this setting. It is also recommended
to treat for HSV with acyclovir when the patient is a neo-
nate or young infant and recent maternal screens are not
available, or there is fever present. This patient will also
require fluid resuscitation and electrolyte supplementation.
Placement of a central venous line to administer inotropic/
vasopressor infusion and fluid boluses may be necessary
and an arterial line may be needed to monitor blood pres-
sure and obtain blood gases.
What medications to treat HSV would be
indicated?
Empiric therapy with acyclovir is definitely recommended
for children with CSF abnormalities and who have
Further Reading
Dong, Y. and Speer, C.P. (2015). Late onset neonatal sepsis:
recent developments. Archives of Disease in Childhood, Fetal
and Neonatal Edition 100: F257–F263. doi: 10.1136/
archdischild-2014-306213.
Good, P.I. and Hooven, T.A. (2019). Evaluating infants at risk
for early onset sepsis. Pediatric Clinics of North America 66:
321–331. doi: 10.1016/j.pcl.2018.12.003.
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Chapter 90 Newborn with Hypothermia 381
encephalitis or focal findings on examination, imaging,
or electroencephalography. However, a body of research
strongly suggests that an infant who is treated for sepsis
should also receive acyclovir until the CSF PCR for HSV
returns negative. Clinical features suggestive of HSV infec-
tion include mucocutaneous vesicles, seizures, lethargy,
respiratory distress, thrombocytopenia, coagulopathy,
blood oozing from intravascular catheter sites, hypother-
mia, sepsis-like illness, hepatomegaly, ascites, or elevated
transaminases.
What additional diagnostic studies would be
warranted at this time?
Obtaining coagulation studies is important to assist in
determining if the infant may be experiencing disseminated
intravascular coagulation, which is a concern for any
patient who is septic. If the infant remains stable and is able
to wean and tolerate extubation without any alterations in
hemodynamic status, routine monitoring of electrolytes
would continue until feedings are on board. It is also impor-
tant to monitor lactate and CRP levels to ensure that they
are decreasing. Evidence regarding mortality in neonates
with sepsis indicates that high CRP and lactate levels are
associated with higher risks of death, especially if levels
do not decrease. Following cultures and identification of
organisms will document definitive treatment.
Thomson, J., Sucharew, H., Cruz, A.T., et al. (2018).
Cerebrospinal fluid reference values for young infants
undergoing lumbar puncture. Pediatrics 141(3): e20173405.
doi: 10.1542/peds.2017-3405.
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383

91

Back Pain in an Active Teenager

Alicia McCarthy
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

A 14-year-old male presents to the outpatient orthopedic Family History

clinic for evaluation of back pain.
Negative for osteogenesis imperfecta or skeletal dysplasias.
Father with degenerative disk disease and disk herniation
History of Present Illness requiring single-level spinal fusion at age 52.

This 14-year-old male fell jumping off a friend’s deck yes-

terday; fall height approximately 1.83 m (6 feet). He landed
on his feet and then fell onto the lower part of his back. He
reports immediate pain after the injury, but was able to
walk. Pain is focused in his midline upper lumbar region,
worsens with movement, and improves with lying supine.
He reports pain is 8/10 in office today with movement, 5/10
when sitting. Family has given him intermittent
acetaminophen which has not provided much relief. Used
a wheelchair to get into clinic today.
Past Medical History
This teen was born full term, with no prenatal or neonatal
complications. Parents cannot recall specific developmen-
tal milestones but feel they were “normal” and similar to
those of his siblings. He was previously treated for low
vitamin D levels by his primary care provider but stopped
taking the supplements 1 year ago. Orthopedic history is
positive for distal non-displaced radius fracture at age 7,
after falling in a soccer game. No previous hospitalizations
or chronic health conditions. He weighs 65.2 kg,
height 176 cm.
Past Surgical History
There is no past surgical history.
Current Status
The patient is able to ask and answer questions appropri-
ately. He walks and changes positions independently but
with obvious signs of pain. No dysmorphic features, sclera
are white. Full neck range of motion without pain. Chest rise
is symmetric on inspiration, and there is no increased work
of breathing. Chest is without deformity. Head is level over
pelvis, sagittal balance is neutral. Focal pain with palpation
of the upper lumbar spine. Significant pain with mild for-
ward bending and hyperextension of the spine in this same
region. Skin is intact with no abrasions, bruises or rashes.
Extremities are pink, warm, no swelling or edema. Radial
and posterior tibial pulses are +2. No pain with palpation
of bilateral upper and lower extremities. There is 5/5
strength in all major muscle groups of upper and lower
extremities. Patellar and Achilles reflexes are at 2+, one beat
of clonus bilaterally. Babinski test is negative.
Questions
Answer the following questions using the details provided.
1 Based on history and physical examination, what are
the differential diagnoses and the most likely diagnosis
for this child?
2 What testing is required for definitive diagnosis?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

384 Cases in Pediatric Acute Care
Continuation of case: The patient is sent for anteroposte-
rior/lateral erect spine radiographs (entire spine on single
cassette, see Figure 91.1), and a total 25-hydroxyvitamin
D level is obtained in the laboratory (result 19 ng/mL).
3 What treatment is recommended to stabilize the spine?
4 What pain relief interventions should be
recommended?
5 Are other interventions required and what follow-up is
needed?
Figure 91.1 Erect spine radiographs.
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Rationale and Evidence-based Practice
Explanation
Based on history and physical examination, what
are the differential diagnoses and the most likely
diagnosis for this child?
Initial differential diagnoses include muscular pain, frac-
ture of the spine, and disk herniation or bulge. Fracture
of the spine is more likely due to focal nature of pain and

midline location. Disk herniation or bulge is less likely due
to absence of radicular and neurologic symptoms and nor-
mal neuromotor testing. The patient was at increased risk
for low vitamin D levels due to his history of this condition,
and the fact that he had stopped supplementation.
What testing is required for definitive diagnosis?
Radiographs should be obtained as a first-line evaluation
for back pain after trauma and for chronic back pain and
should be taken in the erect position. Although radiographs
should generally be limited to the area of concern, in the
case of possible compression fractures, imaging of the
entire spine is important to evaluate a possible impact on
overall alignment.
The radiographs for this patient indicate compression
fractures of L1 and L2, with less than 25% loss of vertebral
height of each vertebra. There is no overall change in his
sagittal alignment. Because of physical examination find-
ings, mild loss of vertebral height and no change in align-
ment on X-ray, no additional imaging (such as MRI or CT)
is required for diagnosis and treatment.
Laboratory work confirms deficiency of vitamin D, which
places the patient at increased risk for fracture.
What treatment is recommended to stabilize
the spine?
External bracing (in this case lumbosacral orthosis) should
be used at all times when not bathing or sleeping. The teen
Further Reading
Booth, T.N., Iyer, R.S., Falcone, R.A., et al. (2017).
ACR Appropriateness Criteria® Back Pain-Child.
Journal of the American College of Radiology 14(5 Suppl):
S13–S24.
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Chapter 91 Back Pain in an Active Teenager 385
may return to school, but should rest from activities that
require prolonged standing and cannot participate in
gym or sports.
What pain relief interventions should be
recommended?
External bracing will provide some amount of pain relief. In
addition, he may take non-steroidal anti-inflammatory
drugs (NSAIDs) for pain relief. Other possible pain relief
methods include heat or ice application as needed, and life-
style modifications (such as sitting in a supportive chair).
Are other interventions required and what
follow-up is needed?
It is also important to supplement vitamin D. Recom-
mended dosing should be 2000 IU of vitamin D daily, with
levels rechecked in 12 weeks. If the level is normal at that
time, the patient should continue on maintenance dosing of
600–1000 IU daily as a long-term medication.
The patient should return in 6 weeks for repeat antero-
posterior/lateral erect spine radiographs (entire spine on
single cassette). At that visit, if symptoms are improved,
he may discontinue his orthosis and start a course of phys-
ical therapy to work on postural training, core strengthen-
ing, back and lower extremity stretching. He could resume
sports to tolerance once cleared by the physical therapist
(likely 4–6 weeks).
Thompson, R.M., Dean, D.M., Goldberg, S., et al. (2017).
Vitamin D insufficiency and fracture risk in urban children.
Journal of Pediatric Orthopedics 37(6): 368–373. doi: 10.1097/
BPO.0000000000000697.

92
Teen with Fever and Abdominal Pain
Sarah Martin
Department of Pediatric Surgery, Ann & Robert H. Lurie Children’s Hospital, Chicago, IL, USA
A 13-year-old-female with a 2-day history of fever and
abdominal pain presents to the emergency department
(ED) for evaluation.
History of Present Illness
This 13-year-old female reports a 2-day history of low-grade
fever and abdominal pain that started at her umbilicus and
now localizes to the right lower quadrant. Her mother
reports a tactile fever and states her daughter has not been
eating or drinking very well. The child has been treated
with acetaminophen every 6 hours as needed for her tactile
fever and Maalox for her abdominal pain. The child has not
had emesis or diarrhea and last voided 10 hours ago. Her
last reported oral intake was a popsicle that she ate 4 hours
prior to her ED presentation. Her last menstrual period was
1 week ago. Her parents deny any recent gastrointestinal
illnesses in the family.
Past Medical History
This teen was adopted and her birth history is unknown.
Her immunizations are up to date and she takes no
prescription or over-the-counter medications regularly.
She was diagnosed with strep throat 6 months ago. She
has never been to an ED or required hospitalization.
Past Surgical History
There is no past surgical history.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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387
Family History
The family history is unknown due to her adoption.
Current Status
On arrival to the ED, the patient reports she has abdominal
pain of 7/10. Her vital signs are as follows: temperature
38 C (100.4 F) orally, heart rate 116 bpm, respiratory rate
15 breaths per minute, blood pressure 108/66 mmHg,
and oxygen saturation 100% on room air.
Anthropometric measurements reflect normal growth
parameters and physical examination reveals an alert teen-
ager watching a movie on her phone. Her head, ears, nose,
throat, and chest examination are unremarkable. Abdomi-
nal examination is significant for right lower quadrant pain
with rebound tenderness; there are no masses or hernias
present. Her extremities are pink, warm, dry with brisk cap-
illary refill time. A basic metabolic panel, complete blood
count, and urinalysis are obtained (see Tables 92.1–92.3).
An abdominal ultrasound is performed done over concern
for appendicitis. A peripheral intravenous catheter is
inserted and intravenous fluids administered at mainte-
nance rate after she receives a 20 mL/kg normal saline fluid
bolus for oliguria. The child receives a dose of morphine for
her abdominal pain. She is ordered nil by mouth until her
diagnosis and treatment plan can be determined.
Abdominal ultrasound identified dilated non-
compressible tubular structure in the right lower quadrant
consistent with an appendix measuring up to 1.1 cm in
maximum transverse diameter. There was adjacent inflam-
matory fat stranding and increased vascularity. Sono-
graphic findings are consistent with acute simple or
uncomplicated acute appendicitis.
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388 Cases in Pediatric Acute Care

Table 92.1 Complete blood count. Continuation of case: The child undergoes a laparoscopic
appendectomy for category 1 appendicitis, which can be
White blood cell count 18 000/mm3
defined as an appendix with acute appendicitis, localized
Hemoglobin 12 g/dL appendicitis, without gangrene, perforation, or abscess
Platelet count 234 000/mm3 (Baxter et al., 2018).
Neutrophils 85%
4 What is appropriate care in the immediate postopera-
Lymphocytes 7.4%
tive period for a child who has undergone a laparo-
Monocytes 7% scopic appendectomy?
Eosinophils 0.2% 5 What would be the plan for hospital discharge and fol-
Basophils 0.2% low-up?

Table 92.2 Basic metabolic panel.

Rationale and Evidence-based Practice
Sodium 137 mEq/L
Explanation
Potassium 3.9 mEq/L
Chloride 102 mEq/L
Based on the data given, what are the differential
Carbon dioxide 18 mEq/L diagnoses that should be considered and the most
Blood urea nitrogen 20 mg/dL likely diagnosis for this teen?
Creatinine 0.56 mg/dL
Differential diagnoses to consider for this child include a
Glucose 98 mg/dL urinary tract infection, ovarian torsion, ovarian cyst, gastro-
enteritis, and appendicitis. Based on this child’s elevated
white blood cell count with a left shift and her ultrasound
Table 92.3 Urinalysis. findings, she most likely has appendicitis. Appendicitis is
the most common emergent indication for abdominal
Specific gravity 1.017 surgery in children. The peak incidence of diagnosis is
Color Yellow 10–19 years of age (Wagner et al., 2018). Although nearly
Nitrite Negative half of the children diagnosed with appendicitis will have
Blood Negative an atypical presentation, this patient had many of the clas-
Glucose Negative sic symptoms including dull periumbilical pain migrating
to the right lower quadrant, fever, and anorexia.

Questions What is the most appropriate testing for this

Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses that should be considered and the most likely
diagnosis for this teen?
2 What is the most appropriate testing for this diagnosis?
Continuation of case: With the confirmation of appendi-
citis based on imaging findings, the child is started on intra-
venous antibiotics and prepared for a laparoscopic
appendectomy. Ceftriaxone and Flagyl were prescribed
with 24-hour dosing. A human chorionic gonadotropin
point-of-care urine test was obtained and was negative.
3 What are appropriate interventions for the treatment of
simple acute appendicitis?
diagnosis?
There are several appendicitis scores (e.g. Alvarado, Appen-
dicitis Inflammatory Response Score, Pediatric Appendici-
tis Score) that can aid clinicians when making the
diagnosis. Depending on the score, a white blood cell count
of greater than 10 000–15 000/mm3 with a neutrophil count
above 75% are laboratory findings that contribute to the
likelihood of appendicitis, along with clinical findings of
right lower quadrant pain, anorexia, and guarding
(Wagner et al., 2018). Radiologic evaluation includes the
use of ultrasound, computed tomography (CT), and mag-
netic resonance imaging (MRI). CT scan is known to be
the most sensitive but does expose children to ionizing radi-
ation. Ultrasound is the most commonly performed radio-
logic test and can be done in the awake toddler and child

without exposure to ionizing radiation. Ultrasound has
high specificity but low sensitivity because operator experi-
ence is variable (Mittal et al., 2013). The findings of
acute appendicitis include an inflamed fluid-filled appen-
dix, a non-compressible blind-ending structure measuring
greater than 6 mm in maximal diameter; an appendicolith
may or may not be present. Negative ultrasound findings
include the organ not visualized with no secondary findings
suggestive of appendicitis. MRI is gaining popularity as it
does not employ ionizing radiation and contrast is not
needed. Most centers can perform the test without sedation
in children older than 5 years (Mittal et al., 2013). Findings
suggestive of appendicitis on MRI include an appendiceal
diameter greater than 7 mm, periappendiceal fat infiltra-
tion, and restricted diffusion of the appendiceal wall
(Mittal et al., 2013). Availability of this modality and high
cost are factors that are making use of this terchnique
variable.
What are appropriate interventions for the
treatment of simple acute appendicitis?
Treatment approaches for appendicitis most often include
operative management with preoperative intravenous anti-
biotics, intravenous fluids, and intravenous pain manage-
ment. Laparoscopic appendectomy is the most common
operative intervention, with these procedures converted
to open operations as indicated. Non-operative intervention
has been recently studied and is an option in some cases of
appendicitis (Gonzalez et al., 2016). Candidates for non-
operative intervention are summarized in Table 92.4.
Non-operative candidates in a hospitalized setting are
administered antibiotics and diet is progressed based on
clinical examination findings. A success rate for non-
operative management is cited as 70–85% for children at
1 year (Gonzalez et al., 2016).
References
Baxter, K.J., Short, H.L., Travers, C.D., et al. (2018).
Implementing a surgeon-reported categorization of
pediatric appendicitis severity. Pediatric Surgery
International 34: 1281–1286. doi: 10.1007/s00383-018-
4364-8.
Benedict, L.A., Sujka, J., Sobrino, J., et al. (2018). Same-day
discharge for nonperforated appendicitis in children: an
updated institutional protocol. Journal of Surgical Research
232: 346–350. doi: 10.1016/j.jss.2018.06.057.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Chapter 92 Teen with Fever and Abdominal Pain 389
Table 92.4 Candidate inclusion criteria for non-operative
management of acute appendicitis.
7–17 years old
Abdominal pain less than 48 hours with no history of chronic
abdominal pain
White blood cell count 5000–18 000/mm3
Ultrasound imaging: hyperemia, <1.1 cm in diameter,
compressible or non-compressible, no abscess, no fecalith/
appendicolith, no phlegmon
No preoperative concern for perforation
No diffuse peritonitis
Not pregnant
No malignancy
Not currently taking antibiotics
What is appropriate care in the immediate
postoperative period for a child who has
undergone a laparoscopic appendectomy?
Care in the immediate postoperative period includes intra-
venous and oral pain medications, diet progression as toler-
ated, and early ambulation with no additional antibiotics
(Hornor et al., 2018). Ambulation and preparation for dis-
charge are also included in the postoperative plans.
What would be the plan for hospital discharge and
follow-up?
Same-day discharge is the evolving benchmark for children
undergoing appendectomy for simple acute appendicitis
(Benedict et al., 2018). Strategies to achieve this include pre-
operative education of the patient and family of planned
care trajectory. This patient would be discharged once afe-
brile, tolerating fluids with minimal nausea, voiding,
ambulating, no bleeding, non-distended abdomen and pain
controlled.
Gonzalez, D.O., Deans, K.J., and Minneci, P.C. (2016). Role of
non-operative management in pediatric appendicitis.
Seminars in Pediatric Surgery 25: 204–207. doi: 10.1053/j.
sempedsurg.2016.05.002.
Hornor, M.A., Liu, J.Y., Hu, Q.L., et al. (2018). Surgical
technical evidence review for acute appendectomy
conducted for the Agency for Healthcare Research
and Quality Safety Program for improving surgical
care and recovery. Journal of the American College of

390 Cases in Pediatric Acute Care
Surgeons 227: 605–617. doi: 10.1016/j.
jamcollsurg.2018.09.024.
Mittal, M.K., Dayan, P.S., Macias, C.G., et al. (2013).
Performance of ultrasound in the diagnosis of appendicitis
in children in a multicenter cohort. Academic Emergency
Medicine 20(7): 697–702. doi: 10.1111/acem.12161.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
Wagner, M., Tubre, D.J., Asensio, J.A., et al. (2018). Evolution
and current trends in the management of acute appendicitis.
Surgical Clinics of North America 98: 1005–1023. doi:
10.1016/j.suc.2018.05.006.

93
Complication in a 6-Year-Old with a Kidney Transplant
Alison Hewitt Torres
Johns Hopkins Children’s Center, Baltimore, MD, USA
A 6-year-old boy with a history of end-stage renal disease
(ESRD) due to obstructive uropathy status post living
related kidney transplant from his father 3 months ago pre-
sents to the clinic with 1-day history of diarrhea and emesis.
History of Present Illness
This 6-year-old boy presented to the nephrology clinic for
evaluation of dehydration, with two episodes of diarrhea
and one episode of vomiting overnight. He is 3 months sta-
tus post living related kidney transplant.
Past Medical History
This patient was diagnosed with presumed posterior ure-
thral valves at 20 weeks’ gestation in the setting of anhy-
dramnios, echogenic kidneys, and dilated bladder. Fetally
placed vesicoperitoneal shunt was attempted and subse-
quently replaced. The shunt again became dislodged and
elective cesarean section was planned for 31 weeks. The
infant then spent 3 months in the neonatal intensive care
unit and was discharged home with parents with an
indwelling suprapubic drain. He continued to have bilat-
eral grade 4–5 reflux despite multiple urologic surgical
interventions and his chronic kidney disease continued to
worsen. By age 5 this boy had reached ESRD and under-
went evaluation for transplant. Urology performed bladder
augmentation with continent stoma creation to optimize
bladder for transplant and received living related kidney
from his father 8 weeks later. Frequent viral infections,
neutropenia, and low-level donor-specific antibodies have
complicated his transplant course. His baseline creatinine
is 0.4 mg/dL.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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391
Past Surgical History
The child is status post two fetal surgeries, both for vesico-
peritoneal shunt placement; and is status post suprapubic
catheter placement in the neonatal period. Bladder aug-
mentation with continent stoma creation was done prior
to transplant in order to facilitate optimal drainage of urine
for transplant. He also had a living related kidney trans-
plant from his father; surgical course was uncomplicated.
Family History
No family history of kidney problems or anyone requiring
dialysis. Paternal grandmother with history of cancer.
Current Status
On arrival to clinic, the patient is lethargic, pale, and ill
appearing. Vital signs are as follows: temporal temperature
37.8 C (100 F), heart rate 145 bpm, respiratory rate
40 breaths per minute, automated blood pressure
65/34 mmHg, and oxygen saturation 95% on room air.
The lips are dry and cracked, capillary refill is delayed at
3–4 seconds. Central pulses are easily palpable, but periph-
eral pulses are thready. Abdomen is distended with Foley
catheter in continent stoma. Basic metabolic panel and
complete blood count are obtained at an outside facility
prior to coming to clinic, and results are shown in
Tables 93.1 and 93.2.
A blood culture, urine culture and urinalysis are obtained
and sent to the inpatient laboratory.
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392 Cases in Pediatric Acute Care

Table 93.1 Basic metabolic panel. normotensive, shock and non-compensated, or hypoten-
sive, shock. This patient’s clinical presentation is consistent
Sodium 138 mEq/L
with non-compensated shock as he is lethargic and ill
Potassium 4.8 mEq/L appearing, with delayed capillary refill and hypotension.
Chloride 100 mEq/L Patients presenting in shock require immediate interven-
Carbon dioxide 9 mEq/L tion. Pediatric patients presenting with hypotensive shock
Blood urea nitrogen 38 mg/dL are in imminent danger of death.
Creatinine 2.2 mg/dL Given this 6-year-old’s recent diarrheal episodes in com-
Glucose 121 mg/dL
bination with his increased urine output due to his history
of obstructive uropathy, hypovolemic shock remains high
Calcium 7.8 mg/dL
on the differential. Another significant consideration in
determining the diagnosis for the patient is his current
immunosuppression regimen as well as his induction reg-
Table 93.2 Complete blood count.
imen related to kidney transplant 3 months ago. Pediatric
White blood cell count 2800/mm3 kidney transplant guidelines recommend that recipients
Hemoglobin 8 g/dL receive a biologic, either an interleukin 2 receptor antago-
nist or a lymphocyte-depleting agent for induction. This
Hematocrit 29.3%
child received a lymphocyte-depleting agent, thymoglobu-
Platelet count 493 000/mm3
lin, for induction. Thymoglobulin will deplete lymphocytes
Neutrophils 17% for 2 months but up to 6 months. Most pediatric kidney
Lymphocytes 59% transplant patients require triple immunosuppression, with
Monocytes 23% a calcineurin inhibitor, an antimetabolite, and a steroid;
Eosinophils 1% however, some patients are on a steroid-sparing protocol.
Absolute neutrophil count 500/mm3 Management goals include balancing sufficient immune
suppression to prevent donor-specific antibody formation
and rejection, while also allowing the immune system to
Questions fight off infection. The patient is neutropenic at presenta-
tion and in addition to his immunocompromised state,
Answer the following questions using the details provided. he has other risk factors for development of urinary tract
infections including a complex urologic system requiring
1 Based on the data, what are the differential diagnoses
intermittent catheterization to avoid urinary stasis.
and the most likely diagnosis for this child?
2 What is the initial management for this child?
3 Which antibiotics would be most appropriate and why? What is the initial management for this child?
Early identification of shock and early interventions are
critical. Because the patient is in the clinic but unstable,
a plan for transfer to a higher level of care should be imme-
Rationale and Evidence-based Practice
diately implemented by activating the code team or calling
Explanation 911. While awaiting help, this 6-year-old needs oxygen
even if he is not having respiratory symptoms. Oxygen
Based on the data, what are the differential
via a non-rebreather mask is recommended. A peripheral
diagnoses and the most likely diagnosis for
intravenous catheter or intraosseous access should be
this child?
obtained and isotonic crystalloid 20 mL/kg bolus should
Based on the presentation and laboratory results, the differ- be rapidly administered using a push–pull method. Reas-
ential diagnoses for this child include viral illness, hypovo- sessment following the fluid administration is important
lemic shock, and septic shock with acute kidney injury. as fluid can worsen clinical status in some cases, such
The most likely diagnosis for this child is hypovolemic as cardiogenic shock and some instances of distribu-
and septic shock due to urosepsis. tive shock.
The American Heart Association defines shock as a The patient will be seen briefly in the emergency depart-
condition in which peripheral tissues and end organs ment, where broad-spectrum antibiotics should be admi-
do not receive adequate oxygenation and nutrients. Pedia- nistered without delay. If shock is not improving after
tric shock is further delineated into compensated, or fluid administration, inotropic support should be initiated.

Based on the previous history, and current sepsis picture,
this child would also be admitted to the critical care unit
for continuous monitoring, especially of cardiorespiratory
status. Because of his chronic steroid requirement, this
child is also at risk for adrenal insufficiency and should
be given stress-dose steroids comprising hydrocortisone
2 mg/kg per dose (100 mg maximum).
Which antibiotics would be most appropriate
and why?
As the patient is toxic in appearance on arrival to the
clinic, broad-spectrum parenteral antibiotics are indicated.
Escherichia coli is the most common organism seen in
urinary tract infections, followed by Klebsiella, Proteus
spp., Staphylococcus saprophyticus, and Staphylococcus
aureus. Children with complex urologic anatomy and those
requiring frequent catheterization experience Enterococcus
and Pseudomonas urinary tract infections more commonly.
To cover the potential infectious organisms for this
patient, best practice would include Gram-positive
Further Reading
Bilgutay, A.N., Roth, D.R., Gonzales, E.T., et al. (2016).
Posterior urethral valves: risk factors for progression to renal
failure. Journal of Pediatric Urology 12(3): 179e1–179e7. doi:
10.1016/j.jpurol.2015.10.009.
Plunket, A. and Tong, J. (2015). Sepsis in children. British
Medical Journal 350: h3017. doi: 10.1136/bmj.h3017.
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Chapter 93 Complication in a 6-Year-Old with a Kidney Transplant 393
coverage with methicillin-resistant organism coverage
and Gram-negative coverage for potential Pseudomonas
or E. coli. The patient appropriately received vancomycin
15 mg/kg and cefepime 50 mg/kg while awaiting culture
results. Vancomycin should be dosed by levels to avoid
nephrotoxicity, with first level obtained prior to the third
dose. Cefepime is dosed every 8 hours.
In the pediatric intensive care unit, a collaborative
approach to care is important. Consult with infectious dis-
ease and continued follow-up with the nephrology team
are indicated to determine continued management. Based
on pediatric sepsis guidelines, laboratory studies include a
complete blood count, serum electrolytes with glucose,
blood culture, blood gas, serum lactate, liver function tests,
coagulation studies, C-reactive protein along with urinal-
ysis with culture and chest radiograph. Serum lactate is
most important as an indicator of inadequate oxygen deliv-
ery, which is suggestive of septic shock. For this patient, a
renal ultrasound should be obtained, although stabiliza-
tion of the patient is the most important priority at
this point.
Porat, A. and Kesler, S. (2018). Urosepsis. In: StatPearls.
Treasure Island, FL: StatPearls Publishing. Available
from https://www.ncbi.nlm.nih.gov/books/
NBK482344/

94
Acute Hypertension and Seizures in a 17-Year-Old Male
Alison Hewitt Torres
Johns Hopkins Children’s Center, Baltimore, MD, USA
A 17-year-old male with no significant medical problems
presents with hypertension, new-onset seizure, and left vis-
ual field abnormality for the past week.
History of Present Illness
This teen presented to the emergency department (ED) via
the emergency medical services after having his first seizure
while standing in line at the grocery store. When parame-
dics arrived, his blood pressure ranged in the 250s mmHg
systolic and 150s mmHg diastolic. The patient has no his-
tory of seizures and no history of elevated blood pressure.
He is a competitive swimmer, and has noticed muscle
cramps and soreness over the last few weeks. He reports
that he has a white spot in the left visual field of his left
eye for the last week.
Past Medical History
The patient’s past history includes failure to thrive (FTT) as
a toddler, attributed to caloric deficiency, urinary tract
infection (UTI) or kidney infection as a toddler, for which
mother reports he was treated with antibiotics. He had no
further problems.
Past Surgical History
No prior surgeries.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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395
Family History
No family history of neurologic problems including sei-
zures or hypertension. Both mother and maternal grand-
mother have histories of UTI and the maternal
grandmother also has renal failure secondary to liver fail-
ure. Mother has a history of kidney stones and malignant
hyperthermia with general anesthesia.
Current Status
On arrival to ED, the patient is alert and reporting a head-
ache, which he attributes to hitting his head during seizure,
and does have a hematoma on his left scalp. He is alert,
oriented, and cooperative with the physical examination.
Pupils are equal, round, reactive to light, and with extrao-
cular movements intact. Cranial nerves III–XII intact, nor-
mal gait, 5/5 strength in all four extremities. Mucous
membranes are moist. Heart rate is regular with normal
sinus rhythm. No murmur on auscultation. Lungs are clear
to auscultation bilaterally with no adventitious sounds
appreciated. Abdomen is soft, non-tender, and non-
distended with no hepatosplenomegaly. Extremities are
without edema. Peripheral pulses are easily palpable and
capillary refill is less than 2 seconds.
Vital signs are as follows: heart rate 76 bpm, respiratory
rate 20 breaths per minute, blood pressure 254/157 mmHg
right arm, and oxygen saturation 100% on room air.
Results of complete blood count, basic metabolic panel,
and urinalysis are shown in Tables 94.1–94.3.
Chest X-ray shows no focal consolidation, pleural effu-
sion, or pneumothorax. There is left basilar subsegmental
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396 Cases in Pediatric Acute Care

Table 94.1 Basic metabolic panel. Questions

Sodium 139 mEq/L
Answer the following questions using the details provided.
Potassium 3.8 mEq/L
Chloride 100 mEq/L 1 Based on the data given, what are the differential diag-
Carbon dioxide 16 mEq/L noses and the most likely diagnosis for this child?
2 Would any other diagnostic studies be recommended?
Blood urea nitrogen 102 mg/dL
3 What specialties should be consulted?
Creatinine 8 mg/dL
4 What is the best way to manage emergent hypertension
Glucose 122 mg/dL and what dosing would be used?
Calcium 7.6 mg/dL

Table 94.2 Complete blood count.

Rationale and Evidence-based Practice
White blood cell count 8650/mm 3 Explanation
Hemoglobin 8.3 g/dL
Based on the data given, what are the differential
Hematocrit 24.2%
diagnoses and the most likely diagnosis for
Platelet count 167 000/mm3
this child?
Neutrophils 71.1%
Lymphocytes 10.5%
This teenage patient is presenting with a hypertensive
emergency. The differential diagnoses include essential
Monocytes 7.5%
hypertension and secondary hypertension, although sec-
Eosinophils 2.7%
ondary hypertension is more likely given the severity of
blood pressure elevation. The differential diagnosis of sec-
ondary hypertension in children more commonly includes
Table 94.3 Urinalysis. renovascular disease or renal parenchymal disease. Other
less prevalent causes of hypertensive emergency in adoles-
Color Clear, yellow cence include secondary hypertension due to aortic coarc-
Specific gravity 1.008 tation, endocrine conditions such as pheochromocytoma or
pH 6.0 Cushing disease, or secondary hypertension related to rec-
Nitrite Negative reational drugs (see Table 94.4).
Ketones Negative The most likely diagnosis is hypertensive emergency sec-
Urobilirubin Negative ondary to renal parenchymal disease given the significantly
elevated serum creatinine level and distant history of UTI
Leukocytes Negative
and FTT. The American Academy of Pediatrics (AAP)
Protein 3+
defines hypertension as a blood pressure ≥95th percentile
Glucose Negative
for age, height, and sex, or above 130/80 mmHg for children
Blood Small 13 years and older. Primary or essential hypertension is
White blood cells 51 per high power field hypertension without a known etiology and is the most
Red blood cells 21 per high power field prevalent cause of hypertension is children over 6 years
Hyaline casts 0 old. Secondary hypertension means that there is an under-
Granular casts 0 lying cause for the hypertension (cardiac, renal, endocrine,
Bacteria Occasional
neurologic, vascular, ingestions, etc.). Hypertensive emer-
gency is defined as hypertension with end-organ damage
(abnormalities in the heart, eyes, kidneys and/or brain).
Prompt recognition and treatment of hypertensive emer-
atelectasis, mild cardiomegaly, and no acute osseous abnor- gency is critical in order to avoid further end-organ damage.
mality. Head computed tomography (CT) shows no evi- Treatment of hypertensive emergency should be targeted at
dence of acute intracranial process; left parietal scalp gradually reducing mean arterial pressure by no more than
swelling noted. 25% in the first hour and then gradually over the next
Toxicology screen is negative. 24–48 hours.
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Chapter 94 Acute Hypertension and Seizures in a 17-Year-Old Male 397

Table 94.4 Causes of hypertension related to age.

Age group Cause of hypertension Associated findings

Newborn Renal: anomalies of the kidneys and urinary tract, vascular
stenosis, thrombosis
Cardiac: coarctation of the aorta
Endocrine: primary hyperaldosteronism, congenital adrenal
hyperplasia, pheochromocytoma, Cushing disease
Preschool Renal: congenital anomalies of kidneys and urinary tract,
through age renal parenchymal disease, vascular stenosis, thrombosis
6 years
Cardiac: coarctation of the aorta
Enodcrine: pheochromocytoma, Cushing disease
School age Essential hypertension
(6–10 years)
Renal: congenital anomalies of kidneys and urinary tract,
renal parenchymal disease, vascular stenosis, thrombosis
Enodcrine: pheochromocytoma, Cushing disease
Adolescence Essential hypertension
Renal: congenital anomalies of kidneys and urinary tract,
renal parenchymal disease, vascular stenosis, thrombosis
Medications and recreational drugs: stimulants, oral
contraceptives, corticosteroids, cocaine, amphetamines
Endocrine: pheochromocytoma, Cushing disease
Elevated serum creatinine, abnormalities on
ultrasound
Discrepant four-extremity blood pressures/perfusion
Tachycardia, palpitations, sweating, elevated serum
cortisol levels
Elevated serum creatinine, abnormalities on
ultrasound
Discrepant four-extremity blood pressures/perfusion
Tachycardia, palpitations, sweating, elevated serum
cortisol levels
Obesity + family history
Elevated serum creatinine, abnormalities on
ultrasound
Tachycardia, palpitations, sweating, elevated serum
cortisol levels
Obesity + family history
Elevated serum creatinine, abnormalities on
ultrasound
Symptoms are related to medication or drug of
exposure
Tachycardia, palpitations, sweating, elevated serum
cortisol levels

Obtaining a detailed history and physical examination
will help guide the hypertension evaluation. Physical exam-
ination should include height, weight, calculated BMI, and
percentiles for age. Children with chronic illness often have
impaired growth, while obesity is associated with primary
hypertension. With stage II hypertension, or systolic blood
pressure ≥95th percentile + 12 mmHg or ≥140/90 mmHg
(whichever is lower), physical examination should include
both upper extremity blood pressure measurements and
one leg blood pressure measurement to evaluate for aortic
coarctation.
Would any other diagnostic studies be
recommended?
Work-up for hypertension includes tests to evaluate for sec-
ondary causes as well as to look for target organ damage.
The work-up should be guided by the age of the patient,
the severity of hypertension, associated comorbid condi-
tions, and findings on history and physical examination.
General work-up typically includes a urinalysis, compre-
hensive metabolic panel, and renal ultrasound with Dop-
pler. Additional work-up may include complete blood
count, thyroid studies, metanephrines, additional imaging
of the renal vasculature such as CT or magnetic resonance
angiogram, and sleep study based on the individual patient.
An eye examination and echocardiogram can help to eval-
uate for end-organ damage from hypertension. In this case
study, renal/bladder ultrasound was obtained later and
confirmed the diagnosis of hypertensive emergency second-
ary to renal parenchymal disease as it showed small echo-
genic kidneys with bilateral hydroureteronephrosis and
thickened bladder wall. He was diagnosed with end-stage
kidney disease due to congenital anomalies of the kidney
and urinary tract.
The AAP 2017 high blood pressure guidelines recom-
mend obtaining a renal ultrasound for children less than
6 years of age or those (regardless of age) with abnormal
urinalysis or renal function. Renal parenchymal disease
combined with renal vascular disease account for 63–74%
of secondary hypertension in children and adolescents.
What specialties should be consulted?
Specialty consultation is recommended for pediatric
patients presenting with stage II hypertension. Nephrology

398 Cases in Pediatric Acute Care
and cardiology are both specialty services that manage
hypertension. Depending on the suspected etiology, one
service may be consulted preferentially over the other. With
vision disturbance and concern for target organ damage, an
ophthalmology consultation is also recommended.
What is the best way to manage emergent
hypertension and what dosing would be used?
Recommended management for hypertensive emergency is
aimed at reducing further end-organ damage by decreasing
blood pressure by no more than 25% in the first hour, with
gradual reduction over the next 24–48 hours. Lowering
blood pressure is done gradually as the vascular beds have
accommodated to the current high pressure and lowering
too quickly will cause hypoperfusion and ischemic injury.
This can best be accomplished in the pediatric intensive
care unit by a continuous infusion of a calcium channel
blocker or beta-blocker with close observation often requir-
ing intra-arterial blood pressure monitoring. In addition to
the goal of reducing blood pressure, it is also important to
Further Reading
Flynn, J.T., Kaelber, D.C., Baker-Smith, C.M., et al. (2017).
Clinical practice guideline for screening and management of
high blood pressure in children and adolescents. Pediatrics
140(3): e20171904. doi: 10.1542/peds.2017-1904.
Seeman, T., Hamdani, G., and Mitsnefes, M. (2018).
Hypertensive crisis in children and adolescents. Pediatric
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plan for supportive care for potential life-threatening com-
plications such as seizures.
Medications used in a hypertensive crisis include the
following.
••
Calcium channel blocker: nicardipine 1–3 μg/kg per min.
Beta-blocker: esmolol 125–500 μg/kg per min or labetalol
0.25–3 mg/kg per hour.
•
Direct vasodilator: diazoxide 1–3 mg/kg every 5–15 min-
utes or hydralazine 0.2–0.6 mg/kg bolus.
Longer-term initial management of hypertension
includes pharmacologic therapy with an angiotensin-
converting enzyme (ACE) inhibitor, angiotensin receptor
blocker, long-acting calcium channel blocker, or a thiazide
diuretic. Beta-blockers are not recommended as first-line
treatment based on adverse effects and adult data that
there is a lack of effect with these agents. There are other
recommendations by the AAP for managing children
with other associated problems, such as diabetes and
proteinuria.
Nephrology 34(12): 2523–2537. doi: 10.1007//s00467-018-
4092-2.
Singh, D., Akingbola, O., Yosypiv, I., and El-Dahr, S. (2012).
Emergency management of hypertension in children.
International Journal of Nephrology 2012: 420247. doi:
10.1155/2012/420247.

95
Jittery 2-Week-Old Infant
Christopher D. Newman
Children’s Hospital Colorado, Aurora, CO, USA
A 2-week-old infant female is brought into your emergency
department because she is “not acting right.”
History of Present Illness
The infant was initially healthy on coming home from the
hospital following her birth, but over the last several days
has been increasingly “jittery,” with difficulty consoling,
rapid breathing and sweating. Until yesterday, she was
taking bottles well, but today she has not been latching
on to the bottle. Approximately an hour ago, her eyes
became unfocused and the patient’s mother decided to
bring her to the emergency department (ED).
Past Medical History
She was born at 39 weeks to a healthy mother and had an
uncomplicated delivery. She was discharged after 24 hours
and received her hepatitis vaccine prior to discharge. She is
scheduled for her 2-week well-child visit tomorrow.
Past Surgical History
She has had no surgeries.
Family History
The infant’s maternal grandmother had gestational diabe-
tes, otherwise there is no significant family history of pre-
natal or childhood illness.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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399
Current Status
In the examination room, she is lying in mother’s arms. She
is pale, diaphoretic, and tachypneic with a respiratory rate
of 55 breaths per minute. Her remaining vital signs are
temperature 35.4 C (95.7 F), heart rate 190 bpm, blood
pressure 80/40 mmHg, and weight 3.5 kg. The patient’s
fontanelle is slightly sunken, and mucous membranes are
dry. Lungs are clear to auscultation; cardiac examination
is normal except for tachycardia and 1+ peripheral pulses.
Abdomen is soft, non-tender, non-distended without hepa-
tosplenomegaly. Extremities are cool, but without defect or
tenderness. Her deep tendon reflexes are 2+ bilaterally. As
the physical examination is concluding, her eyes deviate to
the left and she has a series of tonic–clonic jerks in both
upper and lower extremities. The movements resolve after
15 seconds. A blood glucose level is obtained immediately
with a result of 34 mg/dL.
Questions
Answer the following questions using the details provided.
1 Based on the presentation, what are the differential
diagnoses for this patient’s hypoglycemia?
2 What, if any, additional laboratory tests should be
obtained before initiating treatment?
3 What is the appropriate initial treatment for this
patient?
Continuation of case: Following glucose administration,
the infant is more awake and active. Her repeat blood
glucose is 110 mg/dL. The laboratory results shown in
Table 95.1 are also obtained. Additionally, a glucagon stim-
ulation test is completed, which demonstrates a rise of more
than 30 mg/dL in serum glucose in the first 30 minutes.

400 Cases in Pediatric Acute Care
Table 95.1 Laboratory studies.
Insulin 26 μIU/mL
C-peptide 5 ng/mL
β-Hydroxybutyrate 0.3 mmol/L
Free fatty acids 0.3 mmol/L
Acylcarnitine profile Normal
Lactate 1 mmol/L
Ammonia 80 μg/dL
Urine organic acids Normal
4 What is the most likely cause of the patient’s
hypoglycemia?
5 What is the first-line therapy for this patient’s etiology
of hypoglycemia?
Rationale and Evidence-based Practice
Explanation
Based on the presentation, what are the
differential diagnoses for this patient’s
hypoglycemia?
Hypoglycemia in infancy can generally be divided into
broad categories: inadequate glucose supply (either
through inadequate intake or due to defects in fatty acid
oxidation, carbohydrate metabolism or amino acid metab-
olism) or increased glucose utilization (such as hyperinsu-
linism or some ingestions). Infants will rapidly exhaust
their glycogen stores without glucose intake and are there-
fore less tolerant of fasting. Additionally, illness that
increases metabolic demand can exhaust glucose supply
even in a feeding infant if the infant cannot keep up with
the increased metabolic demand. Common ingestions asso-
ciated with hypoglycemia can include ethanol, salicylates,
sulfonylureas, and beta-blockers.
Hyperinsulinism often presents in the first few weeks of
life. The most common cause is congenital hyperinsulin-
ism, although insulinomas and exogenous insulin adminis-
tration are also possibilities. In these conditions, insulin is
secreted out of proportion to glucose level and the usual
negative feedback loop that disrupts insulin production
when blood glucose levels fall below 60 mg/dL is inacti-
vated. Fatty acid oxidation disorders can include carnitine
deficiency, fatty acid transport defects, and defects of beta-
oxidation enzymes. The most common disorder is medium-
chain acyl-CoA dehydrogenase deficiency. The exact mech-
anism of hypoglycemia is not understood, but since ketone
bodies cannot be manufactured without mobilization of
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free fatty acids, the absence of ketone bodies as an energy
source may play a role. Disorders of carbohydrate metabo-
lism can impact metabolism of glycogen, galactose and
fructose. This group includes the glycogen storage diseases
(such as glycogen synthetase deficiency), disorders of glyco-
sylation (such as phosphoglucomutase 1 deficiency) and
disorders of gluconeogenesis (such as pyruvate carboxylase
deficiency). Although disorders of amino acid metabolism,
such as methylmalonic acidemia and propionic acidemia,
typically present with an episode of profound metabolic aci-
dosis, hypoglycemia may be present due to associated liver
dysfunction or protein malnutrition.
What, if any, additional laboratory tests should be
obtained before initiating treatment?
It is essential for diagnosing the cause of hypoglycemia that
laboratory testing be done on specimens obtained during
the state of hypoglycemia. In an unstable patient, the
patient’s safety comes first. However, ideally, the following
serum studies will be obtained prior to treatment of the
hypoglycemia: plasma glucose, C-peptide, β-hydroxybuty-
rate, free fatty acids, acylcarnitine profile, lactate, and
ammonia. Urine organic acids should also be sent. These
laboratory values can then be used to identify the most
likely cause of hypoglycemia from the differential
diagnosis.
What is the appropriate initial treatment for this
patient?
In older children who are alert enough to swallow safely,
10–20 g of rapidly absorbed glucose, such as honey, table
sugar, glucose tablets or glucose gel, may be administered.
In infants such as this patient, the ideal treatment is intra-
venous administration of dextrose 0.25 g/kg (maximum
25 g). In an unstable patient when intravenous access can-
not be achieved, administer glucagon 0.03 mg/kg intramus-
cularly. Glucagon will effectively treat hypoglycemia due to
increased glucose utilization, but may not treat other causes
and the effect may be transient. Efforts should continue to
establish intravenous access and administer intravenous
dextrose.
What is the most likely cause of the patient’s
hypoglycemia?
The elevated insulin level and appropriate response to glu-
cagon stimulation test are most consistent with hyperinsu-
linemia. Low ketone production (as evidenced by lower
β-hydroxybutyrate) also supports this diagnosis. The time
of presentation and the presence of seizure are most con-
sistent with a focal hyperinsulinemia. In one study, 50%

of patients with focal hyperinsulinemia presented with
seizure.
Because C-peptide and insulin are secreted in equal pro-
portions by the beta cells, the presence of an elevated
C-peptide is most consistent with endogenous insulin pro-
duction. A low C-peptide would raise concern for exoge-
nous insulin administration, either accidental or
intentional. Although ketone bodies would also be low in
fatty acid oxidation defects, free fatty acid levels should
be high and there should be minimal or no response to
the glucagon stimulation test. Disorders of carbohydrate
metabolism may present with different values based on
the specific defect. For example, glucose 6-phosphate dehy-
drogenase is often associated with a lactic acidosis. How-
ever, all will have minimal response to the glucagon
stimulation test and both insulin and C-peptide levels will
Further Reading
Gandhi, K. (2017). Approach to hypoglycemia in infants and
children. Translational Pediatrics 6(4): 408–420.
Hoe, F.M. (2008). Hypoglycemia in infants and children.
Advances in Pediatrics 55: 367–384.
Lord, K., Dzata, E., Snider, K.E., Gallagher, P.R., and De León,
D.D. (2013). Clinical presentation and management of
children with diffuse and focal hyperinsulinism: a review of
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Chapter 95 Jittery 2-Week-Old Infant 401
be low. Additionally, in most circumstances these defects
are associated with hepatomegaly, which this patient does
not have. Disorders of organic or amino acid metabolism
usually have ketosis associated with hypoglycemia. Addi-
tionally, these disorders are commonly associated with hep-
atomegaly and alterations in the urine organic acid profile.
What is the first-line therapy for this patient’s
etiology of hypoglycemia?
The first-line therapy for congenital hyperinsulinemia is
diazoxide, which blocks sulfonylurea receptors in the beta
cells. This in turn opens ATP-dependent potassium chan-
nels, thus decreasing insulin release. Diazoxide 5–15 mg/kg
daily is administered in three divided doses.
223 cases. Journal of Clinical Endocrinology and Metabolism
98(11): E1786–E1789.
Thornton, P.S., Stanley, C.A., De Leon, D.D., et al. (2015).
Recommendations from the Pediatric Endocrine Society for
evaluation and management of persistent hypoglycemia in
neonates, infants, and children. Journal of Pediatrics 167(2):
238–245.

96
Child with Leg and Back Pain
Jill Marks1,2
1
University of Colorado Pediatric Neurology, Boulder, CO, USA
2
Faculty Acute Care Pediatric Nurse Practitioner Program, Rush University College of Nursing, Chicago, IL, USA
A 12-year-old male with no past medical history is admitted
to the emergency department (ED) with a 3-day history of
leg and back pain.
History of Present Illness
He was in his usual state of health until 3 days prior to pres-
entation, when he noted bilateral leg pain and fatigue. He
complained to his parents who administered ibuprofen and
advised he drink extra water. The morning prior to presen-
tation, he was able to go to school, but complained of
feeling tired so came home early. Today, he continues to
complain of leg and lower back pain with fatigue. He
was able to get up and walk to the car, but needed
assistance. Caregivers and patient deny fever, vomiting,
diarrhea, or cold symptoms. He did have a cough and runny
nose a few weeks ago, as did the rest of the family. He
received an influenza vaccination 1 month ago.
Past Medical History
He was born at 37 weeks and was discharged 3 days after
delivery with parents. He has a history of anxiety and
depression. He takes sertraline 25 mg daily and sees a psy-
chologist twice a month. His development has been
typical.
Past Surgical History
He has had no prior surgeries or hospitalizations.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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403
Family History
Mother has a history of anxiety and a thyroid disorder.
Maternal grandfather died of stroke at age 55 years.
Current Status
Arrives to the ED by wheelchair. He is able to stand up and
move on to the gurney on his own with some assistance. He
is a pleasant alert child in no respiratory distress, with lungs
clear and equal. There is a normal S1 and S2 without appre-
ciable murmur. His abdomen is soft and non-distended.
Skin is without rashes. No joint abnormalities are noted.
Neurologic examination reveals cranial nerves II–X with-
out deficits. There is no dysmetria or dysdiadochokinesia.
His strength is 5/5 in the neck, head and upper extremities
and 3/5 in the lower extremities bilaterally. Reflexes are 1+
bilaterally at biceps, triceps, and brachioradialis, and absent
in the patella and Achilles. Sensory examination is unre-
markable. He has an absent Babinski. Gait is not ataxic.
Rectal tone is normal.
Results of basic metabolic profile and cerebrospinal fluid
(CSF) analysis are shown in Tables 96.1 and 96.2.
Questions
Answer the following questions using the details provided.
1 Based on the information given, what are the differential
diagnoses and the most likely diagnosis for this child?
2 Would you obtain any other diagnostic studies?
3 What is the optimal treatment for this neurologic con-
dition and how should it be ordered?
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404 Cases in Pediatric Acute Care

Table 96.1 Basic metabolic profile. (AIDP), acute motor axonal neuropathy and acute motor
and sensory axonal neuropathy (AMAN/AMSAN), multiple
Sodium 140 mEq/L
sclerosis, acute disseminated encephalomyelitis, and trans-
Potassium 4 mEq/L verse myelitis; (ii) spinal injury or compression (vascular
Chloride 100 mEq/L accident, mass); or (iii) infection (encephalitis, meningitis,
Carbon dioxide 23 mEq/L radiculitis, myelitis, epidural abscess). Tick paralysis and
Blood urea nitrogen 10 mg/dL myasthenia gravis can mimic GBS, as can a variety of met-
Creatinine 0.5 mg/dL abolic (thyroid) and electrolyte abnormalities. Myopathy
Glucose 80 mg/dL
and functional neurologic disorder are less likely but should
be considered. Infantile botulism should be considered in
Calcium 10.1 mg/dL
appropriate age groups.
Albumin 4.2 mg/dL
Protein 7.9 mg/dL
Aspartate aminotransferase 40 U/L Would you obtain any other diagnostic studies?
Alanine aminotransferase 42 U/L Further diagnostic studies should be completed to narrow
Bilirubin 0.1 mg/dL the differential. A lumbar puncture should be performed
Alkaline phosphatase 110 U/L and studies sent, including cell counts, glucose, protein
and culture. In patients with concern for elevated intracra-
nial pressure (ICP), head computed tomography (CT)
should be performed first. MRI of the brain and spinal cord
Table 96.2 Cerebrospinal fluid analysis. with and without contrast should be performed as well.
The most likely diagnosis for this child is GBS. CSF
White blood cell count 2/mm3
studies can help confirm the diagnosis of GBS but GBS is
Neutrophils 20%
a diagnosis based on clinical findings so these will be the
Lymphocytes 70% most important. In GBS, CSF will display a classic albumi-
Red blood cell count 100/mm3 nocytologic dissociation (elevated protein level), and nor-
Protein 80 mg/dL mal cell count with lymphocytic predominance. Early in
Glucose 68 mg/dL the course, the CSF profile may be normal, but by the sec-
Opening pressure 14 cmH2O ond week of symptoms more than 90% of patients will have
elevated CSF protein. MRI with gadolinium may show
enhancement of the peripheral nerve roots and cauda
equina, although it is notable that this is not specific and
4 What education would you provide to the family can also be seen following lumbar puncture and with
regarding disease time course? chronic inflammatory neuropathies. Specific anti-
ganglioside antibody testing can be performed as an
adjunct to diagnosis, but is only identifiable in approxi-
mately 50% of children. GBS is a clinical diagnosis and
treatment should be initiated immediately when there is
Rationale and Evidence-based Practice
a high degree of clinical suspicion. Further diagnostic stud-
Explanation ies should be completed to narrow the differential.
GBS is an acute inflammatory polyneuropathy of the
Based on the information given, what are the
peripheral nervous system and the most common cause
differential diagnoses and the most likely
of acute flaccid paralysis in children. Initial symptoms
diagnosis for this child?
include vague, poorly localized pain in the neck, back, but-
Differential diagnoses for patients with nervous system dis- tocks, and lower extremities (up to 50% of patients) fol-
order should include disease localization (in this case can lowed by rapid progression of symmetric ascending
broadly include pathology of the central nervous system, flaccid paralysis. Although absent (or very minimal)
peripheral nervous system, neuromuscular junction, nerve, reflexes are often thought of as a hallmark of GBS, reflexes
and muscle). The differential can be thought of in a may be preserved early in the course. Likewise, although
systematic manner: (i) demyelinating and post-infectious often described as symmetric, clinical cases can have some
conditions including Guillain–Barré syndrome (GBS), degree of asymmetry though not unilaterally. Children are
acute inflammatory demyelinating polyradiculoneuropathy generally afebrile and well appearing. Symptoms usually

peak or plateau around 7–14 days, although for some
patients it can be up to 4 weeks, and recovery occurs
2–4 weeks later. Symptoms may progress to include bulbar
weakness, respiratory failure, and autonomic nervous
system abnormalities (blood pressure instability, tachycar-
dia, pupillary abnormalities, incontinence, and altered
sweating).
Continuation of case: Magnetic resonance imaging (MRI)
of the brain with and without contrast is normal. MRI of the
spine with and without contrast shows enhancement of the
ventral and dorsal nerve roots in the thoracic and lumbar
spine and cauda equina.
What is the optimal treatment for this neurologic
condition and how should it be ordered?
Optimal treatment for GBS includes intravenous gamma-
globulin (IVIG), typically 2 g/kg divided over 2–5 days,
and plasma exchange, both of which shorten the disease
course and time to independent ambulation. They are most
effective when given within 2 weeks of symptom onset.
Although the use of these treatments shortens disease
course, they are not associated with overall difference in
long-term outcome. Patients should be carefully monitored
for bulbar weakness, respiratory muscle weakness, and
autonomic complications. Hospital admission for continu-
ous cardiorespiratory and pulse-oximeter monitoring is
indicated. Patients with neuromuscular weakness will
not demonstrate respiratory distress, and respiratory failure
in GBS can result from deteriorating diaphragmatic muscle
strength. Measures of diaphragm function and gas
exchange including NIF, VC, and exhaled carbon dioxide
should be monitored for patients with GBS. Hypoxia is a
late sign of respiratory failure in patients with GBS. Up
to 20% of patients may require mechanical ventilation dur-
ing the course of their illness. Indications for admission to
the intensive care unit include rapid progression of weak-
ness, flaccid quadriparesis, VC less than 20 mL/kg, bulbar
palsy, and evidence of autonomic dysfunction. A swallow
study should be obtained with any concern for bulbar
weakness. Other complications of GBS include constipa-
tion, urinary retention, pain, skin breakdown, and fatigue.
Rehabilitation service should be consulted and use of phys-
ical, occupational, and speech therapy services should be
initiated. Effective pain management should be a priority,
and psychosocial support for the patient and caregivers
through social work and child life services is imperative
as loss of function for any duration is traumatic for the
entire family.
GBS is generally thought of as a monophasic process with
low recurrence rate (2–5%). The mechanism for GBS
has not been clearly identified, but is most likely an
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Chapter 96 Child with Leg and Back Pain 405
immune-mediated response to a trigger (infection, surgery,
immunization) secondary to molecular mimicry. B and
T cells produce antibodies directed at antigenic proteins
of peripheral nerves. Peripheral nerve components are
attacked by self, secondary to molecular mimicry. Several
subtypes of GBS exist differentiated by the pattern of neu-
rologic deficits that occur, secondary to the variable gangli-
oside expression in different neural tissues. AIDP is the
form classically thought of and accounts for about 75% of
cases. AMAN is most often related to cases associated with
Campylobacter jejuni, thought to be related to the resem-
blance of of C. jejuni expressed lipo-oligosaccharides to
the carbohydrate component of gangliosides. Miller–Fisher
syndrome is a subtype characterized by ataxia, areflexia,
and ophthalmoplegia without weakness, though it can
progress to involve multiple cranial nerves leading to signif-
icant facial paralysis, bulbar weakness and include limb
and truncal weakness at times as well.
Although any virus or infection may trigger GBS, specific
infections implicated in this disease include Mycoplasma,
C. jejuni, pneumococcus, and Haemophilus influenzae.
Post-vaccination cases have been known to occur, particu-
larly related to the influenza vaccine, although they are rare
with risk of influenza morbidity outweighing risk of post-
vaccination GBS.
Continuation of case: Following evaluation in the ED, he
is admitted to the hospital. Blood gas testing reveals normal
carbon dioxide levels. Negative inspiratory force (NIF) and
vital capacity (VC) testing are in the normal range. Post-
void residual reveals more than 300 mL of urine remaining
in the bladder. During the first week of hospitalization, his
examination is notable for increased weakness of all extre-
mities, hypophonia, present but weak cough, dyspnea, and
continued areflexia. Although he does not have respiratory
distress, his blood gas evaluation reveals hyperacarbia and
he demonstrates a steady decline in NIF testing. He is trans-
ferred to the pediatric intensive care unit (PICU) for closer
monitoring. In the PICU he requires frequent oral suction-
ing for secretion management and is started on a cough
assist device to promote pulmonary toileting. Despite air-
way clearance efforts, on day 7 of hospitalization he
requires intubation for respiratory failure and ventilator
support for 1 week. Clean intermittent catheterization
and a bowel management routine are required for effective
elimination patterns. Vital signs are notable for orthostatic
hypotension, intermittent hypertension as well as alternat-
ing bradycardia and tachycardia. During this time, he has
periods of being withdrawn while other times he is very
angry and not adherent with treatment plans. Sleep
hygiene is initiated and with the assistance of psychiatry
his maintenance sertraline dose is increased. Consulting

406 Cases in Pediatric Acute Care
services included neurology, rehabilitation medicine,
speech therapy, physical therapy, psychiatry, occupational
therapy, child life, wound care team, and case manage-
ment. He requires a 5-week hospital stay, including 2 weeks
on the inpatient rehabilitation service.
What education would you provide to the family
regarding disease time course?
Disease course for GBS can be variable and the recovery
phase can be prolonged. Complete recovery can take sev-
eral months, especially if the patient has been intubated
and ventilated for a long period in the PICU. The vast
Further Reading
Centers for Disease Control. (2019). Vaccine Safety. Retrieved
from https://www.cdc.gov/vaccinesafety/concerns/guillain-
barre-syndrome.html.
Centers for Disease Control. (2019). Vaccine
Recommendations and Guidelines of the ACIP:
Contraindications and Precautions. Retrieved from https://
www.cdc.gov/vaccines/hcp/acip-recs/general-recs/
contraindications.html.
Chung, A. and Deimling, M. (2018). Guillain–Barré syndrome.
Pediatrics in Review 39(1): 53–54. doi: 10.1542/pir.2017-0189.
Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
majority of patients have excellent neurologic outcomes,
although a small number of patients will have residual neu-
rologic deficits including weakness or persistent loss of
reflexes. Children may be transferred to a rehabilitation
center for continuing care. Once the child is home, it is
important to immunize with influenza vaccine annually.
If GBS occurs within six weeks of a vaccination, it is impor-
tant to discuss with caregivers the risks and benefits of
future vaccination. The Centers for Disease Control Advi-
sory Council on Immunization Practices recommends pre-
caution rather than absolute contraindication in this
clinical scenario.
GBS/AIDP Foundation International (2012). GBS: An Acute
Care Guide For Medical Professionals. Narberth, PA: GBS/
AIDP Foundation International.
Karalok, Z.S., Taskin, B.D., Yanginlar, Z.B., et al. (2018).
Guillain–Barré syndrome in children: subtypes and
outcome. Childs Nervous System 34(11): 2291–2297.
Ryan, M. (2013). Pediatric Guillian–Barré syndrome. Current
Opinion in Pediatrics 25(6): 689–693. doi: 10.1097/
MOP.0b013e328365ad3f.

97
Fussy, Tired Infant
Charlene Pringle
University of Florida, Gainesville, FL, USA
A 6-week-old infant is admitted to the emergency department
(ED) with excessive crying, fatigue and staring episodes.
History of Present Illness
Infant woke up “screaming” the prior evening and was
fussy, crying, grunting, and “staring off into space” prompt-
ing ED visit. Caregivers deny fever, emesis and diarrhea.
Over the past 2 weeks, the infant has been too tired to finish
its feedings, and the infant’s hands and feet have become
cool when crying.
Past Medical History
Term infant, discharged with mother after birth. Immuni-
zations are up to date. The infant has been followed by the
primary care provider and has gained weight appropriately.
Family History
Non-contributory. Lives with healthy parents and two older
half-siblings with no significant medical problems.
Current Status
On arrival to the ED, the infant is noted to be grunting, and
is bradypneic, lethargic and responding to pain by opening
eyes only. The infant is mottled and gray with very weak
femoral pulses. Her liver is palpable 2 cm below the right
costal margin.
Vital signs on arrival are temperature 35 C (95 F), heart
rate 150 bpm, blood pressure 35/25 mmHg, respiratory rate
22 breaths per minute, weight 4.8 kg, and oxygen saturation
100% on room air.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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407
Diagnostic studies and interventions are indicated emer-
gently. This critically ill infant is provided with high-flow
oxygen immediately and a basic metabolic panel, complete
blood count, arterial blood gases, and lactate level are
obtained when intravenous access is gained. A chest radio-
graph is also obtained (Figure 97.1). The results for basic
metabolic panel, complete blood count, and arterial blood
gas analysis are shown in Tables 97.1–97.3.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this infant?
2 What additional diagnostic study is needed to confirm
your suspected diagnoses?
3 What is the best way to support cardiac function and
increase cardiac output in this infant?
4 How would you determine if the therapy was effective?
5 What other therapies would be recommended at
this time?
6 Which additional diagnostic studies and consults are
needed?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for this
infant?
The differential diagnoses would include cardiomyopathy,
myocarditis, structural heart disease, sepsis, inborn error of
metabolism, and liver failure. The most likely diagnosis for
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408 Cases in Pediatric Acute Care

this infant is cardiomyopathy due to the child’s history, pre-

senting symptoms, and initial imaging.
Cardiomyopathy is defined as an abnormality of the ven-
tricular myocardium that cannot be explained by congeni-
tal heart disease or abnormal loading conditions.
Presentation can be highly variable, ranging from an inci-
dental finding to cardiogenic shock. Cardiogenic shock is a
medical emergency requiring prompt recognition, therapy,
and hospitalization. Management of cardiomyopathy with
cardiogenic shock involves medical therapies to treat the
acutely decompensated heart failure, including potential
mechanical support and/or heart transplantation. Subse-
quent therapy aims to reverse the chronic effects of ventric-
ular remodeling.
There are two main morphologic types of pediatric cardi-
omyopathy: hypertrophic and dilated. Other less common
types include restrictive, non-compaction, mixed, arrhyth-
Figure 97.1 Chest radiograph.
mia-induced, pacemaker induced, and post-exposure to
Table 97.1 Basic metabolic profile. drugs or toxins, including chemotherapy. Morbidity and
mortality are high: only about 20% of children with dilated
Sodium 130 mEq/L cardiomyopathy regain normal function within 2 years,
Potassium 7.8 mEq/L and 40% undergo transplantation or die. Infants with
Chloride 103 mEq/L hypertrophic cardiomyopathy have a 2-year mortality rate
Carbon dioxide 11 mEq/L
of 30%. Cardiomyopathy may present at any age; in infants,
the presentation is often similar to this case. Infants typi-
Blood urea nitrogen 12 mg/dL
cally compensate for heart failure for some time before
Creatinine 0.5 mg/dL
their condition requires emergent treatment.
Glucose 88 mg/dL
What additional diagnostic study is needed to
Table 97.2 Complete blood count. confirm your suspected diagnoses?

White blood cell count 12 000/mm3 Cultures and viral polymerase chain reaction (PCR) were
Hemoglobin 9.9 g/dL obtained and empiric antibiotics are started. An echocardi-
ogram is necessary based on the history, presentation, and
Hematocrit 34.8%
chest radiograph findings. Pediatric cardiology performed a
Platelet count 203 000/mm3
bedside echocardiogram that demonstrated a structurally
Neutrophils 25% normal heart with decreased biventricular function, ven-
Eosinophils 7% tricular hypertrophy, and moderate regurgitation across
Monocytes 18% multiple valves. The ejection fraction was assessed to be
Lymphocytes 41% severely decreased at 14%.
Bands 2%
Metamyelocytes 7% What is the best way to support cardiac function
and increase cardiac output in this infant?

Table 97.3 Arterial blood gas analysis. In addition to increasing cardiac output, the immediate sta-
bilization strategy also includes decreasing the workload of
pH 6.6 the heart. This will include intubation and mechanical ven-
PCO2 75 mmHg tilation, oxygen, and inotropic support aimed at increasing
PaO2 74 mmHg cardiac output, improving diastolic filling, and decreasing
Bicarbonate 8 mEq/L ventricular workload (i.e. afterload reduction). This infant
Base excess –30 was started on milrinone and epinephrine infusions. Milri-
none is a phosphodiesterase inhibitor that results in
Lactate 18 mmol/L
increased inotropy (improved contractility and stroke

volume), lusitropy (improved diastolic relaxation and
decreased diastolic pressure to allow for improved filling)
and vasodilation (afterload reduction). Low-dose epineph-
rine is inotropic, as well as a modest vasodilator.
How would you determine if the therapy
was effective?
Frequent invasive and non-invasive hemodynamic moni-
toring is indicated. Normalizing heart rate, increased blood
pressure and pulse pressure, and improvement in meta-
bolic acidosis would be key markers of efficacy.
What other therapies would be recommended
at this time?
Beta-blockade (if hemodynamically tolerated) may reduce
outflow tract obstruction and allow the heart more time
to fill and empty. Diuretics may be used if there is clinical
volume overload. If medical therapy is insufficient,
mechanical support may be needed. Options could include
veno-arterial extracorporeal membrane oxygenation or a
ventricular assist device, which is a pneumatically driven
device that can be used in infants (e.g. Berlin heart).
Further Reading
Lee, T., Hsu, D., Kantor, P., et al. (2017). Pediatric
cardiomyopathies. Circulation Research 121(7): 855–873.
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Chapter 97 Fussy, Tired Infant 409
Which additional diagnostic studies and consults
are needed?
A 12-lead electrocardiogram (EKG) is indicated and
multiple abnormalities may be present, including biventri-
cular hypertrophy, arrhythmias, and first-degree atrioven-
tricular block. Infants with hypertrophic cardiomyopathy
should be evaluated for inborn errors of metabolism or
the infantile form of Pompe disease. In the case of Pompe
disease, the 12-lead EKG will show very large QRS voltages
as well as a very short PR interval. This infant should have a
genetics consultation. Additionally, this infant presented
with severe liver dysfunction due to acute heart failure
and cardiogenic shock; an abdominal ultrasound with Dop-
pler flow would be recommended to ensure there was no
thrombus from the low-flow state resulting in liver
dysfunction.
Case Resolution
This infant ultimately recovered her end-organ function
while being supported with a ventricular assist device while
awaiting heart transplantation.
Park, M.K. (ed.) (2014). Park’s Pediatric Cardiology for
Practitioners. Philadelphia, PA: Elsevier Saunders.
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411

98

Tics and Something More

Judy Adelizzi-DeLany
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA

A 6-year 7-month-old male with mild to moderate severity Past Surgical History
simple tics diagnosed at age 3.5 years is followed every
10–12 months in neurology clinic. He presented to the Aside from circumcision at birth, there is no past surgical
emergency department (ED) yesterday after 2–3 weeks of history.
worsening tics with headaches, left eye pain, and an event
this morning described as his “gaze stuck up and to the left”
with right eye drifting medially and right arm weakness. Family History

Mother is positive for gestational diabetes and has factor

History of Present Illness
For the last 3 weeks this child has been noted to have wor-
sening photophobia, left eye pain, and head pain in the left
occipital area. When he originally presented to neurology
clinic, he had complaints of left eye motor tics and head-
ache which resolved by the second visit at age 5 years
9 months. His tics have “come and gone” by parental
report. He recently requested “needing sunglasses” both
at school and at home for bright lights. On the morning
of his presentation to the ED, he was unable to hold his
spoon when eating his breakfast, and parents were
concerned.
V Leiden deficiency. History of migraine in mother and
maternal grandmother, hypothyroidism in paternal grand-
mother, and Tourette syndrome in paternal aunt and half-
brother (same biological father). Father has obsessive com-
pulsive disorder and global mental health issues (bipolar);
he also has mild hand tremors related to his medication.
Current Status
In the ED, the patient is noted as an alert, mildly hyperac-
tive, slender male standing without difficulty with obvious
left eye motor tic consisting of rhythmic opening and clos-
ing. All systems are reported negative, except for left eye.
Headache and photophobia are present. Skin is positive
for hyperpigmentation between shoulder blades.

Past Medical History
This patient was born at 37 weeks’ gestation by planned
cesarean section due to maternal placenta previa and ges-
tational diabetes. His birthweight was 2.78 kg. He went
home with mother 4 days later with no need for any oxygen
or respiratory support and no antibiotics or phototherapy.
He fed well and was bottle-fed regular infant formula. He
had reported normal development without any evidence
of developmental delay.
• Physical examination: temperature 37.1 C (98.8 F), heart
rate 86 bpm, blood pressure 95/46 mmHg, and respira-
tory rate 24 breaths per minute. Height is 1.2 m (22%
for age and gender), weight 21 kg (35% for age and gen-
der), and body mass index (BMI) 15.74.
• Ophthamologic examination: conjunctivae and sclera are
clear with equal pupils, left eye with constant tic, fundo-
scopic examination is normal.
• Musculoskeletal examination: No defects are noted, gait
is appropriate without ataxia. Motor: normal strength,
muscle mass, and tone in all extremities.

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

412 Cases in Pediatric Acute Care
• Neurologic examination: alert and awake, cognition,
communication and socialization appropriate for age,
happy and contented child. Cranial nerves as follows:
III, IV and VI, all extraocular movements are intact; V,
facial sensation normal and symmetrical; VII, facial con-
tours and strength symmetrical; VIII, vibratory stimulus
response normal bilaterally; IX and X, uvula midline
with normal soft palate movement; XI, neck rotation
against resistance showed normal sternocleidomastoid
strength bilaterally; XII, tongue protrusion midline, no
fasciculation noted. Sensory: normal to light touch,
reflexes 2+ bilaterally.
• Developmental examination: normal for age, no cogni-
tive or motor delay identified.
• Audiologic examination: hearing intact.
Questions
Answer the following questions using the details provided.
1 Given the history and new-onset photophobia, are there
any initial diagnostic studies that might be useful?
Continuation of case: The routine video electroencepha-
logram (EEG) was normal in wakefulness. There were no
focal or epileptiform abnormalities. Numerous typical
patient events were recorded characterized by frequent
left/right eye twitching or winking movements, left-sided
facial scrunching, upward eye deviation, eye deviation to
the right side followed by downward movement, and eye
deviation to left side. These events were not seizures but
were consistent with motor/ocular tics.
Computed tomography (CT) of the head/brain revealed
no acute intracranial abnormality on non-contrast study,
but a Chiari I malformation with marked reduction of
the cerebrospinal fluid (CSF) was observed at the craniocer-
vical junction.
2 What are the next steps given these unexpected
findings?
Rationale and Evidence-based Practice
Explanation
Given the history and new-onset photophobia, are
there any initial diagnostic studies that might be
useful?
Tic evaluation and work-up is primarily by symptom report
and observation. A sudden and dramatic increase in photo-
phobia accompanied by headache can be explained by a
virus; alternatively, worsening tics causing eye muscle pain
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can exacerbate headache. The morning occurrence of right
arm weakness is a red flag. The best initial approach from
the ED perspective is to order a CT scan to rule out any
structural reason for this event. A second choice, or in addi-
tion to a CT scan, is to obtain an EEG to rule out brain elec-
trical abnormality as the event is suspicious for a possible
seizure given the parental report of abnormal eye and
arm movement.
What are next steps given these unexpected
findings?
Admit the patient and schedule a sedated magnetic reso-
nance imaging (MRI) scan to obtain the best picture of
the Chiari malformation and consult neurosurgery for eval-
uation of the Chiari malformation.
As for the CT scan, MRI reveals the Chiari I malformation
with marked reduction of CSF at the craniocervical junction,
with crowding and/or compression of structures in this
region. There does appear to be edema within the upper cer-
vical cord at C2 at the level of crowding/compression. Par-
tially empty sella is noted, which is non-specific but could
suggest increased intracranial pressure.
Neurosurgery was consulted, and given overall baseline
normal neurologic and general examinations, with the
exception of eye tic, patient was discharged to follow-up
within the week for a scheduled MRI of the cervicothoraco-
lumbar junction to rule out syrinx.
Chiari malformation I is a condition in which brain tis-
sue extends into the spinal canal. It is a structural defect
in the cerebellum, with displacement of one or both cer-
ebellar tonsils through the opening in the base of the
skull known as the foramen magnum. This condition
occurs in about 1% of the pediatric population and
patients typically present in adolescence or early adult-
hood, although the problem is often found incidentally
with imaging at younger ages. The pathogenesis of Chiari
I malformation is poorly understood, but it is a heteroge-
neous condition and there is no other causative pathol-
ogy causing the cerebellar herniation. Congestion at
the foramen magnum and posterior fossa can cause dis-
ruption to flow of CSF that can result in increased intra-
cranial pressure. The defect may also be present with
syrinx (or cyst/cavity) in the spinal canal. There are cur-
rently no specific guidelines in the treatment or decision
for surgery, but most children do eventually have a sur-
gical procedure, especially if increased cranial pressure is
present. In this case, tics were unrelated to the findings of
the Chiari malformation I.
Headache is the most common symptom of Chiari
I malformation, although the frequency and patterns are
not consistent, especially in children. The headache is

typically described as suboccipital and occurring with Val-
salva maneuvers such as coughing or straining (Piper et al.,
2019). Other symptoms include limb weakness and symp-
toms of lower cranial nerve palsy such as dysphagia and
dysphonia. Other findings include non-radicular pain, limb
paresthesia, dissociative loss of pain and temperature sen-
sation, and spasticity.
Around 25% of children have transient tics, while
0.03–1% have Tourette syndrome. Tics typically begin
between 4 and 8 years of age, most before the teenage years.
The male/female ratio is 4 : 1. Tic symptoms fluctuate, wax-
ing and waning over time. Tic severity may peak between
10 and 12 years of age. There may be a genetic component
Reference
Piper, R.J., Pike, M., Harrington, R., and Magdum, S.A. (2019).
Chiari malformations: principles of diagnosis and
Further Reading
Piper, R.J. and Magdum, S.A. (2019). Chiari I malformation
and raised intracranial pressure. Child’s Nervous System
35(10): 1719–1725. doi: 10.1007/s00381-019-04232-x.
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Chapter 98 Tics and Something More 413
to tics but no single gene or genes have been identified.
Diagnosis of tics or Tourette syndrome is made on history
and clinical examination. Features that should be docu-
mented include onset, progression (e.g. motor tics may
manifest in the head and face then migrate distally), fre-
quency, severity or impairment, and family history. The fre-
quency and description of what is witnessed or occurred
during the clinic visit should also be documented. Transient
tics have been present for 4 weeks up to a year. Chronic tics
present for more than a year. Tourette syndrome is a neu-
rodevelopmental disorder characterized by spontaneous
involuntary movements and vocalizations that occur for
at least a year.
management. British Medical Journal 365: l1159. doi:
10.1136/bmj.l1159.
Toldo, I., Tangari, M., Mardari, R., et al. (2014). Headache
in children with Chiari I malformation. Headache 54(5):
899–908. doi: 10.1111/head.12341.

99
Lightheaded Adolescent
Cathy Woodward
Long School of Medicine, University of Texas Health, San Antonio, TX, USA
A 15-year-old female transferred by emergency medical
services to the emergency department (ED) complains of
lightheadedness and near syncope after standing to move
between classes at school.
History of Present Illness
The lightheadedness began within a few moments of stand-
ing from a desk and taking a few steps. A friend helped her
to the floor. When she got up to go to the school nurse’s
office, her lightheadedness worsened and says she “almost
fainted.” Associated symptoms are a mild headache today
and feeling tired most days for over 6 months. She reports
she used to be more physically active, but now does not
have the energy for exercise. She has had this same light-
headedness at least a few times a week for several months
and they are more frequent around the time of her
menstruation.
Past Medical History
The patient has not had any surgeries or trauma requiring
ED or medical visits. She has no chronic medical illnesses.
She has seen her primary care provider twice in the past
6 months for similar symptoms and was initially told she
was dehydrated from not drinking enough water. During
the second medical visit her mother was advised to seek
psychotherapy for a possible anxiety disorder. Her immuni-
zations are up to date for age along with annual influenza
vaccines. She has a weight at the 60th percentile for gender
and age. She takes no medication or vitamins, denies sexual
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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415
activity, and her periods are normal. She reports she is due
to start menstruation any day.
Family History
Her parents are alive and well with no chronic disease. Both
siblings, a 16-year-old old sister and 12-year-old brother,
are healthy with no medical issues. Maternal grandmother
has type 2 diabetes. Fraternal grandparents both have
hypertension.
Current Status
On arrival to the ED she was awake and alert with no acute
distress. She reports a mild posterior headache with almost
complete resolution of her lightheadedness. Her physical
examination is normal. Her initial vital signs include tem-
perature 37 C (98.6 F), heart rate 92 bpm, blood pressure
108/66 mmHg, and oxygen saturation 99% on room air.
She denies chest pain and shortness of breath. The monitor
demonstrates normal sinus rhythm and her 12-lead electro-
cardiogram (EKG) is read as normal. A pregnancy test is
negative.
A basic metabolic profile is obtained to evaluate for elec-
trolyte disturbances and dehydration. A complete blood
count is obtained to evaluate for anemia and signs of infec-
tion. Orthostatic blood pressure measurements are
obtained to evaluate for change in blood pressure in differ-
ent positions. The results of these studies are shown in
Tables 99.1–99.3.
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416 Cases in Pediatric Acute Care

Table 99.1 Basic metabolic profile. Table 99.3 Orthostatic vital signs.

Sodium 141 mEq/L Position Vital signs

Potassium 4.1 mEq/L
Chloride 101 mEq/L Lying down Blood pressure 102/60 mmHg
Heart rate 90 bpm
Carbon dioxide 24 mEq/L
Sitting up with legs dangling Blood pressure 106/62 mmHg
Blood urea nitrogen 12 mg/dL Heart rate 96 bpm
Creatinine 0.6 mg/dL Standing Blood pressure 104/58 mmHg
Heart rate 135 bpm
Glucose 92 mg/dL

Table 99.2 Complete blood count.
White blood cell count 10 000/mm3
Hemoglobin 14 g/dL
Hematocrit 42%
Platelet count 346 000/mm3
After standing, she complains of lightheadedness and
nausea. There is no change in the cardiac rhythm, other
than rate, during the procedure.
Questions
Answer the following questions using the details provided.
1 Based on the information given, what diagnoses are
included in your differential diagnosis?
2 Based on the data, what is the most likely diagnosis for
this adolescent?
3 What other therapies or diagnostic studies would be
recommended at this time?
4 What treatment can be started after diagnosis is
confirmed?
Rationale and Evidence-based Practice
Explanation
Based on the information given, what diagnoses
are included in your differential diagnosis?
The differential diagnoses include orthostatic hypotension,
dehydration, cardiac dysrhythmia, postural tachycardia
syndrome, hyperventilation syndrome, hypoglycemia, anx-
iety disorder.
Based on the data, what is the most likely
diagnosis for this adolescent?
The most likely diagnosis is postural tachycardia syndrome
(POTS). POTS is characterized by orthostatic tachycardia
without associated hypotension intermittently for at least
6 months. It is more common in female adolescents, with
a median onset of symptoms of 13 years of age. The phys-
iology of POTS is understood but the etiology remains
unknown. The pathophysiology of POTS is associated with
a change in vascular tone when standing, with chronotro-
pic sympathetic stimulation and increased heart rate and
resultant impaired cerebral perfusion initiating lighthead-
edness and occasionally syncope.
What other therapies or diagnostic studies would
be recommended at this time?
A formal head-up tilt table test and consideration of Holter
monitor to exclude cardiac dysrhythmia. Plasma norepi-
nephrine levels in supine and standing positions may aid
in the diagnosis. Supine norepinephrine levels can be
high-normal in POTS patients, but will usually be elevated
when upright.
What treatment can be started after diagnosis
is confirmed?
Therapy should be multimodal and include both non-
pharmacologic approaches and possibly medications. Ini-
tially, good sleep hygiene with regular bedtimes and elim-
ination of electronic devices at night must be ensured.
A formal exercise regimen has been shown to reduce ortho-
static tachycardia and improve quality of life. There are no
formal guidelines for pharmacotherapy of these patients,
with only case reports and anecdotal accounts to support
the use of medication for the desired response of maintain-
ing vascular tone and heart rate and decreasing sympa-
thetic response. Hypovolemia may be associated with
POTS and increasing fluid administration during the day
should be advised. Patient education should encourage
avoidance of aggravating factors like heat and dehydration,
and promote the use of support hose (30–40 mmHg pres-
sure). A referral to a cardiologist or center for POTS patients
may be warranted if non-pharmacologic approaches are
unsuccessful.
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417
Chapter 99 Lightheaded Adolescent

Raj, S. (2013). Postural tachycardia syndrome (POTS).

Circulation 127: 2336–2342.
Boris, J. (2018). Postural orthostatic tachycardia syndrome in
children and adolescents. Autonomic Neuroscience: Basic
and Clinical 215: 97–101.
Further Reading
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419

100

Infant with Hyponatremia

Sarah Pihl
Children’s Hospital of Colorado, Aurora, CO, USA

A 5-month-old female with no prior medical history is Family History

admitted to the pediatric intensive care unit (PICU) with
new-onset seizures. No significant family history. No history of seizures.

History of Present Illness Current Status

The patient was in her usual state of health until the day
prior to presentation when she developed increased
malaise. There are no sick contacts. She had adequate oral
intake and no change in the number of wet diapers. Her
parents report she had a “shaking episode” and her lips
turned blue. Emergency medical services were called and
on arrival patient was no longer having signs of seizure
activity. She was transported to the emergency department
(ED) for evaluation. Laboratory studies were obtained and
were notable for a sodium level of 117 mEq/L.
Past Medical History
She was born full term via vaginal delivery and discharged
home on day of life 2 her with parents. Weight checks at the
primary care provider (PCP) office indicate appropriate
weight gain. She is at the 55th percentile for weight and
the 60th percentile for height. Her mother recently stopped
breastfeeding due to decreased supply after returning to
work. Milestones are developmentally appropriate and
include a social smile, rolling onto back, and bringing
hands to mouth. Vaccinations are up to date for her age.
Past Surgical History
None.
On arrival at the PICU she has a generalized tonic–clonic
seizure lasting 35 seconds with apnea and hypoxia, associ-
ated with desaturation to 74% on room air. Lorazepam
0.1 mg/kg is given to treat the seizure and a nasal cannula
is placed. Her physical examination is notable for an ante-
rior fontanelle that is soft, flat and open. Pupils are brisk
bilaterally. General hypotonia is noted. Plasma osmolality
study is sent. Review of history included a feeding history.
On review of the formula mixing technique, it was discov-
ered that during preparation a low amount of formula pow-
der was used for the amount of water that which it
was mixed.
The results of a basic metabolic profile are shown in
Table 100.1.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What is the best way to treat her seizures?
3 What is the best way to correct this electrolyte
derangement?
4 How would you order this intervention?
5 How would you prevent future recurrence of hypona-
tremia in this infant?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

420 Cases in Pediatric Acute Care
Table 100.1 Basic metabolic profile.
Sodium 117 mEq/L
Potassium 4.1 mEq/L
Chloride 94 mEq/L
Carbon dioxide 24 mEq/L
Blood urea 12 mg/dL
nitrogen
Creatinine 0.8 mg/dL
Glucose 81 mg/dL
Serum 289 mosmol/kg (normal range 285–295
osmolality mosmol/kg)
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
This child has hyponatremia, defined as a serum sodium
less than 135 mEq/L; this child’s serum sodium is
117 mEq/L. Differential diagnoses of hyponatremia include
syndrome of inapproriate antidiuretic hormone (SIADH)
secretion, glucocorticoid deficiency, hypothyroidism, water
intoxication, and fluid loss such as gastrointestinal or renal
losses. The most likely diagnosis for this infant is water
intoxication from incorrectly mixed formula. The acute
presentation of low serum sodium with a normal plasma
osmolality supports this conclusion.
What is the best way to treat her seizures?
Seizures caused by hyponatremia should be treated emer-
gently with hypertonic saline, which corrects the electrolyte
abnormality. This type of seizure is not as responsive to
antiepileptic medications as other etiologies of seizure
activity. The administration of hypertonic saline results
in a small increase in serum sodium and decreases the risk
of additional seizures. It is estimated that each 1 mL/kg of
3% hypertonic saline increases serum sodium by 1 mEq/L.
As an example, administration of 5 mL/kg of 3% saline to a
child with a serum sodium of 120 mEq/L would result in an
estimated increase in serum sodium to 125 mEq/L.
The clinical effects of hyponatremia involve the regula-
tion of the blood–brain barrier to allow fluid as free water
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to cross when the osmolarity of extracellular and intracel-
lular fluid is not equal. Sodium is the largest determinant of
osmolarity, so in acute hyponatremia the change in osmo-
larity causes increased fluid volume in the brain, known as
cerebral edema. Other factors, such as hypoxia, can also
contribute to the incidence of seizures in the presence of
hyponatremia and can worsen clinical outcomes in hypo-
natremic encephalopathy as the active transport of sodium
impairs the brain’s adaptive response. Hypoxia can result
from a seizure or can be an effect of hyponatremia.
What is the best way to correct this electrolyte
derangement?
Care must be taken to slowly correct hyponatremia due to
the risk of central pontine myelinolysis. Rapid correction of
serum sodium can result in the loss of some of the myelin
sheath of neural cells and significant neurologic symptoms
including spastic quadriparesis. If seizure activity is pres-
ent, increasing the initial sodium by 5 mEq/L is appropriate
in order to decrease the risk of additional seizures. Serial
serum sodium measurements must be obtained to ensure
correction does not exceed an increase of greater than
10 mEq/L in a 24-hour period. Hyponatremia due to excess
water intake resulting from incorrectly mixed formula is
not uncommon, so it is important to educate parents on
the proper mixing of powder or concentrated formula with
appropriate volumes of water, thus eliminating this source
of free water. Infants are at a much higher risk of hypona-
tremic seizures due to the higher brain to intracranial vol-
ume ratio and immature kidney function, which can
prevent water excretion at adequate levels when serum
sodium is low.
How would you order this intervention?
Use of oral hydration is recommended with mild hypona-
tremia caused by excess water ingestion. This infant has
severe hyponatremia and careful isotonic fluid manage-
ment with measurement of serum sodium levels every
2 hours will allow careful correction of sodium levels.
How would you prevent future recurrence
of hyponatremia in this infant?
Educating the caregivers on proper formula preparation is
key to preventing recurrence. Providing this information in
a non-judgmental manner is essential. Engagement of a
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Chapter 100 Infant with Hyponatremia 421

dietitian can also be helpful. A social work consultation and A detailed neurologic examination will be needed prior to
connection with community resources may be needed to discharge as well as developmental milestone monitoring
ensure the family has access to adequate formula/nutrition. by the PCP.

Further Reading
Greenbaum, L. (2019). Sodium. In: Nelson Textbook of Reid-Adam, J. (2013). Hyponatremia. Pediatrics in Review
Pediatrics, 21st edn (ed. R.M. Kliegman, J.W. St Geme, N.J. 34(9): 417–419.
Blum, S.S. Shah, R.C. Tasker, K.M. Wilson, and R.E.
Behrman), 389–425. Philadelphia, PA: Elsevier Saunders.
 Downloaded from https://onlinelibrary.wiley.com/doi/ by National Institute Of Standard, Wiley Online Library on [06/03/2024]. See the Terms and Conditions (https://onlinelibrary.wiley.com/terms-and-conditions) on Wiley Online Library for rules of use; OA articles are governed by the applicable Creative Commons License
423

101

Bone Marrow Transplant in Sickle Cell Disease

J. Paige Rieckhoff
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA

A 17-year-old female who has undergone bone marrow managed with chronic exchange transfusion. In addition,
transplant (BMT) for sickle cell disease (SCD) presents with she suffers from chronic pain, liver disease due to iron over-
fever and 3 days of cough, rhinorrhea, and wheezing. load from chronic transfusion therapy, obesity, and asthma.
Her immunizations are up to date for age.

History of Present Illness

A 17-year-old female with history of SCD was admitted to

Past Surgical History
the hematology/oncology unit in preparation for a haploi-
She had a tonsillectomy and adenoidectomy at 12 years of
dentical 4/8 match BMT. A preparative regimen including
age for obstructive sleep apnea and a left total hip replace-
thymoglobulin, fludarabine, cyclophosphamide, and thio-
ment at 15 years of age due to damage secondary to avascu-
tepa was administered. The donor, the adolescent’s mother,
lar necrosis.
is a 48-year-old female. A regimen to prevent graft-versus-
host disease (GVHD) including cyclophosphamide on days
+3 and +4 following BMT, sirolimus titrated with a goal
serum trough level of 5–15 ng/mL, and mycophenolate Family History
mofetil 15 mg/kg by mouth three times daily from days
+5 to +35 was administered. She engrafted neutrophils Her brother also has SCD. Her mother has sickle cell trait,
on day +16. On day +21, an erythematous maculopapular hypertension and asthma. Her father’s history is unknown.
rash was noted on her bilateral forearms with associated
erythema of the palms and soles. Topical triamcinolone
cream was prescribed twice daily and the rash resolved.
She was discharged to home on day +23. She presents to Current Status
the emergency department on day +37 post transplant
for fever with 3 days of cough, rhinorrhea, and wheezing. On day +39, an erythematous, pruritic, maculopapular
She is positive for adenovirus on a respiratory virus panel. rash is noted over her face and neck. She is additionally
Serum adenovirus testing reveals an initial level of noted to have palmar and plantar erythema.
11 800 copies/mL. Intravenous cidofovir 5 mg/kg weekly
is initiated.

Past Medical History
The adolescent’s SCD has been complicated by multiple
episodes of acute pain crisis, acute chest syndrome, dacty-
litis, and avascular necrosis. Prior to BMT, her SCD was
Questions
Answer the following questions using the details provided.
1 What differential diagnoses would you consider?
2 Based on her history, what factors increase this adoles-
cent’s risk of developing this condition?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

424 Cases in Pediatric Acute Care
Rationale and Evidence-based Practice
Explanation
What differential diagnoses would you consider?
Conditions to consider for this child include GVHD, drug
eruption, and viral exanthem. With her history of BMT,
GVHD is the most likely diagnosis.
Based on her history, what factors increase this
adolescent’s risk of developing this condition?
In this case, human leukocyte antigen (HLA) disparity,
increased donor parity, and the advanced age of her donor
add increased risk for developing acute GVHD. Other risk
factors include sex mismatching, peripheral blood stem
cells as a source of donor cells, and pre-transplant total
body irradiation.
Additional Questions and Evidence-based
Rationale
3 What three organ systems are targeted by acute GVHD
and what are the manifestations of GVHD in
each organ?
4 How would you grade this child’s GVHD?
5 What treatment is recommended for GVHD?
6 What are your differential diagnoses for her new-onset
diarrhea?
7 How would you grade her GVHD?
8 What is first-line medication management for grade II–
IV GVHD including dose?
9 What side effects of high-dose steroid therapy should be
monitored for?
What three organ systems are targeted by acute
GVHD and what are the manifestations of GVHD in
each organ?
Skin is the most common organ involved, affecting 75% of
patients with acute GVHD. Manifests as a maculopapular
rash or generalized erythema, which can be pruritic or
painful. It usually involves the palms and soles. Gastroin-
testinal GVHD is the second most common form, affecting
50% of patients with acute GVHD. Upper gastrointestinal
GVHD manifests as anorexia, nausea, and dyspepsia.
Lower gastrointestinal manifestations include profuse
watery diarrhea and crampy abdominal pain. Hepatic
GVHD affects only about 20% of patients with acute GHVD
and is manifested as hyperbilirubinemia with jaundice.
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How would you grade this child’s GVHD?
Using the rule of 9s, her skin GVHD is estimated at approx-
imately 10% (3.5% for her face, 1.5% each for hands and
feet). She has no symptoms of gastrointestinal or hepatic
GVHD. Disease is staged as follows: skin, 1; gastrointesti-
nal, 0; liver, 0; overall grade, 1.
What treatment is recommended for GVHD?
Corticosteroid therapy is recommended as first line for all
forms of acute GVHD. For less than 50% body surface area
without other organ involvement, the topical route alone is
sufficient.
Continuation of case: On day +43, she develops large-
volume diarrhea (1.5–2 L/day) without associated crampy
abdominal pain. A colonoscopy with biopsies is performed.
Her bilirubin is closely monitored and remains below
2 mg/dL.
What are your differential diagnoses for her
new-onset diarrhea?
With her initial presentation of fevers and positive serum and
respiratory adenovirus, an infection must be excluded as the
primary cause of diarrhea. Stomach biopsy and stool sample
were positive for adenovirus. Based on the history of trans-
plant and history of skin GVHD, the diarrhea may also be
caused by lower gastrointestinal GVHD. Although GVHD is
a clinical diagnosis, it may be confirmed by biopsy. Her biop-
sies were also positive for GVHD in the colon and stomach.
How would you grade her GVHD?
Her skin score remained at approximately 10%. She now
has diarrhea of 1.5–2 L/day with biopsy confirming gastro-
intestinal GVHD, but there are no signs of liver GVHD,
with bilirubin below 2 mg/dL. Disease is staged as follows:
skin, 1; gastrointestinal, 3; liver, 0; overall grade, 3.
What is first-line medication management for
grade II–IV GVHD including dose?
Methylprednisolone 1–2 mg/kg daily is the standard for
first-line systemic therapy. There are several approaches
regarding such regimens, many with goals of tapering ster-
oids as tolerated due to associated side effects.
What side effects of high-dose steroid therapy
should be monitored for?
High-dose steroid therapy can cause hypertension,
Cushing syndrome, adrenal axis suppression, hyperglyce-
mia, and gastrointestinal bleeding. This additional
immunosuppression also further increases the risk of infec-
tious complications.

Further Reading
Choi, S.W., Levine, J.E., and Ferrara, J.L.M. (2010).
Pathogenesis and management of graft-versus-host disease.
Immunology and Allergy Clinics of North America 30:
75–101.
Flowers, M.E.D., Inamoto, Y., Carpenter, P.A., et al. (2011).
Comparative analysis of risk factors for acute graft-versus-
host disease and for chronic graft-versus-host disease
according to National Institutes of Health consensus
criteria. Blood 117: 3214–3219.
Holtan, S.G., Pasquini, M., and Weisdorf, D.J. (2014). Acute
graft-versus-host disease: a bench-to-bedside update. Blood
124(3): 363–373.
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Chapter 101 Bone Marrow Transplant in Sickle Cell Disease 425
Jacobsohn, D.A. (2008). Acute graft-versus-host disease in
children. Bone Marrow Transplantation 41: 215–222.
Martin, P.J., Rizzo, D., Wingard, J.R., et al. (2012). First and
second-line systemic treatment of acute graft-versus-host
disease: recommendations of the American Society of Blood
and Marrow Transplantation. Biology of Blood and Marrow
Transplantation 18: 1150–1163.
Taketomo, C., Hodding, J., and Kraus, D. (2017). Pediatric
Dosage Handbook. Hudson, OH: Lexi-Comp.
Zeiser, R. and Blazar, B.R. (2017). Acute graft-versus-host
disease: biologic process, prevention, and therapy. New
England Journal of Medicine 377: 2167–2179.

102
Child with Respiratory Failure and Transaminitis
Kim Steanson1,2
1
Monroe Carell Jr. Children’s Hospital at Vanderbilt, Nashville, TN, USA
2
Vanderbilt University College of Nursing, Nashville, TN, USA
A 13-year-old previously healthy girl is admitted to the
pediatric intensive care unit (PICU) with respiratory fail-
ure, hypotension, acute kidney injury, transaminitis, and
anemia.
History of Present Illness
This girl recently returned from a trip to the Dominican
Republic with her family. She stayed with her family at
an all-inclusive resort on the beach. She was able to spend
time on the beach, playing in the ocean and in the pool at
the resort. She and her family left the resort to be involved
in a service project with their church group.
Approximately 1 week after returning to the United
States, she developed fever to 38.3 C (101 F) and body
and muscle aches. On day 2 of the illness, she developed sig-
nificant back pain and chills. She was treated at home with
acetaminophen and ibuprofen for the fever and body aches.
Her parents took her to the emergency department (ED) at
a local hospital for further care on day 3 of the illness.
In the ED, she received a diagnosis of probable flu or viral
illness. She received antiviral medication and was
instructed to take the medication for 5 days. After 5 days,
the symptoms began to worsen and abdominal pain and
headache began. Her parents decided to bring her back to
the ED and she fainted while attempting to walk to the car.
Past Medical History
She was born at term and was discharged with her mother
on day of life 2. She has received all immunizations along
with the influenza vaccine this year. She has never been hos-
pitalized or had surgery. She is active and in the 7th grade.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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427
Past Surgical History
None.
Family History
Maternal grandmother with hypertension, paternal grandfa-
ther with cardiovascular disease. Two older siblings and
mother are healthy, father currently with flu-like symptoms.
Current Status
On arrival to the local ED, she is obtunded with respiratory
distress. She receives midazolam and etomidate for intuba-
tion and 1 L of lactated Ringer’s fluid is started. She is being
transferred to the pediatric intensive care unit (PICU) at a
children’s hospital. She arrives at the PICU intubated and
sedated. A complete metabolic panel and complete blood
count are obtained and the results are shown in Tables 102.1
and 102.2. Influenza (rapid), respiratory viral panel, and
blood cultures are sent. A chest radiograph was obtained
at the outside hospital after intubation and showed
endotracheal tube at level of T1–T2 and bilateral diffuse
pulmonary edema with loss of hemidiaphragms bilaterally.
Questions
Answer the following questions using the details provided.
1 What other historical information would it be impor-
tant to obtain for this patient?
2 What are the differential diagnoses and the most likely
diagnosis for this child?
3 What therapies are necessary to treat the current labo-
ratory abnormalities?
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428 Cases in Pediatric Acute Care

Table 102.1 Basic metabolic profile. symptoms of fever, body and muscle aches, headache,
and abdominal pain, now progressed to respiratory failure,
Sodium 145 mEq/L
anemia, acute kidney injury, transaminitis, and hypoten-
Potassium 3.5 mEq/L sion. The clinical features of malaria are non-specific and
Chloride 90 mEq/L overlap significantly with those of other febrile illnesses.
Carbon dioxide 22 mEq/L The World Health Organization recommends that all
Blood urea nitrogen 82 mg/dL patients have a parasite-specific laboratory test performed
Creatinine 0.75 mg/dL to confirm the clinical impression (Figure 102.1).
Glucose 60 mg/dL
Malaria results from infection with one of the four species
of Plasmodium (P. falciparum, P. vivax, P. ovale, P. malar-
Magnesium 2.8 mg/dL
iae). The most typical means of transmission of malaria is
Aspartate aminotransferase 70 U/L
via the bite of the Anopheles mosquito. Malaria typically
Alanine aminotransferase 50 U/L presents as fever, chills, headache, fatigue, dizziness, and
Alkaline phosphatase 400 U/L malaise. In pediatric patients, fever and headache may be
Total bilirubin 0.3 mg/dL the only presenting symptoms. Initial management of
Direct bilirubin 0.5 mg/dL patients with malaria includes treating acute symptoms
and treatment of the malaria, based on the knowledge of
resistance to chloroquine in the area in which malaria
was contracted (Figure 102.2).
Table 102.2 Complete blood count.

White blood cell count 9000/mm3

What therapies are necessary to treat the current
Hemoglobin 5 g/dL laboratory abnormalities?
Hematocrit 15%
Support and correction of respiratory failure, anemia, and
Platelet count 7900/mm3
hypotension are of the highest importance. This child
was intubated at the outside hospital and will be main-
Rationale and Evidence-based Practice tained on ventilator settings to maintain appropriate oxy-
genation and ventilation. She received a liter of fluid
Explanation

What other historical information would it be

important to obtain for this patient?
Travelers to areas where malaria is endemic may need che-
moprophylaxis. The Centers for Disease Control and Pre-
vention (CDC) provides information about each
destination including the prevailing type of malaria and
the appropriate regimens based on the epidemiology of
the area. Other malaria prevention interventions are also
encouraged, including using mosquito nets and repellants.
In this situation, it is important to know where the
patient traveled and if chemoprophylaxis was prescribed
and administered appropriately.

What are the differential diagnoses and the most

likely diagnosis for this child?
Differential diagnoses for this child include malaria, den-
gue fever, hepatitis A, influenza, measles, leptospirosis,
viral illnesses, and pneumonia.
The most likely diagnosis for this child is malaria because
of the child’s history, travel to a malaria endemic area with-
out prophylaxis, laboratory findings, and presenting Figure 102.1 Rapid diagnostic test (CDC).

Figure 102.2 Malaria transmission areas. Source: Courtesy of CDC.
while being transported to correct the hypotension. Treat-
ment of anemia will be completed with a packed red blood
cell infusion. The metabolic acidosis and renal function
should also be carefully monitored.
Continuation of case: Upon further discussion, the par-
ents did not think that any prophylaxis was needed when
traveling to the Dominican Republic. She is maintained on
the ventilator in pressure-regulated, volume control mode
with the following settings: pressure support 8 cmH2O, pos-
itive end-expiratory pressure (PEEP) 5 cmH2O, rate
18 breaths per minute, and tidal volume 6 mL/kg. She is
transfused with 15 mL/kg of packed red blood cells. Dextrose
5% with 0.45% normal saline is infused at 80 mL/hour. The
infectious disease team is consulted and requests a malaria
rapid diagnostic test (RDT), malaria smear (thick and thin),
and dengue fever polymerase chain reaction (PCR), with
results as follows: malaria RDT is positive; malaria smear
shows 2.6% parasite count.
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Chapter 102 Child with Respiratory Failure and Transaminitis 429
Additional Questions and Evidence-
based Rationale
4 What additional therapies would be recommended at
this time?
5 Should other family members be treated as well?
What additional therapies would be recommended
at this time?
Malaria causes anemia due to the high parasite load,
sequestration, and hemolysis associated with hypersplen-
ism. The choice of malaria medications is based on the
infecting species of Plasmodium, possible drug resistance,
and the severity of the disease. For travel to the Dominican
Republic, where malaria is chloroquine sensitive, travelers
are usually treated with oral chloroquine. However, this
patient’s infection is considered severe because of

430 Cases in Pediatric Acute Care
significant anemia and respiratory failure, so treatment is
started with intravenous quinidine and doxycycline.
Should other family members be treated as well?
If other family members who also traveled to the Dominican
Republic are experiencing symptoms such as fever and mus-
cle and body aches, a malaria RDT and thick and thin smears
should be completed and therapy should commence. Oral
therapy with chloroquine is appropriate in this case after
travel to the Dominican Republic. Family members should
Further Reading
Ashley, E.A., Phyo, A.P., and Woodrow, C.J. (2018). Malaria.
Lancet 391(10130): 1608–1621.
Centers for Disease Control and Prevention (2018). Malaria.
https://www.cdc.gov/parasites/malaria/index.html
(accessed 28 March 2019).
Kimberlin, D.W., Brady, M.T., and Jackson, M.A. (ed.) (2018).
Malaria. In: Red Book: 2018 Report of the Committee on
Infectious Diseases, 31st edn, 527–537. Elk Grove Village, IL:
American Academy of Pediatrics Committee on Infectious
Diseases.
Mathison, B.A. and Pritt, B.S. (2017). Update on malaria
diagnostics and test utilization. Journal of Clinical
Microbiology 55(7): 2009–2017.
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be carefully monitored for signs and symptoms consistent
with malaria for up to 4 weeks after returning from travel.
Case Resolution
The patient received treatment with quinidine and doxycy-
cline and parasite counts continued to decline. She was
extubated on hospital day 3 and remained hospitalized
for 10 days prior to being discharged home.
Olivero, R.M. and Barnett, E.D. (2018). Malaria. In: Feigin and
Cherry’s Textbook of Pediatric Infectious Disease, 8th edn (ed.
J.D. Cherry, G.J. Harrison, S.L. Kaplan et al.), 2156–2178.
Philadelphia, PA: Elsevier.
Taylor, W.R.J., Hanson, J., Turner, G.D.H., White, N.J., and
Dondorp, A.M. (2012). Respiratory manifestations of
malaria. Chest 142(2): 492–505.
White, N.J. (2014). Malaria. In: Manson’s Tropical Diseases,
23rd edn (ed. J. Farrar, P.J. Hotez, T. Junghanss et al.),
532–600. London: Elsevier.
World Health Organization (2018). World malaria report 2018.
https://www.who.int/malaria/publications/world-malaria-
report-2018/en/ (accessed 28 March 2019).

103
A Serious Football Injury
Julie Resler
Riley Hospital for Children, Indianapolis, IN, USA
A 12-year-old male is admitted to the general surgery/
trauma unit for a Grade 3 splenic laceration.
History of Present Illness
This 12-year-old was playing in a football game with full
contact and padding, with helmet. He was carrying the ball
and running down the field when he sustained a hit to his
abdomen with the helmet of a defensive player. He initially
had difficulty catching his breath but was able to participate
in another couple of plays. He then developed significant
abdominal pain and nausea.
Past Medical History
The patient’s birth history includes full-term delivery and
discharge home with his mother. His height and weight
are at the 50th percentile and he recently received age
11 vaccinations and seasonal influenza vaccine. He has a
history of attention deficit hyperactivity disorder and takes
Vyvanse 20 mg daily. He has never been hospitalized.
Past Surgical History
The patient had an uncomplicated circumcision 2 days after
birth and a tonsillectomy and adenoidectomy at age 5 for
recurrent pharyngitis.
Family History
The parents of this patient are both healthy. He has a 5-
year-old brother who has a history of mild intermittent
asthma.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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431
Current Status
On arrival at the emergency department (ED), a primary
survey is completed showing an intact airway, breath
sounds clear and equal bilaterally, strong pulses in arms
and legs, Glasgow Coma Scale (GCS) score 15, and football
clothing in place with pads removed. He has left-sided
abdominal pain with inspiration and positive Kehr’s sign.
Vital signs are as follows: temperature 37.2 C (99 F), heart
rate 120 bpm, respiratory rate 18 breaths per minute, blood
pressure 86/50 mmHg, and oxygen saturation 97% on room
air. Peripheral intravenous catheter is placed and complete
blood count, comprehensive metabolic profile, amylase,
and lipase are obtained, and a urinalysis is done to assess
for blood in the urine (see Tables 103.1–103.4). A 20 mL/kg
fluid bolus of normal saline is given to the patient with
increase in blood pressure to 110/70 mmHg. A chest radi-
ograph is negative. Computed tomography (CT) scan of
the abdomen and pelvis with intravenous contrast is
obtained and indicates a Grade 3 splenic laceration
(Figure 103.1). He is admitted to the pediatric trauma serv-
ice on the general ward, placed on bedrest and is nil by
mouth with intravenous fluids running at a mainte-
nance rate.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses for this child?
2 What is the best treatment plan for this child?
3 What signs would prompt escalation of intervention for
this child and would any other imaging be needed?
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432 Cases in Pediatric Acute Care

Table 103.1 Comprehensive metabolic profile.

Sodium 140 mEq/L

Potassium 4.1 mEq/L
Chloride 100 mEq/L
Carbon dioxide 24 mEq/L
Blood urea nitrogen 14 mg/dL
Creatinine 0.6 mg/dL
Glucose 148 mg/dL
Calcium 9.5 mg/dL
Albumin 3.5 mg/dL
Total protein 5.0 g/dL
Aspartate aminotransferase 25 U/L
Alanine aminotransferase 43 U/L
Alkaline phosphatase 140 U/L
Total bilirubin 0.1 mg/dL
Magnesium 1.8 mEq/L Figure 103.1 CT of abdomen/pelvis.
Phosphorus 4.2 mg/dL

Rationale and Evidence-based Practice

Explanation
Table 103.2 Complete blood count. Based on the data given, what are the differential
3
diagnoses for this child?
White blood cell count 9500/mm
Hemoglobin 10.8 g/dL The differential diagnoses for this child include splenic lac-
Hematocrit 32.6% eration (noted on CT imaging), additional abdominal
injury/trauma, or hypotension from infectious source not
Platelet count 260 000/mm3
yet identified.
The most likely diagnosis is a splenic laceration as noted
on the CT imaging. Splenic lacerations are graded accord-
ing to a scale from the American Association for the Sur-
Table 103.3 Amylase and lipase. gery of Trauma (AAST) and range from a low grade of 1
to a high grade of 5. A grade 3 injury is defined as a subcap-
Amylase 43 U/L
sular hematoma of more than 50% of the surface area of the
Lipase 56 U/L
spleen, ruptured subcapsular or intraparenchymal hema-
toma greater than 5 cm, or parenchymal laceration more
than 3 cm in depth.

Table 103.4 Urinalysis. What is the best treatment plan for this child?
Splenic lacerations rarely require intervention beyond non-
Color Clear, yellow
surgical monitoring and management. A period of bedrest
pH 7.0
followed by limited activity is generally advised. Pulmonary
Specific gravity 1.010
toilet and judicious pain control should be initiated. Avoid-
White blood cells 0 ance of another direct blow to the abdomen is necessary to
Nitrite Negative allow reabsorption of the blood and to allow healing of the
Protein Negative spleen. Serial abdominal examinations should be per-
Glucose Negative formed. Hemoglobin and hematocrit should be checked
Bilirubin Negative until the counts have stabilized and there is no concern
for ongoing bleeding. A diet can be started early and

advanced with toleration. Many institutions have protocols
designed to determine intervals for laboratory draws and
activity restrictions. The newest guidelines, published by
the Pediatric Trauma Society, utilize the ATOMAC
(Arizona–Texas–Oklahoma–Memphis–Arkansas Consor-
tium), which consists of a group of Level I pediatric trauma
centers from across the United States who have participated
in research to improve outcomes for children who suffer
trauma injuries. The publication in 2015 offers a practice
guideline with four highlights. Guidelines include manag-
ing liver and spleen trauma based on hemodynamic stabil-
ity versus grade of injury, a period of as little as 24 hours
bedrest, and limiting transfusions to those who have hemo-
globin levels below 7 g/dL.
What signs would prompt escalation of intervention
for this child and would any other imaging be
needed?
For this patient, follow-up with the trauma surgeon may be
necessary depending on the grade of injury. Ongoing signs
of bleeding (tachycardia, hypotension not responsive to
fluid bolus or blood administration, decreasing hemoglobin
and hematocrit) would prompt one to escalate intervention.
A “blush” on the CT imaging may indicate ongoing and/or
arterial bleeding and may necessitate intervention. If the
patient is stable, interventional radiology can be utilized
Further Reading
Linnaus, M.E., Langlais, C.S., Garcia, N.M., et al. (2017).
Failure of nonoperative management of pediatric blunt liver
and spleen injuries. Journal of Trauma and Acute Care
Surgery 82(4): 672–679.
Notrica, D.M., Eubanks, J.W., Tuggle, D.W., et al. (2015).
Nonoperative management of blunt liver and spleen injury
in children: evaluation of the ATOMAC guideline using
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Chapter 103 A Serious Football Injury 433
to embolize any ongoing bleeding in the spleen. Rarely or
if the patient is unstable, surgical intervention up to and
including a splenectomy may be necessary. This is usually
only required in high-grade (Grade 4 and 5) injuries. Stud-
ies indicate that 96–98% of splenic injuries in children can
be managed with non-operative intervention.
Additional follow-up imaging is not necessary if the
patient is progressing and shows no signs of ongoing bleed-
ing. Continued radiation exposure, such as that with CT
imaging, can increase the lifetime risk of cancer in pediatric
patients.
Case Progression and Resolution
The patient is admitted to the general ward. His hemoglo-
bin and hematocrit are checked every 12 hours until stable.
He is kept on bedrest until hospital day 3 and then allowed
to ambulate. He is started on incentive spirometry while on
bedrest and is allowed a clear liquid diet on hospital day 1
and then advanced to a regular diet. He is given intravenous
opiate medication while nil by mouth and changed to
enteral opiate medication when tolerating a diet. His hemo-
globin and hematocrit remain stable. He is discharged to
home on hospital day 4.
GRADE. Journal of Trauma and Acute Care Surgery
79(4): 683–693.
Wisner, D.H., Kuppermann, N., Cooper, A., et al. (2015).
Management of children with solid organ injuries after
blunt torso trauma. Journal of Trauma and Acute Care
Surgery 79(2): 206–214.
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435

104

Infant with Generalized Weakness

Blythe Pollack
C.S. Mott Children’s Hospital, Ann Arbor, MI, USA

A 5-month-old female with no significant medical history is Past Surgical History

admitted to the pediatric intensive care unit (PICU) with
respiratory failure and generalized weakness of unclear No past surgical history.
etiology.

History of Present Illness
This 5-month-old female presented to the emergency
department (ED) with her mother due to increased work
of breathing and poor oral intake. In the hours preceding
the visit, she had poor latch with breastfeeding and
decreased intake. Bottle feeds were attempted, but unsuc-
cessful. She was taken to the urgent care in the morning
hours for concern of constipation, and sent home with a
prescription of polyethylene glycol. Hours after she
returned home, the infant had a sudden increase in her
work of breathing, audible chest congestion, weak cry,
and lethargy.
Past Medical History
She was a full-term infant, born via non-complicated vagi-
nal delivery. She did not require airway interventions at
birth. She did have coughing/choking episodes with initial
feeds, was diagnosed with reflux, and was repositioned
more upright for feedings. She had no reoccurrence of these
episodes. She was discharged home with her mother fol-
lowing mothers’ postpartum stay. No other hospital admis-
sions or urgent care/ED visits. No preceding illnesses. She is
primarily breast-fed, only taking from a bottle as needed.
She was started on fruit purees a couple of days prior to
presentation. She did not ingest honey. There is some con-
struction happening across the street from their apartment
complex. There are many farms in the surrounding area.
Family History
No significant medical history. No neuromuscular diseases.
Two older siblings, both currently well, one of which
attends public school. Both without medical disease, but
do suffer from the occasional virus.
Current Status
On presentation to the ED at an outside hospital, her vital
signs are stable and she is afebrile. Influenza and respira-
tory syncytial virus swab are both negative. Peripheral
intravenous catheter is placed and blood work obtained.
Secondary to her respiratory compromise, she is presumed
to have bronchiolitis and receives nebulized albuterol. Dur-
ing her albuterol inhalation, she desaturates to 80% but
quickly recovers. Because of her poor oral intake and gen-
eral malaise, she is admitted to the general pediatric floor
for observation and intravenous fluids.
Results of laboratory tests are shown in Tables 104.1 and
104.2. Virus studies were negative for influenza A and
B and respiratory syncytial virus
Questions
Answer the following questions using the details provided.
1 Based on the data given, formulate a differential diag-
nosis for this child?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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436 Cases in Pediatric Acute Care

Table 104.1 Basic metabolic profile. What other diagnostic studies can be completed to
help differentiate diagnosis? How can they change
Sodium 139 mEq/L management?
Potassium 5.4 mEq/L
To gain clarity on her diagnosis a multitude of additional
Chloride 108 mEq/L
diagnostic studies can be completed. Procalcitonin
Carbon dioxide 18 mEq/L
(marker of inflammation) can help differentiate from a
Blood urea nitrogen 12 mg/dL bacterial infection and better elucidate the need for anti-
Creatinine <0.3 mg/dL biotics. A chest radiograph would be helpful to evaluate
Glucose 86 mg/dL for airspace disease, aspiration, consolidation, and ate-
Calcium 10.4 mg/dL lectasis. Continued monitoring of electrolytes will lead
Alkaline phosphatase 137 U/L to supplementation or identification of disturbances.
Albumin 4.3 g/dL Laboratory values can be beneficial in the identification
of fatigue related to hyponatremia or hypoglycemia in
the setting of poor oral intake. A blood gas can be used
for trending in the setting of impending respiratory
Table 104.2 Complete blood count.
failure.
White blood cell count 13 700/mm3 Continuation of case: Within the first 12 hours of admis-
Hemoglobin 12.2 g/dL sion, she continues to have worsening weakness, proximal
Hematocrit 36.8% greater than distal, and intermittent hypoxia. A chest radi-
Platelet count 441 000/mm3 ograph is completed with concern for bilateral airspace dis-
Neutrophils 29% ease, and head computed tomography (CT) is completed
Eosinophils 1%
and interpreted as normal. High-flow nasal cannula is
initiated and she is transferred to the intensive care unit
Monocytes 8%
(ICU). Following transfer to the ICU, she has increased fre-
Lymphocytes 62%
quency of oxygen desaturations, declining mental status,
and hypotonia. Subsequently, her trachea is orally intu-
bated for respiratory support in the setting of neurologic
2 What other diagnostic studies can be completed to decompensation.
help differentiate diagnosis? How can they change
management?
Additional Questions and Evidence-
based Rationale
Rationale and Evidence-based Practice 3 What differential diagnosis are you considering with
Explanation the changes in patient status?
4 What other diagnostic studies can be completed to
Based on the data given, formulate a differential help differentiate diagnosis? How can they change
diagnosis for this child? management?
Based on the presenting symptoms of increased work of
breathing and poor oral intake, it is reasonable to consider
What differential diagnosis are you considering
a primary respiratory issue. Her poor oral intake can be
with the changes in patient status?
related to her work of breathing or other possibilities.
The differential diagnoses include viral bronchiolitis, viral The clinical decompensation in addition to hypotonia
pneumonia, or upper respiratory infection. When consider- expands the diagnosis to include, but not limited to, hypo-
ing her general malaise, constipation, and increased work thyroidism, inborn errors of metabolism, encephalitis, poli-
of breathing, her differential diagnosis should widen. Addi- omyelitis, ingestion/poisoning, neuromuscular junction
tionally, dehydration, failure to thrive, spinal muscular disorders (botulism, myasthenia gravis), demyelinating
atrophy, suspected sepsis, or meningitis should be consid- disorders (Guillain–Barré syndrome/Miller–Fisher syn-
ered. With such vague symptomology the differential drome), acute flaccid myelitis, axonal neuropathy, and
should be expanded and narrowed through the hospi- myopathy/myositis. Symptoms were consistent with bulbar
tal stay. paralysis. Referring to lower brainstem area or medulla

oblongata, control of cranial nerves VII–XII. Term bulbar
can be used for lesions at any level (muscle, neuromuscular
junction, peripheral nerves, motor nuclei).
What other diagnostic studies can be completed to
help differentiate diagnosis? How can they change
management?
Any patient with unknown disease etiology involving the
respiratory system and neurologic decompensation war-
rants a full septic work-up. Blood, urine and respiratory cul-
tures, along with a chest radiograph, should be completed
for further evaluation. Lumbar puncture should be per-
formed to evaluate for meningitis, and viral studies from
cerebrospinal fluid (CSF) should be sent for evaluation.
There are a number of adjunctive tests that can be used
to help determine the cause of a neurologic insult. EEG
should be completed to assess for seizure activity. EMG
can be completed to assess for acute flaccid paralysis or bot-
ulism but results are not always definitive and can cause
discomfort, so the benefits should be weighed. A nerve con-
duction study can typically be completed at the same time
as EMG and is used to evaluate the speed of the electrical
impulse and can eliminate the Miller–Fisher variant. The
Tensilon test, which involves the injection of edrophonium
chloride, can be sensitive for myasthenia gravis. The Tensi-
lon test is influenced by patient response and may produce
false-positive results, with diagnoses such as botulism,
Guillain–Barré syndrome, motor neuron diseases, and
brainstem lesions.
Each diagnostic study by itself is not definitive, though a
diagnosis can be made with additional information from
CSF studies, laboratory results, and clinical presentation.
Continuation of case: During her first few days of ICU
admission, she has decline in many body systems. Routine
blood work is completed, along with blood, urine and spu-
tum cultures. She requires moderate to high ventilator set-
tings, with increased atelectasis and large mucous plugs.
Cardiac echocardiogram is completed with normal func-
tion and anatomy, despite persistent hypertension. She
no longer voids independently, and requires intermittent
straight catheterization. Iatrogenic electrolyte disturbances
are corrected with initiation of continuous feeds. A second
viral swab is completed, which is positive for rhinovirus/
enterovirus. Lumbar puncture is completed twice, with
the results shown in Table 104.3. Electroencephalography
(EEG) shows stage 2 sleep, no wakefulness, and no sei-
zures. Magnetic resonance imaging (MRI) of her brain
and spine is completed without acute findings. Electromy-
ography (EMG) shows active myopathy or primarily
axonal motor neuropathy. A 48-hour course of intrave-
nous gamma globulin (IVIG) is completed without clinical
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Chapter 104 Infant with Generalized Weakness 437
Table 104.3 Initial CSF analysis.
Color Colorless
Clarity Clear
Xanthochromia Absent
Red blood cells 1/mm3
Protein 15 g/L
Glucose 126 mg/dL
change. A muscle biopsy is completed. Viral studies are
sent for evaluation, including human herpesvirus 7, cyto-
megalovirus (CMV), enterovirus, Epstein–Barr virus, her-
pes simplex virus, and West Nile virus. She is treated with
ganciclovir for 5 days for a CMV viral load above 1400
copies/mL. She receives 48 hours of vancomycin and cef-
triaxone for initial concern for sepsis which is discontin-
ued following 48 hours of negative cultures and absence
of fever. Enterovirus sent to the state laboratory for further
subtyping.
The current focused neurologic examination produces
the following results.
•
Mental status: she is encephalopathic with no spontane-
ous eye opening. She has spontaneous, but not vigorous
movements of the bilateral hand and feet, with deep
stimulation.
•
Cranial nerves: pupils are 3 mm with briskly reactive
pupil on the left, 5 mm sluggishly reactive pupil on the
right. Her facial structures appear symmetric. She has
midline tongue movement without fasciculations. No
cough or gag reflex is noted. She has weak dolls eye
and absent corneal reflexes.
• Motor: diffuse peripheral hypotonia with normal bulk
throughout. She has 2+ strength of the fingers, wrists
and elbows of the upper extremities. She has 1+ strength
in the bilateral shoulders, 2+ strength in bilateral ankles
and knees, and 1+ strength in her bilateral hips.
The patient is transferred to a children’s hospital in the
setting of unknown etiology of her neurologic and respira-
tory decompensation. On arrival to the center of higher
care, multiple services are consulted, including neurology,
infectious disease, and pulmonology. The EMG, EEG, brain
and spine MRI, and lumbar puncture are repeated. Blood
and stool specimens for botulism are sent for evaluation.
Medications are avoided which could interfere with neuro-
muscular junction functions. Antibiotics are not initiated,
as the etiology of illness did not appear infectious. BabyBIG
(human botulism immunoglobulin) is administered prior
to any results.

438 Cases in Pediatric Acute Care
The radiology repeat imaging and stool sampling produce
the following results.
• MRI: negative for intracranial mass, midline shift, extra-
axial fluid collection or hemorrhage. The ventricles, sulci
and cisterns are reported as normal. There are no abnor-
mal areas of altered signal intensity or enhancement.
There is no sign of infarction. The visualized aspects of
the orbits, paranasal sinuses, and the mastoid air cells
are normal. MRI brain and spine: susceptibility signals
in the left cerebellar hemispheres may represent a sta-
ble-appearing small cavernoma. No evidence of acute
ischemia or acute parenchymal/extraparenchymal hem-
orrhage present in the study. No convincing evidence of
spinal cord compression present. No abnormal enhance-
ment of the spinal cord. No abnormal thickening of the
cauda equina or the exiting nerve roots noted. No abnor-
mal enhancement of the structures.
• EMG: abnormal examination. There is electrodiagnostic
evidence of a severe motor axonal polyneuropathy with
active denervation, with the differential diagnoses
including acute motor axonal neuropathy (AMAN) and
anterior horn cell disorder. There is electrodiagnostic evi-
dence of a severe sensory neuropathy or a defect in neu-
romuscular junction transmission.
• EEG on day 1 of admission: normal EEG demonstrates
slowing, encephalopathic features. There is also noted
to be normal features of sleep seen intermittently as well.
The background is reactive. No seizures and no epilepti-
form features are recorded.
• EEG on day 4 of admission: abnormal tracing for age.
There is normal sleep with no wakefulness despite nox-
ious stimulation. Intermittent focal slowing is noted over
the left parietal region.
• Muscle biopsy: there is no evidence of abnormal central
nuclei, cytoplasmic inclusions, or ragged red fibers. Focal
mild endomysial inflammation is non-specific but is con-
sistent with immune reactive changes that could be due
to viral infection or an autoimmune process. Clinical cor-
relation is warranted.
••
CSF: results shown in Table 104.4.
Stool specimen: Clostridium botulinum type A confirmed
by mouse inoculation.
Table 104.4 Repeat CSF analysis.
Color Colorless
Clarity Clear
Xanthochromia Absent
Red blood cells 2/mm3
Protein 16 g/L
Glucose 73 mg/dL
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Additional Questions and Evidence-
based Rationale
5 What is the difference between an EEG and EMG? In
what situation would you order either or both studies?
6 Should the provider be concerned for a toxic ingestion
this far into her admission and what could still be eval-
uated for?
7 What is infantile botulism and how can it be prevented?
8 What is BabyBIG and how does it work?
What is the difference between an EEG and EMG?
In what situation would you order either or both
studies?
EEG is the process of attaching electrodes to the scalp to
evaluate the electrical activity of the brain. The activity is
received from the neurons on the outermost layers of the
cerebral cortex. EEG can be used to observe and identify
refractory status epilepticus, achievement of cerebral
silence, treatment monitoring of traumatic brain injury,
metabolic encephalopathies, and neurologic conditions
that inhibit patient responses. EEG continues to be a con-
venient evaluation of continuous, real-time monitoring of
brain function and is supplemental to the clinical
examination.
EMG is a test that measures electrical signals. The proc-
ess consists of inserting into a muscle a small needle that
sends an electrical signal causing the muscle to contract.
The contraction is recorded in as a number, wave or sound,
which is interpreted by a specialist. EMG can be used to
detect nerve or muscle dysfunction, or a dysfunction in
their communication process. In this case, the EMG did
not support a diagnosis of botulism.
Should the provider be concerned for a toxic
ingestion this far into her admission and what
could still be evaluated for?
With an unknown neurologic decompensation, toxic inges-
tions should be considered early and urine and/or blood
samples sent for evaluation. A week into her inpatient stay,
evaluating for several toxic ingestions will not have much
utility in reaching a diagnosis. Evaluating for alcohol is
not useful, as it would have left her system this far into
her course. Acetaminophen and salicylate levels are easily
evaluated with a serum sample but this far into the course
the levels would be decreased to non-existent. Cholinergic
toxidromes can have nicotinic effects with symptoms of
weakness, paralysis, agitation and tachycardia; agents such
as organophosphate pesticides present this way. Typical
presentation following inhalation is respiratory distress,
whereas emesis and drooling are typical clinical signs with

oral ingestion. Airway management, atropine to reverse
muscarinic effects, and benzodiazepines to control seizures
are typical management strategies. Gas chromatography–
mass spectrometry detects compounds even in small quan-
tities in the urine but hold low utility at this time. Consid-
ering further evaluation of organophosphates, lead,
cadmium, and arsenic could narrow the differential and
help direct further treatment.
What is infantile botulism and how can it be
prevented?
In the United States, 99.8% of cases of infantile botulism
have occurred in infants and children under 1-year-old,
with 91.2% found in children under 6 months old. Illness
occurs equally across ethnic and racial groups, with equal
instances in males and females. Clostridium botulinum
toxin is subclassified into types A–F, the most common
being A and B. Infantile botulism occurs when the C. bot-
ulinum toxin is ingested/inhaled and the spores enter the
digestive system. The spores will then grow and release
the toxin. The botulinum toxin binds at the presynaptic seg-
ment of the neuromuscular junction and prevents acetyl-
choline from being released into the neuromuscular
junction.
The commonly discussed causative agent of infant botu-
lism is honey, even though it is uncommon for the ingestion
of honey to be linked with reported cases of infant botulism.
Avoidance of honey in infants under 12 months of age is a
preventative strategy, but does not guarantee the develop-
ment of botulism from a different source. Clostridium bot-
ulinum is a bacterium that inhabits soil and dust and is
therefore found inside and outside the home. The distribu-
tion of confirmed cases nationally has been linked to the
same botulism gene in the soil of a particular area.
Further Reading
Carrillo-Marquez, M. (2016). Botulism. Pediatrics in Review
37(5): 183–192. doi: 10.1542/pir.2015-0018.
Centers for Disease Control and Prevention (2019). Kinds of
botulism. Available at https://www.cdc.gov/botulism/
definition.html (accessed 11 March 2019).
Choi, J. and McCarthy, M. (2017). Novel applications for serum
procalcitonin testing in clinical practice. Expert Review of
Molecular Diagnostics 18(1): 27–34. doi: 10.1080/
14737159.2018.1407244.
Griese, S., Kisselburgh, H., Bartenfeld, M., et al. (2017).
Pediatric botulism and use of equine botulinum antitoxin in
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Chapter 104 Infant with Generalized Weakness 439
What is BabyBIG and how does it work?
BabyBIG is the human botulism immunoglobulin used for
treatment of botulism. It is administered as soon as there is
suspicion of infant botulism. It is an intravenous medica-
tion that helps reverse the toxemia by neutralizing the cir-
culating toxins and preventing further disruption of the
neuromuscular junction. BabyBIG has immediate onset,
with an average half-life of 28 days. The most common
adverse reaction is skin rash, occurring in less than 5% of
patients. As with any immunoglobulin administration,
infusion should be initiated slowly with close monitoring
of vital signs for the first hour; with stability, rate of infusion
can increase. There is risk of anaphylaxis, and if this occurs
the infusion should be discontinued, followed by adminis-
tration of epinephrine. If patients have a known history of
IgA deficiency, they can develop further antibodies to IgA,
which would put them at higher risk for anaphylaxis to any
subsequent blood product transfusions.
Case Resolution
Ten days following admission to the higher level of care,
her stool sample resulted positive for botulism. She demon-
strated improvement over the next weeks as an inpatient.
She was successfully extubated 23 days following her initial
presentation and intubation. Four months following her
admission she was discharged home requiring non-invasive
positive pressure for nocturnal hypoventilation, and naso-
gastric feeds for oral aversion. She was able to tolerate sup-
ported sitting, roll from side to side, though was not yet
supporting her own weight to crawl. She continued occupa-
tional and physical therapy as an outpatient.
children: a systematic review. Clinical Infectious Diseases
66(Suppl 1): S17–S29. doi: 10.1093/cid/cix812.
Opila, T., George, A., El-Ghanem, M., and Souayah, N. (2017).
Trends in outcomes and hospitalization charges of infant
botulism in the United States: a comparative analysis
between kids’ inpatient database and national inpatient
sample. Pediatric Neurology 67: 53–58. doi: 10.1016/j.
pediatrneurol.2016.10.009.
Shaffner, D. and Nichols, D. (ed.) (2016). Rogers’ Textbook of
Pediatric Intensive Care, 5th edn. Philadelphia, PA: Wolters
Kluwer.

105
A 3-Day-Old with Irritability
Christopher D. Newman
Children’s Hospital Colorado, Aurora, CO, USA
A 3-day-old female infant is brought to the emergency
department (ED) by her mother due to irritability and ina-
bility to feed.
History of Present Illness
She was delivered at home with a birth coach via vaginal
delivery. She was initially “a bit floppy” but this resolved
after her first feed. Over the next 48 hours, she appeared
normal and active, but would become irritable, “jittery”
and sweaty as feeding time approached. These symptoms
would all resolve after feeding. Early this morning, the
infant slept for several hours and when her mother
attempted to wake her, she was pale, sweaty, listless and
tremulous. Mother attempted to feed her, but she was list-
less and would not latch or suck consistently. Her mother
reports that the infant’s “heart was racing” and after an
hour of attempting to feed without success, she became
“floppy” and unresponsive, and was brought to the ED
for assistance.
The infant’s mother denies fevers, cough, congestion, or
eye discharge. The child has not vomited nor had diarrhea.
Nobody in the family has been ill recently. She has not tra-
velled and there are no medications in the home.
Past Surgical Hisotry
No surgical history.
Family History
The family are recent immigrants to this country and the
mother admits that she does not have complete details of
family health history. The infant has two older siblings
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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441
who are both healthy. Both were born at home. Mother
reports several cousins that died in infancy, but she is not
sure about the cause of death. The mother has no health
problems, but she believes that the paternal grandfather
had “liver issues.”
Current Status
The infant is examined in her mother’s arms. She is pale,
diaphoretic and listless. Her fontanelle is mildly sunken.
Her pupils are reactive but she does not fix and follow.
Her lips are dry. She is tachypneic with a respiratory rate
of 60 breaths per minute, though her lung fields are clear
to auscultation. She is tachycardic with a heart rate of
180 bpm with a regular rhythm and no murmurs, gallops
or rubs. She has 1+ peripheral pulses and 2-second capil-
lary refill peripherally. The abdomen is soft, with the liver
edge 1 cm below the costal margin. The spleen is not pal-
pated and bowel sounds are sparse but present. There are
no rashes or extremity deformities.
You request that a peripheral intravenous catheter be
placed, laboratory samples obtained, and maintenance
intravenous fluids are started. You order a bedside blood
glucose check, complete blood count, comprehensive met-
abolic panel, blood culture, coagulation studies and procal-
citonin for initial evaluation. The nurse notifies you that the
glucometer blood glucose is 25 mg/dL.
Questions
Answer the following questions using the details provided.
1 Based on the history, clinical presentation and labora-
tory values, what is your differential diagnosis?

442 Cases in Pediatric Acute Care
2 What, if any, additional laboratory testing would
you order?
3 What, if any, treatment would you administer?
Rationale and Evidence-based Practice
Explanation
Based on the history, clinical presentation and
laboratory values, what is your differential
diagnosis?
In any neonate presenting with hypoglycemia, sepsis
should be considered. If there are any infectious symptoms,
treatment should begin immediately while an evaluation is
performed. In this case, there is no clinical evidence of
infection, so laboratory testing for evidence of sepsis is
appropriate. Hypoglycemia in infancy can generally be
divided into broad categories: inadequate glucose supply
(either through inadequate intake or due to defects in fatty
acid oxidation, carbohydrate metabolism or amino acid
metabolism) or increased glucose utilization (such as
hyperinsulinism or some ingestions). Infants will rapidly
exhaust their glycogen stores without glucose intake and
are therefore less tolerant of fasting. Additionally, illness
that increases metabolic demand can exhaust glucose
supply even in a feeding infant if the infant cannot keep
up with the increased metabolic demand. Common inges-
tions associated with hypoglycemia can include ethanol,
salicylates, sulfonylureas, and beta-blockers.
Hyperinsulinism often presents in the first few weeks of
life. The most common cause is congenital hyperinsulin-
ism, although insulinomas and exogenous insulin adminis-
tration are also possibilities. In these conditions, insulin is
secreted out of proportion to glucose level and the usual
negative feedback loop that disrupts insulin production
when blood glucose levels fall below 60 mg/dL is inacti-
vated. Fatty acid oxidation disorders can include carnitine
deficiency, fatty acid transport defects, and defects of beta-
oxidation enzymes. The most common disorder is medium-
chain acyl-CoA dehydrogenase deficiency. The exact mech-
anism of hypoglycemia is not understood, but since ketone
bodies cannot be manufactured without mobilization of
free fatty acids, the absence of ketone bodies as an energy
source may play a role. Disorders of carbohydrate metabo-
lism can impact metabolism of glycogen, galactose and
fructose. This group includes the glycogen storage diseases
(such as glycogen synthetase deficiency), disorders of glyco-
sylation (such as phosphoglucomutase 1 deficiency) and
disorders of gluconeogenesis (such as pyruvate carboxylase
deficiency). Although disorders of amino acid metabolism,
such as methylmalonic acidemia and propionic acidemia,
typically present with an episode of profound metabolic
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acidosis, hypoglycemia may be present due to associated
liver dysfunction or protein malnutrition.
What, if any, additional laboratory testing would
you order?
It is essential in diagnosing the cause of hypoglycemia that
laboratory testing be done on specimens obtained during
the state of hypoglycemia. In an unstable patient, the
patient’s safety comes first. However, ideally, the following
studies will be obtained prior to treatment of the hypogly-
cemia: plasma glucose, C-peptide, β-hydroxybutyrate, free
fatty acids, acylcarnitine profile, lactate, and ammonia.
Urine organic acids should also be sent. These laboratory
values can then be used to identify the most likely cause
of hypoglycemia from the differential diagnosis.
What, if any, treatment would you administer?
In older children who are alert enough to swallow safely,
10–20 g of rapidly absorbed glucose, such as honey, table
sugar, glucose tablets or glucose gel, may be administered.
In the case of infants such as this patient, the ideal treat-
ment is intravenous administration of dextrose 0.25 g/kg
(maximum 25 g). In an unstable patient where intravenous
access cannot be achieved, administer glucagon 0.03 mg/kg
intramuscularly. Glucagon will effectively treat hypoglyce-
mia due to increased glucose utilization, but may not treat
other causes and the effect may be transient. Efforts should
continue to establish intravenous access and administer
intravenous dextrose.
Continuation of case: The results for basic metabolic pro-
file and complete blood count and coagulation are shown in
Tables 105.1 and 105.2. Additional laboratory test results
are as follows:
•• blood culture results pending, Gram stain negative
insulin <2 μIU/mL
•• C-peptide 0.4 ng/mL
β-hydroxybutyrate 0.3 mmol/L
•• free fatty acids 0.3 mmol/L
acylcarnitine profile normal
•• lactate 4 mmol/L
ammonia 80 μg/dL
• urine organic acids normal.
Additional Questions and Evidence-
based Rationale
4 Given the laboratory values, what is the most likely
cause of this patient’s hypoglycemia?
5 How can this diagnosis be confirmed?
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Chapter 105 A 3-Day-Old with Irritability 443

Table 105.1 Basic metabolic profile. they are generally associated with facial dysmorphisms,
developmental delay, and multiorgan impacts and are gen-
Sodium 142 mEq/L
erally not seen in the neonatal period. Given the age at pres-
Potassium 5.2 mEq/L entation, association with fasting, resolution with glucose
Chloride 102 mEq/L administration, presence of lactic acid, and absence of
Carbon dioxide 16 mEq/L ketone bodies, the most likely diagnosis is a glycogen
Blood urea nitrogen 11 mg/dL storage disease. While there are over 20 identified glycogen
Creatinine 0.6 mg/dL storage diseases, only five are associated with
Calcium 2 mmol/L
hypoglycemia.
Glycogen synthetase deficiency (GSD 0) typically pre-
Phosphorus 2 mmol/L
sents with fasting ketosis, postprandial hyperglycemia,
Magnesium 1 mmol/L
and absence of hepatomegaly.
Aspartate aminotransferase 50 U/L Glucose 6-phosphatase deficiency (GSD I) is the most
Alanine aminotransferase 35 U/L common deficiency (accounting for over 80% of cases)
Alkaline phosphatase 200 U/L and has two subtypes, a and b. Typical presentation is hypo-
Total bilirubin 160 μmol/L glycemia with lactic acidemia and hepatomegaly. Mild to
Albumin 3 g/dL moderate ketosis may be present or absent. When patients
Procalcitonin 0.2 μg/L present later in life, additional findings can include growth
retardation, delayed puberty, hyperuricemia, platelet dys-
function, and hyperlipidemia. GSD Ib is similar to GSD
Table 105.2 Complete blood count and coagulation. Ia, but often includes neutropenia. This is the most likely
diagnosis for this patient. Debrancher deficiency (GSD
White blood cell count 10 000/mm3 III) may present with hypoglycemia if there is liver involve-
Hemoglobin 17 g/dL ment. Hypoglycemia is generally milder than in GSD
Hematocrit 55% I because gluconeogenesis still functions. This is a ketotic
Platelet count 310 000/mm3 hypoglycemia and ketone bodies are typically markedly ele-
Prothrombin time (PT) 12 s vated. Hepatic phosphorylase deficiency may present with
Partial thromboplastin time (PTT) 35 s
hypoglycemia, but lactic acid level is usually normal,
ketones are elevated, while transaminases are usually
International normalized ratio 1.0
mildly elevated. Hepatic phosphorylase b kinase deficiency
can present with hypoglycemia, but the disease itself is typ-
ically mild and a presentation this early would be unusual.
Given the laboratory values, what is the most
The hypoglycemia itself is usually mild and there is usually
likely cause of this patient’s hypoglycemia?
an elevated ketone level associated with the hypoglycemia.
A normal white blood cell count and a procalcitonin below
0.5 μg/L in the absence of infectious symptoms make sepsis How can this diagnosis be confirmed?
extremely unlikely. Accidental ingestion in a neonate is
also very unlikely and there are no associated agents in First-line testing is DNA assessment for known mutations.
the home. Salicylate ingestion could explain the hypoglyce- If none of the common mutations are identified, whole
mia and acidosis. If suspicion were higher, a salicylate level exome sequencing can be done to look for less common
could be obtained to exclude this diagnosis. A suppressed mutations. In the event that no mutation can be identified,
insulin level and C-peptide level can exclude hyperinsulin- a liver biopsy may be necessary to confirm the functional
ism as a cause of the hypoglycemia. Similarly, a normal free defect.
fatty acid level and normal organic acid levels can exclude
fatty acid oxidation or amino acid metabolism defects.
The resolution of symptoms following glucose adminis- Case Progression and Resolution
tration, the pattern of symptoms following fasting, the find-
ing of hepatomegaly, and the presence of lactic acidemia Following intravenous administration of dextrose
are all most consistent with a carbohydrate metabolism dis- 0.25 g/kg, the patient is more alert and active. She is less
order. Disorders of gluconeogenesis generally cause ketotic diaphoretic, but continues to be tachycardic. Her repeat
hypoglycemia. glucometer check is 82 mg/dL. Avoidance of fasting, fre-
While there are over 130 recognized glycosylation disor- quent glucose monitoring, and a genetics team consult
ders and many can include hypoglycemia at presentation, are initiated.
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444 Cases in Pediatric Acute Care

Further Reading
Bhattacharya, K. (2015). Investigation and management of the
hepatic glycogen storage diseases. Translational Pediatrics
4(3): 240–248.
Chang, I.J., He, M., and Lam, C.T. (2018). Congenital disorders
of glycosylation. Annals of Translational Medicine
6(24): 477.
Thornton, P.S., Stanley, C.A., De Leon, D.D., et al. (2015).
Recommendations from the Pediatric Endocrine Society for
evaluation and management of persistent hypoglycemia in
neonates, infants, and children. Journal of Pediatrics 167(2):
238–245.
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445

106

Child with Fever and Rash

Misty Evans
Vanderbilt College of Nursing, Nashville, TN, USA

A 7-year-old girl presents with her mother to the emergency Past Medical History
department (ED) with a 3-day history of nausea, vomiting,
stomach ache, fever, and skin rash. Born at term and went home from the hospital with her
mother. She is a healthy child with no significant past med-
ical history. She is up to date on all required vaccines along
with annual influenza vaccine. She weighs 25 kg and height
History of Present Illness is 125 cm, both at 75th percentile for gender and age. No
known allergies.
This 7-year-old girl initially became ill 2 days ago with
fever of 38.3 C (101 F), stomach ache, nausea, vomiting,
and decreased appetite. Mother reports that several chil-
Past Surgical History
dren in the child’s school were recently sick with a “stom-
ach virus.” The child was provided symptomatic care at
She has no prior surgical history.
home with oral fluids, acetaminophen for fever, and rest.
Overnight, the child’s fever increased to 39.3 C (102.7 F).
Her mother administered acetaminophen every 6 hours
and the last dose was given 2 hours ago. This morning,
Family History
a red rash was noted on her wrists and ankles. Worsening
No significant family medical history. Mother, father, and
fever and the new rash prompted her mother to bring the
9-year-old brother are alive and healthy.
child to the ED for further evaluation. The child also com-
plains of intermittent headache and generalized body
aches which are relieved with the acetaminophen given
for fever. She reports abdominal pain with no associated Social History
factors that make the pain better or worse. She vomited
three times yesterday and once this morning. Emesis is She is a first-grade student and lives in a house with her
not bloody or bilious. She is not eating or drinking as usual mother, father, and 9-year-old brother. There is no tobacco
and is having decreased urinary output. She has had reg- exposure in the home. They have one dog in the home. No
ular soft bowel movements with no diarrhea or constipa- other pets.
tion. She has had no sick contacts in the home. She has not
had any recent travel. She has not started any new medi-
cations. She has not used any new detergent, soap or Current Status
lotion. Prior to current illness, she is an active child
who spends a great deal of time playing outdoors. Mother On arrival at the ED, she is pale and lethargic. Physical
reports that she removed a tick from the child’s lower back examination is significant for dry mucous membranes
5 days ago. and tachycardia, with a heart rate of 136 bpm. She has

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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446 Cases in Pediatric Acute Care

generalized abdominal pain that is tender on light palpa- Questions

tion. She is afebrile on presentation. Periorbital edema is
noted. A blanching erythematous rash with macules
2 mm in size is noted on her feet, ankles, hands, wrists,
and forearms bilaterally (Figure 106.1). Blood cultures, uri-
nalysis, complete blood count, and complete metabolic
panel are obtained, with results as shown in Tables 106.1
and 106.2.
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 Would you obtain any other diagnostic studies based on
the working differential diagnoses?
3 What treatment would be recommended at this time?
4 Would you obtain any additional diagnostic studies?
5 What are other potential complications of the illness
that may require an escalation in care?

Rationale and Evidence-based Practice

Explanation

Based on the data given, what are the differential

diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include Rocky Moun-
Figure 106.1 Rash. Source: www.cdc.gov.
tain spotted fever (RMSF), Lyme disease, ehrlichiosis and
granulocytic anaplasmosis, meningitis, meningococcemia,
Table 106.1 Basic metabolic profile. streptococcal infection, gastroenteritis, and acute abdo-
men. Other illnesses such as rubella, measles, and non-
Sodium 140 mEq/L
specific viral illness may be included in the differential
Potassium 3.5 mEq/L
diagnoses.
Chloride 100 mEq/L The most likely diagnosis for this child is RMSF due to
Carbon dioxide 24 mEq/L the child’s history and presenting symptoms of nausea,
Blood urea nitrogen 24 mg/dL vomiting, abdominal pain, headache, fever, and rash fol-
Creatinine 0.7 mg/dL lowing the fever, and known tick bite. Edema around the
Glucose 85 mg/dL eyes is common in early illness. Approximately half of
Alanine aminotransferase 65 U/L patients with RMSF infection do not recall tick exposure,
which can make clinical diagnosis difficult.
Aspartate aminotransferase 50 U/L
RMSF is an infectious tick-borne illness that is curable
but which can be fatal without proper treatment. It is
caused by the intracellular bacterium Rickettsia rickettsii.
Table 106.2 Complete blood count. RMSF is considered one of the most severe tick-borne rick-
ettsial infections, with pre-antibiotic case-fatality rates as
White blood cell count 4500/mm3
high as 80% in some case reports. The disease causes fever,
Hemoglobin 12 g/dL
headache, abdominal pain, and rash in the majority of
Hematocrit 36% patients. The hallmark of RMSF is a blanching erythema-
Platelet count 120 000/mm3 tous rash with macules (1–4 mm in size) that become pete-
Neutrophils 66% chial over time. Although the evolution of skin rash may
Eosinophils 2% vary among patients, it usually begins on the ankles and
Monocytes 2% wrists and spreads to the trunk. A rash that appears on
Lymphocytes 30%
the palms and soles is highly suggestive of RMSF, but usu-
ally occurs in later-stage disease.

Would you obtain any other diagnostic studies
based on the working differential diagnoses?
RMSF is difficult to diagnose because there is no rapid
point-of-care testing available during an acute infection.
Therefore, the clinical diagnosis must be confirmed retro-
spectively by the use of serologic testing.
The standard serologic test for diagnosis of RMSF is the
indirect immunofluorescence antibody (IFA) assay for IgG
using R. rickettsii antigen. IgM and IgG antibodies typically
appear 7–10 days after onset of the illness, and the optimal
time to obtain a convalescent antibody titer is at 14–21 days
after the onset of symptoms. A fourfold rise in IgG titers
between the acute and convalescent stage is diagnostic of
seroconversion and recent illness. Therefore, IgG IFA
assays should be performed on paired acute and convales-
cent serum samples collected 2–4 weeks apart. Because
antibody titers are frequently negative in the first week of
illness, RMSF cannot be confirmed using single acute anti-
body results. IgM IFA assays are available through some
reference laboratories, but results might be less specific
than IgG IFA assays for diagnosing a recent infection.
Another diagnostic option in the evaluation of RMSF is
polymerase chain reaction (PCR) amplification performed
on DNA extracted from peripheral blood and a swab of the
skin rash. Rickettsia rickettsii infects the endothelial cells
that line blood vessels and may not circulate in large num-
bers in the blood until the disease has progressed to a severe
phase of infection. Although a positive PCR result is help-
ful, a negative result does not exclude the diagnosis, and
treatment should not be withheld due to a negative result.
What treatment would be recommended
at this time?
The patient is dehydrated based on the history, physical
examination with tachycardia, and elevated serum creati-
nine and blood urea nitrogen demonstrated on laboratory
findings. The dehydration is secondary to vomiting,
decreased oral intake, and insensible fluid losses from fever.
A 20 mL/kg normal saline bolus is indicated with reevalua-
tion after the bolus is complete. Administration of an anti-
emetic, such as ondansetron, is appropriate given the child
has experienced ongoing nausea and vomiting.
The most important aspect to timely and effective treat-
ment of a rickettsial illness is clinical recognition. When
a rickettsial illness is suspected, rapid empiric treatment
with oral or intravenous doxycycline is the treatment of
choice for both adults and children. Delay in treatment
can lead to severe disease and death or long-term complica-
tions. The dose of doxycycline for children weighing 45 kg
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Chapter 106 Child with Fever and Rash 447
or less is 2.2 mg/kg twice daily (maximum daily dose 200
mg). Children who weigh over 45 kg should receive the
adult dose of 100 mg twice daily. Fever generally resolves
within 24–48 hours after initiation of doxycycline. Doxycy-
cline resistance in RMSF has not been reported. Patients
should be treated with doxycycline for at least 3 days after
fever resolves and clinical improvement has been noted,
and thus minimum duration of treatment is 5–7 days. Tet-
racyclines can cause dental staining when administered to
children younger than 8 years. However, studies have
shown that the risk of dental staining with doxycycline is
minimal if a short course is administered.
Chloramphenicol is typically used in the rare setting
when an individual has a history of severe adverse reaction
to doxycycline (e.g. toxic epidermal necrolysis, severe hepa-
toxicity). The dose of chloramphenicol is 50 mg/kg daily in
four divided doses (maximum 4 g/day); dose reductions are
required if used to treat neonates.
Would you obtain any additional diagnostic
studies?
A lumbar puncture may be indicated in the setting of severe
headache in order to evaluate for meningococcal disease. If
a lumbar puncture is performed in a patient with RMSF,
cerebrospinal fluid (CSF) results typically show a white
blood count of less than 100 cells/mm3 (with lymphocytic
or monocytic predominance), moderately elevated protein
(100–200 mg/dL), and a normal glucose level. Abnormal
coagulation studies may be present in the setting of RMSF.
Coagulation studies including prothrombin time (PT) and
activated partial thromboplastin time (PTT) should be
assessed.
What are other potential complications of the
illness that may require an escalation in care?
Cardiopulmonary manifestations may include myocarditis,
congestive heart failure, dysrhythmia, and pneumonia, but
these are uncommon in patients who are treated early.
Conjunctivitis is the ocular abnormality that is most com-
monly present, but optic edema, retinal arterial occlusion,
retinal hemorrhage, and retinal vein engorgement can also
occur. Neurologic complications can include lethargy,
meningismus, cortical blindness, seizures, cranial nerve
palsies, confusion, photophobia, or psychosis. Hyponatre-
mia is a particularly common finding in patients with
central nervous system involvement. Azotemia may be seen
and is usually due to hypovolemia. Critical illness is
marked by disseminated intravascular coagulation and
acute respiratory distress syndrome.

448 Cases in Pediatric Acute Care
Children are at high risk of death from RMSF, and survi-
vors may have severe complications, including limb ampu-
tation, deafness, and cognitive deficits. Pediatric patients
must be monitored closely as they can quickly progress
Further Reading
Blanton, L. (2019). The rickettsioses. Infectious Disease Clinics
of North America 33(1): 213–229.
Centers for Disease Control and Prevention. Rocky Mountain
spotted fever. https://www.cdc.gov/rmsf/ (accessed
29 March 2019).
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to severe myocarditis, encephalitis, and multiorgan failure.
Early antibiotics are crucial in this population, and reduce
the risk of morbidity and mortality.
Gottlieb, M., Long, B., and Koyfman, A. (2018). The evaluation
and management of Rocky Mountain spotted fever in the
emergency department: a review of the literature. Journal of
Emergency Medicine 55(1): 42–50.

107
A Teen With Vomiting and Diarrhea
Christyne Kyper
Alaska Native Tribal Health Consortium, Anchorage, AK, USA
A 15-year-old female is admitted to the pediatric floor with
abdominal pain, vomiting, diarrhea, and an elevated
lipase level.
History of Present Illness
This 15-year-old female presents with a 3-day history of
abdominal pain and vomiting with occasional loose stools.
She is nauseated any time she tries to eat or drink. Her
abdominal pain is diffuse, and she points to her entire abdo-
men when asked where it hurts, but with persistent ques-
tioning will say it is mostly the upper area. Her pain is
exacerbated by eating, lying flat, or walking. She describes
emesis as “orange liquid with chunks in it.” She reports
drinking approximately two cups of hand sanitizer per
day (about 226 g), which she gets during gym class when
no one is watching. She is currently in a residential treat-
ment program for depression and suicidal ideation.
Past Medical History
This teen’s medical history includes type 2 diabetes, depres-
sion, suicide attempts, self-mutilation, obesity, and
bulimia. She admits to using marijuana and alcohol since
12 years of age.
Her current medications include aripiprazole, Miralax,
fluoxetine, quetiapine, escitalopram, doxycycline, olanza-
pine, metformin, Colace, famotidine, potassium supple-
ments, omega 3, vitamin B complex, vitamin C, ferrous
sulfate, and diphenhydramine. She is allergic to shellfish
and reports a weight gain of 4.5 kg in the past week.
• Abdominal ultrasound: enlarged fatty liver, large amount
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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449
Past Surgical History
The patient had a tonsillectomy and adenoidectomy at
age 7.
Family history includes both parents are healthy. Pater-
nal grandmother has a history of depression and anxiety.
Paternal grandfather is deceased. Maternal grandparents
are alive and well.
Social History
Parents are separated and live in different cities. Patient
reports having multiple altercations with mother and
spends most of her time living with either the father or
paternal grandmother.
Current Status
On presentation to the emergency department (ED), her
vital signs are as follows: temperature 38.4 C (101.2 F),
heart rate 112 bpm, respiratory rate 20 breaths per minute,
blood pressure 151/87 mmHg, and oxygen saturation 95%.
Her weight is 132 kg.
A basic metabolic panel, complete blood count, pan-
creatic enzymes, lipid panel, and urinalysis are obtained
and the results are shown in Tables 107.1–107.5. Hemo-
globin A1c level is 5.2 mg/dL. Urine and blood cultures
are obtained. Pregnancy and influenza screens are
negative.
Imaging results are as follows.
of non-mobile sludge within the gallbladder lumen, mild
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450 Cases in Pediatric Acute Care

Table 107.1 Basic metabolic panel. gallbladder wall thickening. Positive sonographic Mur-
phy sign. Visualized portions of pancreas within normal
Sodium 143 mEq/L
limits.
Potassium
Chloride
3.8 mEq/L
105 mEq/L • Abdominal computed tomography (CT): pancreas is dif-
fusely edematous with surrounding peripancreatic fat
Carbon dioxide 30 mEq/L stranding and peripancreatic fluid. No organized pseudo-
Blood urea nitrogen 5 mg/dL cyst. Mild abdominal ascites, no calcifications in gallblad-
der or biliary tree. Small pleural effusions noted bilaterally.

•
Creatinine 0.6 mg/dL
Glucose 116 mg/dL
Magnetic resonance cholangiopancreatography (MRCP):
findings same as CT.
Calcium 8.5 mg/dL
The patient had another elevation in her lipase 5 days
later when resuming a regular diet, which gradually came
Table 107.2 Complete blood count. down over the next 5 days.
White blood cell count 16 500/mm3
Hemoglobin 11 g/dL
Questions
Hematocrit 35.9%
Platelet count 159 000/mm3 Answer the following questions using the details provided.
Neutrophils 38%
1 What would be included in the differential diagnosis?
Lymphocytes 45%
2 How is the diagnosis of pancreatitis made?
Monocytes 1.3%
3 What is the initial management of acute pancreatitis?
Eosinophils 4% 4 When can you begin to feed the patient?
5 Is any other management needed for this patient?

Table 107.3 Pancreatic enzymes.

Rationale and Evidence-based Practice
Amylase 191 U/L Explanation
Lipase 236 U/L
What would be included in the differential
diagnosis?

Table 107.4 Lipid panel. There are many differential diagnoses for abdominal pain,
including gastroenteritis, cholecystitis, pancreatitis, intra-
Cholesterol 83 mg/dL abdominal infection, gastritis, pelvic inflammatory disease
Lipid 53 mg/dL (PID), ovarian torsion, appendicitis, and constipation. Each
High-density lipoprotein 34 mg/dL of these diagnoses presents with similar symptoms, but
Low-density lipoprotein 35 mg/dL physical findings and diagnostic testing results will be dif-
ferent. For this patient, pancreatitis is a consideration due
to her elevated lipase. It may be associated with her medi-
Table 107.5 Urinalysis. cations or from drinking hand sanitizer and alcohol.
Because her pain is primarily in the upper region, PID,
Color Clear, yellow ovarian torsion, and appendicitis are less likely.
pH 6.5
Specific gravity 1.009 How is the diagnosis of pancreatitis made?
White blood cells 0 The diagnosis of pancreatitis is defined by two of the follow-
Nitrite Negative ing: acute onset of persistent severe epigastric pain often
Protein Negative radiating to the back, elevation in serum lipase or amylase
Glucose Negative to three times or greater the upper limit of normal, or char-
Bilirubin Negative acteristic findings of acute pancreatitis on imaging with
contrast CT, magnetic resonance imaging (MRI) or

transabdominal ultrasound. The diagnosis can be made
without imaging if the first two criteria are met.
What is the initial management of acute
pancreatitis?
Initial management of acute pancreatitis consists of fluid
resuscitation, pain control, and monitoring. Fluid status
must be assessed first. If patients present with hypotension
and tachycardia, fluid boluses of 20 mL/kg of isotonic fluid
should be administered until stable. This patient received
40 mL/kg of normal saline soon after initial evaluation in
the ED. Therapy should be goal-directed and reassessed
at frequent intervals. Adequate fluid replacement should
be based on clinical assessment of vital signs, urine output,
and reduction in hematocrit and blood urea nitrogen
(BUN) over the first 24 hours. Persistent hemoconcentra-
tion at 24 hours has been associated with development of
necrotizing pancreatitis.
Pain control is achieved with opioids. Intravenous hydro-
morphone or fentanyl are typically used, with fentanyl
becoming more widely used due to its better safety profile,
especially related to renal impairment. Morphine can cause
an increase in sphincter of Oddi pressure, but there are no
clinical studies to suggest that morphine can aggravate or
cause pancreatitis.
Further Reading
Tenner, S., Baillie, J., DeWitt, J., and Vege, S.S. (2013).
American College of Gastroenterology Guideline:
management of acute pancreatitis. American Journal of
Gastroenterology 108(9): 1400–1415. doi: 10.1038/
ajg.2013.218.
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Chapter 107 A Teen With Vomiting and Diarrhea 451
Patients with acute pancreatitis should be monitored
closely for the first 24–48 hours. Vital signs including oxy-
gen saturations, urine output, electrolytes, and serum glu-
cose should all be assessed frequently. Hyperglycemia
should be treated because it can increase the risk of second-
ary pancreatic infections.
When can you begin to feed the patient?
Oral feedings can be initiated within 24 hours if there is no
ileus, nausea, or vomiting, the pain is decreasing, and
inflammatory markers are improving. The suggested diet
is low residue, low fat, and soft. If the patient cannot toler-
ate oral feedings within 5 days, it is recommended that
enteral feeds with high-protein, low-fat, semi-elemental
formula be started.
Is any other management needed for this patient?
This patient has an extensive psychiatric history. Because
she has previous attempts at self-harm she should have sui-
cide precautions. She has admitted to drinking hand sani-
tizer so this needs to be carefully monitored while she is in
the hospital. Consults to behavioral health, nutrition, and
endocrinology would also be warranted.
Vege, S.S., DiMagno, M.J., and Forsmark, C.E. (2018). Initial
medical treatment of acute pancreatitis: American
Gastroenterological Association Institute Technical Review.
Gastroenterology 154(4): 1103–1139.

108
Acute Jaundice in a 7-Year-Old Child
Roseann Hausmann
Seattle Children’s Hospital, Seattle, WA, USA
A 7-year-old female presents to the emergency department
(ED) from her primary care provider’s office with a
complaint of fever and abdominal pain.
History of Present Illness
Parents report that 2 weeks ago the patient had a fever to
38.8 C (101.8 F), cough, vomiting, and diarrhea which
resolved without treatment. Today she began to have
dark-colored urine and the primary care provider noted
jaundice of her skin and sclera, and a 5-kg weight gain com-
pared to her last appointment a month ago. She was then
referred to the ED for further evaluation.
Past Medical History
The child was born at term by spontaneous vaginal delivery
with no medical complications and discharged home with
her mother. She has received all immunizations to date, but
did not get influenza vaccine this year. She has never been
hospitalized and does not have any chronic illness.
Past Surgical History
The patient had pressure equalizer tubes placed at 2 years of
age for recurrent otitis media.
Family History
Parents are both alive and healthy. Her 14-year-old brother
was recently sick with a an acute upper respiratory
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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453
infection, but did not see a medical provider or require
treatment.
Current Status
The patient is evaluated in the ED and initially noted to be
sleepy but rousable. She is able to answer questions with
prompting from her mother. Vital signs are stable: temper-
ature 38.9 C (102 F), heart rate 120 bpm, respiratory rate
18 breaths per minute, and blood pressure 100/65 mmHg.
She complains of itching in her arms and legs and right
upper quadrant abdominal pain. She is noted to have scle-
ral icterus. Laboratory studies are obtained and the results
are shown in Tables 108.1–108.3. The ammonia level is
found to be 86 μg/dL.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses for this child?
2 What additional testing would be warranted?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses for this child?
This patient presents with signs of acute liver failure.
Elevation of the liver function enzymes alanine amino-
transferase (ALT) and aspartate aminotransferase (AST)
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454 Cases in Pediatric Acute Care

Table 108.1 Complete blood count. which acetaminophen was given during her acute illness.
It is also important to question the patient and parents
White blood cell count 26 000/mm3
about other potential drugs which could cause liver failure,
Hemoglobin 10 g/dL like some antibiotics, antifungals and ibuprofen, among
Platelet count 85 000/mm3 others.
Neutrophils 8% Immune system dysfunction is another category to con-
Lymphocytes 22% sider within the differential diagnosis. The two forms that
Monocytes 1% are often seen in children are autoimmune hepatitis and
Bands 60%
hemophagocytic lymphohistiocytosis (HLH). While auto-
immune hepatitis presents equally across all ages of pediat-
ric patients, HLH is most typically seen in children under
5 years of age. HLH has been reported in older children
Table 108.2 Complete metabolic profile and liver function test
results. and in adolescence, so while less likely, should be
considered.
Sodium 146 mEq/L Another cause of acute liver failure can be hypoperfusion
Potassium 3.5 mEq/L due to cardiac dysfunction, dehydration, or decreased blood
Chloride 100 mEq/L flow due to sepsis or hepatic artery clot. As the patient has
Carbon dioxide 24 mEq/L
been without symptoms other than fatigue in recent days,
and she has been eating and drinking normally, this cause
Blood urea nitrogen 26 mg/dL
may not be high on the differential list.
Creatinine 1.4 mg/dL
Metabolic conditions or processes are the final concern
Glucose 90 mg/dL for cause of liver failure, which in the school-aged child
Albumin 2.9 g/dL would be Wilson disease or mitochondrial diseases
Alanine aminotransferase 980 U/L (Squires et al., 2019). Screening for Wilson disease should
Aspartate aminotransferase 765 U/L include serum ceruloplasmin, urine copper, Coombs test,
Bilirubin 6.1 mg/dL and ophthalmology examination for Kayser–Fleischer
rings (Squires et al., 2019).

Table 108.3 Coagulation studies.

Prothrombin time (PT) 18 s
Partial thromboplastin time (PTT) 40 s
INR 2.5
Fibrinogen 250 mg/dL
indicates inflammation of the liver, while the elevated
international normalized ratio (INR), bilirubin and ammo-
nia, and clinical findings of encephalopathy indicate that
she has progressed to liver failure. The differential diagno-
sis for cause of pediatric acute liver failure is broad and will
require a detailed evaluation, but some of the potential dif-
ferentials would include drug toxicity, infection, immune
system dysfunction, hypoperfusion, and metabolic disease.
Given the history of viral symptoms, an infectious disease
evaluation should be performed to establish this as a poten-
tial cause of liver failure. Some infectious causes that are
known to result in acute liver failure in pediatric patients
include hepatitis A, B and C, adenovirus, parvovirus,
Epstein–Barr virus, cytomegalovirus, and enterovirus. Ace-
taminophen overdose is another diagnosis to consider, and
it is important to assess the amount and frequency with
What additional testing would be warranted?
In order to consider the list of differential diagnoses and to
finalize a working diagnosis, additional laboratory studies
are indicated. An infectious evaluation should be per-
formed to determine a potential viral cause of acute liver
failure. Depending on facility availability of testing, the
tests can include IgG and IgM to determine current and
past infectious exposures, and quantitative polymerase
chain reaction (PCR) to determine exact viral loads.
A respiratory viral PCR should also be obtained for a broad
screening of viruses prevalent in the local area. It is impor-
tant to note that many of these viruses have no treatment,
so the information provides prognostic indication but may
not change the course of treatment.
Autoimmune hepatitis may be suggested by positive
immune markers, such as anti-nuclear antibody (ANA)
and anti-smooth muscle antibody (SMA), so obtaining this
blood work is important. The treatment for autoimmune
hepatitis will include steroids, so it is important to confirm
the absence of an infectious agent, since an infection will be
made worse by the addition of steroids. HLH can be diag-
nosed with cytopenia in two or more cell lines, and by an
elevated ferritin level. To exclude hypoperfusion injury,
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Chapter 108 Acute Jaundice in a 7-Year-Old Child 455

an abdominal ultrasound with Doppler can be performed to Additional Questions and Evidence-based
monitor blood flow into the liver. Rationale
Continuation of case: The patient is admitted to the pedi-
atric intensive care unit (PICU) for acute liver failure of 3 Given the diagnostic evaluation, what is the most likely
unknown etiology. Over the next 36 hours, she becomes diagnosis for this patient?
unresponsive and requires intubation and is maintained 4 What consults would be recommended at this time?
on a mechanical ventilator. She is given vitamin K with
no change to her INR. On abdominal ultrasound she is
noted to have a large-volume ascites. An ophthalmology Given the diagnostic evaluation, what is the most
examination is completed which shows Kayser–Fleischer likely diagnosis for this patient?
rings. Additional diagnostic laboratory results based on Based on laboratory findings and the ophthalmology exam-
the differential diagnoses are shown in Table 108.4. Liver ination, the most likely diagnosis is Wilson disease. The
function tests have also been trended every 8 hours since American Association for the Study of Liver Diseases
admission and the results are shown in Table 108.5. criteria for the diagnosis of Wilson disease include a low
serum ceruloplasmin (<20 mg/dL), the presence of
Kayser–Fleischer rings on eye examination, and a 24-hour
urine copper level of greater than 40 μg/day.
Table 108.4 Additional diagnostic laboratory results based on Wilson disease is an autosomal recessive disorder that
differential diagnoses. can be associated with liver disease, degenerative changes
in the brain, and the presence of Kayser–Fleischer rings in
Viral respiratory panel Negative
the cornea. The pathologic mechanism is a defect in
Coombs test Negative ATP7B, a copper-transporting ATPase mainly expressed
Hepatitis A Negative in hepatocytes (Schilsky, 2017). When the liver’s capacity
Hepatitis B Negative for copper storage is exceeded or liver cells are damaged,
Hepatitis C Negative there is resulting accumulation of copper in the liver
Epstein–Barr virus PCR Not detected which then spills over into the circulation, and potentially
Cytomegalovirus PCR Not detected into other organs such as the brain, cornea and kidneys.
Wilson disease can present as acute liver failure, progres-
Parvovirus PCR Not detected
sive neurologic disorder, or psychological illness. Most
Adenovirus PCR Not detected
patients with liver disease present in childhood, within
Herpes simplex virus Negative the first 20 years of life, and about 5% of these patients
Ferritin 100 ng/mL develop acute liver failure. If there is a family history of
Anti-nuclear antibody Negative psychological disorders, neurologic disorders that occurred
Anti-smooth muscle antibody Negative in the latter part of life, consideration for Wilson disease
Urine copper 200 μg per 24 hours should be made. There is also a scoring system that
Ceruloplasmin 7 mg/dL includes different laboratory tests, including ceruloplas-
min, urinary copper and chromosome analyses, which
Acetaminophen Not detected
can be used for diagnosis.

Table 108.5 Laboratory trends.

AST (U/L) ALT (U/L) Bilirubin (mg/dL) Albumin (g/dL) Ammonia (μg/L) INR

Monday 10:00 (ED) 895 765 8 2 86 2.6

Monday 18:00 1900 1500 8.5 2.2 90 2.6
Tuesday 02:00 3800 3000 9 2 98 (started 1 (given factor VII
lactulose) for procedure)
Tuesday 10:00 6400 5100 11 1.8 85 1.6
Tuesday 18:00 5800 4800 11.5 1.8 95 2
Wednesday 02:00 3800 3000 14 1.6 100 2.5

456 Cases in Pediatric Acute Care
If identified early, Wilson disease can be managed with a
low-copper diet and chelating agents, such as D-penicilla-
mine, trientine or zinc salts, which increase the urinary
excretion of copper. Foods that are high in copper include
mushrooms, chocolate, liver, and nuts. Patients should also
be monitored with laboratory testing, including complete
blood count, liver panel, serum copper and ceruloplasmin,
and 24-hour urine collection for copper and zinc.
What consults would be recommended at
this time?
The most important and urgent consult is with a pediatric
liver transplant team. When a patient experiences decom-
pensated Wilson disease causing fulminant liver failure,
transplant is often the only treatment option. Current lab-
oratory studies in this case demonstrate a falling AST/ALT
References
Schilsky, M.L. (2017). Wilson disease: diagnosis, treatment and
follow-up. Liver: the Clinics 21: 755–767. doi: 10.1016/j.
cld.2017.06.011.
Squires, J.E., Logan, B., Lorts, A., et al. (2019). A learning
health network for pediatric liver transplantation: inaugural
Further Reading
Bandmann, O., Weiss, K.H., and Kaler, S.G. (2015). Wilson’s
disease and other neurological copper disorders. Lancet
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with a rising bilirubin, ammonia, and INR. These results
are concerning, indicating that the liver does not have suf-
ficient viable tissue to continue producing the enzymes,
nor is it able to filter toxins. Prognosis in Wilson disease
with liver failure and hemolysis is poor without liver trans-
plant. Acute decompensated Wilson disease qualifies a
patient to be listed as a Status 1A on the United Network
for Organ Sharing (UNOS) waiting list for deceased donor
transplants. Liver transplant is curative for Wilson disease,
but transplant in itself is a risk and requires lifelong
follow-up.
A consult with the genetics team would also be indicated.
All siblings and first-degree relatives of the patient should
be screened for Wilson disease. If the disease is identified,
treatment with chelating agents can be begun to slow the
progression and hopefully prevent liver failure.
meeting report from the Starzl Network for Excellence in
Pediatric Transplantation. Pediatric Transplantation 23(6):
e13528. doi: 10.1111/petr.13528.
Neurology 14(1): 102–113. doi: 10.1016/S1474-4422(14)
70190-5.

109
Idiopathic Nephrotic Syndrome
Tamara Hill
University of Maryland Medical Center, Baltimore, MD, USA
A 3-year-old male is brought to the emergency department
(ED) with complaints of weight gain/puffiness, abdominal
pain, and rapid breathing and is admitted to the pediatric
intensive care unit.
History of Present Illness
This 3-year-old child became ill 3 weeks ago when his
mother noticed swelling around his eyes when he woke
up in the morning. After several days of these symptoms,
he was seen by his pediatrician who thought he might have
seasonal allergies and prescribed loratadine. Two weeks
ago, he developed three to four loose watery stools per
day (non-bilious, non-bloody) and has complained inter-
mittently of mild abdominal pain. He was evaluated again
by another provider in the pediatric office and was diag-
nosed with gastroenteritis. Over the past week his mother
noticed that, despite his poor appetite, the waistband of
his pants are getting tighter and his face and legs seem
puffy. He is normally a very energetic and happy child
but for a week now he has been fussy and tired.
Past Medical History
This child was born by spontaneous vaginal delivery at
term and went home with his mother. No complications.
He is current on all immunizations. He has a history of fre-
quent episodes of otitis media. His weight is 99% for gender
and he has a current body mass index (BMI) of 28.5.
Past Surgical History
History of uncomplicated circumcision, but no other surgi-
cal procedures.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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457
Family History
The patient’s father and paternal grandparents have a his-
tory of hypertension. Mother is healthy. There are no
siblings.
Current Status
On arrival to the ED, the patient has mild tachypnea with
respiratory rate of 28 breaths per minute. His lung sounds
are clear. His blood pressure is elevated (122/82 mmHg)
and his weight is up by 1.8 kg from his baseline weight.
He has normal S1 and S2 heart sounds with heart rate of
74 bpm. He has facial, periorbital and scrotal edema with
1–2+ pitting edema up to the knees and ankles; no rashes
or ecchymosis noted. His abdomen is rounded and full with
ascites 2+ noted. He has no organomegaly but he has
abdominal pain 8 on Wong–Baker Faces Scale.
A complete metabolic panel, complete blood count and
urinalysis are obtained, with results as shown in
Tables 109.1–109.3.
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What is the best initial management for this condition?
3 What other therapies would be recommended for fre-
quent relapses of this condition?
4 Would you obtain any other diagnostic studies?
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458 Cases in Pediatric Acute Care

Table 109.1 Complete metabolic panel. Rationale and Evidence-based Practice

Sodium 132 mEq/L
Explanation
Potassium 4.9 mEq/L
Based on the data given, what are the differential
Chloride 105 mEq/L
diagnoses and the most likely diagnosis for
Carbon dioxide 26 mEq/L this child?
Blood urea nitrogen 14 mg/dL
This child is presenting with idiopathic nephrotic syn-
Creatinine 0.36 mg/dL
drome (NS) based on laboratory results and presentation.
Glucose 71 mg/dL
Nephrotic syndrome is characterized by heavy proteinuria,
Calcium 9.8 mg/dL hypoalbuminemia (serum albumin <2.5 g/dL), hyperlipi-
Albumin 2.0 mg/dL demia (serum cholesterol >200 mg/dL), and edema.
Total protein 4.8 g/dL Nephrotic range proteinuria is present if early morning
Aspartate aminotransferase 37 U/L urine protein is 3+/4+ (on dipstick), spot protein/creati-
Alanine aminotransferase 20 U/L nine ratio is above 2 mg/mg, or urine albumin excretion
Alkaline phosphatase 252 U/L
exceeds 40 mg/m2 per hour (on a timed sample). Precise
quantitative assessment of proteinuria, including 24-hour
Total bilirubin 0.6 mg/dL
urine protein measurement, is seldom necessary.
Minimal change nephrotic syndrome (MCNS), focal seg-
mental glomerulosclerosis (FSGS), membranoproliferative
Table 109.2 Complete blood count. glomerulonephritis (MPGN), and IgA nephropathy are
detected via renal biopsy. MCNS is the most common histo-
White blood cell count 8000/mm3
logic form of NS, with males affected more frequently than
Hemoglobin 13.1 g/dL
females. FSGS may present like MCNS or with less profound
Hematocrit 38% proteinuria. MPGN is characterized by hypocomplementemia
Platelet count 275 000/mm3 and glomerular renal disease. IgA nephropathy is commonly
Neutrophils 64% seen in adolescents and children with systemic infections.
Lymphocytes 4%
Monocytes 3.4%
What is the best initial management
Eosinophils 3.4%
for this condition?
The standard medication for treatment of NS is predniso-
lone or prednisone. The initial episode of NS should be trea-
Table 109.3 Urinalysis. ted at a dose of 2 mg/kg daily (maximum 60 mg in single or
divided doses) for 6 weeks, followed by 1.5 mg/kg (maxi-
Color Dark yellow mum 40 mg) as a single morning dose on alternate days
pH 6.0 for the next 6 weeks. The duration of initial prednisolone
Specific gravity 1.008 therapy should be for a minimum of 12 weeks. The benefits
Protein 3+ of sustained remission and reduction in relapse rates are
Nitrites Negative superior if alternate-day treatment is not stopped abruptly
Urobilirubin 1+ at 12 weeks, but tapered over the next 2–4 months.
Blood Small
Leukocytes Negative What other therapies would be recommended
Ketones Negative for frequent relapses of this condition?
Glucose Negative
Treatment for infection is recommended before initiating
White blood cells 11–25 per high power field steroid therapy. Prednisolone is administered at a dose of
Red blood cells 0–2 per high power field 2 mg/kg daily (single or divided doses) until urine protein
Hyaline casts None is trace or nil for three consecutive days. Subsequently, pre-
Bacteria Occasional dnisolone is given in a single morning dose of 1.5 mg/kg on
Crystals None alternate days for 4 weeks and then discontinued. The usual
duration of treatment for a relapse is thus 5–6 weeks.

In case the patient is not in remission despite 2 weeks of
treatment with daily prednisolone, the treatment is
extended for a further 2 weeks. If the patient is steroid
sensitive, he will respond to steroids. If there is steroid
resistance, other immunosuppressive therapy (e.g. myco-
phenolate mofetil or tacrolimus) will need to be considered.
Therapy also includes nutrition education: a low-sodium
and low-protein diet, and fluid restriction. There should be
close monitoring for hypertension and recording of weight.
Would you obtain any other diagnostic studies?
Diagnostic studies during the initial episode should
include urinalysis, complete blood count, and blood
levels of albumin, cholesterol, urea, and creatinine. Esti-
mation of blood levels of antistreptolysin O and C3 is
required in patients with gross or persistent microscopic
Further Reading
Marcdante, K.J. and Kliegman, R. (ed.) (2019). Nelson
Essentials of Pediatrics, 8th edn. Philadelphia, PA: Elsevier.
Nishi, S., Ubara, Y., Utsunomiya, Y., et al. (2016). Evidence-
based clinical practice guidelines for nephrotic syndrome
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Chapter 109 Idiopathic Nephrotic Syndrome 459
hematuria. Appropriate tests are performed, if necessary,
for associated conditions (e.g. chest X-ray and tuberculin
test, hepatitis B surface antigen, and anti-nuclear antibo-
dies). Urine culture is not necessary unless the patient
has clinical features suggestive of a urinary tract
infection.
Children with idiopathic NS who do not have hematuria,
hypertension, or impaired renal function are treated with
corticosteroids without requiring a kidney biopsy.
A biopsy is usually not necessary in patients with frequent
relapses or steroid dependence before starting treatment
with levamisole, cyclophosphamide, or mycophenolate
mofetil, but should be performed before therapy with calci-
neurin inhibitors. A biopsy is required to identify the
underlying renal disease in certain cases (MCNS, FSGS,
MPGN and IgA nephropathy).
2014. Clinical and Experimental Nephrology 20(3): 342–370.
doi: 10.1007/s10157-015-1216-x.
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461

110

School-age Child with Hives and Lip Swelling

Jay M. Hunter
UCSF Benioff Children’s Hospital and UCSF School of Nursing, San Francisco, CA, USA

A 10-year-old male presents to the emergency department Current Status

(ED) with hives and lip swelling.
On arrival to the ED, the patient has facial angioedema,
scattered hives, mild respiratory distress, and appears
History of Present Illness scared. His respiratory rate is 38 breaths per minute with
moderate subcostal retractions. He is wheezing and his oxy-
The boy was outside playing with his friends this afternoon
gen saturations are 92% on 100% oxygen via a non-
when he was stung by a bee. The patient’s mother reports
rebreather mask. His blood pressure is 78/42 mmHg.
that he was crying, screaming, and tearful when she noticed
that his lips were swollen. The patient began to have diffi-
culty breathing, so she called emergency medical services
(EMS). On arrival to the scene, the patient had marked Questions
facial and lip edema and was in significant respiratory dis-
tress, so EMS administered intramuscular epinephrine to Answer the following questions using the details provided.
the patient and transported him to the ED. 1 Based on the currently available data, what are the dif-
ferential diagnoses and the most likely diagnosis for
this child?
Past Medical and Surgical History 2 What is the best way to treat this patient’s current signs
and symptoms on arrival at the ED?
The patient was born full term via vaginal delivery to a
3 How would you determine if the treatment was
healthy mother without complications. The patient has
effective?
completed all vaccinations to date, including an influenza
4 What other treatments would you recommend at
vaccine earlier this year. He has no known medical pro-
this time?
blems. The patient has never had surgery, nor has been
5 What is the patient’s disposition?
admitted to the hospital. He does not take any medications
and has no known allergies to medications or foods.

Rationale and Evidence-based Practice

Family and Social History
Explanation
The patient’s parents are married. His mother is 34 years old,
Based on the currently available data, what are the
healthy, does not report any medical problems, and works as
differential diagnoses and the most likely
a lawyer. His father is 36 years old, reports a history of hyper-
diagnosis for this child?
tension, no other medical problems, and works as a regis-
tered nurse. The patient has one sister who is 12 years old, Differential diagnoses for this child would include
healthy, and does not have any medical problems. The anaphylaxis, acute asthma exacerbation, severe croup,
patient is in the 4th grade and he does well in school. urticaria, foreign body aspiration, isolated angioedema,

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.

462 Cases in Pediatric Acute Care
food poisoning, vasovagal episode, idiopathic capillary leak
syndrome, and other causes of shock. Anaphylaxis is the
most likely diagnosis due to the presentation of facial
angioedema, urticaria, and respiratory compromise follow-
ing an insect sting. Anaphylaxis is commonly misdiagnosed
and underdiagnosed when cutaneous signs are not present,
so it is important to recognize the other signs and symptoms
of anaphylaxis, including, but not limited to, dizziness,
hypotension, cough, stridor, dyspnea, vomiting, diarrhea,
and wheezing.
What is the best way to treat this patient’s current
signs and symptoms on arrival at the ED?
Epinephrine continues to be the mainstay of treatment for
anaphylaxis, with the greatest benefit on administration
prior to ED presentation. For pediatric patients, epineph-
rine should be administered intramuscularly at a dose of
0.01 mg/kg of 1:1000 concentration in the vastus lateralis
muscle, with a maximum dose of 0.3 mg but up to
0.5 mg in prepubertal and teenage children. All other med-
ications, including antihistamines, inhaled beta-agonists
and glucocorticoids, are adjuvant medications that should
be administered only in addition to, but not to replace,
epinephrine.
How would you determine if the treatment
was effective?
Close monitoring for improvement of presenting signs and
symptoms are paramount in determining the effectiveness
of treatment. In addition, close and continuous monitoring
of vital signs, including blood pressure, heart rate, respira-
tory rate, and oxygen saturations, during treatment and
Further Reading
Farbman, K.S. and Michelson, K.A. (2016). Anaphylaxis in
children. Current Opinion in Pediatrics 28(3): 294–297.
doi: 10.1097/MOP.0000000000000340.
Langley, E.W. and Gigante, J. (2013). Anaphylaxis, urticaria,
and angioedema. Pediatrics in Review 34(5): 247–257. doi:
10.1542/pir.34-6-247.
Sicherer, S.H. and Simons, F.E.R. (2017). Epinephrine
for first-aid management of anaphylaxis. Pediatrics
139(3): e20164006. doi: 10.1542/peds.2016-4006.
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until signs and symptoms are resolved are important in
determining if other signs of anaphylaxis and shock are
present.
What other treatments would you recommend
at this time?
Additional systemic symptoms should be treated with sec-
ond-line agents following epinephrine administration, as
appropriate, with particular consideration of biphasic ana-
phylactic reactions. Histamine H1 and H2 receptor antago-
nists, such as diphenhydramine and ranitidine, may be
administered for cutaneous and gastrointestinal symptoms,
and inhaled beta-agonists, such as albuterol, may be admi-
nistered to treat reactive airway. Intravenous fluid replace-
ment and oxygen therapies should be provided for patients
with hypoxia and hypotension.
What is the patient’s disposition?
For those patients with concern for protracted or biphasic
reactions, they should be monitored in the ED for approx-
imately 3–4 hours after administration of epinephrine to
assess for recurrence of symptoms. If symptoms recur, then
medications should be re-dosed for a 24-hour period and
hospitalization is warranted.
Consideration should be made about a referral to an
allergy and immunology specialist for treatment with
venom immunotherapy for insect sting-induced anaphy-
laxis. This treatment can reduce the risk of subsequent ana-
phylaxis to less than 5% if a full course of therapy is
completed.
Simons, F.E.R., Ebisawa, M., Sanchez-Borges, M., et al. (2015).
2015 update of the evidence base: World Allergy
Organization anaphylaxis guidelines. World Allergy
Organization Journal 8(1): 32. doi: 10.1186/s40413-
015-0080-1.

111
A 10-Year-Old with Asthma in the PICU
Catherine M. Haut1,2
1
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
2
Mednax-Pediatrix Medical Group Richardson, TX, USA
A 10-year-old girl with moderate intermittent asthma diag-
nosed at age 4 is admitted to the pediatric intensive care
unit (PICU) with status asthmaticus.
History of Present Illness
This 10-year-old girl became ill 2 days ago with cough and
upper respiratory congestion. She was determined by the
family to be wheezing, so she was given albuterol three times
today at home and twice yesterday with no real improve-
ment of symptoms. She was also treated with over-the-
counter cough preparation which was also ineffective. There
is no history of fever, but the child spent a week at a cousin’s
house where family members were smoking. She is not eat-
ing as usual and has complained of a stomach ache.
Past Medical History
This patient was born at term and went home with her
mother. She has had all required immunizations along with
annual influenza vaccine. Her weight has been at 99% for
gender and age since age 3 and her current body mass index
(BMI) is 28.5. Diagnosis of asthma was made at age 4 after a
2-week inpatient hospitalization for wheezing and pneu-
monia. She required intubation and ventilation during
the hospitalization for respiratory failure, unresponsive to
usual asthma medications, including continuous albuterol,
methylprednisolone, and magnesium sulfate administered
intravenously. She was admitted again to the PICU at age 6,
when she was managed with bilevel positive airway pres-
sure (BiPAP) and was discharged after 4 days in the hospi-
tal. She has had other episodes of wheezing, with two
additional admissions to the general pediatric unit due to
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
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463
hypoxemia with wheezing. Current medications include
Proair MDI two puffs as needed for wheezing, montelukast
5 mg daily, and Advair (fluticasone propionate/salmeterol
100 μg/50 μg) Diskus one puff twice daily. Parents report
compliance with medications. Despite being previously
referred to pulmonology, she has not had an appointment.
Triggers for wheezing include cigarette smoke, drastic
changes in weather, and viral illness such as a cold.
Past Surgical History
The girl had a tonsillectomy and adenoidectomy at age 6 for
obstructive sleep apnea.
Family History
The father of the patient had asthma as a child, but has not
had any wheezing for many years. The patient has a 5-year-
old brother who had one episode of wheezing at age 2.
There is no known family history of allergies or eczema.
Neither parent smokes.
Current Status
On arrival to the emergency department (ED), the patient is
wheezing and hypoxic, with an oxygen saturation of 88% on
room air. Her initial respiratory rate was 45 breaths per
minute with mild subclavicular retractions. She is treated
with oxygen, oral prednisone, and continuous albuterol
administered by facemask. Oxygen is provided at 50% ini-
tially and as she is admitted to the PICU, when she has
an increased oxygen requirement to 75% to maintain
saturations greater than 91%. A complete metabolic panel
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464 Cases in Pediatric Acute Care

Table 111.1 Basic metabolic panel.

Sodium 141 mEq/L

Potassium 3.8 mEq/L
Chloride 101 mEq/L
Carbon dioxide 24 mEq/L
Blood urea nitrogen 12 mg/dL
Creatinine 0.6 mg/dL
Glucose 171 mg/dL
Calcium 8.6 mg/dL

Table 111.2 Complete blood count.

White blood cell count 18 000/mm3

Hematocrit 12 g/dL
Hemoglobin 36%
Platelet count 346 000/mm3
Neutrophils 79%
Lymphocytes 9%
Monocytes 6%
Eosinophils 6%

Figure 111.1 Admission chest radiograph showing

and complete blood count are obtained, with results as
shown in Tables 111.1 and 111.2. A chest X-ray is obtained
and shows hyperinflation with concern for right upper and
middle lobe infiltrates (Figure 111.1).
Questions
Answer the following questions using the details provided.
1 Based on the data given, what are the differential diag-
noses and the most likely diagnosis for this child?
2 What is the best way to increase oxygenation and how
would you order this?
3 How would you determine if the therapy was effective?
Rationale and Evidence-based Practice
Explanation
Based on the data given, what are the differential
diagnoses and the most likely diagnosis for
this child?
Differential diagnoses for this child include status asthma-
ticus (associated with viral illness, exposure to allergens, or
other inciting factors), pneumonia, and aspiration. The
most likely diagnosis for this child is status asthmaticus
hyperinflation in status asthmaticus.
due to the child’s history and presenting symptoms of
wheezing, cough, and hypoxia. This child also has reported
inciting factors of tobacco exposure and viral symptoms
that may have triggered the exacerbation.
Status asthmaticus is defined as a severe asthma exacer-
bation not responsive to standard initial treatment doses of
beta-agonists (e.g. albuterol, levalbuterol). Status asthmati-
cus is a medical emergency requiring prompt recognition,
therapy, and hospitalization. Initial management of status
asthmaticus includes oxygen, beta-agonist therapy (e.g.
continuous albuterol inhalation), and systemic steroids.
What is the best way to increase oxygenation and
how would you order this?
Oxygen therapy often begins with delivery via a facemask;
however, this is frequently not sufficient to support the oxy-
genation needs of these children and some will require the
application of non-invasive ventilation support to assist in
gas exchange and distending pressure to improve oxygena-
tion. Non-invasive ventilation such as continuous positive
airway pressure (CPAP) or bilevel positive airway pressure
(BiPAP) is frequently delivered via facemask or nasal
pillows/prongs, but there is little evidence that documents
the effectiveness of high-flow nasal cannula. When order-
ing this therapy, the following settings will be needed:

amount of oxygen, inspiratory positive airway pressure
(IPAP, similar to pressure support on a conventional venti-
lator), and expiratory positive airway pressure (EPAP, sim-
ilar to positive end-expiratory pressure on a conventional
ventilator).
How would you determine if the therapy was
effective?
Frequent, repeated physical examinations are needed in this
child to determine the effectiveness of therapy. Evaluating
the child’s respiratory rate, work of breathing, inspiratory
to exhalation ratio, presence of wheezing, aeration, ability
to speak, overall level of comfort (e.g. reduction or absence
of anxiousness), and oxygenation status are all needed to
assess the child’s response to therapy.
Continuation of case: The girl is admitted to the PICU
and placed on BiPAP with settings of IPAP +12 cmH2O
and EPAP +6 cmH2O, plus inhaled continuous albuterol
15 mg/hour. She is continued on intravenous methylpred-
nisolone 2 mg/kg daily and started on ceftriaxone for pre-
sumed bacterial pneumonia. She is breathing at a rate of
30 breaths per minute with minimal subclavicular retrac-
tions and is still found to have inspiratory and expiratory
wheezing present. After 3 hours, oxygen has been
decreased to 40%, her respiratory rate is 28 breaths per
minute, and she is still wheezing.
Additional Questions and Evidence-
based Rationale
4 Should albuterol be continued and what other therapies
are recommended at this time?
5 Would you obtain any other diagnostic studies?
Should albuterol be continued and what other
therapies are recommended at this time?
Beta-agonists are the mainstay of therapy for status asthma-
ticus and will be continued through the non-invasive
Further Reading
Henderson, M.B., Schunk, J.E., and Henderson, J.L. (2018).
An assessment of asthma therapy in the pediatric ICU.
Hospital Pediatrics 8(6): 361–367. doi: 10.1542/
hpeds2017-0003.
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Chapter 111 A 10-Year-Old with Asthma in the PICU 465
ventilation circuit. Additional therapies for this child
include continuation of systemic steroids and consideration
of adjuvant pharmacologic therapies including additional
bronchodilators, anticholinergic medications (e.g. ipratro-
pium bromide) and/or intravenous magnesium sulfate. In
some cases, aminophylline could be considered. A small
population of children with status asthmaticus will require
invasive and/or mechanical ventilation if other initial man-
agement strategies do not improve clinical status. Risks
associated with the invasive positive-pressure ventilation
of such children, who have air trapping related to their sta-
tus asthmaticus, include the air leak syndromes (e.g. pneu-
mothorax, pneumomediastinum). In order to avoid such air
leak syndromes, children with status asthmaticus requiring
intubation and mechanical ventilation often need liberal
sedation/analgesia and paralytics. Other strategies that
can be considered include administration of Heliox, a
helium/oxygen gas mixture, or in severe cases, general
anesthesia, although there is limited evidence to support
these therapies.
An assessment of the child’s hydration status is also
needed. Children with respiratory distress have high insen-
sible losses and may have had reduced oral intake prior
to presentation. These children may require fluid boluses
and/or intravenous hydration.
Evaluation for an infectious etiology is also needed.
Asthma exacerbations are most commonly associated with
viral illnesses, though may be associated with a bacterial
superinfection. A close evaluation of the complete blood
count and fever history/fever curve can assist in determin-
ing the need for antibiotics.
Would you obtain any other diagnostic studies?
If the child has a history of a viral prodrome, viral studies
can be sent to assist in identifying the trigger of the exacer-
bation and to appropriately isolate the child if positive.
Blood culture and/or C-reactive protein may be considered
if the history suggests a bacterial illness.
Kaiser, S.V., Rodean, J., and Bekmezian, A. (2018).
Effectiveness of pediatric asthma pathways for hospitalized
children: a multicenter national analysis. Journal of
Pediatrics 197: 165–171.e2. doi: 10.1016/j.jpeds.2018.01.084.
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467

112

Skin Wound in Postoperative Neonate

Hayley Redmond
Norton Children’s Hospital, Louisville, KY, USA

You are called to the bedside of a pediatric cardiac intensive arteriosus. He was treated empirically with antibiotics for
care unit (CICU) patient to evaluate a new skin wound in a 48 hours after birth. The remainder of his NICU course
neonate who has recently undergone heart surgery. was uneventful. He was taken to the operating room for
cardiac repair when he was 3 days old.

History of Present Illness

Past Surgical History
A 10-day-old infant with a past medical history of complex
congenital heart disease was admitted postoperatively to Underwent arterial switch procedure at 3 days of age.
the pediatric CICU 1 week ago following cardiac surgery
in which he underwent an arterial switch procedure. He
Family History
had a difficult operative course, but has been recovering
well and is hemodynamically stable. He remains endotra-
Parents and 1-year-old sibling are healthy.
cheally intubated and mechanically ventilated but is toler-
ating a slow wean of support. Five days ago he had an
arterial catheter placed in the right femoral artery and also
has a peripherally inserted central catheter in the right
Current Status
lower extremity which ends in the femoral vein. Yesterday,
Vital signs are as follows: temperature is elevated to 38 C
during a dressing change, the nurse noted that the area
(100.4 F), heart rate is 121 bpm, respiratory rate 32 breaths
around the arterial catheter in the right groin was swollen
per minute, blood pressure 70/40 mmHg, and oxygen satu-
and had purple discoloration to the skin, but blood return
ration 98% on 30% FiO2 on invasive mechanical ventilatory
from the cannula continued (Figure 112.1). The arterial
support. The daily basic metabolic panel and complete
catheter was removed, but today the area of discoloration
blood count are obtained with results as noted in
is starting to ulcerate and there are new areas of dark
Tables 112.1 and 112.2. A chest X-ray shows good position
and necrotic skin (Figure 112.2).
of endotracheal tube with no infiltrates. An arterial blood
gas analysis shows a pH of 7.43, PaCO2 of 51 mmHg, PaO2
of 74 mmHg, and a bicarbonate of 28 mmol/L. Lactic acid
Past Medical History is 0.8 mmol/L. C-reactive protein is 4.2 mg/L.

The patient was born at 39 weeks’ gestation via cesarean

section. There were no pregnancy complications and his
mother was healthy with negative prenatal laboratory
Questions
results. His cardiac abnormalities were discovered prena-
Answer the following questions using the details provided.
tally on ultrasound. After birth, he was admitted to the neo-
natal intensive care unit (NICU) and started on 1 Based on the data given, what are your differential diag-
prostaglandin infusion to maintain patency of his ductus noses and the most likely diagnosis for this infant?

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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468 Cases in Pediatric Acute Care

Figure 112.1 Discoloration of skin. Source: Courtesy of Megan Figure 112.2 Areas of ulceration. Source: Courtesy of Megan
Boone, MSN, RN. Boone, MSN, RN.

2 What is the best next step in the management of this Table 112.1 Basic metabolic profile.
infant?
3 Would you obtain any other diagnostic studies at Sodium 131 mEq/L
this time? Potassium 3.7 mEq/L
Chloride 96 mEq/L
Carbon dioxide 28 mEq/L
Blood urea nitrogen 26 mg/dL
Rationale and Evidence-based Practice Creatinine 0.5 mg/dL
Explanation Glucose 92 mg/dL

Based on the data given, what are your differential Table 112.2 Complete blood count.
diagnoses and the most likely diagnosis for this
infant? White blood cell count 20 400/mm3
Hemoglobin 11.6 g/dL
Differential diagnoses for this infant include cellulitis,
necrotizing fasciitis, pyoderma gangrenosum, Fournier’s Hematocrit 36%
gangrene, intravenous extravasation, and deep venous Platelet count 334 000/mm3
thrombosis. The most likely diagnosis is necrotizing Neutrophils 62%
fasciitis given the clinical picture and rapid progression. Eosinophils 1%
Necrotizing fasciitis is an infection of the deep soft tissues Monocytes 8%
which results in necrosis of muscle fascia and overlying Lymphocytes 30%
subcutaneous tissue. The infection typically begins with
Bands 0%
trauma to, or a break in, the skin and rapidly spreads to
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Chapter 112 Skin Wound in Postoperative Neonate 469

the muscle fascia. Necrotizing fasciitis is considered a med-

ical and surgical emergency.
The initial presentation consists of pain, edema, and ery-
thema of the soft tissue. The area will feel firm and indu-
rated. These skin changes are similar to those of cellulitis
or abscess, making early diagnosis difficult. However, as
necrotizing fasciitis progresses, a purple discoloration of
the skin may be noted due to bleeding from damaged
blood vessels, bullae formation, ulceration, or presence
of gas in the tissue which results in crepitus. Patients
may or may not develop fever. This condition can rapidly
progress and result in sepsis and septic shock despite anti-
biotic therapy.
Necrotizing fasciitis is caused by bacterial infection, most
commonly with a mixture of various types of bacteria, spe-
cifically Gram-positive cocci including Streptococcus, Figure 112.3 Erythematous spread to scrotum and right lower
Gram-negative rods including Escherichia coli, and anae- extremity. Source: Courtesy of Megan Boone, MSN, RN.
robes such as Clostridium species.

What is the best next step in the management of

this infant?
The diagnosis of necrotizing fasciitis is confirmed with
surgical exploration of the tissues in the operating room.
It is very important not to delay surgical intervention when
there is a clinical suspicion for necrotizing fasciitis, as
delays in surgery are associated with a higher risk of
mortality. The infection is treated with surgical debride-
ment of necrotic tissue and intravenous antibiotics. Antibi-
otic therapy should include activity against Gram-positive,
Gram-negative, and anaerobic organisms.

Would you obtain any other diagnostic studies at

this time?
When necrotizing fasciitis is suspected, laboratory test-
ing is reasonable including inflammatory markers of
infection. Blood cultures should be obtained prior to
Figure 112.4 Erythematous spread to scrotum and right lower
starting antibiotics, if possible. Computed tomography
extremity. Source: Courtesy of Megan Boone, MSN, RN.
(CT) may be able to help determine whether a necrotiz-
ing infection is present, but should not delay surgical
intervention. Additional Question and
Evidence-based Rationale
Continuation of case: Over the next several hours, the
surrounding tissue becomes erythematous and the redness 4 What other therapies should be considered at this time?
spreads rapidly to the scrotum and right lower extremity
(Figures 112.3 and 112.4). The patient is empirically started
What other therapies should be considered at
on broad-spectrum antibiotics including clindamycin, cef-
this time?
triaxone, and vancomycin. The plastic surgery service is
emergently consulted and the patient is taken to the oper- With progression of infection despite antibiotic therapy, the
ating room for surgical exploration and wound debride- patient may show signs of sepsis or septic shock. Fluid
ment. Blood cultures and cultures from the right groin resuscitation and careful monitoring of vital signs, blood
wound are obtained. pressure, and perfusion are important.

470 Cases in Pediatric Acute Care
Further Reading
Hakkarainen, T.W., Kopari, N.M., Pham, T.N., and Evans, H.L.
(2014). Necrotizing soft tissue infections: review and current
concepts in treatment, systems of care, and outcomes.
Current Problems in Surgery 51(8): 344–362.
Stevens, D.L. and Bryant, A.E. (2017). Necrotizing soft-tissue
infections. New England Journal of Medicine 377(23):
2253–2265.
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Stevens, D.L., Bisno, A.L., Chambers, H.F., et. al. (2014).
Practice guidelines for the diagnosis and management of
skin and soft tissue infections: 2014 update by the Infectious
Diseases Society of America. Clinical Infectious Diseases 59
(2): e10–e52.

113
Provider Burnout and Resiliency
Jill Siegrist Thomas
University of Maryland Children’s Hospital, University of Maryland Medical Center, Baltimore, MD, USA
A novice acute-care provider is working in an intermediate-
level pediatric burn unit. She completed a structured orien-
tation 3 months ago and works a rotating day and night
schedule. Clinical responsibilities include the comprehen-
sive diagnosis, assessment and management of infants
and children status post second- and third-degree thermal
and chemical burns. She is active in multidisciplinary
rounds, frequently participates in the admission, transfer
and discharge of patients, and performs moderate sedation
and/or sterile dressing changes daily for assigned patients.
She often works long shifts to complete the required written
documentation and research the literature related to the
patient population. While she states she enjoys her job,
she feels her new occupation consumes her life and has lit-
tle time outside of work to participate in activities she for-
merly enjoyed.
Questions
Answer the following questions using the details provided.
1 What concerns or identified problems are included in
this scenario?
Continuation of case: During multidisciplinary rounds
the novice provider presents her plan to moderately sedate
a patient with ketamine and midazolam. The patient’s
nurse quickly interjects to state her preference of using pro-
pofol for moderate sedation due to institutional “norm.”
The provider educates the nurse that propofol is contrain-
dicated in this patient due to an egg allergy, but feels frus-
trated and undermined and interprets the interaction as not
being trusted by the nursing staff. During the dressing
change the surgical resident does not know she independ-
ently performs dressing changes and tries to educate her on
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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471
how to perform a dressing change. She again feels her
colleagues are questioning her abilities and she is underap-
preciated. She leaves work feeling defeated and questions if
she’s made the right career change.
2 What are the symptoms of burnout syndrome?
3 What interventions potentially prevent burnout syn-
drome and enhance personal well-being?
4 What strategies can this provider employ to foster
resiliency?
Rationale and Evidence-based Practice
Explanation
What concerns or identified problems are included
in this scenario?
Personal characteristics, organizational factors, quality of
working relationships, and exposure to end-of-life issues
place individuals at risk for developing “burnout syn-
drome.” Personal risk factors associated with burnout syn-
drome include work–life imbalance, engagement in
unhelpful coping strategies, sleep deprivation, and being
self-critical, perfectionistic and over-committed. Organiza-
tional factors associated with burnout syndrome include a
highly pressured or chaotic work environment, increasing
workload, loss of control of the work environment, process
or schedule, insufficient rewards, and breakdown of the
work community. These risk factors can lead to unclear
or overly demanding occupational responsibilities, perfor-
mance of menial tasks, and poor alignment of personal
values with organizational leadership (Moss et al., 2016;
Pastores et al., 2019). Poor working relationships can lead
to conflict and foster ineffective communication. Strained
relationships can occur between colleagues, supervisors,

472 Cases in Pediatric Acute Care
Table 113.1 Key resiliency competencies and development strategies.
Competency Associated attributes
Reflective Recognize own strengths and limitations
ability
Emotional Self-awareness: recognize own emotions and their effects,
intelligence accurate self-assessment, have self-confidence (sure about
self-worth)
Self-management: manage disruptive emotions and
impulses, conscientious, self-motivated demonstrating
commitment, initiative and optimism
Social awareness: empathy and culturally sensitive patient-
centered care
Social skills: collaborative, cooperative, skilled
communicator
Social Social confidence, assertiveness, and well-developed
competency communication and conflict resolution skills
Social
support
Positive interactions with others with whom there is a
mutual trust and respect
and patients and their families and is a prominent risk
factor for burnout.
What are the symptoms of burnout syndrome?
Heathcare providers need to recognize the symptoms of
burnout syndrome and seek personal assistance and sup-
port. Symptoms are vague, develop gradually, and can be
both psychological and physical in nature. Psychological
symptoms include feelings of job-related disillusionment,
feeling insufficient at work, anger, frustration, anxiety, dis-
content, and the inability to feel happiness or pleasure.
Physical symptoms include fatigue, exhaustion, insomnia,
headache, muscle tension, and gastrointestinal problems
(Moss et al., 2016).
What interventions potentially prevent burnout
syndrome and enhance personal well-being?
Interventions focused on preventing burnout target the
organizational and personal drivers. Social context and inter-
action with colleagues play a pivotal role in the development
of burnout. Promotion of a healthy work environment
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Development strategies
•• Journaling: narrative writing
••
Debriefing
Utilize a coach or mentor
Find meaning in work and setting realistic short-term
and long-term goals
• Experiential learning, e.g. simulated practice, case
•
studies to facilitate dialogue and reflection
Attend professional conferences, workshops, etc. to
•
build communication, leadership skills
Exercise skills/strategies to reduce stress and frustration
in work environment (e.g. “step away”, take a deep
•
breath, “act rather than react,” establish boundaries)
Maintain physical health (e.g. exercise, obtain adequate
•
sleep, practice good nutrition)
Practice gratitude
• Exercise mindfulness and relaxation techniques
••
(e.g. yoga, meditation, deep breathing activities)
Find time for yourself every day
••
Celebrate successes
Develop rituals to release tension
•
Maintain sense of humor
Ask for help when needed
•• Mentoring and peer coaching
Foster professional relationships and create a caring
community: connect with peers, friends and family on a
•
regular basis
Participate in a novice network (e.g. organizational NP
•
committee)
Develop hobbies outside of work
includes communication training to reduce interpersonal
conflict, appropriate staffing, and meaningful recognition
for completion of occupational duties. Providers may
benefit from self-scheduling practices and time off. Support
groups and cognitive therapy can address the cause of
moral distress and provide coping skills to increase perso-
nal resilience and emotional intelligence.
Team-based interventions to optimize team culture
include use of structured communication tools, team
building, interpersonal skills training activities, and team
debriefings. Healthcare providers are encouraged to partici-
pate in stress reduction training, meditation or relaxation
techniques to increase coping skills. Self-care measures
include provision of adequate rest, exercise, and assurance
of work–life balance through time management and
engagement in social activities (Pastores et al., 2019). The
development of personal coping skills emphasizing the
core principles of emotional intelligence (see Table 113.1) –
self-awareness, self-management, social awareness and
social skills – is essential to optimize personal wellness,
improve stress management skills, and prevent burnout
(Shahid et al., 2018).

What strategies can this provider employ to foster
resiliency?
Resiliency is defined as the capacity to recover after a stress-
ful situation and this attribute allows individuals to thrive
and succeed in the face of adversity and stressful situations
References
Moss, M., Good, V.S., Gozal, D., Kleinpell, R., and Sessler, C.N.
(2016). An official critical care societies collaborative
statement: burnout syndrome in critical care healthcare
professionals: a call for action. Chest 150(1): 17–26. doi:
10.1016/j.chest.2016.02.649.
Pastores, S., Kvetan, V., Coopersmith, C., et al. (2019).
Workforce, workload, and burnout among intensivists and
advanced practice providers: a narrative review. Critical
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Chapter 113 Provider Burnout and Resiliency 473
rather than merely survive. The foundation for building
resiliency is multidimensional and focuses on self-care
and self-awareness. Table 113.1 outlines key resiliency
competencies and associated developmental strategies.
Care Medicine 47(4): 550–557. doi: 10.1097/
CCM.0000000000003637.
Shahid, R., Stirling, J., and Adams, W. (2018). Promoting
wellness and stress management in residents through
emotional intelligence training. Advances in Medical
Education and Practice 9: 681–686. doi: 10.2147/AMEP.
S175299.

114
Kidney Transplant Complication
Cathy C. McAdams
Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE, USA
A 17-year-old African American female with a history of
focal segmental glomerulosclerosis (FSGS) is now 1 year
post successful deceased donor kidney transplant. She
was admitted to the hospital for lethargy, diarrhea, fever,
decreased urine output, and an elevated creatinine.
History of Present Illness
This teen was well up until 2 days ago when she started
feeling tired. She denies any cold symptoms, headaches,
dizziness, vomiting or edema. She does report loose stools
(four to six times over the last 48 hours), decreased oral
fluid intake, decreased urine output, and mild burning with
urination. She has not been checking her temperature at
home because she reports not being able to find the
thermometer, but she states that she feels hot (especially
at night) and has been having chills.
Past Medical History
The patient was born full term with no known complica-
tions. Prior to presentation of her renal disease, her health
history consisted of “several ear infections as a child” and
extraction of molars 3 years ago. Two years ago, the teen
went to the dentist and was found to be hypertensive with
blood pressure 180/110 mmHg She was sent to the local
emergency department where hypertension was confirmed
and laboratory studies obtained. At that time, her creati-
nine was 7.6 mg/dL, and she had large amounts of protein
and blood in her urine. She was sent to a pediatric nephrol-
ogist and underwent a percutaneous kidney biopsy and
subsequently was diagnosed with FSGS and started on
hemodialysis three times a week. After 12 months, she
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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475
received a deceased donor transplant. At the time of trans-
plant, the patient was EBV (Epstein–Barr virus) and CMV
(cytomegalovirus) negative and the donor was both EBV
and CMV positive. Creatinine at discharge was 0.6 mg/
dL, on intravenous fluids; her creatinine post discharge
has been stable at 0.6–0.8 mg/dL depending on oral intake.
Her blood pressure on twice daily amlodipine is 118/
68 mmHg. Her medication regimen is tacrolimus 2 mg
twice daily, mycophenolate mofetil 750 mg twice daily,
prednisone 5 mg daily, amolodipine 5 mg twice daily,
and vitamin D 1000 mg daily.
Past Surgical History
Deceased donor kidney transplant and central dialysis cath-
eter removal after transplant.
Family History
Family history is negative for kidney disease or kidney
stones.
Current Status
The teen presents to the emergency room with diarrhea and
malaise. Her temperature is 39 C (102.3 F), heart rate 135
bpm, and blood pressure 90/42 mmHg. Her weight is 48 kg,
down about 2 kg from baseline and she reports feeling light-
headed. A chest X-ray, transplant ultrasound, urine culture,
and blood cultures are obtained. Blood work on admission
reveals a serum creatinine of 1.4 mg/dL, about a 40% reduc-
tion in her glomerular filtration rate (GFR). The patient is
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476 Cases in Pediatric Acute Care

admitted and a percutaneous kidney biopsy is scheduled for Table 114.3 Urinalysis.
the next day.
Color Dark yellow
Results of a comprehensive metabolic panel, complete
blood count, and urinalysis are shown in Tables 114.1–114.3. pH 6.5
The chest X-ray image is normal, while transplant ultra- Specific gravity 1.030
sound shows no hydronephrosis, although the kidney Protein 2+
may have slightly increased echodensity, thought to be Nitrites Positive
artifactual. Urobilirubin 1+
Blood Large
Leukocytes Positive
Questions Ketones Negative
Glucose Negative
Answer the following questions using the details provided.
1 Based on the data given, what are the most likely differ-
ential diagnoses for this patient?
2 What therapy would you consider starting to try to 3 How would you determine if the therapy is effective?
improve the patient’s kidney function? 4 What additional studies should be ordered?
5 What therapies could be offered if the creatinine does
not improve with rehydration?
Table 114.1 Comprehensive metabolic panel.

Sodium 138 mEq/L

Potassium 5.4 mEq/L Rationale and Evidence-based Practice
Chloride 110 mEq/L Explanation
Carbon dioxide 16 mEq/L
Blood urea nitrogen 42 mg/dL Based on the data given, what are the most likely
Creatinine 1.4 mg/dL differential diagnoses for this patient?
Glucose 126 mg/dL For a stable transplant patient who has had an acute
Calcium 9.8 mg/dL increase in a previously stable creatinine, the differential
Albumin 3.0 mg/dL diagnosis should include:
Total protein
Aspartate aminotransferase
6.0 g/dL
24 U/L • a pre-renal state secondary to poor perfusion related to
diarrheal dehydration and hypotension;
Alanine aminotransferase
Alkaline phosphatase
24 U/L
60 U/L
• intrarenal injury related to drug toxicity (tacrolimus),
rejection, recurrence of her FSGS in the transplanted kid-
ney, or viral/bacterial-related infection;

•
Total bilirubin 0.5 mg/dL
Magnesium 1.6 mEq/L post-renal obstruction related to a kidney stone or
blood clot.
Phosphorus 4.2 mg/dL

What therapy would you consider starting to try

Table 114.2 Complete blood count. to improve the patient’s kidney function?
White blood cell count 10 500/mm3 Because of the patient’s diarrhea and dehydration along
Hemoglobin 12.2 g/dL with weight loss, initial therapy would be administration
Hematocrit 36.6% of an intravenous fluid bolus of normal saline followed
Platelet count 195 000/mm3 by an increased maintenance rate of 1.5 times normal.
Neutrophils 36%
How would you determine if the therapy is
Lymphocytes 42%
effective?
Monocytes 13%
Eosinophils 1% An improvement in the serum creatinine, with return to
baseline values, on rehydration would indicate that the

elevated serum creatinine was due to something that was
either transient or reversible and related to dehydration.
The clinician would also be reassured if the patient
became normotensive, and had a clean urine (free of
protein, blood and/or leukocytes) and near-normal serum
albumin.
What additional studies should be ordered?
In addition to the laboratory and imaging studies already
carried out, it would be important to obtain a tacrolimus
level, viral PCR for EBV, CMV and BK polyoma virus, as
well as donor-specific antibodies. A random urine pro-
tein/creatinine ratio should be sent as well; this would
ideally be a first morning sample.
What therapies could be offered if the creatinine
does not improve with rehydration?
Intravenous fluids should be infused at 1.5 times mainte-
nance if the diarrhea continues. Antidiarrheal agents could
be administered in the short term to prevent loss of electro-
lytes and tacrolimus toxicity. Laboratory work should be
repeated 8–12 hours after starting intravenous fluids to
determine a trend in the creatinine and to evaluate electro-
lytes. Because the urine is positive for blood, protein, and
leukocytes, and the patient had urinary tract symptoms,
antibiotics should be started after the blood and urine cul-
tures are obtained and continued until the 24-hour culture
results are obtained.
Continuation of case: Over the first 12 hours, the
serum creatinine improves to 1.0 mg/dL (still above
baseline), and her blood pressure and hydration status
normalized. Her pre-renal state is reversed and she has
no evidence of obstruction on ultrasound. Her differen-
tial diagnoses at this point still include recurrence of
disease, resolving dehydration effect, tacrolimus toxicity,
acute renal injury, acute rejection, or urinary tract
infection.
Additional Question and
Evidence-based Rationale
6 Based on these supplementary data, what are now the
most likely differential diagnoses for this patient?
Based on these supplementary data, what are now
the most likely differential diagnoses for this
patient?
FSGS is an illness of the kidney that causes scarring in the
glomeruli and can lead to kidney failure. There is about a
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Chapter 114 Kidney Transplant Complication 477
30% chance of recurrence in the transplanted kidney.
Although this is more common immediately after trans-
plant, it can occur at any point and should remain in
the differential, especially if the patient is noted to have
proteinuria and a lower serum albumin.
Dehydration secondary to diarrhea and decreased fluids
is the most likely cause of the patient’s increased serum cre-
atinine. She has experienced several days of frequent loose
stools and a decrease in her urine output from baseline.
Tacrolimus toxicity (or an elevated tacrolimus level) can
be the result of persistent diarrhea and can also result in
an increase in the serum creatinine. Diarrhea and dehydra-
tion, especially if the tacrolimus level is increased, can also
lead to acute renal injury.
Rejection of the kidney transplant can occur at any time.
Although the cause of this in the early months can be
purely immunologic, non-compliance should be consid-
ered in the patient who is further out from transplant. Teen-
agers in particular are at increased risk of losing kidneys
because of skipping or not taking their immunosuppressant
medications.
Another thing to consider in this patient who has previ-
ously had no evidence of CMV or EBV would be a change in
viral status.
Finally, the increase in the creatinine could be the result
of an infection, and in this case where the urine is positive
for protein, blood and leukocytes, there should be higher
suspicion of a urinary tract infection until the urine culture
results show otherwise. In the presence of a fever and
urinary tract symptoms, initiation of antibiotics is war-
ranted while waiting on the urine culture results. Urinary
tract infections (especially pyelonephritis) can damage
the transplanted kidney causing acute or chronic injury.
Case Resolution
At 24 hours post admission, the creatinine has returned to
baseline (0.8 mg/dL), and blood and urine cultures are
negative. The tacrolimus level was elevated to 15 ng/mL
on admission and returned to 5 ng/mL with improvement
of diarrhea and hydration. The patient’s urine has
improved, with only trace blood and protein with negative
leukocytes. Blood and urine cultures were negative at
24 hours and antibiotics were discontinued. At this time,
with a baseline creatinine, no proteinuria, and negative
cultures, recurrence, rejection, infection, and acute kidney
injury related to dehydration or tacrolimus toxicity can be
excluded. The decision was made to cancel her transplant
biopsy and the patient was discharged home on her rou-
tine medications.
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478 Cases in Pediatric Acute Care

Further Reading
Avery, R.K. and Fishman, J.A. (2011). Infectious diseases in Rodig, N.M., Vakili, K., and Harmon, W.E. (2016). Pediatric
transplantation. In: Primer on Transplantation, 3rd edn renal transplantation. In: Pediatric Nephrology, 7th edn
(ed. D. Hricik), 51–72. Chichester: Wiley-Blackwell. (ed. E.D. Avner, W.E. Harmon, P. Niaudet et al.), 2519–2534.
Dharnidharka, V.R. and Araya, C.E. (2016). Complications New York: Springer.
of pediatric renal transplantation. In: Pediatric Nephrology,
7th edn (ed. E.D. Avner, W.E. Harmon, P. Niaudet et al.),
2574–2578. New York: Springer.

115
Infant with Influenza A and New Fever
Lauren Flagg
Yale New Haven Hospital, New Haven, CT, USA
A 4-month-old female, born at 28 weeks, is admitted to the
pediatric intensive care unit (PICU) with respiratory failure
secondary to influenza A infection, now with new onset
of fever.
History of Present Illness
The infant presented to the emergency department (ED)
14 days prior with fever, respiratory distress, lethargy,
and poor oral intake. Her older brother had tested positive
for influenza A several days earlier with similar symptoms.
She was initially admitted to the regular nursing floor for
management. Shortly after arrival, her work of breathing
worsened and she required transfer to the PICU for escala-
tion of monitoring and management. She required intuba-
tion for respiratory failure, with subsequent placement of a
femoral central venous catheter and radial arterial line.
Since PICU admission she has slowly defervesced, with
hemodynamic stability and improved ventilation and oxy-
genation on weaning ventilator settings. Over the past
6 hours, however, she has developed fever to 39.5 C
(103.1 F) with hypotension requiring fluid resuscitation
and initiation of inotropic support.
Past Medical History
The child was born at 28 weeks’ gestation to a gravida 3
para 2 mother with incompetent cervix. Maternal screening
for transmittable infections was unrevealing. She was ini-
tially admitted to the neonatal intensive care unit
(NICU), requiring intubation for 2 days, but was in room
air prior to discharge at age 10 weeks of life. She is fed a for-
tified infant formula by mouth and prior to this admission
had been gaining weight appropriately since NICU
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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479
discharge. A small patent ductus arteriosus was noted
shortly after delivery, for which no intervention has been
required. She is followed by ophthalmology for retinopathy
of prematurity. Her immunizations are up to date.
Past Surgical History
None.
Family History
Has a 4-year-old brother who is otherwise healthy, as are
both parents.
Current Status
The infant is intubated, sedated, mechanically ventilated,
and receiving inotropic support. Mean arterial pressure is
maintained above 45 mmHg on epinephrine 0.1 μg/kg
per min. Over the past 6 hours she has received a total of
40 mL/kg in fluid resuscitation. Her current temperature
is 38.5 C (101.3 F) rectally after 15 mg/kg of intravenous
acetaminophen. Lung sounds are clear with good chest wall
excursion bilaterally. She is tachycardic without cardiac
murmurs. Capillary refill is 5 seconds. There are no rashes.
Central line and peripheral intravenous catheter sites are
without signs of inflammation. A basic metabolic panel
and a complete blood count with differential are obtained.
A chest radiograph from earlier in the day demonstrated
stable, patchy perihilar infiltrates. Respiratory secretions
are unchanged, thin and white. Urine output is decreased
from baseline, but clear and without odor.
Laboratory results are shown in Tables 115.1 and 115.2.
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480 Cases in Pediatric Acute Care

Table 115.1 Basic metabolic profile. Tracheitis should also be considered due to previous viral
illness and prolonged intubation, though is unlikely in
Sodium 140 mEq/L
the setting of unchanged respiratory secretions. Because
Potassium 3.9 mEq/L of her young age, meningitis should also be considered;
Chloride 108 mEq/L however, this infant is not displaying any meningitic signs
Carbon dioxide 18 mEq/L of changes in mental status, so this would be low on the dif-
Blood urea nitrogen 16 mg/dL ferential diagnosis. Another consideration for the differen-
Creatinine 0.4 mg/dL tial diagnosis includes urinary tract infection (UTI), which
Glucose 101 mg/dL
is the second most common infection in the intensive care
unit. However, most UTIs are associated with indwelling
urinary catheters, not present in this infant. Finally, a
new respiratory virus should also be considered on the dif-
Table 115.2 Complete blood count.
ferential diagnosis. While this is possible, it is less likely due
White blood cell count 30 000/mm3 to the unchanged respiratory examination and the promi-
Hemoglobin 11 g/dL nent hemodynamic instability.
Hematocrit 33%
What additional studies would you order?
Platelet count 425 000/mm3
Neutrophils 88% Blood cultures should be obtained in a patient with new
Monocytes 6% fever when the history and clinical evaluation do not indi-
Lymphocytes 6% cate a non-infectious cause. Guidelines recommend that in
patients with indwelling vascular catheters, at least two
blood cultures should be obtained, one from peripheral
venipuncture and one from the intravascular catheter with
Questions suspected infection. Obtaining acute-phase reactants, such
as procalcitonin, may help to evaluate the risk of bacterial
Answer the following questions using the details provided.
infection as the cause of fever. Procalcitonin is specific to
1 Based on these data, what differential diagnoses would bacterial infections. Viral infection, surgery, and chronic
you consider as the cause of the new fever? inflammation are not associated with increased procalcito-
2 What additional studies would you order? nin levels. A procalcitonin level of less than 0.5 ng/mL indi-
3 What empiric management would you begin and why? cates that bacterial infection is not present; levels of 0.6–2
ng/mL are consistent with systemic inflammatory response
syndrome; levels of 2–10 ng/mL are indicative of severe sep-
sis; and levels above 10 ng/mL indicate septic shock. Addi-
Rationale and Evidence-based Practice tionally, to exclude other foci of infection, respiratory and
Explanation urinary cultures are valuable to evaluate for ventilator-
associated pneumonia, tracheitis, and UTI.
Based on these data, what differential diagnoses
would you consider as the cause of the new fever? What empiric management would you begin
and why?
In this case, the combination of leukocytosis and hemody-
namic instability requiring volume resuscitation and ino- Antibiotic coverage should begin after cultures are
tropic support would suggest an infectious process. obtained. However, inability to obtain cultures (such as a
Leading considerations include the following: lumbar puncture) should not delay initiation of antibiotic
Central line-associated bloodstream infection (CLABSI) administration. In this case, the primary concern is the
is high on the differential diagnosis as she has an existing development of CLABSI due to the severity of the clinical
central line and hemodynamic instability. This is the most picture and shock-like state. Empiric antibiotic therapy
likely cause, but other sources of severe infection should should be selected to cover the most common CLABSI
also be considered. organisms, with consideration to common facility-specific
Pneumonia should be considered due to previous respira- nosocomial pathogens as well as institutional protocols.
tory illness with ongoing intubation. It is less likely in the The most common CLABSI pathogens are staphylococcal
setting of normal respiratory examination, normal chest species (including Staphylococcus aureus and the coagu-
radiograph, and unchanged ventilator requirements. lase-negative staphylococci) and enteric Gram-negative

bacilli (such as Escherichia coli and Klebsiella spp.). In
settings with significant rates of methicillin-resistant
S. aureus (MRSA), as well as in patients with high suspicion
for infection (such as known colonization), vancomycin
should be included in the initial therapy. When deter-
mining coverage for Gram-negative bacilli, both local
susceptibility data and the severity of infection should
be considered. Coverage typically should include a
fourth-generation cephalosporin such as carbapenem, or
a β-lactam/β-lactamase inhibitor combination, with or
without the addition of an aminoglycoside. Patients in
whom Pseudomonas aeruginosa is a concern (i.e. those with
immunocompromise, severe sepsis, or known colonization)
should receive an antibiotic regimen with appropriate
coverage. Concern for CLABSI in patients with femoral
vascular access lines should include coverage for Gram-
negative bacilli, with consideration given to Candida spp.
Continuation of case: The patient is empirically started
on vancomycin and cefotaxime after obtaining peripheral
and central blood cultures, urine culture, and respiratory
cultures. Lumbar puncture is deferred due to hemo-
dynamic instability. Procalcitonin resulted at 4.5 ng/mL.
Twelve hours later, preliminary results from both blood
cultures are positive for Gram-positive cocci in clusters.
Perfusion remains decreased, and she continues to receive
epinephrine drip at 0.1 μg/kg per min without further
advancement.
Further Reading
American Academy of Pediatrics (2011). Urinary tract
infection: clinical practice guideline for the diagnosis and
management of the initial UTI in febrile infants and
children 2 to 24 months. Pediatrics 128(3): 595–610. doi:
10.1542/eds.2011-1330.
Mermel, L.A., Allon, M., Bouza, E., et al. (2009). Clinical
practice guidelines for the diagnosis and management of
intravascular catheter-related infection: 2009 update by the
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Chapter 115 Infant with Influenza A and New Fever 481
Additional Questions and
Evidence-based Rationale
4 Based on the blood culture results, what pathogens are
most likely?
5 What additional management changes would you
prioritize?
Based on the blood culture results, what
pathogens are most likely?
Staphylococcal species, specifically S. aureus due to the
severity of the disease. Consider the likelihood for MRSA
based on facility and patient risks.
What additional management changes would you
prioritize?
Removal of central lines in cases of severe CLABSI is a
priority in management. Because of hemodynamic instabil-
ity, she will require a new central line to facilitate manage-
ment. Placement of a new central venous catheter in a new
location is preferable to exchanging the existing line over a
guidewire. Consultation with infectious diseases is recom-
mended for ongoing therapy. Empiric antibiotics should be
continued pending speciation and susceptibilities of the
bacteria, after which coverage may be narrowed.
Infectious Diseases Society of America. Clinical Infectious
Diseases 49(1): 1–45. doi: 10.1086/599376.
O’Grady, N.P., Barie, P.S., Bartlett, J.G., et al. (2008).
Guidelines for evaluation of new fever in critically ill adult
patients: 2008 update from the American College of Critical
Care Medicine and the Infectious Diseases Society of
America. Critical Care Medicine 36(4): 1330–1349. doi:
10.1097/CCM.0b013e318169eda9.

116
A 3-Year-Old with Fever and Painful Urination
Roseann Hausmann
Seattle Children’s Hospital, Seattle, WA, USA
A 3-year-old male presents to the emergency department
(ED) with a fever 38.9 C and painful urination.
History of Present Illness
This 3-year-old male began with fever 24 hours ago which
has been responsive to acetaminophen and ibuprofen at
home. He was potty-trained at 22 months of age, and has
recently begun to have episodes of incontinence. This after-
noon he began complaining that it hurt to go to the bath-
room, prompting parents to bring him to the ED.
Past Medical History
The child was born at term and went home with his mother.
He was not circumcised. His immunization status is up to
date and he has continued to grow along the curve for both
height (50th percentile) and weight (40th percentile). He
had two urinary tract infections (UTIs) as an infant, and
was diagnosed with grade 3 vesicoureteral reflux (VUR)
and is taking prophylactic sulfamethoxazole trimethoprim.
The parents report he has not missed any doses of medica-
tion in the previous month. He was hospitalized at 6 months
for a UTI with urosepsis, but has had no other hospital
admissions.
Past Surgical History
None.
Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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483
Family History
The patient’s father has a history of chronic kidney disease
of unknown etiology that requires dialysis.
Current Status
On arrival at the ED the child appeared ill with a rectal
temperature of 39.3 C (102.7 F). He was tachycardic
with heart rate 150 bpm, blood pressure 80/50 mmHg,
and respiratory rate of 40 breaths per minute. He
received intravenous acetaminophen and a normal
saline fluid bolus of 20 mL/kg. His vital signs have
now stabilized, with temperature 38 C (100.4 F), heart
rate 110 bpm, blood pressure 90/60 mmHg, and respira-
tory rate 25 breaths per minute. Results of complete
blood count, basic metabolic panel, and urinalysis are
shown in Tables 116.1–116.3; results of urine culture
and blood culture are pending.
Questions
Answer the following questions using the details
provided.
1 What is the differential diagnosis and the most likely
diagnosis for this child?
2 What additional testing would be warranted?
3 What medications would you anticipate starting for this
patient?
4 Is surgical intervention indicated for this child?
5 What anticipatory guidance should be provided to the
family?
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484 Cases in Pediatric Acute Care

Table 116.1 Complete blood count. VUR occurs when there is passage of urine from the blad-
der into the upper urinary tract. This predisposes patients to
White blood cell count 5400/mm3
recurrent UTIs as well as acute pyelonephritis due to the
Hemoglobin 11.4 g/dL transportation of bacteria from the bladder into the kidney.
Hematocrit 36% Left untreated, recurrent UTIs may result in renal scarring,
Platelet count 172 000/mm3 hypertension, and eventually end-stage renal disease. VUR
can be primary, due to inadequate closure of the ureterove-
sical junction (UVJ) or to a congenitally short intravesical
Table 116.2 Complete metabolic profile. ureter; or secondary, caused by high pressure in the bladder
that prevents the UVJ from closing during contraction,
Sodium 138 mEq/L
often due to anatomic or functional bladder obstruction.
Potassium 3.9 mEq/L Patients with primary VUR may have spontaneous resolu-
Chloride 100 mEq/L tion, with lower grades having the highest likelihood of this
Carbon dioxide 29 mEq/L response.
Blood urea nitrogen 12 mg/dL
Creatinine 0.24 mg/dL What additionally testing would be warranted?
Glucose 270 mg/dL
A renal and bladder ultrasound is recommended to assess
for pyelonephritis, or perirenal abscess. Close monitoring of
Table 116.3 Urinalysis. vital signs and blood pressure is also warranted. In a patient
who had not previously had a voiding cystourethrogram
Color Dark yellow (VCUG), this would be indicated given that it is a second
Clarity Cloudy febrile UTI and occurred while on prophylactic antibiotics.
pH 5 VCUG is recommended to be completed after resolution of
Specific gravity 1.022 symptoms, while still on antibiotics, or immediately after
the discontinuation of antibiotics.
Glucose 100 mg/dL
Nitrites Positive
Ketones Negative What medications would you anticipate starting
Leukocyte esterase Positive for this patient?
Bilirubin Negative Escherichia coli is the most common cause of UTI in chil-
Protein 100 mg/dL dren, so coverage of Gram-negative bacteria is impera-
Red blood cell count 1 /hpf tive. The patient will require parenteral inpatient
White blod cell count 1 /hpf antibiotic therapy, since he is ill-appearing on presenta-
tion. Antibiotic choice should be influenced by the resist-
ance pattern of E. coli in the local area; in this case the
Rationale and Evidence-based Practice agent of choice was ceftriaxone. Other choices would
include antibiotics in the cephalosporin category, includ-
Explanation
ing cefdinir, ceftibuten, and cefotaxime for best coverage
of Gram-negative organisms, or gentamicin. Once the
What is the differential diagnosis and the most
child has clinically improved and is able to tolerate oral
likely diagnosis for this child?
antibiotics, the agent can be switched based on culture
The differential diagnoses for this patient include UTI with susceptibilities.
urosepsis secondary to VUR, acute viral infection, or sepsis
of unknown origin.
Is surgical intervention indicated for this child?
The most likely diagnosis in this case would be UTI with
urosepsis secondary to VUR. The history of being uncir- Watchful waiting can be successful in the treatment of
cumcised and having VUR, as well as the increased epi- patients who have VUR and this decision was made for
sodes of incontinence in a previously potty-trained child this patient after his initial diagnosis of VUR. He was
and pain with voiding, provide a compelling history for this placed on antibiotic prophylaxis with sulfamethoxazole
diagnosis. Urinalysis demonstrating positive nitrites and trimethoprim during this time. Surgery is needed in
leukocyte esterase also supports the diagnosis. patients who persist with significant reflux past 3 years

of age, as the likelihood of self-resolution decreases at this
point. It is also indicated in patients with higher grade
reflux (>3 years old) who have breakthrough infections
on prophylactic therapy.
What anticipatory guidance should be provided to
the family?
If surgery is performed endoscopically, there is an increased
risk of recurrent VUR compared with patients who have an
open surgical repair. Patients who have undergone treat-
ment for VUR have an increased risk of renal scarring,
which can be associated with chronic kidney disease, and
as such should be followed closely for signs of impaired
renal functioning, including hypertension, poor growth,
Further Reading
American Academy of Pediatrics, Subcommittee on Urinary
Tract Infection, Committee on Quality Improvement,
Subcommittee on Urinary Tract Infection (2016).
Reaffirmation of the Clinical Practice Guideline: the
diagnosis and management of initial urinary tract infection
in febrile infants and young children, 2 to 24 months of age.
Pediatrics 138(6): 843–852.
Hoberman, A., Greenfeld, S.P., Mattoo, T.K., et al. (2014).
Antimicrobial prophylaxis for children with vesicoureteral
reflux. New England Journal of Medicine 370(25):
2367–2376.
ShaikhN.HobermanA.In: Comprehensive Pediatric Hospital
Medicine, 2nd edn (ed. L.B. Zaoutis and V.W. Chiang),
559–565. Philadelphia: Mosby/Elsevier.
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Chapter 116 A 3-Year-Old with Fever and Painful Urination 485
and proteinuria. Constipation and bowel dysfunction can
increase the occurrence of UTIs, so it is important to ensure
patients are having a soft bowel movement daily and may
require stool softeners to achieve this.
Continuation of case: The patient is admitted to the
general acute care floor for management of his febrile
UTI and started on ceftriaxone and intravenous fluids.
Renal and bladder ultrasound demonstrates pyelonephri-
tis. Urology is consulted who plan to take the child to the
operating room when afebrile for 48 hours for manage-
ment of VUR. After 48 hours, the urine culture taken in
the ED demonstrates Escherichia coli that is sensitive to
cefdinir.
Shaikh, N., Borrell, J.L., Evron, J., and Leeflang, M.M. (2015).
Procalcitonin, C-reactive protein, and erythrocyte
sedimentation rate for the diagnosis of acute pyelonephritis
in children. Cochrane Database of Systematic Reviews (1):
CD009185.
Spencer, J.D., Schwaderer, A., McHugh, K., and Hains, D.S.
(2010). Pediatric urinary tract infections: an analysis of
hospitalizations, charges and costs in the USA. Pediatric
Nephrology 25(2): 2469–2475.
Tillman, B. and Wunsch, H. (2018). Epidemiology and
outcomes. Critical Care Clinics 34: 15–27.
White, B. (2011). Diagnosis and management of urinary tract
infection. American Family Physician 83(4): 409–415.
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487

Index

Page locators in bold indicate tables. Page locators in italics indicate figures. This index uses letter-by-letter alphabetization.

a adenosine 70–72
abdominal pain adrenocorticotropic hormone (ACTH) stimulation test 9
acute jaundice 453 airway, breathing, and circulation (ABC) 213
after cardiac surgery 55–56, 56 albuterol 74, 354–355
diarrhea and lethargy 331–334, 332, 333 allergic reactions
fever 387–389, 388–389 billing and coding 38–40
fever and rash 446 hives and lip swelling 461–462
idiopathic nephrotic syndrome 457 vomiting 58
nausea and vomiting 371 allopurinol 63
splenic laceration 431–433, 432, 432 altered mental status 23–27, 24, 25
vomiting and diarrhea 449–451 ambiguous genitalia 261–263, 262
abdominal trauma 29–31, 30, 30 American Academy of Pediatrics (AAP)
abducens nerve palsy 204 acute hypertension and seizures 396–398
abnormal movements 357–359, 358 jaundice 162
acetaminophen otitis media 106
acetaminophen ingestion 207–210, 209–210 quality improvement 134
head injury 255–256, 258–259 respiratory syncytial virus and respiratory failure 52
acetazolamide 229 American College of Critical Care Medicine (ACCM) 179
activated charcoal 208 amiodarone 362
acute abdomen 215–217 amniotic membrane transplantation (AMT) 167
acute hyperglycemia 191–194, 192 amputation 154
acute hypertension and seizures 395–398, 396–397 amylase 432
acute inflammatory demyelinating polyradiculoneuropathy anaphylaxis/anaphylactic shock 212–213, 461–462
(AIDP) 404–405 anemia 265–267, 266, 266
acute jaundice 453–456, 454–455 anesthesia 58–59
acute kidney injury (AKI) 309–310 angiography 122, 122, 130, 131
acute liver failure 453–456, 454–455 angiotensin-converting enzyme (ACE) inhibitors 6
acute lymphoblastic leukemia (ALL) 266, 372–374 anion gap metabolic acidosis 197–198
acute mastoiditis 278–279 anterior mediastinal mass 373–374
acute motor axonal neuropathy/acute motor and sensory antiarrhythmics 117
axonal neuropathy (AMAN/AMSAN) 404–405 antibiotics
acute myeloid leukemia (AML) 266 complication of pneumonia 1–2
acute otitis media (AOM) 277–278, 291 fever and cough 271
acute pancreatitis 450–451 fever and rash 447–448
acute peritonitis 334 fever and redness behind ear 279
acute respiratory distress syndrome (ARDS) 157–159 fever and sore throat 171
acute respiratory failure/distress 251–254, 252, 252, 353 fever, rash and lethargy 320

Cases in Pediatric Acute Care: Strengthening Clinical Decision Making, First Edition. Edited by Andrea M. Kline-Tilford and Catherine M. Haut.
© 2020 John Wiley & Sons Ltd. Published 2020 by John Wiley & Sons Ltd.
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488 Index

antibiotics (cont’d) benzodiazepines 82–84

fussiness and decreased oral intake 156, 157, 159 beta-agonists 465
fussy, tired infant 408 beta-blockers
generalized weakness 437 acute hypertension and seizures 398
influenza A and new fever 480–481 fussy, tired infant 409
kidney transplant complication 392–393, 477 murmur and chest flutter 350
left eye swelling 35 bilateral tympanostomy tubes 105–106
liver transplantation and fever 93–94 bilevel positive airway pressure (BiPAP)
lower respiratory tract infection 112 asthma 463–465
neonatal tachypnea 201–202 fever and cough 271
osteomyelitis 90–91 respiratory syncytial virus and respiratory failure 52–53
scald burn injury 311 sore throat and shortness of breath 337
shunted hydrocephalus 66–67 spinal muscular atrophy 299
skin wound in postoperative neonate 467, 469 biliary atresia 162
upper respiratory infection, headache and fever 237 bilirubin 93
antiemetics 12 billing and coding 37–45
antiepileptic drugs (AED) 82–84 ambulatory and inpatient billing encounters 40–41
antihistamines 315 bundled services 42, 43, 44
antihypertensives 221 charge generating procedure 38
anti-inflammatory medications 347 codes used to bill for services 38, 39
antinuclear antibody (ANA) 244–245 critical care management 41–44, 41
antipsychotics 297 documentation elements 38
antipyretics 17 documentation for critical care services 42
antiretroviral therapy (ART) 176 outpatient case documentation 38–40
apnea PICU case study 42–44
brain death 365 requirements for new providers 37
progressive lethargy and poor feeding 195 services not meriting critical care codes 42
sore throat and shortness of breath 336–337, 337 time-based codes 41–42, 42, 43–44
appendicitis 387–389, 388–389 transfers 41
arterial blood gas analysis see blood gas analysis biobanking 275
arteriovenous malformation (AVM) 121–123, 121–122 biopsy
aspirin 130–131 bone marrow transplant in sickle cell disease 424
asthma generalized weakness 438
acute respiratory failure 252–253 idiopathic nephrotic syndrome 459
outpatient management of asthma 353–356, 355 knee pain 153–154
status asthmaticus 463–465, 464, 464 tachypnea and cool extremities 225
atrioventricular block 140 blisters/bullae 165, 166
atrioventricular septal defect (AVSD) blood gas analysis
oral presentation and communication 339–344, 343 acute hyperglycemia 192
postoperative heart block 285–286, 286 altered mental status 24
autism spectrum disorders (ASD) 48 brain death 364
autoimmune hepatitis 454–455 desaturation in postoperative cardiac surgery infant 78
automatic implantable cardioverter defibrillator (AICD) 350 fussiness and decreased oral intake 156, 157
fussy, tired infant 408
b headache and seizure 120
back pain 385, 403–406, 404 neonatal tachypnea 200
bacteremia 380 outpatient management of asthma 355–356
bacterial meningitis 236–237 postoperative tachycardia 362
basal hormone testing 377 progressive lethargy and poor feeding 196
basic metabolic profile see metabolic profile scald burn injury 308
Beckwith–Weidemann syndrome 102–103 stridor 292
benign neonatal sleep myoclonus 357–359, 358 tachypnea and cool extremities 224
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Index 489

unresponsive patient 326 carditis 346–347

weaning preterm infant from ventilation 248 ceftriaxone 252
blood in stool 85–87, 86 Centers for Disease Control and Prevention (CDC)
blood transfusion 313–315, 314 head injury 257–258
bloody diarrhea 125–126, 126 respiratory failure and transaminitis 428, 428
bone marrow biopsy/aspirate 62 sickle cell disease and fever 175
bone marrow transplant (BMT) 19–21, 20, 423–425 Centers for Medicare and Medicaid Services (CMS) 37–45
botulism 437–439 central line-associated bloodstream infection (CLABSI)
bowel necrosis 334 480–481
bowel perforation 334 central nervous system (CNS) tumors 204–206
brain death 363–366, 364 central venous pressure (CVP) 376–377
Brain Trauma Foundation 328, 328 cerebral angiography 122, 122
breastfeeding cerebral edema 193
neonatal hypoglycemia 101 cerebral palsy 177–181
neonatal tachypnea 202 cerebral salt wasting (CSW) 67, 376–377, 377
neurologic changes and respiratory failure 183 cerebrospinal fluid (CSF)
progressive lethargy and poor feeding 195 fever and rash 447
vomiting 59 generalized weakness 437–438, 437–438
breathlessness headache and seizure 121
sore throat and shortness of breath 335–338, 336, 337 hypothermia 380–381, 380
sudden onset of breathing difficulty 211–213 intractable headache 228, 228
bronchiolitis leg and back pain 404, 405
cough 73–75 neurologic changes and respiratory failure 187
fever and cold symptoms 15–17 progressive lethargy and poor feeding 195–196
fever and cough 270 shunted hydrocephalus 66–67, 66
respiratory syncytial virus and respiratory failure 52–53 tic disorders 412
bronchodilators 16 upper respiratory infection, headache and fever
bronchopulmonary dysplasia (BPD) 248–249 236–237, 236
Brudzinski sign 185 cerebrovascular accident (CVA) 282–283, 282, 284
bruising 61–63, 62, 62–63 cervicalgia 257
bundled services 42, 43, 44 CHARGE syndrome 211–213
burnout and resiliency 471–473, 472 chemotherapy
diplopia and esotropia 206
c knee pain 154
calcium channel blockers (CCB) pineoblastoma and bone marrow transplant 19
acute hypertension and seizures 398 chest flutter 349–351
altered mental status 24–27 chest X-ray
murmur and chest flutter 350 acute hypertension and seizures 395–396
calcium homeostasis 23–26 acute respiratory failure 252, 253
Campylobacter jejuni 405 altered mental status 23, 25
capillary blood gas analysis see blood gas analysis anemia 266
cardiac arrest 311 asthma 464
cardiac catheterization 130, 131 bruising 61–63, 62
cardiac output complication of pneumonia 1–2, 2
desaturation in postoperative cardiac surgery infant 78–79 cough 74–75
fussy, tired infant 408–409 fever and cold symptoms 15, 16, 17
tachypnea and cool extremities 224 fever and cough 270
cardiac surgery 55–56, 56 fussiness and decreased oral intake 157, 158, 158
cardiac tamponade 311, 373 fussy, tired infant 408
cardiomyopathy 407–409 generalized weakness 436
cardiopulmonary arrest 325, 327 headache and seizure 120
cardiopulmonary resuscitation (CPR) 308–310, 363 kidney transplant complication 476
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490 Index

chest X-ray (cont’d) neurologic changes and respiratory failure 183–186,

lower respiratory tract infection 110 185, 186, 187
neonatal tachypnea 200 orthopnea 371–372, 372
neurologic changes and respiratory failure 184 pineoblastoma and bone marrow transplant 19
newborn immediately post delivery 141 right-sided weakness 283, 284
orthopnea 373 seizures 84
outpatient management of asthma 356 shunted hydrocephalus 65, 66
racing heart 116, 117 splenic laceration 431, 432, 433
respiratory decompensation in trached infant 98, 98 sudden onset of breathing difficulty 212
sickle cell disease and fever 174, 174 tic disorders 412
weaning preterm infant from ventilation 248–249 traumatic brain injury 376
Chiari I malformation 322–323, 412 unresponsive patient 326–327, 326–327
child abuse see physical abuse; sexual abuse upper respiratory infection, headache and fever 237
child assent 274–275 vomiting and diarrhea 450
chloramphenicol 447 concussion 257
chloroquine 429–430 confidentiality 275
chronic otitis media 105–107 congenital adrenal hyperplasia (CAH) 262–263
clonazepam 147 congenital hearing loss 48
Clostridium botulinum 438–439 congenital heart disease (CHD) 97–100, 98–99, 200–201
coagulation profile congenital hyperinsulinemia 442
acute jaundice 454 congested cough 15–16
bloody diarrhea 126 conjunctivitis 165, 167
fever and rash 447 continuous positive airway pressure (CPAP)
fussiness and decreased oral intake 158, 158 asthma 464
irritability in 3-day-old 443 desaturation in postoperative cardiac surgery infant 77
progressive lethargy and poor feeding 196 diaphoresis during feedings 6
scald burn injury 308 fever and cough 269, 271
unresponsive patient 326 neonatal tachypnea 201
upper respiratory infection, headache and fever 236 respiratory syncytial virus and respiratory failure 52–53
cognitive impairment 48 sore throat and shortness of breath 337
Collaborative Institutional Training Initiative (CITI) stridor 291, 293
program 274 cool extremities 223–225, 224
communication see oral presentation and communication corneal reflex 364–365, 437
community-acquired pneumonia (CAP) 270 corticosteroids
complex chronic condition 147–150 acute respiratory failure 253
complication of pneumonia 1–4, 2, 2–4 ambiguous genitalia 262–263
compression fractures 385 bone marrow transplant in sickle cell disease 424
computed tomography (CT) cough 74
abdominal pain, diarrhea and lethargy 333 Henoch–Schönlein purpura nephritis 220–221
abdominal trauma 29, 30, 31 outpatient management of asthma 354–355
acute respiratory failure 253 stridor 293
anemia 267 cough 73–75
brain death 363–364 acute respiratory failure 252–253
diplopia and esotropia 205–206 congested cough 15–16
fever and abdominal pain 387 fever and cough 269–272, 270, 270
fever and redness behind ear 278, 279 stridor 291
fever and sore throat 170, 170 C-peptide 401
headache and seizure 120–121, 121 crackles
head injury 256–258 cough 73–74
hypothermia 379 migratory joint pain 345
knee pain 153–154, 154 tachypnea and cool extremities 223
left eye swelling 34, 35 C-reactive protein (CRP)
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Index 491

fever and redness behind ear 278 diazepam 147

hypothermia 380–381 diazoxide 401
migratory joint pain 346 diffuse midline gliomas 204–206
neonatal tachypnea 201 digoxin 6
osteomyelitis 90 dilated cardiomyopathy 408
persistent fever 130 diplopia and esotropia 203–206, 204, 205
critical blood sample 102, 103 disseminated intravascular coagulation (DIC) 159, 236–237
critical care management 41–44, 41 diuretics
croup 292–293 diaphoresis during feedings 6
C-spine injury 257 fussy, tired infant 409
current procedural terminology (CPT) codes 38, 41 murmur and chest flutter 350
Cutibacterium acnes 67 neonatal tachypnea 202
cytomegalovirus (CMV) 174–175, 174, 437 postoperative spinal fusion with complications 368
dizziness 255
d DMAIC process 133–134
Debrancher deficiency 443 domestic minor sex trafficking (DMST) 288–289
deep tendon reflex (DTR) dopamine 7
intractable headache 228 Down syndrome 337
neurologic changes and respiratory failure 185 doxycycline 430, 447
right-sided weakness 282 drooling 165, 167
dehydration duodenal obstruction 216
acute hyperglycemia 192–193
hypothermia 379 e
kidney transplant complication 391, 475–477 early transitional adaptive hypoglycemia 102
neurologic changes and respiratory failure 183, 184 echocardiography
osteomyelitis, electrolyte disturbance and headaches altered mental status 24
303–305 desaturation in postoperative cardiac surgery infant 77
poor weight gain and dehydration 143–145 diaphoresis during feedings 6
vomiting and diarrhea 12–13, 13 fussy, tired infant 408
see also electrolytes/fluid therapy migratory joint pain 346
delirium 295–298 newborn immediately post delivery 139, 141
contributing factors 296 orthopnea 372
environmental versus pharmacologic therapy 297 persistent fever 130–131, 131
pathophysiology 296 poor oral intake and irritability 70–71
screening tools 296–297 tachypnea and cool extremities 224
desaturation in postoperative cardiac surgery infant 77–79, 78 upper respiratory infection, headache and fever 237
desmopressin 377 weaning preterm infant from ventilation 248–249
dexamethasone 204–205, 263 electrocardiogram (EKG)
diabetes insipidus 67, 376–377, 377 altered mental status 23–24, 25
diabetes mellitus (DM) 143–145, 191–194, 192 anemia 267
diabetic ketoacidosis (DKA) 192–193 bruising 63
Diagnostic and Statistical Manual of Mental Disorders (DSM) diaphoresis during feedings 5–6
296–297 fussy, tired infant 409
dialysis 127 lightheadedness 415
diaphoresis during feedings 5–6 migratory joint pain 346
diarrhea murmur and chest flutter 349–351
abdominal pain, diarrhea and lethargy 331–334, 332, 333 newborn immediately post delivery 139–141, 140
bloody diarrhea 125–126, 126 persistent fever 130
idiopathic nephrotic syndrome 457 poor oral intake and irritability 69–72, 71
kidney transplant complication 391–392, 475–477 postoperative heart block 285–286, 286
osteomyelitis, electrolyte disturbance and headaches 303 postoperative tachycardia 361–362, 362
vomiting and diarrhea 11–14, 13, 449–451, 450 racing heart 115–118, 116–117
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492 Index

electrocardiogram (EKG) (cont’d) gaining consensus on plan of care 178–179, 179

scald burn injury 308, 309–310, 310–311 palliative care 180
tachypnea and cool extremities 224 research review 273–275
electroencephalogram (EEG) script of family meeting 178
abnormal movements 358–359, 358 successful processes utilized in family meeting 179–180
brain death 365 evaluation and management (E/M) documentation 38,
generalized weakness 437–438 39, 40–41
headache and seizure 121 exercise/physical activity
neurologic changes and respiratory failure 186 lightheadedness 416
seizures 81–83, 83 murmur and chest flutter 350–351
tic disorders 412 poor weight gain and dehydration 145
electromyelogram (EMG) 437–438 external bracing 385
electronic medical records (EMR) 38, 135 external ventricular drain (EVD) 121
emesis see vomiting extracorporeal cardiopulmonary resuscitation (ECPR) 308–310
emotional intelligence 472 extracorporeal membrane oxygenation (ECMO) 23, 25–27,
empyema 2–4, 4 309–311
encephalitis 187–188, 188 exudative effusion see empyema
end of life 149 eye swelling 33–35, 34–35, 34–35
endoscopic retrograde cholangiopancreatography (ERCP)
31, 94, 162 f
endotracheal tubes 187 facial nerve palsy 204
end-stage renal disease (ESRD) 391–393, 392 failure mode and effects analysis (FMEA) 133–134
enema reduction 333–334 failure to thrive 144
enterovirus 111 febrile neutropenia 20–21
environmental modifications 297 feeding/nutritional support
epididymitis 241 acute jaundice 456
epilepsy blood in stool 87
complex chronic condition 147–149 diaphoresis during feedings 5–6
seizures 81–84, 82, 83 fussiness and decreased oral intake 155–159, 156, 158, 158
seizures in school-age child 231–233, 232 hyponatremia 419–421
epinephrine irritability in 3-day-old 441–443
cough 74 lower respiratory tract infection 111–112
hives and lip swelling 462 neonatal tachypnea 201–202
hypotension in the operating room 9 neurologic changes and respiratory failure 189
stridor 291–293 poor oral intake and irritability 69–72, 70, 71
sudden onset of breathing difficulty 213 poor weight gain and dehydration 143–145
tachypnea and cool extremities 225 progressive lethargy and poor feeding 195–198, 196
unresponsive patient 325 rash and drooling 167
epistaxis 255 spinal muscular atrophy 299–301, 300
Epstein–Barr virus (EBV) 174–175 splenic laceration 432–433
erythema 469 vomiting 59
erythematous rash with macules 318, 446, 446 fentanyl 7
erythrocyte sedimentation rate (ESR) fever
fever and rash 244–245 acute jaundice 453
knee pain 153 and abdominal pain 387–389, 388–389
migratory joint pain 346 and cold symptoms 15–17, 16
Escherichia coli 86–87, 126–127, 393, 484 and cough 269–272, 270, 270
esotropia see diplopia and esotropia and painful urination 483–485, 484
ethics 177–181 and rash 243–246, 244–245, 445–448, 446, 446
case notes and history 177–178 and redness behind ear 277–279, 278, 279
consulting an ethics committee 181 and sore throat 169–171, 170, 171
essential components of family meeting 179, 180 liver transplantation and fever 93–95, 94
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Index 493

osteomyelitis 89 osteomyelitis, electrolyte disturbance and headaches

persistent fever 129–131, 130, 131 303–305, 304
rash and lethargy 317–320, 318, 318 sore throat and shortness of breath 337
sickle cell disease and fever 173–176, 174, 174–175 tic disorders 411–413
upper respiratory infection, headache and fever upper respiratory infection, headache and fever
235–238, 236 235–238, 236
flaccid myelitis 188 head injury 255–259, 256
flexible bronchoscopy 253 Health Insurance Portability and Accountability Act
fludrocortisone 263 (HIPAA) 44
fluorescence in situ hybridization (FISH) 263 hearing loss 48
fluoroscopy 31 heart block 141, 285–286, 286
focal segmental glomerulosclerosis (FSGS) 458, 475–478, Heliox therapy 293
476 hemodialysis
football injury 431–433, 432, 432 altered mental status 26–27
foreign body aspiration 252–253 progressive lethargy and poor feeding 198
fussiness and decreased oral intake 155–159, 156, 158, 158 scald burn injury 311
fussy, tired infant 407–409, 408, 408 hemoglobin/hematocrit 78
[beta-]hemolytic streptococcal pharyngitis 346–347
g hemolytic uremic syndrome (HUS) 86–87, 126–127
gamma-glutamyltransferase (GGT) 93–94 hemophagocytic lymphohistiocytosis (HLH) 454–455
gastroenteritis Henoch–Schönlein purpura (HSP) nephritis
abdominal pain, diarrhea and lethargy 332 219–221, 220
acute abdomen 216 hepatic panel 94
vomiting and diarrhea 12–13 hepatic phosphorylase deficiency 443
gastroesophageal reflux disease (GERD) 58, 216 herpes simplex virus (HSV) 380–381
generalized periodic epileptiform discharges (GPED) 82 high-flow nasal cannula (HFNC)
generalized weakness 435–439, 436–438 complication of pneumonia 1–2
genetic testing 262 cough 74–75
gestational diabetes 101 respiratory syncytial virus and respiratory failure 51–53
Glasgow Coma Scale (GCS) spinal muscular atrophy 299
abdominal trauma 29 stridor 291, 293
brain death 363 history component codes 38, 39
head injury 255–256 HIV/AIDS 174–176, 175
hypothermia 380 hives and lip swelling 461–462
splenic laceration 431 hoarseness 165, 167
traumatic brain injury 375 human leukocyte antigen (HLA) 424
glucose 6-phosphatase deficiency 443 human trafficking 287–289
glucose tests 101–102, 102 screening and identification 289
glycogen synthetase deficiency 443 signs and common complaints 288–289
good clinical practice (GCP) 274 types and prevalence 288
graft-versus-host disease (GVHD) 423–424 hydrocephalus 65–68, 66, 66
grunting 1 hydrocortisone 263
Guillain–Barré syndrome (GBS) 404–406 β-hydroxybutyrate (BOHB) 193
hyperactive delirium 296
h hyperammonemia 197–198
Haemophilus influenzae 35, 236 hyperbilirubinemia 162
headache hyperglycemia 191–194, 192
acute hyperglycemia 193 hyperinsulinemia 400–401, 442
acute hypertension and seizures 395 hyperinsulinemic euglycemia (HIE) therapy 25–26
and seizure 119–123, 120, 121–122 hyperkalemia 62, 63, 309–310
head injury 257–259 hyperleukocytosis 266–267
intractable headache 227–229, 228, 228 hypernatremia 304–305, 376
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494 Index

hyperoxia test 201 immunoglobulin A vasculitis see Henoch–Schönlein purpura

hyperparathyroidism 232 nephritis
hypersensitivity reactions 208 immunosuppressive therapy
hypertension fever and rash 246
acute hypertension and seizures 395–398, Henoch–Schönlein purpura nephritis 221
396–397 idiopathic nephrotic syndrome 458–459
fever and rash 244–245 inborn error of metabolism (IEM) 196–198
hypertrophic cardiomyopathy (HCM) 349–351, 409 indomethacin 248
hypertrophic pyloric stenosis (HPS) 57–58 inflammatory markers 326
hypoactive delirium 296 influenza A and new fever 479–481, 480
hypocalcemia 232–233 influenza vaccine 405–406
hypocomplementemia 245 informed consent 274–275
hypoglycemia inotropes 392–393, 409
irritability in 3-day-old 441–443 insect stings 461–462
jittery 2-week-old infant 399–401, 400 Institutional Review Board (IRB) 136–137, 136, 274
neonatal hypoglycemia 101–103, 102–103 insulin-dependent diabetes mellitus (IDDM) see type 1 diabetes
progressive lethargy and poor feeding 197–198 mellitus
hyponatremia 419–421, 420 insulin therapy 102, 193
acute hyperglycemia 193 intellectual disability 48, 177–181
postoperative spinal fusion with complications 368–369 interleukin (IL)-6 380
shunted hydrocephalus 67 intracranial pressure (ICP) 120–122, 205
traumatic brain injury 377 intractable headache 227–229, 228, 228
hypoparathyroidism 232–233 intravenous immunoglobulin (IVIG)
hypotension leg and back pain 405
altered mental status 23, 26 neurologic changes and respiratory failure 188
in the operating room 7–9, 8 persistent fever 130–131
hypothermia 365, 379–381, 380 rash and drooling 167
hypotonia 177–181, 436–437 intraventricular hemorrhage (IVH) 248, 249
hypovolemia/hypovolemic shock intussusception 216, 332–334, 333
hyponatremia 368 IPASS Handoff program 342–344, 343
kidney transplant complication 392 irritability
lightheadedness 416 in 3-day-old 441–444, 443
scald burn injury 311 poor oral intake and irritability 69–72, 70, 71
traumatic brain injury 376
vomiting and diarrhea 12–13 j
hypoxia jaundice 161–163, 162, 453–456, 454–455
acute respiratory failure 251–253 jittery 2-week-old infant 399–401, 400
asthma 463 jittery 3-day-old 441
complication of pneumonia 1–2 joint pain 220–221
fever and cough 269–271 Jones criteria 346–347, 347
hyponatremia 419–420 junctional ectopic tachycardia (JET) 361–362, 362, 362
outpatient management of asthma 353
sore throat and shortness of breath 337 k
sudden onset of breathing difficulty 212 Kawasaki disease 130, 166
hypoxic ischemic encephalopathy 81, 327–329 Kayser–Fleischer rings 454–455
Kernig sign 185
i ketorolac 258
iatrogenic withdrawal syndrome (IWS) 296 kidney transplant complication 391–393, 392, 475–478, 476
ibuprofen 248, 256, 259 knee pain 151–154, 152–154, 152
idiopathic nephrotic syndrome 457–459, 458
idiopathic scoliosis 367 l
immunocompromised patient 19–21, 392 lactic acidosis 401
immunoglobulin A nephropathy 458 Ladd procedure 216–217
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Index 495

language delay 47–49 malaria 427–430, 428–429, 428

laparoscopic appendectomy 389 malnutrition 299–301, 300
left eye swelling 33–35, 34–35, 34–35 malrotation 215–217
leg and back pain 403–406, 404 mechanical ventilation see ventilation
lethargy meconium aspiration syndrome (MAS) 200–201
abdominal pain, diarrhea and lethargy 331–334, 332, 333 Medicare/Medicaid see Centers for Medicare and Medicaid
fever and rash 445–446 Services
fever, rash and lethargy 317–320, 318, 318 membranoproliferative glomerulonephritis (MPGN) 458
hypothermia 379 meningismus 155
progressive lethargy and poor feeding 195–198, 196 meningitis
vomiting and diarrhea 11 fussiness and decreased oral intake 156, 157, 159
leukemia 62–63, 266–267 hypothermia 380
leukocytosis 62 neurologic changes and respiratory failure 184
lightheadedness 415–415, 416 upper respiratory infection, headache and fever 236–237
lipase 432 meningococcemia 156, 158, 159
lipid emulsion therapy 26–27 mental status
lipid profile 450 altered mental status 23–27, 24, 25
lip swelling 461–462 cognitive impairment 48
literature review 135 generalized weakness 437
liver failure 208, 210, 210 metabolic acidosis 325
liver function test 453–454, 454 metastatic disease 19–21, 153
liver transplantation and fever 93–95, 94 metered-dose inhalers (MDI) 133–137, 136, 143
lorazepam 119 methicillin-resistant Staphylococcus aureus (MRSA)
loss of consciousness (LOC) 255 fever and sore throat 171
lower respiratory tract infection 109–113, 110, 110, 112 fever, rash and lethargy 319–320
lumbar puncture influenza A and new fever 481
fussiness and decreased oral intake 157 osteomyelitis 90
intractable headache 229 shunted hydrocephalus 67
neurologic changes and respiratory failure 186 methicillin-sensitive Staphylococcus aureus (MSSA) 67
Lyme meningitis 228 methylprednisolone 424
lymph nodes 61, 143–144 middle ear ventilation disorders 48
migraine
m headache and seizure 119
magnesium sulfate 355 head injury 258
magnetic resonance cholangiogram (MRCP) 94 intractable headache 227–229, 228, 228
magnetic resonance imaging (MRI) migratory joint pain 345–347, 346–347
diplopia and esotropia 203–206, 205 milk-protein allergy 58
fever and abdominal pain 387–389 milrinone 225
fever and redness behind ear 278 minimal change nephrotic syndrome (MCNS) 458
generalized weakness 437–438 mononucleosis 173
head injury 258 multiorgan dysfunction syndrome (MODS) 309–310
intractable headache 228, 228 murmur 69
knee pain 153, 153 murmur and chest flutter 349–351
left eye swelling 34 myectomy 351
leg and back pain 403, 405 myocarditis 223–225, 224, 447–448
neurologic changes and respiratory failure 186, 187, 187,
188, 189 n
osteomyelitis 90–91, 91 N-acetylcysteine (NAC) 207–208
right-sided weakness 282–283, 282, 284 narrow-complex tachycardia 69–72
scoliosis 322–323 nasal flaring 1
seizures 84 nasogastric (NG) tubes
tic disorders 412 blood in stool 87
unresponsive patient 327 shunted hydrocephalus 65
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496 Index

nasogastric (NG) tubes (cont’d) otitis media (OM)

spinal muscular atrophy 301 acute otitis media 277–278, 291
National Provider Identifier (NPI) 37 chronic otitis media 105–107
nausea and vomiting language delay 48
headache and seizure 119 outpatient management of asthma 353–356, 355
orthopnea 371 oxygen therapy/support
seizures 231 altered mental status 23, 25–27
necrotizing enterocolitis (NEC) 248–249 anemia 266–267
necrotizing fasciitis 467–470, 468–469, 468 asthma 463–465
negative inspiratory force (NIF) 404–405 complication of pneumonia 1–2
Neisseria meningitidis 156, 236–237 cough 74–75
neonatal hypoglycemia 101–103, 102–103, 441–444, 443 diaphoresis during feedings 6
neonatal tachypnea 199–202, 200, 200 fever and cold symptoms 16
nephritis 244–246 fever and cough 269, 271
nephrotic syndrome (NS) 457–459, 458 fussy, tired infant 408–409
neurologic disorders lower respiratory tract infection 111–112
and respiratory failure 183–189, 184, 186, 185–187, 188 neonatal tachypnea 199, 201
headache and seizure 119–123, 120, 121–122 outpatient management of asthma 354–355
seizures 81–84, 82, 83 poor oral intake and irritability 72
newborn immediately post delivery 139–141, 140–141 respiratory decompensation in trached infant 97–99
nocturnal polysomnography 337, 337 respiratory failure and transaminitis 428–429
non-accidental trauma see physical abuse respiratory syncytial virus and respiratory failure 51–53
non-invasive positive pressure ventilation (NIPPV) 271 scald burn injury 309–311
nonsteroidal anti-inflammatory drugs (NSAID) sore throat and shortness of breath 335–337
back pain 385 stridor 291–293
fever and rash 246 tachypnea and cool extremities 224–225
head injury 255–256, 258
Henoch–Schönlein purpura nephritis 220–221 p
norepinephrine 9 pacemakers
nutritional support see feeding/nutritional support newborn immediately post delivery 139–141, 140–141
postoperative heart block 285–286, 286
o packed red blood cell (PRBC) transfusion 158–159
obesity 101 painful urination 483–485, 484
obstructive sleep apnea (OSA) 336–337, 337 palivizumab 75
opioid analgesics 451 palliative care
oral presentation and communication 339–344 brain death 366
barriers to ideal communication during handoff 341–342 ethical case study 180
describing a comprehensive oral presentation 340–341 palpitations 349–351
key components for new admission 340 pancreatic enzymes 450
key components of handoff communication 342–344, 343 pancreatitis 450–451
obtaining important information during handoff 341 panic attacks 212
shift-to-shift handoffs 342 parathyroid hormone (PTH) 232–233
structured/standardized handoff tools 342 parvovirus requiring blood transfusion 313–315, 314
orbital cellulitis 34–35 patent ductus arteriosus (PDA) 247–249
orchiectomy 241 patent foramen ovale (PFO) 70
orthopnea 371–374, 372, 372 pathologic lead point 334
orthostatic blood pressure 415, 416 Pediatric Emergency Care Applied Research Network
Osgood–Schlatter disease 151–152 (PECARN) 257–258
osteomyelitis Pediatric Evaluation of Developmental Status (PEDS)
case study 89–91, 90, 91 tool 47–49
electrolyte disturbance and headaches 303–305, 304 pediatric nurse practitioner (PNP) 37–38, 40–41, 43–44
osteosarcoma 153–154 pericardial effusion 371–374
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Index 497

periodic lateralizing epileptiform discharges (PLED) 82 respiratory decompensation in trached infant 98

periorbital edema 33, 34 respiratory failure and transaminitis 429
peripheral blood smear 63 stridor 291, 293
peripherally inserted central catheter (PICC) 67 post-coarctectomy syndrome 56
peripheral pulmonic stenosis (PPS) 70 postoperative heart block 285–286, 286
peritonsillar abscess 170 postoperative spinal fusion with complications 367–369, 368
persistent emesis 58–59 postoperative tachycardia 78–79, 361–362, 362, 362
persistent fever 129–131, 130, 131 postural tachycardia syndrome (POTS) 415–416, 416
persistent hemodynamic instability 23 prednisolone 263, 458–459
persistent pulmonary hypertension of the newborn (PPHN) prednisone 458
200–201 prenatal laboratory tests 102
petechiae 155–156 preseptal cellulitis 34
petechial rash 61 pressure-equalizing (PE) tubes 105–106
pharyngitis 169 progressive lethargy and poor feeding 195–198, 196
phenobarbital 83 provider burnout and resiliency 471–473, 472
phonophobia 227–228 Pseudomonas aeruginosa 112, 279, 481
photophobia 227–228, 411 pseudotumor cerebri syndrome (PTCS) 228–229
physical abuse pulmonary edema
brain death 363–366, 364 altered mental status 26
unresponsive patient 327–329, 328 desaturation in postoperative cardiac surgery infant 78
Pierre Robin syndrome 337 migratory joint pain 345–347
pineoblastoma and bone marrow transplant 19–21, 20 pulmonary hypertension 78–79
Plan, Do, Study, Act (PDSA) 133–134 pulse oximetry 199
plaques 166 pyloric regimen 59
Plasmodium spp. 428–430 pyloric stenosis (PS) 57–59, 216
pleural effusion 3, 371–374 pyloromyotomy 58–59
pneumonia
acute respiratory failure 252–253 q
billing and coding 42–44 quality improvement (QI) 133–137, 136
complication of pneumonia 1–4, 2, 2–4 appropriate goals for the problem 135
fever and cough 270 appropriate question for case scenario 135
influenza A and new fever 480 barriers to implementation 135–136
upper respiratory infection, headache and fever 236 defining research versus QI 136
point-of-care test (POCT) glucose 191, 193 final steps 137
point-of-care ultrasound (POCUS) 116 Institutional Review Board 136–137, 136
poisoning and toxic events key members of QI team 133–134
acetaminophen ingestion 207–210, 209–210 literature review 135
altered mental status 23–27 measurement of outcomes 136
generalized weakness 438–439 quinidine 430
poliovirus 188
polymerase chain reaction (PCR) r
acute jaundice 454, 455 racing heart 115–118, 116–118, 117
fever and rash 447 radiography
fussy, tired infant 408 abdominal pain after cardiac surgery 56
sickle cell disease and fever 174–175 acute abdomen 216
upper respiratory infection, headache and fever 237 back pain 385
polysomnography (PSG) 337, 337 head injury 256–258, 256
poor oral intake and irritability 69–72, 70, 71 jaundice 162
poor weight gain and dehydration 143–145 knee pain 151–153, 152
positive end-expiratory pressure (PEEP) osteomyelitis 89–90
desaturation in postoperative cardiac surgery infant 77, 79 scoliosis 322
fussiness and decreased oral intake 158 stridor 293
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498 Index

radiography (cont’d) scald burn injury 307–312, 308, 309–310

see also chest X-ray scoliosis 321–323, 322, 367
radiotherapy 206 sedation
range of motion (ROM) 321 delirium 295–296
rapid diagnostic test (RDT) 428, 429 desaturation in postoperative cardiac surgery infant 77–79
rasburicase 63 orthopnea 373
rash seizures 81–84, 82, 83
and drooling 165–168, 166–167, 166 acute hypertension and seizures 395–398, 396–397
bone marrow transplant in sickle cell disease 423 complex chronic condition 147–149
fever and rash 243–246, 244–245, 445–448, 446, 446 headache and seizure 119–123, 120, 121–122
fever, rash and lethargy 317–320, 318, 318 hyponatremia 419–420
redness behind ear 277–279, 278, 279 in school-age child 231–233, 232
refeeding syndrome 300–301 sepsis/septic shock
referral 48 delirium 295
reflective ability 472 fussiness and decreased oral intake 155–156
renal parenchymal disease 396–397 hypothermia 380–381
research review 273–275 irritability in 3-day-old 442
consents and assents 274–275 kidney transplant complication 392
participants 273 parvovirus requiring blood transfusion 315
protection of human subjects 273–274 pineoblastoma and bone marrow transplant 21
responsibilities for data collection 275 scald burn injury 309–310
training programs 274 shunted hydrocephalus 66
resiliency see burnout and resiliency skin wound in postoperative neonate 469
respiratory decompensation in trached infant 97–100, 98–99 upper respiratory infection, headache and fever 236–237
respiratory distress syndrome (RDS) 200–201 septic arthritis 89–90, 151–152
respiratory failure serology 447
generalized weakness 435 serum creatinine 476–477
neurologic changes and respiratory failure 183–189, sexual abuse 287–289
184, 186, 185–187, 188 sexually transmitted infections (STI) 287–288
respiratory syncytial virus 51–53 shiga-like toxin-producing Escherichia coli hemolytic uremic
transaminitis 427–430, 428–429, 428 syndrome (STEC-HUS) 86–87, 126–127
respiratory infections 20–21, 110, 111 short-acting beta-agonists (SABA) 354–355
respiratory syncytial virus (RSV) 51–53, 129 shunted hydrocephalus 65–68, 66, 66
reticulocyte count 314 sickle cell disease (SCD)
retinal hemorrhage 328, 328 and fever 173–176, 174, 174–175
retropharyngeal abscess (RPA) 170–171, 170 bone marrow transplant 423–425
return of spontaneous circulation (ROSC) 325 right-sided weakness 281–284, 282, 282, 284
rheumatic heart disease 346–347, 347 sinus bradycardia 23, 25, 26–27
rhinorrhea 15–16, 195 sinusitis 34
rhinosinusitis 34 sinus tachycardia 5–6
rhinovirus 111 Six Sigma DMAIC process 133–134
rhombencephalitis 187–188 skin wound in postoperative neonate 467–470, 468–469, 468
rhonchi 73 sleep myoclonus 357–359, 358
Rickettsia rickettsii 446–447 SMART objectives 135
right-sided weakness 281–284, 282, 282, 284 social competency 472
Rocky Mountain spotted fever (RMSF) 445–448, 446, 446 social support 472
root cause analysis (RCA) 133–134 sore throat
Rumack–Matthew nomogram 208, 209 and shortness of breath 335–338, 336, 337
fever and 169–171, 170, 171
s speech therapy 49
salbutamol 202 spinal fracture 383
SBAR handoff 354–355, 355 spinal muscular atrophy (SMA) 299–301, 300
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Index 499

splenic laceration 431–433, 432, 432 T-cell acute lymphoblastic leukemia 372–374
sputum culture 112, 112 team culture 472
Staphylococcus aureus testicular torsion 239–241, 240, 240
fever and redness behind ear 279 tethered cord 322–323
fever, rash and lethargy 319 tetralogy of Fallot (TOF) 211–213, 361
influenza A and new fever 480–481 thrombocytopenia 62
left eye swelling 35 thyroid-stimulating hormone (TSH) 116
osteomyelitis 90 tic disorders 411–413
see also methicillin-resistant Staphylococcus aureus time-based codes 41–42, 42, 43–44
status asthmaticus 463–465, 464, 464 tonic–clonic seizures 119
status epilepticus 81, 84, 147 Tourette syndrome 411–413
stay sutures 97, 98, 99 toxic shock syndrome (TSS) 319–320
Stenotrophomonas maltophilia 112 toxic synovitis 89–90
steroid-induced hyperglycemia 144 tracheitis 480
steroids tracheobronchoscopy 99, 99, 100
ambiguous genitalia 262 tracheostomy 97–100, 177–181, 189
neurologic changes and respiratory failure 188 transaminitis 427–430, 428–429, 428
sudden onset of breathing difficulty 213 transcranial Doppler (TCD) 281, 283
see also corticosteroids transfers 41
Stevens–Johnson syndrome and toxic epidermal necrolysis transient hyperinsulinemic hypoglycemia 102
(SJS/TEN) 166–167 transient tachypnea of the newborn (TTN) 199–202, 200, 200
Streptococcus spp. trauma
migratory joint pain 346–347 abdominal trauma 29–31, 30, 30
osteomyelitis 90 splenic laceration 431–433, 432, 432
sore throat and shortness of breath 336 traumatic brain injury (TBI)
upper respiratory infection, headache and fever 236–237 brain death 363–366, 364
stridor 291–294, 292, 335–336 complication of 375–378, 376, 376–377
substance use/abuse 287–289, 449–451 head injury 257–258
sudden cardiac death 350–351 unresponsive patient 327–329, 328
sudden onset of breathing difficulty 211–213 triple flexion reflex 187
sumatriptan 258 trisomy 21 339–344, 343
superior vena cava syndrome 373 tumor lysis syndrome (TLS) 62, 266–267, 373–374
supraventricular tachycardia (SVT) 70–72, 117–118 type 1 diabetes mellitus 143–145, 192–193
swollen eye 33–35, 34–35, 34–35
swollen lip 461–462 u
synchronized cardioversion 72 ulceration 468
synchronized intermittent mandatory ventilation (SIMV) 252 ultrasonography
syndrome of inappropriate antidiuretic hormone (SIADH) 67, abdominal pain, diarrhea and lethargy 333, 333
368–369, 376–377, 377 acute abdomen 216
syrinx 322–323 fever and abdominal pain 387–389
systemic inflammatory response syndrome (SIRS) 20, 309 Henoch–Schönlein purpura nephritis 221
systemic lupus erythematosus (SLE) 139, 244–246, 245 jaundice 162
liver transplantation and fever 94
t neurologic changes and respiratory failure 183
tachypnea racing heart 116
and cool extremities 223–225, 224 testicular torsion 240–241
complication of pneumonia 1 vomiting 58
fever and cough 269–271 vomiting and diarrhea 449–450
migratory joint pain 345 weaning preterm infant from ventilation 249
neonatal tachypnea 199–202, 200, 200 unresponsive patient 325–329, 326–328, 326, 328, 441
oral presentation and communication 339 upper respiratory infection (URI)
respiratory syncytial virus and respiratory failure 51–52 fever and sore throat 169–170
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500 Index

upper respiratory infection (URI) (cont’d) complex chronic condition 148–149

seizures 81 delirium 295
with headache and fever 235–238, 236 fever and cough 271
uric acid 63 fussiness and decreased oral intake 156–158
urinalysis fussy, tired infant 408–409
acute hyperglycemia 192 leg and back pain 404
acute hypertension and seizures 396 lower respiratory tract infection 111–112
blood in stool 86 neurologic changes and respiratory failure 185, 189
bloody diarrhea 126 orthopnea 373
fever and abdominal pain 388 progressive lethargy and poor feeding 198
fever and painful urination 484 respiratory failure and transaminitis 428–429
fever and rash 244 weaning preterm infant from mechanical ventilation
Henoch–Schönlein purpura nephritis 220 247–249, 248–249, 248
idiopathic nephrotic syndrome 458, 459 ventricular septal defect (VSD) 6
kidney transplant complication 476, 477 ventriculoperitoneal (VP) shunt 65–68
neurologic changes and respiratory failure 184 vesicoureteral reflux (VUR) 483–485, 484
pineoblastoma and bone marrow transplant 21 viral encephalitis 228–229
postoperative spinal fusion with complications 368 viral meningitis 236
progressive lethargy and poor feeding 196 vital capacity (VC) 405–406
splenic laceration 432 vitamin D deficiency 232, 385
testicular torsion 240, 241 vomiting 57–59, 59
traumatic brain injury 375 abdominal pain after cardiac surgery 55–56
vomiting and diarrhea 450 acute abdomen 215
urinary tract infection (UTI) and diarrhea 449–451, 450
fever and painful urination 483–485 vomiting and diarrhea 11–14, 13
influenza A and new fever 480 see also nausea and vomiting
kidney transplant complication 392–393, 477
pineoblastoma and bone marrow transplant 20–21 w
wheezing
v acute respiratory failure 252–253
VALUE acronym 179 asthma 463
vancomycin 271 cough 73–74
vasodilators 78–79, 398 fever and cold symptoms 16
vasopressors 157 hives and lip swelling 461
venous–arterial extracorporeal membrane oxygenation (VA- outpatient management of asthma 353
ECMO) 97 respiratory syncytial virus and respiratory failure 51–53
venous blood gas analysis see blood gas analysis sudden onset of breathing difficulty 212
ventilation white blood cell (WBC) count 90
acute respiratory failure 251–253 Wilson disease 454–456, 455
asthma 463–465 Wolff–Parkinson–White (WPW) syndrome 71–72,
brain death 363–364 117–118