Kendig and Chernick's Disorders of the Respiratory Tract in Children E-Book

Robert W. Wilmott; Thomas F. Boat; Andrew Bush; Victor Chernick; Robin R Deterding; Felix Ratjen

BOOK

Kendig and Chernick's Disorders of the Respiratory Tract in Children E-Book

(2012)

Additional Information

Book Details

ISBN: 978-1-4557-4050-5
Edition: 8
Language: English
Pages: 1168
Subjects: Immunology
Paediatric medicine
Neonatal medicine
Cardiothoracic surgery

Abstract

Kendig, Chernick’s Disorders of the Respiratory Tract in Children is the definitive medical reference book to help you confront critical challenges using the latest knowledge and techniques. You’ll get the state-of-the-art answers you need to offer the best care to young patients.

Tackle the toughest challenges and improve patient outcomes with coverage of all the common and rare respiratory problems found in newborns and children worldwide.

Get a solid foundation of knowledge to better understand and treat your patients through coverage of the latest basic science and its relevance to clinical problems.

Get comprehensive, authoritative coverage on today’s hot topics, such as interstitial lung disease, respiratory disorders in the newborn, congenital lung disease, swine flu, genetic testing for disease and the human genome, inflammatory cytokines in the lung, new radiologic techniques, diagnostic imaging of the respiratory tract, and pulmonary function tests.

Learn from the experts with contributions from 100 world authorities in the fields of pediatrics, pulmonology, neurology, microbiology, cardiology, physiology, diagnostic imaging, anesthesiology, otolaryngology, allergy, and surgery.

Section Title	Page	Action	Price
Front Cover	Cover
KENDIG AND CHERNICK'S Disorders OF THE Respiratory Tract IN Children	iii
Copyright	iv
Preface	v
Contributors	vi
Contents	xvi
Section I: General Basic Considerations	1
Chapter 1: Molecular Determinants of Lung Morphogenesis	1
Lung Morphogenesis	2
The Embryonic Period (3 to 6 Weeks Postconception)	2
Pseudoglandular Period (6 to 16 Weeks' Postconception)	4
Canalicular Period (16 to 26 Weeks Postconception)	5
Saccular (26 to 36 Weeks' Postconception) and Alveolar Periods (36 Weeks' Postconception through Adolescence)	5
Control of Gene Transcription During Lung Morphogenesis	6
Transcriptional Cascades/Hierarchies	6
Combinatorial Regulation of Gene Transcription and Expression	7
Influence of Chromatin Structure on Gene Expression	7
Non-Transcriptional Mechanisms	8
Receptor-Mediated Signal Transduction	8
Gradients of Signaling Molecules and Localization of Receptor Molecules	8
Transcriptional Mechanisms Controlling Gene Expression During Pulmonary Development	8
Epithelial-Mesenchymal Interactions and Lung Morphogenesis	10
Branching Morphogenesis, Vascularization, and Sacculation	10
Control of Lung Proliferation During Branching Morphogenesis	10
Role of Extracellular Matrix, Cell Adhesion, and Cell Shape	11
Autocrine-Paracrine Interactions in Lung Injury and Repair	11
Host Defense Systems	11
Innate Defenses	12
Gene Mutations in Lung Development and Function	12
Summary	13
Suggested Reading	13
References	13
Chapter 2:Basic Genetics and Epigenetics of Childhood Lung Disease	14
Background	14
Cystic fibrosis: strategies for the mapping of a single gene disorder	14
Cystic fibrosis: fine-scale heterogeneity in disease causation	15
Novel methods for the identification of genetic modifiers	15
Asthma	16
Genome-wide association	16
Missing heritability	20
Heritable and genetic are not interchangeable terms	20
Implications for the heritability of asthma	21
Suggested Reading	22
References	22
Chapter 3:Gene By Environment Interaction in Respiratory Diseases	23
The definition of gene by environment interaction	23
Gene by environment interaction in asthma	24
Environmental tobacco smoke	25
Air pollution and oxidative stress response pathways	26
Microbial exposures and pattern recognition receptor	26
Genome-wide interaction studies (GWIS)	26
Epigenetics: genetic and environmental factors	27
Conclusion	27
Suggested Reading	28
References	28
Chapter 4:The Surfactant System	29
Surfactant composition	30
metabolism	29
Surfactant metabolism and secretion	30
Alveolar life cycle of surfactant	30
Surfactant function	30
Alveolar Stability	30
Pressure-Volume Curves	31
Host defense functions of surfactant	32
Surfactant deficiency	32
The Preterm Infant with Respiratory Distress Syndrome	32
The injured mature lung	32
Genetic deficiencies of surfactant in mice and humans	33
Surfactant treatment of surfactant deficiency	34
Respiratory Distress Syndrome	34
Acute Respiratory Distress Syndrome	34
Suggested Reading	34
References	34
Chapter 5:The Structural and Physiologic Basis of Respiratory Disease	35
Normal lung anatomy and cell function	35
Airways	35
Alveolar region	38
Pulmonary vascular system	40
Lymphatic system	41
Innervation of the lung	41
Interstitium	41
Growth and development of the lung	42
Prenatal Lung Growth	42
The lung at birth	43
Postnatal lung growth	44
Ventilation and mechanics of breathing	45
Definitions and symbols	46
Properties of Gases	46
Elastic recoil of the lung	47
Compliance of the lung	48
Elastic properties of the chest wall	48
Lung volumes	49
Definition	49
Measurement	50
Interpretation	50
Regional lung volumes	50
Dynamic (flow-resistive) properties of the lung	51
Gas Flow Within Airways	51
Measurement of Resistance	51
Sites of Airway Resistance	52
Factors That Affect Airway Resistance	52
Dynamic Airway Compression	52
Work of Breathing	53
Distribution of ventilation	53
Pulmonary circulation	53
Physiologic Classification of Pulmonary Vessels	54
Pulmonary vascular pressures	54
Pulmonary vascular resistance	54
Distribution of blood flow	55
Methods of evaluating the pulmonary circulation	55
Muscles of respiration	56
Gas exchange	57
Alveolar ventilation	57
Dead space	58
Alveolar Ventilation and Alveolar Gases	59
Diffusion	59
Principles	59
Measurement	61
Shunt and ventilation-perfusion relationships	61
Systemic gas transport	62
Oxygen Transport	62
Assessment of Blood Oxygenation	64
Oxygen Delivery to Tissues	64
Oxygen therapy	65
Increased Inspired Mixtures	65
Administration of Oxygen	66
Hazards of High Oxygen Mixtures	66
Carbon dioxide transport and acid-base balance	66
Buffering and Transport	66
Acid-Base Balance	69
Difference between Additions of CO 2 to Blood In Vitro and In Vivo	70
Tissue respiration	70
Aerobic Metabolism	70
Anaerobic Metabolism	71
Relationship Between o 2 and co 2	71
Regulation of respiration	71
Sensory feedback system	72
O 2 and CO 2	72
The Newborn Infant	73
Metabolic functions of the lung	73
Acknowledgments	73
Suggested Reading	73
Normal Lung Anatomy and Cell Function	73
Pulmonary Circulation	73
Growth and Development of the Lung	73
Lung Physiology	73
Pulmonary Function Testing	74
Respiratory Muscle Testing	74
Control of Breathing	74
References	74
Chapter 6:Biology and Assessment of Airway Inflammation	75
Introduction	75
Acute inflammation	75
Chronic Inflammation	75
Structural Changes and Repair	76
Inflammatory cells	76
Mast Cells	77
Macrophages	77
Dendritic Cells	77
Eosinophils	77
Neutrophils	78
T-Lymphocytes	78
B-Lymphocytes	79
Basophils	79
Platelets	79
Structural cells as sources of mediators	79
Inflammatory mediators	80
Lipid Mediators	80
Cytokines	80
Chemokines	82
Oxidative Stress	82
Growth Factors	82
Neural mechanisms	82
Transcription factors	83
Anti-Inflammatory mechanisms	84
Non-Invasive assessment of airway inflammation	85
Induced Sputum	85
Exhaled Gases	85
Exhaled Nitric Oxide	85
Other Exhaled Gases	86
Exhaled Breath Condensate	86
Is AHR an Inflammatory Surrogate?	86
Other Potential Undirect Inflammatory Markers	86
Is there a role for inflammOmetry in pediatric respiratory disease?	86
Direct measurements of airway inflammation	87
Therapeutic implications	87
Corticosteroid Mechanisms	87
Mechanisms of Corticosteroid Resistance	87
Conclusion	87
Suggested Reading	88
References	88
Chapter 7:Lung Defenses: Intrinsic, Innate, and Adaptive	89
Intrinsic lung defenses	89
Aerodynamic Filtering	89
Humidification	89
Airway Reflexes	90
The Mechanics of Cough and Abnormalities in the Cough Reflex	90
Mucus and Airway Surface Liquid	91
Therapy for Mucus Clearance Disorders	92
Disorders of the Mucociliary System	92
Innate lung defenses	93
Complement	93
Adhesion Proteins	94
Pattern-Recognition Receptors in Lung Innate Immunity	95
Toll-Like Receptors	95
Nod-Like Receptors	96
RIG-Like receptors	96
Cytosolic DNA Sensors	96
Soluble Extracellular Pattern-Recognition Proteins	96
Collectins	97
Ficolins	97
Antimicrobial Peptides	97
Antiproteases	98
Antimicrobial Peptides: Defensins and Cathelicidins	98
Cellular Defenses: At the Crossroads of Innate and Adaptive Immunity	98
Inflammatory Cells in the Lung	98
The Respiratory Epithelium	98
Resident Cell Defenses: At the Interface of Innate and Adaptive Immunity	99
Dendritic Cells	99
Macrophages	99
Mast Cells	101
Recruited Cellular Defenses	102
Neutrophils	102
Eosinophils	103
Innate Lymphocyte Responses in the Lung: Natural Killer Cells, Natural Killer T Cells, and g d T Cells	104
Adaptive Lung Defenses	104
T Lymphocytes and Lung Defense	105
B Lymphocytes	105
Humoral Immunity	105
Immunoglobulin A	106
Immunoglobulin G	106
Immunoglobulin E	106
Immunoglobulin M	107
The Role of Programmed Cell Death and Removal of Dead Cells in Lung Health, Injury, and Repair	107
Summary	108
Suggested Reading	108
References	109
Section II:General Clinical Considerations	110
Chapter 8:The History and Physical Examination	110
The history	110
General principles	110
Structure of the pediatric history	110
The physical examination	112
Inspection	112
Palpation	116
Auscultation	117
Thoracic Acoustics	117
Technique of Auscultation	119
Percussion	122
Taste and Smell	122
Common signs and symptoms of chest disease in children	123
Cough and Sputum Production	123
Noisy Breathing	123
Wheezing	124
Cyanosis	125
Digital Clubbing	126
Cardiovascular Signs	127
Chest Pain	128
Conclusion	129
Suggested Reading	129
General Reading	129
Respiratory Sounds	129
Pulsus Paradoxus	129
Digital Clubbing	130
Cough and Wheezing	130
Chest Pain	130
Cyanosis	130
References	130
Chapter 9:Bronchoscopy and Bronchoalveolar Lavage in Pediatric Patients	131
Instrumentation	131
Care and Maintenance of Bronchoscopes	132
Indications for diagnostic bronchoscopy	132
Bronchoalveolar lavage	133
Indications for BAL	134
Techniques for BAL	135
Processing of BAL Specimens	136
Interpretation of BAL Findings	136
Special Techniques	138
Research Applications	138
Therapeutic indications for bronchoscopy	138
Contraindications to bronchoscopy	139
Anesthesia for bronchoscopy	139
Techniques for bronchoscopy	140
Facilities for Bronchoscopy	140
Rigid Bronchoscopy	141
Flexible Bronchoscopy	141
Special Procedures	142
Complications of bronchoscopy	143
Economic Aspects of Bronchoscopy	144
References	144
Chapter 10:Diagnostic Imaging of the Respiratory Tract	145
Plain radiography	145
Specific features of the chest radiograph in children	145
The Thymus	145
The Cardiothoracic Ratio	145
Kink of the Trachea to the Right	145
Soft Tissue	145
Pleural Fluid	146
Systematic review of the chest radiograph	146
General Degree of Lung Inflation	146
Asymmetrical Lung Volume	146
Lobar Overinflation	147
Mediastinal Distortion	147
Hilar Expansion	149
Lung Opacities	149
Focal and Multifocal Lung Densities	151
Pulmonary Interstitial Emphysema	152
Lung Abscess	152
Diffuse Interstitial Lung Disease	153
Pneumothorax	153
Skeletal Abnormalities Associated with Respiratory Disorders	153
Fluoroscopic techniques	154
Spiral computed tomography	154
Isotropic computed tomography	155
Data processing	155
Multi-Planar Reconstruction	156
Multi-Planar Volume Reconstructions: Maximum Intensity Projection and Minimum Intensity Projection	156
Shaded-Surface Display	157
Volume-Rendering Technique	157
Virtual Bronchoscopy	158
Review of findings	158
Pitfalls	158
Dose	159
High-resolution computed tomography	161
Dose	162
Controlled ventilation technique	162
Interpretation	162
Regional or Generalized Increased Density	162
Regional or Generalized Decreased Density	163
Septal Thickening	164
Nodules	164
Bronchial Change	164
Architectural Distortion	165
Angiography	165
Magnetic resonance imaging	165
Endobronchial ultrasonography	167
Ultrasonography	167
Radionuclide imaging	168
Conclusion	168
Suggested Reading	168
References	168
Chapter 11:Pulmonary Function Tests in Infants and Preschool Children	169
Historical background	169
Overview	169
Differences in assessing lung function in infants and preschool children	170
Infants and Toddlers Younger Than 2 Years of Age	170
Developmental changes pertinent to infant PFTs 4	170
Influence of the Upper Airways	170
Compliance of the Chest Wall and Dynamic Elevation of End-Expiratory Level	170
Sleep state, sedation, and duration of the testing procedure	171
Studies in Unsedated Infants	171
Studies in Sedated Infants	171
Safety issues and posture	171
Equipment requirements	171
Leaks and dead space	172
Preschool children	172
Anthropometry and background details	173
Methods of assessing pulmonary function in infants and young children	173
Assessment of lung volume and ventilation	174
Why Measure Lung Volumes?	174
Tidal breathing parameters	175
Equipment and Procedure	175
Calculation and Reporting of Results	176
Advantages and Limitations	176
Future Directions	177
Plethysmographic assessment of lung volumes	178
Infants	178
Advantages and Limitations	178
Preschool Children	179
Gas dilution or washout techniques to assess lung volumes	179
Multiple-Breath Inert Gas Washout	180
Methodological Considerations	180
Advantages and Disadvantages	181
Gas-mixing efficiency	181
Theoretical Background	181
Advantages and Limitations	183
Clinical Applications	183
Respiratory mechanics: resistance and compliance	184
Introduction	184
Plethysmographic assessments of airway resistance	184
Methodological and Theoretical Considerations	185
Equipment and Procedure	185
Infants	185
Preschool Children	186
Reporting of Results	186
Advantages and Limitations	188
Passive respiratory mechanics	188
Methodological and Theoretical Considerations	188
Advantages and Limitations	189
Interrupter technique	189
Theoretical and Methodological Considerations	190
Advantages and Limitations	191
Forced Oscillation Technique	192
Methodological and Theoretical Considerations	192
Advantages and Limitations	193
Chest wall mechanics	193
Forced expiratory maneuvers	194
Assessments in infants	194
Partial Forced Expiratory Maneuvers	194
Methodological Considerations	194
Advantages and Limitations	195
Forced Expiratory Maneuvers From Raised Lung Volume	195
Methodological Considerations	197
Analysis and Reporting of Results	197
Advantages and Limitations	199
Preschool Spirometry	199
Data Collection	199
Reporting of Results	200
Quality Control	201
Interpretation of Results	201
Assessment of bronchial responsiveness	203
Methodological Issues	203
Choice of Tests	203
Choice of Provocative Stimulus	203
Administration of an Inhaled Agent	204
Evaluation of Response	204
Age-Related Changes in Hyperresponsiveness	204
Future Directions	204
Applications of pfts in infants and preschool children	205
Epidemiologic Research into Early Determinants of Respiratory Function	205
Infant lung function as a predictor of subsequent respiratory morbidity	205
Applications during clinical research studies	206
Difficulties in Assessing Lung Function in Infants and Young Children with Respiratory Disease	206
Clinical Research Studies	206
Applications During and After Intensive Care	207
Interpretation of Lung Function Results in Infants and Young Children and Their Role in Clinical Management	208
What Is Normal?	208
Can Lung Function Tests Be Used in the Clinical Management of Individual Infants and Preschool Children?	209
Future Directions	209
Summary and conclusions	209
Suggested Reading	210
References	210
Chapter 12:Pulmonary Function Testing in Children	211
Indications for testing	211
Laboratory environment	211
Lung volumes	211
Gas dilution techniques	211
Helium Dilution	211
Nitrogen Washout	213
Plethysmography	214
Forced expiratory spirometry	215
Historical Background	215
Flow limitation	215
Equipment	218
Testing procedure	218
Calculation of results	219
Assessing airway responsiveness	220
Interpretation of results	221
Reference standards	222
Type of pulmonary dysfunction	223
Degree of pulmonary dysfunction	224
Assessing changes in degree of pulmonary dysfunction	226
Other tests of function	227
Maximal Respiratory Pressure	227
Diffusing capacity for carbon monoxide	228
Measures of resistance	230
Interrupter Resistance	230
Forced oscillation	230
Plethysmographic airway resistance	231
Multiple breath washout	231
Exhaled nitric oxide	232
Suggested Reading	233
References	233
Chapter 13:Exercise and Lung Function in Child Health and Disease	234
The biologic relevance of exercise in the growing child	234
Physical activity and growth in children—early developmental factors	234
A biologic approach toward exercise testing in children	236
Real patterns of physical activity in children	237
The cardiorespiratory response to exercise	237
Maturation of Cardiorespiratory Responses to Exercise	238
Useful variables of exercise testing in children	242
Alternatives to maximal testing	243
The problem of size	245
Methods of assessing exercise responses in children	245
Safety of exercise testing in children	246
Normal values	247
Exercise-induced asthma and other tests for bronchial reactivity in asthmatic children	247
Exercise and other tests for bronchial reactivity	249
Exercise and exhaled NO	249
Summary	250
References	250
Chapter 14:Integrating Patient-Reported Outcomes into Research and Clinical Practice	251
Definition of a PRO	251
Development and utilization of PROs	251
Health-related quality of life measures	251
Developmental considerations	252
Use of proxy respondents	253
Clinical utility	253
Summary	254
Review of disease-specific respiratory HRQOL measures	255
Asthma Measures	255
Section III:Respiratory Disorders in theNewborn	317
Clinical approach	317
Describe What Is Actually Seen	318
Use Common Language	318
Use a Systematic Approach	318
Keep Clinical and Pathologic Descriptions Separate	318
Epidemiology of congenital malformations of the lung	318
Age-related presentations of congenital lung disease	319
Antenatal Presentation	319
Presentation in the Newborn Period	319
Later Presentation of Congenital Lung Disease	320
Antenatal diagnosis and treatment	320
What Can We Diagnose and When?	320
Congenital Diaphragmatic Hernia	321
CTM Subsequently Diagnosed Pathologically as CCAM	323
CTM Subsequently Diagnosed Pathologically as Bronchopulmonary Sequestration	325
Upper Respiratory Tract Atresia	325
Postnatal features of congenital lung disease: airway and lung parenchyma	327
Congenital Abnormalities of the Upper Airway	327
Management of Neonatal Respiratory Distress	328
Severe Distress at Birth	328
Assessment of the Nonintubated Neonate	328
Assessment of the Intubated Neonate	329
Congenital Abnormalities of the Larynx	329
Laryngeal Atresia	329
Laryngeal Webs	329
Laryngeal Cleft	330
Management of Type 1 Clefts	330
Management of Type 2 and 3 Clefts	330
Management of Type 4 Clefts	331
Laryngomalacia	331
	331
Vocal Cord Paralysis	331
Saccular Cysts and Laryngocoeles	332
Subglottic Hemangioma	332
Subglottic Stenosis	333
Congenital Abnormalities of the Trachea	333
Treatment	334
Congenital Tracheal Stenosis (Complete Tracheal Rings)	334
Bronchial Abnormalities	334
Congenital Bronchial Stenosis and Atresia	334
Abnormal Bronchial Origin and Bronchial Branching	335
Disorders of Bronchial Laterality	335
Other Disorders of the Bronchial Walls	335
Pulmonary Agenesis, Aplasia (Absent Lung), and Hypoplasia (Small Lung)	336
Ectopia	337
Congenital cystic lesions	337
Foregut (Bronchogenic) Cysts	337
Congenital Cystic Adenomatoid Malformation (CCAM)	338
Type 0 CCAM	338
Type 1 CCAM	338
Type 2 CCAM	338
Type 3 CCAM	339
Type 4 CCAM	339
Postnatal Treatment Decisions in Congenital Cystic Lung Disease	340
Prevention of (Nonmalignant) Complications	340
Optimizing Lung Growth	341
Preventing Malignant Transformation	341
Conclusion	341
Pulmonary Sequestration	341
Congenital Large Hyperlucent Lobe (Congenital Lobar Emphysema)	342
Presentation in Infancy	342
Differential Diagnosis	342
Treatment	343
Abnormal connections between the bronchial tree and other structures	343
Tracheoesophageal Fistula and Esophageal Atresia	343
Etiology	343
Pathology	343
Associations	344
Presentation	344
Preoperative Care	345
Surgery	345
Postoperative Course	345
Other Abnormal Connections	345
Congenital disease of the pulmonary arterial tree	345
Disorders of Pulmonary Artery Arrangement	345
Absent or Small Pulmonary Artery	346
Congenital disease of the systemic arterial tree	347
Congenital disease of the pulmonary venous tree	348
Abnormal Pulmonary Venous Drainage	348
Congenital Absence of the Pulmonary Veins	348
Abnormalities of the connections between the pulmonary arterial and venous trees	349
Congenital Alveolar Capillary Dysplasia	349
Pulmonary Arteriovenous Malformations	349
Etiology	349
Diagnosis	349
Treatment	349
Complications	350
Congenital disease of the lymphatic tree	350
Other relevant issues	350
Congenital Disorders of the Chest Wall	350
Congenital Diaphragmatic Hernia	350
Pathologic Anatomy	350
Postnatal Presentation	350
Surgery	351
Outcome	351
Anterior Diaphragmatic Hernia	352
Diaphragmatic Eventration	352
Congenital cardiac disorders	353
Multisystem congenital disorders that affect the lung	353
Computed Tomography	354
Magnetic Resonance Imaging	354
Barium Swallow	354
Esophageal Tube Injection	354
Angiography	354
Bronchoscopy	354
Low-Contrast Volume Bronchography	357
Specific treatment issues	357
Long-term follow-up	357
Summary	357
References	357
Chapter 22:Respiratory Disorders in the Newborn	358
Section IV: Infections of the RespiratoryTract	399
Laboratory Dignosis of Respiratory viral Infections	399
Brief Overview of Viruses Involved in Respiratory Illness	399
Treatment of Clinical Samples	401
Blood Specimen	401
Upper Respiratory Tract Specimen	401
Lower Respiratory Tract Specimens	402
Diagnostic Techniques	402
Virus Cultures	402
Cell Culture	402
Eggs	403
Animals	404
Detection of Whole Viruses by Electron Microscopy	404
Fluid Samples	404
Biopsy Specimens	404
Electron Microscopic Appearance of Respiratory Viruses	404
Other Whole-Virus Detection Methods	405
Antigen Detection	405
Immunoassays	406
Agglutination Assays	408
Serologic Methods	408
Immunoassays	409
Immunofluorescence	409
Enzyme Immunoassays	409
Other Immunoassays	409
Complement Fixation	410
Agglutination Assays	411
Detection of Viral Nucleic Acids	411
Hybridization	411
Polymerase Chain Reaction	411
Real-Time Polymerase Chain Reaction	413
Other Methods	414
Practical Considerations in the Use of Nucleic Acid–Based Techniques	414
Techniques to Diagnose Respiratory Viruses in Clinical Practice	414
Developing Techniques and Future Directions	415
Laboratory diagnosis of respiratory bacterial infections	415
Upper Respiratory Tract Infections	415
Upper Respiratory Tract Specimens	415
Microscopic Examination	416
Culture	416
Lower Respiratory Tract Infections (LRTI)	417
Blood Specimens and Culture	417
Urine Specimens	418
Lower Respiratory Tract Specimens	418
Macroscopic and Microscopic Examination of an LRT Specimen	419
Culture of Lower Respiratory Tract Specimen	419
Qualitative (or Semiquantitative) Culture	419
Uncommon Microbes:	420
Detection of Elastin Fibers in LRT Specimen	421
Antigen Detection	421
Serology	421
Detection of bacterial nucleic acid	423
Chapter 25:Acute Infections that Produce Upper Airway Obstruction	424
Viral laryngotracheobronchitis	424
Etiology	424
Epidemiology	425
Pathophysiology	425
Clinical Presentation and Diagnosis	426
Mild	426
Moderate	426
Severe	426
Recurrent or Spasmodic Croup	427
Non-Infective Causes of Acute Airway Obstruction	427
Who Should Be Evaluated?	427
Management of Viral Laryngotracheobronchitis	428
Supportive Care	428
Humidification	428
Corticosteroids	428
Optimal Route of Administration, Formulation, and Dosing Regimen	428
Epiglottitis	429
Etiology	429
Epidemiology	429
Pathophysiology	430
Clinical Presentation and Diagnosis	430
Management	431
Bacterial tracheitis	431
Section V:Infections of the Respiratory Tract Due to Specific Organisms	489
Chapter 31:Influenza	489
Properties of influenza viruses	489
Epidemiology and immunity	490
Laboratory diagnosis	491
Clinical manifestations	491
Prevention	491
References	492
Chapter 32:Atypical Pneumonias in Children	493
Hantavirus pulmonary syndrome	493
Virology	493
Epidemiology	493
Pathophysiology	493
Clinical Manifestations	493
Laboratory Findings and Diagnosis	494
Treatment	494
Prevention	494
Legionnaire's disease	494
Microbiology	494
Epidemiology	494
Pathology	494
Clinical Manifestations	495
Laboratory Findings and Diagnosis	495
Treatment	495
Prevention	495
Psittacosis	495
Microbiology	496
Epidemiology	496
Pathophysiology	496
Clinical Manifestations	496
Laboratory Findings and Diagnosis	496
Treatment	496
Prevention	496
Chlamydophila pneumoniae	496
Microbiology	497
Epidemiology	497
Pathology	497
Clinical Manifestations	497
Laboratory Findings and Diagnosis	497
Treatment	497
Prevention	498
Mycoplasma pneumoniae	498
Microbiology	498
Epidemiology	498
Pathophysiology	498
Clinical Manifestations	498
Laboratory Findings and Diagnosis	498
Treatment	498
Prevention	499
Inhalational anthrax	499
Microbiology	499
Epidemiology	499
Pathology	500
Clinical Manifestations	500
Laboratory Findings/Diagnosis	500
Treatment	500
Prevention	500
Tularemia	501
Microbiology	501
Epidemiology	501
Pathology	501
Clinical Manifestations	501
Laboratory Findings and Diagnosis	502
Treatment	502
Prevention	502
Q fever	502
Microbiology	502
Epidemiology	502
Pathophysiology	503
Clinical Manifestations	503
Laboratory Findings and Diagnosis	503
Treatment	503
Prevention	503
Pneumonic plague	503
Microbiology	503
Epidemiology	503
Pathophysiology	504
Clinical Manifestations	504
Laboratory Findings and Diagnosis	504
Treatment	504
Prevention	505
References	505
Chapter 33:Tuberculosis and Nontuberculous Mycobacterial Disease	506
Mycobacteriology	506
Immunology	507
Mycobacterium tuberculosis	508
Epidemiology	508
Transmission	508
Incidence and Prevalence	508
TB-HIV Co-infection	510
Clinical Manifestations	510
Pathophysiology	510
Evolution of Clinical Disease in Children	511
Intrathoracic Tuberculosis	512
Extrathoracic Tuberculosis	515
Tuberculosis and HIV	516
Diagnosis	516
Tuberculin Skin Test	516
Interferon-gamma Release Assays	518
Interpretation of Tests for M. tuberculosis Infection	518
Laboratory Diagnosis	519
Staining and Microscopic Examination	519
Culture	520
DNA Methodologies	520
Antibody and Antigen Detection	520
Therapy	520
Latent Tuberculosis Infection	520
Window Prophylaxis	521
Tuberculosis Disease	522
Principles of Treatment	522
Pulmonary Tuberculosis	522
Extrapulmonary Tuberculosis	523
Drug-Resistant Tuberculosis	523
Adjunctive Therapy	523
Follow-up During Antituberculosis Therapy	523
Control and Prevention	523
Bacillus Calmette-Guérin Vaccination	523
Public Health Involvement	524
Pulmonary disease caused by nontuberculous mycobacteria	524
Epidemiology	524
Clinical manifestations	525
Mycobacterium Kansasii	525
Mycobacterium Avium Complex	526
Rapidly Growing Mycobacteria	527
Other Mycobacterial Species	527
Mycobacterial Infection in Cystic Fibrosis	527
Diagnosis	528
Treatment	528
Prevention	530
Suggested reading	530
References	530
Chapter 34:The Mycoses	531
Endemic mycoses	531
Histoplasmosis	531
Histoplasmosis—The Microbe	531
Epidemiology	531
Pathogenesis	531
Clinical Manifestations	531
Pulmonary Histoplasmosis	531
Acute Disseminated Histoplasmosis	532
Fibrosing Mediastinitis	533
Chronic Disseminated Histoplasmosis	533
Rarer Clinical Presentations	533
Diagnosis	534
Treatment	534
Coccidioidomycosis	534
Blastomycosis	535
Opportunistic pulmonary fungal infections	536
Aspergillosis—Invasive Pulmonary Disease	536
Aspergillus—The Microbe	536
Immunity	536
Predisposing Factors	537
Clinical Presentation	538
Diagnosis	538
Treatment and Prognosis	539
Cryptococcosis	540
Cryptococcosis—The Microbe	540
Immunity	540
Clinical Manifestations	540
Diagnosis	541
Treatment	541
Candidiasis	542
Zygomycosis	542
Other uncommon pulmonary mycoses	543
Acknowledgments	544
References	544
Chapter 35:Pertussis and Other Bordetella Infections of the Respiratory Tract	545
Microbiology, pathogenesis, and immunity	545
Epidemiology	545
Clinical characteristics	548
Diagnosis and differential diagnosis	548
Treatment	550
Prevention	551
Suggested Reading	551
References	551
Chapter 36:Toxocariasis, Hydatid Disease of the Lung, Strongyloidiasis, and Pulmonary Paragonimiasis	552
Toxocariasis (Visceral Larvamigrans)	552
Etiology	552
Epidemiology	552
Pathogenesis	552
Clinical Manifestations	552
Complications	553
Diagnosis	553
Treatment	554
Prevention	554
Hydatid disease of the lung (pulmonary hydatidosis)	554
Cystic Hydatid Disease	554
Clinical Features	554
Diagnosis	555
Treatment	557
Prevention and Follow-up	558
Sylvatic Alaskan-Canadian Variant	558
Alveolar Hydatid Disease	559
Polycystic Hydatid Cyst	560
Strongyloidiasis	560
Pulmonary paragonimiasis (lung fluke disease)	561
Suggested Reading	562
References	563
Section VI:Noninfectious Disorders of the Respiratory Tract	564
Chapter 37:Atelectasis	564
What is atelectasis?	564
Why does atelectasis occur?	565
Classification of Atelectasis	565
Surfactant Inibition	565
Gas Resorption	565
Compression Atelectasis	565
Consequences of Atelectasis	566
Impaired Gas Exchange	566
Impaired Lung Mechanics	566
Increased Pulmonary Vascular Resistance	566
Worsening of Lung Injury	567
Diagnosis	567
Clinical Features	567
Radiological Features	567
Factors Modifying the Development of Atelectasis	567
Posture	567
Developmental Influences	568
Supplemental Oxygen	568
Pre-existing Lung Disease	568
Treatment of Atelectasis	568
Recruitment Maneuvers	568
Chest Physiotherapy	569
Removal of Endobronchial Obstruction	569
Summary	569
References	569
Chapter 38:Pulmonary Edema	570
Anatomic considerations	570
Factors responsible for fluid movement	571
Vascular Forces	572
Interstitial Forces	572
Microvascular Filtration Coefficient and Vascular Permeability	572
Lymphatic Clearance	573
Surface Tension	573
Safety Factors That Oppose Edema Formation	573
Mechanisms that cause pulmonary edema	573
Increased Hydrostatic Pressure (Pmv) in the Pulmonary Microvasculature	573
Decreased Plasma Colloid Osmotic Pressure ( p mv)	573
Decreased Interstitial Hydrostatic Pressure (Ppmv)	573
Increased Pulmonary Vascular Surface Area	574
Increased Vascular Permeability in Fluid-Exchanging Vessels	574
Clearance of pulmonary edema fluid	574
Pathophysiologic consequences of edema	575
Clinical presentation	576
Physical Examination	576
Pulmonary Function Tests	576
Imaging Studies	576
The Chest Radiograph	577
Computed Tomography (CT) and Magnetic Resonance Imaging (MRI)	577
Distinguishing High Pressure from Low Pressure Pulmonary Edema	578
Quantitation of Pulmonary Edema in Patients	579
Clinical disorders causing pulmonary edema	579
High-Pressure Pulmonary Edema	579
Airway Obstruction	579
Reexpansion Pulmonary Edema	580
Neonatal Respiratory Distress Syndrome	580
Neurogenic Pulmonary Edema	580
Acute Lung Injury and the Acute Respiratory Distress Syndrome	581
High-Altitude Pulmonary Edema	581
Inhalation of Toxic Agents	581
Intravenous Agents	582
Narcotic-Induced and Medication-Induced Pulmonary Edema	582
Therapy	582
Reversing the Hypoxemia	582
Reduce the Rate of Fluid Filtration	583
Minimize Treatment-Related Lung Damage	583
Augment the Rate of Clearance of Airspace Fluid	584
Suggested Reading	585
References	585
Chapter 39:Acute Respiratory Distress Syndrome	586
Definition and diagnosis	586
Pathophysiology	586
Pulmonary (direct) versus nonpulmonary (indirect) ALI/ARDS	587
Epidemiology and Outcome	588
Severity Score	588
Genetic Modifiers of ALI/ARDS	588
Differences Between Children and Adults	589
Treatment	589
Conventional Mechanical Ventilation	589
Other Modalities of Ventilation	590
Noninvasive Ventilation	590
High Frequency Oscillatory Ventilation (HFOV)	590
Airway Pressure Release Ventilation (APRV)	590
Neurally Adjusted Ventilatory Assist (NAVA)	590
Adjuvants to Mechanical Ventilation	591
Prone Positioning	591
Inhaled Nitric Oxide (iNO)	591
Surfactant	591
Corticosteroids	591
Neuromuscular Blocking Agents	592
Beta-Adrenergic Agonists	592
Extracorporeal Life Support (ECLS) in Pediatric ARDS	592
Tracheostomy	592
References	592
Chapter 40:Lung Injury from Hydrocarbon Aspiration and Smoke Inhalation	593
Lung injury from hydrocarbon aspiration	593
Pathology	593
Pathophysiology	593
Clinical findings	594
Management	594
Hydrocarbon “sniffing”	595
Respiratory complications of smoke inhalation	595
Pathogenesis	595
Carbon monoxide poisoning	596
Pathology	596
Pathophysiology	597
Clinical findings	597
Treatment	598
The relationship of pulmonary injury from smoke inhalation to the pulmonary complications of surface burns	599
Suggested Reading	599
References	599
Chapter 41:Drowning	600
Drowning	600
Epidemiology	600
Drowning Sequence	600
Sequelae of submersion/immersion events	600
Pulmonary Injury	600
Nonpulmonary Sequelae	601
Hypothermia	601
Electrolyte Imbalances	601
Trauma	602
Hypoxic-Ischemic Damage	602
Management of Pulmonary Injury	602
Outcome of Pulmonary Injury	602
Outcome Prediction of Neurologic Injury	602
Prevention of Drowning	603
References	604
Chapter 42:Tumors of the Chest	605
Diagnosis of Pulmonary or Mediastinal Tumor	605
Pulmonary tumors	607
Benign Pulmonary Tumors	608
Hamartoma	608
Mesodermal Tumors	609
Benign Parenchymal Tumors	609
Bronchial Adenoma	609
Tracheal Tumors	610
Papilloma of the Trachea and Bronchi	610
Fibroma of the Trachea	611
Hemangioma of the Trachea	611
Leiomyoma of the Lung	611
Neurogenic Tumors	612
Malignant Pulmonary Tumors	612
Bronchogenic Carcinoma	612
Fibrosarcoma of the Bronchus	612
Leiomyosarcoma	612
Multiple Myeloma	612
Chorioepithelioma	612
Systemic Neoplasms Affecting the Lung	612
Metastatic Pulmonary Tumors	612
Mediastinal tumors	613
Signs and Symptoms of Mediastinal Tumor	613
Respiratory Symptoms	613
Gastrointestinal Symptoms	614
Neurologic Symptoms	614
Vascular Symptoms	614
Miscellaneous Symptoms	614
Physical Findings	614
Diagnostic Procedures	614
Primary Mediastinal Cysts	614
Bronchogenic Cysts	615
Esophageal Cysts (Duplication)	615
Gastroenteric Cysts	616
Pericardial Coelomic Cysts	619
Intrathoracic Meningoceles	619
Tumors of the Thymus	620
Hyperplasia of the Thymus	620
Neoplasm of the Thymus	620
Benign Thymoma	620
Thymic Cysts	620
Teratoma of the Thymus	621
Tuberculosis of the Thymus Gland	621
Teratoid Mediastinal Tumors	621
Benign Cystic Teratoma	621
Benign Solid Teratoid Tumors and Malignant Teratoid Tumors	623
Neurogenic Mediastinal Tumors	623
Mediastinal Lymph Node Abnormalities	625
Inflammatory Disorders	626
Vascular-Lymphatic Abnormalities of the Mediastinum	626
Mediastinal Lipoma, Liposarcoma, and Lipoblastoma	627
Thyroid disorders	627
Primary cardiac and pericardial tumors	627
Tumors of the diaphragm	628
Primary tumors of the chest wall	628
Suggested Reading	629
General	629
Benign Pulmonary Tumors	629
Primary and Metastatic Malignant Pulmonary Tumors	629
Mediastinal Tumors	629
Cardiac Tumors	630
Diaphragm and Chest Wall Tumors	630
Chapter 43:Chest Wall and Respiratory Muscle Disorders	631
General considerations	631
Respiratory Muscle Fatigue	631
Assessment of Respiratory Function in Children with Chest Wall Dysfunction	631
Management of Children with Chest Wall Dysfunction	632
Causes of Chest Wall Dysfunction	633
Diseases of the motor neurons	634
Spinal Cord Injury and Cerebral Palsy	634
Spinal Cord Injury	634
Cerebral Palsy	634
Spinal Muscular Atrophies	634
Perspectives for Drug Therapy in SMA Patients	635
SMA with Respiratory Distress Type 1	635
Poliomyelitis	635
Diaphragmatic Paralysis	635
Guillain-Barré Syndrome	636
Muscle diseases that affect the chest wall	636
Muscular Dystrophies	636
Duchenne Muscular Dystrophy	636
Long-Term Management of Respiratory Disability	637
Management of Acute Respiratory Deteriorations	637
Corticosteroid Therapy	637
Future Perspectives on Duchenne Muscular Dystrophy Treatment	639
Myotonic Dystrophy	639
Congenital Myotonic Dystrophy	639
Classic Myotonic Dystrophy	639
Congenital Muscular Dystrophy	639
Other Heritable Myopathies	639
Neuromuscular junction diseases	640
Myasthenia Gravis and Congenital Myasthenic Syndromes	640
Juvenile Myasthenia Gravis	640
Transient Neonatal Myasthenia	640
Congenital Myasthenic Syndromes	640
Botulism	641
Skeletal anomalies	641
Scoliosis	641
Classification and Natural History	641
Pulmonary Function in Scoliosis	642
Lung Function	642
Lung Growth	642
Management	643
Adolescent Idiopathic Scoliosis	643
Juvenile Scoliosis	643
Congenital and Infantile Scoliosis	643
Spine Deformities in Neuromuscular Diseases	643
Postoperative Pulmonary Function in Scoliosis	644
Hypoplastic Thorax Syndromes	644
Miscellaneous disorders that cause chest wall dysfunction	645
The Chest Wall in Obstructive Pulmonary Disease	645
Obesity	646
The Chest Wall of the Newborn	646
Acknowledgments	646
References	646
Section VII:Asthma	647
Chapter 44:The Epidemiology of Asthma	647
Definition And Measurement In Epidemiologic Studies	647
Definitions	647
Study Designs	648
The Prevalence Of Asthma	648
Variation of Prevalence Between Countries and Regions	648
Differences Between Rural and Urban Settings	649
Changes with Migration	650
Change of Prevalence with Time	650
Prevalence by Age, Gender, and Ethnicity	651
Age	651
Gender	651
Ethnicity	651
The Burden Of Asthma	652
Sleep Disturbance, School Absenteeism, and Quality of Life	652
Hospitalization	653
Trends Over Time in Asthma Hospitalizations: 1960 to 1990	654
Trends Over Time in Asthma Hospitalizations: 1990 to 2007	654
Risk and Protective Factors for Hospital Admission	654
Prehospital Care	654
Socioeconomics/Poverty and Ethnicity	654
Climate and Environmental Exposures	654
Age, Gender, and Obesity	655
Disease Severity	655
Viral Infection	655
Risk Factors for Admission to Intensive Care	655
Mortality	655
Trends in Asthma Mortality Over Time	656
Mortality Risk Factors	657
Genetic, Ethnic, and Socioeconomic Determinants	657
Season	657
Asthma Severity, Setting, and Psychological Factors	658
Disability-Adjusted Life Years Lost	658
Economic Burden	658
Etiology or “Predisposing, Precipitating, Perpetuating, and Protective” Factors	658
The Genetic Determinants of Asthma	659
The Relationship Between Asthma, Atopy, and Allergy	659
Atopy	659
Allergens	660
Birth Weight, Growth, Physical Activity, and Obesity	661
Birth Weight and Growth	661
Physical Activity	662
Obesity	662
Underweight	663
Diet	663
Fish	663
Vegetables, Fruit, and Antioxidants	663
Cereals and Rice	664
Polyunsaturated Fat	664
Trans Fatty Acids	664
Vitamin D	664
Chemicals and Trace Elements	664
Food Preservatives and Additives	665
Mediterranean Diet	665
Breast Feeding	665
Maternal Diet in Pregnancy	665
Toxins And Pollution	666
Environmental Tobacco Smoke	666
Outdoor Air Pollution	667
Infection, Immunization, and Microbial Exposure	668
Infection	668
Immunization	669
Other Microbial Exposure	670
Housing, Animals, and Climate	671
Damp and Mold	671
Animals	671
Other Indoor Factors	672
Climate and Weather	672
Economic Factors	673
Natural History	674
Preschool Asthma	674
School-Age Asthma and Beyond	674
Conclusion	676
Suggested Reading	676
References	676
Chapter 45:The Immunopathogenesis of Asthma	677
The relationship between allergens, allergen sensitization, and asthma	677
The allergens associated with asthma	677
Allergen proteins	679
Airborne particles carrying foreign proteins, relevance to exposure, and deposition in the chest	679
The paradoxical effects of cat ownership	681
Relevance of different allergens to total serum ige and the associated risk for acute asthma	681
The interaction between viral infection and allergic responses in children with acute episodes of asthma	682
Mechanisms of inflammation in the respiratory tract	683
Relationship between immune responses, inflammation, and symptoms	683
T cell peptide responses	684
Treatment with monoclonal antibodies to immunoglobulin e	684
A modified th2 response to cat allergen is not associated with symptoms of asthma or lung inflammation	684
Summary	685
References	685
Chapter 46:Asthma in the Preschool-Age Child	686
Asthma in the preschool-age child	686
What Is Asthma?	686
The epidemiology of early childhood asthma	686
When Does Asthma Start?	686
Who Gets Asthma?	687
The pathophysiology of early childhood asthma	688
The natural history of early childhood asthma	688
Diagnosis and assessment	690
Clinical Presentation	690
Wheezing and Cough: When Is It Asthma, and When Is It Not?	691
The Clinical Patterns of Asthma	692
Evaluation of the Preschool Child with Asthma	692
Recognizing an Exacerbation of Asthma	693
Treatment	693
Intervention Measures	694
Maintenance Therapy	695
Limitation of Maintenance Medication	696
Environmental Aspects of Treatment	696
Immunotherapy (Allergy Shots) for Environmental Aeroallergens	697
Family Education	697
Summary	697
Suggested Reading	698
References	698
Chapter 47: Wheezing in Older Children: Asthma	699
Introduction	699
Pathology	699
Pathophysiology	700
Inflammatory Cell Biology and Asthma Etiology and Pathophysiology	701
Natural History and Prognosis	702
Asthma Mortality	703
Diagnosis of Asthma	703
Physical Examination	704
Asthma Triggers	705
Allergens	705
Irritants	705
Weather Changes	705
Infections	705
Exercise	706
Emotional Factors	706
Gastroesophageal Reflux	706
Allergic Rhinitis and Sinusitis	706
Nonallergic Hypersensitivity to Drugs and Chemicals	706
Endocrine Factors	707
Nocturnal Asthma	707
Laboratory Diagnosis	707
Lung Function Tests	707
Bronchial Challenge Tests	708
Exercise Challenge Test	709
Other Tests	709
Complete Blood Cell Count	709
Cytologic Examination of Sputum	709
Exhaled Nitric Oxide	709
Serum Tests	710
Sweat Test	710
Radiographs	710
Allergy Testing	710
Therapeutic Considerations	710
Classification of Asthma	712
Intermittent Asthma	712
Mild Persistent Asthma	712
Moderate Persistent Asthma	713
Severe Persistent Asthma	713
Other Measures for Assessing Asthma Severity and Control	714
Pharmacologic Management of Asthma in Children Older than 5 Years of Age	714
Reliever Medications: Short-Acting b Agonists	715
Anticholinergic Agents	717
Controller Medications	717
Inhaled Corticosteroids	717
Fluticasone Propionate	717
Budesonide	718
Beclomethasone	718
Mometasone	718
Ciclesonide	719
Other Inhaled Corticosteroids	719
Mechanism of Action and Clinical Use	719
Systemic Corticosteroids	720
Long-acting b Agonists	720
Leukotriene Antagonists	722
Other Drugs	723
Methylxanthines: Theophylline	723
Cromolyn Sodium and Nedocromil	724
Management of Chronic Asthma	725
Intermittent Asthma	726
Mild Persistent Asthma	726
Moderate Persistent Asthma	727
Severe Persistent Asthma	728
Holding Chambers and Spacer Devices	728
Peak Expiratory Flow Rate Monitoring	729
Nonpharmacologic Measures	731
Management of an Acute Episode	731
Hospital Management of Asthma	733
General Treatment	733
Suggested Reading	735
References	735
Chapter 48:Severe Asthma	736
Pediatric and adult severe asthma: similarities and differences	736
The initial label: problematic severe asthma	736
Group 1: Wrong Diagnosis (Not Asthma)	737
Group 2: Co-Morbidities (Asthma Plus)	737
Group 3: The Real Deal, But What Is the Deal?	737
The next evaluation: does the child have difficult or severe therapy-resistant asthma?	737
Adherence and Other Medication Issues	737
Cigarette Smoke Exposure	738
Allergen Exposure	738
Psychosocial Issues	738
Multidisciplinary Team Assessment	738
The final evaluation: discordant phenotypes, pattern of airway inflammation, steroid responsiveness, and persistent air flo ...	738
Assessment of Phenotype Discordance and the Pattern of Airway Inflammation	739
Assessment of Steroid Responsiveness	739
Does the Child Have PAL?	740
Options for phenotype therapy	740
Persistent Air Flow Limitation	740
Steroid-Sensitive and Steroid-Resistant Eosinophilic Inflammation	740
Omalizumab	740
Immunomodulatory Agents	740
Gold Salts	740
Section VIII:Cystic Fibrosis	753
Chapter 50:Genetics and Pathophysiology of Cystic Fibrosis	753
CFTR gene	753
Structure	753
Splicing	754
Mutations	754
CFTR Protein	755
Characteristics	755
Biogenesis	755
Function	755
Cellular Distribution and Function	755
Developmental Expression and Function	755
Postnatal Expression and Function	756
Tissue Distribution	756
Genotype-Phenotype Correlations	756
Molecular Consequences of Mutations	756
Clinical Consequences of Mutations	757
Pathophysiology	758
Airways Dehydration and Disruption of Mucociliary Clearance	758
Characteristics of systemic disease	759
Airways, Upper and Lower	759
Inflammation	760
Infection	760
Gastrointestinal Disease	761
Sweat Gland Effects	761
Reproductive Tissues	761
Disorders Related to the Cystic Fibrosis Transmembrane Conductance Regulator	761
Congenital Bilateral Absence of the Vas Deferens	761
Pancreatitis	762
Sinusitis	762
References	762
Chapter 51:Diagnosis and Presentation of Cystic Fibrosis	763
Diagnostic criteria for cystic fibrosis	763
Making the diagnosis of CF	763
Clinical Suspicion	763
The Sweat Test	764
Mutation Analysis	765
Assessment for End Organ Involvement	766
Transepithelial Potential Difference Measurements	766
Antenatal testing for CF	767
Neonatal screening for cystic fibrosis	767
Genotype-phenotype correlations	768
Cystic fibrosis phenotypes	769
Classic CF	769
Atypical CF (with Symptoms)	769
Atypical CF (Without Symptoms)	769
Suggested Reading	769
References	769
Chapter 52:Pulmonary Disease in Cystic Fibrosis	770
Pathogenesis of lung disease	770
Clinical manifestations and complications	772
Prognosis	773
Evaluations	774
Treatment	775
Airway Clearance	775
Inhaled Mucolytics	776
Antibiotic Therapy in Cystic Fibrosis	776
Anti-inflammatory Therapy in Cystic Fibrosis	777
Preventative Care	778
Lung Transplantation in Cystic Fibrosis	778
Emerging Therapies in Cystic Fibrosis	779
Conclusions	780
Suggested Reading	780
References	780
Chapter 53:Nonpulmonary Manifestations of Cystic Fibrosis	781
Pancreatic disease	781
Pathobiology	781
Pancreatic Phenotype	781
Genotype-Phenotype Correlations	781
Clinical Presentation	782
Pancreatic Insufficiency	782
Pancreatic Sufficiency	782
Assessment of Pancreatic Function	782
Management of Pancreatic Insufficiency	783
Complications of Therapy	784
Fibrosing Colonopathy	784
Allergy	784
Cystic fibrosis–related diabetes mellitus (CFRD)	784
Pathobiology	784
Diagnosis	785
Management	785
Intestinal disease	786
Pathobiology	786
Clinical Manifestations of Intestinal Disease	786
CFTR-related Intestinal Diseases	786
Meconium Ileus	786
Distal Intestinal Obstruction Syndrome	787
Constipation	788
Rectal Prolapse	788
Appendicitis	788
Intussusception	788
Secondary Intestinal Complications	788
Pseudomembranous Colitis	788
Gastroesophageal Reflux Disease	788
Associated Conditions	789
Inflammatory Bowel Disease (IBD)	789
Gastrointestinal Malignancies	789
Hepatobiliary disease	789
Pathobiology	789
Clinical Presentation	789
Disease Manifestations	790
Extrahepatic Complications	790
Microgallbladder	790
Cholelithiasis	790
Abnormalities of the Biliary Tree	791
Cholangiocarcinoma	791
Intrahepatic Complications	791
Neonatal Cholestasis	791
Hepatic Steatosis	791
Focal Biliary Cirrhosis	791
Multilobular Biliary Cirrhosis	792
Management of Liver Disease	792
Pharmacologic Interventions	792
Nutrition	792
Portal Hypertension and Hypersplenism	792
Liver Failure	793
Nutrition	793
Pathobiology	793
Nutritional Management	794
Vitamin Supplementation	794
Single organ manifestations of cftr dysfunction	795
Male Infertility	795
CFTR and Pancreatitis	795
Conclusion	795
References	795
Section IX: Child	796
Chapter 54:New Concepts in Children's Interstitial Lung Disease and Diffuse Lung Disease	796
New concepts, terminology, and classification	796
General diagnostic approach	797
Resources for families and physicians	799
Suggested Reading	799
References	799
Chapter 55:Childhood Interstitial Lung Disease Disorders More Prevalent in Infancy	800
Alveolar capillary dysplasia with misalignment of pulmonary veins	800
Clinical Presentation	800
Radiographic Findings	800
Histologic Findings	800
Epidemiology	801
Etiology and Pathogenesis	801
Diagnostic Approach	802
Differential Diagnosis	802
Treatment and Prognosis	802
Lung growth abnormalities presenting as childhood ild	802
Clinical Presentation	802
Radiographic Findings	802
Histologic Findings	802
Epidemiology	804
Etiology and Pathogenesis	804
Diagnostic Approach	804
Differential Diagnosis	804
Treatment and Prognosis	804
Pulmonary interstitial glycogenosis	804
Clinical Presentation	804
Radiographic Findings	805
Histologic Findings	805
Epidemiology	805
Etiology and Pathogenesis	805
Diagnostic Approach	805
Differential Diagnosis	805
Treatment and Prognosis	805
Neuroendocrine cell hyperplasia of infancy	807
Clinical Presentation	807
Radiographic Findings	807
Histologic Findings	807
Epidemiology	808
Etiology and Pathogenesis	808
Diagnostic Approach	808
Differential Diagnosis	808
Treatment and Prognosis	808
Summary	809
Suggested Reading	809
References	809
Chapter 56:Lung Diseases Associated with Disruption of Pulmonary Surfactant Homeostasis	810
Overview of surfactant composition and metabolism	810
Disorders of surfactant production	812
Surfactant Protein B (SP-B) and Hereditary SP-B Deficiency	812
Surfactant Protein C Genetic Abnormalities and Lung Disease	812
ABCA3 Deficiency	815
NKX2.1 Haploinsufficiency	816
Disorders of surfactant clearance	817
Pathogenesis	817
Nomenclature	817
Autoimmune PAP	818
PAP Caused by Autosomal Recessive CSF2RA Mutations	818
PAP Caused by Autosomal Recessive CSF2RB Mutations	818
Diagnostic approach	819
Genetic Testing	819
Lung Histopathology	820
Lung and Serum Biomarkers	820
Summary	820
Acknowledgements	821
References	821
Chapter 57:Pulmonary Involvement in the Systemic Inflammatory Diseases of Childhood	822
Juvenile idiopathic arthritis (JIA)	822
Epidemiology	822
Etiology and Pathogenesis	822
Clinical Manifestations	823
Classification and Diagnosis of JIA	824
Pulmonary Involvement in JIA	824
Treatment	826
Prognosis	826
Systemic lupus erythematosus (SLE)	826
Epidemiology	826
Pathogenesis	827
Clinical Features	827
Pulmonary Involvement	827
Treatment	829
Prognosis	829
Juvenile dermatomyositis	829
Epidemiology	829
Pathogenesis	829
Clinical Manifestations	830
Diagnosis	830
Pulmonary Involvement	830
Treatment	831
Prognosis	831
Scleroderma (SSc)	831
Epidemiology	831
Clinical Features	832
Pathogenesis	833
Pulmonary Involvement	833
Treatment	834
Prognosis	834
Mixed connective tissue disease (MCTD)	834
Epidemiology and Pathogenesis	834
Clinical Manifestations and Diagnosis	834
Pulmonary Involvement	835
Treatment and Prognosis	835
Sarcoidosis	835
Epidemiology	836
Etiology and Pathogenesis	836
Clinical Manifestations	836
Pulmonary Involvement	837
Diagnosis	837
Treatment	838
Prognosis	839
Childhood vasculitides	839
Granulomatosis with Polyangitis (GP)	840
Epidemiology	840
Pathogenesis	841
Clinical Manifestations	841
Pulmonary Involvement	841
Diagnosis	842
Treatment	843
Prognosis	844
Other systemic inflammatory diseases with significant pulmonary involvement	844
Sjögren's Syndrome	844
Inflammatory Bowel Disease	845
Clinical approach to diagnosis and management of pulmonary involvement in the systemic inflammatory diseases of childhood	845
Suggested Reading	847
References	847
Chapter 58: Diffuse Alveolar Hemorrhage in Children	848
Etiology of Diffuse Alveolar Hemorrhage	848
Immune-Mediated Alveolar Hemorrhage	848
Pathophysiology	848
Pulmonary-Renal Syndromes	850
Wegener's Granulomatosis	850
Microscopic Polyangiitis	852
Goodpasture's Syndrome	853
Isolated Pulmonary Capillaritis	853
Non-Immune-Mediated Alveolar Hemorrhage	854
Idiopathic Pulmonary Hemosiderosis	854
Acute Idiopathic Pulmonary Hemorrhage of Infancy	854
Approach to the Child with Pulmonary Hemorrhage	855
Diagnosis	855
Treatment	856
Summary	857
References	857
Suggested Reading	857
Chapter 59:Environmental Exposures in the Normal Host	858
Pathogenesis	858
Type III and Type IV Hypersensitivity Responses	858
Cells, Cytokines, and Other Pulmonary Factors	859
Susceptibility Factors	859
Clinical Manifestations	860
Acute Stage	860
Subacute Stage	860
Chronic Stage	860
Immunologic Studies	860
Radiologic Findings	861
Pulmonary Function Testing and Bronchial Challenge	862
Bronchoalveolar Lavage and Lung Biopsy	862
Etiology	862
Bird Fancier's Lung	863
Other Environmental Exposures	863
Therapeutic Considerations and Prognosis	863
Eosinophilic pulmonary diseases	864
Biology of Eosinophils	864
Drug-Induced Eosinophilia	864
Helminth-Associated Eosinophilic Lung Diseases	865
Transpulmonary Passage of Helminth Larvae	865
Hookworms	866
Hematogenous Seeding with Helminth Larvae	866
Allergic Bronchopulmonary Aspergillosis (ABPA)	867
Pathology	867
Pathogenesis	867
Clinical Manifestations	868
Differential Diagnosis	868
Clinical Staging	868
Radiographic Findings	869
Laboratory Investigations	869
Therapy	869
Prognosis	869
Acute Eosinophilic Pneumonia	870
Chronic Eosinophilic Pneumonia	871
Eosinophilic Granuloma	871
Churg-Strauss Syndrome—Allergic Angiitis and Granulomatosis	871
Hypereosinophilic Syndrome	872
Bronchiolitis obliterans	873
References	876
Chapter 60:Rare Childhood Lung Disorders	877
Introduction	877
Respiratory Disorders of the Lymphatic System	877
Pulmonary Lymphangiectasia	877
Lymphangiomatosis and Gorham-Stout Disease	878
Lymphangioleiomyomatosis	879
Pulmonary Alveolar Microlithiasis	879
Gaucher Disease	881
Niemann-Pick Disease Type B	881
Neurofibromatosis	881
Dyskeratosis Congenita	882
Hermansky-Pudlak Syndrome	882
Alpha-1 Antitrypsin Deficiency	883
Genetics and Pathophysiology	883
Epidemiology and Clinical Presentation	884
Diagnosis	884
Natural Course and Treatment	884
Behçet Disease	885
Summary	885
Suggested Reading	885
Selected Foundations	885
References	885
Section X:Disorders of the Immunocompromised Child	886
Chapter 61:Primary Immunodeficiency: Chronic Granulomatous Disease and Common Variable Immunodeficiency Disorders	886
Chronic granulomatous disease	886
Clinical features	886
Pulmonary complications	888
Laboratory findings and diagnosis	890
NADPH oxidase	890
Molecular defects and inheritance	891
Management	894
Hematopoietic cell transplantation and gene therapy	894
Common variable immunodeficiency disorders	895
Clinical features	895
Pulmonary complications	897
Laboratory findings and diagnosis	897
Molecular defects and inheritance	898
Management	898
Suggested Reading	898
References	898
Chapter 62:Pulmonary Disease In The Pediatric Patient With Acquired Immunodeficiency States	899
Introduction	899
Pulmonary Infections In The Immunocompromised Pediatric Host	899
Clinical Presentation Of Pulmonary Infection In The Immunocompromised Child	899
Infectious Risks Shared By Malignancy, Solid-Organ Transplantation, And Hematopoietic Stem Cell Transplantation	900
Common Pulmonary Infectious Agents In The Immunocompromised Pediatric Host	900
Viral Pathogens	900
Cytomegalovirus	900
Respiratory Syncytial Virus and Other Common Respiratory Viruses	902
Varicella-Zoster Virus and Herpes Simplex Virus	902
Herpesvirus Type 6	902
Human Metapneumovirus	902
Adenovirus	903
Fungal Pathogens (See also Chapter 34)	903
Pneumocystis Jirovecii (Formerly P. Carinii)	903
Aspergillus Species	904
Mucor and Rhizopus	904
Candida Species	905
Histoplasmosis and Blastomycosis	905
Cryptococcus Neoformans	905
Rarer Fungal Pneumonias	905
Bacterial Pathogens	906
Mycobacteria	906
Legionella pneumophila	906
Parasitic Agents (See also Chapter 36)	906
Toxoplasma Gondii and Cryptosporidium Parvum	906
Pulmonary Co-Infections	907
Non-Infectious Pulmonary Complications In The Immunocompromised Pediatric Host	907
Pulmonary Complications Of Childhood Tumors And Their Treatment	907
Pulmonary Complications Following Solid-Organ Transplantation	908
Pulmonary Edema, Pleural Effusions, and ARDS	908
Impairment of Respiratory Mechanics	909
Medication Toxicity	909
Posttransplant Lymphoproliferative Disease (PTLD)	909
Other Noninfectious Pulmonary Complications of Solid-Organ Transplantation	911
Pulmonary Complications Of Hematopoietic Stem Cell Transplantation	911
Pretransplant Factors	911
Early Noninfectious Posttransplant Complications (See also Figure 62-6)	912
Oral and Perioral Complications	913
Pulmonary Edema and Capillary Leak Syndrome	913
Peri-Engraftment Respiratory Distress Syndrome	913
Idiopathic Pneumonia Syndrome	913
Diffuse Alveolar Hemorrhage (DAH)	914
Pulmonary and Hepatic Veno-Occlcusive Disease (VOD)	914
Pulmonary Function Following HSCT	914
Late Noninfectious Posttransplant Complications	914
Bronchiolitis Obliterans	915
Interstitial Lung Diseases	916
Posttransplant Lymphoproliferative Disorder	916
Pulmonary Alveolar Proteinosis	916
Respiratory Failure	916
Diagnostic Approach To Pulmonary Disease In The Immunocompromised Pediatric Host	917
Radiographic Findings	917
Noninvasive Diagnostic Studies	917
Invasive Diagnostic Studies	918
Flexible Bronchoscopy and Bronchoalveolar Lavage	918
Transthoracic Needle Aspiration Biopsy	918
Open-Lung Biopsy	919
Suggested Reading	919
References	919
Chapter 63: Respiratory Disorders in Pediatric HIV Infection	920
Etiology and Pathogenesis	920
Definition	920
Epidemiology	921
Diagnosis	921
Natural History of HIV-1 Infection in Children	922
HIV and Lung Defense	922
Pulmonary Disorders	922
Bacterial Pneumonia	923
Tuberculosis	924
Mycobacterium Avium Complex	926
Viral Infection	926
Pneumocystis Jirovecii Pneumonia	927
Fungal Infections	929
Chronic Lung Disease	929
Lymphoid Interstitial Pneumonitis/Pulmonary Lymphoid Hyperplasia	929
Chronic Infection	930
Bronchiectasis	931
Immune Reconstitution Inflammatory Syndrome	931
Diffuse Alveolar Damage	932
Pulmonary Tumors	932
Upper Airway Disease	932
Approach to the HIV-1–Infected Patient with Respiratory Symptoms	932
Summary	932
Suggested Reading	933
References	933
Chapter 64:Pediatric Lung Transplantation	934
Indications and timing	934
Contraindications	935
Surgical technique	936
Posttransplant management	936
Immunosuppressive Regimen	936
Antimicrobial Regimen	937
Management Issues Unique to Pediatrics	937
Complications	937
Immediate Posttransplant Phase	937
Early Phase (1 to 6 Months)	939
Infection	939
Rejection	940
Medication Side Effects	940
Late Phase (More than 6 Months)	940
Posttransplant Lymphoproliferative Disease	940
Bronchiolitis Obliterans and Bronchiolitis Obliterans Syndrome	941
Outcomes	941
Survival	941
Transplant Benefit, Functional Outcome, and Quality of Life	942
Growth	943
Causes of Death	943
Future Directions	943
Suggested Reading	944
References	944
Section XI:Aerodigestive Disease	945
Chapter 65:The Aerodigestive Model	945
Patient overview	945
The Interdisciplinary Team	945
Screening and Evaluating Potential Patients	946
Summary	946
Chapter 66:Aspiration	947
Aspiration caused by swallowing dysfunction	947
Development of Swallowing	948
Mechanisms of Aspiration During Swallowing	948
Swallowing Dysfunction in Specific Populations	949
Evaluation of swallowing	950
Radiographic Evaluation of Swallowing	950
Endoscopic Evaluation of Swallowing	951
Role of Radiographic and Endoscopic Tests in the Evaluation of Aspiration	951
Aspiration of gastroesophageal reflux	952
Evaluation of Reflux Aspiration	952
Lipid-Laden Macrophage Index	952
Other Biomarkers of Reflux Aspiration	952
Treatment Considerations for Reflux Aspiration	953
Aspiration of oral secretions	953
Evaluation of Salivary Aspiration	954
Treatment of Salivary Aspiration	954
Evaluation of lung injury caused by aspiration	954
Multidisciplinary approach to chronic aspiration	955
Evaluation of the Aerodigestive Tract	955
Suggested Reading	956
References	956
Chapter 67:Feeding, Swallowing, and Voice Disorders	957
Anatomy, physiology, and neural control pertinent to feeding, swallowing, and phonation	957
Feeding Skill Development	958
Phases of Swallowing	958
Conditions associated with feeding, swallowing, and airway protection problems	959
Specific Airway Conditions and the Effect on Feeding, Swallowing, and Voice	960
Evaluation and management	960
Instrumental Assessment of Swallowing Function and Airway Protection	960
Additional Evaluations	962
Management Strategies	962
Voice	962
Pediatric Voice Assessment	963
Treatment of Pediatric Voice Disorders	964
Medical-Surgical Treatment	964
Behavioral Treatment	964
Summary	965
References	965
Chapter 68:Gastroesophageal Reflux Disease and Eosinophilic Esophagitis in Children with Complex Airway Disease	966
Gastroesophageal Reflux Disease	966
Diagnosis	967
Treatment	967
Disorders of Motility	967
Eosinophilic Esophagitis	967
Diagnosis	968
Treatment	968
Suggested Reading	968
References	968
Chapter 69:Laryngeal and Tracheal Airway Disorders	969
Operative assessment	969
Flexible Bronchoscopy	969
Microlaryngoscopy and Rigid Bronchoscopy	969
Esophagoduodenoscopy	969
Swallowing and Voice Evaluations	970
Mitigating factors	970
Bacterial Colonization	970
Eosinophilic Esophagitis	970
Gastroesophageal Reflux Disease	971
Obstructive Sleep Apnea	971
Pulmonary Disease	971
Inappropriate Patient Selection	971
Optimization	971
Management of airway pathology	971
Laryngomalacia	971
Vocal Cord Paralysis	972
Laryngeal Webs	973
Subglottic Stenosis	973
Vascular Compression	974
Posterior Laryngeal Clefts	974
Tracheomalacia	974
Complete Tracheal Rings	975
References	975
Section XII:Other Diseases with a Prominent Respiratory Component	976
Chapter 70:Air and Liquid in the Pleural Space	976
Anatomy of the pleural space	976
Anatomic Features	976
Parietal Pleura	976
Visceral Pleura	976
Physiology of the pleural space	977
Formation and Absorption of Pleural Fluid	977
Maintenance of an Air-Free Pleural Space	977
Accumulation of excess pleural liquid	978
Pathogenesis	978
Functional Pathology	979
History and Physical Examination	979
Chest Imaging	980
Examination of Pleural Fluid	982
Pleural Biopsy	985
Management of Noninflammatory Pleural Effusions and Transudates	985
Hemothorax	986
Chylothorax	986
Management of Exudates and Empyema	986
Prognosis	989
Air in the pleural space	989
Etiology and Pathogenesis	989
Functional Pathology	991
History and Physical Examination	992
Management	993
Prognosis	994
Suggested Reading	994
References	994
Chapter 71:Primary Ciliary Dyskinesia	995
Historical background and nomenclature	995
Cilia: structure and function	995
Motile Cilia	995
Embryonic Nodal Cilia and Primary (Sensory) Cilia	996
Ultrastructural ciliary defects in primary ciliary dyskinesia	996
Dynein arm defects	996
Radial spoke defects	997
Microtubular transposition defect	997
Other defects	997
Functional ciliary defects in primary ciliary dyskinesia	997
Nasal nitric oxide in primary ciliary dyskinesia	998
Genetics	998
Clinical features of primary ciliary dyskinesia in children	999
Lower respiratory tract manifestations	1000
Nose and paranasal sinuses	1000
Middle ear	1000
Situs inversus totalis and other laterality defects	1001
Genitourinary system	1001
Other	1001
Diagnosis of primary ciliary dyskinesia	1001
Treatment	1001
Prognosis	1002
PCD and potential overlap with primary ciliopathies	1002
References	1002
Chapter 72:Childhood Pulmonary Arterial Hypertension	1003
Definition and classification	1003
Neonatal pulmonary hypertension	1004
Persistent Pulmonary Hypertension of the Newborn	1004
Pulmonary arterial hypertension in childhood	1005
Epidemiology	1005
Natural History	1006
Pathobiology	1006
Vasoconstriction	1006
Endothelial Dysfunction	1007
Pathophysiology	1008
Diagnosis and Assessment	1009
Hemodynamics/Cardiac Catheterization	1009
Clinical Presentation	1011
Physical Examination	1011
Physical Signs	1011
Treatment	1011
General Measures	1011
Anticoagulation	1012
Calcium Channel Blockade	1012
Serial Reevaluations	1013
Targeted Therapy	1013
Prostacyclin Analogs	1014
Intravenous Prostacyclin (Epoprostenol)	1014
Inhaled Prostacyclin Analogs (Iloprost, Treprostinil)	1014
Subcutaneous/Intravenous Prostacyclin Analog (Treprostinil)	1015
Endothelin Receptor Antagonists	1015
Nitric Oxide	1015
Phosphodiesterase Inhibitors	1016
Gene Therapy	1016
Oxygen	1016
Additional Pharmacotherapy: Cardiac Glycosides, Diuretics, Antiarrhythmic Therapy, Inotropic Agents, and Nitrates	1016
Atrial Septostomy	1017
Transplantation	1017
Summary	1017
References	1018
Chapter 73:The Lung in Sickle Cell Disease	1019
Definitions, epidemiology	1019
Pathophysiology	1019
Sickling and Inflammation	1019
Clinical manifestations	1019
Acute Chest Syndrome	1019
Chronic Lung Disease	1020
Role of abnormal pulmonary function tests and future morbidity	1021
Low O 2 Saturation	1021
Sleep-Disordered Breathing	1021
Pulmonary Hypertension	1021
Airway Lability	1022
Role of Asthma in Worsening Morbidity	1022
Atopy	1022
Care	1023
Chronic	1023
Care for acute episodes	1024
Pain	1024
Acute Chest Syndrome	1024
Summary	1024
Suggested Reading	1025
References	1025
Chapter 74:Lung Injury Caused by Pharmacologic Agents	1026
Cytotoxic drugs used in cancer therapy	1026
Antibiotics	1026
Bleomycin	1026
Alkylating agents	1028
Cyclophosphamide	1028
Chlorambucil	1029
Other Alkylating Agents	1029
Nitrosoureas	1029
Carmustine	1030
6-Mercaptopurine, Cytosine Arabinoside, and Gemcitabine	1030
Other Cytotoxic Agents	1030
Non-cytotoxic and other drugs	1031
Nitrofurantoin	1031
Sulfasalazine	1032
Diphenylhydantoin, Carbamazepine, and Levetiracetam	1032
Minocycline	1032
Penicillamine	1032
Leflunomide	1033
Azathioprine	1033
Other Immunomodulatory Agents	1033
Amiodarone	1033
Other agents	1034
Summary	1035
Suggested Reading	1035
References	1035
Chapter 75:Disorders of the Respiratory Tract Caused by Trauma	1036
General considerations	1036
Features of the pediatric thorax	1036
Sternal fractures	1037
Rib fractures and flail chest	1037
Tracheostomy in chest wall injury	1038
Traumatic pneumothorax	1039
Hemothorax	1040
Tracheobronchial trauma	1041
Pulmonary compression injury (traumatic asphyxia)	1041
Posttraumatic atelectasis	1041
Cardiac trauma	1042
Traumatic rupture of the thoracic aorta	1042
Injuries to the esophagus	1042
Traumatic blunt rupture of the diaphragm	1044
Thoracoabdominal injuries	1045
References	1045
Chapter 76:Sudden Infant Death Syndrome and Apparent Life-Threatening Events	1046
Apparent life-threatening events in infants and apnea in premature infants	1046
Apparent Life-Threatening Events	1046
Definitions	1046
Apparent life-threatening events and recommendations for home cardiorespiratory monitoring	1046
Evaluation of an infant with an apparent life-threatening event	1046
A potential mechanism for apparent life-threatening events after gastroesophageal reflux or respiratory syncytial virus inf ...	1049
Relevant epidemiology and physiology of gastroesophageal reflux	1049
Characteristics of laryngeal chemoreflex apnea	1050
Some risk factor for sids and alte may be explained by lcra	1051
Laryngeal Chemoreflex Apnea and Nicotine Exposure	1051
Laryngeal Chemoreflex Apnea and Respiratory Syncytial Virus Infections	1051
Summary	1051
Apnea in premature infants	1052
Definition and Differential Diagnosis	1052
Hypotheses for pathogenesis of apnea of prematurity	1052
The natural history of apnea of prematurity	1054
Therapies for apnea of prematurity and monitoring premature infants at home	1054
Monitoring Premature Infants at Home	1055
Sudden infant death syndrome: new explanations within a new definition	1055
Triple-Risk Model and New Infant Variables	1055
Multifactorial explanations, not a single cause	1057
Newer approaches to pathology of sudden infant death syndrome	1057
Programmed Cell Death Within the Central Nervous System	1057
Neural Receptors and Sudden Infant Death Syndrome	1058
Fatty Acid Oxidation Deficiencies and the Contribution of Genetic Diseases of Metabolism to Sudden Infant Death Syndrome	1059
Prolonged Electrocardiogram QT Intervals and Lethal Cardiac Arrhythmias	1060
Current understanding based on investigations of the scene and circumstances of sudden infant death	1060
Sleep practices and the decline in rates of sudden infant death syndrome—the changing epidemiology	1061
Success Story for Epidemiology	1061
Epidemiology of sudden infant death syndrome before and after back-to-sleep interventions	1061
The impact of scene investigations on the understanding of sudden death and diagnostic shifting	1062
Physiology Related to Sleep Position That May Make Infants More Vulnerable	1062
Abnormal arousal and the impact of sleep position	1063
Abnormalities in ventilatory response and threats within the sleep environment	1064
Soft bedding as an effect modifier: physiologic implications	1064
Sharing beds, racial disparities, and continued high rates of sudden unexpected infant deaths	1065
Summary	1066
Suggested Reading	1066
References	1066
Chapter 77:Disorders of Breathing During Sleep	1067
Basic mechanisms and architecture of normal sleep	1067
Neural Circuitry of Sleep and Waking	1067
Developmental aspects of sleep	1067
Respiratory control mechanisms	1068
The respiratory rhythm generator	1068
Sleep and breathing during development	1068
Central chemoreceptors and their development	1069
Multiplicity of central chemosensitive centers	1070
Peripheral chemoreflexes	1070
The upper airway	1071
Upper Airway Control	1071
Upper Airway Dysfunction	1072
Apnea	1073
Central Apnea or Hypoventilation Syndromes	1073
Congenital central hypoventilation syndrome	1073
Diagnosis and Clinical Management	1074
Secondary central hypoventilation syndromes	1075
Apnea of prematurity	1076
Pathophysiology	1076
Diagnosis	1077
Treatment	1077
Role of Continuous Positive Airway Pressure	1078
Obstructive sleep apnea	1078
Epidemiology	1080
Pathophysiology	1082
Conditions Associated with Obstructive Sleep Apnea	1082
Clinical Evaluation and Diagnosis of Obstructive Sleep Apnea	1083
Polysomnography	1083
Short-Term and Long-Term Morbidity of Obstructive Sleep Apnea	1084
Treatment of Obstructive Sleep Apnea	1086
References	1086
Index	1087

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