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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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x ■ Contributors

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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xi

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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xii ■ Reviewers

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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