Pediatric - Pulmonology - Front 2

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Pulmonology
2ND EDITION
Copyright © 2023. American Academy of Pediatrics. All rights reserved.
Section, on Pediatric Pulmonology and Sleep Medicine, American Academy of Pediatrics. Pediatric Pulmonology, edited by
Michael J. Light, and Hook, Kristin Van, American Academy of Pediatrics, 2023. ProQuest Ebook Central, http://ebookcentral.proquest.com/lib/ucla/detail.acti
Created from ucla on 2024-01-12 17:30:05.
Pediatric
Pulmonology
2ND EDITION
Author
American Academy of Pediatrics
Section on Pediatric Pulmonology and Sleep Medicine
Editors in Chief
Michael J. Light, MD, FAAP
Kristin Van Hook, MD, MPH, FAAP
Associate Editors
Lee J. Brooks, MD, FAAP
Mary Bono Cataletto, MD, FAAP
Allen J. Dozor, MD, FAAP
Thomas G. Keens, MD, FAAP
Richard M. Kravitz, MD, FAAP

Shruti M. Paranjape, MD, FAAP
Michael S. Schechter, MD, MPH, FAAP
Girish D. Sharma, MD, FAAP, FCCP
Dennis C. Stokes, MD, MPH, FAAP
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Section, onPP 2ND ED.indd
Pediatric 1
Pulmonology and Sleep Medicine, American Academy of Pediatrics. Pediatric Pulmonology, edited by Michael J. 11/7/23 8:03 AM
Light, and Hook, Kristin Van, American Academy of Pediatrics, 2023. ProQuest Ebook Central, http://ebookcentral.proquest.com/lib/ucla/detail.action?docID=3105
American Academy of Pediatrics Publishing Staff
Mary Lou White, Chief Product and Services Officer/SVP, Membership, Marketing, and Publishing
Mark Grimes, Vice President, Publishing
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Soraya Alem, Digital Production Specialist
Mary Louise Carr, MBA, Marketing Manager, Clinical Publications
Published by the American Academy of Pediatrics
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The American Academy of Pediatrics is an organization of 67,000 primary care pediatricians, pediatric medical
subspecialists, and pediatric surgical specialists dedicated to the health, safety, and well-being of all infants, children,
adolescents, and young adults.
The recommendations in this publication do not indicate an exclusive course of treatment or serve as a standard of medical
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This publication has been developed by the American Academy of Pediatrics. The authors, editors, and contributors are
expert authorities in the field of pediatrics. No commercial involvement of any kind has been solicited or accepted in the
development of the content of this publication. Disclosures: Dr Ariel Berlinski disclosed an advisor relationship with IPAC-
RS and a financial relationship with Therapeutics Development Network, NIH, Cystic Fibrosis Foundation, and Vertex. Dr
Lee Brooks disclosed a financial relationship with Astra Zeneca. Dr Thomas Ferkol disclosed a financial relationship with
ReCode Therapeutics, Translate Bio, Arrowhead Pharmaceuticals, and NIH; and a principal investigator relationship with
Parion Sciences. Dr Christopher Harris has disclosed a financial relationship with Enata Pharmaceuticals and Vertex
Pharmaceuticals. Dr Jordana Hoppe disclosed a financial relationship with Vertex Pharmaceuticals. Dr Susanna McColley
disclosed a financial relationship with Vertex Pharmaceuticals. Dr Kenneth Olivier disclosed a financial relationship with
AN2 Therapeutics, Insmed Inc./Kaplan, Mannkind Corporation, and Spero Therapeutics. Dr Michael Schecter disclosed a
financial relationship with Astra Zeneca, Vertex Pharmaceuticals, and Sanofi. Dr Jade Tam-Williams disclosed a financial
relationship with Merck. Any relevant disclosures have been mitigated through a process approved by the AAP Board of
Directors.
Every effort has been made to ensure that the drug selection and dosages set forth in this publication are in accordance
with the current recommendations and practice at the time of publication. It is the responsibility of the health care
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Every effort is made to keep Pediatric Pulmonology consistent with the most recent advice and information available from
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eBook: 978-1-61002-653-6
Cover and publication design by LSD DESIGN LLC
Library of Congress Control Number: 2022901614
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Pediatric 2
American Academy of Pediatrics Reviewers
Board of Directors Reviewer
Madeline M. Joseph, MD, FAAP
Committees, Councils, and Sections

Committee on Hospital Care
Committee on Infectious Diseases
Committee on Native American Child Health
Committee on Pediatric and Adolescent HIV
Committee on Substance Use and Prevention
Council on Children With Disabilities
Council on Genetics
Council on Immigrant Child and Family Health
Council on Injury, Violence, and Poison Prevention
Section on Anesthesiology and Pain Medicine
Section on Endocrinology
Section on Epidemiology, Public Health, and Evidence
Section on Gastroenterology, Hepatology, and Nutrition
Section on Hematology/Oncology
Section on Hospice and Palliative Medicine
Section on Hospital Medicine
Section on Infectious Diseases
Section on Minority Health, Equity, and Inclusion

Section on Neurology
Section on Oral Health
Section on Radiology
Section on Rheumatology
III
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Pediatric 4
American Academy of Pediatrics
Section on Pediatric Pulmonology and Sleep Medicine
2022–2023
Executive Committee
Chairperson
Charlottesville, VA
Rajeev Bhatia, MD, FAAP

Scottsdale, AZ
Lauren Elizabeth (L.E.) Faricy, MD, FAAP

Burlington, VT
Theresa Guilbert, MD, FAAP

Cincinnati, OH
Brooke Gustafson, MD, FAAP

Fellowship Trainee Member
Columbus, OH
Binal Kancherla, MD, FAAP

Sugar Land, TX
Rebekah Nevel, MD, FAAP

Columbia, MO
Christopher Oermann, MD, FAAP

Kansas City, MO
Andrew Sokolow, MD, FAAP

Nashville, TN

Immediate Past Chairperson
New Orleans, LA
Staff
Laura Laskosz, MPH
Manager, Committees and Sections
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Contributors
Editors in Chief
Michael J. Light, MD, FAAP
Orlando Pediatric Pulmonary and Sleep Associates
Orlando, FL
Ochsner Hospital for Children
New Orleans, LA
Associate Editors
Lee J. Brooks, MD, FAAP
Professor of Pediatrics
Rowan University
School of Osteopathic Medicine
Stratford, NJ
Mary Bono Cataletto, MD, FAAP
Clinical Professor of Pediatrics
Division of Pediatric Pulmonology
NYU–Long Island School of Medicine
Mineola, NY
Allen J. Dozor, MD, FAAP
President, Boston Children’s Health Physicians
Associate Physician-in-Chief
Maria Fareri Children’s Hospital at WMCHealth
Professor of Pediatrics and Clinical Public Health
New York Medical College
Valhalla, NY
Thomas G. Keens, MD, FAAP
Professor Emeritus of Pediatrics, Physiology, and Neuroscience
Keck School of Medicine of USC
Children’s Hospital Los Angeles
Los Angeles, CA
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VIII
Contributors

Division Chief, Division of Pediatric Respiratory Medicine
University of Virginia School of Medicine
Charlottesville, VA
Shruti M. Paranjape, MD, FAAP
Eudowood Division of Pediatric Respiratory Sciences
Johns Hopkins University
Baltimore, MD
Professor and Chief
Division of Pulmonary Medicine
Department of Pediatrics
Virginia Commonwealth University
Children’s Hospital of Richmond at VCU
Richmond, VA
Rush University
Chief, Division of Pediatric Pulmonology
Director, Rush Cystic Fibrosis Center
Rush University Medical Center
Chicago, IL
Dennis C. Stokes, MD, MPH, FAAP
Vanderbilt University School of Medicine
Division of Allergy, Immunology, and Pulmonary Medicine
Monroe Carell Jr Children’s Hospital at Vanderbilt
Nashville, TN
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IX
Contributors
Chapter Authors
Mutasim Abu-Hasan, MD
Senior Attending Physician
Pediatric Pulmonology
Sidra Medicine
Doha, Qatar
Jennifer Accardo, MD, MSCE
Associate Professor of Pediatrics and Neurology
Virginia Commonwealth University School of Medicine
Richmond, VA
Suzanne E. Beck, MD
Professor of Clinical Pediatrics
Perelman School of Medicine at the University of Pennsylvania
Division of Pulmonary and Sleep Medicine
Children’s Hospital of Philadelphia
Philadelphia, PA
Ariel Berlinski, MD, FAAP, FAARC
Division of Pediatric Pulmonology and Sleep Medicine
University of Arkansas for Medical Sciences
Pediatric Aerosol Research Laboratory
Arkansas Children’s Research Institute
Little Rock, AR
Shyall Bhela, MD
Boston Children’s Health Physicians
Maria Fareri Children’s Hospital

Westchester Medical Center
Westchester, NY
Ellen K. Bowser, MS, RDN, LDN, RN, FAND
Associate in Pediatrics
Codirector and Nutrition Faculty
Pediatric Pulmonary Center Leadership Training Program
Pediatric Pulmonary Division
University of Florida
Gainesville, FL
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X
Contributors
John S. Bradley, MD, FAAP

Distinguished Professor
Division of Infectious Diseases
UCSD School of Medicine
Rady Children’s Hospital San Diego
San Diego, CA
Deborah M. Brooks, MD
Assistant Professor of Child and Adolescent Psychiatry
University of Maryland School of Medicine
Baltimore, MD
Pi Chun Cheng, MD, MS
Assistant Professor of Pediatrics
Division of Pediatric Pulmonology, Allergy, and Sleep Medicine
Riley Hospital for Children
Indiana University School of Medicine
Indianapolis, IN
Christopher M. Cielo, DO, MS
University of Pennsylvania
Division of Pulmonary & Sleep Medicine
Philadelphia, PA
Robyn T. Cohen, MD, MPH
Division of Pediatric Pulmonary and Allergy
Boston Medical Center
Associate Professor of Pediatrics
Chobanian and Avedisian Boston University School of Medicine

Boston, MA
Carol Conrad, MD
Director, Pediatric Lung Transplant Program
Stanford University
Palo Alto, CA
Zarmina Ehsan, MD, FAAP
University of Missouri–Kansas City
Division of Pulmonary and Sleep Medicine Children’s Mercy–Kansas City
Kansas City, MO
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Pediatric 10 and Sleep Medicine, American Academy of Pediatrics. Pediatric Pulmonology, edited by Michael J.
Pulmonology 11/7/23 8:03 AM
XI
Contributors
Laurie C. Eldredge, MD, PhD

University of Washington School of Medicine
Seattle Children’s Hospital
Seattle, WA
Harold J. Farber, MD, MSPH, FAAP
Professor of Pediatrics, Pulmonary Division
Baylor College of Medicine
Texas Children’s Hospital
Houston, TX
David Fedele, PhD, ABPP
Associate Professor
Department of Clinical and Health Psychology
Gainesville, FL
Thomas W. Ferkol, MD
Division of Pulmonology
University of North Carolina at Chapel Hill
Chapel Hill, NC
Julie L. Fierro, MD, MPH
Assistant Professor
Philadelphia, PA
Edward W. Fong, MD
Assistant Clinical Professor of Pediatrics
University of Hawaii
Kapiolani Medical Center for Women and Children
Honolulu, HI
Emily S. Gillett, MD, PhD, FAAP
Assistant Professor of Clinical Pediatrics
Los Angeles, CA
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Contributors
Samuel B. Goldfarb, MD
Codirector, Pediatric Cystic Fibrosis Program
Codirector, University of Minnesota Cystic Fibrosis Center
Division of Pediatric Pulmonary and Sleep Medicine
University of Minnesota
Minneapolis, MN
Madeleine Grigg-Damberger, MD
Professor of Neurology
University of New Mexico School of Medicine
Medical Director, Pediatric Sleep Medicine Services
Associate Director, University of New Mexico Neurodiagnostic Laboratory
Associate Director, Clinical Neurophysiology Fellowship Program
University of New Mexico
Albuquerque, NM
Pallav Halani, MD, FAAP
Fellow, Division of Pediatric Pulmonology
University of North Carolina
Chapel Hill, NC
Karen Ann Hardy, MD, FATS
Clinical Professor
Division of Pediatric Pulmonary, Asthma, and Sleep Medicine
Stanford University
Palo Alto, CA
Christopher E. Harris, MD, FAAP
Medical Director
Enanta Pharmaceuticals
Watertown, MA
Leslie Hendeles, PharmD
Professor Emeritus, College of Pharmacy
Courtesy Professor of Pediatrics (Pulmonary)
Gainesville, FL
Jordana E. Hoppe, MD, MSCS
University of Colorado School of Medicine
Aurora, CO
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Contributors
Paul Houin, MD
Senior Instructor
Department of Pediatric Pulmonology and Sleep Medicine
University of Colorado School of Medicine Anschutz Medical Campus
Pediatric Pulmonologist
Children’s Hospital Colorado
Aurora, CO
Caitlin Hurley, MD
Assistant Member
Division of Critical Care Medicine and Department of Bone Marrow
Transplantation and Cellular Therapy
Medical Director, Continuing Medical Education
St. Jude Children’s Research Hospital
Memphis, TN
Manju S. Hurvitz, MD, FAAP
Fellow, Pediatric Pulmonology
Division of Respiratory Medicine
University of California San Diego
San Diego, CA
Maki Ishizuka, MD
Philadelphia, PA
Jay Jin, MD, PhD
Assistant Professor of Clinical Pediatrics
Section of Allergy and Immunology

Riley Hospital for Children at Indiana University Health
Indianapolis, IN
Katharine Kevill, MD, MHCDS, FAAP
Stony Brook Children’s Hospital
Stony Brook, NY
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Contributors
T. Bernard Kinane, MD
Massachusetts General Hospital for Children
Harvard Medical School
Boston, MA
Sankaran Krishnan, MD, MPH
Associate Professor of Pediatrics and Public Health
Division Chief, Pediatric Pulmonology, Allergy, Immunology, and Sleep
Medicine
Maria Fareri Children’s Hospital at Westchester Medical Center
Valhalla, NY
Patricia Lenhart-Pendergrass, MD, PhD
Division of Respiratory Medicine
San Diego, CA
Evans M. Machogu, MBChB, MS, FAAP
Section of Pediatric Pulmonology, Allergy, and Sleep Medicine
Indiana University School of Medicine
Riley Hospital for Children
Indianapolis, IN
Stacey L. Martiniano, MD
Section of Pediatric Pulmonology and Sleep Medicine

University of Colorado Denver
Aurora, CO
Oscar Henry Mayer, MD
Medical Director, Pulmonary Function Testing Laboratory
The Perelman School of Medicine at the University of Pennsylvania
Philadelphia, PA
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Contributors
Susanna A. McColley, MD, FAAP

Associate Director for Child Health
Northwestern University Clinical and Translational Sciences Institute
Scientific Director, Interdisciplinary Research Partnerships
Stanley Manne Children’s Research Institute
Ann & Robert H. Lurie Children’s Hospital of Chicago
Professor of Pediatrics in Pulmonary and Sleep Medicine
Northwestern University Feinberg School of Medicine
Chicago, IL
Peter H. Michelson, MD, MS
Medical Director, Clinical Development
Vertex Pharmaceuticals, Inc
Boston, MA
Kenneth N. Olivier, MD, MPH
Michael E. Hatcher Distinguished Professor
Department of Medicine
Division of Pulmonary Diseases and Critical Care Medicine
University of North Carolina at Chapel Hill
Chapel Hill, NC
Brian P. O’Sullivan, MD
Geisel School of Medicine at Dartmouth
Hanover, NH
Howard B. Panitch, MD
Director, Technology Dependence Center

The Perelman School of Medicine at the University of Pennsylvania
Philadelphia, PA
Madhuri Penugonda, MD
University of Florida College of Medicine
Pensacola, FL
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Contributors
Iris A. Perez, MD, FAAP

Associate Professor of Clinical Pediatrics
Los Angeles, CA
Yehudit Pollack, MD
Division of Pediatric Pulmonology, Allergy, Immunology, and
Sleep Medicine
Valhalla, NY
Priya Prashad, MD, MSCE, DABSM
Division of Pediatric Pulmonology, Allergy, Immunology, and
Sleep Medicine
Valhalla, NY
Courtney M. Quinlan, DO, MS
University of South Florida College of Medicine
Lehigh Valley Reilly Children’s Hospital
Allentown, PA
Nanda Ramchandar, MD, MPH, FAAP
Uniformed Services University
Voluntary Assistant Clinical Professor of Pediatrics
San Diego, CA
Clement L. Ren, MD, MBA
Professor of Pediatrics and Richard B. Johnston Jr Chair in Pediatrics
University of Pennsylvania Perelman School of Medicine
Cystic Fibrosis Center Director
Philadelphia, PA
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Contributors
Bruce K. Rubin, MEngr, MD, MBA, FRCPC, FAAP

Jessie Ball duPont Distinguished Professor, Department of Pediatrics
Professor and Chair Emeritus of Pediatrics
Professor of Biomedical Engineering
Virginia Commonwealth University School of Medicine
Richmond, VA
Danieli B. Salinas, MD, MS
Associate Professor of Pediatrics and Preventive Medicine
Associate Director, Cystic Fibrosis Center
Los Angeles, CA
Adrienne P. Savant, MD, MS
Service Line Chief, Pediatric Pulmonology
Children’s Hospital of New Orleans
Chief, Pediatric Pulmonology
Tulane University School of Medicine
New Orleans, LA
Mark H. Sawyer, MD
Rady Children’s Hospital San Diego
San Diego, CA
James W. Schroeder Jr, MD, MBA, FACS, FAAP

Vice Chair of Education–Department of Surgery
Ann & Robert H. Lurie Children’s Hospital of Chicago
Professor
Departments of Otolaryngology Head and Neck Surgery and
Medical Education
Northwestern University Feinberg School of Medicine
Chicago, IL
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Contributors
Marianna Martin Sockrider, MD, DrPH, FAAP

Professor of Pediatrics, Pulmonary Division
Baylor College of Medicine
Chief of Pulmonary Clinics
Texas Children’s Hospital
Houston, TX
Paul C. Stillwell, MD, FAAP
Senior Instructor, Department of Pediatrics
University of Colorado School of Medicine Anschutz Medical Campus
Pediatric Pulmonologist
Children’s Hospital Colorado
Aurora, CO
Jade B. Tam-Williams, MD, FAAP
Assistant Professor of Pediatric Pulmonology
Children’s Mercy–Kansas City
Kansas City, MO
Danna Tauber, MD, MPH
University of Pennsylvania School of Medicine
Philadelphia, PA
Nadav Traeger, MD, DABSM, FCCP, FAAP
Associate Professor of Clinical Pediatrics

Valhalla, NY
Timothy J. Vece, MD
Director, Rare and Genetic Lung Disease Program
University of North Carolina–Chapel Hill
Chapel Hill, NC
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Contributors
Nico W. Vehse, MD
Associate Professor, UMass Chan Medical School–Baystate
Chief, Pediatric Pulmonology
Medical Director, CF Center
Medical Director, Pediatric Respiratory Care
Baystate Children’s Hospital
Springfield, MA
Karen Z. Voter, MD, FAAP
University of Rochester School of Medicine
Rochester, NY
Mary H. Wagner, MD
Division of Pediatric Pulmonary and Sleep Medicine
Gainesville, FL
Miles Weinberger, MD, FAAP
Professor Emeritus
University of Iowa
Visiting Clinical Professor of Pediatrics
Rady Children’s Hospital
San Diego, CA
Pnina Weiss, MD, MHPE, FAAP
Vice Chair for Education
Section on Pulmonology, Allergy, Immunology, and Sleep Medicine

Yale School of Medicine
New Haven, CT
John Welter, MD
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Contributors
Valhalla, NY
Amy S. Whigham, MD, MS-HPEd
Associate Professor
Department of Otolaryngology–Head and Neck Surgery
Division of Pediatric Otolaryngology
Vanderbilt University School of Medicine
Nashville, TN
Eric D. Zee, MD
Clinical Associate Professor of Pediatrics
Stanford University School of Medicine
Palo Alto, CA
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Equity, Diversity, and Inclusion Statement
The American Academy of Pediatrics is committed to principles of equity,
diversity, and inclusion in its publishing program. Editorial boards, author
selections, and author transitions (publication succession plans) are designed
to include diverse voices that reflect society as a whole. Editor and author
teams are encouraged to actively seek out diverse authors and reviewers at all
stages of the editorial process. Publishing staff are committed to promoting
equity, diversity, and inclusion in all aspects of publication writing, review, and
production.
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Contents
Preface..................................................................................................................................... XXIX
Part 1. Foundation....................................................................................... 1
Chapter 1. Anatomy of the Lung............................................................................................ 3
Michael J. Light, MD
Chapter 2. Pulmonary Physiology........................................................................................15
Pnina Weiss, MD, MHPE, FAAP
Chapter 3. Applied Pulmonary Physiology....................................................................... 39
Maki Ishizuka, MD, and Christopher M. Cielo, DO, MS
Chapter 4. Taking the Pulmonary History........................................................................ 55
Chapter 5. The Pulmonary Physical Examination......................................................... 65
Chapter 6. Pulmonary Function Testing.......................................................................... 77
Carol J. Blaisdell, MD, and Allen J. Dozor, MD, FAAP
Chapter 7. Pulmonary Imaging........................................................................................... 101
Michael J. Light, MD; Julieta M. Oneto, MD; and Ricardo Restrepo, MD
Chapter 8. Bronchoscopy......................................................................................................141
Pi Chun Cheng, MD, MS, and Samuel B. Goldfarb, MD
Part 2. Allergic Conditions.................................................................... 163

Chapter 9. Allergic Bronchopulmonary Aspergillosis................................................ 165
Chapter 10. Hypersensitivity Pneumonitis.................................................................... 183

Katharine Kevill, MD, MHCDS, FAAP
Chapter 11. Eosinophilic Pneumonia................................................................................ 197
Peter H. Michelson, MD, MS
Chapter 12. Asthma................................................................................................................ 207
Miles Weinberger, MD, FAAP; Mutasim Abu-Hasan, MD; and Leslie Hendeles,
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Contents
Part 3. Anatomical Disorders................................................................................. 239

Chapter 13. Congenital Abnormalities of the Upper Airway.................................... 241
James W. Schroeder Jr, MD, MBA, FACS, FAAP; Susanna A. McColley, MD, FAAP;
and Mary Bono Cataletto, MD, FAAP
Chapter 14. Congenital Lung Anomalies......................................................................... 253
Brian P. O’Sullivan, MD, and T. Bernard Kinane, MD
Chapter 15. Chest Wall and Spinal Deformities.......................................................... 279
Julie L. Fierro, MD, MPH, and Oscar Henry Mayer, MD
Part 4. Upper Airway Infections........................................................................... 309

Chapter 16. Croup, Epiglottitis, and Bacterial Tracheitis.......................................... 311
Part 5. Lower Airway Infections............................................................................327

Chapter 17. Bronchiectasis................................................................................................... 329
Jordana E. Hoppe, MD, MSCS, and Paul C. Stillwell, MD, FAAP
Chapter 18. Bronchiolitis...................................................................................................... 341
Chapter 19. Viral Pneumonia (Including COVID-19)................................................. 353
Michael J. Light, MD; Paul C. Stillwell, MD, FAAP; Nanda Ramchandar, MD, MPH,
FAAP; and Mark H. Sawyer, MD
Chapter 20. Nonviral Pneumonia...................................................................................... 365
Michael J. Light, MD; Paul C. Stillwell, MD, FAAP; Nanda Ramchandar, MD, MPH,
FAAP; and Mark H. Sawyer, MD
Chapter 21. Complications of Pneumonia...................................................................... 387
Paul C. Stillwell, MD, FAAP

Chapter 22. Recurrent Pneumonia....................................................................................401
Paul Houin, MD, and Paul C. Stillwell, MD, FAAP
Chapter 23. Tuberculosis.......................................................................................................411
Carol Conrad, MD
Chapter 24. Nontuberculous Mycobacteria................................................................... 453
Stacey L. Martiniano, MD; Patricia Lenhart-Pendergrass, MD, PhD;
and Kenneth N. Olivier, MD, MPH
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Part 6. Noninfectious Pulmonary Disorders................................................ 467

Chapter 25. Atelectasis......................................................................................................... 469
Chapter 26. Respiratory Disorders Associated With Systemic Inflammatory
Diseases...................................................................................................................................... 481
Paul C. Stillwell, MD, FAAP, and Eric D. Zee, MD
Chapter 27. Interstitial Lung Disease...............................................................................505
Pallav Halani, MD, FAAP; Timothy J. Vece, MD; and Adrienne P. Savant, MD, MS
Chapter 28. Bronchopulmonary Dysplasia..................................................................... 521
Laurie C. Eldredge, MD, PhD, and Susanna A. McColley, MD, FAAP
Chapter 29. Pleural Effusion (Noninfectious).............................................................. 541
Chapter 30. Pneumothorax and Pneumomediastinum............................................. 553
Danna Tauber, MD, MPH
Chapter 31. Pulmonary Hemorrhage................................................................................ 563
Evans M. Machogu, MBChB, MS, FAAP, and Karen Z. Voter, MD, FAAP
Part 7. Pediatric Sleep Medicine.......................................................................... 579

Chapter 32. Development and Maturation of Sleep................................................... 581
Madeleine Grigg-Damberger, MD
Chapter 33. Obstructive Sleep Apnea Syndrome........................................................ 603
Courtney M. Quinlan, DO, MS, and Suzanne E. Beck, MD
Chapter 34. Sleep Testing in the Laboratory and Home........................................... 617
Deborah M. Brooks, MD, and Lee J. Brooks, MD, FAAP
Chapter 35. Sleep-Related Movement Disorders......................................................... 633

Jennifer Accardo, MD, MSCE
Chapter 36. Insomnia............................................................................................................. 643
Chapter 37. Parasomnias....................................................................................................... 651
Chapter 38. Narcolepsy and Other Disorders of Excessive Somnolence............659
Nadav Traeger, MD, DABSM, FCCP, FAAP
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Chapter 39. Sudden Infant Death Syndrome and Brief, Resolved,

Unexplained Events............................................................................................................... 671
Zarmina Ehsan, MD, FAAP
Chapter 40. Congenital Central Hypoventilation Syndrome.................................. 681
Iris A. Perez, MD, FAAP; Emily S. Gillett, MD, PhD, FAAP; and Thomas G. Keens,
MD, FAAP
Part 8. Other Pulmonary Issues........................................................................... 697

Chapter 41. Acute Aspiration and Chronic Aspiration–Related
Lung Disease............................................................................................................................ 699
Jade B. Tam-Williams, MD, FAAP
Chapter 42. Lung Transplantation.................................................................................... 721
Carol Conrad, MD
Chapter 43. Asthma and Other Respiratory Disorders Associated
With Obesity............................................................................................................................. 747
Mutasim Abu-Hasan, MD, and David Fedele, PhD, ABPP
Chapter 44. Functional Respiratory Disorders............................................................. 763
Miles Weinberger, MD, FAAP; Manju S. Hurvitz, MD, FAAP;
and Mutasim Abu-Hasan, MD
Part 9. Genetic Disorders......................................................................................... 779

Chapter 45. Cystic Fibrosis.................................................................................................. 781
Chapter 46. Cystic Fibrosis Newborn Screening and CFTR-Related
Metabolic Syndrome............................................................................................................. 807
Danieli B. Salinas, MD, MS, and Clement L. Ren, MD, MBA
Chapter 47. Primary Ciliary Dyskinesia and Other Genetic Lung Diseases....... 817
Madhuri Penugonda, MD; Nico W. Vehse, MD; Thomas W. Ferkol, MD; and
Bruce K. Rubin, MEngr, MD, MBA, FRCPC, FAAP
Part 10. Lung Disease Associated With Systemic Disorders.............. 849

Chapter 48. Respiratory Considerations in Children With Cardiac Disease..... 851
Yehudit Pollack, MD, and Sankaran Krishnan, MD, MPH
Chapter 49. Lung Disease Associated With Endocrine Disorders........................ 869
Michael J. Light, MD, and Martin Draznin, MD
Chapter 50. Pulmonary Complications of Gastrointestinal Diseases.................. 875
Edward W. Fong, MD
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Contents
Chapter 51. Pulmonary Complications of Sickle Cell Disease................................ 891

Robyn T. Cohen, MD, MPH
Chapter 52. Pulmonary Manifestations of Oncologic Disease
and Treatment.......................................................................................................................... 913
Mary Bono Cataletto, MD, FAAP, and Caitlin Hurley, MD
Chapter 53. Pulmonary Complications of Immunologic Disorders..................... 929
Jay Jin, MD, PhD, and Clement L. Ren, MD, MBA
Chapter 54. Pulmonary Complications of Neuromuscular Disorders.................947
Part 11. Treating and Managing Pulmonary Disease...............................969

Chapter 55. Airway Clearance Techniques..................................................................... 971
Karen Ann Hardy, MD, FATS
Chapter 56. Aerosol Delivery of Medication.................................................................. 991
Ariel Berlinski, MD, FAAP, FAARC
Chapter 57. Bronchodilators............................................................................................. 1005
John Welter, MD; H. William Kelly, PharmD; and Leslie Hendeles, PharmD
Chapter 58. Antibiotics for Pulmonary Conditions.................................................. 1019
Michael J. Light, MD; Nanda Ramchandar, MD, MPH, FAAP; and John S. Bradley,
MD, FAAP
Chapter 59. Nutritional Aspects of Pulmonary Conditions...................................1045
Ellen K. Bowser, MS, RDN, LDN, RN, FAND, and Mary H. Wagner, MD
Chapter 60. Oxygen Therapy............................................................................................ 1061
Shyall Bhela, MD, and Sankaran Krishnan, MD, MPH
Chapter 61. Nicotine and Tobacco..................................................................................1083

Harold J. Farber, MD, MSPH, FAAP, and Marianna Martin Sockrider, MD, DrPH,
FAAP
Chapter 62. Tracheostomy.................................................................................................. 1111
Amy S. Whigham, MD, MS-HPEd
Chapter 63. Home Ventilation...........................................................................................1125
Howard B. Panitch, MD, and Thomas G. Keens, MD, FAAP
Index.............................................................................................................................1145
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Preface
We are pleased to present the second edition of Pediatric Pulmonology. While
the first edition was intended for primary care physicians, many pediatric
pulmonologists have come to rely on “the blue book” as an excellent refer-
ence. In the second edition, we attempted to provide a broad-based reference
that would be useful to subspecialists while still being a most useful reference
for primary care physicians, residents, and students. Recognizing that an
ever-growing number of pulmonology sections and practices include sleep
medicine specialists, this edition dedicates a full section to pediatric sleep
medicine—expanding beyond apparent life-threatening events (now termed
brief, resolved, unexplained events) and sleep-disordered breathing to include
information on normal sleep, sleep-related movement disorders, sleep-wake
disorders, and disorders of excessive somnolence.
As with the first, this edition begins by covering the fundamental topics of
lung anatomy, pulmonary physiology, and the basics of eliciting a thorough
pulmonary history and physical examination. Among the many updates
throughout the book, the chapters on cystic fibrosis and cystic fibrosis trans-
membrane conductance regulator (CFTR)-related metabolic syndrome are
significantly different, as they reflect the many developments and advances in
the treatment of these disorders since the first edition was published in 2011.
In addition, the section on lower airway infections was updated and expanded
with more in-depth coverage of pneumonia and the complications of pneumo-
nia, including pneumonia caused by COVID-19.
Dr Light, editor in chief of the first edition, returns to lend his experience and
expertise to this revision, collaborating this time with Dr Van Hook, past chair
of the American Academy of Pediatrics (AAP) Section on Pediatric Pulmon-

ology and Sleep Medicine, as well as an esteemed team of associate editors.
This update would not have been possible without the tireless efforts of Chris
Wiberg, our liaison with American Academy of Pediatrics Publishing; we are
indebted to him for his assistance. We would also like to thank the authors of
the individual chapters, who spent countless hours researching and revising
their individual contributions to make this book possible.
Michael J. Light, MD
XXIX
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Richard M. Kravitz, MD, FAAP

Committees, Councils, and Sections

Section on Minority Health, Equity, and Inclusion

Rajeev Bhatia, MD, FAAP

Lauren Elizabeth (L.E.) Faricy, MD, FAAP

Theresa Guilbert, MD, FAAP

Brooke Gustafson, MD, FAAP

Binal Kancherla, MD, FAAP

Rebekah Nevel, MD, FAAP

Christopher Oermann, MD, FAAP

Andrew Sokolow, MD, FAAP

Kristin Van Hook, MD, MPH, FAAP

Richard M. Kravitz, MD, FAAP

Maria Fareri Children’s Hospital

John S. Bradley, MD, FAAP

Chobanian and Avedisian Boston University School of Medicine

Laurie C. Eldredge, MD, PhD

Division of Pediatric Pulmonology, Allergy, and Sleep Medicine

Section of Pediatric Pulmonology and Sleep Medicine

Susanna A. McColley, MD, FAAP

Children’s Hospital of Philadelphia

Iris A. Perez, MD, FAAP

Bruce K. Rubin, MEngr, MD, MBA, FRCPC, FAAP

James W. Schroeder Jr, MD, MBA, FACS, FAAP

Marianna Martin Sockrider, MD, DrPH, FAAP

Maria Fareri Children’s Hospital at Westchester Medical Center

Section on Pulmonology, Allergy, Immunology, and Sleep Medicine

Part 2. Allergic Conditions.................................................................... 163

Chapter 10. Hypersensitivity Pneumonitis.................................................................... 183

Part 3. Anatomical Disorders................................................................................. 239

Part 4. Upper Airway Infections........................................................................... 309

Part 5. Lower Airway Infections............................................................................327

Paul C. Stillwell, MD, FAAP

Part 6. Noninfectious Pulmonary Disorders................................................ 467

Part 7. Pediatric Sleep Medicine.......................................................................... 579

Chapter 35. Sleep-Related Movement Disorders......................................................... 633

Chapter 39. Sudden Infant Death Syndrome and Brief, Resolved,

Part 8. Other Pulmonary Issues........................................................................... 697

Part 9. Genetic Disorders......................................................................................... 779

Part 10. Lung Disease Associated With Systemic Disorders.............. 849

Chapter 51. Pulmonary Complications of Sickle Cell Disease................................ 891

Part 11. Treating and Managing Pulmonary Disease...............................969

Chapter 61. Nicotine and Tobacco..................................................................................1083

of the American Academy of Pediatrics (AAP) Section on Pediatric Pulmon-

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