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CURRENT Diagnosis & Treatment

Pediatrics 24th Edition Edition William


Hay
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a LANGE medical book

CURRENT
Diagnosis & Treatment
Pediatrics
TWENTY-FOURTH EDITION

Edited by
William W. Hay, Jr., MD Robin R. Deterding, MD
Professor, Department of Pediatrics Professor, Department of Pediatrics
Section of Neonatology and Chief, Section of Pediatric Pulmonary Medicine
Division of Perinatal Medicine University of Colorado School of Medicine and
University of Colorado School of Medicine Children’s Hospital Colorado
and Children’s Hospital Colorado Medical Director, Breathing Institute Children’s
Hospital Colorado
Myron J. Levin, MD
Professor, Departments of Pediatrics and Medicine Mark J. Abzug, MD
Section of Pediatric Infectious Diseases Professor, Department of Pediatrics
University of Colorado School of Medicine Section of Pediatric Infectious Diseases
and Children’s Hospital Colorado Associate Vice Chair for Academic Affairs,
Department of Pediatrics
University of Colorado School of Medicine and
Children’s Hospital Colorado

and Associate Authors

The Department of Pediatrics at the University of Colorado School of Medicine


is affiliated with Children’s Hospital Colorado.

New York Chicago San Francisco Athens London Madrid Mexico City Milan
New Delhi Singapore Sydney Toronto

Hay_FM_pi-xxxii.indd 1 30/03/18 11:40 AM


Copyright © 2018 by McGraw-Hill Education. All rights reserved. Except as permitted under the United States Copyright Act of
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Contents
Authors xix Esophageal Atresia & Tracheoesophageal
Preface xxix Fistula 48
Selected Highlighted Topics in the Intestinal Obstruction 49
24th Edition of CDT-P xxxi Abdominal Wall Defects 50
Diaphragmatic Hernia 51
1. Advancing the Quality Gastrointestinal Bleeding 51
& Safety of Care 1 Gastroesophageal Reflux 51
Infections in the Newborn Infant 52
Daniel Hyman, MD Bacterial Infections 52
Introduction 1 Fungal Sepsis 55
Current Context 1 Congenital Infections 56
Strategies & Models for Perinatally Acquired Infections 58
Quality Improvement (QI) 4 Hematologic Disorders in the Newborn Infant 59
Principles of Patient Safety Bleeding Disorders 59
(Incident Reporting, Just Culture, Anemia 60
Disclosure, FMEA, RCA, Polycythemia 61
Reliability, Checklists) 7 Renal Disorders in the Newborn Infant 62
Renal Failure 62
2. The Newborn Infant 10 Urinary Tract Anomalies 62
Renal Vein Thrombosis 63
Danielle Smith, MD Neurologic Problems in the Newborn Infant 63
Theresa Grover, MD Seizures 63
Introduction 10 Intracranial Hemorrhage 64
The Neonatal History 10 Metabolic Disorders in the Newborn Infant 65
Assessment of Growth & Gestational Age 10 Hyperglycemia 65
Examination at Birth 12 Hypocalcemia 65
Examination in the Nursery 13 Inborn Errors of Metabolism 66
Care of the Well Neonate 15 Quality Assessment & Improvement
Feeding the Well Neonate 15 in the Newborn Nursery & NICU 66
Early Discharge of the Newborn Infant 17
Hearing Screening 18 3. Child Development & Behavior 67
Common Problems in the Term Newborn 18
Neonatal Jaundice 18 Edward Goldson, MD
Hypoglycemia 25 Ann Reynolds, MD
Respiratory Distress in the Term Introduction 67
Newborn Infant 26 Normal Development 67
Heart Murmurs 28 The First 2 Years 67
Birth Trauma 28 Ages 2–4 Years 73
Infants of Mothers Who Abuse Drugs 29 Early School Years: Ages 5–7 Years 74
Multiple Births 31 Middle Childhood: Ages 7–11 Years 74
Neonatal Intensive Care 32 Behavioral & Developmental Variations 74
Perinatal Resuscitation 32 Normality & Temperament 74
The Preterm Infant 36 Enuresis & Encopresis 75
The Late Preterm Infant 45 Enuresis 75
Cardiac Problems in the Newborn Infant 46 Encopresis 76
Structural Heart Disease 46 Common Developmental Concerns 77
Persistent Pulmonary Hypertension 47 Colic 77
Arrhythmias 48 Feeding Disorders in Infants &
Gastrointestinal & Abdominal Surgical Young Children 79
Conditions in the Newborn Infant 48 Sleep Disorders 80
iii

Hay_FM_pi-xxxii.indd 3 30/03/18 11:40 AM


iv Contents

Parasomnias 81 Predicting the Progression from


Insomnia 81 use to Abuse 148
Sleep-Disordered Breathing 82 Evaluation of Substance Abuse 149
Restless Legs Syndrome & Periodic Treatment & Referral 152
Limb Movement Disorder 82 Prevention 154
Temper Tantrums & Breath-Holding Spells 83 References 155
Well-Child Surveillance & Screening 85
Developmental Disorders 85 6. Eating Disorders 156
Attention-Deficit/Hyperactivity Disorder 87
Autism Spectrum Disorders 89 Eric J. Sigel, MD
Intellectual Disability 93 Introduction 156
Specific Forms of Intellectual Disability & Etiology 156
Associated Treatment Issues 95 Incidence 157
References 97 Predisposing Factors & Clinical Profiles 157
Anorexia Nervosa 157
4. Adolescence 98 Bulimia Nervosa 163
Binge-Eating Disorder 165
Amy E. Sass, MD, MPH Avoidant/Restrictive Food Intake Disorder 166
Molly J. Richards, MD Prognosis, Quality Assessment,
Introduction 98 & Outcomes Metrics 166
Epidemiology 98 Resources for Practitioners & Families 167
Mortality Data 98
Morbidity Data 98 7. Child & Adolescent Psychiatric
Providing a Medical Home for Adolescents 99 Disorders & Psychosocial Aspects
Relating to the Adolescent Patient 100 of Pediatrics 168
The Structure of the Visit 101
Guidelines for Adolescent Preventive Services 107 Adam Burstein, DO
Adolescent Screening 107 Kimberly Kelsay, MD
Promoting Healthy Behaviors 108 Ayelet Talmi, PhD
Transition to Adult Care 112 Introduction 168
Growth & Development 114 Models of Care Encompassing
Puberty 114 Behavioral Health in the
Physical Growth 114 Primary Care Setting 169
Sexual Maturation 114 Prevention, Early Identification, &
Psychosocial Development 115 Developmental Context 170
Adolescent Gynecology & Lifestyle Recommendations 171
Reproductive Health 117 Identification & Assessment During Health
Breast Examination 117 Supervision Visits 171
Breast Masses 118 Psychiatric Disorders of Childhood
Nipple Discharge & Galactorrhea 119 & Adolescence 180
Gynecomastia 120 Anxiety Disorders 181
Gynecologic Disorders in Adolescence 122 Obsessive-Compulsive & Related Disorders 189
Contraception 132 Attention-Deficit/Hyperactivity Disorder 192
Pregnancy 139 Mood Disorders 196
Suicide in Children & Adolescents 202
5. Adolescent Substance Abuse 142 Disruptive, Impulse-Control
& Conduct Disorders 204
Paritosh Kaul, MD High-Risk Patients & Homicide 205
Scope of the Problem 142 Somatic Symptom & Related Disorders 207
Morbidity Data 147 Adjustment Disorders 209
Psychotic Disorders 209
Other Psychiatric Conditions 212

Hay_FM_pi-xxxii.indd 4 30/03/18 11:40 AM


Contents v

8. Child Abuse & Neglect 213 Vaccination in Special Circumstances 254


Hepatitis B Vaccination 255
Antonia Chiesa, MD Rotavirus Vaccination 258
Andrew P. Sirotnak, MD Diphtheria-Tetanus-Acellular
Introduction 213 Pertussis Vaccination 259
Prevention 214 Haemophilus Influenzae Type B
Clinical Findings 214 Vaccination 261
Differential Diagnosis 220 Pneumococcal Vaccination 262
Treatment 220 Poliomyelitis Vaccination 264
Prognosis 221 Influenza Vaccination 265
Measles, Mumps, & Rubella Vaccination 266
9. Ambulatory & Office Pediatrics 222 Varicella Vaccination 268
Hepatitis A Vaccination 270
Meghan Treitz, MD
Meningococcal Vaccination 271
Daniel Nicklas, MD
Tetanus-Reduced Diphtheria-Acellular
Maya Bunik, MD, MSPH
Pertussis Vaccination
David Fox, MD
(Adolescents & Adults) 273
Introduction 222 Human Papillomavirus Vaccination 273
Pediatric History 222 Vaccinations for Special Situations 274
Pediatric Physical Examination 224 Rabies Vaccination 274
Health Supervision Visits 225 Typhoid Fever Vaccination 276
Developmental & Behavioral Assessment 225 Cholera Vaccination 277
Growth Parameters 228 Japanese Encephalitis Vaccination 277
Blood Pressure 229 Tuberculosis Vaccination 277
Vision & Hearing Screening 230 Yellow Fever Vaccination 278
Anticipatory Guidance 234 Passive Prophylaxis 278
Nutrition Counseling 235
Counseling About Television & 11. Normal Childhood Nutrition
Other Media 236 & Its Disorders 280
Immunizations 237
Acute-Care Visits 237 Matthew A. Haemer, MD, MPH
Prenatal Visits 238 Laura E. Primak, RD, CNSD
Sports Physicals 238 Nancy F. Krebs, MD, MS
Chronic Disease Management 238 Nutritional Requirements 280
Medical Home 239 Nutrition & Growth 280
Mental & Behavioral Health 239 Energy 280
Telephone Management & Web-Based Protein 281
Information 240 Lipids 282
Advocacy & Community Pediatrics 240 Carbohydrates 283
Common General Pediatric Issues 241 Major Minerals 284
Fever 241 Trace Elements 284
Growth Deficiency 243 Vitamins 284
Infant Feeding 288
10. Immunization 245 Breast-Feeding 288
Special Dietary Products for Infants 295
Matthew F. Daley, MD
Nutrition for Children 2 Years & Older 296
Sean T. O’Leary, MD, MPH
Pediatric Undernutrition 297
Ann-Christine Nyquist, MD, MSPH
Pediatric Overweight & Obesity 298
Standards for Pediatric Immunization Nutrition Support 302
Practices 246 Enteral 302
Routine Childhood & Adolescent Immunization Parenteral Nutrition 303
Schedules 247 Nutrient Requirements & Delivery 304
Vaccine Safety 247

Hay_FM_pi-xxxii.indd 5 30/03/18 11:40 AM


vi Contents

12. Emergencies & Injuries 309 Barbiturates & Benzodiazepines 342


Belladonna Alkaloids (Atropine, Jimsonweed,
Kevin P. Carney, MD Potato Leaves, Scopolamine, Stramonium) 342
Kelley Roswell, MD b-Blockers & Calcium Channel Blockers 343
Introduction to Pediatric Carbon Monoxide 343
Emergencies & Injuries 309 Caustics 344
Initial Approach to the Acutely Ill Central a2-Adrenergic Agonist 344
Infant or Child 309 Cocaine 345
The ABCs of Resuscitation 310 Cosmetics & Related Products 345
Management of Shock 314 Cyclic Antidepressants 345
Summary of Initial Approach to the Digitalis & Other Cardiac Glycosides 346
Acutely Ill Infant or Child 315 Diphenoxylate with Atropine
Emergency Pediatric Drugs 316 (Lomotil) & Loperamide (Imodium) 346
Approach to the Pediatric Trauma Patient 316 Disinfectants & Deodorizers 347
Mechanism of Injury 317 Disk-Shaped “Button” Batteries 347
Initial Assessment & Management 317 Ethylene Glycol & Methanol 348
Primary Survey 317 g-Hydroxybutyrate, g-Butyrolactone,
Secondary Survey 319 Butanediol, Flunitrazepam, & Ketamine 348
Head Injury 321 Hydrocarbons (Benzene, Charcoal
Burns 323 Lighter Fluid, Gasoline, Kerosene,
Thermal Burns 323 Petroleum Distillates, Turpentine) 349
Electrical Burns 325 Ibuprofen 349
Disorders due to Extremes of Environment 326 Insect Stings (Bee, Wasp, & Hornet) 349
Heat-Related Illnesses & Heat Stroke 326 Insecticides 349
Hypothermia 326 Iron 350
Submersion Injuries 327 Lead 351
Lacerations 328 Magnets 352
Animal & Human Bites 329 Marijuana 352
Dog Bites 329 Mushrooms 352
Cat Bites 330 Nitrites, Nitrates, Aniline,
Human Bites 330 Pentachlorophenol, & Dinitrophenol 353
Pain Management & Procedural Sedation 330 Opioids & Opiates 353
Oral Anti-Diabetics
13. Poisoning 332 (Sulfonylureas, Metformin) 354
Antipsychotics (Typical & Atypical) 355
Richard C. Dart, MD, PhD
Plants 355
Barry H. Rumack, MD
Psychotropic Drugs 355
George Sam Wang, MD
Salicylates 357
Introduction 332 Scorpion Stings 358
Pharmacologic Principles of Toxicology 332 Serotonin Reuptake Inhibitors 358
Preventing Childhood Poisonings 333 Snakebite 358
General Treatment of Poisoning 333 Soaps & Detergents 359
Initial Telephone Contact 333 Spider Bites 360
Initial Emergency Department Contact 336 Vitamins 360
Definitive Therapy of Poisoning 336 Warfarin (Coumadin) & Newer Oral
Management of Specific Common Poisonings 337 Anticoagulants (NOAC) 361
Acetaminophen (Paracetamol) 337
Alcohol, Ethyl (Ethanol) 339
Amphetamines & Related Drugs
(Stimulants, Methamphetamine, MDMA) 340
Anesthetics, Local 340
Antihistamines & Cough & Cold
Preparations 341

Hay_FM_pi-xxxii.indd 6 30/03/18 11:40 AM


Contents vii

14. Critical Care 362 Fungal Infections of the Skin 409


Viral Infections of the Skin 410
Todd C. Carpenter, MD Virus-Induced Tumors 411
Amy Clevenger, MD, PhD Insect Infestations 412
Angela S. Czaja, MD, MSc Dermatitis (Eczema) 412
Eva N. Grayck, MD Common Skin Tumors 415
Cameron F. Gunville, DO Papulosquamous Eruptions 416
Leslie Ridall, DO Hair Loss (Alopecia) 417
Carleen Zebuhr, MD Reactive Erythemas 418
Introduction 362 Miscellaneous Skin Disorders Seen
Respiratory Critical Care 362 in Pediatric Practice 418
Acute Respiratory Failure 362
Conventional Mechanical Ventilation 366 16. Eye 420
Other Modes of Respiratory Support 369
Major Respiratory Diseases in the Jennifer Lee Jung, MD
Pediatric ICU 370 Rebecca Sands Braverman, MD
Acute Respiratory Distress Syndrome 370 Introduction 420
Status Asthmaticus 373 Common Nonspecific Signs & Symptoms 420
Cardiovascular Critical Care 376 Red Eye 420
Shock 376 Tearing 420
Sepsis 379 Discharge 420
Neurocritical Care 383 Pain & Foreign Body Sensation 420
Traumatic Brain Injury 383 Photophobia 420
Hypoxic-Ischemic Encephalopathy 387 Leukocoria 421
Acute Kidney Injury & Renal Replacement Refractive Errors 421
Therapy 388 Myopia (Nearsightedness) 422
Fluid Management & Nutritional Support Hyperopia (Farsightedness) 422
of the Critically Ill Child 389 Astigmatism 422
Sedation & Analgesia in the Pediatric ICU 391 Ophthalmic Examination 422
End-of-Life Care & Death in the PICU 395 History 422
Quality Improvement Initiatives Visual Acuity 422
in the PICU 398 External Examination 423
Pupils 424
15. Skin 399 Alignment & Motility Evaluation 424
Ophthalmoscopic Examination 425
Lori D. Prok, MD Ocular Trauma 426
Carla X. Torres-Zegarra, MD Ocular Foreign Bodies 426
General Principles 399 Corneal Abrasion 427
Diagnosis of Skin Disorders 399 Intraocular Foreign Bodies & Perforating
Treatment of Skin Disorders 399 Ocular Injuries 428
Disorders of the Skin in Newborns 401 Blunt Orbital Trauma 429
Transient Diseases in Newborns 401 Lacerations 429
Pigment Cell Birthmarks, Nevi, Burns 430
& Melanoma 402 Hyphema 431
Melanocytic Nevi 402 Abusive Head Trauma & Nonaccidental
Vascular Birthmarks 403 Trauma 432
Epidermal Birthmarks 404 Prevention of Ocular Injuries 432
Connective Tissue Birthmarks Disorders of the Ocular Structures 433
(Juvenile Elastoma, Collagenoma) 404 Diseases of the Eyelids 433
Hereditary Skin Disorders 405 Viral Eyelid Disease 434
Common Skin Diseases in Infants, Miscellaneous Eyelid Infections 435
Children, & Adolescents 406 Eyelid Ptosis 435
Acne 406 Horner Syndrome 436
Bacterial Infections of the Skin 408 Eyelid Tics 437

Hay_FM_pi-xxxii.indd 7 30/03/18 11:40 AM


viii Contents

Disorders of the Nasolacrimal System 437 17. Oral Medicine & Dentistry 466
Nasolacrimal Duct Obstruction 437
Congenital Dacryocystocele 438 Ulrich Klein, DMD, DDS, MS
Dacryocystitis 438 Roopa P. Gandhi, BDS, MSD
Diseases of the Conjunctiva 439 Camille V. Gannam, DMD, MS
Ophthalmia Neonatorum 439 Issues in Pediatric Oral Health 466
Bacterial Conjunctivitis 440 Oral Examination of Children 467
Viral Conjunctivitis 440 Eruption of the Teeth 469
Allergic Conjunctivitis 441 Dental Caries 471
Mucocutaneous Diseases 442 Periodontal Disease 473
Disorders of the Iris 443 Dental Emergencies 474
Iris Coloboma 443 Special Patient Populations 475
Aniridia 444 Orthodontic Referral 477
Albinism 444
Miscellaneous Iris Conditions 445 18. Ear, Nose, & Throat 478
Glaucoma 445
Patricia J. Yoon, MD
Uveitis 445
Melissa A. Scholes, MD
Anterior Uveitis/Iridocyclitis/Iritis 446
Norman R. Friedman, MD
Posterior Uveitis 446
Intermediate Uveitis 447 The Ear 478
Disorders of the Cornea 448 Infections of the Ear 478
Cloudy Cornea 448 Acute Trauma to the Middle Ear 488
Viral Keratitis 448 Ear Canal Foreign Body &
Corneal Ulcers 449 Cerumen Impaction 488
Disorders of the Lens 450 Auricular Hematoma 489
Cataracts 450 Congenital Ear Malformations 489
Dislocated Lenses/Ectopia Lentis 450 Identification & Management of
Disorders of the Retina 451 Hearing Loss 489
Retinal Hemorrhages in the Newborn 451 The Nose & Paranasal Sinuses 492
Retinopathy of Prematurity 451 Acute Viral Rhinitis 492
Retinoblastoma 452 Rhinosinusitis 493
Retinal Detachment 453 Choanal Atresia 495
Diabetic Retinopathy 454 Recurrent Rhinitis 495
Diseases of the Optic Nerve 454 Epistaxis 496
Optic Neuropathy 454 Nasal Infection 497
Optic Nerve Hypoplasia 455 Nasal Trauma 497
Papilledema 456 Foreign Bodies in the Nose 497
Optic Neuritis 457 The Throat & Oral Cavity 497
Optic Atrophy 458 Acute Stomatitis 497
Diseases of the Orbit 458 Pharyngitis 498
Periorbital & Orbital Cellulitis 458 Peritonsillar Cellulitis or Abscess (Quinsy) 501
Craniofacial Anomalies 459 Retropharyngeal Abscess 501
Orbital Tumors 459 Ludwig Angina 501
Nystagmus 460 Acute Cervical Adenitis 502
Amblyopia 461 Snoring, Mouth Breathing, & Upper Airway
Strabismus 462 Obstruction 503
Unexplained Decreased Vision Tonsillectomy & Adenoidectomy 505
in Infants & Children 464 Disorders of the Lips 506
Learning Disabilities & Dyslexia 464 Disorders of the Tongue 506
Halitosis 506
Salivary Gland Disorders 507
Congenital Oral Malformations 507

Hay_FM_pi-xxxii.indd 8 30/03/18 11:40 AM


Contents ix

19. Respiratory Tract & Mediastinum 509 Congenital Malformations


of the Lung Parenchyma 529
Monica J. Federico, MD Pulmonary Agenesis & Hypoplasia 529
Christopher D. Baker, MD Pulmonary Sequestration 530
Emily M. Deboer, MD Congenital Lobar Overinflation 531
Ann C. Halbower, MD Congenital Pulmonary Airway Malformation
Oren Kupfer, MD (Cystic Adenomatoid Malformation) 532
Stacey L. Martiniano, MD Acquired Abnormalities of the Lung
Scott D. Sagel, MD Parenchyma 533
Paul Stillwell, MD Bronchopulmonary Dysplasia 533
Edith T. Zemanick, MD Community-Acquired Bacterial Pneumonia 534
Michelle Caraballo, MD Parapneumonic Effusion & Empyema 536
Stephen Hawkins, MD, DO Atypical Pneumonias 537
Respiratory Tract 509 Viral Pneumonia 537
Growth & Development 509 Bronchiolitis 538
Diagnostic Aids 510 Mycoplasma Pneumonia 539
Physical Examination of the Aspiration Pneumonia 540
Respiratory Tract 510 Pneumonia in the Immunocompromised
Pulmonary Function Tests 510 Host 541
Assessment of Oxygenation & Ventilation 511 Lung Abscess 542
Diagnosis of Respiratory Tract Infections 512 Bronchiolitis Obliterans 543
Imaging of the Respiratory Tract 514 Children’s Interstitial Lung Disease
Laryngoscopy & Bronchoscopy 515 Syndrome 544
General Therapy for Pediatric Lung Diseases 515 Hypersensitivity Pneumonitis 545
Oxygen Therapy 515 Diseases of the Pulmonary Circulation 546
Inhalation of Medications 516 Pulmonary Hemorrhage 546
Airway Clearance Therapy 516 Pulmonary Embolism 548
Avoidance of Environmental Hazards 517 Pulmonary Edema 549
Disorders of the Conducting Airways 517 Congenital Pulmonary Lymphangiectasia 549
Laryngomalacia & Congenital Disorders Disorders of the Chest Wall 550
of the Extrathoracic Airway 518 Scoliosis 550
Laryngomalacia 518 Pectus Carinatum 550
Other Causes of Congenital Extrathoracic Pectus Excavatum 550
Obstruction 518 Neuromuscular Disorders & Lung Disease 551
Acquired Disorders of the Disorders of the Pleura & Pleural Cavity 551
Extrathoracic Airway 519 Hemothorax 552
Foreign Body Aspiration in the Chylothorax 552
Extrathoracic Airway 519 Pneumothorax & Related Air Leak
Croup Syndromes 520 Syndromes 552
Vocal Fold Immobility 522 Mediastinum 553
Subglottic Stenosis 522 Mediastinal Masses 553
Congenital Causes of Intrathoracic Airway Sleep-Disordered Breathing 554
Obstruction 523 Obstructive & Central Sleep Apnea 554
Malacia of Airways 523 Apparent Life-Threatening Events 555
Vascular Rings & Slings 524 Sudden Unexpected Infant Death &
Bronchogenic Cysts 524 Sudden Infant Death Syndrome 558
Acquired Causes of Intrathoracic Quality Assessment & Outcomes Metrics 559
Airway Obstruction 525
Foreign Body Aspiration in the
Intrathoracic Airway 525
Disorders of Mucociliary Clearance 525
Cystic Fibrosis 526
Primary Ciliary Dyskinesia 527
Bronchiectasis 528

Hay_FM_pi-xxxii.indd 9 30/03/18 11:40 AM


x Contents

20. Cardiovascular Diseases 561 Cardiac Transplantation 611


Quality Improvement for Pediatric Heart
Pei-Ni Jone, MD Transplantation 612
Johannes VonAlvensleben, MD Pulmonary Hypertension 613
Dale Burkett, MD Disorders of Rate & Rhythm 614
Jeffrey R. Darst, MD Disorders of the Sinus Node 614
Kathryn K. Collins, MD Premature Beats 615
Shelley D. Miyamoto, MD Supraventricular Tachycardia 616
Introduction 561 Ventricular Tachycardia 620
Diagnostic Evaluation 561 Long QT Syndrome 621
History 561 Sudden Death 621
Physical Examination 561 Disorders of Atrioventricular Conduction 621
Electrocardiography 564 Syncope (Fainting) 623
Chest Radiograph 566 Introduction 623
Echocardiography 567 Diagnostic Evaluation 624
Magnetic Resonance Imaging 568 Vasovagal/Neurocardiogenic Syncope 624
Cardiopulmonary Stress Testing 568
Arterial Blood Gases 568 21. Gastrointestinal Tract 626
Cardiac Catheterization & Angiocardiography 569
Perinatal & Neonatal Circulation 570 Edward J. Hoffenberg, MD
Heart Failure 571 Glenn T. Furuta, MD
Genetic Basis of Congenital Heart Disease 573 Gregory Kobak, MD
Acyanotic Congenital Heart Disease 574 Thomas Walker, MD
Defects in Septation 574 Jason Soden, MD
Patent (Persistent) Ductus Arteriosus 578 Robert E. Kramer, MD
Right-Sided Obstructive Lesions 580 David Brumbaugh, MD
Left-Sided Lesions 582 Disorders of the Esophagus 626
Diseases of the Aorta 586 Gastroesophageal Reflux & GERD 626
Coronary Artery Abnormalities 587 Eosinophilic Esophagitis 628
Cyanotic Congenital Heart Disease 588 Achalasia of the Esophagus 629
Tetralogy of Fallot 588 Caustic Burns of the Esophagus 630
Pulmonary Atresia with Ventricular Foreign Bodies in the Alimentary Tract 631
Septal Defect 590 Disorders of the Stomach & Duodenum 633
Pulmonary Atresia with Intact Hiatal Hernia 633
Ventricular Septum 590 Pyloric Stenosis 633
Tricuspid Atresia 592 Gastric & Duodenal Ulcer 634
Hypoplastic Left Heart Syndrome 593 Congenital Diaphragmatic Hernia 635
Transposition of the Great Arteries 594 Congenital Duodenal Obstruction 636
Total Anomalous Pulmonary Disorders of the Small Intestine 637
Venous Return 596 Intestinal Atresia & Stenosis 637
Truncus Arteriosus 597 Intestinal Malrotation 638
Quality Improvement in Congenital Short Bowel Syndrome 638
Heart Disease 598 Intussusception 640
Acquired Heart Disease 599 Inguinal Hernia 640
Rheumatic Fever 599 Umbilical Hernia 641
Kawasaki Disease 601 Patent Omphalomesenteric Duct 641
Infective Endocarditis 602 Meckel Diverticulum 641
Pericarditis 604 Acute Appendicitis 642
Cardiomyopathy 605 Duplications of the Gastrointestinal Tract 643
Myocarditis 608 Disorders of the Colon 643
Preventive Cardiology 609 Congenital Aganglionic Megacolon
Hypertension 609 (Hirschsprung Disease) 643
Atherosclerosis & Dyslipidemias 609 Constipation 644
Chest Pain 610 Anal Fissure 646

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Contents xi

Congenital Anorectal Anomalies 646 Pancreatic Disorders 708


Clostridium Difficile Infection in Children 647 Acute Pancreatitis 708
Disorders of the Peritoneal Cavity 648 Chronic Pancreatitis 710
Peritonitis 648 Gastrointestinal & Hepatobiliary
Chylous Ascites 648 Manifestations of Cystic Fibrosis 711
Gastrointestinal Tumors & Malignancies 649 Syndromes with Pancreatic
Juvenile Polyps 649 Exocrine Insufficiency 711
Cancers of the Esophagus, Small Isolated Exocrine Pancreatic
Bowel, & Colon 651 Enzyme Defect 714
Mesenteric Cysts 651 Pancreatic Tumors 714
Intestinal Hemangiomas & References 715
Vascular Malformations 651
Major Gastrointestinal Symptoms & Signs 652 23. Fluid, Electrolyte, & Acid-Base
Acute Diarrhea 652 Disorders & Therapy 716
Chronic Diarrhea 653
Gastrointestinal Bleeding 655 Melisha G. Hanna, MD, MS
Vomiting 658 Margret Bock, MD, MS
Abdominal Pain 660 Regulation of Body Fluids, Electrolytes,
Acute Abdomen 661 & Tonicity 716
Malabsorption Syndromes 661 Acid-Base Balance 717
Inflammatory Bowel Disease 665 Fluid & Electrolyte Management 717
Dehydration 719
22. Liver & Pancreas 669 Hyponatremia 721
Hypernatremia 722
Ronald J Sokol, MD, FAASLD Potassium Disorders 722
Michael R. Narkewicz, MD Acid-Base Disturbances 724
Shikha S Sundaram, MD, MSCI Metabolic Acidosis 724
Cara L. Mack, MD Metabolic Alkalosis 725
Liver Disorders 669 Respiratory Acidosis 725
Neonatal Cholestatic Jaundice 669 Respiratory Alkalosis 725
Intrahepatic Cholestasis 669
Extrahepatic Neonatal Cholestasis 678 24. Kidney & Urinary Tract 726
Other Neonatal Hyperbilirubinemic
Conditions (Noncholestatic Melissa A. Cadnapaphornchai, MD
Nonhemolytic) 680 Gary M. Lum, MD
Hepatitis A 683 Evaluation of the Kidney & Urinary Tract 726
Hepatitis B 685 History 726
Hepatitis C 687 Physical Examination 726
Hepatitis D (Delta Agent) 688 Laboratory Evaluation of Renal Function 726
Hepatitis E 688 Laboratory Evaluation of Immunologic
Other Hepatitis Viruses 688 Function 730
Acute Liver Failure 689 Radiographic Evaluation 730
Autoimmune Hepatitis 690 Renal Biopsy 730
Nonalcoholic Fatty Liver Disease 692 Congenital Anomalies of the Urinary Tract 730
a1-Antitrypsin Deficiency Liver Disease 693 Renal Parenchymal Anomalies 730
Wilson Disease (Hepatolenticular Distal Urinary Tract Anomalies 731
Degeneration) 694 Hematuria & Glomerular Disease 732
Drug-Induced Liver Disease 696 Microhematuria 732
Cirrhosis 696 Glomerulonephritis 732
Portal Hypertension 698 Tubulointerstitial Disease 734
Biliary Tract Disease 701 Acute Interstitial Nephritis 734
Pyogenic & Amebic Liver Abscess 705 Proteinuria & Renal Disease 734
Liver Tumors 706 Congenital Nephrosis (Finnish Type) 735
Liver Transplantation 708

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xii Contents

Idiopathic Nephrotic Syndrome Acute Ataxias of Childhood 794


of Childhood 735 Chronic & Episodic Ataxias 797
Focal Segmental Glomerulosclerosis 736 Movement Disorders 797
Membranous Nephropathy Cerebral Palsy 800
(Membranous Glomerulonephritis) 736 Infections & Inflammatory Disorders
Diseases of the Renal Vessels 736 of the Central Nervous System 801
Renal Vein Thrombosis 736 Bacterial Meningitis 802
Renal Arterial Disease 737 Brain Abscess 803
Hemolytic-Uremic Syndrome 737 Viral Infections 804
Renal Failure 738 Encephalopathy of Human
Acute Kidney Injury 738 Immunodeficiency Virus Infection 804
Chronic Renal Failure 741 Other Infections 805
Hypertension 742 Noninfectious Inflammatory Disorders
Inherited or Developmental of the Central Nervous System 805
Defects of the Kidneys 745 Multiple Sclerosis 806
Disorders of the Renal Tubules 746 Syndromes Presenting as Acute
Cystinosis 746 Flaccid Weakness 807
Oculocerebrorenal Syndrome Disorders of Childhood Affecting Muscles 810
(Lowe Syndrome) 747 Benign Acute Childhood Myositis 817
Hypokalemic Alkalosis (Bartter Syndrome, Myasthenic Syndromes 817
Gitelman Syndrome, & Liddle Syndrome) 747 Peripheral Nerve Palsies 819
Nephrogenic Diabetes Insipidus (NDI) 747 Chronic Polyneuropathy 820
Nephrolithiasis 748 Miscellaneous Neuromuscular Disorders 821
Urinary Tract Infections 749 Floppy Infant Syndrome 821
QA/QI in Pediatric Nephrology 750
26. Orthopedics 824
25. Neurologic & Muscular Disorders 752
Jason Rhodes, MD, MS
Teri L. Schreiner, MD, MPH Mark A. Erickson, MD, MMM
Michele L. Yang, MD Alex Tagawa, BS
Jan A. Martin, MD Cameron Niswander, BA
Ricka Messer, MD, PhD Disturbances of Prenatal Origin 824
Scott Demarest, MD Congenital Amputations &
Diana Walleigh, MD Limb Deficiencies 824
Neurologic Assessment & Neurodiagnostics 752 Deformities of the Extremities 825
History & Examination 752 Common Foot Problems 825
Diagnostic Testing 752 Genu Varum & Genu Valgum 827
Pediatric Neuroradiologic Procedures 754 Tibial Torsion & Femoral Anteversion 828
Disorders Affecting the Nervous System Developmental Dysplasia of the Hip Joint 828
in Infants & Children 758 Slipped Capital Femoral Epiphysis 830
Altered States of Consciousness (Coma) 758 Common Spine Conditions 831
Seizures & Epilepsy 761 Torticollis 831
Sleep Disorders 773 Scoliosis 832
Headaches 774 Kyphosis 832
Pseudotumor Cerebri (Idiopathic Inflammatory Conditions 833
Intracranial Hypertension) 777 Arthritis & Tenosynovitis 833
Cerebrovascular Disease 779 Syndromes with Musculoskeletal Involvement 834
Congenital Malformations of the Arthrogryposis Multiplex Congenita
Nervous System 782 (Amyoplasia Congenita) 834
Abnormal Head Size 785 Klippel-Feil Syndrome 834
Neurocutaneous Dysplasias 787 Neurologic Disorders Involving the
Central Nervous System Degenerative Musculoskeletal System 836
Disorders of Infancy & Childhood 794 Trauma 837
Ataxias of Childhood 794 Traumatic Subluxations & Dislocations 839

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Contents xiii

Fractures 840 30. Hematologic Disorders 898


Infections of the Bones & Joints 842
Vascular Lesions & Avascular Necrosis Daniel R. Ambruso, MD
(Osteochondroses) 845 Rachelle Nuss, MD
Neoplasia of the Musculoskeletal System 846 Michael Wang, MD
Miscellaneous Diseases of Bone & Joint 848 Normal Hematologic Values 898
Fibrous Dysplasia 848 Bone Marrow Failure 898
Bone Cysts, Baker Cyst, & Ganglions 849 Constitutional Aplastic Anemia
Quality Assurance/Improvement Initiatives (Fanconi Anemia) 898
in Orthopedics 849 Acquired Aplastic Anemia 900
Anemias 901
27. Sports Medicine 850 Approach to the Child with Anemia 901
Pure Red Cell Aplasia 903
Katherine S. Dahab, MD, FAAP, CAQSM Nutritional Anemias 904
Kyle B. Nagle, MD, MPH, FAAP, CAQSM Anemia of Chronic Disorders 906
Armando Vidal, MD Congenital Hemolytic Anemias:
Basic Principles 850 Red Cell Membrane Defects 907
Fitness & Conditioning 850 Congenital Hemolytic Anemias:
Sports Nutrition 851 Hemoglobinopathies 909
Preparticipation Physical Evaluation 851 Congenital Hemolytic Anemias:
Rehabilitation of Sports Injuries 859 Disorders of Red Cell Metabolism 916
Common Sports Medicine Issues & Injuries 860 Acquired Hemolytic Anemia 917
Infectious Diseases 860 Polycythemia & Methemoglobinemia 919
Head & Neck Injuries 860 Methemoglobinemia 920
Spine Injuries 864 Disorders of Leukocytes 920
Shoulder Injuries 865 Neutropenia 920
Elbow Injuries 867 Neutrophilia 922
Hand & Wrist Injuries 869 Disorders of Neutrophil Function 923
Hip Injuries 871 Lymphocytosis 925
Knee Injuries 873 Eosinophilia 925
Foot & Ankle Injuries 877 Bleeding Disorders 925
Prevention 878 Abnormalities of Platelet Number
or Function 927
28. Rehabilitation Medicine 880 Inherited Bleeding Disorders 931
Von Willebrand Disease 935
Gerald H. Clayton, PhD Acquired Bleeding Disorders 936
Pamela E. Wilson, MD Vascular Abnormalities Associated
Pediatric Brain Injury 880 with Bleeding 938
Spinal Cord Injury 883 Thrombotic Disorders 939
Brachial Plexus Lesions & Splenic Abnormalities 943
Typical Features 885 Splenomegaly & Hypersplenism 943
Common Rehabilitation Problems 886 Asplenia & Splenectomy 943
Quality Assurance/Improvement Transfusion Medicine 944
Initiatives in Rehabilitation Medicine 888 Donor Screening & Blood Processing:
Risk Management 944
29. Rheumatic Diseases 889 Storage & Preservation of Blood & Blood
Components 944
Jennifer B. Soep, MD
Pretransfusion Testing 945
Juvenile Idiopathic Arthritis 889 Transfusion Practice 949
Systemic Lupus Erythematosus 892 References 952
Dermatomyositis 894
Vasculitis 895
Raynaud Phenomenon 896
Noninflammatory Pain Syndromes 896

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xiv Contents

31. Neoplastic Disease 953 Children’s Concept of Death 992


Spiritual & Cultural Support 993
John A. Craddock, MD Withdrawal of Medical Life Support 994
Ralph R. Quinones, MD Advance Care Planning 994
Amy K. Keating, MD References 996
Kelly Maloney, MD
Jean M. Mulcahy Levy, MD 33. Immunodeficiency 997
Roger H. Giller, MD
Brian S. Greffe, MD Jordan K. Abbott, MD
Anna Franklin, MD Pia J. Hauk, MD
Timothy Garrington, MD Immunodeficiency Evaluation: Primary
Major Pediatric Neoplastic Diseases 953 Considerations 997
Acute Lymphoblastic Leukemia 953 Severe Combined Immunodeficiency
Acute Myeloid Leukemia 957 Diseases 1001
Myeloproliferative Diseases 959 SCID Classification 1002
Brain Tumors 961 Other Combined Immunodeficiencies 1004
Lymphomas & Lymphoproliferative Antibody Deficiency Syndromes 1006
Disorders 965 Phagocyte Disorders 1010
Neuroblastoma 970 Deficiencies of the Innate Immune System 1011
Wilms Tumor (Nephroblastoma) 972 Immunodeficiency Diseases
Bone Tumors 974 That Present with Autoimmunity 1013
Rhabdomyosarcoma 976 Immunodeficiency Diseases
Retinoblastoma 977 That Overlap with Allergy 1014
Hepatic Tumors 978
Langerhans Cell Histiocytosis 980 34. Endocrine Disorders 1016
Hematopoietic Stem Cell Transplant 981
General Considerations 981 Sarah Bartz, MD
Late Effects of Pediatric Cancer Therapy 983 Jennifer M. Barker, MD
Growth Complications 983 Michael S. Kappy, MD, PhD
Endocrine Complications 983 Megan Moriarty Kelsey, MD, MS
Cardiopulmonary Complications 984 Sharon H. Travers, MD
Renal Complications 984 Philip S. Zeitler, MD, PhD
Neuropsychological Complications 984 General Concepts 1016
Second Malignancies 985 Hormone Types 1016
Feedback Control of Hormone Secretion 1016
32. Pain Management & Pediatric Disturbances of Growth 1017
Palliative & End-of-Life Care 986 Target Height & Skeletal Maturation 1018
Short Stature 1018
Brian S. Greffe, MD Tall Stature 1024
Jeffrey L. Galinkin, MD, FAAP Disorders of the Posterior Pituitary Gland 1025
Nancy A. King, MSN, RN, CPNP Arginine Vasopressin
Introduction 986 (Antidiuretic Hormone) Physiology 1025
Pain Assessment 986 Central Diabetes Insipidus 1025
Acute Pain 986 Thyroid Gland 1026
Chronic Pain Management 989 Fetal Development of the Thyroid 1026
Pediatric Palliative & End-of-Life Care 991 Physiology 1026
Introduction 991 Hypothyroidism (Congenital & Acquired) 1027
Children Who May Benefit from Palliative Thyroiditis 1028
Care Interventions 991 Hyperthyroidism 1029
Pain Management in Pediatric Palliative Care 992 Thyroid Cancer 1031
Quality-of-Life Adjuncts & Symptom Disorders of Calcium & Phosphorus
Management in Pediatric Palliative Care 992 Metabolism 1031
Psychosocial Aspects of Pediatric Hypocalcemic Disorders 1031
Palliative Care 992

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Contents xv

Pseudohypoparathyroidism 36. Inborn Errors of Metabolism 1065


(Resistance to Parathyroid
Hormone Action) 1035 Janet A. Thomas, MD
Hypercalcemic States 1035 Johan L.K. Van Hove, MD, PhD, MBA
Familial Hypocalciuric Hypercalcemia Peter R. Baker II, MD
(Familial Benign Hypercalcemia) 1037 Introduction 1065
Hypervitaminosis D 1037 Diagnosis 1065
Idiopathic Hypercalcemia of Suspecting Inborn Errors 1065
Infancy (Williams Syndrome) 1037 Laboratory Studies 1066
Immobilization Hypercalcemia 1037 Common Clinical Situations 1066
Hypophosphatasia 1037 Management of Metabolic Emergencies 1067
Gonads (Ovaries & Testes) 1037 Newborn Screening 1067
Development 1037 Disorders of Carbohydrate Metabolism 1068
Disorders of Sexual Development 1038 Glycogen Storage Diseases 1068
Abnormalities in Female Pubertal Galactosemia 1069
Development & Ovarian Function 1040 Hereditary Fructose Intolerance 1070
Abnormalities in Male Pubertal Disorders of Energy Metabolism 1071
Development & Testicular Function 1043 Disorders of Amino Acid Metabolism 1073
Adrenal Cortex 1045 Disorders of the Urea Cycle 1073
Adrenocortical Insufficiency Phenylketonuria & the
(Adrenal Crisis, Addison Disease) 1046 Hyperphenylalaninemias 1074
Congenital Adrenal Hyperplasias 1048 Hereditary Tyrosinemia 1076
Adrenocortical Hyperfunction Maple Syrup Urine Disease
(Cushing Disease, Cushing Syndrome) 1050 (Branched-Chain Ketoaciduria) 1077
Primary Hyperaldosteronism 1051 Homocystinuria 1077
Uses of Glucocorticoids & Adrenocorticotropic Nonketotic Hyperglycinemia 1078
Hormone in Treatment of Organic Acidemias 1079
Nonendocrine Diseases 1051 Propionic & Methylmalonic
Adrenal Medulla Pheochromocytoma 1053 Acidemia (Ketotic Hyperglycinemias) 1079
Carboxylase Deficiency 1081
35. Diabetes Mellitus 1054 Glutaric Acidemia Type I 1081
Disorders of Fatty Acid O ­ xidation
Brigitte I. Frohnert, MD, PhD & Carnitine 1082
H. Peter Chase, MD Fatty Acid Oxidation Disorders 1082
Marian Rewers, MD, PhD Carnitine 1083
General Considerations 1054 Purine Metabolism Disorders 1083
Pathogenesis 1055 Lysosomal Diseases 1084
Prevention 1055 Peroxisomal Diseases 1087
Clinical Findings 1055 Congenital Disorders of Glycosylation 1088
Differential Diagnosis 1056 Smith-Lemli-Opitz Syndrome & D ­ isorders
Complications 1056 of Cholesterol Synthesis 1089
Associated Autoimmune Diseases in Disorders of Brain Specific M ­ etabolism:
Type 1 Diabetes 1059 Neurotransmitters, Synthesis
Associated Conditions with Type 2 of Amino Acids & Sphingolipids 1090
Diabetes 1060 Creatine Synthesis Disorders 1091
Treatment 1060 Quality Initiatives in the Field of
Quality Assessment (QA) & Outcomes Metabolic Disease 1092
Metrics 1064
Prognosis 1064
References 1064

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xvi Contents

37. Genetics & Dysmorphology 1093 Urticaria & Angioedema 1152


Anaphylaxis 1154
Naomi J.L. Meeks, MD Adverse Reactions to Drugs & Biologicals 1156
Margarita Saenz, MD Food Allergy 1159
Anne Chun-Hui Tsai, MD, MSc Insect Allergy 1161
Ellen R. Elias, MD
Foundations of Genetic Diagnosis 1093 39. Antimicrobial Therapy 1163
Cytogenetics 1093
Molecular Genetics 1098 Sarah K. Parker, MD
Principles of Inherited Human Disorders 1099 Jason Child, PharmD
Mendelian Inheritance 1099 Justin Searns, MD
Multifactorial Inheritance 1102 John W. Ogle, MD
Nonmendelian Inheritance 1103 Principles of Antimicrobial Therapy 1163
Family History & Pedigree 1104 Susceptibility Testing & Drug Dosing
Dysmorphology & Human Embryology 1105 Properties 1163
Mechanisms 1105 Pharmacodynamic Concepts 1164
Clinical Dysmorphology 1106 Use of Antimicrobial Agents to
Chromosomal Disorders: Prevent Disease 1165
Abnormal Number 1107 Concepts for Judicious Use of
Trisomies 1107 Antimicrobials 1165
Sex Chromosome Abnormalities 1108 Choice of Antimicrobial Agents 1167
Chromosomal Abnormalities: Specific Antimicrobial Agents 1167
Abnormal Structure 1109 b-Lactam Antibiotics
Chromosome Deletion Disorders 1109 (Step 1, Figure 39–2) 1167
Contiguous Gene Disorders 1109 b-Lactam: Penicillins 1175
Mendelian Disorders 1110 b-Lactam: Cephems 1181
Autosomal Dominant Disorders 1110 b-Lactam: Monobactams 1181
Autosomal Recessive Disorders 1113 b-Lactam: Carbapenems 1181
X-Linked Disorders 1114 Glycopeptide Agents (Step 2, Figure 39–2) 1182
Nonmendelian Disorders 1115 Daptomycin (Step 4, Figure 39–2) 1182
Disorders of Imprinting 1115 Trimethoprim/Sulfamethoxazole
Disorders Associated with Anticipation 1116 (Step 5, Figure 39–2) 1182
Disorders of Multifactorial Inheritance 1116 Metronidazole (Step 6, Figure 39–2) 1183
Cleft Lip & Cleft Palate 1117 Macrolides (Step 6, Figure 39–2) 1183
Neural Tube Defects 1118 Lincosamides (Step 6, Figure 39–2) 1183
Common Recognizable Disorders Oxazolidinones (Step 6, Figure 39–2) 1184
with Variable or Unknown Cause 1119 Tetracyclines (Step 6, Figure 39–2) 1184
Genetic Evaluation of the Child Aminoglycosides (Step 6, Figure 39–2) 1184
with Developmental Disabilities 1120 Rifamycins (Step 7, Figure 39–2) 1185
Perinatal Genetics 1122 Fluoroquinolones (Step 8, Figure 39–2) 1185
Teratogens 1122 References 1185
Assisted Reproduction 1122
Prenatal Diagnosis 1123 40. Infections: Viral & Rickettsial 1187
Myron J. Levin, MD
38. Allergic Disorders 1124 Daniel Olson,
Ronina A. Covar, MD Edwin J. Asturias, MD
David M. Fleischer, MD Adriana Weinberg, MD
Christine Cho, MD Viral Infections 1187
Mark Boguniewicz, MD Respiratory Infections 1187
Introduction 1124 Viruses Causing the Common Cold 1187
Asthma 1124 Infections due to Adenoviruses 1190
Allergic Rhinoconjunctivitis 1143
Atopic Dermatitis 1148

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Contents xvii

Influenza 1194 42. Infections: Bacterial & Spirochetal 1246


Parainfluenza (Croup) 1195
Respiratory Syncytial Virus Disease 1196 James Gaensbauer, MD, MScPH
Human Metapneumovirus Infection 1198 Yosuke Nomura, MD
Infections due to Enteroviruses John W. Ogle, MD
& Parechoviruses 1198 Marsha S. Anderson, MD
Acute Febrile Illness 1199 Bacterial Infections 1246
Respiratory Tract Illnesses 1199 Group A Streptococcal Infections 1246
Rashes (Including Hand-Foot-&- Group B Streptococcal Infections 1250
Mouth Disease) 1200 Streptococcal Infections with Organisms
Central Nervous System Illnesses 1200 Other than Group A or B 1253
Infections due to Herpesviruses 1203 Pneumococcal Infections 1254
Herpes Simplex Infections 1203 Staphylococcal Infections 1257
Varicella & Herpes Zoster 1206 Meningococcal Infections 1261
Roseola Infantum (Exanthem Subitum) 1208 Gonococcal Infections 1264
Cytomegalovirus Infections 1209 Botulism 1267
Infectious Mononucleosis Tetanus 1269
(Epstein-Barr Virus) 1212 Gas Gangrene 1270
Viral Infections Spread by Insect Vectors 1214 Diphtheria 1271
Encephalitis 1214 Infections due to Enterobacteriaceae 1273
Dengue 1218 Pseudomonas Infections 1275
Chikungunya 1219 Salmonella Gastroenteritis 1277
Zika 1220 Typhoid Fever & Paratyphoid Fever 1279
Colorado Tick Fever 1221 Shigellosis (Bacillary Dysentery) 1280
Other Major Viral Childhood Exanthems 1222 Cholera 1281
Erythema Infectiosum 1222 Campylobacter Infection 1283
Measles (Rubeola) 1223 Tularemia 1284
Rubella 1225 Plague 1285
Infections due to Other Viruses 1226 Haemophilus Influenzae Type B Infections 1287
Hantavirus Cardiopulmonary Syndrome 1226 Pertussis (Whooping Cough) 1289
Mumps 1227 Listeriosis 1291
Rabies 1228 Tuberculosis 1293
Rickettsial Infections & Q Fever 1229 Infections with Nontuberculous
Human Ehrlichiosis & Anaplasmosis 1230 Mycobacteria 1296
Rocky Mountain Spotted Fever 1231 Legionella Infection 1298
Endemic Typhus (Murine Typhus) 1231 Chlamydophila & Chlamydia Infections
Q Fever 1232 (Psittacosis, C Pneumoniae, &
C Trachomatis) 1299
41. Human Immunodeficiency Cat-Scratch Disease 1301
Virus Infection 1233 Spirochetal Infections 1302
Syphilis 1302
Elizabeth J. McFarland, MD Relapsing Fever 1305
Perinatally HIV-Exposed Infant 1235 Leptospirosis 1306
Adolescents with HIV Exposure 1236 Lyme Disease 1308
Progressive HIV Disease 1237
Treatment 1240
Prevention 1244
Quality Assurance & Outcome Metrics 1245

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xviii Contents

43. Infections: Parasitic & Mycotic 1311 Vaginal Discharge 1364


Genital Ulcerations 1365
James Gaensbauer, MD, MScPH Genital Warts & Human Papillomavirus 1368
Kevin Messacar, MD Other Viral Infections 1370
Samuel R. Dominguez, MD, PhD Ectoparasitic Infections 1371
Myron J. Levin, MD References 1372
Parasitic Infections 1311
Protozoal Infections 1312 45. Travel Medicine 1373
Systemic Infections 1312
Gastrointestinal Infections 1319 Suchitra Rao, MBBS
Trichomoniasis 1324 Introduction 1373
Metazoal Infections 1325 Preparing Children & Infants
Nematode Infections 1325 for Travel 1373
Cestode Infections (Flukes) 1331 Vaccinations—Routine Childhood
Trematode Infections 1334 Vaccines Modified for Travel 1375
Mycotic Infections 1335 Vaccinations—Travel-Specific 1377
Pneumocystis & Other Opportunistic Traveler’s Diarrhea 1379
Fungal Infections 1346 Malaria Prophylaxis & Prevention 1380
Pneumocystis Jiroveci Infection 1348 Visits to Friends & Relatives (VFR)
in High-Risk Areas 1381
44. Sexually Transmitted Infections 1351 HIV & Sexually Transmitted Diseases 1381
Fever in the Returned Traveler 1384
Daniel H. Reirden, MD References 1388
Ann-Christine Nyquist, MD, MSPH
Adolescent Sexuality 1351 46. Chemistry & Hematology Reference
Risk Factors 1352 Intervals 1389
Prevention of Sexually
Transmitted Infections 1352 Jordana E. Hoppe, MD
Screening for Sexually Robert Snyder, MT (ASCP)
Transmitted Infections 1352 Frank J. Accurso, MD
Signs & Symptoms 1353 Georgette Siparsky, PhD
The Most Common Antibiotic- Challenges in Determining & Interpreting
Responsive Sexually Transmitted Pediatric Reference Intervals 1389
Infections 1353 Guidelines for Use of Data in a Reference
Chlamydia Trachomatis Infection 1353 Range Study 1389
Neisseria Gonorrhoeae Infection 1354 Statistical Computation of
The Spectrum of Signs & Symptoms of Reference Intervals 1390
Sexually Transmitted Infections 1360 Why Reference Intervals Vary 1391
Cervicitis 1360 Sensitivity & Specificity 1391
Pelvic Inflammatory Disease 1361 Pediatric Reference Intervals 1392
Urethritis 1362
Epididymitis 1363 Index 1405
Proctitis, Proctocolitis, & Enteritis 1363

Hay_FM_pi-xxxii.indd 18 30/03/18 11:40 AM


Authors
Jordan K. Abbott, MD Sarah Bartz, MD
Assistant Professor, Department of Pediatrics, University of Assistant Professor, Department of Pediatrics, Section of
Colorado School of Medicine and Children’s Hospital Pediatric Endocrinology, University of Colorado School of
Colorado; Division of Pediatric Allergy, Asthma and Clinical Medicine and Children’s Hospital Colorado
Immunology, National Jewish Health Chapter 34: Endocrine Disorders
Chapter 33: Immunodeficiency
Margret Bock, MD, MS
Frank J. Accurso, MD Assistant Professor, Department of Pediatrics, Section of
Professor, Department of Pediatrics, Section of Pediatric Pediatric Nephrology, University of Colorado and Children’s
Pulmonary Medicine, University of Colorado School of Hospital Colorado
Medicine and Children’s Hospital Colorado Chapter 23: Fluid, Electrolyte, & Acid-Base Disorders & Therapy
Chapter 46: Chemistry & Hematology Reference Intervals
Mark Boguniewicz, MD
Daniel R. Ambruso, MD Professor, Department of Pediatrics, University of Colorado
Professor, Department of Pediatrics, Section of Pediatric School of Medicine and Children’s Hospital Colorado;
Hematology, Oncology, and Bone Marrow Transplant, Division of Pediatric Allergy, Asthma and Clinical
University of Colorado School of Medicine and Children’s Immunology, National Jewish Health
Hospital Colorado Chapter 38: Allergic Disorders
Chapter 30: Hematologic Disorders
Rebecca Sands Braverman, MD
Marsha S. Anderson, MD Associate Professor, Department of Ophthalmology,
Professor, Department of Pediatrics, Section of University of Colorado School of Medicine
Pediatric Infectious Diseases, University of Colorado School Chapter 16: Eye
of Medicine and Children’s Hospital Colorado
Chapter 42: Infections: Bacterial & Spirochetal David Brumbaugh, MD
Associate Professor, Department of Pediatrics, Section of
Edwin J. Asturias, MD Pediatric Gastroenterology Hepatology and Nutrition,
Associate Professor, Departments of Pediatrics and University of Colorado School of Medicine and Children’s
Epidemiology and Infectious Diseases, University of Hospital Colorado
Colorado School of Medicine; Associate Director, Center for Chapter 21: Gastrointestinal Tract
Global Health, Colorado School of Public Health
Chapter 40: Infections: Viral & Rickettsial
Maya Bunik, MD, MSPH
Professor, Department of Pediatrics, Section of General
Christopher D. Baker, MD Academic Pediatrics, Director of the Child Health Clinic,
Associate Professor, Department of Pediatrics, Section of University of Colorado Denver School of Medicine and
Pediatric Pulmonary Medicine, University of Colorado Children’s Hospital Colorado
School of Medicine and Children’s Hospital Colorado Chapter 9: Ambulatory & Office Pediatrics
Chapter 19: Respiratory Tract & Mediastinum

Peter R. Baker II, MD Dale Burkett, MD


Assistant Professor, Department of Pediatrics, Section of
Assistant Professor, Department of Pediatrics, Section of
Pediatric Cardiology, University of Colorado School of
Clinical Genetics and Metabolism, University of Colorado
Medicine and Children’s Hospital Colorado
School of Medicine and Children’s Hospital Colorado
Chapter 20: Cardiovascular Diseases
Chapter 36: Inborn Errors of Metabolism

Jennifer M. Barker, MD Adam Burstein, DO


Senior Clinical Instructor, Department of Child & Adolescent
Associate Professor, Department of Pediatrics, Section of
Psychiatry, Psychiatry Resident Training Programs,
Pediatric Endocrinology, University of Colorado School of
University of Colorado School of Medicine and Children’s
Medicine and Children’s Hospital Colorado
Hospital Colorado
Chapter 34: Endocrine Disorders
Chapter 7: Child & Adolescent Psychiatric Disorders &
Psychosocial Aspects of Pediatrics

xix

Hay_FM_pi-xxxii.indd 19 30/03/18 11:40 AM


xx AUTHORS

Melissa A. Cadnapaphornchai, MD Gerald H. Clayton, PhD


Professor, Departments of Pediatrics and Medicine, Instructor, Department of Physical Medicine and
University of Colorado School of Medicine, Section of Rehabilitation, University of Colorado School of Medicine
Pediatric Nephrology, Director, Pediatric Continuous Renal and Children’s Hospital Colorado
Replacement Therapy, Hemodialysis, and Peritoneal Chapter 28: Rehabilitation Medicine
Dialysis, The Kidney Center, Children’s Hospital Colorado
Chapter 24: Kidney & Urinary Tract Amy Clevenger, MD, PhD
Assistant Professor, Department of Pediatrics, Section of
Michelle Caraballo, MD Pediatric Critical Care Medicine, University of Colorado
Former Fellow, Department of Pediatrics, Section of Pediatric School of Medicine and Children’s Hospital Colorado
Pulmonary Medicine, University of Colorado School of Chapter 14: Critical Care
Medicine and Children’s Hospital Colorado
Assistant Professor, Department of Pediatrics, Division of Kathryn K. Collins, MD
Respiratory Medicine, University of Texas Southwestern Professor, Department of Pediatrics, Section of
Medical Center Pediatric Cardiology, University of Colorado School of
Chapter 19: Respiratory Tract & Mediastinum Medicine and Children’s Hospital Colorado
Chapter 20: Cardiovascular Diseases
Kevin P. Carney, MD
Assistant Professor, Department of Pediatrics, Section of Ronina A. Covar, MD
Emergency Medicine, University of Colorado School of Associate Professor, Department of Pediatrics, University of
Medicine, Director of Quality Improvement, Children’s Colorado School of Medicine and Children’s Hospital
Hospital Colorado Colorado; Division of Pediatric Allergy, Asthma and Clinical
Chapter 12: Emergencies & Injuries Immunology, National Jewish Health
Chapter 38: Allergic Disorders
Todd C. Carpenter, MD
Associate Professor, Department of Pediatrics, Section of John A. Craddock, MD
Pediatric Critical Care Medicine, University of Colorado Assistant Professor, Department of Pediatrics, Section of
School of Medicine and Children’s Hospital Colorado Pediatric Hematology, Oncology, and Bone Marrow
Chapter 14: Critical Care Transplant, Center for Cancer and Blood Disorders,
University of Colorado School of Medicine and Children’s
H. Peter Chase, MD Hospital Colorado
Professor, Department of Pediatrics, Clinical Director Emeritus, Chapter 31: Neoplastic Disease
Barbara Davis Center for Childhood Diabetes, University of
Colorado School of Medicine Angela S. Czaja, MD, MSc
Chapter 35: Diabetes Mellitus Associate Professor, Department of Pediatrics, Section of
Pediatric Critical Care Medicine, University of Colorado
Antonia Chiesa, MD School of Medicine and Children’s Hospital Colorado
Associate Professor, Department of Pediatrics, Kempe Child Chapter 14: Critical Care
Protection Team, University of Colorado School of
Medicine, Children’s Hospital Colorado and Kempe Katherine S. Dahab, MD, FAAP, CAQSM
Center for the Prevention and Treatment of Child Abuse Assistant Professor, Department of Orthopedics, Fellowship
and Neglect Director, Pediatric Primary Care Sports Medicine
Chapter 8: Child Abuse & Neglect Fellowship, University of Colorado School of Medicine and
Children’s Hospital Colorado
Jason Child, PharmD Chapter 27: Sports Medicine
Co-Director of Antimicrobial Stewardship, Infectious Disease
Clinical Specialist, Department of Pharmacy, University of Matthew F. Daley, MD
Colorado Skaggs School of Pharmacy and Pharmaceutical Associate Professor, Department of Pediatrics, Section of
Sciences and Children’s Hospital Colorado General Academic Pediatrics, University of Colorado School
Chapter 39: Antimicrobial Therapy of Medicine and Children’s Hospital Colorado
Chapter 10: Immunization
Christine Cho, MD
Assistant Professor, Department of Pediatrics, University Jeffrey R. Darst, MD
Colorado School of Medicine and Children’s Hospital Associate Professor, Department of Pediatrics, Section of
Colorado; Division of Allergy, Asthma and Clinical Pediatric Cardiology, University of Colorado School of
Immunology, National Jewish Health Medicine and Children’s Hospital Colorado
Chapter 38: Allergic Disorders Chapter 20: Cardiovascular Diseases

Hay_FM_pi-xxxii.indd 20 30/03/18 11:40 AM


AUTHORS xxi

Richard C. Dart, MD, PhD Anna Franklin, MD


Professor, Departments of Regulatory Compliance and Associate Professor, Department of Pediatrics,
Emergency Medicine, University of Colorado School of Section of Pediatric Hematology, Oncology, and
Medicine; Director, Rocky Mountain Poison and Drug Bone Marrow Transplant, University of Colorado
Center, Denver Health and Hospital Authority School of Medicine and Children’s Hospital Colorado
Chapter 13: Poison Chapter 31: Neoplastic Disease

Emily M. Deboer, MD Norman R. Friedman, MD


Assistant Professor, Department of Pediatrics, Section of Associate Professor, Departments of Otolaryngology &
Pediatric Pulmonary Medicine, University of Colorado Pediatrics, University of Colorado School of Medicine and
School of Medicine and Children’s Hospital Colorado Children’s Hospital Colorado
Chapter 19: Respiratory Tract & Mediastinum Chapter 18: Ear, Nose, & Throat

Scott Demarest, MD Brigitte I. Frohnert, MD, PhD


Assistant Professor, Department of Pediatrics, Neurology, Assistant Professor, Department of Pediatrics, Barbara Davis
University of Colorado School of Medicine and Children’s Center for Childhood Diabetes, University of Colorado
Hospital Colorado School of Medicine
Chapter 25: Neurologic & Muscular Disorders Chapter 35: Diabetes Mellitus

Samuel R. Dominguez, MD, PhD Glenn T. Furuta, MD


Associate Professor, Department of Pediatrics, Section of Professor, Department of Pediatrics, Section of
Pediatric Infectious Diseases, University of Colorado School Gastroenterology, Hepatology and Nutrition, Attending
of Medicine and Children’s Hospital Colorado Physician, Digestive Health Institute; Director,
Chapter 43: Infections: Parasitic & Mycotic Gastrointestinal Eosinophil Disease Program, University
of Colorado School of Medicine and Children’s Hospital
Ellen R. Elias, MD Colorado
Professor, Departments of Pediatrics and Genetics, Director, Chapter 21: Gastrointestinal Tract
Special Care Clinic, University of Colorado School of
Medicine and Children’s Hospital Colorado James Gaensbauer, MD, MScPH
Chapter 37: Genetics & Dysmorphology Assistant Professor, University of Colorado, Children’s
Hospital Colorado Center for Global Health, Colorado
Mark A. Erickson, MD, MMM School of Public Health Pavilion for Women and Children
Chairman, Department of Pediatric Orthopaedics, Professor Chapter 42: Infections: Bacterial & Spirochetal
of Orthopaedic Surgery, Orthopedic Institute, University Chapter 43: Infections: Parasitic & Mycotic
of Colorado School of Medicine and Children’s Hospital
Colorado Jeffrey L. Galinkin, MD, FAAP
Chapter 26: Orthopedics Professor, Department of Anesthesiology, University of
Colorado School of Medicine and Children’s
Monica J. Federico, MD Hospital Colorado
Associate Professor, Department of Pediatrics, Section of Chapter 32: Pain Management & Pediatric Palliative &
Pediatric Pulmonary Medicine, University of Colorado End-of-Life Care
School of Medicine and Children’s Hospital Colorado
Chapter 19: Respiratory Tract & Mediastinum Roopa Gandhi, BDS, MSD
Assistant Professor, Residency Program Director, Department
David M. Fleischer, MD of Pediatric Dentistry, University of Colorado School of
Associate Professor, Department of Pediatrics, University of Dental Medicine and Children’s Hospital Colorado
Colorado School of Medicine and Children’s Hospital Chapter 17: Oral Medicine & Dentistry
Colorado; Division of Pediatric Allergy, Asthma and Clinical
Immunology, National Jewish Health Camille Gannam, DMD, MS
Chapter 38: Allergic Disorders Assistant Professor, Department of Pediatric Dentistry,
University of Colorado School of Dental Medicine and
David Fox, MD Children’s Hospital Colorado
Associate Professor, Department of Pediatrics, Section of Chapter 17: Oral Medicine & Dentistry
General Academic Pediatrics, University of Colorado School
of Medicine and Children’s Hospital Colorado
Chapter 9: Ambulatory & Office Pediatrics

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xxii AUTHORS

Timothy Garrington, MD Melisha G. Hanna, MD, MS


Associate Professor, Department of Pediatrics, Section of Assistant Professor, Department of Pediatrics, Section of
Pediatric Hematology, Oncology, and Bone Marrow Pediatric Nephrology, University of Colorado School of
Transplant, University of Colorado School of Medicine and Medicine and Children’s Hospital Colorado
Children’s Hospital Colorado Chapter 23: Fluid, Electrolyte, & Acid-Base Disorders & Therapy
Chapter 31: Neoplastic Disease
Pia J. Hauk, MD
Roger H. Giller, MD Associate Professor, Department of Pediatrics, University of
Professor, Department of Pediatrics, Section of Pediatric Colorado School of Medicine and National Jewish Health
Hematology, Oncology, and Bone Marrow Transplant, Chapter 33: Immunodeficiency
Director, Pediatric BMT Program, University of Colorado
School of Medicine and Children’s Hospital Colorado Stephen Hawkins, MD, DO
Chapter 31: Neoplastic Disease Assistant Professor, Department of Pediatrics, Section of
Pediatric Pulmonary Medicine, University of Colorado
Edward Goldson, MD School of Medicine and Children’s Hospital Colorado
Professor, Department of Pediatrics, Section of Developmental Chapter 19: Respiratory Tract & Mediastinum
Pediatrics, University of Colorado School of Medicine and
Children’s Hospital Colorado Edward J. Hoffenberg, MD
Chapter 3: Child Development & Behavior Professor, Department of Pediatrics, Section of
Pediatric Gastroenterology, Hepatology and Nutrition,
Eva N. Grayck, MD University of Colorado School of Medicine and Children’s
Professor, Department of Pediatrics, Section of Hospital Colorado
Pediatric Critical Care Medicine, University of Colorado Chapter 21: Gastrointestinal Tract
School of Medicine and Children’s Hospital Colorado
Chapter 14: Critical Care Daniel Hyman, MD
Associate Professor, Department of Pediatrics, University of
Brian S. Greffe, MD Colorado School of Medicine, Chief Quality Officer,
Professor, Department of Pediatrics, Section of Pediatric Children’s Hospital Colorado
Hematology, Oncology, and Bone Marrow Transplant, Chapter 1: Advancing the Quality & Safety of Care
University of Colorado School of Medicine and Children’s
Hospital Colorado Pei-Ni Jone, MD
Chapter 31: Neoplastic Disease Associate Professor, Department of Pediatrics, Section of
Chapter 32: Pain Management & Pediatric Palliative & Pediatric Cardiology, Children’s Hospital Colorado and
End-of-Life Care University of Colorado School of Medicine
Chapter 20: Cardiovascular Diseases
Theresa Grover, MD
Associate Professor, Department of Pediatrics, Section of Jennifer Lee Jung, MD
Neonatology, University of Colorado School of Medicine Assistant Professor, Department of Ophthalmology, University of
and Children’s Hospital Colorado Colorado School of Medicine, Children’s Hospital Colorado
Chapter 2: The Newborn Infant Chapter 16: Eye

Cameron F. Gunville, DO Michael S. Kappy, MD, PhD


Assistant Professor, Department of Pediatrics, Section of Professor, Department of Pediatrics, Section of Pediatric
Pediatric Critical Care Medicine, University of Colorado Endocrinology, University of Colorado School of Medicine
School of Medicine and Children’s Hospital and Children’s Hospital Colorado
Chapter 14: Critical Care Chapter 34: Endocrine Disorders

Matthew A. Haemer, MD, MPH Paritosh Kaul, MD


Associate Professor, Department of Pediatrics, Section of Professor, Department of Pediatrics, Section of Adolescent
Pediatric Nutrition, University of Colorado School of Medicine, University of Colorado School of Medicine,
Medicine and Children’s Hospital Colorado Children’s Hospital Colorado, and Denver Health Medical
Chapter 11: Normal Childhood Nutrition & Its Disorders Center
Chapter 5: Adolescent Substance Abuse
Ann C. Halbower, MD
Professor, Department of Pediatrics, Section of Pediatric
Pulmonology, University of Colorado School of Medicine
and Children’s Hospital Colorado
Chapter 19: Respiratory Tract & Mediastinum

Hay_FM_pi-xxxii.indd 22 30/03/18 11:40 AM


AUTHORS xxiii

Amy K. Keating, MD Oren Kupfer, MD


Associate Professor, Department of Pediatrics, Section of Assistant Professor, Department of Pediatrics, Section of
Pediatric Hematology, Oncology, and Bone Marrow Pediatric Pulmonary Medicine, University of Colorado
Transplant, Center for Cancer and Blood Disorders, School of Medicine and Children’s Hospital Colorado
University of Colorado School of Medicine and Children’s Chapter 19: Respiratory Tract & Mediastinum
Hospital Colorado
Chapter 31: Neoplastic Disease Myron J. Levin, MD
Professor, Departments of Pediatrics and Medicine,
Kimberly Kelsay, MD Section of Pediatric Infectious Diseases, University
Associate Professor, Department of Psychiatry and Behavioral of Colorado School of Medicine and Children’s Hospital
Sciences, University of Colorado School of Medicine and Colorado
Children’s Hospital Colorado Chapter 40: Infections: Viral & Rickettsial
Chapter 7: Child & Adolescent Psychiatric Disorders & Chapter 43: Infections: Parasitic & Mycotic
Psychosocial Aspects of Pediatrics
Gary M. Lum, MD
Megan Moriarty Kelsey, MD, MS Clinical Professor, Departments of Pediatrics and Medicine,
Associate Professor, Department of Pediatrics, Section of Section of Pediatric Nephrology, University of Colorado
Pediatric Endocrinology, University of Colorado School of School of Medicine and Children’s Hospital Colorado
Medicine and Children’s Hospital Colorado Chapter 24: Kidney & Urinary Tract
Chapter 34: Endocrine Disorders
Cara L. Mack, MD
Nancy A. King, MSN, RN, CPNP Associate Professor, Department of Pediatrics, Section of
Inpatient Oncology Advanced Practice Nurse, Department of Pediatric Gastroenterology, Hepatology and Nutrition,
Pediatrics, Section of Hematology, Oncology, and Bone University of Colorado School of Medicine and Children’s
Marrow Transplant, University of Colorado School of Hospital Colorado
Medicine and Children’s Hospital Colorado Chapter 22: Liver & Pancreas
Chapter 32: Pain Management & Pediatric Palliative
& End-of-Life Care Kelly Maloney, MD
Professor, Department of Pediatrics, Section of
Ulrich Klein, DMD, DDS, MS Pediatric Gastroenterology, Hepatology and Nutrition,
Associate Professor and Chair, Department of Pediatric University of Colorado School of Medicine and Children’s
Dentistry, Director of Pediatric Dentistry Residency Hospital Colorado
Program, University of Colorado School of Dental Medicine Chapter 31: Neoplastic Disease
and Children’s Hospital Colorado
Chapter 17: Oral Medicine & Dentistry Jan A. Martin, MD
Assistant Professor, Department of Pediatrics, Section of
Gregory Kobak, MD Neurology Genops, University of Colorado School of
Senior Instructor, Department of Pediatrics, Section of Medicine and Children’s Hospital Colorado
Pediatric Gastroenterology, Hepatology and Nutrition, Chapter 25: Neurologic & Muscular Disorders
Digestive Health Institute, University of Colorado School of
Medicine and Children’s Hospital Colorado Stacey L. Martiniano, MD
Chapter 21: Gastrointestinal Tract Assistant Professor, Department of Pediatrics, Section of
Pediatric Pulmonary Medicine, University of Colorado
Robert E. Kramer, MD School of Medicine and Children’s Hospital Colorado
Professor, Department of Pediatrics, Section of Chapter 19: Respiratory Tract & Mediastinum
Pediatric Gastroenterology, Hepatology and Nutrition,
University of Colorado School of Medicine and Children’s Elizabeth J. McFarland, MD
Hospital Colorado Professor, Department of Pediatrics, Section of Pediatric
Chapter 21: Gastrointestinal Tract Infectious Diseases, University of Colorado School of
Medicine and Children’s Hospital Colorado
Nancy F. Krebs, MD, MS Chapter 41: Human Immunodeficiency Virus Infection
Professor, Department of Pediatrics, Section of Pediatric
Nutrition, University of Colorado School of Medicine and Naomi J. L. Meeks, MD
Children’s Hospital Colorado Assistant Professor, Department of Pediatrics, Section of
Chapter 11: Normal Childhood Nutrition & Its Disorders Clinical Genetics and Metabolism, University of Colorado
School of Medicine
Chapter 37: Genetics & Dysmorphology

Hay_FM_pi-xxxii.indd 23 30/03/18 11:40 AM


xxiv AUTHORS

Kevin Messacar, MD Rachelle Nuss, MD


Assistant Professor, Department of Pediatrics, Section of Professor, Department of Pediatrics, Section of Pediatric
Pediatric Infectious Diseases, University of Colorado School Hematology, Oncology, and Bone Marrow Transplant;
of Medicine and Children’s Hospital Colorado Associate Director, Colorado Sickle Cell Treatment and
Chapter 43: Infections: Parasitic & Mycotic Research Center at University of Colorado School of
Medicine; Director Pediatric Sickle Cell Program, Center for
Ricka Messer, MD, PhD Cancer and Blood Disorders, Children’s Hospital Colorado
Assistant Professor, Department of Pediatrics, Neurology, Chapter 30: Hematologic Disorders
University of Colorado School of Medicine and Children’s
Hospital Colorado Ann-Christine Nyquist, MD, MSPH
Chapter 25: Neurologic & Muscular Disorders Professor, Department of Pediatrics, Section of Pediatric
Infectious Diseases, University of Colorado School of
Shelley D. Miyamoto, MD Medicine and Children’s Hospital Colorado
Associate Professor, Department of Pediatrics, Section of Chapter 10: Immunization
Pediatric Cardiology, University of Colorado School of Chapter 44: Sexually Transmitted Infections
Medicine and Children’s Hospital Colorado
Chapter 20: Cardiovascular Diseases Sean T. O’Leary, MD, MPH
Associate Professor, Department of Pediatrics, Section
Jean M. Mulcahy Levy, MD of Pediatric Infectious Diseases, University of Colorado
Assistant Professor, Department of Pediatrics, Center for School of Medicine and Children’s Hospital Colorado
Cancer and Blood Disorders, University of Colorado Chapter 10: Immunization
Chapter 31: Neoplastic Disease
John W. Ogle, MD
Kyle B. Nagle, MD, MPH, FAAP, CAQSM Professor and Vice Chairman Emeritus, Department of
Assistant Professor, Department of Orthopedics, University of Pediatrics, University of Colorado School of Medicine
Colorado School of Medicine, Sports Medicine for Young Chapter 39: Antimicrobial Therapy
Athletes, Children’s Hospital of Colorado Orthopedics Chapter 42: Infections: Bacterial & Spirochetal
Institute
Chapter 27: Sports Medicine Sarah K. Parker, MD
Associate Professor, Department of Pediatrics, Section of
Michael R. Narkewicz, MD Pediatric Infectious Diseases, University of Colorado School
Professor, Department of Pediatrics, Fellowship Program of Medicine and Children’s Hospital Colorado
Director, Section of Pediatric Gastroenterology, Chapter 39: Antimicrobial Therapy
Hepatology and Nutrition, Hewitt Andrews Chair in
Pediatric Liver Diseases, Medical Director, Pediatric Liver Laura E. Primak, RD, CNSD
Center and Liver Transplant Pediatric Services Professional Research Assistant, Coordinator of Nutrition
Associate Clinical Director/Associate Dean for Pediatric Electives, Section of Pediatric Nutrition, University of
Clinical Affairs, University of Colorado School of Medicine Colorado School of Medicine and Children’s Hospital
and Children’s Hospital Colorado Colorado
Chapter 22: Liver & Pancreas Chapter 11: Normal Childhood Nutrition & Its Disorders

Daniel Nicklas, MD Lori D. Prok, MD


Assistant Professor of Pediatrics, Director of Primary Care Associate Professor, Departments of Pediatrics and
Education, Children’s Hospital Colorado Residency Dermatology, Section of Pediatric Dermatology, University
Program, University of Colorado and Children’s Hospital of Colorado School of Medicine and Children’s Hospital
Colorado Colorado
Chapter 9: Ambulatory & Office Pediatrics Chapter 15: Skin

Cameron Niswander, BA Ralph R. Quinones, MD


Third Year Medical Student Clinical Professor, Department of Pediatrics, Section of
University of Colorado School of Medicine Pediatric Hematology, Oncology, and Bone Marrow
Chapter 26: Orthopedics Transplant, Center for Cancer and Blood Disorders,
University of Colorado School of Medicine and Children’s
Yosuke Nomura, MD Hospital Colorado
Pediatrician and Instructor of Pediatrics, Denver Health, Chapter 31: Neoplastic Disease
University of Colorado School of Medicine
Chapter 42: Infections: Bacterial & Spirochetal

Hay_FM_pi-xxxii.indd 24 30/03/18 11:40 AM


AUTHORS xxv

Suchitra Rao, MBBS Margarita Sifuentes Saenz, MD


Assistant Professor, Department of Pediatrics, Section of Assistant Professor, Department of Pediatrics, Section of
Pediatric Infectious Disease, University of Colorado School Clinical Genetics and Metabolism, University of Colorado
of Medicine and Children’s Hospital Colorado School of Medicine and Children’s Hospital Colorado
Chapter 45: Travel Medicine Chapter 37: Genetics & Dysmorphology

Daniel H. Reirden, MD Scott D. Sagel, MD


Associate Professor, Department of Pediatrics, Section of Professor, Department of Pediatrics, Section of
Adolescent Medicine and Internal Medicine, Medical Pediatric Pulmonary Medicine, University of Colorado
Director, CHIP Youth Clinic, Director of Internal School of Medicine and Children’s Hospital Colorado
Medicine—Pediatric Residency, University of Colorado Chapter 19: Respiratory Tract & Mediastinum
School of Medicine and Children’s Hospital Colorado
Chapter 44: Sexually Transmitted Infections Amy E. Sass, MD, MPH
Associate Professor, Department of Pediatrics, Section of
Marian Rewers, MD, PhD Adolescent Medicine, University of Colorado School of
Professor, Department of Pediatrics, Clinical Director, Barbara Medicine and Children’s Hospital Colorado
Davis Center for Childhood Diabetes, University of Chapter 4: Adolescence
Colorado School of Medicine
Chapter 35: Diabetes Mellitus
Melissa A. Scholes, MD
Ann Reynolds, MD Assistant Professor, Department of Pediatric Otolaryngology,
Associate Professor, Department of Pediatrics, Section of University of Colorado School of Medicine and Children’s
Developmental Pediatrics, University of Colorado School of Hospital Colorado
Medicine; Director, The Child Development Unit, Children’s Chapter 18: Ear, Nose, Throat
Hospital Colorado
Chapter 3: Child Development & Behavior Teri L. Schreiner, MD, MPH
Associate Professor, Departments of Pediatrics and
Jason Rhodes, MD, MS Neurology, Section of Pediatric Neurology, University
Associate Professor, Departments of Mechanical and Materials of Colorado School of Medicine and Children’s Hospital
Engineering, Director, Cerebral Palsy Program, Orthopedics Colorado
Institute, University of Colorado School of Medicine and Chapter 25: Neurologic & Muscular Disorders
Children’s Hospital Colorado
Chapter 26: Orthopedics Justin Searns, MD
Pediatric Infectious Diseases Fellow, Department of Pediatrics,
Molly J. Richards, MD Section of Pediatric Infectious Diseases, University of
Associate Professor, Department of Pediatrics, Section of Colorado School of Medicine and Children’s
Adolescent Medicine, University of Colorado School of Hospital Colorado
Medicine and Children’s Hospital Colorado Chapter 39: Antimicrobial Therapy
Chapter 4: Adolescence
Eric J. Sigel, MD
Leslie Ridall, DO Professor, Department of Pediatrics, Section of
Assistant Professor, Department of Pediatrics, Section of Adolescent Medicine, University of Colorado School of
Pediatric Critical Care Medicine, University of Colorado Medicine and Children’s Hospital Colorado
School of Medicine and Children’s Hospital Colorado Chapter 6: Eating Disorders
Chapter 14: Critical Care
Georgette Siparsky, PhD
Kelley Roswell, MD Clinical Data Analyst II, Department of Clinical Informatics,
Assistant Professor, Department of Pediatrics, Section of
Children’s Hospital Colorado, and University of Colorado
Emergency Medicine, Co-Director Medical Student
School of Medicine
Emergency Care Clerkship, University of Colorado School
Chapter 46: Chemistry & Hematology Reference Intervals
of Medicine and Children’s Hospital Colorado
Chapter 12: Emergencies & Injuries
Andrew P. Sirotnak, MD
Barry H. Rumack, MD Professor and Vice Chair for Faculty Affairs, Department of
Clinical Professor, Department of Pediatrics, University of Pediatrics, Director, Child Protection Team, University of
Colorado School of Medicine; Director Emeritus, Rocky Colorado School of Medicine, Children’s Hospital Colorado
Mountain Poison and Drug Center, Denver Health Chapter 8: Child Abuse & Neglect
Authority
Chapter 13: Poison

Hay_FM_pi-xxxii.indd 25 30/03/18 11:40 AM


xxvi AUTHORS

Danielle Smith, MD Ayelet Talmi, PhD


Assistant Professor, Department of Pediatrics, Section of Assistant Professor, Departments of Psychiatry and Pediatrics,
Neonatology, University of Colorado School of Medicine Associate Director, Irving Harris Program in Child
and Children’s Hospital Colorado Development and Infant Mental Health, University of
Chapter 2: The Newborn Infant Colorado School of Medicine and Children’s Hospital
Colorado
Robert Snyder, MT (ASCP) Chapter 7: Child & Adolescent Psychiatric Disorders &
Clinical Chemistry Supervisor, Department of Pathology and Psychosocial Aspects of Pediatrics
Laboratory Medicine, University of Colorado, Children’s
Hospital Colorado Janet A. Thomas, MD
Chapter 46: Chemistry & Hematology Reference Intervals Associate Professor, Department of Pediatrics, Section of
Clinical Genetics and Metabolism, Director—IMD Clinic,
Jason Soden, MD University of Colorado School of Medicine and Children’s
Associate Professor, Department of Pediatrics, Section of Hospital Colorado
Pediatric Gastroenterology, Hepatology and Nutrition, Chapter 36: Inborn Errors of Metabolism
University of Colorado School of Medicine and Children’s
Hospital Colorado Carla X. Torres-Zegarra, MD
Chapter 21: Gastrointestinal Tract Assistant Professor, Pediatric Dermatology Fellow, Pediatric
Dermatology, Department of Dermatology, University of
Jennifer B. Soep, MD Colorado and Children’s Hospital Colorado
Associate Professor, Department of Pediatrics, Section of Chapter 15: Skin
Pediatric Rheumatology, University of Colorado School of
Medicine and Children’s Hospital Colorado Sharon H. Travers, MD
Chapter 29: Rheumatic Diseases Associate Professor, Department of Pediatrics, Section of
Pediatric Endocrinology, University of Colorado School of
Ronald J. Sokol, MD, FAASLD Medicine and Children’s Hospital Colorado
Professor and Vice Chair, Department of Pediatrics, Arnold Chapter 34: Endocrine Disorders
Silverman MD Endowed Chair in Digestive Health; Head,
Section of Pediatric Gastroenterology, Hepatology and Meghan Treitz, MD
Nutrition and the Digestive Health Institute, University of Assistant Professor, Department of Pediatrics, Section of
Colorado School of Medicine and Children’s Hospital General Academic Pediatrics, University of Colorado
Colorado; Director, Colorado Clinical and Translational Denver School of Medicine and Children’s Hospital
Sciences Institute and Assistant Vice Chancellor for Clinical Colorado
and Translational Science, University of Colorado Denver Chapter 9: Ambulatory & Office Pediatrics
Chapter 22: Liver & Pancreas
Anne Chun-Hui Tsai, MD, MSc
Paul Stillwell, MD Professor, Department of Pediatrics, Section of
Senior Instructor, Department of Pediatrics, Section of Pediatric Clinical Genetics and Metabolism, University
Pediatric Pulmonary Medicine, University of Colorado of Colorado School of Medicine and Children’s Hospital
School of Medicine and Children’s Hospital Colorado Colorado
Chapter 19: Respiratory Tract & Mediastinum Chapter 37: Genetics & Dysmorphology

Shikha S. Sundaram, MD, MSCI Johan L. K. Van Hove, MD, PhD, MBA
Associate Professor, Department of Pediatrics, Section of Professor, Department of Pediatrics, Head, Section of Clinical
Pediatric Gastroenterology, Hepatology and Nutrition, Genetics and Metabolism, University of Colorado School of
University of Colorado School of Medicine and Children’s Medicine and Children’s Hospital Colorado
Hospital Colorado Chapter 36: Inborn Errors of Metabolism
Chapter 22: Liver & Pancreas
Armando Vidal, MD
Alex Tagawa, BS Associate Professor, Department of Orthopedics—Sports
Research Assistant, Center for Gait and Movement Analysis, Medicine Program, University of Colorado School of
Department of Pediatric Orthopedics, University Medicine and Children’s Hospital Colorado
of Colorado School of Medicine and Children’s Hospital Chapter 27: Sports Medicine
Colorado
Chapter 26: Orthopedics

Hay_FM_pi-xxxii.indd 26 30/03/18 11:40 AM


Contents xxvii

Johannes Von Alvensleben, MD Pamela E. Wilson, MD


Assistant Professor, Department of Pediatrics, Section of Associate Professor, Department of Physical Medicine &
Pediatric Cardiology, University of Colorado School of Rehabilitation Medicine, University of Colorado School of
Medicine and Children’s Hospital Colorado Medicine and Children’s Hospital Colorado
Chapter 20: Cardiovascular Diseases Chapter 28: Rehabilitation Medicine

Thomas Walker Michele L. Yang, MD


Professor of Pediatrics, Section of Pediatric Gastroenterology, Assistant Professor, Departments of Pediatrics and Neurology,
Hepatology and Nutrition, Digestive Health Institute, Section of Child Neurology, University of Colorado School
University of Colorado School of Medicine and Children’s of Medicine and Children’s Hospital Colorado
Hospital Colorado Chapter 25: Neurologic & Muscular Disorders
Chapter 21: Gastrointestinal Tract
Patricia J. Yoon, MD
Diana Walleigh, MD Associate Professor, Department of Otolaryngology, Associate
Assistant Professor, Department of Pediatrics, Neurology, Director, Bill Daniels Center for Children’s Hearing,
University of Colorado School of Medicine and Children’s University of Colorado School of Medicine and Children’s
Hospital Colorado Hospital Colorado
Chapter 25: Neurologic & Muscular Disorders Chapter 18: Ear, Nose, & Throat

George Sam Wang, MD Carleen Zebuhr, MD


Assistant Professor, Department of Pediatrics, Section of Assistant Professor, Department of Pediatrics, Section of
Emergency Medicine, Medical Toxicology, University of Pediatric Critical Care Medicine, University of Colorado
Colorado School of Medicine and Children’s Hospital School of Medicine and Children’s Hospital Colorado
Colorado Chapter 14: Critical Care
Chapter 13: Poison
Philip S. Zeitler, MD, PhD
Michael Wang, MD Professor, Department of Pediatrics, Head, Section of Pediatric
Associate Professor, Department of Pediatrics, Section of Endocrinology, University of Colorado School of Medicine
Pediatric Hematology, Oncology, and Bone Marrow and Children’s Hospital Colorado
Transplant, Center for Cancer and Blood Disorders, Chapter 34: Endocrine Disorders
University of Colorado School of Medicine and Children’s
Hospital Colorado Edith T. Zemanick, MD
Chapter 30: Hematologic Disorders Associate Professor, Department of Pediatrics Section of
Pediatric Pulmonary Medicine, University of Colorado
Adriana Weinberg, MD School of Medicine and Children’s Hospital Colorado
Professor, Departments of Pediatrics and Medicine, Director of Chapter 19: Respiratory Tract & Mediastinum
Clinical Virology Laboratory, Section of Pediatric Infectious
Diseases, University of Colorado School of Medicine and
Children’s Hospital Colorado
Chapter 40: Infections: Viral & Rickettsial

Hay_FM_pi-xxxii.indd 27 30/03/18 11:40 AM


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Preface
The 24th edition of Current Diagnosis & Treatment: Pediatrics (CDTP) features practical, up-to-date, well-referenced informa-
tion on the care of children from birth through infancy, childhood, and adolescence. CDTP emphasizes the clinical aspects
of pediatric care in the context of important underlying principles. CDTP provides a guide to diagnosis, understanding, and
treatment of the medical problems of all pediatric patients in an easy-to-use and readable formaat.

INTENDED AUDIENCE
Like all Lange medical books, CDTP provides a concise yet comprehensive source of current information. Students will find
CDTP an authoritative introduction to pediatrics and an excellent source for reference and review. CDTP provides excellent
coverage of The Council on Medical Student Education in Pediatrics (COMSEP) curriculum used in pediatric clerkships.
Residents in pediatrics (and other specialties) will appreciate the detailed descriptions of diseases as well as diagnostic and
therapeutic procedures. Pediatricians, family practitioners, nurses, nurse practitioners, physician assistants, and other health
care providers who work with infants, children, and adolescents will find CDTP a useful reference on management aspects of
pediatric medicine.

COVERAGE
Forty-six chapters cover a wide range of topics, including neonatal medicine, child development and behavior, emergency and
critical care medicine, and diagnosis and treatment of specific disorders according to major problems, etiologies, and organ
systems. A wealth of tables and figures provides quick access to important information, such as acute and critical care pro-
cedures in the delivery room, the office, the emergency room, and the critical care unit; anti-infective agents; drug dosages;
immunization schedules; differential diagnosis; and developmental disorders.

NEW TO THIS EDITION


The 24th edition of CDTP has been revised comprehensively by the editors and contributing authors. All chapters have been
revised to reduce excessive text and condense text into tables. New references as well as up-to-date and useful websites have
been added, permitting the reader to consult original material and to go beyond the confines of the textbook. As editors and
practicing pediatricians, we have tried to ensure that each chapter reflects the needs and realities of day-to-day practice.

CHAPTERS WITH MAJOR REVISIONS INCLUDE:


4 Adolescence
7 Child & Adolescent Psychiatric Disorders & Psychosocial Aspects of Pediatrics
9 Ambulatory & Office Pediatrics
10 Immunization
16 Eye
17 Oral Medicine & Dentistry
19 Respiratory Tract & Mediastinum
21 Gastrointestinal Tract
22 Liver & Pancreas
23 Fluid, Electrolyte, & Acid-Base Disorders & Therapy
25 Neurologic & Muscular Disorders
31 Neoplastic Disease
33 Immunodeficiency

xxix

Hay_FM_pi-xxxii.indd 29 30/03/18 11:40 AM


xxx Preface

36 Inborn Errors of Metabolism


37 Genetics & Dysmorphology
38 Allergic Disorders
41 Human Immunodeficiency Virus Infection
42 Infections: Bacterial & Spirochetal
43 Infections: Parasitic & Mycotic

CHAPTER REVISIONS
The 19 chapters that have been extensively revised, with new authors added in several cases, reflect the substantially updated
material in each of their areas of pediatric medicine. Especially important are updates to the chapters on immunizations, endo-
crinology, neurologic and muscular disorders, and adolescence. The chapter on HIV includes current guidelines for prevention
and treatment of HIV and updates information on the new antiretroviral therapies that have become available. The chapter on
immunizations contains the most recently published recommendations, discusses the contraindications and precautions rel-
evant to special populations, and includes the new vaccines licensed since the last edition of this book. Chapters on adolescence,
skin, immunodeficiency, inborn errors of metabolism, and neoplastic disease are markedly updated with the latest information.
All laboratory tables in Chapter 46 Pediatric Laboratory Medicine and Reference Ranges that includes reference ranges and
reference intervals have been updated. All other chapters are substantially revised and references have been updated. Twenty-
six new authors have contributed to these revisions.

ACKNOWLEDGMENTS
The editors would like to thank Bonnie Savone for her expert assistance in managing the flow of manuscripts and materials
among the chapter authors, editors, and publishers. Her attention to detail was enormously helpful. The editors also would like
to thank Tia Brayman at Children’s Hospital Colorado who produced the cover photographs.

William W. Hay, Jr., MD


Myron J. Levin, MD
Robin R. Deterding, MD
Mark J. Abzug, MD
Aurora, Colorado

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Selected Highlighted Topics in the
24th Edition of CDT-P
The Adolescence chapter includes information about patient-centered care strategies and use of motivational interviewing.
The Child Abuse & Neglect chapter includes the use of nucleic acid amplification tests to diagnose sexually transmitted dis-
eases in pre-pubertal children evaluated for sexual abuse.
The Immunization chapter includes new discussions about how HPV vaccination can prevent cancer and how a newer HPV
vaccine protects against additional HPV types. There also is new discussion about how most parents in the United States
choose to vaccinate their children (in 2013, < 1% of young children received no vaccines) and the problems associated with
parents’ concerns about vaccines and the increasing number of parents who are choosing to delay or decline vaccination for
their children.
The Poisoning chapter includes new recommendations for evaluation and treatment for common ingestions and exposures in
the pediatric population, including pharmaceuticals, household products, and designer drugs.
The Skin chapter includes the use of propranolol for infantile hemangiomas.
The Eye chapter describes how instrument-based vision screening is a novel means of detecting vision-threatening amblyo-
genic risk factors in preverbal, preliterate, and developmentally delayed children, and is endorsed by the American Academy
of Pediatrics.
The Oral Medicine & Dentistry chapter includes preventive dental treatment (fluoride varnish) by physicians for
high-risk populations.
The Respiratory Tract & Mediastinum chapter introduces the new cystic fibrosis drug, Orkambi.
The Gastrointestinal Tract chapter identifies the life-threatening impact of button batteries as startling and significant health
care problem.
The Liver & Pancreas chapter identifies two new oral therapies that are available for children with chronic hepatitis B: entecavir
for children 3 years or older and tenofovir for children 12 years or older.
The Hematologic Disorders chapter notes that hydroxyurea therapy is now recommended for all children with sickle cell
anemia or sickle β-thalassemia.
The Immunodeficiency chapter describes the use of genetic testing in the evaluation of children with immunodeficiency and
states of immune dysregulation.
The Endocrine Disorders chapter includes the use of recombinant alkaline phosphatase (asfotase alfa) for the treatment of
hypophosphatasia and how it has revolutionized therapy for this disorder.
The Inborn Errors of Metabolism chapter includes updated information about diagnostic testing for a variety of disorders.
The Genetics & Dysmorphology chapter includes information on microarray testing and whole exome sequencing.
The Viral and Rickettsial chapter includes the latest information about Zika virus and dengue.
The Antimicrobial Therapy chapter includes information on antimicrobial stewardship.
And many more!

xxxi

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1

1
Advancing the Quality &
Safety of Care
Daniel Hyman, MD

INTRODUCTION and, indeed throughout the world, have accelerated their indi-
vidual and collective involvement in analyzing and improving
Hippocrates’ famous dictum primum non nocere 2500 years care. In the United States, numerous governmental agencies,
ago may have been the earliest reflection of the importance large employer groups, health insurance plans, consumers/
of patient safety, but the Institute of Medicine’s (IOM) 1999 patients, health care providers, and delivery systems are
landmark report To Err Is Human truly galvanized the cur- among the key constituencies calling for and working toward
rent focus on eliminating preventable harm from health better and safer care at lower cost. Similar efforts are occur-
care. Its most quoted statistic, that between 44,000 and ring internationally. Indeed, the concept of the Triple Aim
98,000 Americans die every year because of medical error, has been widely accepted as an organizing framework for con-
was based upon studies of hospital mortality in Colorado, sidering the country’s overall health care improvement goals.
Utah, and New York and extrapolated to an annual estimate
for the country. The IOM followed up this report with a Committee on Quality Health Care in America, Institute of Medi-
second publication, Crossing the Quality Chasm, in which cine: Crossing the Quality Chasm: a New Health System for the
they said, “Health care today harms too frequently, and 21st Century. Washington, DC: National Academy Press, 2001.
routinely fails to deliver its potential benefits…. Between the Kohn L, Corrigan JM: To Err Is Human: Building a Safer Health
health care we have and the care we could have lies not just a System. Washington, DC: National Academy Press, 2000.
gap, but a chasm.” These two reports have served as central
elements in an advocacy movement that has engaged stake-
holders across the continuum of our health care delivery
CURRENT CONTEXT
system and changed the nature of how we think about the The health care industry is in a period of transformation
quality and safety of the care we provide, and receive. being driven by at least four converging factors: (1) the rec-
In Crossing the Quality Chasm, the IOM included a ognition of serious gaps in the safety and quality of care we
simple but elegant definition of the word “Quality” as it provide (and receive), (2) the unsustainable increases in the
applies to health care. They defined six domains of health cost of care as a percent of the national economy, (3) the
care quality: (1) SAFE—free from preventable harm, (2) aging of the population, and (4) the emerging role of health
EFFECTIVE—optimal clinical outcomes; doing what we care information technology as a potential tool to improve
should do, not what we should not do according to the care. These are impacting health care organizations as well as
evidence, (3) EFFICIENT—without waste of resources— individual practitioners in numerous ways that can also be
human, financial, supplies/equipment, (4) TIMELY—without traced to expectations regarding transparency and increas-
unnecessary delay, (5) PATIENT/FAMILY CENTERED— ing accountability for results. As depicted in Figure 1–1,
according to the wishes and values of patients and their the Quadruple Aim (advancing the original concept of the
families, and (6) EQUITABLE—eliminating disparities in Triple Aim) includes the simultaneous goals of achieving
outcomes between patients of different race, gender, and better care (outcomes/experience) for individual patients and
socioeconomic status. families, better health status for the population, lower overall
In the years since these two reports were published, the cost of care, and enhancing the experience of the health care
multiple stakeholders have been concerned about the effec- workforce. Practitioners and trainees (and health care workers
tiveness, safety, and cost of health care in the United States in general) must adapt to a new set of priorities that focus

Hay_CH01_p0001-0009.indd 1 16/03/18 5:57 PM


2 CHAPTER 1

*IHI Quadruple Aim population. The agency has also enabled and advocated
for greater transparency of results and makes available
on its website comparative measures of performance for
its Medicare population. CMS is also increasingly utiliz-
ing its standards under which hospitals and other health
Improved care provider organizations are licensed to provide care
*Better clinician as tools to ensure greater compliance with these regula-
outcomes experience tions. It has instituted a Hospital-Acquired Condition
Reduction program (https://www.cms.gov/medicare/
medicare-fee-for-service-payment/acuteinpatientpps/
HAC-reduction-program.html) which targets a range
of conditions, with payment reductions from Medicare
*Improved based upon rates of occurrence. It is worth noting that
*Lower
costs
patient CMS is able to generate comparative national data only
experience for its Medicare population because it, unlike Medicaid,
is a single federal program with a single financial data-
base. Because the Medicaid program functions as 51
state/federal partnership arrangements, patient expe-

▲▲Figure 1–1.
rience and costs are captured in 51 separate state-based
Quadruple aim. program databases. This segmentation has limited the
development of national measures for pediatric care
attention on new goals to extend our historic focus on the in both inpatient and ambulatory settings. Similarly,
doctor/patient relationship and autonophysician decision while the reporting of HACs is uniform across the
making. Instead, our system’s new imperatives are evidence- United States for Medicare patients, in the Medicaid
based medicine, advancing safety, and reducing unnecessary population it varies by individual state and payment
expense. There is recognition that we need to care for our implications are both limited and state based.
workforce and understand their challenges if we are to achieve 2. National Quality Forum—www.qualityforum.org/Home
our patient oriented goals. Staff must be safe, both physically National Quality Forum (NQF) is a private, not-for-
and psychologically, and their resiliency and stress is increas- profit organization whose members include consumer
ingly a focus of health care systems around the country. advocacy groups, health care providers, accrediting
The impact of health care quality improvement will bodies, employers and other purchasers of care, and
increasingly influence clinical practice and the delivery of research organizations. Its mission is to promote
pediatric care in the future. This chapter provides a sum- improvement in the quality of American health care
mary of some of the central elements of health care quality primarily through defining priorities for improvement,
improvement and patient safety, and offers resources for the approving consensus standards and metrics for perfor-
reader to obtain additional information and understanding mance reporting, and through educational efforts. The
about these topics. NQF, for example, has endorsed a list of 29 “serious
To understand the external influences driving many of reportable events” in health care that include events
these changes, there are at least six key national organiza- related to surgical or invasive procedures, products or
tions central to the transitions occurring: device failures, patient protection, care management,
1. Center for Medicare and Medicaid Services (Depart- environmental issues, radiologic events, and potential
ment of Health and Human Services)—www.cms.gov criminal events. This list and the CMS list of HACs
Center for Medicare and Medicaid Services (CMS) are both being used by insurers to reduce payment to
oversees federally funded health care programs of the hospitals/providers as well as to require reporting to
United States, including Medicare, Medicaid, and other state agencies for public review. In 2012, NQF released
related programs. CMS and the Veterans Affairs a set of 44 measures for the quality of pediatric care,
Divisions together now provide funding for more than largely representing outpatient preventive services and
1 trillion of the total $3.2 trillion the United States management of chronic conditions, and population-
spends annually on health care expense. CMS is increas- based measures applicable to health plans, for example,
ingly promoting payment mechanisms that withhold immunization rates and frequency of well-child care.
payment for the costs of preventable complications of 3. Leapfrog—www.leapfroggroup.org
care and giving incentives to providers for achieving bet- Leapfrog is a group of large employers who seek to
ter outcomes for their patients, primarily in its Medicare use their purchasing power to influence the health

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ADVANCING THE QUALITY & SAFETY OF CARE 3

care community to achieve big “leaps” in health care organizations, including hospitals, nursing homes, and
safety and quality. It promotes transparency and issues other health care provider entities in the United States
public reports of how well individual hospitals meet as well as internationally. Its mission is to continuously
their recommended standards, including computerized improve the quality of care through evaluation, educa-
physician-order entry, ICU staffing models, and rates tion, and enforcement of regulatory standards. Since
of hospital-acquired infections. There is some evi- 2003, JC has annually adopted a set of National Patient
dence that meeting these standards is associated with Safety Goals designed to help advance the safety of care
improved hospital quality and/or mortality outcomes. provided in all health care settings. Examples include
4. Agency for Healthcare Research and Quality—www. the use of two patient identifiers to reduce the risk of
ahrq.gov care being provided to an unintended patient; the use
Agency for Healthcare Research and Quality (AHRQ) of time-outs and a universal protocol to improve surgi-
is currently one of 11 agencies within the US Department cal safety and reduce the risk of wrong site procedures;
of Health and Human Services, although this is one adherence to hand hygiene recommendations to reduce
of many things subject to change with the Trump the risk of spreading hospital-acquired infections, to
administration’s 2018 budget proposal. AHRQ’s primary name just a few. These goals often become regulatory
mission has been to support health services research standards with time and widespread adoption. Failure
initiatives that seek to improve the quality of health care to meet these standards can result in actions against
in the United States. Its activities extend well beyond the licensure of the health care provider, or more com-
the support of research and now include the develop- monly, requires corrective action plans, measurement
ment of measurements of quality and patient safety, to demonstrate improvement, and resurveying depend-
reports on disparities in performance, measures of ing upon the severity of findings. The JC publishes
patient safety culture in organizations, and promotion a monthly journal on quality and safety, available at
of tools to improve care among others. AHRQ also con- http://store.jcrinc.com/the-joint-commission-journal-
venes expert panels to assess national efforts to advance on-quality-and-patient-safety/.
quality and patient safety and to recommend strategies
Finally, ongoing governmental impacts on quality and
to accelerate progress.
safety will be subject to modifications in the US government’s
5. Specialty Society Boards anticipated repeal, replacement or changes in the Patient
Specialty Society Boards, for example, American Board Protection and Affordable Care Act (PPACA) enacted by
of Pediatrics (ABP). The ABP, along with other spe- the US government in 2010. This federal health care leg-
cialty certification organizations, has responded to the islation sought to provide near-universal access to health
call for greater accountability to consumers by enhanc- care through discounted health care exchanges that sup-
ing its maintenance of certification programs (MOC). plemented the existing, largely employer-based system.
All trainees, and an increasing proportion of active Changes in payment mechanisms for health care will con-
practitioners, are now subject to the requirements of tinue irrespective of what happens to the PPACA, and cur-
the MOC program, including participation in qual- rent and future providers’ practices will be economically,
ity improvement activities in the diplomate’s clinical structurally, and functionally impacted by these emerging
practice. The Board’s mission is focused on assuring trends. Furthermore, changes in the funding and structure
the public that certificate holders have been trained of the US health care system may ultimately also result in
according to their standards and also meet continuous changes in other countries. Many countries have single-
evaluation requirements in six areas of core compe- payer systems for providing health care to their citizens and
tency: patient care, medical knowledge, practice-based often are leaders in defining new strategies for health care
learning and improvement, interpersonal and com- improvement.
munication skills, professionalism, and systems-based
practice. These are the same competencies as required
Agency for Healthcare Research and Quality. www.ahrq.gov.
of residents in training programs as certified by the Accessed March 23, 2017.
Accreditation Council on Graduate Medical Educa- American Board of Pediatrics. https://www.abp.org. Accessed
tion. Providers need not only to be familiar with the March 23, 2017.
principles of quality improvement and patient safety, Berwick D, Nolan T, Whittington J: The triple aim: care,
but also must demonstrate having implemented qual- health, and cost. Health Aff (Millwood) 2008;27(3):759–769.
ity improvement efforts within their practice settings. doi:10.1377/hlthaff.27.3.759 [PMID: 18474969].
Center for Medicare and Medicaid Services: Triple Aim.
6. The Joint Commission—www.jointcommission.org Center for Medicare and Medicaid Services. www.cms.gov.
The Joint Commission (JC) is a private, nonprofit Accessed March 23, 2017.
agency that is licensed to accredit health care provider

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4 CHAPTER 1

CMS list of Hospital-Acquired Conditions. https://www.cms.gov/


simple questions that guide the work of the improvement
medicare/medicare-fee-for-service-payment/acuteinpatientpps/ leader and team. The model’s framework includes an Aim
HAC-reduction-program.html. Accessed March 23, 2017. statement, a measurement strategy, and then the use of
Connors E, Gostin L: Health care reform—a historic moment in “rapid cycle” changes to achieve the aim. The IHI website,
US social policy. JAMA 2010;303(24):2521–2522. doi:10.1001/ www.ihi.org, has an extensive resource library, and hosts
jama.2010.856 [PMID: 20571019]. an “Open School” that includes a QI/Patient safety modular
Hawkins RE, Weiss KB: Commentary: building the evidence base curriculum for health professional students and their faculty
in support of the American Board of Medical Specialties main-
tenance of certification program. Acad Med 2011;86(1):67. at www.ihi.org/openschool.
doi:10.1097/ACM. 0b013e318201801b [PMID: 21191200].
http://www.nejm.org/doi/full/10.1056/NEJMp0804658. Accessed
March 23, 2017. AIM STATEMENT
Jha A, Oray J, Ridgway A, Zheng J, Eptein A: Does the Leapfrog The Aim statement answers the question, “What do we
program help identify high-quality hospitals? Jt Comm J Qual
Patient Saf 2008;34(6):318–325 [PMID: 18595377].
want to accomplish?” The measure question is “How will
Leapfrog. www.leapfroggroup.org. Accessed March 23, 2017. we know that a change is an improvement?” and the change
Miller T, Leatherman S: The National Quality Forum: a “me-too” component is focused on “What changes can we make that
or a breakthrough in quality measurement and reporting? will result in improvement?” This model is represented in
Health Aff (Millwood) 1999;18(6):233–237 [PMID: 10650707]. Figure 1–2.
NQF 29 Serious Reported Events List. http://www.qualityforum. Aim statements are a written description of what the
org/Topics/SREs/List_of_SREs.aspx. Accessed March 23, 2017. team’s improvement goal is, and also include informa-
National Health Expenditure Data https://www.cms.gov/
Research-Statistics-Data-and-Systems/Statistics-Trends-and-
tion on who comprises the patient population and a time
Reports/NationalHealthExpendData/index.html. Accessed frame within which the improvement will be achieved.
11/11/2017. They identify a “stretch” but achievable improvement target
Reference on 44 NQF Pediatric measures. http://www. goal, and, often, some general statement regarding how the
qualityforum.org/Publications/2012/01/Child_Health_Quality_ improvement will be achieved. Aim statements are some-
Measures_2010_Final_Report.aspx. Accessed March 23, 2017. times characterized using the mnemonic SMAART: Specific,
Rosenthal M: Beyond pay for performance—emerging models of Measurable, Achievable, Actionable, Relevant, and Timely.
provider-payment reform. New Engl J Med 2008;359:1197–1200
[PMID: 18799554]. Aim statements should be unambiguous and understand-
Shekelle P et al: Advancing the science of patient safety. Ann able to the stakeholders, and are most likely to be achieved
Intern Med 2011;154(10):693–696 [PMID: 21576538]. if they are aligned with the strategic goals of the team or
Straube B, Blum JD: The policy on paying for treating hospital- organization.
acquired conditions: CMS officials respond. Health Aff (Millwood) For example, the following statement meets the criteria
2009;28(5):1494–1497 [PMID: 19738268]. for a SMAART aim statement, “We will reduce the fre-
The Joint Commission. www.jointcommission.org. Accessed quency of emergency department visits and hospitalizations
March 23, 2017.

STRATEGIES & MODELS FOR QUALITY Model for Improvement


IMPROVEMENT (QI) What are we trying to accomplish?
While there are many approaches to improving the qual-
How will we know that a change is an improvement?
ity of care in health care settings, the following represent
three common tools for conducting clinical improvement What changes can we make that will result in an
work. The Model for Improvement (MFI) is primarily improvement?
emphasized because of its ease of adoption, and because it
is the foundation for most improvement efforts included
in the Maintenance of Certification program of the ABP.
Act Plan
Briefer summaries of Lean and Six-Sigma methods are also
included, with listings of resources where the reader can find
additional information. Study Do

▲▲Figure 1–2. Model for improvement. (Adapted


“MODEL FOR IMPROVEMENT”
Widely taught and promoted by the Boston-based educa- with permission from Langley G, Moen R, Nolan K, et al:
tional and advocacy organization the Institute for Health- The Improvement Guide: AIM Model for Improvement.
care Improvement (IHI), the MFI is grounded in three San Francisco: Jossey-Bass; 2009.)

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ADVANCING THE QUALITY & SAFETY OF CARE 5

for patients with asthma seen at E Street Pediatrics by 25% the treatment plan into action as a part of the process of
by December 31, 2017,” whereas this next statement does care, and that the health status or outcome measure will be
not, “We will improve the care for patients with asthma by improved by fully improving the measured processes of care,
appropriately prescribing indicated medications and better including patient adherence to the treatment plan.
educating families in their use.” Measures are essential elements of any improvement
The first example provides a specific measurable goal, a work. It is a good idea to choose a manageable (4–6) number
time frame, and clarity with respect to who the patients are. of measures, all of which can be obtained with limited or no
A 25% reduction in ED/inpatient asthma visits will require a extra effort, and with a mix of outcome, process, and balanc-
change in the system for asthma care delivery for the entire ing measures. Ideally, the best process measures are those
population of children with asthma; that extent of level of that are directly linked to the outcome goal. The hypoth-
improvement is a stretch, but it is much more achievable esis in this specific example would be that assessing asthma
than a goal would be if it was set to “eliminate” such encoun- severity and appropriately using controller medications and
ters. The second example is unclear in terms of the measure action plans would all contribute to reducing the number
for improvement, the time frame for the goal to be met, and or frequency of missed school days and the need for ED/
even the population in question. The statement provides hospital utilization.
some sense of processes that could be utilized to improve It is important to note that measurement in the setting
asthma care but is missing needed specificity. of an improvement project is different from measurement in
a research study. Improvement projects require “just enough”
MEASURES data to guide the team’s continuing efforts. Often, the result
seen in a sequence of 10 patients is enough to tell you whether a
Specific measures provide a means to assess whether or not particular system is functioning consistently or not. For exam-
the improvement effort is on track. Three types of measures ple, considering the first process measure in Table 1–1, if in the
are useful. Outcome measures answer questions concerning last 10 patients seen with asthma, only two had their asthma
the health care impact for the patients, such as how has their severity documented, how many more charts need be checked
health status changed? Process measures are related to the to conclude that the system is not functioning as intended and
health care delivery system itself. They answer questions that changes are needed? Other measures may require larger
about how the system is performing. Balancing measures sample sizes, especially when assessing the impact of care
seek to identify potential unintended consequences that are changes on a population of patients with a particular condi-
related to the improvement effort being undertaken. Exam- tion. See Randolph’s excellent summary for a fuller description
ples are helpful to contextualize these conceptual definitions. of measurement for improvement.
Table 1–1 reflects some examples of types of measures
that might be employed in an asthma QI initiative.
One might argue that adherence to a treatment plan
CHANGES & IDEAS
(third process measure) is an outcome of the work of Once the team’s aim is established and the measures are
the practice/practitioner prescribing the medication. It is selected, the third component of the MFI draws from indus-
more consistent, however, to consider the translation of trial engineering and focuses on what changes in the system

Table 1–1. Examples of types of measures used in an asthma QI initiative.

Outcome Measures Process Measures Balancing Measures

Percent of children with asthma in the Proportion of children with an asthma Average difference in the time between
practice seen in the ED or hospitalized severity assessment in their medical record the last office patient’s scheduled
for asthma in the past 6 months in the past year appointment time and the actual office
close time
Percent of children with persistent asthma Percent of children with persistent asthma, of any Staff satisfaction with their job
in the practice with fewer than five missed severity, prescribed a controller medication at their
school days due to asthma in the past year most recent visit
Percent of children prescribed a controller
medication who report taking their medicine
Percent of children in a practice asthma registry
provided with a complete asthma action plan in the
past 12 months

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6 CHAPTER 1

must be made that will result in the targeted improve- “LEAN”


ments. To answer the question “What changes will result in
improvement?” the improvement team should incorporate Also grounded in industrial engineering, an increasingly
“Plan-Do-Study (or check)-Act” cycles, typically referred to popular method for driving improvement efforts in health
as PDSA cycles. The cycles include the following steps. care settings is “Lean” or “Lean processing.” Early thinking
about Lean processes is credited to the Toyota Manufacturing
• Plan: What will we do that will likely improve the process
Company in Japan. The crossover to health care from manu-
measures linked to the outcome target goal? Who will do
facturing is a relatively recent phenomenon, but numerous
it? Where? When? How? How will the data be collected?
hospitals and health care delivery settings, including indi-
• Do: Implementation of the planned change(s). TIP! It is vidual clinics, have benefited from the application of these
good to make the change cycles as small as possible, for principles to their clinical operations. Lean improvement
example, trying a new process on the next five patients methods focus on reducing errors and variability in repetitive
being seen by one provider as opposed to wide scale steps that are part of any process. In health care, examples
implementation of a new chart documentation form of repeated processes would include how patients are regis-
across an entire clinic. tered and their information obtained; how medications are
• Study (or check): Once the small test of change is tried, ordered, compounded, distributed, and administered; how
its results are assessed. How many times did the process consent forms are accurately completed in a timely manner
work as planned for the five patients included in the cycle? prior to surgical procedures; and how antibiotics are reliably
• Act: Based upon the results of the study of the cycle, rec- and efficiently delivered prior to surgical procedures.
ommendations are made as to what the next steps ought Lean is a philosophy of continuous improvement. It is
to be to achieve the goal. At this point, the cycle then grounded in recognizing that the way we do things today is
resumes and planning begins for the next cycle. merely “current state.” With time, effort, focus, and long-
term thinking, we can create a “future state” that is better
Over the course of an improvement effort, multiple tests than the status quo. It does so by focusing on identifying the
of change might be implemented for any or all of the process value of all steps in any process and eliminating those steps
measures felt to be likely to impact the outcome measures that do not contribute to the value sought by the customer,
relevant to the project. or in health care, the patient/family. In doing so, improve-
The MFI has been used by improvement teams across ments in outcomes, including cost and productivity, and in
numerous health care settings around the world. Further clinical measures of effectiveness can be realized. See Young
information about the model and examples can be found for an early critical assessment of the incorporation of Lean
at www.ihi.org/openschool, or in The Improvement Guide into health care settings.
(Langley et al). There are four categories that describe the essential
The IHI Open School modules are an excellent online elements of Toyota’s adoption of “Lean” as a manage-
resource for clinicians interested in learning more about the ment strategy. These four categories are: (1) philosophy
fundamentals of quality improvement and patient safety. (emphasize long-term thinking over short-term gain); (2)
These educational lessons are free of charge to health profes- process (eliminate waste through very defined approaches
sional students, residents, and university faculty members, including an emphasis on process flow and the use of
and for a modest subscription fee to other clinicians. They pull systems to reduce overproduction, for example); (3)
are also free to health care practitioners in the developing people/partners (respect, challenge, and grow staff); and
world. An excellent original resource on implementing this (4) problem solving (create a culture of continuous learning
model in clinical practice is in Berwick’s summary article and improvement).
from 1998. There are a number of hospitals that have fully integrated
Lean management as a primary basis for its organizational
Berwick D: The science of improvement. JAMA 2008:299(10):
approach to improvement. Several were featured in a “White
11821184 [PMID: 18334694]. Paper” published by the IHI in 2005.
Berwick DM. Developing and testing changes in delivery of care.
Ann Intern Med 1998;128:651–656 [PMID: 9537939]. Going Lean in Health Care, Innovation Series White Paper. Insti-
Langley G, Moen R, Nolan K, Nolan T, Norman C, Provost L: The tute for Healthcare Improvement. 2005. http://www.ihi.org/
Improvement Guide: AIM Model for Improvement. San Francisco, knowledge/Pages/IHIWhitePapers/GoingLeaninHealthCare.
CA: Jossey-Bass; 2009:24. aspx. Accessed March 25, 2017.
Randolph G, Esporas M, Provost L, Massie S, Bundy D: Model Liker: The Toyota Way. Madison, WI: McGraw-Hill; 2004.
for Improvement—Part Two: Measurement and feedback Young TP, McClean SI: A critical look at Lean Thinking in
for quality improvement efforts. Pediatr Clin North Am healthcare. BMJ Qual Saf Health Care 2008;17:382–386 [PMID:
2009;56(4):779–798 [PMID: 19660627]. 18842980].

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ADVANCING THE QUALITY & SAFETY OF CARE 7

“SIX SIGMA” way things are done now, and then implementing planned
changes to see how they impact the outputs or outcomes.
A third quality-improvement methodology also arose in the For additional information on Lean and Six Sigma, see
manufacturing industry. Motorola is generally credited with www.isixsigma.com or www.asq.org/sixsigma.
promoting Six Sigma as a management strategy designed
to reduce the variability in its processes and thereby reduc-
ing the number of defects in its outputs. Organizations Pande P, Holpp L: What Is Six Sigma? New York, NY: McGraw-
adopting Six Sigma as an improvement strategy utilize mea- Hill; 2002.
surement-based strategies that focus on process improve-
ment and variation reduction to eliminate defects in their
work and to reduce cycle times, thereby increasing profit-
PRINCIPLES OF PATIENT SAFETY (INCIDENT
ability and enhancing customer satisfaction. Sigma is the sta-
REPORTING, JUST CULTURE, DISCLOSURE,
tistical measure of standard deviation and Motorola adopted
FMEA, RCA, RELIABILITY, CHECKLISTS)
Six Sigma as a performance indicator, promoting consistency Safe patient care avoids preventable harm; it is care that
of processes in order to have fewer than 3.4 defects per million does not cause harm as it seeks to cure. The list of adverse
opportunities. This performance goal has since become the events that are considered to be preventable is evolving. As
common descriptor for this approach to improvement both mentioned earlier, both CMS and NQF have endorsed lists
in manufacturing and in service industries, including health of various complications of care as being “never events” or
care. Similar to Lean, the translation of business manufac- “serious reportable events” for which providers are often
turing strategies into health care has various challenges, but now not reimbursed, and which are increasingly reportable
there are many processes that repeatedly occur in health to the public through various state transparency programs.
care that can be routinized and made more consistent. Many A national network of Children’s Hospitals has been
health care processes fail far more frequently than 3.4 times active since 2012, collaborating on a shared aim of eliminat-
per million opportunities. Consider pharmacy dispensing ing serious harm in their now more than 110 organizations.
errors, medication ordering or administration errors, and Initially funded as a Hospital Engagement Network by the
patient-scheduling errors, just to name a few. These are a Center for Medicare and Medicaid Innovation program,
few of many examples of processes that could potentially Children’s Hospitals Solutions for Patient Safety merges
benefit from the kind of rigorous analysis that is integral to approaches to achieving reliability in evidence-based pre-
the Six-Sigma approach. vention practices (“bundles”) with safety practices at both
In a typical Six-Sigma structured improvement project, staff and leadership levels to reducing serious safety events
there are five phases generally referred to as DMAIC: (1) (SSEs) and HACs in general. Numerous hospitals have
Define (what is the problem, what is the goal?), (2) Measure demonstrated dramatic reductions in the occurrence rates
(quantify the problem and improvement opportunity), (3) of both HACs and SSEs with the following interventions:
Analyze (use of observations and data to identify causes), (4) (1) implementation of structured processes to improve
Improve (implementation of solutions based on data analy- consistency of prevention processes, (2) education and rein-
sis), and finally, (5) Control (sustainable change). forcement of principles of patient safety culture designed to
One of the central aspects of Six Sigma as an improve- improve communication and reduce error, and (3) effective,
ment strategy is its defined focus on understanding the structured cause analyses after SSEs.
reasons for defects in any process. By understanding these Irrespective of one’s views about whether various compli-
drivers, it is then possible to revise the approach to either cations are entirely preventable at the current state of science
the manufacturing process or the service functions in order or not, these approaches reflect a changing paradigm that is
to reduce these errors and failures. impacting many aspects of health care delivery. Transpar-
“Lean-Six Sigma” is a newer entity that draws from both ency of results is increasingly expected. Perspectives and
methodologies in order to simplify the improvement work data on how these kinds of efforts are impacting actual
where possible, but retain the rigorous statistical method improvement in outcomes are mixed.
that is a hallmark of Six-Sigma projects. Lean focuses on Given these trends, health care providers need to have
where time is lost in any process and can identify opportu- robust systems for measuring and improving the safety of
nities to eliminate steps or reduce time. Six Sigma aims to care provided to patients. The methods for improving qual-
reduce or eliminate defects in the process, thereby resulting ity reviewed earlier are frequently used to reduce harm, just
in a higher quality product through a more efficient and as they can be used to improve effectiveness or efficiency.
lower cost process. For example, hospitals attempting to reduce infections
Regardless of the method used, improvement happens have successfully used these types of process improvement
because an organization, team, or individual sets a goal to approaches to improve antibiotic use prior to surgical pro-
improve a current process through systematic analysis of the cedures or to improve hand hygiene practices.

Hay_CH01_p0001-0009.indd 7 16/03/18 5:57 PM


8 CHAPTER 1

Common patient safety tools are summarized here. Failure modes and effects analyses (FMEA): An FMEA is a
Incident-reporting systems: Efforts to advance safety in any systematic methodology used to proactively identify ways
organization require a clear understanding of the kinds of in which any process might fail, and then to prioritize
harm occurring within that organization, as well as the among strategies for reducing the risk or impact of identi-
kinds of “near-misses” that are occurring. These reporting fied potential failures. In conducting an FMEA, which all
systems can range from a simple paper reporting form to hospitals are required to do annually, a team will carefully
a “telephone hot-line” to a computerized database that describe and analyze each step in a particular process,
is available to staff (and potentially patients) within the consider what and how anything might go wrong, why
organization. Events are traditionally graded according to it would happen and what the impact would be of such
the severity of harm that resulted from the incident. One failures. Like Lean and Six Sigma, the FMEA has been
example is the NCC MERP Index, which grades events adopted into health care from its origins in military and
from A (potential to cause harm) to I (resulting in patient industrial settings. The FMEA is an effective method for
death). Errors that are recognized represent only a frac- identifying strategies to reduce risks in health care set-
tion of the actual errors and near-misses that are present tings, thereby protecting patients if interventions are put
in the system. Incident-reporting systems depend upon into place as a result of the analysis. A tool to use in con-
people recognizing the error or near-miss, being comfort- ducting an FMEA is available from the IHI (http://www.
able reporting it, knowing how and when to report, and ihi.org/knowledge/Pages/Tools/FailureModesandEffects-
then actually doing so. It is no surprise therefore that esti- AnalysisTool.aspx). Their site includes additional infor-
mates for how frequently incidents that could or should mation and resources about the FMEA process.
be reported into incident-reporting systems range from Root-cause analyses (RCA) (postevent reviews): As con-
1.5% to 30% depending on the type of adverse or near- trasted with the proactive FMEA process, an RCA is
miss event. “Trigger tools” (either manual chart reviews a retrospective analysis of an adverse occurrence (or
for indications of adverse events, or automated reports near-miss) that has already happened. It too is a sys-
from electronic medical records) are increasingly being tematic process that in this case allows a team to reach
used to increase the recognition of episodes of harm in an understanding of why certain things occurred, what
health care settings. systems factors and human factors contributed to the
“Just culture”: The effectiveness of incident-reporting occurrence, and what defects in the system might be
systems is highly dependent upon the culture of the orga- changed in order to reduce the likelihood of recurrence.
nization within which the reporting is occurring. Aviation Key to an effective RCA process, and similar to the prin-
industry safety-reporting systems are often highlighted ciples discussed above related to “just culture,” RCAs are
for their successes over the past few decades in promot- designed not to ask who was at fault, but rather what sys-
ing reporting of aviation events that might have led to tem reasons contributed to the event. “Why,” not “who,”
accidents. The Aviation Safety Reporting System (ASRS) is the essential question to be asked. The answer to the
prioritizes confidentiality in order to encourage reporting question “why did this occur” almost invariably results
and protects reporters from punishment, with certain in a combination of factors, often illustrated by a series of
limitations when they report incidents, even if related pieces of Swiss cheese where the holes all line up. Taken
to non-adherence to aviation regulations. Although the from the writings of James Reason, the “Swiss Cheese
system is voluntary, more than 880,000 reports have been Model” illustrates the many possible system failures that
submitted and used by the Federal Aviation Administra- can contribute to an error, and contributes to identifying
tion to improve air travel safety. potential system changes that reduce the risk of error
In health care, the variable recognition of adverse recurrence. Strategies for approaching retrospective RCA
events as well as any fear about reprisal for reporting and the causes of human and system error are available at
events both work to reduce the consistent reporting http://www.ncbi.nlm.nih.gov/pmc/articles/pmc1117770.
of events. The concept of “just culture” has been The use of retrospective review processes to learn from
promoted as a strategy to increase the comfort of staff adverse events is frequently now referred to as “Safety 1”
members to report the occurrence of errors or near- approach to health care improvement. Increasingly, safety
misses, even if they may have done something incorrectly. experts are focusing on “Safety 2,” which is complemen-
See the work of David Marx and the “Just Culture tary strategy oriented to learning “when things go right,”
Community” for more information on how to evaluate and designing systems for reliability using approaches
error so as to support reporting and safer practices in common in the field of human factors engineering. A few
organizations. A great deal more information about “just selected reading materials for the interested learner are
culture” principles is available at http://www.justculture.org. listed later.

Hay_CH01_p0001-0009.indd 8 16/03/18 5:57 PM


ADVANCING THE QUALITY & SAFETY OF CARE 9

Communication and team training: Because failures of


Ashley L, Armitage G, Neary M, Hoolingsworth G: A practical
communication are the most common identified factors guide to failure mode and effects analysis in health care: making
in the analysis of reported serious health care events, many the most of the team and its meetings. Jt Comm J Qual Patient
health care organizations have incorporated tools from Saf 2010;36(8):351–358 [PMID: 20860241].
other industries, particularly aviation, in order to enhance Children’s Hospitals Solutions for Patient Safety. http://www.
patient safety. As a result of the knowledge gained through solutionsforpatientsafety.org. Accessed March 25, 2017.
analysis of tragic aviation accidents, the airline industry Hollnagel E. A Tale of Two Safeties. http://www.resilienthealth-
care.net/A_tale_of_two_safeties.pdf. Accessed March 25, 2017.
implemented methods like crew resource management
http://www.ihi.org/resources/Pages/Tools/FailureModesand
training to ensure that communication among cockpit EffectsAnalysisTool.aspx. Accessed November, 11, 2017.
team members is effective and clear, thereby reducing http://www.macrmi.info/files/5213/5482/2320/Marx_just_culture_
risk of air accidents. Similar methods have been used to copy.pdf. Accessed November 11, 2017
train teams in operating rooms, delivery rooms, and other Incident Reporting Systems. http://www.nccmerp.org/. Accessed
team-based settings. Most of these curricula include a March 25, 2017.
few common elements: introductions to be sure all team Marx D: A Primer for Health Care Executives: Patient Safety
members know one another’s name, promoting the likeli- and the “Just Culture.” New York, NY: Columbia University;
2011.
hood of speaking up; leader clarity with team members Mills R. Collaborating With Industry to Ensure Regulatory Over-
about the expectation that all will speak up if anyone has sight: The Use of Voluntary Safety Reporting Programs by the
a concern; and structured language and other tools like Federal Aviation Administration. Kent State University, College
verbal read-back of critical information to ensure clarity in of Arts and Sciences; 2011. [dissertation]. https://etd.ohiolink.
interpersonal or interdisciplinary communications. Such edu/pg_10?10229231243643::NO:10:P10_ETD_SUBID:54852.
training also seeks to flatten the hierarchy, making it more Accessed March 25, 2017.
Reason J: Human error: models and management. BMJ
likely that potential risks or problems will be identified
2000;320:768. doi: 10.1136/bmj.320.7237.768:2000 [PMID:
and effectively addressed. Common tools used in pro- 10720363].
moting effective team communication include structured Reference on 29 Serious Reportable Events NQF. http://www.
language like SBAR (Situation, Background, Assessment, qualityforum.org/Topics/SREs/List_of_SREs.aspx. Accessed
and Recommendation), taken from the navy, to promote March 25, 2017.
clarity of communication. Shekelle P et al. Advancing the science of patient safety. Ann
A number of resources exist in the public domain Intern Med 2011;154(10):693–696 [PMID: 21576538].
Stockwell DC et al: Development of an electronic pediatric
to support better teamwork and communication. One
all-cause harm measurement tool using a modified Delphi
good place to start is the TeamSTEPPS program from the method. J Patient Saf 2014 Aug 26. http://www.ncbi.nlm.nih.
Agency for Healthcare Research and Quality. It can be gov/pubmed/25162206. Accessed March 25, 2017.
found at http://teamstepps.ahrq.gov/.

Hay_CH01_p0001-0009.indd 9 16/03/18 5:57 PM


10

2
The Newborn Infant
Danielle Smith, MD
Theresa Grover, MD

INTRODUCTION such as ultrasound, amniocentesis, screening tests (rubella


antibody, hepatitis B surface antigen, serum quadruple
The newborn period is defined as the first 28 days of life. In screen for genetic disorders, HIV [human immunodefi-
practice, however, sick or very immature infants may require ciency virus]), and antepartum tests of fetal well-being (eg,
neonatal care for many months. There are three levels of biophysical profiles, nonstress tests, or Doppler assessment
newborn care. Level 1 refers to basic care of well newborns, of fetal blood flow patterns). Pregnancy-related maternal
neonatal resuscitation, and stabilization prior to transport. complications such as urinary tract infection, pregnancy-
Level 2 refers to specialty neonatal care of premature infants induced hypertension, eclampsia, gestational diabetes, vagi-
greater than 1500 g or more than 32 weeks’ gestation. Level nal bleeding, and preterm labor should be documented.
3 is subspecialty care of higher complexity ranging from 3A Significant peripartum events include duration of rup-
to 3C based on newborn size and gestational age. Level 4 tured membranes, maternal fever, fetal distress, meconium-
includes availability of general surgery, cardiac surgery, and stained amniotic fluid, type of delivery (vaginal or cesarean
extracorporeal membrane oxygenation (ECMO). Level 4 section), anesthesia and analgesia used, reason for operative
care is often part of a perinatal center offering critical care or forceps delivery, infant status at birth, resuscitative mea-
and transport to the high-risk mother and fetus as well as sures, and Apgar scores.
the newborn infant.

ººASSESSMENT OF GROWTH &


ººTHE NEONATAL HISTORY
GESTATIONAL AGE
The newborn medical history has three key components:
It is important to know the infant’s gestational age because
1. Maternal and paternal medical and genetic history normal behavior and possible medical problems can be
2. Maternal past obstetric history predicted on this basis. The date of the last menstrual period
3. Current antepartum and intrapartum obstetric history is the best indicator of gestational age with early fetal ultra-
sound providing supporting information. Postnatal physical
The mother’s medical history includes chronic medical characteristics and neurologic development are also clues to
conditions, medications taken during pregnancy, unusual gestational age. Table 2–1 lists the physical and neurologic
dietary habits, smoking history, substance abuse history, criteria of maturity used to estimate gestational age by the
occupational exposure to chemicals or infections of potential Ballard method. Adding the scores assigned to each neonatal
risk to the fetus, and any social history that might increase physical and neuromuscular sign yields a score correspond-
the risk for parenting problems and child abuse. Family ill- ing to gestational age.
nesses and a history of congenital anomalies with genetic If the physical examination indicates a gestational age
implications should be sought. The past obstetric history within 2 weeks of that predicted by the obstetric dates, the
includes maternal age, gravidity, parity, blood type, and gestational age is as assigned by the obstetric date. Birth
pregnancy outcomes. The current obstetric history includes weight and gestational age are plotted on standard grids
the results of procedures during the current pregnancy to determine whether the birth weight is appropriate for

Hay_CH02_p0010-0066.indd 10 30/03/18 4:05 PM


THE NEWBORN INFANT 11

Table 2–1. New Ballard score for assessment of fetal maturation of newly born infants.a
Neuromuscular Maturity
Score
Neuromuscular Record Score
Maturity Sign –1 0 1 2 3 4 5 Here
Posture

Square window
(wrist)

Arm recoil

Popliteal angle

Scarf sign

Heel to ear

Total Neuromuscular Maturity Score


Physical Maturity
Score
Physical Maturity Record Score
Sign –1 0 1 2 3 4 5 Here
Skin Sticky, friable, Gelatinous, Smooth, pink, Superficial Cracking, pale Parchment, Leathery,
transparent red, visible veins peeling and/or areas; rare veins deep cracking; cracked,
translucent rash; few veins no vessels wrinkled
Lanugo None Sparse Abundant Thinning Bald areas Mostly bald
Plantar surface Heel toe > 50 mm: Faint red Anterior Creases Creases over
40–50 mm: –1 no crease marks transverse anterior 2/3 entire sole
< 40 mm: –2 crease only
Breast Imperceptible Barely Flat areola; Stippled areola; Raised areola; Full areola;
perceptible no bud 1- to 2-mm bud 3- to 4-mm bud 5- to 10-mm
bud
Eye/ear Lids fused Lids open; Slightly curved Well-curved Formed and Thick
loosely: –1 pinna flat; pinna; soft; pinna; soft but firm instant cartilage;
tightly: –2 stays folded slow recoil ready recoil recoil ear stiff
Genitals (male) Scrotum flat, Scrotum Testes in Testes Testes down; Testes
smooth empty; faint upper canal; descending; good rugae pendulous;
rugae rare rugae few rugae deep rugae
Genitals Clitoris Prominent Prominent Majora and Majora large; Majora cover
(female) prominent clitoris and clitoris and minora equally minora small clitoris and
and labia flat small labia enlarging prominent minora
minora minora
Total Physical Maturity Score
Maturity Score –10 –5 0 5 10 15 20 25 30 35 40 45 50
Rating Weeks 20 22 24 26 28 30 32 34 36 38 40 42 44
a
See text for a description of the clinical gestational age examination. (Reproduced with permission from Ballard JL, Khoury JC, Wedig K, et al:
New Ballard Score, expanded to include extremely premature infants. J Pediatr 1991 Sep;119(3):417–423.)

Hay_CH02_p0010-0066.indd 11 30/03/18 4:05 PM


12 CHAPTER 2

gestational age (AGA), small for gestational age (SGA, also congenital anomalies, cardiomyopathy, hyperbilirubinemia,
known as intrauterine growth restriction [IUGR]), or large and hypocalcemia. SGA infants are at risk for fetal distress
for gestational age (LGA). Birth weight for gestational age in during labor and delivery, polycythemia, hypoglycemia, and
normal neonates varies with gender, race, maternal nutri- hypocalcemia.
tion, access to obstetric care, and environmental factors such
as altitude, smoking, and drug and alcohol use. Whenever ººEXAMINATION AT BIRTH
possible, standards for newborn weight and gestational age
based on local or regional data should be used. Birth weight The extent of the newborn physical examination depends
related to gestational age is a screening tool that should be on the condition of the infant and the setting. Examina-
supplemented by clinical data when entertaining a diagno- tion in the delivery room consists largely of observation
sis of IUGR or excessive fetal growth. These data include plus auscultation of the chest and inspection for congenital
the infant’s physical examination and other factors such as anomalies and birth trauma. Major congenital anomalies
parental size and the birth weight–gestational age of siblings. occur in 1.5% of live births and account for 20%–25% of
An important distinction, particularly in SGA infants, is perinatal and neonatal deaths. Because infants are physically
whether a growth disorder is symmetrical (weight, length, stressed during parturition, the delivery room examination
and occipitofrontal circumference [OFC] all ≤ 10%) or should not be extensive. The Apgar score (Table 2–2) should
asymmetrical (only weight ≤ 10%). Asymmetrical growth be recorded at 1 and 5 minutes of age. In severely depressed
restriction implies a problem late in pregnancy, such as infants, scores can be recorded out to 20 minutes. Although
pregnancy-induced hypertension or placental insufficiency. the 1- and 5-minute Apgar scores have almost no predictive
Symmetrical growth restriction implies an event of early value for long-term outcome, serial scores provide a useful
pregnancy: chromosomal abnormality, drug or alcohol use, description of the severity of perinatal depression and the
or congenital viral infections. In general, the outlook for response to resuscitative efforts.
normal growth and development is better in asymmetrically Skin color is an indicator of cardiac output because of
growth-restricted infants whose intrauterine brain growth the normal high blood flow to the skin. Stress that triggers a
has been spared. catecholamine response redirects cardiac output away from
The fact that SGA infants have fewer problems (such the skin to preserve oxygen delivery to more critical organs.
as respiratory distress syndrome) than AGA infants of the Cyanosis and pallor are thus two useful signs suggestive of
same birth weight, but a lower gestational age has led to the inadequate cardiac output.
misconception that SGA infants have accelerated matura- Skeletal examination at delivery serves to detect obvious
tion. SGA infants, when compared with AGA infants of the congenital anomalies and to identify birth trauma, particu-
same gestational age, actually have increased morbidity and larly in LGA infants or those born after a protracted second
mortality rates. stage of labor where a fractured clavicle or humerus might
Knowledge of birth weight in relation to gestational be found.
age allows anticipation of some neonatal problems. LGA The placenta and umbilical cord should be examined at
infants are at risk for birth trauma. LGA infants of diabetic delivery. The number of umbilical cord vessels should be
mothers are also at risk for hypoglycemia, polycythemia, determined. Normally, there are two arteries and one vein.

Table 2–2. Infant evaluation at birth—Apgar score.a


Score

0 1 2

Heart rate Absent Slow (< 100) > 100


Respiratory effort Absent Slow, irregular Good, crying
Muscle tone Limp Some flexion Active motion
Response to catheter in nostrilb No response Grimace Cough or sneeze
Color Blue or pale Body pink; extremities blue Completely pink
a
One minute and 5 minutes after complete birth of the infant (disregarding the cord and the placenta), the following objective signs should
be observed and recorded.
b
Tested after the oropharynx is clear.
Data from Apgar V, Holaday DA, James LS, et al: Evaluation of the newborn infant—second report. J Am Med Assoc 1958 Dec 13;168(15):
1985–1988.

Hay_CH02_p0010-0066.indd 12 30/03/18 4:05 PM


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