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ContributorsJANE BENSON, MDAssistant ProfessorPediatric RadiologyThe Johns Hopkins School
of MedicineBaltimore, MD
RENEE BOSS, MD, MHSAssistant Professor, Division of
Neonatology, Department of Pediatrics
The Johns Hopkins School of Medicine
The Johns Hopkins Berman Instituteof Bioethics
Baltimore, MD
MARISSA BRUNETTI, MDAttending IntensivistThe Children’s Hospital
of PhiladelphiaPhiladelphia, PA
HEATHER CHANDLER, MD,MPH
Assistant Professor, Pediatric Critical Care
Emory University Atlanta, GA
SHAWN COLBOURN, AS, RRT-NPSPediatric Respiratory TherapistThe Children’s Hospital
of PhiladelphiaPhiladelphia, PA
ELIZABETH CRISTOFALO, MDAssistant Professor of PediatricsThe Johns Hopkins School
of Medicine Baltimore, MD
ELLEN DEUTSCH, MD, FACS,FAAP
Director, Simulation and Systems Integration
Center for Simulation, Advanced Education, and Innovation
The Children’s Hospital of Philadelphia
Philadelphia, PA
DHEERAJ GOSWAMI, MDPediatric Critical Care FellowThe Johns Hopkins HospitalBaltimore, MD
ROBERTA HALES, MHA, RRT-NPS, RN
Simulation EducatorCenter for Simulation, Advanced
Education and InnovationThe Children’s Hospital
of PhiladelphiaPhiladelphia, PA
ANDREA L. HONESTO, BS, RRT
Respiratory Care Practitioner IICystic Fibrosis CenterThe Johns Hopkins HospitalBaltimore, MD
ELIZABETH A. HUNT, MD,MPH, PHD
Associate Professor of Anesthesiologyand Critical Care Medicine
Associate Professor of PediatricsThe Johns Hopkins School
of MedicineBaltimore, MD
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SAFEENA KHERANI, MDFRCSC
Assistant ProfessorUniversity of OttawaOttawa, ON
HOLLY LOOSEN, PTPhysical Therapist IICystic Fibrosis CenterThe Johns Hopkins HospitalBaltimore, MD
STACEY MANN, BS, RRTManager, Pediatric Respiratory Care
and ECMO ServicesThe Johns Hopkins HospitalBaltimore, MD
MEGAN MCCABE, MD, FAAPAssistant ProfessorSection of Pediatric Critical Care
Medicine, Department of Pediatrics
Director, Pediatric Critical Care Fellowship Program
Yale University School of MedicineNew Haven, CT
SHARON MCGRATH-MORROW,MD, MBA
Associate Professor of PediatricsDivision of Pediatric PulmonaryThe Johns Hopkins School
of MedicineBaltimore, MD
PETER MOGAYZEL JR., MD, PHDAssociate Professor of PediatricsThe Johns Hopkins School
of MedicineDirector, Cystic Fibrosis CenterThe Johns Hopkins HospitalBaltimore, MD
SARAH JANN MOLA, MDAssistant Professor of Pediatrics Medical Director, Maryland
Regional Neonatal Transport Program, University of MarylandSchool of Medicine
Baltimore, MD
WYNNE MORRISON, MD, MBEAssistant Professor, Department of
Anesthesiology and Critical Care,Perelman School of Medicine at theUniversity of Pennsylvania
Children’s Hospital of PhiladelphiaPhiladelphia, PA
VINAY NADKARNI, MD, MSEndowed Chair, Pediatric Critical
Care MedicineAssociate Professor, Anesthesia,
Critical Care and Pediatrics, University of Pennsylvania School of Medicine
The Children’s Hospital of Philadelphia
Philadelphia, PA
GARY OLDENBURG, RRT-NPSECMO and VAD Program ManagerChildren’s National Medical CenterWashington, DC
STACEY B. PEDDY, MDDirector of Simulation, Cardiac
Intensive Care UnitAssistant Professor, Anesthesiology
and Critical Care MedicineThe Children’s Hospital
of PhiladelphiaPhiladelphia, PA
SHANNON POLING, BS, RRTSimulation EducatorThe Johns Hopkins Medicine
Simulation CenterBaltimore, MD
SUZANNE PRESTWICH, MDMedical Director, Inpatient Pediatric
Rehabilitation UnitKennedy Krieger InstituteBaltimore, MD
WEBRA PRICE-DOUGLAS PHD,CRNP, IBCLC
Coordinator, Maryland Regional Neonatal Transport Program
Baltimore, MD
JENNIFER SCHUETTE, MDAttending Physician, Cardiac
ICUChildren’s National Medical
CenterWashington, DC
JAMIE MCELRATH SCHWARTZ,MD
Medical Director, Cardiac Procedure Recovery Unit Anesthesiology and Pain Medicine
Children’s National Medical CenterWashington, DC
THEODORA STAVROUDIS, MD
Assistant Professor of PediatricsUniversity of Southern California Los Angeles, CA
MATTHEW TROJANOWSKI, BA,RRT
Manager, Adult Respiratory CareServices
The Johns Hopkins HospitalBaltimore, MD
KAREN VON BERG, PTPhysical Therapist IICystic Fibrosis CenterThe Johns Hopkins HospitalBaltimore, MD
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ReviewersTERI ALLEN, BSHA, RRT-NPSNeonatal/Pediatric Clinical Education
CoordinatorUniversity of California–IrvineOrange, CA
NICKI SUE BLY, BS, RRT-NPSDirector of Clinical Education,
Respiratory CareHighline Community CollegeDes Moines, WA
LISA CONRY, MA, RRTDirector of Clinical EducationSpartanburg Community CollegeSpartanburg, SC
HELEN S. CORNING, RRTRegistered Respiratory TherapistShands/UF Medical CenterJacksonville, FL
JUANITA DAVIS, RRTFacultySouthern Alberta Institute of
TechnologyAlberta, Canada
MARTHA DESILVA, MED, RRT-AC-E
Program Director, Respiratory CareMassasoit Community CollegeBrockton, MA
LEZLI HEYLAND, BS, RRT-NPSProgram Director, Respiratory Care
ProgramOklahoma City Community CollegeOklahoma City, OK
NANCY A. JOHNSON, RRT-NPSPediatric Respiratory Education
CoordinatorUniversity Hospitals of ClevelandRainbow BabiesChildren’s HospitalCleveland, OH
ROBERT L. JOYNER, JR., PHD,RRT, FAARC
Associate Dean, Henson School ofScience & Technology
Director, Respiratory Therapy Program
Salisbury UniversitySalisbury, MD
CHRISTY J. KANE, PHD, RRT-NPS
Department Chair, Respiratory Therapy Department
Bellarmine UniversityLouisville, KY
ALETA KAY MARTIN, BSRC,RRT-NPS
Clinical EducatorChildren’s Medical CenterDallas, TX
KERRY J. MCNIVEN, MS, RRTProfessor, DirectorManchester Community CollegeManchester, CT
LISA J. PIERCE, MSA, BS, RRTClinical Director, Respiratory CareAugusta Technical CollegeAugusta, GA
GEORGETTE ROSENFELD, PHD,RRT, RN
Department Chair, Respiratory CareIndian River State CollegeFort Pierce, FL
MELISSA D. SMITH, BS, RRTRespiratory Care Education
CoordinatorChapter 8 President, Louisiana
Society for Respiratory CareSt. Tammany Parish HospitalCovington, LA
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STEPHEN G. SMITH, MPA, RT,RRT
Clinical Assistant ProfessorStony Brook UniversityStony Brook, NY
JANA STAFFORD, BS, RRT, RCPProfessor of Respiratory TherapyRose State CollegeMidwest City, OK
GEORGE SWEAT, RN, RRTRespiratory Therapy ManagerHoward County General HospitalColumbia, MD
ROBERT J. TRALONGO, MBA,RT, RRT-NPS, CPFT, AE-C
Program Director, Respiratory CareMolloy CollegeRockville Centre, NY
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ContentsChapter 1
Making Sense of Caring for Kids: A Different Approach to Respiratory Care
Julianne S. Perretta, MSEd, RRT-NPS, CHSE 1Anatomical and Physiological Differences Between Children
and Adults 3Patient Assessment 6Pharmacology 14Equipment Selection 16Intensive Care Unit Classifications 17Evidence-Based Medicine 18
Section OneFetal Development and Transition to Extrauterine Life
Chapter 2
Fetal Cardiopulmonary DevelopmentJulianne S. Perretta, MSEd, RRT-NPS, CHSE 31
Determining Gestational Age 33Fetal Lung Development 33Fetal Cardiac Development 35Fetal Neurological Development 36Fetal Circulation 37Changes to Fetal Circulation at Delivery 38Assessing Fetal Lung Maturity 39Tocolytics and Glucocorticoids During Preterm Labor 41Implications of Development on Neonatal Course 41
Chapter 3
Resuscitation of the Newborn During Transition to Extrauterine Life
Theodora A. Stavroudis, MD 45The Three Stages of Labor 46Fetal Monitoring During Labor and Delivery 47Transition to the Extrauterine World 47
Section TwoPerinatal Lung Diseases and Complications
Chapter 4
Respiratory Distress SyndromeJulianne S. Perretta MSEd, RRT-NPS, CHSE 65
Hyaline Membrane Disease/Respiratory Distress Syndrome 67
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Chapter 5
Apnea of Prematurity and Bronchopulmonary Dysplasia
Julianne S. Perretta, MSEd, RRT-NPS, CHSE 111Apnea of Prematurity 112Bronchopulmonary Dysplasia 118
Chapter 6
Disease of Full-Term InfantsSara Jann Mola, MD • Webra Price-Douglas, PhD, CRNP, IBCLC • Julianne S.Perretta, MSEd, RRT-NPS, CHSE • Shannon Poling, BS, RRT Gary Oldenburg, RRT-NPS 135
Persistent Pulmonary Hypertension of the Newborn 136Meconium Aspiration Syndrome 151Transient Tachypnea of the Newborn 157
Section ThreeCommon Neonatal Complications
Chapter 7
Pulmonary ComplicationsJulianne S. Perretta, MSEd, RRT-NPS, CHSE 165
Atelectasis 166Pulmonary Interstitial Emphysema 170Pneumothorax 174Pneumomediastinum 176Pneumopericardium 177Pneumonia 178
Chapter 8
Multisystem ComplicationsElizabeth Cristofalo, MD • Julianne S. Perretta, MSEd, RRT-NPS, CHSEMatthew Trojanowski, BA, RRT 185
Intraventricular Hemorrhage 187Patent Ductus Arteriosus 193Necrotizing Enterocolitis 199Retinopathy of Prematurity 204
Section FourCongenital Anomalies
Chapter 9
Abdominal DefectsJulianne S. Perretta, MSEd, RRT-NPS, CHSE 213
Congenital Diaphragmatic Hernia 214Gastroschisis 226Omphalocele 228
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Chapter 10
Airway Abnormalities Ellen Deutch, MD FACS, FAAP • Safeena Kherani, MD FRCSC 237
Airway Abnormalities of the Nasal Passages and Nasopharynx 239Choanal Atresia 239Other Conditions Affecting the Nasal Passages and
Nasopharynx 241Airway Abnormalities of the Oral Cavity and Oropharynx 242Robin Sequence 242Other Conditions Affecting the Oral Cavity and Oropharynx 244Airway Abnormalities of the Supraglottis 244Laryngomalacia 245Other Conditions Affecting the Supraglottis 246Airway Abnormalities of the Glottis 247Recurrent Respiratory Papillomatosis 247Other Conditions Affecting the Glottis 248Airway Abnormalities of the Subglottis 249Subglottic Stenosis 249Other Conditions Affecting the Subglottis 251Airway Abnormalities of the Trachea and Bronchi 251Tracheoesophageal Fistula 251Other Conditions Affecting the Trachea and Bronchi 253Tracheotomies 253
Chapter 11
Acyanotic Heart Defects Stacey Peddy, MD • Heather Chandler, MD, MPH 261
Atrial Septal Defect 263Ventricular Septal Defect 266Atrioventricular Septal Defect 267Aortic Stenosis 268Pulmonic Stenosis 270Coarctation of the Aorta 271Double Aortic Arch 273
Chapter 12
Cyanotic Heart DefectsMarissa Brunetti, MD • Jamie McElrath Schwartz, MD 279
Tetralogy of Fallot 282Total Anomalous Pulmonary Venous Return 285Transposition of the Great Arteries 288Hypoplastic Left Heart Syndrome 291Ebstein Anomaly 296Truncus Arteriosus 298
Section FivePediatric Diseases With Respiratory Implications
Chapter 13
AsthmaJulianne S. Perretta, MSEd, RRT-NPS, CHSE 307
Asthma 308
Chapter 14
Cystic FibrosisPeter Mogayzel, Jr. MD, PhD, • Holly Loosen, PTKaren Von Berg, PT, • Andrea L Honesto, BA, RRT 355
Cystic Fibrosis 356
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Chapter 15
Pediatric Pulmonary DiseasesVinay Nadkarni, MD • Roberta Hales MHA, RRT-NPS, RNShawn Colbourn, AS, RRT-NPS 381
Bronchiolitis 382Pneumonia 393Acute Respiratory Distress Syndrome 399
Chapter 16
Pediatric Infectious Airway DiseasesStacey Mann, BS, RRT 415
Epiglottitis 417Croup 422Bacterial Tracheitis 426
Chapter 17
Neuromuscular DisordersSharon McGrath-Morrow, MD, MBA • Suzanne Prestwich, MD 433
Guillain- Barré Syndrome 438Myasthenia Gravis 441Tetanus 442Botulism 443Muscular Dystrophy 445Spinal Muscular Atrophies 447
Chapter 18
Pediatric Accidents With Pulmonary InvolvementElizabeth A. Hunt, MD, MPH, PhD • Dheeraj Goswami, MD 453
Near-Drowning 454Inhalation Injuries 462Upper-Airway Injury 462Direct Lung Injury 463Systemic Complications 465Cyanide Poisoning 466Aspiration 467Foreign Body Aspiration 468Chemical Pneumonitis 470Hydrocarbons 470Lipoids 471Secretions 472Gastric Secretions 474
Chapter 19
Pediatric Neurological AccidentsJennifer Schuette, MD 481
Traumatic Brain Injury 482Spinal Cord Injury 492Neurogenic Pulmonary Edema 495
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Section SixEthics and Pediatric Palliative Care
Chapter 20
Ethics in Perinatal and Pediatric Respiratory Care Renee Boss, MD, MHS • Megan McCabe, MD, FAAP 503
Principles of Bioethics 504Professional Ethics 506Clinical Ethics 509Clinical Scenarios With Ethical Implications for Pediatric
Respiratory Care 514
Chapter 21
Palliative and End-of-Life CareWynne Morrison, MD, MBE 525
Framing the Discussion About Appropriate Goals of Care 528Early Referral/Concurrent Care 530Do Not Attempt Resuscitation Orders 531Discontinuing Mechanical Ventilation 533Treating Dyspnea 535Location of Death 538Supporting the Family 539Supporting the Team 539
Glossary 545
Index 563
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