Index

Note: Page numbers followed by b indicate boxes; f, figures; and t, tables.

A Acute cerebral insult, 590 Acute respiratory distress syndrome (ARDS)

A disintegrin and metalloproteinase domain 33 Acute chest syndrome, 904–905 (Continued)
(ADAM33), in asthma, 653t clinical features of, 905, 905f airway pressure release ventilation in,
AAD. see Assisted autogenic drainage management of, 906–907, 906t 611
AAQOL. see Adolescent Asthma Quality of Life risk factors of, 904–905 beta-adrenergic agonists in, 613
Questionnaire Acute eosinophilic pneumonia, 959–960 conventional mechanical ventilation in,
ABCA3 deficiency, indication of, 58 Acute eosinophilic pneumonitis, 609–610
Abdominal binders, 285 characteristics of, 959t corticosteroids in, 612
Abdominal wall, movement of, 5 Acute exposures, to environmental factors, in high-frequency oscillatory ventilation in,
Aberdeen cohort, 253 hospitalization, in asthma, 647 611
Aberdeen SEATON study, in asthma, 654 Acute exudative phase, characterization of, inhaled nitric oxide in, 612–613
Aberrant right subclavian artery, in Down 607 neurally adjusted ventilatory assist in,
syndrome, 1002f Acute hypersensitivity pneumonitis, 948 611–612
About My Asthma (AMA), 234–235, Acute hypoxia, vasoconstrictor response, 373 neuromuscular blocking agents in, 613
236t–237t Acute illness, 2 noninvasive ventilation in, 610–611
ABPA. see Allergic bronchopulmonary Acute inflammation, 101, 102f prone positioning in, 612
aspergillosis Acute inflammatory process, resolution, 102 surfactant in, 613
Abscess, lung, 154, 155f Acute laryngotracheobronchitis (croup), 287 tracheostomy in, 614
Absent-in-melanoma-like receptors, 125 Acute lower respiratory infection (ALRI), Acute respiratory failure, in neonates, gene
Acapella, 275f, 281f 441–443 mutations and, 38–39
Accidental deaths, in drowning, 634 frequency, increase, 444 Acute respiratory illness, environmental
Accuhaler, 265f, 266 risk, increase, 443 contributions to, 55
ACD. see Alveolar capillary dysplasia Acute lung injury Acute tamponade, physical findings with,
ACDMPV. see Alveolar capillary dysplasia with acute respiratory distress syndrome and, 1067
misalignment of pulmonary veins 590 Acute traumatic diaphragmatic injuries
Acetaminophen hospital mortality in, plot of, 592f (treatment), surgical reduction (usage),
antenatal effects of, 249–250 inflammation-mediated lung injury in, 1070, 1071f
during pregnancy, asthma and, 653–654 occurrence of, 584 Adaptive lung defenses, 129–132
Acid-base balance, 94–97, 96t Acute mountain sickness (AMS), 590–591 ADCC. see Antibody-dependent cellular
buffering and transport, 94–96, 95f Acute pneumonia, tuberculosis and, 481 cytotoxicity
Acidemia, 96 Acute pneumonitis, rarity of, 855 Adenocarcinoma, 313
Acidosis, metabolic, asthma and, 688 Acute pulmonary edema, fluid accumulation Adenoidectomy, usage, 1159
Acinar area, in postnatal lung growth, 72–73 sequence during, schematic Adenoids, enlargement, 1054–1055
Acinar dysplasia, 314 representation of, 585f Adenosine triphosphate, NBD affinity, 759
Acinus, 63 Acute rejection, after pediatric lung Adenosine triphosphate-binding cassette A3
as portion of lung parenchyma, 66–67 transplantation, 986 (ABCA3), 45
Acquired atelectasis, 1027 Acute respiratory acidosis, 96 absence, 61
Acquired bilateral vocal cord paralysis, Acute respiratory alkalosis, 96 deficiency, 837–838
1121–1122 Acute respiratory deterioration, management predicted incidence of, 837–838
Acquired digital clubbing, 19 of, 1058 ultrastructural findings associated,
Acquired immune deficiency syndrome Acute respiratory disease, in human 842f
(AIDS), nontuberculous mycobacterial immunodeficiency virus–infected children location of, 836
disease with, 499 and adolescents, 968–975, 969b mutations of, 815
Acquired immunity, in pulmonary host Acute respiratory distress syndrome (ARDS), production of, 836
defense systems, 37–38 60, 62, 351–352, 606–614 Adenotonsillar hypertrophy, childhood OSA,
Acquired immunodeficiency, in children, 923 acute lung injury and, 590 1154
Acquired immunodeficiency states, pulmonary clinical presentation of, 351 Adenovirus, 472, 927
pathogens associated with, 924t definition of, 606–607 infection, 472–474
Acquired thrombophilia, 888 diagnosis of, 606–607, 607t characteristics of, 467t
Acrocyanosis, 17 differences between children and adults, clinical features of, 472–473
ACT. see Airway clearance technique 609 diagnosis of, 473
Activator protein-1 (AP-1), as epidemiology of, 606 differential diagnosis of, 473
proinflammatory transcription factors, etiology of, 606, 607b epidemiology of, 472
109–110 following solid-organ transplantation, 931 etiology of, 472
Active cycle of breathing techniques, 275f genetic modifiers of, 609 imaging of, 473, 473f
modification, 284 inflammation-mediated lung injury in, laboratory findings on, 473
teaching, 279–281 occurrence of, 584 management and treatment of, 473–474
Active immunization, for pertussis, 533 management of, 351–352 physical findings on, 473
Active smoking, 252 mechanical ventilation in, 551–552 prevention of, 474
severe asthma and, 729 outcome of, 606 prognosis of, 474
Acute alveolitis, in hydrocarbon aspiration, pathophysiology of, 351, 607–609 pulmonary function tests for, 473
626 pediatric, 607t symptoms of, 473
Acute asthma extracorporeal life support in, 613–614 transmission of, 472
episode of, management of, 717–719 pulmonary (direct) versus extrapulmonary Adenylate cyclase, in Bordetella pertussis,
management of, in medical facility, (indirect), 608, 608t 530t
algorithm for, 718f severity score for, 609 ADHD. see Attention deficit hyperactivity
Acute bronchitis, etiologic agents with, 397t treatment of, 609–614 disorder

1161
1162 Index
Adherence
electronic monitoring and, 728, 728f
home visit and, 727
parental supervision and, 727
to prescription records, 727
severe asthma and, 727–728
to treatment regimen, for asthma, 717
use of inhaler devices and, 727–728
Adhesion proteins, 122–123, 123f
Adjunct therapy, for preschool asthma,
681–682
Adolescent Asthma Quality of Life
Questionnaire (AAQOL), 236t–237t
Adolescent idiopathic scoliosis, 1052,
1058–1059
Adolescents
allergic rhinitis, 737
asthma in, 689
authority, challenge, 244–245
body mass index (BMI), 216
bronchoscopy, chest radiograph, 766f
cardiopulmonary exercise testing, 215–216
chronic respiratory disorder, transition, 241
confidentiality, establishment, 243
cystic fibrosis, spiral chest CT scan, 766f
development, understanding, 241–242
exercise, physiological responses, 216, 217f
exploratory behaviors, 244–245
exposure pathways in, 50t
health issues, addressing, 244–245
health-risk behaviors, 244–245
independence/autonomy, increase, 241
lung apices, parenchymal opacifications
(chest radiograph) in, 865f
nasal passageways of, inspection of, 16
obesity, 215–216
pulmonary embolism in, 888, 888t
self-management, promotion, 244
study of CPET in, 216
tasks of, 242b
Adrenal suppression, corticosteroids and, 706
Adrenaline, 410–411
Adult dermatomyositis, 857
Adult disease, pediatric origins, 212
Adult onset DM (morbidity/mortality), ILD
(impact) of, 858
Adult type tuberculosis, 481, 482f
Adulthood, pediatric respiratory disorders,
242b
Adults
allergic rhinitis, 737
bronchiolitis obliterans organizing
pneumonia (BOOP) developed in, 880
care models, differences, 242
chronic respiratory disorder, transition, 241
exposure pathways in, 50t
hamartomas, 1072
health care needs/patterns, 242
lungs in
children and, contrast between, 609
microvascular bed in, perfusion of,
583–584
self-management, promotion, 244
services
preparation, 245
trust/respect, absence, 246
shrinking lung syndrome in, 856
Advanced Trauma Life Support (ATLS),
guidelines, 1062
Adventitious respiratory sounds, indication of,
11
Adventitious sounds, in categories of
respiratory sounds, 14f
AECs. see Alveolar epithelial cells
Aerobic metabolism, 97
Aerobic training, 218
Aerodigestive model
health care value for complex patients
through coordinate care, 1094–1096
historical perspective of, 1094
interdisciplinary team in, 1095, 1095f
patient evaluation in, 1094–1095
patient selection in, 1094
Aerodigestive program referral, typical
complaints leading to, 1095t
Aerodigestive tract
anatomic abnormalities, 1100
evaluation of, 1104–1105
Aerodynamic filtering, 120
Aerosol devices, 120, 264–270
Aerosol drug-delivery device design,
270
Aerosol generation, 261–263
Aerosol therapy, 257
advantages of, 257
advice and training for parents, 271
aerosol generation, 261–263
assessment techniques, 261–264
brownian motion/diffusion, 260
delivery, 263
and deposition, 258–260
delivery system, progress, 271
disadvantages of, 257
dosage considerations, 270–271
calculation of, 270
patient considerations, 270
electrostatic attraction, 260
gravitational sedimentation, 259–260
inertial impact, 259, 262, 262f
laser diffraction, 262–263
particle size, 258–259, 258t
distribution, 261–263
patient-related variables, 260–261
age, 260
regimen and device compliance,
260–261
systemic dose, from inhaled drugs, 270–271
total drug output, 263
in vivo deposition, 263–264, 264f
Afferent fibers, stimulation of, 120–121
African American boy, skin lesions (history/
biopsy) of, 864f
Agar gel immunodiffusion, 949f
Age, in immunopathogenesis of pediatric
asthma, 665
b2-agonist, for hydrocarbon aspiration,
627–628
AHLM. see Alveolar hemosiderin-laden
macrophages
AHR. see Airway hyperresponsiveness
Air bronchograms, presence of, 858f
Air embolism, 312
Air leak syndromes, as complication of
mechanical ventilation, 601, 601f
Air quality, 221
in asthma, 661–664
indoor, 662–663
Air-liquid interfaces, collapsing forces in, 26
Airspace fluid, clearance rate of
(augmentation), 593
Airspaces, of lungs, distention of, 71
Airway clearance
methods, 454
respiratory physiotherapy, 274–282
treatment options, 274
Airway clearance technique (ACT), 275f–278f
review, 282
variety, 274
Airway compression
due to vascular rings and slings, 615–619,
616f–618f
clinical presentation of, 619
Airway compression (Continued)
diagnosis of, 619
treatment of, 619, 619f–620f
dynamic, 80
Airway endoscopy abnormalities, in Down
syndrome, 994f
Airway eosinophilia, in pediatric allergic
asthma, 667
Airway hyperreactivity
asthma and, 686, 689
cystic fibrosis and, 781
in human immunodeficiency virus–infected
children and adolescents, 979
reduced symptoms of, 715
to substances, 695
Airway hyperresponsiveness (AHR), 102–103,
118
in asthma development, 665
Airway inflammation, 101–119, 221
direct measurement of, 114–115,
114f–115f
due to asthma, 1027
indirect inflammatory markers, 118
neural control, two-way interaction, 112f
noninvasive assessment of, 115–118
in pediatric respiratory disease, 118
therapeutic implications for, 118
Airway obstruction, 589
asthma and, 688
bronchiolitis, 421
diagnostic bronchoscopy for, 138
in infants, 180–181
pulmonary edema and, 587
Airway pressure release ventilation, in acute
respiratory distress syndrome, 611
Airway protection
instrumental assessment of, 1109–1112
pharyngeal phase, 1107–1108
problems, 1108–1109
neurologic conditions, 1109
Airway remodeling, 689–690
in asthma development, 665, 669f
Airway resistance
factors that affect, 80
increase in, 585–586
plethysmographic measurement of,
198–199
sites of, 79–80
Airway smooth muscle, in pulmonary
immunity and asthma pathogenesis, 671
Airway surface liquid (ASL), 121–122, 762
depletion, 122
Airways, 63–66
anastomosis, dehiscence of, 985
branching, model of, 65f
cartilage, congenital deficiency of, 65–66
cellularity, dominant, 458t
compression, diagnosis, 1123
compromise, risk of, 1067
conditions, impact, 1109
defense functions, 121
dehydration, impact, 762–763
diameter, 72f
increased, 83–84
dilation, 444–445
dryness of, 872
epithelium, 110
examination of, bronchoscopy for, 134
flaccidity, 450
function of, prostaglandins (PG) in, 110
functional compartments of, 63
gas flow within, 78–79
guy wire effect on, 80
inflammation
and neural control, 112f
pathogenic processes (impact), 446–447

Airways (Continued)
inflammatory response of, neural
mechanism in, 111–112
models of, sound generation in, 10
mucosal abnormality, 450f
mucus production/secretion (reduction),
antisecretagogues, 453–454
muscles, bronchoconstriction of, 66
narrowing, maximum, 1149
nerves, release neurotransmitter from, 112
oxygen and carbon dioxide in, partial
pressures of, 85f
patency, maintaining, 65–66
pathology, management of, 1121–1124
radiologic evaluation of, 1122
reflexes, 120–121
resin cast of, 64f
smooth muscle content of, 66
submucosa in, 65–66
tortuosity, 444–445
vascular compression, 1123
wall structure of, 65f
AKITA JET Inhalation system, 269, 270f
Alae nasi, flaring of, 5
Albendazole, for echinococcosis, 542
Albuterol (Salbutamol), 219
for asthma, 702–703
acute episodes of, 717
nebulized, in emergency department, 717
Alemtuzumab (Campath), 883
Alkalemia, 96
Alkaline phosphatase (ALP), elevation of, 792
Alkylating agents, 878–879
Allergen immunotherapy, 747–750
administration, 747–748
allergic sensitivity development, prevention
of, 749
asthma, prevention of, 749
asthma control, impact on, 748–749
clinical application, 750
indication of, 747
mechanism of action, 748, 748f
patient selection, 747
persistent clinical effects, after
discontinuation, 749
safety, 749–750
Allergens
asthma and, 692
avoidance, 741
strategies, 741b
exposure to, severe asthma and, 728–729
immunotherapy, 742
provocation studies, 738
removal of, 715
Allergic angiitis, 962
Allergic bronchopulmonary aspergillosis
(ABPA), 781, 952–958
central bronchiectasis in, 956f
clinical features of, 955–957
clinical staging of, 955–956, 956t
cystic fibrosis with, lung biopsy of, 953f
development of, genetic risk factors in, 954b
diagnostic criteria for, 952b
differential diagnosis of, 957
eosinophils, biology of, 953–955
epidemiology of, 952
etiology of, 952
IL-4, increased sensitivity to, 954
immunotherapy for, 958
laboratory findings in, 956–957
management and treatment of, 957–958
pathogenesis of, 952–955
pathology of, 952–955
physical findings in, 955
prednisone in, dose of, 957
prognosis of, 958
Allergic bronchopulmonary aspergillosis
(ABPA) (Continued)
radiographic findings in, 956
serum IgE in, elevated, 955
symptoms of, 955
as Th2 hypersensitivity lung disease, 952
Allergic fungal sinusitis, 744
Allergic inflammation, 101
cells predominate in, 103
characterization of, 101
in environmental exposures, 51–53, 53f
infiltration with eosinophils, 101
Allergic inflammatory cytokines, in asthma,
665
Allergic rhinitis, 737–742, 1054–1055
asthma and, 693
association, 737–738
epidemiologic relationship, 737–738
pathophysiologic connections, 739–740
burden, addition, 738
diagnosis, 741
diagnostic/therapeutic implications,
741–742
histologic studies, 738
immunopathology, 738
pharmacotherapy, 741–742, 746b
therapeutic implications, 741–742
Allergic salutes, transverse crease from, 6
Allergic sensitivity development, prevention of,
749–750
Allergic sensitization
in asthma development, 665, 666f
in environmental exposures, 51–53, 53f
Allergic shiners, 6
Allergy
to food, asthma and, 725
testing, for asthma, 698
All-trans retinoic acid (ATRA), 881
Alpha-1 antitrypsin (ATT) deficiency,
823–824
clinical features of, 824
diagnosis of, 824
epidemiology of, 823–824
etiology of, 824
management of, 824
pathobiology of, 824
predispositional testing of, 824
treatment of, 824
ALRI. see Acute lower respiratory infection
ALTE. see Apparent life-threatening event
Altered responsiveness, BRUE definition and
factors for inclusion and exclusion, 1126t
Alternaria spp., 526
Altitude, exercise, 226
Alveolar capillary dysplasia (ACD), 298, 999
Alveolar capillary dysplasia with misalignment
of pulmonary veins (ACDMPV),
825–826, 827f
clinical features of, 826
diagnosis of, 826
differential diagnosis of, 826
epidemiology of, 825
etiology of, 825–826
FOX F1 gene and, 825
histologic findings in, 826
management of, 826
pathogenesis of, 825–826
presentation of, 826
prognosis of, 826
radiologic findings in, 826
treatment of, 826
Alveolar capillary membrane
integrity, 344
permeability, increased, 584
Alveolar development, 252
Alveolar edema, in smoke inhalation, 630
Index 1163
Alveolar epithelial cells (AECs), 26
type II, 67f
Alveolar filling disorders, therapeutic
bronchoscopy for, 145
Alveolar gases, humidification of, 26
Alveolar hemorrhage, incidence of, 879
Alveolar hemosiderin-laden macrophages
(AHLM), presence of, 1140
Alveolar hypoventilation, 17–18
treatment of, 390–391
Alveolar macrophages (AM), 66–67, 70, 104,
105f
prevention of, from catabolizing surfactant,
granulocyte-macrophage colony-
stimulating factor deficiency and, 58
Alveolar period, 28f, 30–31
Alveolar proteinosis
clinical syndrome of, 58
gene mutations and, 38–39
Alveolar region, 66–68
Alveolar septum, membranes in, fusion of,
581f
Alveolar spaces
protein clearance from, 584
protein presence in, 584
Alveolar stability, 58–59
Alveolar type II epithelial cell (AEC2), storage
of, 836
Alveolar ventilation, 86
alveolar gases and, 86–88
effect of changing, 87f
Alveolarization, 72–73
Alveoli
epithelial cells in, 67
portrayal of, 580
Alveologenesis, 35–36
AM. see Alveolar macrophages
AMA. see About My Asthma
American College of Rheumatology, SLE
classification criteria of, 855b
Amikacin, for nontuberculous mycobacterial
infections, 504t
Aminophylline, effects of, 21
4-aminopteroylglutamic acid, 879–880
Amiodarone, 883–884
Amphotericin B
for candidiasis, 519–520
for coccidioidomycosis, 513
for fungal infections, 975
for invasive aspergillosis, 517–518
for pulmonary mycoses, 507
for sporotrichosis, 524
Amphotericin B deoxycholate, for candidiasis,
520
Ampicillin, for early onset septicemia, 346
AMS. see Acute mountain sickness
Anaerobic metabolism, 97–98
Anaerobic threshold, 215
Anastomotic narrowing, development of,
985
ANCA. see Antineutrophil cytoplasmic
antibody
Aneuploidy, 292
Angiogenesis, increased number of blood
vessel, 102–103, 103f
Angiography, 168, 334, 336f
Annexin, 102
Anomalous innominate artery, 309
Ansa-cervicalis reinnervation, 1121
Antenatal lung development, 248
Antenatal steroids, exposure, absence,
343–344
Anterior axillary fold, chest wall and, 148
Anterior diaphragmatic hernias, 331–332,
332f
Anterior diverticulum, 248
1164 Index

Anterior left upper lobe, in spectra of Aplasia, 320–322 Asphyxiating thoracic dystrophy, 384,
respiratory sounds, 11f pulmonary, 321–322 1005t–1006t, 1049, 1052–1054, 1054f,
Anterior malignant teratoma, posteroanterior/ Apnea, 1149–1159 1060–1061
lateral radiographs of, 1078f bronchiolitis and, 422 Aspiration, 1094, 1097–1105
Anterior mediastinum, widening of, thymus caffeine treatment, 343 evaluation, radiographic and endoscopic
and, 147 central apnea, 1149–1152 tests, role of, 1100–1101
Anterior right upper lobe, fluid-filled cysts in, differential diagnosis, 1130 of gastroesophageal reflux, 1101–1103
541f home monitors, 1129–1130 hydrocarbon, lung injury from, 626–633
Anterior superior mediastinum, benign laryngeal chemoreflex apnea, 1126 clinical findings of, 627, 627f
thymoma (location), 1076f newborn infants, 343 diagnosis and differential diagnosis of,
Anterolateral right upper lobe, bilateral short duration, 1145 627
circumscribed densities in, 540f Apnea of prematurity (AOP), 1125 epidemiology of, 626
Anteroposterior (AP) chest diameters, mean first-line treatment of, 1133 etiology of, 626
values of, 8f management and treatment of, 1133–1134 management and treatment of, 627–628
Antiarrhythmic therapy, in pulmonary arterial methylxanthines for, 1133 pathology of, 626
hypertension, 578 natural history of, 1132 pathophysiology of, 626–627
Antibiotics pathogenesis of, 1130–1131, 1132f prevention of, 628
for asthma during pregnancy, 653–654 prognosis of, 1132–1133 prognosis of, 628
bacterial resistance and, 432 treatment, high-flow therapy, efficacy of, of oral secretions, 1103
for pneumonia, in human 1133–1134 protection against, 1097–1098
immunodeficiency virus–infected Apoptosis, 132–133 swallowing dysfunction, caused by,
children and adolescents, 970t in pulmonary arterial hypertension, 568 1097–1100
for pregnant, 250 Apoptosome, 132–133 upper airway infection, relationship, 443
for preschool asthma, 685 Apparent life-threatening event (ALTE), 1125 Aspiration pneumonia, 354, 354f
Antibody-dependent cellular cytotoxicity clinical approach to, 1129, 1129f Aspiration pneumonitis, in human
(ADCC), eosinophil degranulation, clinical features of, 1128 immunodeficiency virus–infected children
953 differential diagnosis of, 1126b and adolescents, 978
Anticholinergic agents, for asthma, 703 GER, impact, 1126 Aspirin, 884
Anticoagulation, in pulmonary arterial laboratory findings, 1129 asthma and, 693–694
hypertension, 572–573 management and treatment of, 1128–1129 Assist/Control (A/C) mode, 388
Antigen detection assays, 402 physical findings, 1128 Assisted autogenic drainage (AAD), 279
Antigen presenting cells, 102f Appendicitis, 796 Associated hypereosinophilic syndrome, 966b
Antigenic drift, in influenza virus, 460 Architectural distortion, 168 Associated pulmonary arterial hypertension
Antigenic shift, in influenza virus, 460 ARDS. see Acute respiratory distress syndrome with congenital heart disease (APAH-
Antiglomerular basement membrane Area of reactance (AX), asthma and, 695 CHD), 561–572
(anti-GBM) disease, 897–898, 899f Arnold-Chiari malformation, 306, survival in, 564
Antihistamines, 739 1148–1149 Asthma, 221, 286, 686–721
Antiinflammatory mechanisms, 113–114 Arousal, sudden infant death syndrome and, acute episode of, management of, 717–719
Antimetabolites, 879–880 1140 in medical facility, algorithm for, 718f
Antimicrobial peptides, 125–126 ARQOLS. see Asthma-Related Quality of Life adherence to treatment regimen for, 717
Antimicrobial regimen, for pediatric lung Scale allergens and, 692
transplantation, 984 Arterial blood, reduced hemoglobin in, 17 allergic bronchopulmonary aspergillosis in,
Antimicrobial susceptibility testing methods, Arterial hemoglobin desaturation, 952b
403–404 mechanisms of, 17–18 allergic rhinitis
Antimicrobial-resistant bacteria, in human Arthritis-like signs, 448 association, 737–742
immunodeficiency virus–infected children Arthrogryposis multiplex congenita, epidemiologic relationship, 737–738
and adolescents, 969 1005t–1006t pathophysiologic connections, 739–740
Antineutrophil cytoplasmic antibody (ANCA), Aryepiglottic folds, 303 and sinusitis, 693
867–869 Ascaris lumbricoides, 962 allergy testing for, 698
ANCA-associated vasculitis, 867–869 ASL. see Airway surface liquid altitude, 226–227
Antinuclear antibodies (ANA), presence of, ASOT. see Antistreptolysin O titer anticholinergic agents for, 703
850–852 Aspergillosis antiinflammatory mechanisms in, 113
Antioxidant defense, in environmental in cystic fibrosis, 955 atopic, 650–651
exposures, 51, 52f invasive, 515–518 beclomethasone for, 704–705
Antiphospholipid antibodies (APLA) clinical features of, 516 biologic therapy for, 710
presence of, 856, 895 diagnosis of, 516–517 biomarkers for, 701
Antiphospholipid syndrome, 572–573 differential diagnosis of, 517 breast-feeding diet and, 655–656
Anti-PIV-specific immunoglobulin E (IgE), role epidemiology of, 515 bronchial challenge and, 695–696, 695t
of, 407–408 etiology of, 516 budesonide for, 704
Antiretroviral therapy (ART), in human imaging of, 516 chronic, management of, 711, 712f
immunodeficiency virus–infected children laboratory findings on, 516 sample treatment plan for, 716f
and adolescents, 968 management and treatment of, chronic sinusitis, 745
Antisecretagogues, 453–454 517–518 epidemiologic relationship, 745
impact, 453–454 pathology/pathogenesis of, 516 pathophysiologic links, 745
Antistreptolysin O titer (ASOT), 1018 prevention of, 518 ciclesonide for, 705
Antiviral immunization, 379 prognosis of, 518 classification of, 699–701
Antiviral treatment, for influenza, 463 pulmonary function testing for, 516 complete blood cell count for, 696
AOP. see Apnea of prematurity Aspergillus flavus, 516 control of
Aorta, preductal coarctation, 18 Aspergillus fumigatus, 319, 516, 781 determination of, 700–701, 700t
Aortic arch, and derivatives, 616f Aspergillus nidulans, 516 impairment domain of, 698–699
Aortic bodies, peripheral chemoreceptors, Aspergillus pneumonia, 928, 928f measures for, 701
location of, 342 Aspergillus species, 516 risk domain of, 698–699, 711
Aortopexy, 310 in human immunodeficiency virus–infected controller medications for, 703–705
APAH-CHD. see Associated pulmonary arterial children and adolescents, 975 corticosteroids for
hypertension with congenital heart pulmonary infection and, 928–929 inhaled, 703–704, 704t
disease Aspergillus terreus, 516 systemic, 706–709

Asthma (Continued)
cough-variant, 690–691
cytokines in, 109–110
cytologic examination of sputum and, 696
deaths, 647
defined, 686
diagnosis of, 641, 690–693
diagnostic testing, 741
difficult, 726–730
early childhood and, 655–657
economic impact of, 648
education for, 729
emotional factors and, 693
endocrine factors of, 694
environmental contributions to, 55,
55t–56t
environmental influences on, 653–664
epidemiologic studies, defining, 641–642
epidemiology of, 640–664
epigenetics of, 652–653
etiology of, 688–689
exacerbations, immunology of, 674
exercise and, 217–223, 692–693
role of, 217–218
exercise challenge test for, 696
exhaled nitric oxide and, 696–697, 697t
fluticasone propionate for, 704
food allergy and, 725
gastroesophageal reflux and, 693, 725
gene environment interactions in, 652,
653t
general treatment of, 719–721
genetics of, 651–652
high risk of death from, 723b
holding chambers and spacer devices for,
714, 714t
hospital management of, 719
hospitalization in, 646–647
HRQoL measures, 236t–237t
in human immunodeficiency virus–infected
children and adolescents, 979
immunopathogenesis of, 665–676
altered pulmonary immunity in, 665,
666f
cells, molecules, and cytokines involved
in, 665–669
multiple interactions and factors leading
to, 666f
preschool wheeze, 671–674
school age allergic asthma, 669–670
specific clinical scenarios for
considerations in, 674–675
structural airway cells in, 670–671
immunopathology, 738
infections and, 657–659, 658f, 692–693
inflammation in, 104f
inflammatory subtypes of, 650
intermittent, 711–712
irritants and, 692
labile, 690
laboratory diagnosis of, 695
leukotriene antagonists for, 709–710
long-acting b-agonists for, 706–709
long-acting muscarinic antagonists for, 710
lung attacks, 734
lung function tests for, 695
maternal stress in, 250–251, 250b
measures, 234–235
recommendation, 235
mild, 689
persistent, 712–713
moderate, 689
persistent, 713
modern molecular therapies, for application
in managing childhood, 747–755
mometasone for, 705
Asthma (Continued)
mortality of, 646–647, 690
natural history and prognosis of, 689–690
nocturnal, 694
nonallergic hypersensitivity to drugs and
chemicals and, 693–694
nonatopic, 650–651
nonpharmacologic measures for, 715–717
obesity and, 656–657, 725
overview of, 640
pathology of, 686–687, 687f–688f
pathophysiology of, 687–688
inflammatory cell biology and, 688–689
peak expiratory flow rate monitoring for,
714–715
pharmacologic management of, 701
phenotypic variation in, 648–651
physical examination for, 691–692, 692f
plus extrapulmonary comorbidities,
724–726
pregnancy and childbirth and, 653–655
fetal growth and birth size, 654–655
maternal factors, 653–654
maternal lifestyle and environment, 654
mode of delivery, 655
in preschool age child, 677–685
clinical patterns of wheeze in, 679
diagnosis of, 677–678, 678t
environmental factors of, 679
epidemiology of, 677, 678f
natural history of, 679
pathology of, 679
treatment of, 680–685, 684f
prevalence of, 642–646
childhood, outcomes of, 646
in gender and ethnicity, 645–646
spatial variation in, 644–645
temporal variation in, 642–644,
643f–644f
prevention of, 749
quality of life in, 648
radiographs for, 698, 699f
reliever medications for, 701–703
rhinitis therapy, effects, 738–739
rhinosinusitis and, 725–726
safe sleeping zone, 715
serum tests for, 697–698
severe, 647, 650, 722–736
adherence and, 727–728
airway inflammation assessment, 731–732
airways disease deconstructed, 723, 731b
definitions of, 722–723
differential diagnoses of, 725t
environmental factors of, 728–729
exacerbating phenotype, treatment of,
734–735
hospital admission for, 729–730, 730b
investigation of, modified diagnostic
protocol for, 724, 724f
overarching principles of, 722
persistent, 713–714
persistent airflow limitation in,
assessment of, 732–733
problematic, 723b
psychosocial morbidity and, 729
steroid responsiveness assessment, 732,
732t
therapy-resistant, 730–732
types of, 723
severity of, 223f, 646–647, 698, 700–701
measures for, 701
short-acting b agonists for, 701–703
sinus therapy
effects, 738–739
medical treatment, 744–745, 746b
surgical treatment, 744
Index 1165
Asthma (Continued)
sleep disordered breathing and, 726
study of, epidemiologic approaches to,
640–641
sweat test and, 698
symptoms
improvement, 739
reporting, 729
syndromes of vocal cord dysfunction and,
726
systemic corticosteroid treatment of,
tapering of, 962
temporal progression of symptoms of,
648–650, 649f
theophylline for, 710
therapeutic considerations for, 698–699
therapy, 454
transcription factors and, 112, 113f
treatment considerations, 221–223
triggers of, 692
avoidance of, 715
uncontrolled, 218, 722
weather changes and, 692
Asthma-Related Quality of Life Scale
(ARQOLS), 236t–237t
Asthmatics
radiologic sinusitis
features, 743–744
prevalence, 743t
responses, 692
Astra, 280f
Asymmetrical lung volume, 148–149, 149f
Asymptomatic pulmonary function
abnormalities, 859
Ataxia telangiectasia (AT), 821, 921
Atelectasis, 1027–1033
during anesthesia, 1027
causes of, 1028b
chest physiotherapy of, 1031
clinical manifestations of, 1028–1029,
1029f
congenital, 1027
cystic fibrosis and, 780
definition of, 1027
diagnosis of, 1029–1031, 1030f, 1030t
bronchography, 1031, 1032f
chest radiography, 1029–1030
CT usage, 1030, 1030f–1031f
fluoroscopy, 1029–1030, 1029f
magnetic resonance imaging, 1030–
1031, 1031f
ultrasound, 1031, 1031f
etiology of, 1027
as mechanical ventilation complication,
599–600
pathogenesis of, 1027
pathogenic mechanisms of, 1027–1028
presence of, 1028
prevention of, 1032–1033
secondary, 1027
in smoke inhalation, 630
sound transmission and, 8–9
total obstruction, lesion persistence, 1088f
treatment of, 1031–1032
ultrasonography, usage, 1031
Atopic asthma, 650–651
Atopic status, independent, 103
Atopic wheezers, 679
Atopy, 725
ATRA. see All-trans retinoic acid
Atresia
bronchial, 321f
congenital bronchial stenosis, 311
esophageal, 322–324, 1003
laryngeal, 304–306
pulmonary, 622–624
1166 Index
Atresia (Continued)
tracheal, 298, 299f, 299t
upper respiratory tract, 298, 299f
Atrial premature contractions, 21
Atrial septostomy, in pulmonary arterial
hypertension, 578
Attention deficit hyperactivity disorder
(ADHD)
OSA and, 1158
periodic leg movements, 1155f
Attentional capacity, changes, 232–233
Auerbach’s plexuses, presence, 1085
Aureobasidium pullulans, contamination with,
946–947
Auscultation, 10–14
technique of, 12–14, 13f–14f, 13t–14t
thoracic acoustics, 10–12, 10f–11f
Auscultatory signs, basis of, 14
Autocrine-paracrine interactions, in lung
injury and repair, 37
Autogenic drainage, 278f, 280f
Autoimmune pulmonary alveolar proteinosis
term, usage of, 839
whole lung lavage for, 847–849
Autonomic nervous system
development of, 384–385
dysfunction, 1150–1151
Autophagy, 132
Autosomal dominant disorders, 41
Autosomal recessive CSF2RA mutations,
839–840
Autosomal recessive CSF2RB mutations, 840
Autosomal recessive neurodegenerative
disorders, 1047
Autosomal-recessive CGD, 909
Avian antigens, in hypersensitivity
pneumonitis, 944
Azathioprine, 883
Azithromycin
for cystic fibrosis, 785
for nontuberculous mycobacterial
infections, 504t
for pertussis, 533
B Birth size, on asthma, 654–655
Bacillus Calmette-Guérin (BCG) vaccination,
6–7
for tuberculosis, 496
Back-to-Sleep campaign, 1140
Bacterial antigens, detection, 430
Bacterial colonization, 1119–1120, 1120t
Bacterial pneumonia, in human
immunodeficiency virus–infected children
and adolescents, 969–970
antibiotic treatment of, 970t
Bacterial tracheitis
clinical features of, 415
epidemiology of, 414
etiology of, 414
management of, 415
pathology of, 414
Bactericidal assays, 913
Bacteriologic complications, of bronchoscopy,
146
Bag mask valve, 284f
BALF. see Bronchoalveolar lavage fluid
Barium swallow, 156, 334, 336f
for vascular rings, 619
Barrel-shaped chest, 6
Basal cells (BCs), 66f
Basement membrane, thickening of, asthma
and, 686–687, 687f
Basophils, 108
B-cell immunodeficiencies, 920
BCG vaccine, in HIV-infected children, 972
Beckett, Katie, 382
Beclomethasone, for asthma, 704–705
Bed sharing, sudden infant death syndrome
and, 1141
Bedaquiline, for drug-resistant tuberculosis,
494
Benign cystic teratoma, 1075–1077
mediastinal dermoid cyst, 1075–1076
Benign neurofibromas, pediatric rarity, 1080
Benign solid teratoid tumors, 1077
Benign thymoma, 1075
anterior superior mediastinum location,
1076f
Benralizumab, 754
Berkson fallacy, 724–725
Bernoulli principle, 406
Beta sympathomimetics, effects of, 21
Beta-adrenergic agonists, for acute respiratory
distress syndrome, 613
BHR. see Bronchial hyperresponsiveness
Bilateral congenital small lungs, causes of,
322t
Bilateral diaphragmatic paralysis, 384
Bilateral paralysis, 1121
Bile flow (improvement), ursodeoxycholic acid
(usage) and, 793
Biogenesis, 759
Biologic therapy, for asthma, 710
Biological agents, for severe therapy-resistant
asthma, 734
Biologics, 754
Biomarkers, for asthma, 701
Biopsy, 143
liver, for non-CF-related liver disease, 792
lung, in hypersensitivity pneumonitis,
950–951, 951f
Biot breathing, 5
Bipolaris spp., 526
Bird fancier’s lung, 944
Birth
changes in the circulation at, 354–355
depression, 339
functional residual capacity, 338
respiration, initiation, 338
Birth cohort study, in asthma, 640–641
Blastomyces dermatitidis, 514
Blastomycosis, 514–515, 514f
pulmonary infection and, 929–930
Bleomycin
lung injury caused by, 876–878
withdrawal, 878–879
Blood, gas analysis, 609
Blood clots, therapeutic bronchoscopy for, 144
Blood flow, distribution of, 82–83, 83f
Blood oxygenation, assessment of, 91–92, 92f
Blood specimens, 401
Blood vessels, increased number of
(angiogenesis), 102–103, 103f
Blood volume, increase in, 589
Blue light lasers, for pediatric airways, 144
B-lymphocytes, 107–108
in IgE production, 107–108
lung defense and, 130–131
BMPR2 mutations, in pulmonary arterial
hypertension, 560
Body, interior of, visualization of, 134
Body mass index (BMI), percentile, 239
Body plan, formation of, 26
Body plethysmography, 176–177
Body ventilators, 387–388
BOOP. see Bronchiolitis obliterans organizing
pneumonia
Bordetella, 528
Bordetella bronchiseptica, 528
Bordetella holmesii, 528
Bordetella parapertussis, 528
Bordetella pertussis, 528
pneumonia associated with, 969
Bordetella petrii, 528
Bordetella resistance to killing factor (brkA), in
Bordetella pertussis, 530t
Bordetella trematum, 528
Botulinum toxin injection, 1103
Boyle’s law, 74, 177
Boys
chest diameters of, mean values of, 8f
neuroblastoma in, 1081f
thymic cyst in, 1077f
Bradycardia, presence of, 346–347
Bradypnea, 4
Brain, after drowning, magnetic resonance
imaging of, 637f
Brain stem, dysfunction, 1150–1151
Branching morphogenesis, 26–27, 35–36
lung proliferation during, control of, 36
Breast-feeding diet, on asthma, 655–657
Breast milk, as exposure pathway, 49
Breath condensate, exhaled, 117–118
Breath print, 697
Breathing, 190
absent, decreased, or irregular, BRUE
definition and factors for inclusion and
exclusion, 1126t
exercises, 287
noisy, 16, 17b
pattern
disorders, 286
inspection of, 4
rhythm of, changes in, 4–5
sleep disordered, asthma and, 726
technique, active cycle, 280f
ventilation and mechanics of, 73
work of, 80–81
support, 3
Breathlessness, exercise-induced, 726
differential diagnosis of, 726b
BRICHOS, 838
Bronchi (bronchus), lumens, lesions of, 1088f
Bronchial abnormalities
branching, 311–312, 311f
congenital, 311–313
laterality, 312
Bronchial adenomas, 1072–1073
histologic types, definition of, 1072–1073
symptoms/signs of, 1073
Bronchial arteries, 68
Bronchial brushing, 143
Bronchial buds, 1008f
Bronchial challenge, 950
asthma and, 695–696, 695t
Bronchial change, 167–168, 168f
Bronchial circulation, 893
role of, 580
Bronchial disease, in tuberculosis, 479–480,
480f–481f
Bronchial diverticula, 311–312
Bronchial epithelial function, altered, in
asthma, 665
Bronchial hyperreactivity, 872–873
Bronchial hyperresponsiveness (BHR),
641–642
Bronchial lumen occlusion, 1027
Bronchial reactivity, exercise, 220
Bronchial rupture, management of, 1066
Bronchial stenosis, 144
therapeutic bronchoscopy for, 144
Bronchial tree, as proximal conducting zone,
63
Bronchiectasis, 14–15, 439–459, 440f, 1041
airway clearance methods, 454
antiinflammatory agents, 453

Bronchiectasis (Continued)
antimicrobials, 453
antioxidant agents, 453
antisecretagogues, 453–454
asthma therapy, 454
Bhalla score, 450–451
bronchial structural changes, vascular
changes, 445
bronchoscopic findings, 449–450
causes, 442t
chest HRCT scans
diagnosis, 449b
features, 448b
c-HRCT evidence, 439
clinical features, 447–448
comorbid conditions, 447–448
CSLD, contrast, 439
CT scan findings, 445f
definition of, 439–440
development, 446f
radiologic risk factors, 447
diagnosis of
delay, 447
median age, 447
diagnostic criteria, 448
diagnostic evaluations, 448–452
disease progression, assessment, 451
economic cost, 441
environmental modification, 454
epidemiology of, 440–444
etiologic evaluation, 449
etiologic risk factors, 441
etiologies of
evaluation, 449t
studies, 443t
exacerbations, assessment/importance, 452
genetics, 444
health care, 455
in human immunodeficiency virus–infected
children and adolescents, 978
infection, assessment, 451–452
initial trigger, 445
lobectomy, indications/contraindications,
455b
macro malnutrition/micro malnutrition,
impact, 443–444
malnutrition, predisposition, 443–444
management
issues, review, 452b
principles, 452–455
mucoactive agents, 453–454
mucociliary clearance, 445
negative predictive value, 451
nonsteroidal antiinflammatory agents, 453
pathology/pathophysiology, 444–447
positive predictive value level, 451
predisposing factor, 444
presenting clinical features, 447
prevalence, 440–444
prevention, 454
prognosis, 455–456
public health issues, 443–444
in pulmonary tuberculosis, 480–481, 481f
radiographic finding, 448
radiographic scoring systems, 450–451
severity, 447
assessment, 450–451
signet ring appearance, high-resolution
finding, 448f
social determinants, 455
surgical considerations, 454–455
technology dependence, 385
upper airway infection/aspiration, 443
vaccines, impact, 454
varicose/cystic changes, 444f
worsening, 454
Bronchiolar lining layer, 65
Bronchiole, light micrograph of, 66f
Bronchiolitis, 287, 420–426
associated with all respiratory viruses
high-risk population for, 420
population risk for, 420
clinical features of, 421–422
clinical scores, 422, 423t
disease severity, 422
imaging, 422
laboratory findings, 422
physical findings, 422
symptoms, 421–422
diagnosis of, 404, 422–425
differential diagnosis of, 422–425
epidemiology of, 420–421
etiologic agents with, 397t
etiology of, 421
guidelines for, 424, 424t
management of, 425
mechanical ventilation in, 553
pathology/pathogenesis of, 421
physical therapy, impact, 287
in preschool asthma, 677
prevention of, 425–426
prognosis of, 426
respiratory syncytial virus
high-risk population for, 421, 421f
population risk for, 420
treatment of, 425
Bronchiolitis obliterans (BO)
clubbing in, 19
in human immunodeficiency virus–infected
children and adolescents, 978, 978f
in pediatric lung transplantation, 988
Bronchiolitis obliterans organizing pneumonia
(BOOP), 850
Bronchiolitis obliterans syndrome, 938–939,
938f
in pediatric lung transplantation, 988
Bronchitis, protracted bacterial bronchitis,
456–459
Bronchoalveolar lavage (BAL), 139–142,
941
across studies
limitation, 1104–1105
lipid-laden macrophage index versus,
1102f
assays, 142b
bacterial presence, 443
cell profiles, usage of, 866
as diagnostic tool, 815
excessive volumes of topical anesthetic,
140
findings, interpretation of, 141–142
human defensins in, 126
in hypersensitivity pneumonitis, 950–951
increasingly utilized, 876
indications for, 139–140, 139b
performing, 141
processing of, 141
research application of, 142
samples of, 134
sampling, allowance of, 1088–1090
severe asthma and, 729
techniques for, 140–141
Bronchoalveolar lavage fluid (BALF), 61
from CF, 778–779
culturing, 772
in hypersensitivity pneumonitis, 948
surface tension lowering properties,
reduction of, 842
Bronchobiliary fistula, occurrence of, 539
Bronchoconstriction, 221–223
as airway reflexes, 120–121
inadequate, 66
Index 1167
Bronchodilation, 69
Bronchodilators
for preschool asthma, 683
response, assessment of, 189, 190f
use of, 379
Bronchogenic carcinoma, 1073
Bronchogenic cysts (foregut cysts), 1081–
1083, 1083f
asymptomatic characteristic, 1082
classification of, 1081
Bronchography, of atelectasis, 1031, 1032f
Bronchomalacia, 450f
Bronchoprovocation challenge testing
direct, 695
indirect, 695–696
Bronchopulmonary anatomy, nomenclature
of, 64f
Bronchopulmonary dysplasia (BPD),
367–381
altitude, 227
antiviral immunization, 379
bronchodilators, 379
cardiovascular abnormalities, 374–375
chest radiographs of, 368f
clinical findings of, 375
corticosteroid therapy, 377
definition of, 367–369, 369f
diagnostic criteria for, 369t
diuretics, usage, 379
drug therapies, 379
epidemiologic studies of, 370–371
epidemiology of, 369–370
etiology of, 370–372
genetic susceptibility, 371
histologic features of, 368f
HRQoL measure, 240
imaging of, 375
incidence of, 370, 370f
in infants, chest radiograph of, 373f
inflammation, 372–373
laboratory findings of, 375
late pulmonary hypertension, mortality
with, 374f
long-term outcome, 376
lung pathophysiology, 374f
oxygen toxicity, 372
pathogenesis, of, 372–373, 372f
pathologic features of, 369t
pathophysiology of, 373–375
physical findings of, 375
postnatal factors of, 371–372
prenatal factors of, 371
prevention of, 376–378
prognosis of, 380
pulmonary circulation, 373–375
pulmonary function tests, 375
pulmonary hypertension and, 357–358,
379–380, 560–561
respiratory disease with risk for, 370f
respiratory function, 373
steroids, use of, 379
systemic administration, adverse effects,
377
technology dependence, 385
treatment of, 378–380
ventilator-induced lung injury, 372
Bronchopulmonary secretion, removal of,
physical therapy and, 273
Bronchopulmonary sequestration, 296
Bronchoscope
care and maintenance of, 135
flexible, 134–135
manipulation of, 136
nomenclature, 134
rigid, 134
Bronchoscopic intubation, 145
1168 Index
Bronchoscopy, 134–146, 334–335
anesthesia for, 136–137, 138f
complications of, 145–146
contraindications to, 139
economic aspects of, 146
for examination of airway, 134
facilities for, 135
flexible, 136, 136t
instrumentation for, 134–135
rigid, 135–136
specimens, 400
techniques for, 135–136
therapeutic, 143–145
for vascular rings, 619
Bronchospasm, asthma and, 688
Bronchus, bronchoscope, wedged into, 140
Brownian motion/diffusion, 260
BRUE, 1125
definition and factors for inclusion and
exclusion, 1126t
Bruxism, 1154f
bteA, in Bordetella pertussis, 530t
B-type natriuretic peptide (BNP), usage of, 587
Budesonide, for asthma, 704
Bulbar musculature, weakness of, 384
Burkholderia cepacia complex (BCC) organisms,
982
Busulfan (Myleran), 879
BVG intermediate-phase, in Bordetella
pertussis, 530t
C Cardiomyopathy, in human immunodeficiency
CAD. see Congenital alveolar dysplasia
Caesarean section delivery, 251
Calcineurin inhibitor (CNI), 984
Calcitonin-gene-related peptide (CGRP), 112,
120–121
Calcium channel blockade, 573–575
epoprostenol treatment for, 564–565
Calcium channel blockers, use of, 380
Calcium-dependent chloride channels (ClCa),
777
Calor, inflammation and, 101
Calreticulin/CD91, actions of, 60
Camellote sign, 539
CA-MRSA. see Community-associated MRSA
Canalicular period, 28f, 30
Canalicular phase, 248
Canalicular stage, 70
Cancer therapy, cytotoxic drugs used in,
876–881, 877t
Candida species, 518
pulmonary infection due to, 929
Candidate gene approach, in asthma,
651–652
Candidiasis, pulmonary, 518–520
clinical features of, 519
diagnosis of, 519
differential diagnosis of, 519
epidemiology of, 518
etiology of, 518
imaging of, 519
laboratory findings on, 519
management and treatment of, 519–520
pathology/pathogenesis of, 518–519
prevention of, 520
prognosis of, 520
pulmonary function testing for, 519
CAP. see Community-acquired pneumonia
Capillaries
absolute concentration of reduced
hemoglobin in, 17
endothelial membrane of, 580
Capillary leak syndrome, 936
Capillary malformations, 329
Capnometry, 390–391
CAQ. see Childhood Asthma Questionnaire
Carbamazepine, 882
Carbohydrate recognition domain (CRD), 57
Carbon dioxide
addition of, to blood, 97
in blood, 96t
dissociation curve, 95f
level of, elevated, 18
in sensory feedback system, 99
transport, 94–97
Carbon monoxide
in diffusing capacity, 88
poisoning, 629, 629f
transfer, 254
Cardiac arrhythmia, 634
detection of, 21
sudden infant death syndrome and, 1139
Cardiac catheterization
in pediatric pulmonary arterial
hypertension, 571–572
studies of, 373
Cardiac disorders, congenital, 332
Cardiac effusions, in Down syndrome, 999
Cardiac failure, pulmonary edema and, 589
Cardiac glycosides, in pulmonary arterial
hypertension, 578
Cardiac output, decreased, 18
Cardiac surgery, pulmonary complications of,
625, 1002t
Cardiac trauma, 1067–1068, 1068f
Cardiac wounds, suspicion, 1067
virus infected children, 979–980
Cardiopulmonary bypass, pulmonary
complications related to, 625
Cardiopulmonary exercise testing (CPET),
213–214, 213f, 572
challenge, 215–216
modern, 214
normal values, 216
protocol, 214–215
variables, 215f
Cardiorespiratory compromise, 1109
Cardiorespiratory monitoring, use of,
390–391
Cardiorespiratory sounds, 12
Cardiothoracic ratio, 147
Cardiovascular abnormalities, 374–375
Cardiovascular signs, 19–21
Cardiovascular system, assessment of, 21
Care, pediatric/adult models (differences),
242
Carinal bronchogenic cyst, posteroanterior
chest radiograph (overexposure), 1082f
Carmustine (BCNU), 879
Carotid bodies, peripheral chemoreceptors,
location, 342
Carpal tunnel syndrome, 860–861
Cartilaginous congenital subglottic stenosis,
308
Cascades, 38
Caseating consolidation, in pulmonary
tuberculosis, 480, 481f
Case-control study, in asthma, 640–641
CASI. see Composite Asthma Severity Index
Casoni skin test, 540–541, 541f
Caspofungin, for mucormycosis, 523
b-catenin, in gene expression, 26
Cathelicidin, 126
CBAVD. see Congenital bilateral absence of the
vas deferens
CCAM. see Congenital cystic adenomatoid
malformation
CCHS. see Congenital central hypoventilation
syndrome
CCSP. see Club cell secretory protein
CD4/CD8 ratio, depressed, 880
CDH. see Congenital diaphragmatic hernia
Cefoxitin, for nontuberculous mycobacterial
infections, 504t
Cell adhesion, role of, 36–37
Cell proliferation, in pulmonary arterial
hypertension, 568
Cell shape, role of, 36–37
Cells
function of, 63
junctions (zonulae occludentes), 67
in pediatric allergic asthma, 665–669
Cell-to-cell contact, promotion of, 372–373
Cellular debris, 844
Cellular defenses, 126–129
Cellular distribution/function, 759
Centers for Disease Control and Prevention
(CDC), in quality of life, in asthma,
648
Central apnea, 1149–1152
Central chemoreceptors
development of, 1145–1146
impact of, 342
Central chemosensitive centers, multiplicity of,
1146
Central hypoventilation syndrome (CHS),
1149–1150, 1149b
Central intracellular R domain, presence,
758–759
Central nervous system (CNS), programmed
cell death, 1137–1138
Central nervous system disease, in
extrathoracic tuberculosis, 483–484
Centrilobular nodules, in hypersensitivity
pneumonitis, 949–950
Centrioles, cilia derivation, 63–65
Cephalosporin, 414
Cerebral hydrogen 1 magnetic resonance
spectroscopy, 638f
Cerebrocostomandibular syndrome,
1005t–1006t
Cervical injury, impact of, 384
Cervical lymphadenopathy, 418
Cervicomediastinal lymphangioma, diagnosis
of, 1079
Cetuximab, 880–881
CF. see Cystic fibrosis
CF transmembrane conductance regulator
(CFTR), 777
C-fiber receptors, 69
CFRD. see Cystic fibrosis-related diabetes
mellitus
CFTR. see CF transmembrane conductance
regulator
CFTR-related metabolic syndrome (CRMS),
773–774
CGD. see Chronic granulomatous disease
CGRP. see Calcitonin-gene-related peptide
Challenge materials, studies, 740
Challenge to personalizing therapy, for
preschool asthma, 680
CHAOS. see Congenital high airway
obstruction syndrome
Charcot-Leyden crystal protein, 953
CHARGE association, 1099–1100
Charles’ law, 74
Chemicals, nonallergic hypersensitivity to,
asthma and, 693–694
Chemokines, 105, 110–111, 111f, 121
Chemoprophylaxis, for influenza, 464
Chest
action, diagram of, 1063f
auscultation, 10–14
performing, 12
cardiovascular signs in, 19–21
 Index 1169

Chest (Continued) Chest wall disorders (Continued) Children (Continued)

circumference management of, 1058–1061 airway obstruction in, pulmonary edema
distribution of, 7f pathogenesis of, 1048–1049 and, 587
mamillary level of, 6 management of, 1054–1061 anterior malignant teratoma,
configuration, objective documentation of, 6 pathogenesis of, 1047–1049 posteroanterior/lateral radiographs of,
deformities, 6f Chest wall dysfunction 1078f
expansion, symmetry of, 8–9 causes of, 1047t asthma and, HRQoL measures, 236t–237t
fluid-filled cysts in, CT confirmation of, 541f general management of children with, auscultation of, 10–14
high-resolution computed tomography scan 1054–1055 body mass index (BMI), 216
of, in hypersensitivity pneumonitis, respiratory function, assessment, breathing of, rhythm of, changes in, 4–5
949f 1046–1047 bronchiectasis, etiologies (studies), 443t
inspection, palpation and, 8 Cheyne-Stokes breathing, occurrence of, 5 bruxism, 1154f
movement of, 5 Chiari malformation, 384–385 cardiopulmonary exercise testing, 215–216
percussion of, 9–10 ChILD. see Childhood interstitial lung disease CFQ-R profile, 233f
performing, 9f Child Health and Illness Profile, 232 chest disease in, signs and symptoms of,
radiographic examination of, 1087–1088 Childbirth, pregnancy and, asthma on, 14–23
surface of 653–655 chest pain in, causes of, 23b
pulmonary lobes on, projections of, 13f fetal growth and birth size, 654–655 chest percussion for, 9
symmetric sites of, 9 maternal factors, 653–654 chest radiography for, 147–148
tube insertion, 1065 maternal lifestyle and environment, chest wall dysfunction
tumors of, 1072–1092 654 general management of, 1054–1055
epidemiology of, 1072 mode of delivery, 655 respiratory function, assessment,
etiology of, 1072 Childhood. see also Children 1046–1047
vertical reference lines of, 9f asthma chronic anticoagulation in, consideration of,
Chest disease, in children, signs and symptoms diet and, 656 572–573
of, 14–23 management of, 110 chronic aspiration, prevalence, 1099
cardiovascular signs, 19–21 outcomes of, 646 chronic mechanical ventilation,
chest pain, 22–23, 23b chronic granulomatous disease, 909 requirement of, 382–383
cough and sputum, 14–16, 16t genetic variation, lung disease and with chronic respiratory diseases, 3
cyanosis, 17–18, 18f interpretation of, 43 chylothorax, causes, 1023t
digital clubbing, 18–19, 19b, 20f research study designs to, 44–46, 45t community-acquired pneumonia (CAP),
exercise limitation and dyspnea, 21, 22f technologies to identify, 41–43 antibiotics, 433
noisy breathing, 16, 17b type of, 40–41, 41f, 43t cyanosis in, 17–18
wheezing, 16–17, 17b, 18f lung disease and, 40–48, 41f, 42t with cystic fibrosis
Chest high-resolution computerized clinical genetic testing, 44, 44f HRQoL measures, 238t
tomography (c-HRCT) OSA, adenotonsillar hypertrophy, 1154 infant pulmonary function testing for,
irreversible dilatation, 439–440 pulmonary arterial hypertension, 556–579 814–815
radiological features, 439 sternum, fractures of, 1063 diagnosis, tachypnea (usage), 429
scans, timing, 440 systemic inflammatory disease of, diffuse alveolar hemorrhage in, 893–902,
Chest pain, 22–23 pulmonary involvement in, 850–875 894f
causes of, 23b approach to, 874b anti-GBM disease, 897–898, 899f
treatment of, oral/sublingual nitrates in, 578 diagnosis/management of, 873–874 approach to, 899–901
Chest physiotherapy, 287, 1031 serologic test for, 851t causes of, 894b
Chest radiography, 333, 333f significant, 872–873 clinical manifestations of, 901t
for atelectasis, 1029–1030 systemic scleroderma in, rarity of, 860 COPA syndrome, 899
for bronchiolitis, 422 taste and smell, examination of, 14 diagnosis of, 899–901
features of, 147–148 Childhood Asthma Management Program etiology of, 893–899
lateral view of, 150f (CAMP) research group, 646 immune-mediated, 893–897, 898f
for pulmonary edema, 586–587 Childhood Asthma Questionnaire (CAQ), 234, isolated pulmonary capillaritis, 898
systematic review of, 148–156 236t–237t nonimmune-mediated, 899
for vascular rings, 619 Childhood Asthma Research and Education pathophysiology of, 893–895
Chest wall Network, study on asthmatic children by, treatment of, 901–902
anterior axillary fold and, 148 708 digital clubbing in, 18–19
augment inspection of, 6 Childhood Cancer Survivor Study, bleomycin, disease in, diagnosis of, 2
compliance, increase of, 1148–1149 multivariate analyses of, 877–878 with Down syndrome, 992
elastic properties of, 76, 77f Childhood interstitial lung disease (ChILD), drowning in, 637–638, 638f
function, assessment, 1046b 837–838, 999 drug administration, by inhalation,
injury, tracheostomy in, 1064 consideration of, 825 257–272
movements, 1046 diagnosis of, 826 exercise, physiological responses, 216, 217f
in newborn, 1044, 1045f forms of, 834t exercise-induced bronchoconstriction, 219f
respiratory muscle disorders and, lung growth abnormalities presenting as, exposure pathways in, 50t
1044–1061 826–828 gastroesophageal reflux, commonness, 1101
considerations in, 1044–1045 more prevalent in infancy, 825–835 heavy nocturnal snoring in, clinical
tumors, 1073–1074 overview of, 825 symptoms of, 17b
Chest wall deformities Childhood Onset of Asthma (COAST) study, in hemothorax, 1025
prevention of, 284 asthma, 658–659 immunocompromised, pulmonary infections
visual perception of, 6 Childhood vasculitides, 867–869 in, 923
optical markers in, 6f classification of, 868t approach to, 940
Chest wall disorders, 287 pediatric classification scheme, European clinical presentation of, 923–924
clinical features of, 1049–1053 League Against Rheumatism/Paediatric fungal pathogens, 927–930
congenital, 329–332 Rheumatology European Society infectious agents in, 924–931
diagnosis of, 1053–1054 development, 867 invasive diagnostic studies for, 941–942
etiologies of, 1046–1047 Childhood-onset myotonic dystrophy, 1051 noninvasive diagnostic studies for,
involving bony structure of Children 940–941
clinical features of, 1051–1053 with ABPA, immunotherapy for, 958 parasitic agents, 931
diagnosis of, 1054 airway mucosa abnormality, 450f prevention strategies for, 942
1170 Index
Children (Continued)
pulmonary coinfections, 931
radiographic testing for, 940, 940t
viral pathogens in, 924–927
indications and special considerations for,
175–176
intrathoracic lipoma rarity in, 1079
laryngeal anatomy of, 406
lung sounds of, objective acoustic
measurements of, 11
lungs in, adults and, contrast between, 609
malnutrition in, 3
mechanical ventilation in, studies on, 610
medical history of, 3
methacholine bronchial provocation in, 18f
nasal interfaces for, 389
nasal passageways of, inspection of, 16
neck, adenoid tissue enlargement/OSA,
lateral roentgenogram, 1153f
neurological injury in, 637
obesity, 215–216
asthmatic, 219
obstructive sleep apnea
conditions, 1154–1156, 1156b
polysomnographic measures, 1152f
with oligoarticular disease, ANA presence
in, 850–852
palpation in, 8–9
physical activity
biological importance, 212
levels, reduction, 212
physical development of, 3
physical inactivity, effects, 212
pneumonia in, 427–438
posteroanterior film, 147
prior tests and immunizations in, detailed
report, 4
psychosocial concerns for, 983
REM-associated obstructive apneic events,
polygraphic tracing, 1153f
respiratory symptoms, presentation of, 3
respiratory tract, diseases in, 3
secondary pneumothorax of, 1010t
sleep-disordered breathing,
polysomnographic montage, 1157b
sleep-related breathing disorders, HRQoL
measures, 239t
study of CPET in, 216
with suspected tumors of chest, general
approach to evaluation of, 1087–1090
systemic inflammatory disease in,
pulmonary manifestations of, 851t
systemic lupus erythematosus (SLE) in
pulmonary hemorrhage, rarity of, 855
pulmonary hypertension, rarity of,
855–856
technical and staff requirements for,
175–176, 176f
traumatic thoracic injury in, 1062
tuberculosis in, 478, 478t, 479f
with Turner syndrome, 839–840
upper airways of, partial obstruction of, 16
upper respiratory tract infection,
posteroanterior chest radiograph,
1084f
vulnerability of, to adverse environmental
exposures, 49–54
Children’s interstitial lung disease (chILD)
bronchoalveolar lavage (BAL) in, usage of, 815
bronchoscopy in, usage of, 815
classification of, 813–814
diagnostic approach of, 814–816
diffuse pediatric lung disease classification,
proposal, 814t
families and physicians, resources for, 816
genetic testing in, 815
Children’s interstitial lung disease (chILD)
(Continued)
lung biopsy for, 816
new concepts in, 813–816
noninvasive testing, completion of, 814
retrospective studies in, 814
terminology in, 813–814
Children’s Interstitial Lung Disease Research
Network (ChILDRN)
in lung biopsy identification, 825
study, 829–830
ChILDRN. see Children’s Interstitial Lung
Disease Research Network
Chlamydia organism, 106
Chlamydia pneumoniae, infections, occurrence,
427
Chloral hydrate, for infant sedation, 175
Chloramphenicol, intravenous, 414
Chlorine gas, inhalation of, 591
Cholesterol needles, 844
Chondromas, 1074
“Chord” compliance (Cchord), 193–194
c-HRCT. see Chest high-resolution
computerized tomography
Chromatin state, 34
Chromatin topology/3D structure, 34
Chromosomal microarray (CMA), 41, 45t
Chronic anticoagulation, consideration of,
572–573
Chronic aspiration
causes of, 1098f
histopathology of, 1098f
lung injury, 1104f
multidisciplinary approach, 1104–1105
rigid bronchoscopy, consideration, 1104
Chronic asthma
clubbing in, 19
management of, 711, 712f
sample treatment plan for, 716f
Chronic cough
causes of, 16t
long-term risk for, 878
Chronic disease, self-management, 244
Chronic eosinophilic leukemia, not otherwise
specified, criteria for, 965b
Chronic eosinophilic pneumonia, 960–961
Chronic eosinophilic pneumonitis,
characteristics of, 959t
Chronic granulomatous disease (CGD),
909–917
chest roentgenogram for, 911f
clinical features of, 909–910
clinical findings in, 910t
defect in phagocyte function and, 909
diagnosis of, 911–913
encapsulated pneumonia in, 912f
fluorescence-based screening assays on, 913
gene therapy for, 916–917
hematopoietic cell transplantation for,
916–917
“honeycombing” in, 911
inheritance, 914–915
laboratory findings on, 911–913
management of, 915–916
molecular defects in, 914–915, 914t
nitroblue tetrazolium (NBT) test for, 913
positive screening test for, 913
pulmonary complications of, 910–911
pulmonary disease, expression of, 911
pulmonary infections, range of microbial
agents causing, 910–911, 911t
purulent rhinitis, clinical features, 909
registry, patient enrollment for, 909, 910t
screening tests for, 911, 913
Chronic hypersensitivity pneumonitis, 949
Chronic illness, 2
Chronic infection, in human
immunodeficiency virus–infected children
and adolescents, 977
Chronic inflammation, 102–103, 102f
Chronic interstitial lung disease, gene
mutations and, 38–39
Chronic lung allograft dysfunction, pediatric
lung transplantation, 988–989
Chronic lung disease (CLD)
in human immunodeficiency virus–infected
children and adolescents, 969b,
975–979
factors associated with, 976f
lung function in, 979
of prematurity, infant pulmonary function
testing for, 814–815
Chronic lung injury, development of, 1101
Chronic mechanical ventilatory support, need
for, 385–386
Chronic myelogenous leukemia, Busulfan
(Myleran) for, 879
Chronic nasal symptoms, differential diagnosis
of, 741t
Chronic neonatal lung disease, 828–829
Chronic obstructive pulmonary disease
(COPD)
chronic inflammation and, 102
development of, 380
end stage, 255
misdiagnosis, 440
Chronic otitis media, severity, 1041
Chronic pulmonary aspiration, 1097
conditions associated, 1099b
evaluation/treatment, 1104
peripheral neurologic diseases and injury,
impact, 1099–1100
Chronic pulmonary tuberculosis, 481,
482f
Chronic respiratory disorders, 1097
services for, 245
Chronic respiratory failure
causes of, 382
follow-up, patient, 391–392
mechanical ventilation, reduction, 391
noninvasive ventilation for, 386–387
treatment of, 386–389
Chronic respiratory muscle fatigue,
identification (problems), 1045–1046
Chronic respiratory support, epidemiology of,
382–383
Chronic rhinosinusitis, 742–746
clinical features, 743, 743f
with nasal polyps, 743, 744f
Chronic sinusitis
asthma
association, 745
pathophysiologic links, 745
clinical features, 743
histopathology, 743–744
microbiology, 744
Chronic suppurative lung disease (CSLD),
439–459, 440f
bronchiectasis, contrast, 439
clinical syndrome, 439
comorbid conditions, 447–448
definition, 439–440
diagnosis, delay, 447
diagnostic criteria, 448
diagnostic evaluations, 448–452
economic cost, 441
epidemiology of, 440–444
etiologic risk factors, 441
genetics, 444
macro malnutrition/micro malnutrition,
impact, 443–444
pathology/pathophysiology, 444–447

Chronic suppurative lung disease (CSLD)
(Continued)
prevalence, 440–444
public health issues, 443–444
upper airway infection/aspiration, 443
Chrysosporium spp., 525–526
CHS. see Central hypoventilation syndrome
CHSA, 236t–237t
Churg-Strauss syndrome, 962–964
characteristics of, 963b
Chyle fluid, 1021–1022, 1022f
Chylomicrons, chylothorax, 1021–1022
Chylothorax, 1021–1025
associated with Down syndrome, 999
in cardiac surgery, 1002t
clinical manifestations of, 1023
etiology, 1022–1023
investigations of, 1023–1025, 1024f
lymphatic system, 1021–1022
management of, 1025
neonate, chest x-rays, 1024f
pharmacologic therapy of, 1025
physical and chemical characteristics,
1021b
traumatic, 1023
Chylous leak, identification of specific site of,
1023–1025
Ciclesonide, for asthma, 705
CIE. see Counterimmunoelectrophoresis
Cilia, types of, 1035
Cilia-dependent mucus clearance, 122
Ciliary beat, frequency of, 122
Ciliary dyskinesia, 256
Ciliated cells (CiCs), 63–65, 66f
Cincinnati Children’s Hospital Medical Center
aerodigestive model, 1094
Clarithromycin, for nontuberculous
mycobacterial infections, 504t
“Classic” hypereosinophilic syndrome, 965
Classical cystic fibrosis, 775
ClCa. see Calcium-dependent chloride
channels
Cleft, laryngeal, 306
type 1, 306
type 2, 306
type 3, 306
type 4, 306
Cleft palate, inspection of, 6–7
CLHL. see Congenital large hyperlucent lobe
Climate, in respiratory disease, 55
Clinic visits, HRQoL assessments (example),
234
Clinical genetic testing, 44, 44f
Clinical microbiology laboratory, 396–404
Clinical scores, for bronchiolitis, 422, 423t
Clofazimine, for nontuberculous mycobacterial
infections, 504t
Closing volume, 81
Clotting abnormalities, in single ventricle
physiology, 624
Club cell secretory protein (CCSP), 65
Clubbing, 448
digital, 18–19, 20f
nonpulmonary diseases associated with,
19b
in physical findings, 6–7
Cluster of differentiation 14 (CD14), in
asthma, 653t
CMA. see Chromosomal microarray
CMD. see Count median diameter
CMV. see Cytomegalovirus
CNI. see Calcineurin inhibitor
Coatomer-associated protein alpha (COPA)
syndrome, 899
Coccidioides immitis, 512
Coccidioides posadasii, 512
Coccidioidomycosis, 512–514
clinical features of, 512–513
diagnosis of, 513
differential diagnosis of, 513
epidemiology of, 512, 512f
etiology of, 512
imaging of, 513
laboratory findings on, 513
management and treatment of, 513
pathology/pathogenesis of, 512
prevention of, 513
prognosis of, 513–514
pulmonary function testing for, 513
Cochrane systematic review, 379, 410
Cognitive capacity, changes, 232–233
Cognitive risks, of bronchoscopy, 146
Cohen’s classification system, of laryngeal
web, 305
Cohort study, in asthma, 640–641
Colistin, 268, 269f
Collagen-like triple helix, formation of, 57
Collapse-consolidation lesions, 479–480, 480f
Collectin locus, 57
Collectins, 125
Combination therapy, 804–805
Combined cellular and humoral
immunodeficiencies, 920
Comfort, mechanical ventilation and, 553
Commercial inhalers, 258, 259f
Common cold
etiologic agents with, 398t
manifestations of, 404
Community variable immunodeficiency
disorders, 917–920, 918t
clinical features of, 917–918
diagnosis of, 919
inheritance, 919
laboratory findings for, 919
management of, 919–920
molecular defects of, 919
pulmonary complications of, 918
Community-acquired pneumonia (CAP)
agents, impact, 428t
antibiotics
choice, 432–433
treatment, 433t–434t
antigen, 430
antimicrobials, treatment, 432–433
bacterial resistance, problem, 432
chest radiographs, impact, 435
clinical features of, 428–430
diagnosis of, 431
differential diagnosis of, 431
epidemiology of, 427–428
etiologic diagnosis of, 430–431
etiology of, 427–428
health problems, 427
hospitalized patients, 432
laboratory tests, 429
management of, 432
pathogenesis of, 428
physical findings of, 429
polymerase chain reaction, 431
prevention of, 437
prognosis of, 437–438
radiologic findings of, 429–430
serologic tests for, 430
sputum examination for, 430
treatment of, antibiotic dosages, 434b
tuberculosis, consideration, 431
Community-associated MRSA (CA-MRSA),
433, 435
Comorbidities, extrapulmonary, asthma and,
724–726
Comparative auscultation, 12
Complement proteins, in lung defense, 125
Index 1171
Complement-derived chemoattractants,
778–779
Complete blood cell count, for asthma, 696
Complete tracheal rings, 1124
Compliance, 189–190
of lungs, 75–76
measurement of, 189–205
forced oscillation technique in, 199–205,
200f–201f, 203f–204f
general considerations for, 194,
194f–195f
interpretation of, 191–194, 191f–193f
measurement of interrupter resistance in,
196–198, 197f
physiological principles and assumptions
in, 190–191
plethysmographic measurement of
airway resistance and, 198–199,
199f
single-breath occlusion technique,
194–196, 195f
Composite Asthma Severity Index (CASI),
701
Compression phase, 121
Computed tomography (CT), 149–150, 157,
157f, 334, 335f
for cystic fibrosis, 792, 792f
for pulmonary edema, 587, 588f
for vascular rings, 619
Computed tomography (CT) angiography, for
pulmonary embolism, 889
Confidentiality, establishment, 243
Congenital abnormalities of the trachea,
309–310
Congenital alveolar capillary dysplasia, 327
Congenital alveolar dysplasia (CAD), 999
Congenital alveolar proteinosis, term, 840
Congenital atelectasis, 1027
Congenital bilateral absence of the vas
deferens (CBAVD), 767–768
Congenital central hypoventilation syndrome
(CCHS), 384–385, 1149–1150
clinical management of, 1150–1151
clinical manifestations of, 1150
clinical presentation of, 1150
diagnosis of, 1150–1151, 1151b
genetic origin, 1150
Congenital cystic adenomatoid malformation
(CCAM), 289, 425
Congenital diaphragmatic hernia (CDH), 58,
291–292, 329–331, 330f
outcomes of, 293t
specific diagnoses of, 292–298, 293f
Congenital gastrointestinal defects, in Down
syndrome, 1003
Congenital heart disease
associated pulmonary arterial hypertension
with, 561–572
in Down syndrome, 1000–1001
mechanical ventilation in, 553
pediatric patients with, special pulmonary
considerations in, 604
pulmonary disease associated with,
615–625
with Fontan repair of single ventricle
physiology, 624–625
pulmonary hypertension and
postoperative pulmonary
hypertensive crisis, 619–621
pulmonary vein stenosis, 621–622,
622f–623f
related to cardiac surgery and
cardiopulmonary bypass, 624–625
reperfusion pulmonary edema, 622–624
vascular rings and slings, 615–619,
616f–618f
1172 Index
Congenital heart disease (Continued)
pulmonary hypertension and postoperative
pulmonary hypertensive crisis
background of, 619–620
evaluation and monitoring of, 621
pathophysiology of, 621
risk factors of, 620–621
treatment of, 621
vascular rings and slings
clinical presentation of, 619
diagnosis of, 619
treatment of, 619, 619f–620f
Congenital high airway obstruction syndrome
(CHAOS), 291–292, 304–305
Congenital large hyperlucent lobe (CLHL), 290
Congenital lesions, bronchiolitis and, 425
Congenital lobar emphysema, 65–66,
320–321, 320f–321f
Congenital lung disease
age-related presentations of, 290–291, 291t
antenatal presentation of, 290–292, 291t
in childhood and adult, presentations of,
312–313
clinical approach of, 289–290
clinical/pathologic descriptions of, 290
epidemiology of, 290
systematic approach in, use of, 290
Congenital lung malformations, clinical
appearances, 290t
Congenital myasthenic syndromes, 1047,
1050, 1053, 1056
Congenital pulmonary airway, 1027
Congenital pulmonary airway malformation
(CPAM), 314–316
antenatal diagnosis and prognosis of,
294–295, 295f
antenatal treatment options of, 295–296
rare, 316
type 0, 314
type 1, 314–315, 315f
type 2, 315–316
type 3, 316
type 4, 316, 316f
Congenital pulmonary lymphangiectasis,
histopathology in, 999, 1000f
Congenital scoliosis, 1048, 1052, 1055f,
1059
Congenital small lung (CSL), 290
Congenital thoracic malformation (CTM),
314
antenatal diagnosis of, 292b
diagnosis
bronchopulmonary sequestration,
296
CPAM, 294–296
long-term follow-up cystic, 320
malignant transformation, prevention of,
318
management of, 297–298
optimizing lung growth, 318
postnatal treatment decisions in, 316–317,
317f
presentation of, in postnatal period,
296–298, 297f
prevention of complications of, 317–318
requirements for fetal surgery for, 296b
Congenital tracheal stenosis, 310, 310f–311f
Congenital tracheobronchomegaly, 312
Congenital vocal cord paralysis, 1121
Congestive heart failure (CHF) identification,
BNP usage in, 587
Congestive vasculopathy, 899, 900f
Connective tissue disease, mixed, 862–863
Constipation, 795
Continuation phase, of multidrug therapy, for
pulmonary tuberculosis, 493
Continuous positive airway pressure (CPAP), Crack cocaine users, in Loeffler’s-like
274f, 310, 367 syndrome, 958
application of, 339, 346 Crackles, 11, 13–14
patient transfer, 284 CRD. see Carbohydrate recognition domain
usage, 273, 388 C-reactive protein, 60–61
Continuous spontaneous ventilation, 550 Crescent sign, 539
Controlled mechanical ventilation, 1064 Cribriform membrane, 1007–1008
Controlled ventilation technique, 165 Cricopharyngeal achalasia, aspiration
Conventional ventilation opportunities, 1099–1100
mechanical, in acute respiratory distress Cricotracheal resection (CTR), 308
syndrome, 609–610 Critical closing pressure (Pcrit), 1147
oscillatory versus, in pediatric intensive care, Critical exercise-asthma, 218
596–598, 598f CRMS. see CFTR-related metabolic syndrome
Coordinate care Cross-sectional study, in asthma, 640–641
aerodigestive model health care value for Croup, 406
complex patients through, 1094–1096 diagnosis of, 404, 409
value in, 1096 humidification, 410
Copenhagen Study on Asthma in Childhood pharyngitis associated with, 397t
(COPSAC), in asthma, 659 recurrent or spasmodic, 409
Cor pulmonale, 19–21, 781 supportive care for, 410
Cord blood studies, 254 Cryptococcosis, 520–522
Correctors, 804 clinical features of, 521
Corticosteroids, 377, 879 diagnosis of, 521–522
for acute respiratory distress syndrome, 612 differential diagnosis of, 522
DMD usage, 1058 epidemiology of, 520
inhaled etiology of, 520–521
adverse effects of, 706 imaging of, 521
asthma and, 703–704, 704t laboratory findings on, 521
mechanism of action and clinical use of, management and treatment of, 522
705–706 pathology/pathogenesis of, 521
systemic, for asthma, 706–709 prognosis of, 522
use of, 410 pulmonary function testing for, 521
Costimulatory molecules, aid of, 105 Cryptococcus, 520–521
Costophrenic angles, blunting, 148 Cryptococcus gattii, 520–521
Cotrimoxazole prophylaxis, in human Cryptococcus neoformans, 520–521
immunodeficiency virus infected children, pulmonary infection and, 930
970, 975t Cryptogenic organizing pneumonia, 939
Cotton-Meyer grading system, 308 Cryptosporidium parvum, 930
Cough C-shaped rings, 65–66
anterolateral right upper lobe and, bilateral CSL. see Congenital small lung
circumscribed densities in, 540f CSLD. see Chronic suppurative lung disease
cardinal manifestation of, 14–15 CTM. see Congenital thoracic malformation
cause of, 15 CTR. see Cricotracheal resection
in chest disease in children, 14–16 C-type lectin receptors, 124
dependent clearance, 122 Culture, 401–402
detailed history of, 15 Cumbo sign, 539
expulsion of, 121 Cumulative dose (PD20), 695
in hydrocarbon aspiration, 627 Curvularia spp., 526
irritant and, 121 Cutaneous lesions, in Churg-Strauss
manually assisted, 284f syndrome, 962–963
mechanics of, 121 Cutaneous or mucocutaneous stimulation, 98
production, 14 Cutis laxa, 312
reflex, abnormalities in, 121 CXC chemokines, 111
spikes, superimposition, 283f CXCL1 and CXCL8, 108
strength (measurement), peak cough flow Cyanosis, 17–18
(usage), 283 BRUE definition and factors for inclusion
Cough-assist, 599 and exclusion, 1126t
devices, 121, 285f clinical impression of, 18
Cough-variant asthma, 690–691 in physical findings, 6–7
Coughing reduced hemoglobin, requirement, 18f
act, components, 283 Cyclic adenosine monophosphate (cAMP),
components, impairment, 283 777
compression phase of, 121 response element, 757
expulsive phase of, 121 Cyclic GMP
glottic closure, 121 augmentation, 380
inspiratory phase of, 121 breakdown, prevention of, 577
irritation phase of, 121 Cyclophosphamide, 878–879
phases of, 15, 121 Cyclosporine, 984
relaxation phase for, 121 Cysteine leukotrienes, in asthma, 667
timing of, importance of, 15 Cysteinyl leukotrienes (cysLTs), 709
Count median diameter (CMD), 261 bronchiolitis and, 421
Counterimmunoelectrophoresis (CIE), 949 Cystic adenomatoid malformation, 289
CPAM. see Congenital pulmonary airway Cystic fibrosis (CF), 256, 439
malformation airway
CPAP. see Continuous positive airway pressure clearance, 782–783
CPET. see Cardiopulmonary exercise testing inflammation, 766
 Index 1173

Cystic fibrosis (CF) (Continued) Cystic fibrosis (CF) (Continued) Cystic fibrosis transmembrane conductance
allergic bronchopulmonary aspergillosis in, nonclassic forms (mild forms), mutations regulator (CFTR) (Continued)
952b (functional consequences), 762f postnatal expression/function, 759
altitude, 227 nonpulmonary manifestations of, 788–799 role, importance, 763
antenatal testing, 772–773 constipation, 795 splicing, 757–758
antibiotic therapy for, 783–784 distal intestinal obstruction syndrome structure, 757
antiinflammatory therapy, 784–785 (DIOS), 796 three-dimensional topology, 759
aspergillosis in, 955 fibrosing colonopathy, 790 tissue distribution, 759
at-risk couples, preimplantation diagnosis, gallbladder disease, 794 transcription, 760
773 gastric emptying, 796 modulators, small molecule, 800–801
atypical, 775 GI cancers, 797 candidate drugs, identifying, 800, 801t
diagnosis of, 762 hepatobiliary disease, 791–794, 791f on protein expression, maturation, or
bacterial colonization, 763 infertility, 794 function, 800–801, 802f
bronchiectasis, 101 nutrition in, 797–798, 798f, 798t protein with, 801, 802f
chest radiograph, 765f–766f pancreatic disease, 788–790 mutations, 771b
chronic mechanical ventilation for, 385 pancreatitis, 791 analysis, 771–772
chronic sinusitis, 768 rectal prolapse, 796 clinical consequences, 761–762, 772t
classical CF, 775 small bowel motility, 796 molecular consequences, 760–761
clinical features of, 780–782 small intestine bacterial overgrowth, severity, 775
clinical suspicion, 769, 770b 796–797 protein, 758–759
diagnosis of, 769–776 pancreatitis and, 768 characteristics, 758–759
diagnostic criteria, 769 pathogenesis of, 777–780, 778f–779f in small bowel, 794–795
list, 770b pathophysiology of, 757–768 Cystic fibrosis transmembrane conductance
DNA testing, sensitivity (enhancement), percussion and, 279f regulator-related disorders, 775, 775b
771 phenotypes, 775–776 Cystic fibrosis transmembrane conductance
emerging therapies, 786 physical activity and, 223–225 regulator-related metabolic syndrome,
end organ involvement, assessment, positive expiratory pressure and, 280f–281f 775–776
772 presentation of, 769–776 Cystic fibrosis-related diabetes mellitus (CFRD),
energy imbalance, development model of, preventative care for, 785–786 798–799
798f prognosis of, 383, 786–787 Cystic hydatidosis, of lung, occurrence of,
environmental factors, 773–774 pulmonary disease in, 777–787 539–540
epidemiology of, 777 reproductive tissues, 767 Cystic hygroma, 301, 301f
etiologic agents with, 398t respiratory physiotherapy, 274–283 confinement of, 1079
etiology of, 777–780, 778f–779f result, respiratory epithelial inducible Cystic lung lesions, 292t
exercise, 223–225 chloride channel, 122 outcomes and postnatal management in,
reduction, 224 spirometry for, 188 298t
on trampet, 282f sweat gland effects, 767 Cystic masses, impact, 1076–1077
fitness levels, 223f sweat test, 769–771, 770b Cystic teratomas, commonness, 1076
gastrointestinal disease, 766–767 symptoms and physical findings, 780–781, Cytochrome b558, 913
gender differences, 223 780f Cytochrome oxidase, decrease in, 590
gene transepithelial potential difference Cytochrome P-450-dependent detoxification,
clinical features of, 770b measurements, 772 65
mutations and, 38–39 transmembrane conductance regulator, 788 Cytokines, 109–110, 109f, 111f, 121
genetic basis, 757 modulators, 784, 785f antiinflammatory actions of, 113
genetics, 757–768 treatment of, 782–786, 783f in pediatric allergic asthma, 665–669
HRQoL measures, 235–237 ultrasonic nebulizer and, 282f release of, 106–107
list, 238t Cystic fibrosis gene therapy, 805–810, 805b Cytomegalovirus (CMV), 924–926
imaging and laboratory studies, 781 lung and, 806 clinical manifestations of, 987
infant, physiotherapy, 279f outstanding questions on, 808–810 incidence of, 987
infection, 404, 766 recent progress in, 806–808, 807f pneumonia, 925, 973
inhaled mucolytics for, 783 vector development and upcoming clinical antibiotic treatment of, 970t
ion transport in, 122 trials of, 808, 808f–809f reduced, significance of, 984
8-isoprostane in, increased concentration of, viral versus nonviral, 806 Cytosine arabinoside, 880
111 Cystic fibrosis liver disease (CFLD), 791 Cytosolic oxidase components, 914
kyphosis, onset, 274f clinical findings in, 791 Cytotoxic drugs, 880–881
liver disease and, 791 early diagnosis of, 792 usage, 876–881, 877t
lung disease and, 782 Cystic Fibrosis Quality of Life Questionnaire
lung transplantation and, 786 (CFQoL), 238t
management of, 782–786, 783f Cystic Fibrosis Questionnaire-Revised D
measures, 235–239 Respiratory Symptom Scale (CFQ-R), 231, DAH. see Diffuse alveolar hemorrhage
recommendation, 239 233f, 238t, 239 Daily preventive monotherapy, for preschool
molecular therapies of, 800–811 Cystic Fibrosis Respiratory Symptom Diary asthma, 680–681
challenges for, 810–811 (CFRSD) measures, 237–239, 238t Daily therapy dose, for preschool asthma, 683
combination therapy, 804–805 Cystic fibrosis screen positive, inconclusive Daily versus preemptive asthma controller, for
correctors, 804 diagnosis, 775–776 preschool asthma, 683
declaration of interests in, 811 Cystic fibrosis transmembrane conductance Dalton’s law, 74
evaluation of, 810 regulator (CFTR), 757, 788 Data processing, 158–161
gene therapy, 805–810, 805b control, 762–763 Daytime sleepiness, excessive, 1151–1152
“Go/No-Go” decision point, 809 dysfunction of, 788 DC. see Dyskeratosis congenita
potentiators, 803–804 gene, 757–758 DCTN4 (dynactin subunit 4), 45
read-through agents, 801–803 biogenesis, 759 De novo SFTPC mutations, impact of, 845–846
transmembrane conductance regulator cellular distribution/function, 759 De novo synthesized lipid mediators, 953
modulators, small molecule, developmental expression/function, 759 Dead space, 86–88
800–801, 808 function, alteration, 757–758 Death
mutations causing, 771t other disorders, 767–768 in asthma, 647
neonatal screening, 773–774 polypeptide, translation, 759 in pediatric lung transplantation, 990, 991f
1174 Index
Death inducing signaling complex, 132–133
Decannulation, achievement, 1121
Deep inspiration, 283
in coughing, 15
Deformities, stereographic measurements, 6
Delamanid, for drug-resistant tuberculosis,
494
Deletions, 41
Delivery room resuscitation, 339–341
Dendritic cells (DCs), 105, 126–127
in asthma, 665
Dennie lines, 6
Deoxyribonuclease (RhDNase), mucoactive
agents, 282
Deoxyribonucleic acid (DNA), polymerized
actin and, 122
Dermonecrotic toxin, in Bordetella pertussis,
530t
Dexamethasone, use of, 410
Diagnostic bronchoscopy, indications for,
137–139, 138t, 139f
Diaphragm
chest pieces, placement of, 12
dysfunction of, 1044–1045
flattening of, 148
in muscles of respiration, 83
paralysis of, 384
penetration of, 1085
relaxation of, in coughing, 15
traumatic blunt rupture of, 1070, 1070f
tumors of, 1087
Diaphragm paralysis
clinical features of, 1049
diagnosis of, 1053
management of, 1055
pathogenesis of, 1047
treatment of, 1055
Diaphragmatic dome, 63
flattening of, 148
Diaphragmatic dysfunction, ventilator
induced, 554
Diaphragmatic eventration, 332
Diaphragmatic hernia, 292–298
Diaphragmatic muscle, in muscles of
respiration, 84
Diaphragmatic paralysis, in cardiac surgery,
1002t
Diaphragmatic weakness, 1051
Diastolic murmurs, appearance of, 19–21
Dichotomous branching, 63
Diet, maternal, on asthma, 654
Differential auscultation, 12
Difficult asthma, 726–730
misdiagnosis, 440
Diffuse alveolar hemorrhage (DAH), 867, 937
in children, 893–902, 894f
anti-GBM disease, 897–898, 899f
approach to, 899–901
causes of, 894b
clinical manifestations of, 901t
COPA syndrome, 899
diagnosis of, 899–901
etiology of, 893–899
immune-mediated, 893–897, 898f
isolated pulmonary capillaritis, 898
nonimmune-mediated, 899
pathophysiology of, 893–895
treatment of, 901–902
Diffuse interstitial lung disease, 154, 155f,
813–816
classification of, 813–814
diagnostic approach of, 814–816
diffuse pediatric lung disease classification,
proposal, 814t
families and physicians, resources for, 816
new concepts in, 813–816
Diffuse interstitial lung disease (Continued)
new genetic mutations associated with, 815t
noninvasive testing, completion of, 814
pattern (determination) of, imaging (usage),
814
pediatric-specific classification for,
designation of, 813–814
terminology in, 813–814
Diffuse lung disease, of surfactant deficiency,
845
Diffuse Pediatric Lung Disease Classification,
814t
Diffuse small airway obstruction, 589
Diffusing capacity (DLCO), 88
increase in, 586
measurement of, 181–182
Diffusion, 88–89
capacity for carbon monoxide, decreased,
948
impairment of, 17–18
measurement of, 88–89
principles of, 88
Diffusion-weighted magnetic resonance
imaging, 637
Digital clubbing, 18–19, 781
asthma and, 691
cystic fibrosis and, 781
DIHS. see Drug-induced hypersensitivity
syndrome
DIOS. see Distal intestinal obstruction
syndrome
Dipalmitoyl phosphatidylcholine (DPPC), 836
Diphenylhydantoin, 882
Diphtheria, 415–416
clinical features of, 415–416, 416f
epidemiology of, 415
etiology of, 415
management of, 416
pathology of, 415
Direct hemodynamic monitoring, in
pulmonary hypertension, 621
Direct lung injury, occurrence of, 584
DISABKIDS
Asthma Module, 234, 236t–237t
Cystic Fibrosis Module, 238t
Disaturated phosphatidylcholine (DSPC), 836
levels/content, reduction of, 842
Disease
diagnosis of, 2
onset of, 2
physical inactivity and, 213f
Disease-specific HRQoL instruments, 232
design, 232
Disease-specific respiratory health-related
quality of life measures, 234–240
review of, 234–240
asthma measures, 234–235
Distal intestinal obstruction syndrome (DIOS),
796
abdominal pain and, 796
Gastrografin enemas for, 796
management of, 796
Distal phalangeal diameter, to interphalangeal
diameter ratio, 19, 20f
Distal respiratory zone, 63
Diuretics
detection of, 21
in pulmonary arterial hypertension, 578
Diverticulum bifurcates, 248
DMD. see Duchenne muscular dystrophy
DNA methylation, 34, 46–47, 47f
in asthma, 652–653
in respiratory disease, 50–51
Docetaxel, 880
Docosohexanoic acid prostaglandin D
(PD)1-derived mediator, 114
Dolor, inflammation and, 101, 112
“Domino” transplant, 981
Doppler ultrasound, 296
for cystic fibrosis liver disease, 792
Double aortic arch, 309, 615, 616f–617f,
619f
Down syndrome
aberrant right subclavian artery in, 1002f
airway endoscopy abnormalities in, 994f
cardiorespiratory interactions in,
1000–1003
congenital heart disease in, 1000–1001
disease complicating respiratory illness in,
993t
effusions in, 999
endoscopic findings in, 994t
endotracheal tube diameter in, size of,
1004t
gastrointestinal disorders in, treatment of,
1005
gastrointestinal-respiratory interactions in,
1003
genetic anomalies in, 1005–1006,
1005t–1006t
hemosiderosis in, 999f
imaging abnormalities in, 1001f
immune function in, 998t
laryngomalacia in, 993–994
lower respiratory tract in, 996–999
imaging of, 999–1000
infection of, 996–998, 1004–1005
magnetic resonance imaging of, 995f
obesity in, 1003–1005
pneumonia in, 1003f
pulmonary edema in, cardiac disease
leading to, 1001t
pulmonary vasculature in, 1000–1003
radial count in, 996, 997f
respiratory complications of, 992–1006
treatment of, 1004
respiratory disease in, 1001–1003
patterns of, 993t
respiratory disturbance index in, 995,
996f
sleep-related breathing disorders in,
995–996
subglottic stenosis in, 995
subpleural cysts in, 997f
survival in, 993f
trachea, 992–995
tracheal bronchus in, 1002f
tracheal stenosis in, 995
tracheomalacia in, 993–994
upper airway in
anatomy of, 992–993
disease of, 992–995, 993t
upper respiratory tract disease in
clinical presentation of, 995
treating, considerations when, 1004
wheeze in, 998–999
DPIs. see Dry powder inhalers
DPPC. see Dipalmitoyl phosphatidylcholine
DRESS. see Drug rash with eosinophilia and
systemic symptoms
Drowning, 634–638
accidental deaths in, 634
animal data in, 635
broad-spectrum antibiotics for, 636
chest radiograph of, victim of, 635f
initial, 635f
definitions of, 634
electrolyte imbalances in, 635–636
epidemiology of, 634
in hypertonic fluid, 635
hypothermia in, 635
hypoxic-ischemic damage in, 636

Drowning (Continued)
neuroprotection following, in induced
hypothermia, 636
nonpulmonary sequelae in, 635–636
postulated, 635
prevention, 637–638
as primary event, 634
sequence, 634
trauma in, 636
victims, in hypothermia, 635
“Drug holidays”, 261
Drug rash with eosinophilia and systemic
symptoms (DRESS), 876
Drug susceptibility testing (DST), for
Mycobacterium tuberculosis, 477
Drug-induced eosinophilia, 958–959, 958t
Drug-induced hypersensitivity syndrome
(DIHS), 876
Drugs
administration, by inhalation, in children,
257–272
nonallergic hypersensitivity to, asthma and,
693–694
Dry powder inhalers (DPIs), 257, 266–267
DSPC. see Disaturated phosphatidylcholine
Duchenne muscular dystrophy (DMD), 1047,
1050–1051, 1053, 1099
acute respiratory deteriorations,
management of, 1058
corticosteroid therapy of, 1058
daytime noninvasive respiratory aids, usage,
1058
exon skipping of, 1058
natural history of, 1056
obesity, frequency, 1057
prognosis of, 383
respiratory care in, consensus on, 1057b
respiratory disability, long-term
management of, 1056–1057
treatment, perspectives on, 1058
Duct ligation, 1103
Dupilumab, 754
Duplications, 41
Dutch Generation R study, in asthma,
654–655
Dynamic airway compression, 80
Dynein, 1038–1039
Dyskeratosis congenita (DC), 823, 823f
Dyskinesia, primary ciliary, 1034–1043
Dysphagia, 860
Dyspnea, 21
long-term risk for, 878
Dystrophin gene, location of, 1047
E etiologic agents with, 398t
Early antigen responses, 692
Early onset sarcoidosis (EOS), 863
Early-onset pneumonia, 352–353, 353f
Early-onset scoliosis, 384
classification of growth-friendly spine
implants for, 1059b
EBB. see Endobronchial biopsy
EBC. see Exhaled breath condensate
EBV. see Epstein-Barr virus
Echinocandins
for candidiasis, 519–520
for pulmonary mycoses, 508
Echinococcosis (hydatid disease), 538–543
clinical features of, 539–540, 540f–541f
diagnosis of, 540–541, 541f
differential diagnosis of, 540–541
epidemiology of, 538
etiology of, 538
management for, 541–543
pathology/pathogenesis of, 538–539, 538f
Index 1175
Echinococcosis (hydatid disease) (Continued) Endoderm, 26
prognosis for, 543 Endoplasmic reticulum (ER)
sylvatic Alaskan-Canadian variant of, 543 CFTR polypeptide translation, 759
treatment of, 537t, 541–543 stress, 843
Echocardiography, 333–334 surfactant, synthesis, 344
monitoring, 375 Endothelial basement membranes, epithelial
in pediatric pulmonary arterial membranes and, fusion of, 581f
hypertension, 570–571 Endothelial cell injury, assessment of,
in pulmonary hypertension, 621 587–588
Echogenicity, increase, 289–290 Endothelial dysfunction, in pulmonary arterial
Economic impact, of asthma, 648 hypertension, 567–568
Ectopia, 320–322 Endothelial nitric oxide synthase (eNOS),
Edema, 860 696–697
formation of, opposition in, factors in, 583 Endothelin receptor antagonists, in pulmonary
pathophysiologic consequences of, 585–586 arterial hypertension, 576–577
presence of, 585–586 Endotoxin, in asthma, 659–660
Edwards syndrome, 1005t–1006t Endotracheal aspirates, 400
Efferocytosis, 133 Endotracheal intubation
Effusions, in Down syndrome, 999 recorded, 3
EGFR. see Epidermal growth factor receptor in smoke inhalation, 632
Ehlers-Danlos syndrome, 312 Endotracheal tube diameter, in Down
EIB. see Exercise-induced bronchoconstriction syndrome, 1004t
E-ILO. see Exercise-induced laryngeal eNOS. see Endothelial nitric oxide synthase
obstruction Enterogenous cysts, 314
Elastance, in respiratory resistance, 193 Enterovirus D68 (ED68), 466
Elastic pulmonary arteries, 68 infection
Elastin, in interstitium, 69–70 characteristics of, 467t
Electrocardiogram QT intervals, prolongation, clinical features of, 466
sudden infant death syndrome and, 1139 diagnosis of, 467
Electrolyte imbalances, in nonpulmonary differential diagnosis of, 467
sequelae, 635–636 epidemiology of, 466
Electronic noses, 697 etiology of, 466, 467t
Electrostatic attraction, 260 imaging of, 467
Embolization, 319 laboratory findings on, 467
Embryo management and treatment of, 467
anterior-posterior (A-P) axes, formation of, pathology/pathogenesis of, 466
26 physical findings on, 466–467
organization of, principles in, 26 prevention of, 467
Embryonic period, 27–29, 27t, 28f prognosis of, 467
Embryonic phase, 248 pulmonary function tests for, 467
Embryonic pleural space, 1007 symptoms of, 466
Embryonic stage, of fetal lung, 70 Enthesitis-related arthritis (ERA), development
Emergent airway, 301 of, 850–852
Emotional factors, asthma and, 693 Entrainment or breath-enhanced nebulizers,
Emphysema, 290 268, 268f
gene mutations and, 38–39 Environment, maternal, on asthma, 654
in hydrocarbon aspiration, 626 Environmental exposures
pulmonary interstitial, 359–360 adverse
Empyema, 287, 1014–1021 in home environment, 50t
bacterial causes of, 1016b increased disease risk in, mechanism of,
chest drain, 1019, 1020f 50–54
clinical manifestations of, 1016 vulnerability of children in, 49–54, 50t
complications of, 1021 asthma and, 715
diagnosis of, 404 Environmental modification, 454
epidemiology of, 1015 Environmental tobacco smoke (ETS), effects,
444
etiology of, 1014–1015 Enzyme immunoassay (EIA), for
follow-up of, 1021, 1021f histoplasmosis, 510
hemithorax opacity and mediastinal Enzyme-linked immunosorbent assays (ELISA),
deviation, 435f in hypersensitivity pneumonitis, 949
investigations, 1016–1019, 1017f–1018f EOS. see Early onset sarcoidosis
management of, 1019–1021 Eosin, stains with, 844
microbiology of, 1015–1016 Eosinophilia, 105
monitoring progress, 1021 asthma and, 696
pleural effusion, relationship, 435–436 control of, 966–967
EMTU. see Epithelial-mesenchymal trophic unit drug-induced, 958–959
ENaC. see Epithelial sodium channel Eosinophilic esophagitis, 1116, 1120
Encapsulated pneumonia, 911 diagnosis of, 1116
chronic granulomatous disease and, 912f endoscopic features of, 1116
ENCODE (Encyclopedia of DNA elements) symptom from, 1116
project, 33–34 treatment of, 1116
End organ involvement, assessment, 772 Eosinophilic granuloma, 961–962
End-expiratory lung volume, 77 characteristics of, 961t
Endobronchial biopsy (EBB), usage of, 866 Eosinophilic granulomatosis with polyangiitis,
Endobronchial lesions, biopsy for, 143 962–964
Endobronchial ultrasonography, 170 characteristics of, 963b
1176 Index
Eosinophilic inflammation
activate, 101
in preschool wheeze
inception, 672
persistence, 672–673
in school age allergic asthma, 670
noninvasive biomarkers of, 670
stability of, 670
Eosinophilic lung diseases, 944–967
drugs that cause, 958t
etiology of, 952, 952b
helminth associated, 955b
Eosinophils, 105–106, 128–129, 750–751
adhesion of, 105
airway, asthma and, 686–687
biology of, 953–955
in pediatric allergic asthma, 665–667, 667b
recruitment, 105
Epidemiologic approaches, to asthma, study of,
640–641
Epidemiology and Natural History of Asthma:
Outcomes and Treatment Regimens study,
650
Epidermal growth factor receptor (EGFR),
gefitinib, 880–881
Epigenetics, 46–47, 47f
of asthma, 652–653
Epigenome-wide association studies (EWAS),
in asthma, 652–653
Epiglottitis, 412–414
clinical features of, 413, 413f
diagnosis of, 404
epidemiology of, 412
etiologic agents with, 398t
etiology of, 412
management of, 413–414
pathology of, 412–413
swollen, 413f
Epiglottoplasty, 1121
Episodic viral wheeze (EVW), 103, 649–650
Epistaxis, in bronchoscopy, 145
Epithelial cell
injury in, by inflammatory mediators,
60–61
surface of, hydration of, 26
Epithelial membranes, endothelial basement
membranes and, fusion of, 581f
Epithelial sodium channel (ENaC), 777
CFTR control, 762–763
Epithelial-mesenchymal interactions, lung
morphogenesis and, 35
Epithelial-mesenchymal trophic unit (EMTU),
formation of, 954–955
Epithelium, reduction of, 65f
Epoprostenol (intravenous prostacyclin), 575
Epstein-Barr virus (EBV), 418
ERA. see Enthesitis-related arthritis
Ergosterol, for pulmonary mycoses, 507–508
Erythrocytes, transit time of, 18
Erythromycin, 1115
for pertussis, 532
Esophageal atresia, 322–324, 1003
Esophageal cysts, 314
duplication of, 1083–1085
location of, 1083
types of, 1084
Esophageal dysmotility, 1115
Esophageal perforation, management in, 1070
Esophageal surgery, 1121
Esophageal tube injection, 334
Esophagitis, eosinophilic, 1116
Esophagogastroduodenoscopy, 1119
Esophagus
injuries to, 1069–1070
motility disorders, treatment, 1115
plain chest radiographs, usage, 1070
Estrogens, females receiving, pulmonary
embolism and, 888
Ethambutol
for nontuberculous mycobacterial
infections, 504t
for tuberculosis, 493t
Ethnicity, in prevalence of asthma, 645–646
Etiologic agents, 396
with acute bronchitis, 397t
associated with, 397t
with bronchiolitis, 397t
with common cold, 398t
with croup, 397t
with cystic fibrosis, 398t
with epiglottitis, 398t
with lung abscess, 398t
with pleural effusion and empyema, 398t
with pneumonia, 397t
with sinusitis, 397t
Etiology, age-related differences, 427
Euchromatin, 47
EUROCAT. see European Surveillance of
Congenital Anomalies
European Respiratory Society Task Force, in
asthma, 649–650
European Surveillance of Congenital
Anomalies (EUROCAT), 290
EVLW. see Extravascular lung water
EVW. see Episodic viral wheeze
EWAS. see Epigenome-wide association studies
Ewing’s sarcoma, 1074
Ex utero intrapartum treatment (EXIT
procedure), 298, 304–305
Examination, in diagnosis of preschool
asthma, 677–678
Exercise, 221
in age of omics, 229–230
asthma and, 217–223, 693
role of, 217–218
biological importance, 212
biomarkers, physical fitness, 214
brief, effect of, 229
capacity, 225
child health and disease, 212–230
cystic fibrosis and, 223–225
recommendations, 225
safety, 225
laryngeal obstruction and, 227–228
lung disease and, 225
response to, 219f
physiologic, 214–215
therapy, importance, 285
treatment considerations, 221–223
Exercise challenge test, 219–220, 219f
for asthma, 696
pretest, 219–220
Exercise limitation, 21, 22f
Exercise-induced asthma (EIA), 692–693
Exercise-induced bronchoconstriction (EIB),
218–219
caffeine, 222
children, 219f
diagnosis of, 222f
in nonasthmatics, 221
nonpharmacological approaches, 221–222
pharmacological approaches, 222–223
recommendations, 221
treatment of, 221, 222f
Exercise-induced bronchospasm, 693
Exercise-induced laryngeal obstruction
(E-ILO), 228f, 726
diagnosis, 228
mechanism of, 228
research, 228
therapeutic modalities, 228
Exercise-induced laryngomalacia, 691–692
Exhaled breath condensate (EBC), 117–118,
697
Exhaled gases, 116–118
Exhaled nitric oxide
for asthma, 696–697, 697t
for eosinophilic inflammation, 670
Exophiala spp., 526
Expiratory muscles
contraction of, in coughing, 15
effective contraction, 283
Expiratory wheezing, 11
Expulsive phase, 121
Extensive drug resistance, 494
External PEEP valve, use of, 388
External stents, 310
Extracellular matrix
amount, increase of, 30
role of, 36–37
Extracorporeal life support, in pediatric
acute respiratory distress syndrome,
613–614
Extracorporeal membrane oxygenation
(ECMO) therapy, 330–331
in pediatric intensive care, 598
Extrapulmonary nontuberculous
mycobacterial disease
clinical manifestations of, 502–503
management and treatment of, 505
Extrapulmonary tuberculosis
clinical features of, 482–483, 483f
in human immunodeficiency virus–infected
children and adolescents, 968
treatment of, 494
Extrathoracic airways, 16–17
Extrathoracic regions, inspection of, 6
Extrathoracic tuberculosis, 483–484,
483f
Extravascular lung water (EVLW)
measurement of, 588
mortality function of, 589f
Extrinsic allergic vasculitis, clubbing in, 19
Extubation, mechanical ventilation in, 554
Eyelids, heliotrope rash over, 857
F
Factor V Leiden, factor II 20210A variant,
572–573
FADS. see Fetal akinesia deformation sequence
Failed extubation, 302, 303f
Familial hypereosinophilic syndrome, 966b
Familial pulmonary arterial hypertension
(FPAH), 556
Family history
asthma and, 691
inquiry of, 3
Famine exposure, 249
Farming studies, in asthma, 659–660
FAST. see Focused Assessment with
Sonography in Trauma
Fast-twitch fibers, in muscles of respiration,
84
Fatty acid oxidation deficiencies, 1138–1139
Fecal pancreatic elastase-1, test, 790
Feeding
behavioral issues, 1113
conditions, 1108–1109
development of, 1107–1108, 1107t
neurologic conditions, 1109
treatment strategies, 1112–1113
Feeding and swallowing disorders,
1106–1113
epidemiology of, 1106
FEES. see Fiberoptic endoscopic evaluation of
swallowing
FEF25-75, airway obstruction and, 695
 Index 1177

Females (women), chest radiograph for Fluoroscopy, of atelectasis, 1029–1030, 1029f G

with cystic fibrosis, 765f
with juvenile dermatomyositis, 858f
Fentanyl, for pulmonary hypertension, 621
Fetal akinesia deformation sequence (FADS),
1005t–1006t
Fetal breathing activity, importance of, 338
Fetal echocardiography, 293–294
Fetal endoscopic tracheal occlusion (FETO),
293–294, 294f
Fetal growth, on asthma, 654–655
Fetal hemoglobin, oxygen-binding curve of, 18
Fetal lung
abnormalities, prenatal detection, 291
embryonic stage of, 70
fluid, of newborn infants, 346
liquid secretion, 71
polarized epithelium lining in, 72f
Fetal oxyhemoglobin dissociation curve, 91
FETO. see Fetal endoscopic tracheal occlusion
FGF. see Fibroblast growth factor
FHA. see Filamentous hemagglutinin
Fiberoptic endoscopic evaluation of swallowing
(FEES), 1100, 1110–1112, 1110t, 1112f
pediatric, 1110t
videofluoroscopic swallow studies versus,
1100t
Fiberoptic nasoendoscopy (FNE), 300–301,
301f
Fibrinogen, 60–61
Fibrinous septations, empyema and, 1014f
Fibroblast growth factor (FGF), 314
in lung morphogenesis, 26–27
mutations in, 38
Fibroblasts, in interstitium, 70
Fibrosing colonopathy, 790
treatment of, 790
Ficolins, 125
Filamentous hemagglutinin (FHA), in
Bordetella pertussis, 530t
Fimbriae 2, in Bordetella pertussis, 530t
Fimbriae 3, in Bordetella pertussis, 530t
Finger clubbing, measurement of, 20f
FISH. see Fluorescent in situ hybridization
Flail chest, 5–6, 1063–1064
action, diagram of, 1063f
paradoxical respiratory excursions with,
1064
Flexible bronchoscopes, 134–135
in contrast to rigid bronchoscopes, 135
Flexible bronchoscopy, 136, 136t, 941, 1118
Flexible nasoendoscopy, 305
Flow limitation, 182–183
Fluconazole
for candidiasis, 519–520
for coccidioidomycosis, 513
for pulmonary mycoses, 507–508
Flucytosine, for pulmonary mycoses, 508
Fluid accumulation, sequence of, schematic
representation of, 585f
Fluid dynamics, laws of, 406
Fluid filtration rate, reduction in, 592–593
Fluid leakage rate, reduction in, 592
Fluid management, mechanical ventilation in,
554
Fluid movement, factors for, 581–583
Fluid restriction, 376
Fluid-exchanging membranes, fluid
equilibrium across, 581
Fluid-exchanging vessels, vascular
permeability in, increase in, 584
Flunisolide, 705
Fluorescence-based screening assays, 913
Fluorescent in situ hybridization (FISH), 41,
45t
Fluoroscopic technique, 156–157, 157f
Fluticasone furoate, for asthma, 704 Galactomannan antigen assay, 928–929
Fluticasone propionate, for asthma, 704 for invasive aspergillosis, 517
Flutter, 275f, 278f, 280f Gallstones, occurrence of, 794
FNE. see Fiberoptic nasoendoscopy Gamma-aminobutyric acid (GABA),
Focal biliary cirrhosis, 792 importance of, 342
Focal bronchopneumonia, in hydrocarbon Ganciclovir
aspiration, 626 availability of, 984
Focal lung densities, 153, 154f for CMV pneumonia, 973
Focused Assessment with Sonography in Ganglioneuroblastoma, 1081
Trauma (FAST), 1067 Ganglioneuroma, 1081
Food allergy, asthma and, 725 Gas dilution technique, 176–180, 179f
Forced expiration, huffing, 279 Gas exchange, 85–86, 85f
Forced expiration measurement, 182–189 breath-by-breath analysis, 215
assessment of bronchodilator response and, HR variables, relationships, 215
189, 190f measurement, 214–215
flow and volume occurrence of, 67–68
cooperative subjects, 184–185 protocol, 215
in infants, 185–189 Gas humidification, mechanical ventilation in,
physiological principles and assumptions of, 554
182–184, 183f Gas mixing, measurement of, 205–210
quality control, acceptability criteria, and multiple breath washout technique,
limitations of, 184–185 205–207, 206f–210f
raised volume-rapid thoracoabdominal Gasdermin B (GSDMB), in asthma, 653t
compression in, 185–186, 186f–187f Gases
rapid thoracoabdominal compression in, in barriers, 88
185–186 definitions and symbols in, 73–74
Forced oscillation technique, 199–205, 199f, diffusing, amount of (Q), 88
201f diffusion rate of, 88
asthma and, 695 exhaled, 116–118
Forced vital capacity (FVC), measurement, flow of, 79
sitting position, 1046–1047 in physical solution, 74
Foregut pressure, measurement of, 74
duplication cysts, 1081 properties of, 74
embryologic maldevelopment, 1081f Gastric contents, reflux, 1097
structure of, 1081 Gastric emptying
Foregut cysts, 313. see also Bronchogenic cysts in cystic fibrosis, 796
Foregut patterning, 27f disorders, 1115
Foreign bodies Gastric pepsin, elevation of, 1101–1102
in atelectasis, 1027 Gastroenteric cysts (duplication cysts), 314,
removal of, therapeutic bronchoscopy for, 1085
143–144 Gastroesophageal reflux (GER)
Forkhead box A2 (FOXA2) aspiration of, 1101–1103
in gene expression, 26 asthma and, 693, 725
as transcription factor, 32 GERD versus, 1114
Forkhead box transcription factor 1 (FOXF1), in newborn infants, 343, 366
coding sequence (heterozygous role of, 1127–1128
mutations) of, 825 treatment considerations for, 1102–1103
Formoterol Gastroesophageal reflux disease (GERD),
for asthma, 707 1114–1115, 1120
combined with inhaled corticosteroids, 709 coexist, 447–448
FOXF1, mutations in, 38 diagnosis, 1115
FPAH. see Familial pulmonary arterial in Down syndrome, 1003
hypertension management for, 1115
F protein, 407–408 medical therapy, 1115
Fraction exhaled nitric oxide, measurement of, reconstructive airway surgery and,
in asthma, 650 1114–1115
Fractional nitric oxide, 696–697, 697t, 701 symptoms, 1114
Fractures, sternal, 1063 weight gain and obesity, association,
Frameshift variant, 40, 41f 1114
Fraser syndrome, 298 Gastrografin enemas, usage of, 796
FRC. see Functional residual capacity Gastrointestinal (GI) cancers, 797
Frequency dependence, 194 Gastrointestinal disease, 766–767
Functional electrical stimulation, 286f in Down syndrome, treatment of,
Functional residual capacity (FRC), 76, 120 1005
formation of, 338 Gastrointestinal-respiratory interactions, in
maintained, 60–62 Down syndrome, 1003
volume of, 581 GATA-3 transcription factor, 112–113
Functional surfactant, alveolar pool of, 58 GATA-binding protein 6 (GATA6), in gene
Fungal infection, in human immunodeficiency expression, 26
virus–infected children and adolescents, Gaucher disease, 821
973–975 chest radiograph in, 822f
Fungal pulmonary infections, 923–924 clinical features of, 821
Fungal spores, asthma and, 692 epidemiology of, 821
Funnel chest deformity, change, 6f etiology of, 821
Fusarium spp., 525–526 management of, 821
1178 Index
Gaucher disease (Continued)
pathobiology of, 821
prognosis of, 821
treatment of, 821
Gefitinib, as tyrosine kinase inhibitors,
880–881
Gender
in asthma prevalence, 645–646
pulmonary arterial hypertension and,
559–560
switch, in asthma, 645–646
Gene
by environmental interactions, 50–53
therapy, HPAH gene, identification of, 577
Gene environment interactions, of asthma,
652, 653t
Gene expression
cis-regulatory networks controlling, 33–34
control of, 31f
exercise, 229f
transcriptional mechanisms in, 31–34
Gene regulatory networks, 31–33
Gene therapy, cystic fibrosis, 805–810, 805b
lungs and, 806
outstanding questions on, 808–810
recent progress in, 806–808, 807f
vector development and upcoming clinical
trials of, 808, 808f–809f
viral versus nonviral, 806
Generalized pulmonary underinflation, 148
Generic HRQoL instruments, 232
Genetic ablation, 26
Genetic abnormalities (identification), of
surfactant production disorders (impact),
845
Genetic anomalies, in Down syndrome,
1005–1006
Genetic susceptibility, 371
Genetic variation
interpretation of, 43
research study designs to, 44–46, 45t
risk for respiratory disease, 50
technologies to identify, 41–43
type of, 40–41, 41f, 43t
Genetics, of asthma, 651–652
Genome-wide approaches, in asthma,
651–652
Genome-wide association studies (GWAS), 40,
46
Genome-wide interaction study (GEWIS), in
asthma, 652
Genotype-phenotype associations, 47–48
Genotype-phenotype correlations, 760–762,
774–775
Gentamicin, for early onset septicemia, 346
GERD. see Gastroesophageal reflux disease
Germ cell tumors, nonseminomatous,
1078–1079
Ghon focus, 478
Gibson and Cooke technique, 770
Girls. see also Females
chest diameters of, mean values of, 8f
with SLE, chest radiographs in, 856f
Gland excision, 1103
Globulin, 60–61
Glottic closure, 121
in coughing, 15
intact bulbar function, requirement, 283
Glottic openness, in coughing, 15
(1-3)-b-D-glucan (BDG), 517
Glucocorticoid response element (GRE),
705
Glucocorticoids, in pediatric allergic asthma,
667
Glutathione-S-transferase Mu 1 (GSTM1), in
asthma, 653t
Glutathione-S-transferase Pi 1 (GSTP1), in
asthma, 653t
Glutathione-S-transferase Theta 1 (GSTT1), in
asthma, 653t
Glycerol-phosphorylcholine backbone, 57
Glycine, importance of, 342
Glycocalyx, 68
Glycoproteins, 121–122
Glycosaminoglycans, in interstitium, 69–70
GM-CSF. see Granulocyte-macrophage
colony-stimulating factor
Goblet cells, 66f
replacement of, 65
Gomori-Grocott (methenamine-silver) stain,
for bronchoalveolar lavage, 142
Goodpasture syndrome, 894–895
Gorham-Stout disease, 819
Gottron’s papules, presence of, 857
G-protein-coupled receptors, transmembrane
receptor superfamily of, 110
Graft-versus-host disease (GVHD), pediatric
intensive care and, 604–605
Gram stain appearance, of bacterial
respiratory pathogens, 398t
Granulocyte-macrophage colony-stimulating
factor (GM-CSF)
impact of, 836–837
lung/serum biomarkers, 846
production of, genetically determined
primary defects in, 840
serum autoantibody testing for, 846
Granulocyte-macrophage colony-stimulating
factor deficiency, 58
Granulocytes, in pulmonary endothelium, 70
Granulomatosis with polyangiitis, 869–872,
895–896, 895f–896f
airways of, bronchoscopic photographs of,
873f
clinical manifestation of, 869–870
CT scan in, 872f
diagnosis of, 871
epidemiology of, 869
HRCT scan in, 870f
induction therapy for, 872
lung biopsy, photomicrograph of, 869f
pathogenesis of, 869
prognosis of, 872
pulmonary involvement in, 870–871
pulmonary symptoms of, 870
pulmonary-renal syndrome and, 871
soft tissue radiograph in, 871f
treatment of, 871–872
Granulomatous lesions, occurrence of, 864
Granulomatous lung lesions, progression of,
865
Grass pollen, asthma and, 692
Gravitational sedimentation, aerosol, 259–260
GRE. see Glucocorticoid response element
Great arteries, transposition of, 18
Green zone, preventive management of, in
preschool asthma, 680, 681f
Group A Streptococcus, 427
Group B Streptococcus clearance, 61
Group O Rh-negative blood, administration of,
339–341
Growth, exercise (relationship), 213f
Growth factors, 111, 112f
in pulmonary arterial hypertension, 568
Guillain-Barré syndrome, 1046
GWAS. see Genome-wide association studies
H Hepatic steatosis, 792
Haemophilus influenzae, 396
in asthma, 659
clearance, 61
Haemophilus influenzae b (Hib)
vaccine, 427
in bacterial pneumonia, 969–970
in HIV-infected ART-naïve children, 970
Hamartomas, 1072
Hamman-Rich disease, 944
HAPE. see High-altitude pulmonary edema
HapMap project, in asthma, 651–652
HEADSS (Home Education Activities Drugs
Sexuality Suicide) framework, 244b
Health, exercise and, 213f
Health-related quality of life (HRQoL)
assessment, 233
children, 232
clinical utility, 233–234
errors, reduction, 232–233
feedback, impact, 233–234
integration, feasibility, 234
measures, 231–232
development, 232
developmental considerations, 232–233
PRO type, 231–232
proxy-respondent, usage, 233
Heart
primary sarcoma of, 1086
primary tumors of, 1086
Heart rate, increase, 391
Heart-lung transplantation, 983
success in, 578–579
Heated humidified high-flow nasal cannula
(HHHFNC), 347
Heavy nocturnal snoring, clinical symptoms
of, 17b
Heliox, for acute episode of asthma, 719
Helium dilution technique, 179f
Helminth associated eosinophilic lung disease,
962
Helminth larvae, 962
Hemagglutinin, in influenza virus, 460
Hemangiomas, 307–309
Hematopoietic stem cell transplantation
pulmonary complications of, 935–940,
935t
early noninfectious posttransplant
complications, 936–937, 936f
late noninfectious posttransplant
complications, 938–940
pretransplant factors, 935–936
special pulmonary considerations in
pediatric patients with, 604–605, 605t
Hemianomalous pulmonary venous drainage,
298
Hemiplegia, 1046
Hemodynamics, in pediatric pulmonary
arterial hypertension, 571–572
Hemoglobin
reduced, 18f
in transport of carbon dioxide, 94
Hemoglobin A, 903
Hemoptysis, cystic fibrosis and, 781
Hemorrhage
in hydrocarbon aspiration, 626
pulmonary, as complication of mechanical
ventilation, 603
Hemosiderosis, in Down syndrome, 999f
Hemothorax, 1065–1066
bleeding sources, abundance, 1065–1066
children, 1025
clotting/loculation/infection, 1066
management of, 1066
Hepatic engorgement, 21
Hepatic function, abnormality of, 540
Hepatobiliary disease, 791–794, 791f
blood test, usage of, 791
clinical presentation of, 791

Hepatobiliary disease (Continued)
diagnosis of, 792, 792f
management of, 793–794
ultrasound in, 792
Hepatopulmonary syndrome (HPS), following
solid-organ transplantation, 933
Hereditary clubbing, 19
Hereditary SP-B deficiency, recognition of, 837
Hering-Breuer reflex, 69, 185–186
lung inflation stimulation, 342–343
Heritable pulmonary arterial hypertension
(HPAH), 564
epidemiology of, 564
gene identification of, 577
incidence and prevalence of, 560
natural history of, 564–565
Hermansky-Pudlak syndrome (HPS), 823
Herpes simples virus (HSV), 926
Herpesvirus type 6, 926
HES. see Hypereosinophilic syndrome
Heterotaxia, 1041
Heterotaxy, 1041
Heterotaxy syndrome, in single ventricle
physiology, 624–625
HFCWO. see High-frequency chest wall
oscillation
HFJV. see High-frequency jet ventilation
HFOV. see High-frequency oscillatory
ventilation
HFV. see High-frequency ventilation
HGVS. see Human Genome and Variome
Society
HHHFNC. see Heated humidified high-flow
nasal cannula
HIES. see Hyper IgE syndromes
High altitude (HA), 226
asthma, 226–227
bronchopulmonary dysplasia, 227
chronic exposure, 227
cystic fibrosis, 227
muscle performance, 226
High-altitude pulmonary edema (HAPE),
590–591, 1003
High-frequency chest wall oscillation
(HFCWO), 281f
usage, 281, 284
High-frequency jet ventilation (HFJV),
randomized trials, results, 376
High-frequency oscillatory ventilation (HFOV)
in acute respiratory distress syndrome, 611
randomized trials, results, 376
High-frequency ventilation (HFV), 550–551
gas-transport mechanisms during, 597, 598f
High intensity interval training (HIIT),
224–225
High-permeability pulmonary edema, 584
High pressure pulmonary edema, 584,
588–589
distinguishing low pressure from, 587–588
High-resolution computed tomography
(HRCT), 165, 165f
interpretation, 165–168, 166f–167f
sensitivity, 1103–1104
for severe problematic asthma, 724
HIIT. see High intensity interval training
Hilar adenopathy, in pulmonary tuberculosis,
479f
Hilar bronchogenic cyst, esophagram of,
1083f
Hilar expansion, 151
Hippocratic fingers, 18–19
Hirschsprung disease, 1150
Histamine, 220
in asthma, 667
direct bronchoprovocation testing and, 695
presence, 739
Index 1179
Histones, 47 Human immunodeficiency virus–infected
Histoplasma capsulatum, 508 children and adolescents (Continued)
Histoplasmosis, 508–512 cardiomyopathy in, 979–980
diagnosis of, 509–510, 511f chronic infection in, 977
differential diagnosis of, 510 chronic lung disease in, 975–979, 976f
disseminated, 509 lung function in, 979
epidemiology of, 508, 508f cotrimoxazole prophylaxis in, 975t
etiology of, 508 fungal infection in, 973–975
imaging of, 509, 510f immune reconstitution inflammatory
laboratory findings on, 509 syndrome in, 978–979
management and treatment of, 510–511 lymphoid interstitial pneumonitis in, 977,
pathology/pathogenesis of, 508–509 977f
prevention of, 511 Mycobacterium avium complex (MAC) in,
prognosis of, 511–512 972
pulmonary, 509 pneumonia in, 968–969
pulmonary function testing for, 509 antibiotic treatment of, 970t
pulmonary infection and, 929–930 bacterial, 969–970
symptoms and physical findings of, 509 Pneumocystis jirovecii, 973–975, 974f
Historical cohort study, in asthma, 640–641 prevention of, strategies for, 975b
History, 2–25 pulmonary complications of, 969b
in diagnosis of preschool asthma, 677–678 pulmonary hypertension in, 979–980
pediatric, 2–4 pulmonary tumors in, 977–978
principles of, 2 respiratory disorders in, 968–980
HIV. see Human immunodeficiency virus acute, 968–975
HMPV. see Human metapneumovirus spectrum of, 968
HOA. see Hypertrophic osteoarthropathy tuberculosis in, 970–972, 971f
Hoarseness, 305 upper airway disease in, 980
Hodgkin disease, relapsing, 878 viral infection in, 972–973
Holding chambers, for asthma, 714, 714t Human metapneumovirus (HMPV), 469,
Home care, considerations for, 385–386 926–927
Home mechanical ventilators, 389–390 infection, 469–471
Home positive pressure mechanical characteristics of, 467t
ventilators, 390 clinical features of, 469
Home visit, adherence and, 727 diagnosis of, 470
Homeobox 2B (PHOX2B) gene, 384–385 differential diagnosis of, 470
Homeostasis, in type II alveolar epithelial cells, epidemiology of, 469
67 etiology of, 469
Hookworms, 962 imaging of, 470, 470f
Hoover sign, 5 laboratory findings on, 470
Horner’s syndrome, 1080–1081 management and treatment of, 470
Hospital pathology/pathogenesis of, 469
admission to, severe asthma and, 729–730, physical findings of, 470
730b prevention of, 470
management of asthma in, 719 prognosis of, 470–471
Hospitalization, in asthma, 646–647 pulmonary function tests for, 470
Host defense functions, of surfactant, 59–60 symptoms of, 469–470
House dust mites, pet sensitization and, Humanized monoclonal antibody,
728–729 administered, 106
How Are You? (HAY), 236t–237t Humans
HP. see Hypersensitivity pneumonitis surfactant in, genetic deficiencies in, 61
HPAH. see Heritable pulmonary arterial toxocariasis and, 535
hypertension Humidification, 120, 410
HPS. see Hermansky-Pudlak syndrome Humoral immunity, 131–132
HRQoL. see Health-related quality of life Hyaline membrane disease, 343–344
HS. see Hyperinfection syndrome Hyalohyphomycosis, 525–526
HSV. see Herpes simples virus Hydatid disease, 538–543. see also
Human genome, sequencing, 229 Echinococcosis
Human Genome and Variome Society (HGVS) Hydrocarbon aspiration, 1097
CFTR guidelines, 758 lung injury from, 626–633
nomenclature, 758 clinical findings of, 627, 627f
Human Genome Project, 41, 43 diagnosis and differential diagnosis of,
Human immunodeficiency virus (HIV) 627
adolescent, 968 epidemiology of, 626
nontuberculous mycobacterial disease with, etiology of, 626
499 management and treatment of, 627–628
pediatric, epidemiology of, 968 pathology of, 626
with tuberculosis pathophysiology of, 626–627
clinical features of, 484 prevention of, 628
epidemiology of, 476–477 prognosis of, 628
Human immunodeficiency virus–infected Hydrocarbon pneumonia, 627f
children and adolescents Hydrocarbon sniffing, 628
airway hyperreactivity/asthma in, 979 Hydrocarbon toxicity, 626
annual screening of, 970–971 Hydrocephalus
aspiration pneumonitis in, 978 report, 1042
bronchiectasis in, 978 in tuberculosis meningitis, 483–484,
bronchiolitis obliterans in, 978, 978f 483f
1180 Index
Hydrophilic particles, enlarged by
humidification, 120
Hydrostatic pressure, increased, in pulmonary
microvasculature, 583
Hydroxymethylglutaryl coenzyme A (HMG-
CoA) reductase inhibitors, 884
Hydroxyurea, 880
Hyper IgE syndromes (HIES), 132, 921–922
Hyperacute rejection, after pediatric lung
transplantation, 986
Hypercapnia
demonstration of, 384
pump failure of, impact of, 383
response, 385
Hypercarbia, clinical signs of, 18
Hyperemia, in hydrocarbon aspiration,
626
Hypereosinophilia, evaluation of, 966
Hypereosinophilic syndrome (HES), 964–967,
966b
associated, 966b
clinical features of, 965–966
epidemiology of, 964
etiology of, 964–965
familial, 966b
laboratory findings in, 966
management and treatment of, 966–967
myeloproliferative variants of, 966b
overlap, 966b
pathology/pathogenesis of, 965
physical findings in, 965–966
prognosis of, 967
symptoms of, 965–966
T lymphocytic variant of, 966b
undefined, 966b
Hypergammaglobulinemia, 537
Hyperinfection syndrome (HS), occurrence of,
544
Hyperoxia, 342
Hyperplasia, thymus, 1074–1075, 1075f
Hyperpnea, 4
Hyperreactive airway disease, wheezing
associated with, 17
Hypersensitivity pneumonitis (HP), 944–967
acute stage of, 948
Bird fancier’s lung in, 944
bronchial challenge in, 950
bronchoalveolar lavage in, 950–951
chronic stage of, 949
clinical features of, 948–951
counterimmunoelectrophoresis (CIE) in,
949
cytokines in, 948
diagnostic criteria for, 948b
diffusion capacity for carbon monoxide,
decreased, 948
ELISA in, 949
environmental exposures in, 946–947
epidemiology of, 944
etiologic antigens and sources in,
945t–946t
etiology of, 944–947
high-resolution computed tomography scan
in, 949f
histologic findings in, 950–951
immunologic studies in, 949
lung biopsy in, 950–951, 951f
management and treatment of, 951
Ouchterlony double immunodiffusion in,
949f
pathogenesis of, 947–948, 947f
physical findings of, 948
prevention of, 951
prognosis of, 951–952
pulmonary function testing in, 950
radioimmunoassay (RIA) in, 949
Hypersensitivity pneumonitis (HP) (Continued)
radiologic findings in, 949–950
subacute stage of, 949
symptoms of, 948
Hypertension, maternal, 250
Hyperthyroidism, asthma and, 694
Hypertonia, BRUE definition and factors for
inclusion and exclusion, 1126t
Hypertonic saline
for cystic fibrosis, 782
mucoactive agents, 282
Hypertrophic osteoarthropathy (HOA), 19,
448
Hypertrophic pulmonary osteoarthropathy,
781
Hyperventilation,asthma and, 691–692
Hypocarbia, avoidance, 376
Hypopharynx, occurrence, 1097–1098
Hypoplasia, 321–322
secondary pulmonary, 360–361, 361f
Hypoplastic lung, 289
Hypoplastic right lung, chest radiograph for,
149f
Hypoplastic thorax syndromes, 1049,
1052–1054
management of, 1060–1061
Hypopnea, 4
Hypoproteinemia, presence of, 583
Hypothermia, 634
in nonpulmonary sequelae, 635
Hypotonia, BRUE definition and factors for
inclusion and exclusion, 1126t
Hypoventilation
episodes of, 384
syndromes, 1149–1152
Hypoxemia
development of, 1131
result, 383
reversing, 592
in smoke inhalation, 630
Hypoxia, 93b
Hypoxic ventilatory roll-off, 1146–1147
Hypoxic-ischemic damage, in drowning, 636
I Immunosuppressives
ICAM. see Intracellular adhesion molecule
ICAM-1. see Intercellular adhesion molecule-1
ICS. see Inhaled corticosteroids
Idiopathic clubbing, 19
Idiopathic interstitial pneumonitis, 937
Idiopathic kyphoscoliosis, 1048
Idiopathic pneumonia syndrome, 937
Idiopathic pulmonary arterial hypertension
(IPAH), 564
epidemiology of, 564
natural history of, 564–565
Idiopathic pulmonary fibrosis (IPF), 813, 944
Idiopathic pulmonary hemosiderosis, 899,
901f
IgE-mediated sensitization, asthma and,
688–689
ILAR. see International League of Associations
for Rheumatology
ILCOR. see International Liaison Committee on
Resuscitation
ILD. see Interstitial lung disease
Iloprost (inhaled prostacyclin analogues),
575–576
Imaging modalities, utility of, 147
Imatinib, 880–881
Imidazoles, for pulmonary mycoses, 507–508
Imipenem, for nontuberculous mycobacterial
infections, 504t
Immediate posttransplant phase, of pediatric
lung transplantation, 985–986
Immersion accidents, serious, 636
Immersion events, 634–638
Immotile cilia syndrome, 63–65, 1034
Immune dysfunction, in Down syndrome, 998
Immune dysregulation, primary
immunodeficiency and other diseases
with, 1062–1071
Immune function, in Down syndrome, 998t
Immune maturation, delayed, environmental
exposures in, 53, 54f
Immune-mediated alveolar hemorrhage,
893–897, 898f
Immune-mediated inflammation, impact, 766
Immune pathways, stimulation and inhibition
of, 60
Immune reconstitution inflammatory
syndrome (IRIS), in human
immunodeficiency virus–infected children
and adolescents, 978–979
Immune response, suppress, regulatory T cells
(Tregs) and, 106–107
Immunocompromised child, pulmonary
infections in, 923
approach to, 940
clinical presentation of, 923–924
fungal pathogens, 927–930
infectious agents in, 924–931
invasive diagnostic studies for, 941–942
noninvasive diagnostic studies for, 940–941
parasitic agents, 931
pathogens associated with, 924t
prevention strategies for, 942
pulmonary coinfections, 931
radiographic testing for, 940, 940t
viral pathogens in, 924–927
Immunoglobulin A (IgA), 131
levels of, asthma and, 697–698
Immunoglobulin E (IgE), 132
production of, B-lymphocytes and, 107–108
Immunoglobulin G (IgG), 131
levels of, asthma and, 697–698
Immunoglobulin infusions, for severe
therapy-resistant asthma, 734
Immunoglobulin M (IgM), 132
levels of, asthma and, 697–698
for pediatric lung transplantation, 984
for severe therapy-resistant asthma, 734
Impaction, facilitated, 120
Impedance (Zrs) curves, in terms of resistance,
202f
IMV. see Intermittent mandatory ventilation
In vitro measure of allergen-specific IgE
(s-IGE), asthma and, 698
In vivo deposition, aerosol, 263–264, 264f
Indoor air quality, in asthma, 662–663
Induced hypothermia, 636
Induced sputum, 116
Inducible form of NOS (iNOS), increased
expression of, 111
Inertial impaction, aerosol, 262, 262f
Infancy, neuroendocrine cell hyperplasia of,
832–834
characteristic radiographic and pathologic
findings in, 833f
clinical features of, 832
diagnosis of, 833–834
differential diagnosis of, 833–834
epidemiology of, 832
etiology of, 832
histologic findings in, 833
management of, 834
pathogenesis of, 832
prognosis of, 834
radiologic findings in, 832–833
treatment of, 834

Infant pulmonary function test (iPFT),
814–815, 832
Infanticide, 1139–1140
Infantile hemangiomas, 307
Infantile idiopathic scoliosis, 1052, 1055f,
1059
Infants
community-acquired pneumonia (CAP),
antibiotics, 433
congenital heart disease in, 18
delivery room resuscitation, 339–341
exposure pathways in, 50t
heart, primary tumors (impact) in, 1086
hemoptysis in, 1085
lung sounds of, objective acoustic
measurements of, 11
lung volumes in, 180–181
meconium ileus, signs/symptoms of, 795
pneumonia in, 58
positive expiratory pressure (PEP), 279
pulmonary embolism in, 888, 888t
pulmonary function tests in, 174–211
forced expiratory flow and volume in,
185–189
indications and special considerations for,
174–175
interpretation of lung volumes in,
180–181
sedated, 175
technical and staff requirements for,
174–175, 175f
respiratory distress, thymus (enlargement),
1075f
respiratory distress syndrome (RDS) in,
alveolar surfactant pool sizes in, 58
solid benign teratoma in, 1078f
sternum, fractures of, 1063
traumatic thoracic injury in, 1062
Infections
in asthma, 657–659, 658f, 660f
asthma and, 692–693
in human immunodeficiency virus–infected
children and adolescents
chronic, 977
fungal, 973–975
viral, 972–973, 973f
in pediatric lung transplantation, 986–987
Infectious mononucleosis, 418–419
Infectious processes, BAL indicated for,
139–140
Inflammation
asthma and, 688
development, 665, 669f
BPD, relationship, 372–373
characterization of, 101
occurrence of, 3
in pulmonary arterial hypertension, 568
resulting from infection, 406
Inflammation-mediated lung injury in,
occurrence of, 584
Inflammatory cells, 103–108, 104f
assessment of, in asthma, 650
from growth factors, 111
Inflammatory enzymes, 112
Inflammatory markers, measurement of, in
asthma, 650
Inflammatory mediators, 108–111
Inflammatory pseudotumor (plasma cell
granuloma), 1072
Inflammatory subtypes, of asthma, 650
“Inflammometer,” characteristics of, 115–116,
116t
Influenza, 460–465
chemoprophylaxis for, 464
clinical features of, 461–462
complications of, 461t, 462
Influenza (Continued)
diagnosis of, 462
differential diagnosis of, 462, 463t
epidemiology of, 460, 461t
management of, 462–464
pathology/pathogenesis of, 460–461
physical findings on, 461
prevention of, 464, 464t
prognosis/outcome of, 464–465
radiographic findings on, 461–462, 462f
symptoms of, 461
treatment of, 462–464, 463t
Influenza A virus infection, antibiotic
treatment of, 970t
Influenza B virus infection, antibiotic
treatment of, 970t
Influenza viruses, 460, 461f
Infraclavicular fossae, as horizontal reference
points, 9f
Infrapulmonary/subpulmonary effusion,
1017f
INH prophylaxis, for HIV-infected children,
972
Inhalation
in children, drug administration by,
257–272
smoke, respiratory complications of,
626–633
carbon monoxide poisoning, 629, 629f
clinical findings of, 631
diagnosis and differential diagnosis of,
631
epidemiology of, 628–629
etiology of, 629
management and treatment of, 631–632
pathogenesis of, 629
pathology of, 629–630
pathophysiology of, 630–631
prevention of, 633
prognosis of, 633
surface burns and, pulmonary
complications of, 632–633
therapies, 282
Inhaled aerosolized drugs, 257, 258t
Inhaled corticosteroids (ICS), 219–220, 454
fine-particle, for severe therapy-resistant
asthma, 733
high-dose, for severe therapy-resistant
asthma, 733
for preschool asthma, 685
Inhaled foreign body, 287
Inhaled iloprost, approval of, 576
Inhaled mucolytics, for cystic fibrosis, 783
Inhaled nitric oxide (iNO), 826
for acute respiratory distress syndrome,
612–613
in pulmonary arterial hypertension, 577
Inhaled prostacyclin analogs (Iloprost/
Treprostinil), 575–576
Inhaled therapies, timing, 283f
Inhaler devices, use of, adherence and,
727–728
Inherited thrombophilia, 888
Initiator caspases, 132–133
Injured mature lung, 60–61
Innate defenses, in pulmonary host defense
systems, 38
Innate immune receptors, expression, 447
Innate immune system, deficiencies of, 922
Innate immunity, in pulmonary host defense
systems, 37–38
Innate lung defenses, 121–129
Innate lymphocytes, 129
Innate lymphoid cells, 107
in pediatric allergic asthma, 668–669, 668f,
668t
Index 1181
Inotropic agents, in pulmonary arterial
hypertension, 578
Inspiration, supraclavicular fossae in,
indrawing of, 5
Inspiratory indrawing, 5
Inspiratory load, 1131
Inspiratory lung sounds, 10
spectra of, 11f
Inspiratory phase, 121
Inspired air, humidification of, 120
Inspired mixtures, increased, 92–93, 93b
Instruments, quality of life, in asthma, 648
INSURE technique, usage, 346
Integrated Therapeutics Group Child Asthma
Short Form (ITG-CASF), 236t–237t
Intensive care, pediatric
history of, 596
intubation in, 596
mechanical ventilation in
complications of, 598–605
indications for, 596, 597t
respiratory complications of, 596–605
special pulmonary considerations in
in patients with congenital heart disease,
604
in patients with malignancy or
hematopoietic stem cell
transplantation, 604–605, 605t
in patients with neuromuscular disorders,
603
ventilation strategies in, 596–598
conventional versus oscillatory
ventilation, 596–598, 598f
extracorporeal membranous oxygenation,
598
Intensive care unit neuromyopathy, as
complication of mechanical ventilation,
603–604
Intensive phase, of multidrug therapy, for
pulmonary tuberculosis, 493
Intercellular adhesion molecule-1 (ICAM-1),
105
glycoprotein, 372–373
Intercostal muscles, contraction of, 384
Intercostal tone, loss of, 1131
Interdisciplinary team, in aerodigestive model,
1095, 1095f
Interferon (IFN)-g, 102
Interferon-gamma release assays, for
tuberculosis, 486–488, 488f, 489t
Interleukin-1 (IL-1)
inhibitors of, efficacy of, 850–852
production of, 947
Interleukin-2 (IL-2), 881
Interleukin-5 (IL-5), 752
administered, 106
in pediatric allergic asthma, 665–667, 667f
Interleukin-6 (IL-6), levels of, elevation of,
850–852
Interleukin-8 (IL-8), 101
production of, 947
Interleukin-10 (IL-10), 113
Interleukin-23 (IL-23) axis, 102
Interleukin-25 (IL-25), in pediatric allergic
asthma, 668–669
Interleukin-33 (IL-33)
in pediatric allergic asthma, 668–669
severe asthma and, 732
Intermittent asthma, 711–712
Intermittent hypoxia, consequence, 1158
Intermittent mandatory ventilation (IMV),
550
Intermittent positive pressure breathing
(IPPB), 275f–277f
benefit, 285
device, 284f
1182 Index
Internal stents, 310
International HapMap Project, 41
International League of Associations for
Rheumatology (ILAR), JIA classification
criteria of, 852
International Liaison Committee on
Resuscitation (ILCOR), newborn
breathing inadequacy procedures,
339–341
International Study of Asthma and Allergies
in Children (ISAAC) survey, in
epidemiologic studies of asthma,
641–642
International Union against Tuberculosis and
Lung Disease (IUTLD), in epidemiologic
studies of asthma, 641–642
Interrupter resistance, measurement of,
196–198, 197f
Interstitial edema, in smoke inhalation, 630
Interstitial fluid, clearance pathways in, 584
Interstitial forces, 582
Interstitial glycogenosis, pulmonary, 830–832
clinical features of, 830
diagnosis of, 830–832
differential diagnosis of, 830–832
epidemiology of, 830
etiology of, 830
histologic findings in, 830, 831f
management of, 832
pathogenesis of, 830
presentation of, 830
prognosis of, 832
radiologic findings in, 830
treatment of, 832
Interstitial hydrostatic pressure, decreased,
583
Interstitial lung disease (ILD), 287, 287f, 354,
939
anti-Scl-70 antibodies and, 862
HRQoL measure, 240
impact of, 861f
Niemann-Pick disease and, 822
onset of, 861f
Interstitial macrophage, 70
Interstitial spaces, protein presence in, 584
Interstitium, 69–70, 69f
continuous fiber scaffold in, 69–70
Intestinal mucous gland hyperplasia, 767
Intraalveolar edema, in hydrocarbon
aspiration, 626
Intraarticular corticosteroid injections, usage
of, 854
Intrabronchial mucus stasis, 777, 779f
Intracellular adhesion molecule (ICAM),
corticosteroids and, 705
Intractable air leaks, therapeutic bronchoscopy
for, 145
Intralobar sequestrations, 319
Intranasal corticosteroids, 738–739
effects, 738–739
Intrapulmonary percussive ventilation (IPV),
281f
device, 281
Intrathoracic airway disease, 385
Intrathoracic lipoma, rarity, 1079
Intrathoracic meningoceles, 1085–1086
Intrathoracic tuberculosis, 478–479
Intravenous agents, pulmonary edema and,
591
Intravenous prostacyclin (epoprostenol),
575
Intravenous prostacyclin analogue
(treprostinil), 576
Intrinsic laryngeal muscles, actions of,
1097–1098, 1108
Intrinsic lung defense, 120–121
Intrinsic sensory pathway, neurotransmitters, Juvenile idiopathic arthritis (JIA) (Continued)
1146 diagnosis of, 852
Intubation, in pediatric intensive care, 596 epidemiology of, 850
Invariant natural killer T (iNKT) cell, 130 etiology of, 850–852
Invasive fungal infections, 778 intraarticular corticosteroid injections for,
Invasive pulmonary aspergillosis, 516 usage of, 854
Invasive ventilation, via tracheostomy, 387 macrophage activation syndrome (MAS)
IPAH. see Idiopathic pulmonary arterial and, 852
hypertension mortality of, 853
IPF. see Idiopathic pulmonary fibrosis nonsteroidal anti-inflammatory drugs
iPFT. see Infant pulmonary function test (NSAIDs) for, usage of, 854
IPPB. see Intermittent positive pressure pathogenesis of, 850–852
breathing prognosis of, 854
Ipratropium bromide, for asthma, 703 pulmonary function in, studies of, 853–854
nebulized, 717 pulmonary involvement in, 853–854
IPV. see Intrapulmonary percussive ventilation pulmonary manifestations with, 853
IRIS. see Immune reconstitution inflammatory subtypes of, 852t
syndrome systemic onset, HRCT scan in, 853f
Irreversible dilatation, 439–440 treatment of, 854
Irritant receptors, 69 Juvenile myasthenia gravis, 1047, 1050,
Irritants, asthma and, 692 1053, 1056
Irritation phase, 121 Juvenile scoliosis, 1059
ISAAC study, in prevalence of asthma, Juxtacapillary (J) receptors, distribution of,
642–646, 661–662 581
Isavuconazole
for invasive aspergillosis, 517–518
for mucormycosis, 523 K
Isolated pulmonary capillaritis, 898 Kartagener syndrome, 63–65
Isomerism, 312 Karyotype, 41, 45t
Isoniazid Kasukawa’s criteria, 863
for nontuberculous mycobacterial KCO, 88
infections, 504t Kenny-Caffey syndrome, 312
for tuberculosis, 491–492 Keratoconjunctivitis sicca, 872
8-isoprostane, increased concentration of, 111 Kerley B lines, 958
Isotropic computed tomography, 157–158 Kerley lines, 586
ITG-CASF. see Integrated Therapeutics Group example of, 587f
Child Asthma Short Form KL4 polypeptide, 346–347
Itraconazole Kreisler (mouse) MAF-related leucine zipper
for ABPA, 957 homolog, 1144
for blastomycosis, 515 Kyphoscoliosis, 1048–1049, 1052, 1054,
for histoplasmosis, 510 1058–1060, 1085
for sporotrichosis, 524 classification of, 1048
IUTLD. see International Union against respiratory effects of, 1048–1049
Tuberculosis and Lung Disease respiratory function testing in, 1052
Ivemark syndrome, 312 Kyphosis
Ivermectin, for strongyloidiasis, 545 onset, 274f
severe, 384
J
Jarcho-Levin syndrome, 1049, 1053–1054, L
1055f, 1061 Labile asthma, 690
JDM. see Juvenile dermatomyositis Lactoferrin, 126
Jet nebulizers, 267–268, 267f LAM. see Lymphangioleiomyomatosis
Jeune syndrome, 1005t–1006t Lamellar bodies, 67, 838
JIA. see Juvenile idiopathic arthritis absence, 61
Job’s syndrome, neutrophil dysfunction, 101 LAQ, 234–235
J receptors, 69 LAR. see Laryngeal adductor reflex
Jugular venous distention, 21 Larsen and Fryn syndrome, 312
Juvenile dermatomyositis (JDM), 857–860 Larsen syndrome, 1005t–1006t
asymptomatic pulmonary function Laryngeal adductor reflex (LAR), adequacy,
abnormalities in, 859 1111
chest radiograph in, 858f Laryngeal agenesis, 291
posteroanterior/lateral view of, 859f Laryngeal atresia, 304–306
clinical manifestations of, 857 Laryngeal chemoreflex apnea (LCRA), 1126
diagnosis of, 857–858 feeding infants with exaggerated,
epidemiology of, 857 1129–1130
pathogenesis of, 857 in infant, 1127f
prognosis of, 860 Laryngeal cleft, 306
pulmonary involvement in, 858–859, 858f type 1, 306, 306f
treatment of, 859–860 type 2, 306
Juvenile idiopathic arthritis (JIA), 850–854 type 3, 306
classification of, 852 type 4, 306
International League of Associations for Laryngeal closure, prevention of, 121
Rheumatology (ILAR) criteria for, Laryngeal disorders, 121, 1118–1124
852 operative assessment, 1118–1119
clinical manifestations of, 852 Laryngeal electromyography, 306–307
 Index 1183

Laryngeal function, impaired, 1097 Lipid-laden macrophage index (LLMI), 1101 Lower respiratory tract infection (LRTI)
Laryngeal mist airway (LMA), 346–347 bronchoalveolar lavage across studies (Continued)
Laryngeal obstruction, 227–228, 228f versus, 1102f severity of, 998
Laryngeal receptor reflex, 98–99 Lipid mediators, 102, 110 treatment of, 1004–1005
Laryngeal webs, 304–306, 305f, 1122 Lipid stains, for bronchoalveolar lavage, in human immunodeficiency virus–infected
Laryngoceles, 307 142 children and adolescents, 968–969
Laryngoesophagotracheal cleft, evaluation of, Lipoblastoma, 1079–1080 outcome, chest radiographs (impact), 429
1105f Lipopolysaccharide-induced expression, signs/symptoms, 428–429
Laryngomalacia, 16, 302–304, 726, 1121 inhibition of, 60 Low-viscosity periciliary gel layer (PCL),
in Down syndrome, 993–994 Lipoxin (LX), 114 121–122
presentation of, 304f, 317f Lipoxin A4 (LAX4), 102 LRTI. see Lower respiratory tract infection
Laryngotracheal reconstruction (LTR), 308 Liver biopsy, for non-CF-related liver disease, LTEC. see Laryngotracheoesophageal cleft
Laryngotracheobronchitis (LTB), viral, 792 LTR. see Laryngotracheal reconstruction
406–409 Liver disease Lucinactant (Surfaxin), 346–347
Laryngotracheoesophageal cleft (LTEC), 1100 clinical presentation of, 791 Ludwig angle, 9f
Larynx, 1106 diagnosis of, 792, 792f Lung(s)
abnormalities of, congenital, 300t, fat-soluble vitamin dosage, 798t abnormalities of, 374
302–306 management of, 793–794 adaptive defense of, 70
laryngeal atresia, 304–305 Living donor lobar lung transplantation, airway
laryngeal cleft, 306 success with, 983–984 branching of, 65f
laryngeal webs, 305 LKLF. see Lung Kruppel-like factor resin cast of, 64f
laryngomalacia, 302–304 LLMI. see Lipid-laden macrophage index alveoli-containing gas-exchanging area, 63
congenital conditions above, causing LMA. see Laryngeal mist airway biomarkers of, 846
neonatal airway obstruction, 299t Load compensation, 1131 biopsy of, usage of, 816
LAS. see “Lung Allocation System” Lobar overinflation, 149–150, 150f at birth, 71, 72f
Laser diffraction, aerosol, 262–263 Lobar pneumonia, 287, 353f capacities of, determined, 77
Late-onset pneumonia, 353–354 Lobectomy, 316–317 cellular characteristics of, 67t
Late preterm infants, autonomic instability, Local breath sounds, absence or presence of, changes in, 71
1132–1133 12 circulation, early injury, 373–374
Late pulmonary hypertension, mortality, Localized thoracic neurogenic tumors, primary closing volume in, 77
374f therapy for, 1081 compliance of, 75–76
Lateral chest Loculation, 435 congenital abnormalities of, 71
diameters, mean values of, 8f Loeffler’s-like syndrome, 958 congenital malformations, epidemiology of,
inspiratory indrawing of, 5 Löffler syndrome, 962 290
Lateral neck radiograph, 414 Lomustine (CCNU), 879 cystic fibrosis gene therapy and, 806
Laterality defects, 1041 Long-acting b-agonists cystic hydatidosis of, occurrence of,
LCRA. see Laryngeal chemoreflex apnea for asthma, 706–709 539–540
Lecithin to sphingomyelin ratio (L:S ratio), overuse of, 708 cysts, detection of, 539
344 Long-acting b-2 agonists, role, 454 development, relationship of, 371f
Leflunomide, 883 Long-acting muscarinic antagonists, for distal, 75f
Left atrial (LA) pressure, differences in, 582 asthma, 710 dried human, 64f
Left hemidiaphragm, blunt traumatic rupture Long-chain polyunsaturated fatty acids, in dynamic (flow-resistive) properties of,
of, 1070f asthma, 654 78–81
Left hilar bronchogenic cyst, 1082f Long-term injection medialization, 1121 elastic recoil of, 75, 75f–76f
Left lower lobe Long-term mechanical ventilation, 392 increased, 75
bronchus, obstructive emphysema (chest Long-term pulmonary fibrosis, hydrocarbon responsibility of, 75
radiograph), 1087f aspiration, 1097 elasticity of, 75
inflammatory stricture of, 1088f Loop diuretics, effects of, 379 in excretion of acids, 94
total atelectasis, chest radiograph of, 1088f Low-affinity IgE receptors (Fce:RII), 105 fibrous network of, structure of, 80
Left mediastinal outline, semicircular left Low contrast volume bronchography, 335 fibrous support of, 69f
convex distortion of, 149f Low dose carbon dioxide, 343 fluid movement in, 594f
Left-sided pneumothorax, 1011f Low lung function fluke disease in, 545–546
Left-sided primary spontaneous environmental exposures in, 53, 54f function decline, acceleration
pneumothorax, 1013f risk for respiratory disease, 50 (determinants), 452
Left ventricular hypertrophy (LVH), 374 Low oxygen saturation, 907 growth of
Legionella pneumophila, 931 Low pressure pulmonary edema, abnormal, 829f
Leigh disease, 384–385 distinguishing high pressure from, deficient, conditions associated with, 828t
Lesions, parenchymal, 313–320 587–588 and development, 70–71, 71f
Lethal pterygium syndrome, 1005t–1006t Lower airway optimization, 318
Leukemia, chlorambucil, 879 bronchoscope in, 140 hila of, 69
Leukocytes, in pulmonary endothelium, 70 histamine, presence, 739 histopathology of, 846–847
Leukotriene antagonists, 884 nasal inflammation, systemic effects, 739 hyperinflation of, 67–68
for asthma, 709–710 systemic disease, 765–766 inflation, degree of, 148
Leukotriene receptor antagonist, 708 upper airway disease on, influence of, innate defense of, 70
Levalbuterol, for asthma, 702–703 737–746 innervation of, 69
Levetiracetam, 882 Lower cranial nerve function, impact, interstitial matrix of, 69–70
LHR. see Lung-to-head ratio 1099–1100 lesion, cystic, 292t
Life measures, health-related quality, 231–232 Lower respiratory tract infection (LRTI) lymph flow in, pulmonary microvascular
Ligand-binding alpha chain (CD116), in Down syndrome, 996–999 pressures and, relationship of, 593f
836–837 clinical presentation of, 998 mechanics, modifications, sleep (impact) of,
Lights Criteria, pleural fluid, 1014t epidemiology of, 996–998 1044
Line probe assays, for tuberculosis, 491 etiology of, 996 metabolic functions of, 99–100, 100t
Linezolid, for nontuberculous mycobacterial histopathology of, 996 microvascular pressures in, changes in, 592
infections, 504t imaging of, 999–1000 multisystem congenital disorders that affect,
Lipid-containing macrophages, elevation of, prevention of, 1004–1005 332
1101–1102 rare diseases of, 999 new and emerging infections of, 466–474
1184 Index
Lung(s) (Continued)
normal
anatomy of, 63
mechanical ventilation in, 551
in number, 67t
parenchyma, acinus in, 66–67
perfusion, regional differences in, 83
post pneumonectomy, 73
pressure-volume curve of, 75–76, 75f–76f,
79f
recoil of, 80
resistance, measurement of, 79
sensory nerves from, 69
shape, 63
stabilization of, methods in, 75
structural features of, 580
total lung compliance, changes in, 76
ventilation of, 73
Lung abscess, 154, 155f, 436–437
clinical presentation, 437
diagnosis of, 404
etiologic agents with, 398t
parenteral antibiotic, usage, 437
“Lung Allocation System” (LAS), 981–982
Lung biopsy, 1091f
in hypersensitivity pneumonitis, 950–951,
951f
for invasive aspergillosis, 517
Lung bud morphogenesis, 27f
Lung clearance index, 206, 209f–210f
Lung compliance, reduction, 383
Lung defense, 120–133
adaptive, 129–132
innate, 121–129
intrinsic, 120–121
Lung densities, focal and multifocal, 153, 153f
Lung development, 254
adverse effects
across the life course, 248–249
operating antenatally, 249–251
aging, 254
antenatal, 248
antibiotics, 254
asthma and, 252–253
atopy and, 252–253
birth weight, and gestational age, 251
delivery, long-term effects of events, 251
early viral infections, 254
environmental pollution, 252
in health and disease, 248–255
microbiome, 254
molecular mechanisms directing, 31–37
morphogenetic periods of, 27t
nutrition on life course of, 252
postnatal lung growth, 251–252, 251b
stages of, 28f
stress and outcomes, 253
wheeze, acute attack and, 252–253
Lung diseases
associated with disruption, of pulmonary
surfactant homeostasis, 836–849
diagnosis and differential diagnosis of,
845–847
epidemiology of, 836–838
etiology of, 838–840
management, treatment, and prognosis
of, 847–849
pathology/pathogenesis of, 840–844
CF expression, 765
due to NKX2.1, incidence of, 838
eosinophilic, 944–967
drugs that cause, 958t
etiology of, 952, 952b
helminth-associated, 955b
pulmonary function/gas exchange
impairment in, 874t
Lung epithelial cell
development, 32f
morphogenesis and differentiation of, 33f
Lung function
abnormalities, 905–906
child health and disease, 212–230
in chronic lung disease, 979
low, environmental exposures in, 53, 54f
tests, for asthma, 695
Lung growth, 70–71, 71f
postnatal, 71–73, 72f–73f
prenatal, 70–71
reduced, environmental exposures in, 53
and relationship to disease outcomes,
252–254
Lung growth abnormalities, 826–828
clinical features of, 827–828
diagnosis of, 829
differential diagnosis of, 829
epidemiology of, 826–828
etiology of, 826–827
histologic findings in, 828–829
management of, 829
pathogenesis of, 826–827
prognosis of, 829–830
radiologic findings in, 828–830, 828f
treatment of, 829
Lung injury
chronic aspiration, impact, 1104f
evaluation, aspiration, 1103–1104
ventilator-induced, 600–601
Lung Kruppel-like factor (LKLF), 102,
113–114
Lung morphogenesis, 26–31
autocrine-paracrine signaling in,
importance of, 37
epithelial-mesenchymal interactions and,
35
gene transcription in, regulating, 32
molecular determinants of, 26–39
Nkx2-1 in, ablation of, 32–33
nontranscriptional mechanisms regulating,
34–35
reciprocal signaling during, 29f
secreted growth factors influencing, 36b
Lung opacities, 151–153, 151f–153f
Lung proliferation, control of, 36
Lung sounds, 10
asymmetry of, 10–11
nomenclature, 13t
objective acoustic measurements of, 11
Lung tissue, 401
compression, 1027
granuloma of, 912f
Lung transplantation
in cystic fibrosis, 786
dependence, 61
pediatric, 981–991
acute rejection after, 986
antimicrobial regimen for, 984
benefit of, 990
bronchiolitis obliterans in, 988
bronchiolitis obliterans syndrome in, 988
chronic lung allograft dysfunction in,
988–989
complications after, 985–989, 985f
contraindications to, 982–983, 983t
death, causes of, 990, 991f
early phase of, 986–987
functional outcome after, 990
future directions in, 990–991
ganciclovir, availability of, 984
hyperacute rejection after, 986
immediate posttransplant phase of,
985–986
immunosuppressive regimen for, 984
Lung transplantation (Continued)
indications for, 981–982, 982f
infection in, 986–987
late phase of, 987–989
Liou study in, 990
management issues in, 984–985
medication side effects in, 987
outcomes of, 989–990
posttransplant lymphoproliferative disease
in, 987–988
posttransplant management for, 984
quality of life in, 990
referral of, timing of, 981
rejection after, 985–987
somatic growth after, 990
surgical technique for, 983–984
survival after, 989, 989f
timing of, 981–982
viral infections in, 986
in pulmonary arterial hypertension,
578–579
Lung volumes, 76–78
asymmetrical lung volumes, 148–149,
150f, 150t
and capacities, 179f
definition of, 76–77, 77f
determined, 77
interpretation of, 78
measurement of, 78, 176–181
gas dilution technique for, 178–180,
179f–180f
whole body plethysmography for,
177–178, 177f
-pressure relations, changes in, 76
regional, 78, 79f
Lung-gut axis, 47–48
Lung-protective ventilatory strategies, usage
of, 593
Lung-to-head ratio (LHR), 292–293
LVH. see Left ventricular hypertrophy
Lymph node disease, complicated, in
tuberculosis, 480–483, 481f
Lymph nodes, biopsy of, 1090
Lymphadenitis, 910
Lymphadenopathy, isolated, in tuberculosis,
479, 479f
Lymphangiography, 1023–1025
Lymphangioleiomyomatosis (LAM),
819–820
gene mutations and, 38–39
Lymphangioma, 819
Lymphangiomatosis, 819
Lymphatic capillaries, 68–69
Lymphatic clearance, 582–583
Lymphatic system, 68–69, 1021–1022
development of, 1009f
respiratory disorders of, 817–819
Lymphatic tree, congenital disease of, 329
Lymphatic vessels, 68–69
location, 1007
Lymphocytic interstitial pneumonitis, 850
Lymphoid cells, in pediatric allergic asthma,
667–669
Lymphoid interstitial pneumonitis, in human
immunodeficiency virus–infected children
and adolescents, 977, 977f
Lymphomas, 1074
Lymphosarcoma, 1075
Lymphoscintigraphy, 1023–1025
Lysozyme, in airway, 125
M
MAb338, for HMPV infection, 470
MAC. see Mycobacterium avium complex
Macro malnutrition, impact, 443–444

Macrolide antibiotics
for pertussis, 532–533
for severe therapy-resistant asthma, 734
Macrophage activation syndrome (MAS), 852
Macrophage inflammatory protein (MIP)-1a,
947
Macrophages, 104–105, 127
Magnesium sulfate (MgSO4)
for acute episode of asthma, 717–719
for preschool asthma, 685
Magnetic resonance imaging (MRI), 169–170,
169f–170f, 334
for atelectasis, 1030–1031, 1031f
for cystic fibrosis, 792
for Down syndrome, 995, 995f
for pediatric pulmonary arterial
hypertension, 572
for pulmonary edema, 587
for pulmonary embolism, 890–891
for vascular rings, 619
Main pulmonary artery, dilation of, 19–21
Major bronchi, lumens (lesions), 1088f
Male, hypereosinophilic syndrome in, 965
Malignancy, special pulmonary considerations
in pediatric patients with, 604–605
Malignant teratoid tumors, 1077–1078
Malinosculation, 289
Malnutrition
in chest circumference, 6
predisposition, 443–444
Mannitol, as indirect bronchoprovocation
agent, 695–696
Manual hyperinflation, 286f
Manual techniques, 286f
MARS. see Medication adherence rating scale
MAS. see Macrophage activation syndrome
Mass lesions, therapeutic bronchoscopy for,
144
Mass median aerosol diameter (MMAD), 258
Mast cells, 103–104
in asthma, 667
Maternal antibiotic, 250
Maternal exposures, in respiratory disease,
55
Maternal factors, on asthma, 653
Maternal hypertension, 250
Maternal lifestyle, on asthma, 654
Maternal malnutrition, 249
Maternal obesity, 249
on asthma, 653
Maternal smoking, in respiratory disease,
50–51
Matrix protein inhibitors, for influenza, 463
Matrix proteins, in influenza virus, 460
Matthew-Wood syndrome, 1005t–1006t
Mature lung, injured, 60–61
Maximal exercise tests, 214
Maximal expiratory flow, cough spikes,
superimposition, 283f
Maximum intensity projection, 158–159
MCTD. see Mixed connective tissue disease
mDCs. see Myeloid dendritic cells
Measles virus infection, antibiotic treatment
of, 970t
Mechanical insufflation-exsufflation (MI-E),
599
NIV, combination, 284
Mechanical ventilation, 548–555
adjuvants to, 612–614
beta-adrenergic agonists, 613
corticosteroids, 612
extracorporeal life support, 613–614
inhaled nitric oxide, 612–613
neuromuscular blocking agents, 613
prone positioning, 612
surfactant, 613
Mechanical ventilation (Continued)
tracheostomy, 614
chronic dependency, causes of, 382
complications of, 554
composition of, 548, 549f
external monitors use of, 390–391
hemodynamic effects of, 551, 552f
history of, 548
indications for, 548
in pediatric intensive care, 596, 597t
management during, 553–554
comfort and, 553
in fluid management, 554
in gas humidification, 554
in monitoring, 553
in neuromuscular blockade, 554
suctioning and, 553
synchronization and, 554
weaning and extubation, 554
modes of, 548–550, 549f
in pediatric intensive care, complications of,
598–605
air leak syndromes as, 601, 601f
atelectasis as, 599–600
intensive care unit neuromyopathy as,
603–604
lung injury as, 600–601
mucus plugging as, 598–599
pneumonia as, 601–603, 602t,
603f
pulmonary hemorrhage as, 603
reduction, 391
in specific conditions, 551–553
in acute respiratory distress syndrome,
551–552
in bronchiolitis, 553
in congenital heart disease, 553
in neonate, 553
in normal lungs, 551
in status asthmaticus, 552–553
Mechanically ventilated patients, devices for,
269–270
Meconium aspiration syndrome, 58,
349–351
clinical features of, 350, 350f
epidemiology of, 349–350
management of, 350–351
pathophysiology of, 350
prevention of, 351
prognosis of, 351
Meconium ileus (MI), 794–795, 795f
nonoperative management of, 794–795
operative management of, 795
Meconium-stained amniotic fluid (MSAF),
349–350
Mediastinal bronchogenic cyst, 313–314,
313f–314f
Mediastinal chemodectomas, 1081
Mediastinal cysts
primary, 1081–1086
vertebral anomalies, presence of, 1085
Mediastinal dermoid cyst (benign cystic
teratoma), 1075–1076
Mediastinal distortion, 150–151, 151f
Mediastinal lipoma, 1079–1080
presence, reports of, 1079–1080
Mediastinal shift, 294–295
Mediastinal tumors, 1074–1086
neurogenic, 1080–1081, 1080f
Mediastinum
CT scan of, 1089f
mobility of, 1062
posterior gastroenteric foregut cysts of,
1085
vascular-lymphatic abnormalities of, 1079,
1079f
Index 1185
Mediators, 109f
antiinflammatory mechanisms of, 113–114
multiplicity and redundancy of, 108–109
Medical history
health status, impression of, 3
use of, 2
Medical Research Council (MRC), in
epidemiologic studies of asthma,
641–642
Medical stability, criteria for, 386t
Medical treatment, compliance, 4
Medication adherence rating scale (MARS),
727
Medication toxicity, following solid-organ
transplantation, 932
Medication-induced pulmonary edema, 591
Medium-chain acyl-CoA dehydrogenase
(MCAD) deficiency, 1139
Medulla oblongata, serotonin (5HT) neurons
importance of, 342
Melbourne Children’s Asthma Study, 646
Membranous subglottic stenosis, 308
Mendelian randomization studies, in asthma,
641f
Mendelian susceptibility, to nontuberculosis
mycobacterial disease, 499, 500t
Meniscus sign, 539
Menstruation, asthma and, 694
Mepolizumab, 752–754
for asthma, 710
biomarkers on direct use, 753
clinical application, 754
clinical studies, 753–754
mechanism of action, 752–753, 752f
mode of administration and dosing,
753
pharmacodynamics, 753
pharmacokinetics, 753
pharmacology, 753
safety, 754
target effects, 752–753, 752f
6-Mercaptopurine, 880
MERS-CoV. see Middle East respiratory
syndrome coronavirus
Mesalazine, 882
Mesenchymal cells, transient proliferative
capacity of, 830
Mesh nebulizers, 268, 269f
Mesodermal derivatives, 1077
Metabisulfite, asthma and, 694
Metabolic acidosis, 96
asthma and, 688
Metabolic alkalosis, 96
Metastatic pulmonary disease, CT study of,
1089f
Metered-dose inhaler (MDI), 120
directions for use of, with and without a
spacer, 714t
Methacholine (MCH), 220
bronchial provocation in children, 18f
direct bronchoprovocation testing and, 695,
695t, 701
Methemoglobinemia, 18
Methicillin-resistant Staphylococcus aureus
(MRSA) emergence, 766
Methicillin-sensitive Staphylococcus aureus
(MSSA), 435
Methotrexate, 879–880
4-aminopteroylglutamic acid, 879–880
Methotrexate-induced pneumonitis, diagnosis
of, 880
Methylation, 229–230
Methylprednisolone, for histoplasmosis,
510
Methylxanthines, for asthma, 710
M-HES. see Myeloproliferative variants HES
1186 Index
Mice
gene-targeted, lacking SP-B, 61
surfactant in, genetic deficiencies, 61
Micro malnutrition, impact, 443–444
Microbial recognition, in environmental
exposures, 51, 52f
Microbiology
empyema, 1015–1016
protracted bacterial bronchitis, 456–457
Microbiome, 47–48, 404–405, 796–797
of airway, asthma and, 701
Microgallbladder, 794
Microlaryngobronchoscopy (MLB), 300,
307–308
Microlaryngoscopy, 1118–1119
MicroRNA (miRNA), 47
Microscopic polyangiitis (MPA), 867–869,
896–897, 897f
Microscopic-observation drug-susceptibility
(MODS), for Mycobacterium tuberculosis,
490
Microscopy, 401
Microtubules, of primary ciliary dyskinesia,
1034
Microvascular filtration coefficient, 582
Middle ear, 1041
Middle East respiratory syndrome coronavirus
(MERS-CoV), 468
infection, 467–469
characteristics of, 467t
clinical features of, 468
diagnosis of, 468–469
differential diagnosis of, 468–469
epidemiology of, 467–468
etiology of, 468
imaging of, 468
laboratory findings on, 468
management and treatment of, 469
pathology/pathogenesis of, 468
physical findings on, 468
prevention of, 469
prognosis of, 469
pulmonary function tests for, 468
symptoms of, 468
Middle-lobe syndrome, 1027
Mild persistent asthma, 712–713
Miliary tuberculosis, 482, 483f
Milrinone infusion, for pulmonary
hypertension, 621
Minimal important difference (MID) score
establishment, 234
identification, 231
Minimally invasive surfactant therapy (MIST),
for newborn infants, 346
Minimum intensity projection, 158–159, 160f
Minocycline, 882
for nontuberculous mycobacterial
infections, 504t
6-minute walk distance (6MWD) test, 572
MIST. see Minimally invasive surfactant
therapy
Mitochondrial superoxide dismutase, decrease
in, 590
Mixed connective tissue disease (MCTD),
862–863
clinical manifestations of, 863
diagnosis of, 863
diagnostic criteria for, 863
epidemiology of, 862
Kasukawa’s criteria for, 863
manifestation of, 863
PAH in, impact of, 863
pathogenesis of, 862
prognosis of, 863
pulmonary involvement in, 863
treatment of, 863
MLB. see Microlaryngobronchoscopy
MMAD. see Mass median aerosol diameter
MMF. see Mycophenolate mofetil
Möbius syndrome, 1099–1100
Mode of delivery, on asthma, 655
Moderate asthma, National Asthma Education
Program definition, 738
Moderate persistent asthma, 713
Molecular methods, 403
Molecular terms, used in diagnostics, 399t
Molecular therapies, for cystic fibrosis,
800–811
challenges for, 810–811
combination therapy, 804–805
correctors, 804
declaration of interests in, 811
evaluation of, 810
gene therapy, 805–810, 805b
lungs and, 806
outstanding questions on, 808–810
recent progress in, 806–808, 807f
vector development and upcoming
clinical trials of, 808, 808f–809f
viral versus nonviral, 806
“Go/No-Go” decision point, 809
potentiators, 803–804
read-through agents, 801–803
transmembrane conductance regulator
modulators, small molecule, 800–801,
808
candidate drugs, identifying, 800, 801t
on protein expression, maturation, or
function, 800–801, 802f
protein with, 801, 802f
restoration of, 809
Molecules, in pediatric allergic asthma,
665–669
Mometasone, for asthma, 705
Monitoring, mechanical ventilation in, 553
Monocyte chemoattractant protein (MCP)-1,
947
Monocytic leukocytes, 407–408
Monoresistant tuberculosis, 494
Montelukast
adverse effects of, 710
for asthma, 708–709, 713, 719
leukotriene D4 receptor antagonist, 742
Moraxella catarrhalis, in asthma, 659
Morbidity
adult health care needs/patterns, 242
psychosocial, severe asthma and, 729
Morgagni hernia, 331–332, 331f
Mortality, in asthma, 646–647, 690
Motility disorders, 1115
treatment of, 1115
Mounier-Kuhn syndrome, 66, 312
Mouth, breathing, nasal obstruction (impact),
740
Moxifloxacin, for nontuberculous
mycobacterial infections, 504t
MPA. see Microscopic polyangiitis
MSAF. see Meconium-stained amniotic fluid
MSSA. see Methicillin-sensitive Staphylococcus
aureus
Mucin gel, properties of, 122
Mucoactive agents, 453–454
Mucociliary clearance
disruption, airways dehydration (impact),
762–763
measurement of, 122, 1040
in pulmonary host defense systems, 37
Mucociliary system, disorders of, 122
Mucociliary transport system, 63–65
Mucoepidermoid, 313
Mucoid impaction, 955
Mucor, pneumonia due to, 929
Mucorales, 522
Mucormycosis, 522–523
Mucosal edema, asthma and, 688
Mucosal function, impairment, 739–740,
740f
Mucus, 121–122
airway, 121
clearance, cilia-dependent, 122
layer, cilia and, 121
water contents of, 122
Mucus plugging
as complication of mechanical ventilation,
598–599
therapeutic bronchoscopy for, 144
Multichannel intraluminal impedance plus
pH-metry (MII-pH), 1115
Multidisciplinary coordinated care, 1094
Multi-drug resistant tuberculosis, 494, 495t,
972
Multifocal lung densities, 153, 153f–154f
Multilobular biliary cirrhosis, 792
Multi-omics approaches, for genotype-
phenotype associations, 47–48
Multiplanar reconstruction, 158, 158f
Multiplanar volume reconstruction, 158–159,
159f
Multiple aortopulmonary collateral arteries,
reperfusion pulmonary edema in,
622–624
Multiple breath washout technique, 205–207
clinical applications in infants and young
children, 209–210
feasibility in older children
quality control, acceptability criteria, and
limitations of, 207–209
main outcome measures of, 206–207,
207f–208f
physiological principles and assumptions in,
205–206, 206f
Multiplex ligation-dependent probe
amplification (MLPA), 40–41, 45t
Multitrigger wheeze (MTW), 649–650
Munchausen syndrome by proxy, 1139–1140
Muscarinic antagonists, long-acting, 710
Muscle fiber, in muscles of respiration, 84
Muscles, of respiration, 83–85
Muscular dystrophy, 121
Muscular pulmonary arteries, 68
Muscularization, of arteries, 68
Mutation, 40
Myasthenia gravis, 1047
Mycobacteria, 930f, 931
Mycobacterial disease, nontuberculous,
498–506
acquisition and potential for transmission
of, 500–501
alternative drugs for, 506
drug toxicity and clinical response of,
505–506
environmental risk factors and spatial
clusters of, 499–500
epidemiology of, 499–500
extrapulmonary
clinical manifestations of, 502–503
management and treatment of, 505
with human immunodeficiency virus
infection/acquired immune deficiency
syndrome, 499
management and treatment of, 503–506,
504t
Mendelian susceptibility to, 499, 500t
microbiology of, 498
nonpharmacologic treatment options for, 505
outcomes of, 506
prevalence of, 499
prevention of, 506

Mycobacterial disease, nontuberculous Nasogastric lavage, for hydrocarbon
(Continued) aspiration, 627–628
pulmonary Nasopharyngeal airway (NPA), 301
clinical criteria of, 502, 502b Nasopharyngeal specimens, 400
clinical manifestations of, 501–502, Nasopharyngeal swab, 396–397
501t, 502f National Asthma Education and Prevention
diagnosis of, 502–503 Program (NAEPP) guidelines, 711
management and treatment of, 503 National surveillance surveys, in asthma
microbiologic criteria of, 502 deaths, 647
Mycobacterium abscessus, 504–505 NATs. see Nucleic acid detection tests
Mycobacterium avium, 503 Natural killer (NK) cells, 129
Mycobacterium kansasii, 503–504 increased, 948
surgical treatment options for, 505 NCPAP. see Nasal continuous positive airway
in vulnerable populations and in modern pressure
environment, 501 ncRNA. see Non-coding RNAs
Mycobacterium, 477, 498 Near-obligatory nasal breathers, 1144
Mycobacterium abscessus species complex Nebulization, 410
pulmonary disease, treatment of, Nebulized epinephrine, 410–411
504–505 Nebulizers, 264, 267–269
Mycobacterium avium complex (MAC), 972 Neck, adenoid tissue enlargement/OSA, lateral
pulmonary disease, treatment of, 503 roentgenogram, 1153f
Mycobacterium kansasii pulmonary disease, Necrosis, in smoke inhalation, 630
treatment of, 503–504 Necrotizing bronchitis, pertussis and, 529,
Mycobacterium tuberculosis, 475 529f
culture of, 490 Negative intrathoracic pressure, 1008
DNA methodologies for, 490–491 Negative pressure breath, supply of, 387–388
immunology of, 477, 478f NEHI. see Neuroendocrine cell hyperplasia of
mycobacteriology of, 477 infancy
Mycophenolate mofetil (MMF), 984 Neonatal airway obstruction, 299t
Mycoplasma organism, 106 examination of a child with, 300–301
Mycoses, 507–527 Neonatal hyperoxia, neoalveolarization and,
Myeloid dendritic cells (mDCs), 105 254
Myeloid malignancies, classification of, 964b Neonatal intensive care unit
Myeloproliferative variants HES (M-HES), 966b apnea episodes, 1130
Myer-Cotton grading scale, 1119f pediatric pulmonologists, basis of, 1134
Myocardial contusions, blunt trauma of, Neonatal myasthenia gravis, 1047, 1050,
1067–1068 1053, 1056
Myocardial failure, 589 Neonatal pulmonary hypertension, 560–561
Myopathies, 1046 Neonatal respiratory distress syndrome,
clinical features of, 1050–1051 pulmonary edema and, 590
diagnosis of, 1053 Neonates
management of, 1056–1058 acute airway compromise, 1123
pathogenesis of, 1047–1048 breathing, nasal/oral route change,
Myositis-specific antibodies, presence of, 1144–1145
857–858 bronchopulmonary anatomy of, 63
Myotonic dystrophy, 40–41, 1051, 1053 chylothorax, 1024f
congenital, 1051 community-acquired pneumonia (CAP),
management of, 1058 treatment, 433
muscular, 1099 mechanical ventilation in, 553
Myxoma, commonness, 1086 reflexes, potentiated, 1144
NERD. see Nonerosive reflux disease
Nerve growth factor (NGF), 112
N Neural mechanisms, in inflammatory airways,
Nail fold capillary abnormalities, 860–861 111–112, 112f
Naloxone, use of, 339–341 Neurally adjusted ventilator assistance, 551
Narcotic-induced pulmonary edema, 591 in acute respiratory distress syndrome,
Nasal cavity, anatomic abnormalities in, 611–612
1108–1109 Neurally mediated bronchospasm, 740
Nasal congestion, 1041 Neuraminidase, in influenza virus, 460
NIV high flow, impact, 387 Neuraminidase inhibitors, for influenza,
Nasal continuous positive airway pressure 463
(NCPAP), methylxanthine response, Neurilemomas, pediatric rarity, 1080
failure, 1133–1134 Neuroblastoma, 1080–1081
Nasal discharge, 4 boys, presence of, 1081f
Nasal flaring, presence of, 345 Neuroendocrine cell hyperplasia of infancy
Nasal inflammation, systemic effects, 739 (NEHI), 425, 832–834
Nasal interfaces, 389 characteristic radiographic and pathologic
Nasal mask, 285f findings in, 833f
Nasal nitric oxide, usage, 1040 clinical features of, 832
Nasal obstruction, 4 diagnosis of, 833–834
Nasal passageways, inspection of, 16 differential diagnosis of, 833–834
Nasal polyps epidemiology of, 832
asthma and, 691 etiology of, 832
frequency of, 6 histologic findings in, 833
Nasal potential difference (PD), 773f management of, 834
Nasal-bronchial reflex, 740 pathogenesis of, 832
Index 1187
Neuroendocrine cell hyperplasia of infancy
(NEHI) (Continued)
prognosis of, 834
radiologic findings in, 832–833
treatment of, 834
Neuroendocrine cell prominence, small airway
obstruction severity (correlation) and,
832
Neuroepithelial bodies, oxygen-sensing
functions of, 65
Neurofibromatosis, 822–823
Neurofibromatosis type 1 (NF-1), 822–823
CT sagittal section in, 822f
Neurogenic mediastinal tumors, 1080–1081,
1080f
Neurogenic pulmonary edema, 590
Neurogenic tumors, radiographic examination
in, 1081
Neurokinin A, 112
Neurologic arch, 1108
Neurological injury, 1097
outcome prediction of, 637
Neuromuscular blockade, mechanical
ventilation in, 554
Neuromuscular blocking agents, for acute
respiratory distress syndrome, 613
Neuromuscular disease (NMD), 283–284
inadequate pump out, 383–384
physical therapy, 273
respiratory function testing in, 1051
special pulmonary considerations in
pediatric patients with, 603
spine deformities in, 1059
Neuromuscular junction diseases
clinical features of, 1050
diagnosis of, 1053
management of, 1056
pathogenesis of, 1047
Neuromyopathy, intensive care unit, as
complication of mechanical ventilation,
603–604
Neuroprotection, following drowning, in
induced hypothermia, 636
Neutrophilic airway inflammation, sputum
markers, 447
Neutrophil-mediated proteolytic activity, in
smoke inhalation, 631
Neutrophils, 106
bronchospasm, 220–221
characterized, 128
dysfunction (Job’s syndrome), 102
in pediatric allergic asthma, 669
Newborn infants
acute respiratory distress syndrome,
351–352
clinical presentation of, 351
management of, 351–352
pathophysiology of, 351
airway muscles of, bronchoconstriction of,
inadequate, 66
apnea, 343
aspiration pneumonia, 354, 354f
aspiration susceptibility, 1099
bronchopulmonary dysplasia, 367–381
chest wall in, 1044, 1045f
CPAP for, 347
early-onset pneumonia, 352–353
clinical presentation of, 352–353
management of, 353
gastroesophageal reflux (GER), 343, 366
high-frequency jet ventilation (HFJV),
347–348
high-frequency oscillatory ventilation
(HFOV), 347–348
hyaline membrane disease, 343–344
interstitial lung disease, 354
1188 Index
Newborn infants (Continued)
late-onset pneumonia, 353–354
lung maturation, factors, 345
meconium aspiration syndrome, 349–351
clinical features of, 350, 350f
epidemiology of, 349–350
management of, 350–351
pathophysiology of, 350
prevention of, 351
prognosis of, 351
periodic breathing, 343
persistent pulmonary hypertension of,
354–357
diagnosis and differential diagnosis of,
355–356
management of, 356–357
mortality and morbidity of, 357
pathophysiology of, 355
pneumomediastinum, 358
pneumopericardium, 359, 359f
pneumothorax, 358, 358f–359f
preterm, 347–348
pulmonary edema, 363–364
pulmonary hemorrhage, 364–365,
364f
pulmonary interstitial emphysema,
359–360, 360f
respiratory activity, 342–343
respiratory distress syndrome, 343–349
clinical presentation of, 345
diagnosis of, 345–346, 345f
differential diagnosis of, 345–346,
345f
epidemiology of, 344
management of, 346–348
morbidity of, 348–349
mortality of, 348–349
pathophysiology of, 344–345
preventative strategies of, 348
resuscitation, 340f
secondary pulmonary hypoplasia, 360–361,
361f
sensory feedback system in, 99
transient tachypnea of, 349
clinical presentation of, 349, 349f
epidemiology of, 349
management of, 349
pathophysiology of, 349
upper airway obstruction, 365–366
Newborn screening, 774f
Nexin, PCD and, 1034
NGF. see Nerve growth factor
NGS Panel, 45t
Nicotinamide adenine dinucleotide phosphate
(NADPH)
catalytic component of, cytochrome b558,
914
as electron source, 909
oxidase, 913–914, 913f
Nicotine
exposure, in pregnancy, 249
neoalveolarization and, 254
Niemann-Pick disease (NPD), 821–822
clinical features of, 822
epidemiology of, 821
etiology of, 821
management of, 822
pathobiology of, 821
prognosis of, 822
treatment of, 822
type B, 822
NIPPV. see Noninvasive positive pressure
ventilation
Nissen fundoplication, 1115
Nitrates, in pulmonary arterial hypertension,
578
Nitric oxide, 111, 577, 696–697
decreased, 903
endothelial, 696–697
exhaled, 116–117
fractional, 696–697, 697t, 701
inhaled, for pulmonary hypertension, 621
as short-acting selective vasodilator, 612
Nitric oxide synthase (NOS)
inducible form (iNOS), 111
NO formation, 1040
Nitroblue tetrazolium (NBT) test, 913
Nitrofurantoin, 881
Nitrosoureas, 879
NIV. see Noninvasive ventilation
Nkx2-1
ablation of, 32–33
mutations in, 38
NMD. see Neuromuscular disease
NNRTIs. see Nonnucleoside reverse
transcriptase inhibitors
Nocturnal asthma, 694
Nodlike receptors, 124–125
Nodules, 167, 168f
Noisy breathing, 16
causes, clarification of, 16
Nonadrenergic noncholinergic nervous
system, 69
Nonatopic asthma, 650–651
Nonatopic wheezers, 679
Noncardiac congenital anomalies, infant/
toddler diagnoses, 382
Non-CF related bronchiectasis, hospitalization
rate, 440
Nonciliated bronchiolar epithelial cells, 65
Non-coding RNAs (ncRNA), 34–35
Noncystic fibrosis bronchiectasis, respiratory
physiotherapy, 274–283
Noncystic fibrosis transmembrane
conductance regulator, 805
Noncytotoxic drugs, 877t, 881–884, 884t
Nonerosive reflux disease (NERD), 1115
Nonimmune-mediated alveolar hemorrhage,
899
Noninvasive nocturnal ventilatory support,
recommendation, 1055
Noninvasive positive pressure ventilation
(NIPPV), usage of, 610–611
Noninvasive ventilation (NIV), 386–387, 550
in acute respiratory distress syndrome,
610–611
MI-E, combination, 284
nocturnal, 386–387
Nonleukocyte-derived vasoactive mediators,
584
Nonnucleoside reverse transcriptase inhibitors
(NNRTIs), 971–972
Non-rapid eye movement (NREM) sleep, 1143
neural sites and, 1143
Nonresolving pneumonia, persistence/
recurrence, 431
Nonresponder, term (usage), 573
Nonsense variant, 40, 41f
Nonspecific interstitial pneumonia (NSIP)-like
pattern, 950–951
Nonsteroidal antiinflammatory drugs
(NSAIDs), 854, 884
asthma and, 693–694
Non-synonymous variant, 41f
Nontuberculosis mycobacteria (NTM)
in human immunodeficiency virus–infected
children and adolescents, 972
infections, 930
Nontuberculosis mycobacterial disease,
498–506
acquisition and potential for transmission
of, 500–501
Nontuberculosis mycobacterial disease
(Continued)
alternative drugs for, 506
drug toxicity and clinical response of,
505–506
environmental risk factors and spatial
clusters of, 499–500
epidemiology of, 499–500
extrapulmonary
clinical manifestations of, 502–503
management and treatment of, 505
with human immunodeficiency virus
infection/acquired immune deficiency
syndrome, 499
management and treatment of, 503–506,
504t
Mendelian susceptibility to, 499, 500t
microbiology of, 498
nonpharmacologic treatment options for,
505
outcomes of, 506
prevalence of, 499
prevention of, 506
pulmonary
clinical criteria of, 502, 502b
clinical manifestations of, 501–502,
501t, 502f
diagnosis of, 502–503
management and treatment of, 503
microbiologic criteria of, 502
Mycobacterium abscessus, 504–505
Mycobacterium avium, 503
Mycobacterium kansasii, 503–504
surgical treatment options for, 505
in vulnerable populations and in modern
environment, 501
Nontypable H. influenzae association, 427
Nonviral gene transfer agents, 806
Nonvolatile lipid-containing liquids, 1097
Normal respiratory flora, 399t
Normal (basic) sounds, in categories of
respiratory sounds, 14f
Northway’s old bronchopulmonary dysplasia,
66
Norwood cardiac repair, 1121
NOS. see Nitric oxide synthase
Nose, humidification at, 120
NPA. see Nasopharyngeal airway
NPD. see Niemann-Pick disease
NRTIs. see Nucleoside reverse transcriptase
inhibitors
NSAIDs. see Nonsteroidal antiinflammatory
drugs
Nuclear factor-kB (NF-kB), 109–110
activation of, 60
corticosteroid action and, 705
Nucleic acid detection tests (NATs), 403
Nucleoside reverse transcriptase inhibitors
(NRTIs), 971–972
Nursery, graduates, death prevention,
1134
Nutrition, 797–798, 798f, 798t
O
Obesity
asthma and, 656–657, 725
maternal, 653
asthmatic children and, 219
in Down syndrome, 1003–1005
gastroesophageal reflux disease and,
1114
Obligatory nasal breathers, 1144
Obliterative bronchiolitis, 938–939, 938f
Obstructing lesion, persistence of, 1089f
Obstructive apnea, 384
 Index 1189

Obstructive emphysema Oral endotracheal tube, tracheostomy Pancreatic disease, 788–799

chest radiograph revealing, 1087f techniques, 1064f enzyme supplements, 790
partial obstruction, 1088f Oral glucose tolerance test (OGTT), 794 fibrosing colonopathy, 790
Obstructive sleep apnea (OSA), 1120, 1147 Oral H1 antihistamines, usage, 741–742 treatment of, 790
adenotonsillar hypertrophy, association of, Oral motor development, 1107t pathobiology of, 788
1154 Oral secretions, aspiration of, 1103 pathophysiology of, 789f
ADHD, association, 1158 ORCC. see Outwardly rectifying chloride Pancreatic enzyme replacement therapy
animal models, 1159 channel (PERT), 790
conditions associated with, 1154–1156 Organ Procurement and Transplantation Pancreatic enzymes, 793
diagnosis of, 1156 Network (OPTN), 981–982 CF produces, 789
in Down syndrome, 995–996 Organ systems, review of, 4 release of, 788, 794
dynamic factors, 1148 Organ-restricted hypereosinophilic syndrome, Pancreatic function
early reports, 1157 966b assessment of, 790
epidemiology, 1152–1153 ORM1-like protein 3 (ORMDL3), in asthma, fecal pancreatic elastase-1 test, 790
intermittent hypoxia, consequence, 1158 653t testing, 772
inverse relationships, 1158–1159 Oronasal interfaces, 389 Pancreatic insufficiency (PI), 788
laryngomalacia and, 303 Oropharyngeal swabs, culturing, 772 management of, 790
long-term morbidity, 1157–1159 Oropharynx, inspection of, 6–7 mutations, impact, 761f
mechanisms of, 1158 Orthopnea, existence of, 5 Pancreatic sufficiency (PS), 789
oxygen desaturations, 1157–1158 OSA. see Obstructive sleep apnea Pancreatitis, 768, 791
pathophysiology, 1153–1154 OSAS. see Obstructive sleep apnea syndrome Pansinusitis, cystic fibrosis, 781
contribution, 1145 Oscillatory positive expiratory pressure, PAP. see Pulmonary alveolar proteinosis
polysomnographic measures, 1152f 279–281 PAPA. see Practicalities and Perceptions
polysomnography, 1156–1157 Oscillatory ventilation, conventional versus, in Approach
respiratory compromise, risk for, 1159 pediatric intensive care, 596–598, 598f PAQLQ. see Pediatric Asthma Quality of Life
risk factors, 1148–1149 Oseltamivir, for influenza, 463, 463t Questionnaire
short-term morbidity, 1157–1159 OT. see Ocular toxocariasis Paradoxical breathing, 5, 1145
treatment of, 1159 Ouchterlony double immunodiffusion, 949f Paradoxical respiratory excursions, 1064
ventilatory control, 1148 Outdoor air pollution, in asthma, 661–663 Paragonimiasis, pulmonary
Obstructive sleep apnea syndrome (OSAS), Out-of-hospital cardiac arrest, 637–638 clinical features of, 545–546
905 Outwardly rectifying chloride channel (ORCC), diagnosis of, 546
Obstructive sleep apnea-18 (OSA-18), 239, chloride transportation, 758–759 differential diagnosis of, 546
239t OVA-induced eosinophil, 218 epidemiology of, 545
Obstructive sleep disorders-6 (OSD-6), 239, Ovalbumin (OVA), 218 etiology of, 545
239t Ova/parasites, in stool examination, 537 management for, 546
Occlusive chest wall dressing, application of, Overexpansion, 320–322 pathology/pathogenesis of, 545
1066f Overlap hypereosinophilic syndrome, 966b prognosis for, 546
Ocular larva migrans, 535 Overlap syndrome, 689–690 treatment of, 537t, 546
Ocular toxocariasis (OT), 535 Oxaliplatin, 880 Paragonimus westermani, 545
OGTT. see Oral glucose tolerance test Oxidants, in smoke inhalation, 630 Parainfluenza pneumonia, 931
Older patient, respiratory disease in, 3 Oxidative stress, 111 Parainfluenza virus (PIV), 407
Oligohydramnios, 826 Oxygen Paramyxoviridae, 407
Omalizumab, 750–752 administration of, 93 Paranasal sinuses, 1041
for ABPA, 957–958 for bronchiolitis, 425 Paranasal sinusitis, 14
for asthma, 710 delivery, to tissues, 92 Parasites
bioavailability, 751 desaturations, 1157–1158 extirpation of, 542
clinical application, 752 in diffusing capacity, 88 in stool examination, 537
clinical studies, 751 humidified, for asthma, 719 Parasympathetic nerves, 69
in adult, 751 mixtures, hazards of, 93–94 Paratracheal nodes, in pulmonary
in children, 751 in pulmonary arterial hypertension, tuberculosis, 480–481
distribution, 751 577–578 Parenchymal lesions, 313–320
dosing strategies, 751 in sensory feedback system, 99 Parental reporting, of wheeze, 677–678
elimination, 751 therapy, 92–94 Parental supervision, adherence and, 727
mechanism of action, 750–751, 750f toxicity, 372 Pari PEP device, 281f
mode of transmission, 751 transport, 89–91, 90f, 91t Parietal pleura, 1007
pharmacodynamics, 751 treatment, 3 Paroxysmal coughing, 1028–1029
pharmacokinetics, 751 Oxygen radical production, 61 Paroxysmal supraventricular tachycardia,
pharmacology, 751 Oxygenation 21
safety, 751–752 inhaled nitric oxide (iNO), 377–378 Partial SP-B deficiency, 847
for severe therapy-resistant asthma, ventilation index and, contrast to, 609 Partially muscular pulmonary arteries, 68
733 Oxyhemoglobin dissociation curves, 90f Particle size, 258–259
Omics, 229–230 aerosol, 258t
Open, sucking thoracic wall injury, 1065f PAS staining. see Periodic acid-Schiff staining
“Open tube” bronchoscope, 134 P Passive smoking, 252
Open-lung biopsy, 942 Paclitaxel, 880 severe asthma and, 729
Opitz-Frias syndrome, 306 PAH. see Pulmonary arterial hypertension Patau syndrome, 1005t–1006t
Opportunistic infections (OIs), in human Paired-like homeobox 2B (PHOX2b), 1150 Patent ductus arteriosus (PDA), 18, 361–363
immunodeficiency virus–infected children Palivizumab, 426 clinical features of, 362, 362f
and adolescents, 968 use of, 379 clinical importance of, 346–347
fungal, 975 Pallister-Hall syndrome, 306 epidemiology of, 361–363
Optical coherence tomography, 170 Pallor, BRUE definition and factors for etiology of, 361
OPTN. see Organ Procurement and inclusion and exclusion, 1126t management of, 362–363
Transplantation Network Palpable lymph nodes, biopsy of, 1090 pathogenesis of, 361–362
Oral contraceptive pill, asthma and, 694 Palpation, 8–9 prevention of, 362
Oral corticosteroids, for preschool asthma, order, 8 prognosis of, 363
684–685 PAM. see Pulmonary alveolar microlithiasis repair, 1121
1190 Index
Patient/provider discussion (facilitation),
HRQoL (usage), 233
Patient-related variables, 258t, 260–261
Patient-reported outcomes (PRO), 231–240
definition, 231
development, 231
importance, FDA recognition, 231
psychometric criteria, 232t
utilization, 231
Pattern-recognition receptors, 123–125
PAVM. see Pulmonary arteriovenous
malformation
PCD. see Primary ciliary dyskinesia
PCR. see Polymerase chain reaction
PCV. see Pneumococcus conjugated vaccine
PDA. see Patent ductus arteriosus
PDH. see Progressive disseminated
histoplasmosis
Peak expiratory flow, 183–184
rate
handheld devices for, 715
monitoring of, for asthma, 714–715
zones for, 715
Peak flow meter, asthma and, 695
Pectus excavatum, 1048, 1051–1052, 1058
Pediatric acute respiratory distress syndrome,
607t
extracorporeal life support in, 613–614
Pediatric asthma
allergic, cells, molecules, and cytokines
involved in, 665–669
NO measurement for, 117
Pediatric Asthma Caregiver’s Quality of Life
Questionnaire, 235, 236t–237t
Pediatric Asthma Quality of Life Questionnaire
(PAQLQ), 234, 236t–237t
Pediatric blunt chest trauma, 1062
Pediatric care models, differences, 242
Pediatric chest radiology, 147
Pediatric deaths, occurrence, 1062
Pediatric fiberoptic endoscopic evaluation of
swallowing, advantages and
disadvantages, 1110t, 1111
Pediatric history, structure of, 2–4
Pediatric Index of Mortality (PIM2), 637
Pediatric intensive care unit (PICU)
history of, 596
intubation in, 596
mechanical ventilation in
complications of, 598–605
indications for, 596, 597t
respiratory complications of, 596–605
special pulmonary considerations in
in patients with congenital heart disease,
604
in patients with malignancy or
hematopoietic stem cell
transplantation, 604–605, 605t
in patients with neuromuscular disorders,
603
transfer, 386
ventilation strategies in, 596–598
conventional versus oscillatory
ventilation, 596–598, 598f
extracorporeal membranous oxygenation,
598
Pediatric Laryngology and Bronchoesophagology,
406
Pediatric lung transplantation, 981–991
antimicrobial regimen for, 984
benefit in, 990
causes of death after, 990, 991f
complications after, 985–989, 985f
contraindications to, 982–983, 983t
early phase in, 986–987
future directions in, 990–991
Pediatric lung transplantation (Continued)
immediate posttransplant phase in,
985–986
immunosuppressive regimen for, 984
indications for, 981–982, 982f
issues in, 984–985
late phase in, 987–989
outcomes of, 989–990
functional, 990
posttransplant management for, 984
quality of life in, 990
somatic growth after, 990
survival after, 989, 989f
technique in, 983–984
timing for, 981–982
Pediatric OSA, clinical findings, 1156b
Pediatric patients, 134–146
flexible bronchoscopes for, 134–135
Pediatric Quality of Life Inventory, 232
Pediatric respiratory disorders, 242b
physical therapies, 273–288
Pediatric Risk of Mortality (PRISM), 637
Pediatric systemic inflammatory diseases, with
pulmonary involvement, serologic tests
for, 851t
Pediatric thorax, features of, 1062–1063
Pediatric videofluoroscopic swallow study,
1110
Pediatric Voice Handicap Index (pVHI), 235t
Pediatric Voice Outcome Survey (PVOS),
235t
Pediatric Voice-Related Quality of Life
(PVRQOL), 235t
PedsQL-Asthma Module, 236t–237t
Pena Shokeir syndrome, 1005t–1006t
Pena-Shokeir phenotype, 291
Pendelluft, 197
Penicillamine, 882–883
PEP. see Positive expiratory pressure
Percussion, 9–10
subjective assessment of, 10
Perfusion scanning, 333
Pericardial coelomic cysts, 1085
Pericardial cyst, location (posteroanterior/
lateral chest radiographs), 1086f
Pericardial tumors, 1086
Pericardioperitoneal canals (primorial pleura
cavity), 1008f
Pericardium, primary neoplasms of, 1086
Pericyte, in interstitium, 70
Peri-Engraftment respiratory distress
syndrome, 936–937
Periodic acid-Schiff (PAS) staining
for bronchoalveolar lavage, 142
usage of, 844
Periodic breathing, 4–5
definition, 1145
epochs, 1131
newborn infants, 343
Periodic leg movements, 1155f
Periostosis, 448
Peripheral blood mononuclear cells (PBMCs),
methylation, 229–230
Peripheral chemoreflexes, 1146–1147
Peripheral cyanosis, 17
Peripheral edema, 21
Peripheral interlobular septal thickening,
HRCT scan in, 861f
Peripheral vascular disease, 860–861
Peritonsillar abscess, 417–418
Permanent medialization laryngoplasty,
1121
Persistent airflow limitation, severe asthma
and, 732–733
Persistent bacterial bronchitis, inflammation
and, 101
Persistent pulmonary hypertension of
newborn (PPHN), 354–357, 560,
1001–1002
clinical presentation of, 355
diagnosis and differential diagnosis of,
355–356
management of, 356–357
mechanical ventilation in, 553
mortality and morbidity of, 357
pathophysiology of, 355
treatment of, 577
Persistent rhinitis, diagnostic testing, 741
PERT. see Pancreatic enzyme replacement
therapy
Pertactin, in Bordetella pertussis, 530t
Pertussis, 287, 528–534
clinical features of, 530–531
diagnosis of, 532
differential diagnosis of, 532
epidemiology of, 528, 529t
etiology of, 528
imaging of, 531–532
incidence, 437
laboratory findings of, 531–532
management of, 532–533
pathology/pathogenesis of, 528–530, 529f,
530t
physical findings on, 531, 531t
prevention of, 533
prognosis of, 533
pulmonary function testing for, 531–532
symptoms of, 530–531
treatment of, 532–533
Pertussis toxin (PTX), 530t
PET. see Positron emission tomography
PFTs. see Pulmonary function tests
PGD. see Primary graft dysfunction
pH, alterations in homeostasis in, asthma and,
688
PHACES syndrome, 307
Phaeohyphomycosis, 526
Phagocyte function, defect in, 909
Pharmacologic agents, lung injury caused by,
876–885, 884t
Pharmacologic therapy, of chylothorax, 1025
Pharyngitis
diagnosis of, 404
etiologic agents associated with, 397t
Pharyngoconjunctival fever, in adenoviral
infections, 473
Phase III slope (SIII) analysis, for washout
curve, 207, 208f
Phenotype, 45
genotype correlations, 760–762, 774–775
Phenotypic variation, of asthma, 648–651
atopic and nonatopic asthma, 650
inflammatory subtypes of, 650
severe asthma, 650
temporal progression of symptoms of,
648–650, 649f
Phonation, 1108
Phosgene gas, inhalation of, 591
Phosphatidylcholine (PC), presence of, 836
Phosphatidylglycerol (PG), requirement, 344
Phosphodiesterase inhibitors, in pulmonary
arterial hypertension, 577
Phosphodiesterase type 5 (PDE5) inhibitors,
577
Phospholipids
content of, 61
phosphatidylcholine species of, 57
Physical activity
in asthma, 660–661
cystic fibrosis, 223–225
insufficient, 230
lung disease, 225

Physical examination, 4–14
auscultation in, 10–14
technique of, 12–14, 13f–14f, 13t–14t
thoracic acoustics, 10–12, 10f–11f
inspection in, 4–7, 5f–8f
palpation in, 8–9
percussion in, 9–10, 9f
taste and smell in, 14
Physical fitness, 212–213
Physical irritation, occurrence of, 3
Physical therapy
offering, 274f
secretion-mobilizing techniques, 284
Physiologic complications, of bronchoscopy,
146
Physiologic measures, integration, 234
Physiotherapy
chest, 1031
conditions, 287
importance, 273
inhaled therapies, timing, 283f
preoperative and postoperative
management, 285
principles, 273
role, 273
treatments, 273
PI. see Pancreatic insufficiency
PIG. see Pulmonary interstitial glycogenosis
PIM2. see Pediatric Index of Mortality
PIV. see Parainfluenza virus
Piwi-interacting RNA (piRNA), 47
PL. see Pulmonary lymphangiectasia
Plain radiography, 147
Plasma
bacterial antigens, detection, 430
colloid osmotic pressure, decreased, 583
Plasma cell granuloma (inflammatory
pseudotumor), 1072
Plasmacytoid DCs (pDCs), 105
Plastic bronchitis, in single ventricle
physiology, 624
Platelet-activating factor (PAF), as
inflammatory mediators, 110
Plethysmography, 177–178, 177f
Pleural cavity, blood in, 1065–1066
Pleural effusion, 1013–1014
causes, 1014t
chest radiographs, obtaining, 435
clinical course, 435
CT scans, usage, 435–436
diagnosis of, 404, 436
empyema, relationship, 435–436
etiologic agents with, 398t
following solid-organ transplantation, 931
outcomes, 436
randomized trials, 436
signs of, 148
treatment, 436
Pleural fluid, 148
culture, 430
drainage, 436
formation, 1008–1010
Gram stain, 436
Lights Criteria, 1014t
microbiological testing, 436
ultrasonogram of, 1089f
Pleural liquid excess, accumulation,
1008–1010
Pleural space, 1007–1026
anatomy, 1007–1008
bacteria, entry, 435
embryology, 1007–1008
fluid, 1013–1025
physiology, 1008–1010
Pleural tuberculosis, 482, 482f
Pleuroperitoneal shunt, chylothorax, 1025
Pleuropulmonary blastoma, 1073
Pleximeter, placement of, 9
pMDIs. see Pressurized metered dose inhalers
PMN. see Polymorphonuclear leukocytes
Pneumatoceles, association, 435
Pneumococcal pneumonia, 430f
presumptive diagnosis, 434f
Pneumococcus conjugated vaccine (PCV)
in bacterial pneumonia, 969–970
in HIV-infected children, 969–970
impact, 437
Pneumocystis jirovecii, 927–928
Pneumocystis jirovecii pneumonia, in human
immunodeficiency virus–infected children
and adolescents, 973–975
antibiotic treatment of, 970t
Pneumomediastinum, 358, 359f
Pneumonectomy, 316–317
Pneumonia
in children, 427–438
definition of, 427
diagnosis of, 404, 431
in Down syndrome, 1003f
environmental contributions to, 55
etiologic agents with, 397t
in human immunodeficiency virus–infected
children and adolescents, 968–969
antibiotic treatment of, 970t
associated with Bordetella pertussis, 969
bacterial, 969–970
cytomegalovirus in, 973
Pneumocystis jirovecii, 973–975, 974f
prevention of, strategies for, 975b
varicella zoster, chest radiograph of, 973f
hydrocarbon, 627f
incidence, vaccines (impact), 427
necrotizing pneumonia, 435
prevention, 437
prognosis, 437–438
slowly resolving pneumonias, 433–435
vaccine trials, efficacy estimates, 427
ventilator-associated, 554, 601–603, 602t,
603f
Pneumopericardium, 359, 359f
Pneumothorax, 358, 358f–359f
absence of, 148, 154–156, 156f
cystic fibrosis and, 781
flying and diving after, 1013
left-sided, 1011f
measurement of, 1012f
pleural space, 1010–1013
primary spontaneous, 1012f
quantify, methods, 1011t
secondary, causes of, 1010t
traumatic, 1064–1065
treatment of, 1011
Pollen-sensitive asthma, exacerbations, 117
Pollution, antenatal effects of, 249
Polyclonal hypergammaglobulinemia, 911
Polydrug resistance, 494
Polyhydramnios, 291–292, 294–295
Polymerase chain reaction (PCR)
CAP analysis, 431
for invasive aspergillosis, 517
for pertussis, 532
Polymorphisms, 40, 61
in hospitalization, in asthma, 647
occurrence, 1137
Polymorphonuclear leukocytes (PMN),
407–408, 590
Polymyositis (morbidity/mortality), ILD
(impact) of, 858
Polyphonic wheezing, 17
Poor adherence, in hospitalization, in asthma,
647
Pores of Kohn, 66–67
Index 1191
Portable suction machines, 390
Portable ventilators, 388
Portal hypertension, 791–793
Porto-pulmonary hypertension (PPHTN),
following-solid organ transplantation,
933
Posaconazole
for mucormycosis, 523
for pulmonary mycoses, 507–508
Positioning, 287f
Positive end expiratory pressure (PEEP)
delivery of, 347–348
intrinsic, offsetting, 388
Positive expiratory pressure (PEP), 279,
280f–281f
Positive pressure devices, 388–389
Positron emission tomography (PET), 173
Post-effort breath sound, 12
Posterior cricoid cartilage grafting,
1121–1122
Posterior gastroenteric foregut cysts, 1085
Posterior laryngeal clefts, 1123
Posterior mediastinal cyst, enteric
characteristic of, 1085
Posteroanterior chest radiograph,
overexposure, 1082f
Posteroanterior film, in children, 147
Postinfectious bronchiolitis obliterans, 447
Postmenstrual age, 1130–1131
monitoring, 1134
Postnasal drip, inflammatory material, 740
Postnatal pulmonary vasculature, 69
Postoperative atelectasis, 1029
Postoperative pulmonary hypertensive crisis,
619–621
background of, 619–620
evaluation and monitoring of, 621
pathophysiology of, 621
risk factors for, 620–621
treatment of, 621
Posttransplant lymphoproliferative disease,
932–933, 933f, 939
in pediatric lung transplantation, 987–988
Posttraumatic atelectasis, 1067, 1069f
Postural drainage (PD), positions, ACBT
(usage), 279–281
Postural hypotension, effect (minimization),
285
Potentiators, 803–804
Potts shunt, in severe pulmonary arterial
hypertension, 578
PPHN. see Persistent pulmonary hypertension
of newborn
Practicalities and Perceptions Approach
(PAPA), 727
Prader-Willi syndrome (PWS), 385
sleep-related/breathing-related
manifestations, 1151–1152
Praziquantel, for pulmonary paragonimiasis,
546
Pre-Bötzinger complex, 1144
Prednisolone, for severe therapy-resistant
asthma, 733
Prednisone, in ABPA, 957
Preemptive inhaled corticosteroids, for
preschool asthma, 682, 682f
Preemptive leukotriene receptor antagonists,
for preschool asthma, 682
Preemptive oral corticosteroids, for preschool
asthma, 682–683
Preemptive strategy, effective, for preschool
asthma, 683
Pregnancy and childbirth, asthma on,
653–655
fetal growth and birth size, 654–655
maternal factors, 653–654
1192 Index
Pregnancy and childbirth, asthma on
(Continued)
maternal lifestyle and environment, 654
mode of delivery, 655
Pregnant women, immunization of, in HIV,
973
Premature emphysema, tobacco exposure and,
249
Premature infants
apnea, 1125, 1130–1134
spontaneous, 1133f
home monitoring, 1134
intercostal tone, loss, 1131
Premature neonates, quiet sleep, nonapneic
controls, 1131
Premature termination, 184–185
Prematurity, 255–256, 255b, 826
chronic lung disease of, infant pulmonary
function testing for, 814–815
lung disease, 225
Prematurity and Respiratory Outcomes
Program (PROP), 378f
Prenatal exposures
pathway, 50t
in respiratory disease, 55
Prenatal maternal stress, 250
Prenatal onset pulmonary hypoplasia,
828–829
ranges of, 826–827
Prenatal ultrasound, 291
Prenatal (saccular) walls, in postnatal lung
growth, 73
Preoperative swallowing, 1119
Preschool age child, asthma in, 677–685. see
also Preschool asthma
Preschool asthma, 677–685
clinical patterns of wheeze in, 679
diagnosis of, 677–678, 678t
environmental factors of, 679
epidemiology of, 677, 678f
natural history of, 679
pathology of, 679
treatment of, 680–685
future directions in, 685
initial management of exacerbations in,
683–685
nonpharmacological, 680
pharmacological, 680–683
Preschool wheeze, 671–674
developing airway microbiome, 673
differential diagnosis of, 678t
eosinophilic inflammation on
inception, 672
persistence, 672–673
episodes of, 677
exacerbations
during and in between, airway pathology
in, 672, 672f
infection associated, airway inflammation
in, 671–672
impact of immune maturation on, 673
phenotypes of, 671
progression to asthma of, 674
protection from, 673
response to antiinflammatory therapies,
671
severity and frequency of, 674
Preschool-age children
wheezing in, preventive strategy for, 681
Prescription records, adherence and, 727
Pressure control ventilation, 549, 549f
Pressure support ventilation, 549–550, 549f
Pressure-volume curves, 59
of lung, 75f–76f
Pressurized metered dose inhalers (pMDIs),
257, 264–266, 265f
Preterm infants
aspiration susceptibility, 1099
periodic breathing, epochs, 1131
with respiratory distress syndrome, 60
Preventive strategy, for specific wheezy
children, 681
Primary cardiac tumors, 1086
myxoma of, 1086
Primary ciliary dyskinesia (PCD), 63–65, 122,
256, 1034–1043
classification of proteins mutated in,
1038f
clinical features of, 1040–1042, 1040t
diagnosis of, 1042
epidemiology of, 1034
etiology of, 1034–1036
functional defects of, 1039–1040, 1039f
gene mutations and, 38–39, 1037t
genetic heterogeneity of, 1036
genetics, 1036–1038
HRQoL measure, 239
management of, 1042–1043
nasal nitric oxide, 1040
normal motile ciliary structure and function
of, 1034–1036, 1035f–1036f
pathogenesis of, 1036–1040
pathology of, 1036–1040
primary ciliopathies, overlap, 1035–1036,
1042
prognosis of, 1043
pulmonary function tests of, 1041
recommendation, 240
respiratory physiotherapy, 274–283
specific ciliary defects causes, 1038f
surgical intervention of, 1043
treatment of, 1042–1043
ultrastructural ciliary defects, 1038–1039,
1039t
Primary graft dysfunction (PGD), 986
Primary intrathoracic malignancy, 318
Primary mediastinal cysts, 1081–1086
Primary pulmonary lesions
benign, 1072
malignant, 1072–1073
Primary spontaneous pneumothorax, 1012f
Prior pleurodesis, 982
PRO. see Patient-reported outcomes
PROBIT trial, in asthma, 655–656
Problematic severe asthma, 723b
initial evaluation of, 723–724
not asthma at all, 724
nurse-led assessments in, 727t
Procarbazine, 880
Procaspase 8, 132–133
Progressive disseminated histoplasmosis
(PDH), 509
Progressive primary infection, in tuberculosis,
479, 479f
Progressive right heart failure, in untreated
IPAH, 564–565
Proinflammatory cytokines, 60
Proinflammatory transcription factors,
109–110
Prone positioning
in acute respiratory distress syndrome, 612
sudden infant death syndrome and, 1140
PROP. see Prematurity and Respiratory
Outcomes Program
Prophylactic caffeine, use of, 343
Propranolol therapy, for tracheal
hemangioma, 308, 308f
Prostacyclin, inhaled, for pulmonary
hypertension, 621
Prostacyclin analogues, 575–576
inhaled, 575–576
oral, 576
Prostaglandins (PG), 110
Protectin D1 (PD1), 102
Protein C/S deficiency, 572–573
Proteinase 3 (PR3), 869
Proteoglycans, in interstitium, 69–70
Protracted bacterial bronchitis, 439–440,
440f, 456–459
bronchoscopic appearances, 456f
children, outcomes, 456–457
clinical features of, 457
defining, 457–458
diagnosis of, 457–459
diagnostic criteria of, 458b
differential diagnosis of, 457–459
disease burden, 456
epidemiology of, 456
management of, 459
pathology/pathogenesis of, 456–457
prognosis of, 459
treatment of, 459
Protracted hematogenous tuberculosis, 482
Proximal airway wall, T-lymphocytes in,
103
Proximal conducting zone (bronchial tree),
63
Proximal upper airway lesions, subcutaneous
emphysema (presentation), 1066
Proxy-respondents, use of, 233
PS. see Pancreatic sufficiency
Pseudallescheria boydii, 525–526
Pseudoglandular period, 28f, 29–30
Pseudoglandular phase, 248
Pseudoglandular stage, 70
Pseudomonas aeruginosa, 319, 777
airways colonization, 757
Psychological stress, in hospitalization, in
asthma, 647
Psychosocial development, quality of life and,
383
PTE. see Pulmonary thromboembolism
Pubertal growth spurt, respiratory failure
during, 384
Pulmonary agenesis, 321–322, 321b
Pulmonary alveolar microlithiasis (PAM),
820–821
clinical features of, 820
diagnosis of, 820
differential diagnosis of, 820
epidemiology of, 820
etiology of, 820
management of, 820–821
pathogenesis of, 820
prognosis of, 820–821
radiographic features of, 820f
Pulmonary alveolar proteinosis (PAP), 939
autoimmune, 839
autosomal recessive CSF2RA mutations,
impact, 839–840
autosomal recessive CSF2RB mutations,
impact, 840
as surfactant homeostasis disorder, 836
syndrome, genetic disorders and, 840
Pulmonary arterial hypertension (PAH),
850
assessment of, 570–572
atrial septostomy in, 578
childhood, 556–579
classification of, 556–557
clinical presentation of, 569
definition of, 556–557
diagnosis of, 570–572
epidemiology of, 557–560
etiology of, 557–560
genetics of, 560
impact of, 863
lung transplantation in, 578–579

Pulmonary arterial hypertension (PAH)
(Continued)
management of, 572–577
anticoagulation in, 572–573
calcium channel blockade in, 573–575
endothelin receptor antagonists in,
576–577
general measures in, 572
phosphodiesterase inhibitors in, 577
prostacyclin analogues in, 575–576
pathogenesis and pathobiology of, 565–568
apoptosis in, 568
cell proliferation in, 568
endothelial dysfunction in, 567–568
growth factors in, 568
inflammation in, 568
pathways responsible for vasoconstriction
in, 566–567, 566f
thrombosis in, 568
pathophysiology of, 568–569
pediatric
diagnostic workup for, algorithm
illustrating, 570f
pharmacological approach to, algorithm
illustrating, 574f
severity of disease in, 574f
physical examination findings of, 569–570
severe, Potts shunt in, 578
targeted therapy for, 573–575
targets for current or emerging therapies in,
566f
Pulmonary arterial (PA) pressure, 582
Pulmonary arterial tree
absent or small, 324–326, 325f–326f
arrangement, disorders of, 324–326,
325f
congenital disease of, 324–326
Pulmonary arteries, 68
Pulmonary arteriovenous malformation
(PAVM), 298, 327–329
associated disorders of, 328
clinical features of, 327–328, 328f
clubbing in, 19
complications, 329
diagnosis of, 328
etiology of, 327
prevalence of, 327
screening, 328
treatment of, 328
Pulmonary artery sling, 309, 309f, 615–619,
618f
clinical presentation of, 619
diagnosis of, 619
slide tracheoplasty for, 620f
treatment of, 619, 619f–620f
Pulmonary atresia, reperfusion pulmonary
edema in, 622–624
Pulmonary blood
flow
increased, 589
ventilation and, 214
vessels, definition of, 580–581
volumes, increased, 585
Pulmonary capillaries, endothelium in,
continuity of, 580
Pulmonary capillaritis, isolated, 898
Pulmonary capillary bed, 68
reduction of, 18
Pulmonary capillary pressure, 582
Pulmonary changes (evaluation), CT scanning
(usage), 772
Pulmonary circulation, 81, 893
methods of evaluating, 83
physiology of, 568–569
Pulmonary compression injury (traumatic
asphyxia), 1067
Pulmonary disease, 1120
bleomycin and, 877–878
cardiovascular signs of, 6–7
in cystic fibrosis, 777–787
presence of, 863
Pulmonary disease associated with congenital
heart disease, 615–625
with Fontan repair of single ventricle
physiology, 624–625
pulmonary hypertension and postoperative
pulmonary hypertensive crisis,
619–621
background of, 619–620
evaluation and monitoring of, 621
pathophysiology of, 621
risk factors of, 620–621
treatment of, 621
pulmonary vein stenosis, 621–622,
622f–623f
related to cardiac surgery and
cardiopulmonary bypass, 624–625
reperfusion pulmonary edema, 622–624
vascular rings and slings, 615–619,
616f–618f
clinical presentation of, 619
diagnosis of, 619
treatment of, 619, 619f–620f
Pulmonary edema, 287, 363–364, 363f,
580–595, 936
anatomic considerations in, 580–581
clinical disorders causing, 588–591
clinical presentation of, 586–588
in Down syndrome, 1001t
fluid clearance in, 584–585
fluid movement in, factors for, 581–583
interstitial forces, 582
lymphatic clearance, 582–583
microvascular filtration coefficient, 582
surface tension, 583
vascular forces, 582
vascular permeability, 582
following solid-organ transplantation,
931
formation of, opposition in, factors in,
583
high altitude, 590–591
high pressure, 584, 588–589
distinguishing low pressure from,
587–588
low pressure, distinguishing high pressure
from, 587–588
mechanisms causing, 583–584
medication-induced, 591
narcotic-induced, 591
neurogenic, 590
pathophysiologic consequences of,
585–586
quantitation of, in patients, 588
reexpansion, 589–590
in smoke inhalation, 630
therapy for, 591–593
Pulmonary epithelium, in pulmonary
immunity and asthma pathogenesis,
670–671
Pulmonary function tests (PFTs), 219, 586
abnormalities of, 854–855
asthma and, 691–692
for children, 175–176, 176f
for Churg-Strauss syndrome, 962–963
cystic fibrosis and, 782
for forced expiration measurement,
182–189
assessment of bronchodilator response
and, 189, 190f
physiological principles and assumptions
of, 182–184, 183f
Index 1193
Pulmonary function tests (PFTs) (Continued)
quality control, acceptability criteria, and
limitations of, 184–185
raised volume-rapid thoracoabdominal
compression in, 185–186,
186f–187f
rapid thoracoabdominal compression in,
185–186
for gas mixing measurement, 205–210
multiple breath washout technique,
205–207, 206f–210f
for hypersensitivity pneumonitis, 950
for infants, 174–175, 175f
for lung volume measurement, 176–181
gas dilution technique for, 178–180,
179f–180f
whole body plethysmography for,
177–178, 177f
for resistance and compliance measurement,
189–205
forced oscillation technique in, 199–205,
200f–201f, 203f–204f
general considerations for, 194,
194f–195f
interpretation of, 191–194, 191f–193f
measurement of interrupter resistance in,
196–198, 197f
physiological principles and assumptions
in, 190–191
plethysmographic measurement of
airway resistance and, 198–199,
199f
single-breath occlusion technique,
194–196, 195f
Pulmonary heart disease, 19–21
Pulmonary hemorrhage, 364–365, 364f,
893, 894b
as complication of mechanical ventilation,
603
rarity of, 855
Pulmonary host defense
surfactant protein D (SP-D) in, 57
systems, development of, 37–38
Pulmonary hypertension (PH), 19–21,
619–621, 905
background of, 619–620
bronchopulmonary dysplasia and,
357–358, 560–561
classification of, evolution of, 556–557,
557b–559b, 557t
decrease, 373
evaluation and monitoring of, 621
in human immunodeficiency virus infected
children, 979–980
management of, 907
pathophysiology of, 621
pediatric
clinical presentation of, 569
pharmacological therapy for,
561t–563t
rarity of, 855–856
risk factors for, 620–621
treatment of, 621
vascular changes in, pathologic mechanisms
underlying, 565f
Pulmonary hypertensive vascular disease,
pediatric
broad schema of 10 basic categories of,
557b, 557t
classification of, 558b–559b
Pulmonary hypoplasia, 291
associated with oligohydramnios,
1005t–1006t
Pulmonary immunity
altered, in asthma, 665, 666f
structural airway cells in, 670–671
1194 Index
Pulmonary infections, in
immunocompromised child, 923
approach to, 940
clinical presentation of, 923–924
fungal pathogens, 927–930
infectious agents in, 924–931
invasive diagnostic studies for, 941–942
noninvasive diagnostic studies for, 940–941
parasitic agents, 931
pathogens associated with, 924t
prevention strategies for, 942
pulmonary coinfections, 931
radiographic testing for, 940, 940t
viral pathogens in, 924–927
Pulmonary inflammation, exaggeration/
persistence, 445–446
Pulmonary injury, 634–635
management of, 636
outcome of, 636–637
surfactant depletion, 634–635
Pulmonary interstitial emphysema, 154, 155f,
359–360, 360f
Pulmonary interstitial glycogenosis (PIG),
830–832, 999, 1000f
clinical features of, 830
diagnosis of, 830–832
differential diagnosis of, 830–832
epidemiology of, 830
etiology of, 830
histologic findings in, 830, 831f
management of, 832
pathogenesis of, 830
presentation of, 830
prognosis of, 832
radiologic findings in, 830
treatment of, 832
Pulmonary involvement
small vessel vasculitides with, 868t
vasculitis syndromes and, 867–869,
868t
Pulmonary Langerhans cell histiocytosis,
961–962
Pulmonary lesions
in hydrocarbon aspiration, 627
x-ray for, 911
Pulmonary lobes, projections of, 13f
Pulmonary lymphangiectasia (PL), 817–819
chest HRCT in, 818f
clinical features of, 817–818
epidemiology of, 817
etiology of, 817
imaging of, 818
management of, 818
pathogenesis of, 817
prognosis of, 818–819
Pulmonary microstructure, forces on, 58–59
Pulmonary microvascular pressures, lung
lymph flow to, relationship of, 593f
Pulmonary mycoses, 507–527
antifungal drugs for, 507–508
endemic, 508–515
in hosts with impaired immunity, 515–526
Pulmonary parenchyma, tumor (presence) of,
1089f
Pulmonary resistance, increased, 120–121
Pulmonary sarcoidosis
decision to treat, 866
presenting symptoms of, 864–865
Pulmonary sequestration, 319–320
Pulmonary surfactant, 57, 836
homeostasis, lung diseases associated with
disruption of, 836–849
diagnosis and differential diagnosis of,
845–847
epidemiology of, 836–838
etiology of, 838–840
Pulmonary surfactant (Continued)
management, treatment, and prognosis
of, 847–849
pathology/pathogenesis of, 840–844
Pulmonary thromboembolism (PTE),
887–892
clinical features of, 889
diagnosis of, 889–890
differential diagnosis of, 889–890
epidemiology of, 887
etiology of, 887–888, 888t
imaging for, 888, 890f
Pulmonary toxicity, BCNU and, 879
Pulmonary tuberculosis
in human immunodeficiency virus–infected
children and adolescents, 968,
971
empiric therapy for, 971
treatment of, 493–494, 493t
Pulmonary tumors, in human
immunodeficiency virus–infected children
and adolescents, 977–978
Pulmonary vascular disease, 374, 565–566
Pulmonary vascular pressures, 81
Pulmonary vascular resistance, 82, 82f
in single ventricle physiology, 624
structural changes, impact, 373
Pulmonary vascular surface area, increased,
583–584
Pulmonary vascular system, 68, 68f
endothelium of, 68
Pulmonary vasculature, in Down syndrome,
1001–1003
Pulmonary vasodilation, for pulmonary
hypertension, 621
Pulmonary veins, 68
misalignment of, 825–826
Pulmonary venous tree
abnormal, 326
congenital disease of, 326–327
veins, absence of, 326–327, 327f
Pulmonary vessels, physiologic classification
of, 81
Pulmonary-renal syndrome, 867, 871
Pulse oximetry monitoring, 390–391
Pulsus paradoxus, 21
Pump failure, impact, 383
Pump output, inadequacy, 383–384
Purulent rhinitis, 909–910
pVHI. see Pediatric Voice Handicap Index
PVOS. see Pediatric Voice Outcome Survey
PVRQOL. see Pediatric Voice-Related Quality of
Life
Pyrazinamide, for tuberculosis, 493t
Q Reexpansion pulmonary edema, 589–590
Quadriplegia, 1045
Quality adjusted life years (QALYs), in asthma,
648
Quality of life (QOL)
in asthma, 648
measures, 453
maximum, 452
Quiet sleep, 1143–1144
Quinsy, 417–418
R Renal crisis, 860–861
Racial disparities, sudden infant death
syndrome and, 1141
Radial spoke, primary ciliary dyskinesia and,
1034
Radiation fibrosis, 934, 934f
Radiographic swallow studies, value,
1100–1101
Radiography
for asthma, 698, 699f
chest, for tuberculosis, 484–485, 484f–485f
Radioimmunoassay (RIA), in hypersensitivity
pneumonitis, 949
Radionuclide imaging, 172–173, 172f
Raised volume-rapid thoracoabdominal
compression, 185–186, 186f–187f
Rales, 13–14
Randomized control trial (RCT), in asthma,
640, 641f
RANTES. see Regulated on activation, normal
T cell expressed and secreted
Rapid antigen tests, for influenza, 462
Rapid eye movement (REM)
hypoventilation during, 384
REM-associated obstructive apneic events,
polygraphic tracing, 1153f
Rapid eye movement (REM) sleep, 1143
neural sites and, 1143
physiologic characteristics of, 1143
Rapid thoracoabdominal compression, 184f,
185–186
Rapidly adapting vagal (irritant) receptors, 69
RAR. see Retinoic acid receptors
Rare childhood lung disorders, 817–824
Raster lines, projection of, 6
Rasterstereography, 6f
Raynaud phenomenon, 860–861
vasodilating drugs for, 863
RDI. see Respiratory disturbance index
RDS. see Respiratory distress syndrome
Reactivation pulmonary tuberculosis, 481,
482f
Reactive oxygen species (ROS), production of,
372
Reactive thrombocytosis, 1018
Rebound tenderness, clinical signs of, 1071
Receptor molecules, localization of, 35
Receptor-mediated signal transduction, 35
Reciprocal signaling, during lung
morphogenesis, 29f
Recombinant human CuZn superoxide
dismutase (rhSOD), association, 372
Reconstructive airway surgery, GERD and,
1114–1115
Recruited cellular defenses, 127–129
Rectal prolapse, 796
Recurrent illness, 2
Recurrent laryngeal nerve palsy, in cardiac
surgery, 1002t
Recurrent pneumonia, long-term risk of,
878
Recurrent respiratory infections, in Down
syndrome, 993t
Recurrent syncope, 578
Reflux aspiration
biomarkers, 1101–1102
evaluation, 1101
treatment considerations for, 1102–1103
Regional lung volumes, 78, 79f
Regulated on activation, normal T cell
expressed and secreted (RANTES), 947
Regulatory T cells (Tregs), 106–107
Rejection, after pediatric lung transplantation,
985–987
Relaxation phase, 121
Reproductive tissues, cystic fibrosis, 767
Resident cell defenses, 126–127
Residential mold contamination, 946–947
Residual pancreatic function, development,
767
Residual volume (RV), 76
determining, 77

Resistance, 189–190
forced oscillation technique in, 199–205,
200f–201f, 203f–204f
general considerations for, 194, 194f–195f
interpretation of, 191–194, 191f–193f
measurement of interrupter resistance in,
196–198, 197f
physiological principles and assumptions in,
190–191
plethysmographic measurement of airway
resistance and, 198–199, 199f
single-breath occlusion technique,
194–196, 195f
Reslizumab, 754
Resolvin E1 (RvE1), 102
Resonant sounds, 10
Respiration
initiation, birth, 338
Lavoisier, stipulation, 1044
muscles of, 83–85
regulation of, 98, 98f
tissue, 97–98
Respiratory activity, impact of, 342–343
Respiratory bronchiole (RB), light micrograph
of, 66f
Respiratory compromise, risk, 1159
Respiratory control mechanisms, 1144–1147
Respiratory depression, at birth, 339
Respiratory disability, long-term management
of, 1056–1057
Respiratory disease
adventitious respiratory sounds, indication
of, 11
care, HRQoL/physiologic measures
(integration feasibility), 234
disease-specific aspects, 242
in Down syndrome, 1001–1003
environmental contributions to, in children,
49–56
epigenetic mechanisms in, 50–53
family tree of, 54f
pathways in, increased, 51f, 53–54
risk of, increased, 54f
vulnerability of children in, 49–54, 50t
impact of, 2
long-term consequences of, in children,
247–256, 248b, 248t
of lymphatic system, 817–819
manifestations, description of, 3
in newborn, 338–366
progressiveness, 765
structural and physiologic basis of, 63–100
Respiratory distress, 1080–1081
in pulmonary tuberculosis, 480f
Respiratory distress syndrome (RDS)
acute, 351–352
alveolar surfactant pool sizes in, 58
clinical presentation of, 345, 349
diagnosis of, 345–346, 345f
differential diagnosis of, 345–346, 345f
epidemiology of, 344
management of, 346–348
morbidity of, 348–349
mortality of, 348–349
newborn infants, 343–349
pathophysiology of, 344
preterm infant with, 60
preventive strategies of, 348
risk, decreased, 60
surfactant homeostasis, disruption of, 836
surfactant treatment of, 61–62
type II alveolar epithelial cells and, 67
Respiratory disturbance index (RDI), in Down
syndrome, 995, 996f
Respiratory drive, abnormal, 384–385
Respiratory epithelium, 126
Respiratory failure, 939–940
pathophysiology of, 383
progression of, 384
Respiratory function, 373
Respiratory heat loss (RHL), 220
Respiratory illness
microbiological diagnosis of, 396–405
microbiological tools for, 401–404
molecular assays commonly use for
diagnosis of, 399t
Respiratory infections, occurrence, 1079
Respiratory mechanics, impairment of,
931–932
Respiratory morbidity, progression of,
383–384
Respiratory muscles
disorders
chest wall and, 1044–1061
considerations, 1044–1045
failure, clinical signs of, 1046b
fatigue, 84, 1045–1046
weakness, NMD (impact), 283–285
Respiratory pathogens, 396
diagnosis, clinical specimens for, 399–401
Respiratory pattern, changes in, 1065f
Respiratory physiotherapist/therapist,
physiologic knowledge, 273
Respiratory physiotherapy, 274–287
Respiratory pump, abnormal, 384
Respiratory rate, 5f
increase, 391
longitudinal documentation of, 4
Respiratory rhythm, generator, 1144
Respiratory sounds, 14t
abnormal, 12
characteristics of, 13
documentation of, 13
intensity of, 12
spectra of, 11f
Respiratory specimens
and diagnostic testing, 398t
screening of, 399t
Respiratory symptoms
daily activities, relationship between, 3
reduction, efficacy (decrease), 1102–1103
Respiratory syncytial virus (RSV), 926
in asthma, 657–658
infections
occurrence, 427
severity, 442–443
treatment of, 379
Respiratory syncytial virus bronchiolitis
coinfection, 421
high-risk population for, 421
population risk for, 420
reinfection, 421
Respiratory syncytial virus-positive
nasopharyngeal aspirate, presence,
431f
Respiratory tract
acute inflammation, 101
diagnostic imaging for, 147–173
pitfalls of, 162–163
radiation dose for, 163–164, 164t–165t
review of findings in, 161–162, 161t,
162f–164f
Resting conditions, diaphragm (usage), 1044
Retinoic acid receptors (RAR), in gene
expression, 26
Retropharyngeal abscess, 416–417, 417f
Reverse transcription polymerase chain
reaction (RT-PCR), influenza-specific,
462
Rhabdomyoma, impact of, 1086
Rhinitis therapy, effects, 738–739
Rhinorrhea, bronchiolitis and, 421–422
Index 1195
Rhinosinusitis
asthma and, 725–726
invasive, 516
Rhinovirus, asthma and, 657–658, 692–693
Rhinovirus C infection, 471–472, 472f
characteristics of, 467t
clinical features of, 471
diagnosis of, 472
differential diagnosis of, 472
epidemiology of, 471
etiology of, 471
imaging of, 472, 472f
laboratory findings on, 472
management and treatment of, 472
pathology/pathogenesis of, 471
physical findings on, 471
prevention of, 472
prognosis of, 472
pulmonary function tests for, 472
symptoms of, 471
Rhizopus, pneumonia due to, 929
RHL. see Respiratory heat loss
Rhonchus, 13–14
Ribonucleic acid (RNA)
interference, for HMPV infection, 470
noncoding, 34–35
Ribosomal RNA (rRNA), 47
Ribs
breakage, illustration of, 1063f
explosive blasts, 1067
fractures, 1063–1064
multiple fractures, 1063
uncomplicated fractures, treatment, 1064
Rifabutin, 971–972
for nontuberculous mycobacterial
infections, 504t
Rifampicin, 971–972
resistance, 494
Rifampin, for tuberculosis, 492, 493t
Right aortic arch, in association with aberrant
left subclavian artery, 615, 617f, 620f
Right hemithorax, chest radiograph for, 149f
Right scalene lymph node group, right lung
drainage, 1090f
Right ventricle, adaptation of, 568–569
Right ventricle hypertrophies, 568–569
Right ventricular outflow tract, dilation of,
19–21
Right-to-left shunting, 17–18
Rigid bronchoscope, 134
“open tube” bronchoscope, 134
size for, 134
Rigid bronchoscopy, 135–136, 1118–1119
RIG-like receptors, 125
Roberts syndrome, 1005t–1006t
ROS. see Reactive oxygen species
Round pneumonia, 431f
Routine immunization, for cystic fibrosis, 785
RSV. see Respiratory syncytial virus
Rubor, inflammation and, 101
RV. see Residual volume
S
SABAs. see Short-acting b agonists
Saccular bronchiectasis, CT scan findings,
445f
Saccular cysts, 307, 307f
Saccular outgrowth, in foregut endoderm,
26–27
Saccular period, 28f, 30–31
Saccular phase, 248
Saccular stage, 70–71
Saccular walls, in postnatal lung growth, 73
Saccule, 70
Safe sleeping zone, for asthma, 715
1196 Index

Safety profile, in preschool asthma, 683 Sensitization, IgE-mediated, asthma and, Short-rib thoracic dysplasia 11, 1005t–1006t
Salivary aspiration 688–689 Shrinking lung syndrome, 856
evaluation, 1103 Sensory feedback system, 98–99 chest radiograph in, 856f
treatment, 1103 Sensory nerve endings, inflammatory products Shunt, 89
Salmeterol on, 112 Shunt-like effect, 89
for asthma, 707 Sepsis, occurrence of, 635 SIBO. see Small intestine bacterial overgrowth
with fluticasone, 708 Septal thickening, 166–167, 167f Sickle cell anemia, 903
Salmeterol Multicenter Asthma Research Trial Sequestrated segment, 289 Sickle cell disease, 903–908
(SMART) trial, 707–708 Sequestration, pulmonary, 319–320 clinical features of, 904–906
Sanger sequencing, 45t Serial reevaluations, 573 diagnosis of, 906
Sarcoid airway, bronchoscopy picture of, 865f Serology, 402–403 epidemiology of, 903
Sarcoidosis, 863–867 Serum, biomarkers of, 846 etiology of, 903
airway involvement in, 865 Serum albumin, 60–61 management of, 906–907
cardiac/neurologic/ocular/renal Serum eosinophilic cationic protein, in pathogenesis of, 903–904
involvement in, 867 eosinophilic inflammation, 670 prevention of, 907–908
clinical manifestations of, 864 Serum immunoreactive trypsinogen, 788 prognosis of, 908
clubbing in, 19 Serum tests, for asthma, 697–698 sickling, 904
diagnosis of, 865–866 17q21 gene, in asthma, 653t Sickle chronic lung disease, 905
clinical evaluation of, 866b Severe asthma, 647, 650, 722–736 Sickle hemoglobin C disease (HbSC), 903
early onset, 863 adherence and, 727–728 SIDS. see Sudden infant death syndrome
endobronchial biopsy for, usage of, 866 electronic monitoring and, 728, 728f Signal regulating protein a (SIRPa), actions of,
epidemiology of, 864 home visit and, 727 60
etiology of, 864 parental supervision and, 727 Signaling molecules, gradients of, 35
granulomatous lesion of, 864 prescription records and, 727 Sildenafil
granulomatous lung lesion of, progression use of inhaler devices and, 727–728 for pulmonary hypertension, 621
of, 865 airway inflammation assessment in, use of, 380
incidence of, 864 731–732 Single mediator, 108–109
laboratory abnormalities of, 864 airways disease deconstructed, 723, 731b Single nucleotide polymorphisms (SNPs),
pathogenesis of, 864 definitions of, 722–723 40
prognosis of, 867 differential diagnoses of, 725t Single nucleotide variants (SNVs), 40, 41f
pulmonary involvement in, 864–865 environmental factors of, 728–729 Single-breath occlusion technique, 194–196,
transbronchial lung biopsy (TBLB) for, usage exacerbating phenotype, treatment of, 195f
of, 866 734–735 Sinuses
treatment of, 866–867 with fungal sensitization, 733–734, 734b aspirates, culturing, 772
SARP. see Severe Asthma Research Program diagnostic criteria for, 734t disease, incidence definition (attempts),
SC phocomelia syndrome, 1005t–1006t hospital admission for, 729–730, 730b 742
Scedosporium apiospermum, 525 investigation of, modified diagnostic protocol Sinusitis, 768
Scedosporium prolificans, 526 for, 724, 724f asthma and, 693
School age allergic asthma National Asthma Education Program diagnosis of, 404
eosinophilic inflammation in, clinical definition, 738 etiologic agents associated with, 397t
relevance of, 670 overarching principles of, 722 Sirolimus (Rapamycin), 883
noninvasive biomarkers of, 670 persistent airflow limitation in, assessment Situs inversus, totalis, 1034
stability of, 670 of, 732–733 Skin abnormalities, 860
mechanisms of, 669–670 problematic, 723b Skin testing, for asthma, 698
pathology of, 669–670, 669f initial evaluation of, 723–724 Skin-prick testing, 747
Scimitar syndrome, 326, 326f not asthma at all, 724 SLE. see Systemic lupus erythematosus
Scleroderma, 850, 860–862 nurse-led assessments in, 727t Sleep
clinical features of, 860–861 psychosocial morbidity and, 729 architecture, mechanisms, 1143
diagnosed with, ILD onset and, 861f steroid responsiveness assessment in, 732, arousal, 1145
epidemiology of, 860 732t developmental aspects, 1143–1144
lung disease and, 861f therapy-resistant, 730–732 neural circuitry, 1143
pathogenesis of, 861 annual assessment of, 735 practices, risk factors, 1136–1137
prognosis of, 862 invasive investigation of, 730–731, 731b, types, differentiation, 1143
pulmonary involvement in, 861–862 731f Sleep-disordered breathing, 905
treatment of, 862 monitoring child with, 735 asthma and, 726
Scoliosis treatment of, 733–735 bruxism, 1154f
classification of, 1048b types of, 723 polysomnographic montage, 1157b
congenital, 1048, 1052 Severe Asthma Research Program (SARP), spectrum of, 1152
postoperative pulmonary function, 650 Sleep-related breathing disorders, 239
1059–1060 Severe combined immunodeficiencies, in Down syndrome, 995–996
Screening sinus computed tomography, 698 920–921 HRQoL measures, 239
Scrofula, in extrathoracic tuberculosis, 483 Severe persistent asthma, 713–714 list, 239t
Secondary central hypoventilation syndromes, Severe therapy resistant asthma, 674–675, recommendation, 239
1151–1152 675f SLIT. see Sublingual immunotherapy
Secondary hypoalbuminemia, 1018 Sex discordance, in asthma, 645–646 Slowly adapting (stretch) receptors, 69
Secondary pneumothorax Sex-determining region Y (SRY)-related Slowly resolving pneumonias, 433–435
causes of, 1010t HMG-box (SOX) 17 (SOX17), in gene pneumococcal pneumonia, diagnosis of,
treatment of, 1012–1013 expression, 26 430f
Secondary pulmonary hypoplasia, 360–361, SGS. see Subglottic stenosis Slow-twitch fibers, in muscles of respiration,
361f Short interfering RNA (siRNA), 47 84
Secretion-mobilizing techniques, 284 Short-acting b agonists (SABAs) Small airway obstruction severity
Sedation, for infants, in lung function test, adverse effects of, 703 (correlation), neuroendocrine cell
175 for asthma, 701–703 prominence and, 832
Segmental tracheomalacia, presence, 1123 Short-acting b-2 agonists, role, 454 Small bowel motility, 796
Segmentectomy, 316–317 Short-latency somatosensory evoked potentials Small intestine bacterial overgrowth (SIBO),
Self-management, promotion, 244 (SSEP), 637 796–797

Small lungs
bilateral congenital, causes of, 322t
due to extrapulmonary mechanical factors,
322t
Small molecule cystic fibrosis transmembrane
conductance regulator modulators,
800–801, 808
candidate drugs, identifying, 800, 801t
on protein expression, maturation, or
function, 800–801, 802f
protein with, 801, 802f
restoration of, 809
Small primary pneumothorax, treatment of, 1012
Small volume liquid inhalers, 269
Small-cell carcinoma, CT scan of, 1089f
SMART regime, for severe therapy-resistant
asthma, 733
SMN. see Survival motor neuron
Smoke, 630
Smoke inhalation, respiratory complications
of, 626–633
carbon monoxide poisoning, 629, 629f
clinical findings of, 631
diagnosis and differential diagnosis of, 631
epidemiology of, 628–629
etiology of, 629
management and treatment of, 631–632
pathogenesis of, 629
pathology of, 629–630
pathophysiology of, 630–631
prevention of, 633
prognosis of, 633
surface burns and, pulmonary
complications of, 632–633
Smooth muscle cells, in interstitium, 70
Sneezing, as airway reflexes, 120–121
Sniffing, hydrocarbon, 628
Snoring, 905, 1147
SNPs. see Single nucleotide polymorphisms
SNVs. see Single nucleotide variants
Socioeconomic factors, in increase disease risk,
49–50
SOD. see Superoxide dismutase
Sodium bicarbonate, use of, 339–341
Sodium channel, 122
Sodium transport model, alveolar epithelium
(usage), 585f
Soft bedding, 1140–1141
Soft tissue, 148
Solid organ transplantation
infectious risks in, 924, 925f
pulmonary complications following,
931–934
Soluble extracellular pattern-recognition
proteins, 125–126
Somatic gene therapy, 786
Somatic growth
after lung transplantation, 990
restriction, environmental exposures in, 53,
54f
Somatostatin, chylothorax, 1025
Sonic hedgehog (SHH), mutations in, 38
Sounds
digital respirosonogram of, 10f
pathways of, 10–11
reflection of, 12
transmission of, 12
Southern California Children’s Health Study
(CHS), in asthma, 660–661
SOX2
in gene expression, 26
mutations in, 38
SOX9, mutations in, 38
Spacer devices, for asthma, 714, 714t
Spatial variation, in prevalence of asthma,
644–645
SP-B protein, 344–345
Spear syndrome, 1005t–1006t
Specific airway resistance (sRaw), 198
Specific resistive loops, 199, 199f
Specific T helper cell phenotypes, 667f
Specimens
blood, 401
bronchoscopy, 400
clinical, for respiratory pathogen diagnosis,
399–401
throat and nasopharyngeal, 400
Spinal cord injury, 285
pump output, inadequacy, 383–384
Spinal muscular atrophy (SMA), 121
clinical features of, 1049–1050
type I, 1049
type II, 1049–1050
diagnosis of, 1053
drug therapy, perspectives, 1056
management of, 1055–1056
type I, 1055–1056
NIV/MI-E, combination, 284
pathogenesis of, 1047
prognosis of, 383
severity, antenatal onset, 291
type II, 1050f, 1056
type III, 1050, 1056
Spinal muscular atrophy with respiratory
distress type I (SMARD I), 1055–1056
Spirometric measurements of lung volumes in
cooperative children, 178
Spirometry, 184
for asthma, 695
Spondylocostal dysostosis, 1049
Spondylocostal dysostosis 1, 1005t–1006t
Spondyloepiphyseal dysplasia congenita,
1005t–1006t
Spondylothoracic dysplasia, 1049
Sporothrix schenckii, 523–524
Sporotrichosis, 523–524
Sputum, 396–397
in chest disease in children, 14–16
culturing, 772
cytologic examination of, asthma and, 696
induced, 116, 400
production, 14
samples, 402, 402f
Squawk, 11
SSEP. see Short-latency somatosensory evoked
potentials
Standard sweat test (Gibson and Cooke
technique), 770
Staphylococcus aureus, 427–428
pneumonia, illness presentation, 427–428
Starling’s equation, 592–593
“Static compliance” (Cst), 194
Statistical methods, usage, 231
Status asthmaticus, 719
mechanical ventilation in, 552–553
Stem cell transplantation
infectious risks in, 924
pulmonary function following, 937–938
Stenosis
cartilaginous congenital subglottic, 308
congenital tracheal, 310, 310f–311f
membranous subglottic, 308
pulmonary vein, 621–622, 622f–623f
subglottic, 298, 1122–1123
tracheal or bronchial, therapeutic
bronchoscopy for, 144
Stents, for therapeutic bronchoscopy, 145
Sternocleidomastoid muscles, visible
contraction of, 5
Sternum
fractures of, 1063
physical examination of, 1063
Index 1197
Steroid-resistant asthma, 312–313
Steroids
assessment of responsiveness to, severe
asthma and, 732, 732t
for influenza, 463–464
use of, 379
Streptococcus pneumoniae, 396, 427
antibiotic resistance, 432–433
in asthma, 659
in bacterial pneumonia, 969
empyema, 1022f
pneumococcus serotypes, implications, 427
Streptococcus pyogenes, 427
pharyngitis caused by, 396
Stress/inflammatory immune system,
stimulant, 220–221
Stretch receptors, 69
Stridor, 12, 300
Strongyloides stercoralis, 543
Strongyloidiasis, 543–545
clinical features of, 544
diagnosis of, 544–545
differential diagnosis of, 544–545
epidemiology of, 543
etiology of, 543
pathology/pathogenesis of, 543–544
treatment of, 537t, 545
Structural cell, 102
as sources of mediators, 108, 108f
Structural changes and repair, 102–103
Subacute hypersensitivity pneumonitis, 949
Subacute illness, 2
Subcarinal nodes, in pulmonary tuberculosis,
480–481
Subcutaneous emphysema, 1067
Subcutaneous prostacyclin analogue
(treprostinil), 576
Subglottic cysts, 303f
Subglottic hemangiomas, 307
Subglottic stenosis (SGS), 298, 1122–1123
acquired, 1122
in cardiac surgery, 1002t
congenital, 308–309, 1122
in Down syndrome, 995
mechanical ventilation and, 554
severity, levels of, 1122
Sublingual immunotherapy (SLIT), 747
usage, 742
Submersion accidents, 635
Submersion events, 634–638
Subpleural cysts, in Down syndrome, 997f
Substance P, 112, 120–121
Sucking wound, occlusive chest wall dressing
(application), 1066f
Suctioning, mechanical ventilation and, 553
Sudden infant death syndrome (SIDS),
1125–1142
alveolar hemosiderin-laden macrophages,
presence of, 1140
arousal, abnormality, 1140
bed sharing, 1141
cardiac arrhythmias, lethality, 1139
central nervous system, programmed cell
death, 1137–1138
circumstances of, 1139–1140
CO2, increase concentration, 1140–1141
diagnostic shifting, scene data, 1140
electrocardiogram QT intervals,
prolongation, 1139
epidemiologic findings, 1137
epidemiology, 1136–1137, 1136f–1137f
Back-to-Sleep campaign, 1140
success, 1141
fatty acid oxidation deficiencies,
1138–1139
metabolism, genetic diseases, 1138–1139
1198 Index

Sudden infant death syndrome (SIDS) Surfactant-deficient lungs Sweat chloride concentration, 770
(Continued) surfactant effects on, 59 Sweat gland effects, 767
neural receptors, 1138 surfactant treatment on, 60f Sweat test, 769–771, 770b
pathogenesis of, 1134–1135, 1135f Surfactant dysfunction mutations, type II for asthma, 698
pathology and physiology, 1137 alveolar epithelial cells and, 67 chloride analyte, 770
polymorphisms, occurrence, 1137 Surfactant homeostasis description, 769–770
prone position in, 1140 disruption of, 836–849 Gibson and Cooke, 770
racial disparities, 1141 genetic disorders, 841f Sylvatic Alaskan-Canadian variant, 543
serotonin binding, 1138 pulmonary, lung diseases associated with Sympathetic nerves, 69
sleep practices, 1137 disruption of, 836–849 Symptomatic vascular compression, surgical
soft bedding, effect modifier, 1140–1141 diagnosis and differential diagnosis of, management of, 1123
sudden death, scene investigations, 845–847 Synchronization, mechanical ventilation and,
1139–1140 epidemiology of, 836–838 554
ventilatory response, abnormalities, etiology of, 838–840 Synchronized intermittent mandatory
1140–1141 management, treatment, and prognosis ventilation (SIMV) mode, 388
Sudden unexpected early neonatal death of, 847–849 Syndromes, 396
(SUEND), 1137 pathology/pathogenesis of, 840–844 diagnostic approach by, 404
SUEND. see Sudden unexpected early neonatal Surfactant production Synonymous variant, 40, 41f
death disorders of Systemic corticosteroid, for asthma, 962
Sulfasalazine, 882 clinical features of, 844–845 Systemic disease, characteristics, 765–767
Superficial gel layer, 63–65 epidemiology of, 837–838 Systemic gas transport, 89–92
Superior left lower lobe etiology of, 838–839 Systemic hypertension, 374
bilateral circumscribed densities in, 540f imaging, pulmonary function testing, and Systemic inflammatory conditions, 874t
fluid-filled cysts in, CT confirmation of, laboratory testing in, 845 Systemic inflammatory disease, of childhood
541f pathology/pathogenesis of, 840–844 acute respiratory deterioration in, causes of,
Superior mediastinum, space-occupying presentation, symptoms, and physical 875t
structure in, 147 findings in, 844–845 pulmonary involvement in, 850–875
Superoxide dismutase (SOD), mitochondrial, genetic testing of, 846 approach to, 874b
decrease in, 590 hereditary disorders of, features of, 849t diagnosis/management of, 873–874
Supplemental oxygen overview of, 837f serologic test for, 851t
chronic dependency, causes, 382 Surfactant protein A (SP-A), 57, 125 significant, 872–873
for hydrocarbon aspiration, 627–628 encoding, 57 pulmonary manifestations of, 851t
long-term risk, increase, 878 misfolding and trapping of, 61 Systemic lupus erythematosus (SLE), 850,
Supraclavicular fossae as pattern recognition molecule, 59 854–857
as horizontal reference points, 9f polymorphisms of, 421 acute pneumonitis and, rarity, 855
indrawing of, 5 Surfactant protein B (SP-B), 57, 815 antiphospholipid antibodies in, presence of,
Supraglottic airways, resonances from, 10 deficiency 856
Supraglottitis, 413 as autosomal recessive disorder, 838 chest radiographs, of, 856f
Supraglottoplasty, 1121 ultrastructural findings associated, 842f classification criteria (American College of
Surface burns, smoke inhalation and, hereditary deficiency, 837 Rheumatology) for, 855b
632–633 as hydrophobic surfactant proteins, 60 clinical features of, 854
Surface tension, 583 as pattern recognition molecule, 59 epidemiology of, 854
reduction of, 836 Surfactant protein C (SP-C), 57 pathogenesis of, 854
Surfactant, 57–62 deficient mice, 61 prognosis of, 857
for acute respiratory distress syndrome, 613 gene, 57 pulmonary hemorrhage and, rarity, 855
alveolar life cycle of, 58, 59f as hydrophobic surfactant protein, 60 pulmonary hypertension and, rarity,
alveolar pool size of, 58 mutations of, lung incidence/prevalence 855–856
composition of, 57, 58f, 836–837 and, 838 pulmonary involvement in, 854–857
associated protein, 57, 58f Surfactant protein D (SP-D), 57 shrinking lung syndrome and, 856
function, 58–59 as 43-kD hydrophilic collectin, 57 treatment of, 857
genetic deficiencies in, 61 Surgery, preoperative management, 285 Systemic onset juvenile idiopathic arthritis,
host defense functions of, 59–60 Survival motor neuron (SMN), 1047 HRCT scan in, 853f
impairment of, with ARDS, 60–61 Susceptibility, individual, environmental Systemic scleroderma, 860
instillation of, 636 exposures in, 50–53 Systemic sclerosis (SSc), 860
metabolism, 57–58, 836–837 Swallowing classification of, 860–861
kinetics, 58 airway conditions, impact, 1109 clinical features of, 860
phosphatidylcholine, 58 anatomy of, 1106–1108 pathogenesis of, 861
production of, 344 behavioral issues, 1113 prognosis of, 862
secretion, 58 conditions, 1108–1109 pulmonary involvement in, 861
basal rate, 58 delayed initiation, 1098–1099 treatment of, 862
static effects of, 59 development of, 1098 Systemic steroids, neoalveolarization and, 254
surface tension decrease, 58–59 dysfunction, 1098–1100 Systemic-to-pulmonary collateral vessels,
synthesis of, 58 endoscopic evaluation, 1100–1101 development, 375
therapy, 346–347 esophageal phase, dysfunction, 1108t Systemic vascular pressures (reduction),
treatment, effect of, 60f evaluation of, 1100–1101 diuretics/drugs (usage) in, 593
Surfactant clearance function, instrumental assessment of,
disorders of 1109–1112
epidemiology of, 838 neural control, 1106 T
etiology of, 839–840 neurologic conditions, 1109 Tachypnea, 4
pathology/pathogenesis of, 844 oral phase, dysfunction, 1108t usage, 429
hereditary disorder of, features of, 849t pharyngeal phase, dysfunction, 1108t TACQOL-Asthma, 234–235, 236t–237t
Surfactant deficiency, 60–61 phases of, 1107–1108 Tacrolimus, 984
genetic testing of, 846 physiology of, 1106–1108 TagSNPs, 41
in preterm babies, 180 radiographic evaluation of, 1100 Talaromyces marneffei, 525–526
states, 57 treatment strategies, 1112–1113 TBB. see Transbronchial biopsy
surfactant treatment of, 61–62 voice evaluations and, 1119 TBNA. see Transbronchial needle aspiration
 Index 1199

T-box family of transcription factors, in gene Thrombophilia Tracer concentration, 209f

expression, 26
T cells
in immune response to, Mycobacterium
tuberculosis, 477
regulatory, 106–107
in lungs, 667–668
Technetium, 263
Technology dependence support, objectives of,
383, 383t
TEF. see Tracheoesophageal fistula
Temporal progression of symptoms, in
asthma, 648–650, 649f
Temporal variation, in prevalence of asthma,
642–644, 643f–644f
Tendon friction rub, 860–861
Tension pneumothorax, creation of, 1065
Teratoid mediastinal tumors, 1075–1079
Terbutaline
for asthma, 702–703
subcutaneous, for severe therapy-resistant
asthma, 734
Terminal sac, 70
Tetralogy of Fallot, 311–312
with absent pulmonary valve, 615–619,
618f
reperfusion pulmonary edema in,
622–624
Th0 naïve T cells, 667f
TH17 pathway, severe asthma and, 732
Thanatophoric dysplasia, 1005t–1006t
T-helper 2 (Th2) cells, 101, 667
chemoattractant receptor of (DP2-receptor),
110
development of, 105
T-helper 2 (Th2) lymphocyte, 667–668, 668f,
668t
T-helper type 1(Th1) phenotypes, 123–124
T-helper type 2 (Th2) phenotypes, 123–124
Theophylline
for asthma, 710
low-dose, for severe therapy-resistant
asthma, 733
Therapeutic bronchoscopy, 143–145
Thiabendazole, for strongyloidiasis, 545
Third-party payers, financial support, 386
Thoracentesis, empyema, 1019
Thoracic acoustics, 10–12
signs, assessment of regional ventilation by,
12
Thoracic aorta, traumatic rupture of,
1068–1069
Thoracic cage, distortion of, 384
Thoracic diagnostic workup, MRI (usage),
1087–1088
“Thoracic insufficiency syndrome”, 1049
Thoracic tumors, management and treatment
of, 1090–1091
Thoracic wall, injury, 1065f
Thoracoabdominal injuries, 1070–1071
Thoracoscopy, usage, 1090
Thoracotomy, 1020–1021, 1020f
usage, 1090
Thorax
local sound generation of, 12
sound transmission characteristics of,
12
Throat specimens, 400
Throat swab, 396–397
Thromboembolism, pulmonary, 887–892
clinical features of, 889
diagnosis of, 889–890
differential diagnosis of, 889–890
epidemiology of, 887
etiology of, 887–888, 888t
imaging for, 888, 890f
acquired, 888
inherited, 888
Thrombosis, in pulmonary arterial
hypertension, 568
Thumbs, divergence of, 6
Thymic cysts, 1075, 1076f
Thymic shadow, size/shape, variation of, 1075
Thymic stromal lymphopoietin (TSLP), 110,
110f
in pediatric allergic asthma, 668–669
Thymus
and anterior mediastinum, widening of, 147
chest radiograph for, 147, 148f
enlargement, 1075f
hyperplasia of, 1074–1075, 1075f
neoplasms of, 1075
teratoma of, 1075
Thyroid transcription factor 1 (TTF1), 836
Tidal volume (VT), 76
Tigecycline, for nontuberculous mycobacterial
infections, 504t
Tiotropium, 710
added to ICS and ICS and LABA, 710
TIPPV. see Tracheostomy intermittent positive
pressure ventilation
Tissue distribution, 759
Tissue-engineered tracheal transplantation,
310
Tissue resistance (Rti), 192–193
Tissue respiration, 97–98
Tissues, boundary of, 12
TIVA. see Total intravenous anesthesia
TLC. see Total lung capacity
T-lymphocytes, 101, 106–107, 107f
in pediatric allergic asthma, 667–668
quiescence, LKLF, 113–114
T lymphocytes, and lung defense, 130
gd T lymphocytes, 130
T lymphocytic variant HES (L-HES), 966b
Tobacco exposure, in pregnancy, 249
Tobacco smoke, in hospitalization, in asthma,
647
Toll-like receptors (TLRs), 123–124, 124f
interaction, 948
Tongue, oral cavity, 1106–1107
Tonsillectomy, usage, 1159
Torr unit, 74
Total atelectasis, chest radiograph revealing,
1088f
Total drug output, aerosol, 263
Total intravenous anesthesia (TIVA), 301
Total lung capacity (TLC), 76
Total obstruction, lesion persistence of, 1088f
Total respiratory resistance (Rrs), 192, 192f
Total respiratory system, compliance of,
195–196, 195f
Total surfactant pool size, 58
Toxic agents, inhalation of, 591
Toxic lung injury, induction of, 591
Toxocara canis, in helminth associated
eosinophilic lung disease, 962
Toxocariasis, 535–538
clinical features of, 536–537
diagnosis of, 537
differential diagnosis of, 537
epidemiology of, 535
etiology of, 535
management for, 537
pathology/pathogenesis of, 535–536
prognosis for, 537–538
treatment of, 537, 537t
Toxoplasma gondii, 930
tPAD (trans-Nasal Pulmonary Aerosol
Delivery), 271
T-piece jet nebulizer, 267–268, 268f
Trachea
congenital abnormalities of, 309–310
congenital tracheal stenosis, 309f–311f,
310
tracheomalacia, 309–310
deviation of, 8
kink, 147
lacerations of, 1066
position of, assessment of, 8
resonances from, 10
sounds, spectra of, 11f
Tracheal agenesis, 291
Tracheal airway disorders, operative
assessment, 1118–1119
Tracheal atresia, upper respiratory tract, 298,
299f, 299t
Tracheal bronchus, in Down syndrome, 994,
1002f
Tracheal colonization, in Bordetella pertussis,
530t
Tracheal cytotoxin, in Bordetella pertussis,
530t
Tracheal hemangiomas, 307–309, 308f
Tracheal stenosis
in Down syndrome, 995
therapeutic bronchoscopy for, 144
Tracheal tug, examination of, 8
Tracheobronchial stenosis, 867
Tracheobronchial trauma, 1066
chest radiographs, usage, 1066
Tracheobronchitis
Aspergillus, 516
in smoke inhalation, 630
Tracheoesophageal fistula (TEF), 291, 313,
313f, 322–324
anatomic abnormalities, 1100
associations of, 323
condition, 1114
etiology of, 322–323
pathology of, 322–323, 323f
postoperative care of, 324
preoperative care for, 323–324
presentation of, 323, 323t
surgery for, 324
Tracheomalacia, 293–294, 309–310, 309f,
1124
aortopexy, 310
congenital tracheal anomaly, 1124
in Down syndrome, 993–994
surgery, 310
technology dependence, 385
tracheostomy, 310
treatment of, 310
Tracheostomy, 310
for acute respiratory distress syndrome, 614
in chest wall injury, 1064
complication, 1097
operation, morbidity, 1064
placement, 391
presence of, 387
techniques of, 1064f
Tracheostomy intermittent positive pressure
ventilation (TIPPV), 285, 286f
Tracheostomy tube, presence of, 121
Traditional jet nebulizers, 257
Traffic-related air pollution, in asthma, 661
Transbronchial biopsy (TBB), 733
Transbronchial lung biopsy (TBLB), usage of,
866
Transbronchial needle aspiration (TBNA),
143
Transcription factors
in gene expression, 31–33
in inflammatory airways, 112f
inflammatory airways and, 112–113
1200 Index
Transcriptional mechanisms, in gene
expression, 31–34
Transepithelial potential difference
measurements, 772
Transfer RNA (tRNA), 47
Transient hypoxemia, presence of, 346–347
Transient infant wheezers, 679
Transient tachypnea, of newborn, 349, 349f
Transition
approach, 243–246
barriers, 246
definition, 241
evidence base, 242–243
importance, reasons, 241–243
from pediatric to adult care, 241–246
process, principles (development), 242
readiness, checklist (sample), 245b
Society for Adolescent Health and Medicine
definition, 241
transition, preparation, 243
young adult preparation, 243
Transplacental transmission, 49
Transporter associated with antigen
presentation (TAP) gene polymorphisms,
444
Transpulmonary pressure, 1008
increase in, achievement of, 592
Transthoracic lung aspiration, 400–401
Transthoracic needle aspiration biopsy,
941–942
Trastuzumab, 883
Trauma
in drowning, 636
physiologic compensation to, 1062–1063
Traumatic asphyxia (pulmonary compression
injury), 1067
Traumatic chylothorax, 1023
Traumatic pneumothorax, 1064–1065
Traumatic thoracic injury, 1062
Treatment-related lung damage, minimization
of, 593
Treprostinil (subcutaneous/intravenous
prostacyclin analogue), 576
Triamcinolone, intramuscular, for severe
therapy-resistant asthma, 733
Trichinella spiralis, in helminth associated
eosinophilic lung disease, 962
Trichosporon species, 524
Trichosporonosis, 524–525
Triclabendazole, for pulmonary
paragonimiasis, 546
Tricuspid regurgitant jet velocity (TRV), 907
Triggers, of asthma, 692
avoidance of, 715
Trimethoprim-sulfamethoxazole, for
nontuberculous mycobacterial infections,
504t
Trinucleotide (CTG), instability (expansion),
1047–1048
Triple endoscopy, 1104–1105
Triple risk model, of sudden infant death
syndrome, 1134–1135, 1135f
Tropical pulmonary eosinophilia, 962
True acidic GER, mucosal injury, 1114
True vocal folds (TVFs), 1106
adduction, 1107–1108
Trunk, flexion of, splinting with, 5–6
TRV. see Tricuspid regurgitant jet velocity
TSC. see Tuberous sclerosis complex
TSLP. see Thymic stromal lymphopoietin
Tuberculin skin test, for tuberculosis,
485–486, 486b, 488f, 489t
Tuberculins, 485
Tuberculosis (TB), 475–497
acute pneumonia and, 481
adult type, 481, 482f
Tuberculosis (TB) (Continued)
bacillus Calmette-Guérin vaccination for,
496
bronchial disease in, 479–480, 480f–481f
CAP consideration, 431
chest radiography for, 484–485, 484f–485f
in children, 478, 478t, 479f
clinical features of, 478–484
complicated lymph node disease in,
480–483, 481f
control and prevention of, 496
diagnosis of, 484–485
diagnostic imaging for, 484
disease, 493–495
adjunctive therapy for, 495
follow-up for, 495–496
treatment of, 493
drug-resistant, 494–495, 495t
epidemiology of, 475–477
exposure, treatment of, 491–492
extrapulmonary
clinical features of, 482–483, 483f
treatment of, 494
extrathoracic, 483–484, 483f
with human immunodeficiency virus
clinical features of, 484
epidemiology of, 476–477
in human immunodeficiency virus–infected
children and adolescents, 970–972
chest radiographs of, 971f
extrapulmonary, 968
IRIS, 978–979
multidrug-resistant, 972
pulmonary, 968, 971
incidence and prevalence of, 476
infection, treatment of, 492–493
interferon-gamma release assays for,
486–488, 488f, 489t
intrathoracic, 478–479
isolated lymphadenopathy in, 479, 479f
laboratory diagnosis of, 488–491, 489t
line probe assays for, 491
pathophysiology of, 477–478
pleural, 482, 482f
progressive primary infection in, 479, 479f
public health involvement for, 496
pulmonary, treatment of, 493–494, 493t
staining and microscopic examination of,
489–490
transmission of, 475–476
treatment of, 491–496
tuberculin skin test for, 485–486, 486b,
488f, 489t
Tuberous sclerosis, 290
Tuberous sclerosis complex (TSC), 819–820,
819f
Tubular bones, periostosis, 448
Tubular myelin, 58
Tucson Children’s Respiratory Study
in asthma, 648–649
in preschool wheeze, 679
Tumor necrosis factor (TNF), in asthma,
653t
Tumor necrosis factor-a (TNF-a), 123
blocking, 109–110
Tumors
benign, 1073–1074
of chest, 1072–1092
childhood, pulmonary complications of,
934–935
of diaphragm, 1087
malignant, 1074
Turbinates, edema of, from viral infections or
allergies, 120
Turbuhaler, 265f, 266–267
Turner syndrome, 839–840
“Turnover” (TO), in multiple breath washout,
206
TVFs. see True vocal folds
Twin studies, in asthma, 651
Type 2 innate lymphoid cells (ILC2), 668t
Type I alveolar epithelial cells, 67
Type II alveolar epithelial cells, 67, 67f
Type II cells
in surfactant, 58
ultrastructural abnormalities of, 58
Type III hypersensitivity response, 947–948
Type III secretion system (bsc), in Bordetella
pertussis, 530t
Type IV hypersensitivity response, 947–948
U
UIP. see Usual interstitial pneumonia
Ultrasonic nebulizers, 269
Ultrasonography, 170–172, 171b, 171f–172f,
333–334
of atelectasis, 1031, 1031f
Ultrastructural ciliary defects, 1038–1039,
1039t
Uncomplicated fractures, treatment of, 1064
Uncontrolled asthma, 218
Undefined hypereosinophilic syndrome,
966b
Unfolded protein response (UPR)
increased levels of, 843–844
triggering, 843–844
Unilateral cord palsy, 307
Unilateral diaphragmatic paralysis, 384
Unilateral diseases, 5–6
Unilateral intrathoracic abnormalities,
indication of, 8–9
Unilateral paralysis, management of, 1121
Unscreened populations, cystic fibrosis
diagnosis (clinical features), 770b
Untreated idiopathic pulmonary arterial
hypertension, progressive right heart
failure in, 564–565
Upper abdominal tenderness, clinical signs of,
1071
Upper aerodigestive tract, 1108
Upper airway, 1147–1148
congenital abnormalities of, 298–300
cough, 300
cyanosis, 300
neonatal intubation, 300
stridor, 300
voice/cry, 300
control, 1147–1148
in Down syndrome
anatomy of, 992–993
disease of, 992–995, 993t
obstruction of, 993t
dysfunction, 1148–1149
in muscles of respiration, 84
partial obstruction of, 16
reflexes, origination, 1144
systemic disease, 765–766
Upper airway disease
in human immunodeficiency virus infected
children, 980
on lower airway, influence of, 737–746
Upper airway endoscopy, requirements for,
302b
Upper airway infection, aspiration,
relationship, 443
Upper airway obstruction, 365–366,
406–419, 407f
acute, infectious and noninfectious causes
of, 409, 409b
diagnosis and differential diagnosis of,
409–412

Upper airway obstruction (Continued)
differentiation of principal infective causes
of, 408t
diphtheria, 415–416
epiglottitis, 412–414
load compensation, 1131
Upper mediastinal contour, chest radiograph,
148f
Upper respiratory tract atresias, 298, 299f
Upper respiratory tract disease, in Down
syndrome
clinical presentation of, 995
treating, considerations when, 1004
Upper respiratory tract infection (URTI),
posteroanterior chest radiograph of,
1084f
UPR. see Unfolded protein response
Urbanization, in prevalence of asthma,
644–645
Urinary antigen testing, 414
Urine, 401
bacterial antigens (detection), 430
Ursodeoxycholic acid (UDCA), in cystic fibrosis
liver disease, 793
URTI. see Upper respiratory tract infection
US National Health and Nutrition
Examination Survey (NHANES), in
asthma, 642–644
Usual interstitial pneumonia (UIP), 813
Usual interstitial pneumonia (UIP)-like
pattern, 950–951
Uvulopharyngopalatoplasty, 1159
V Ventilator-associated lung injury (VALI), risk
Vaccination, for influenza, 464, 464t
Vaccine-preventable infections, 396
Vagus nerve, and stimulation of afferent fibers,
120–121
VALI. see Ventilator-associated lung injury
Value, in coordinated aerodigestive care, 1096
VAP. see Ventilation associated pneumonia
Variceal bleeding, portal hypertension and,
793
Varicella zoster pneumonia, chest radiograph
of, 973f
Varicella-zoster virus (VZV), 926
Vas deferens, congenital bilateral absence,
767–768
Vascular abnormalities, 298
Vascular cell adhesion molecule (VCAM),
corticosteroids and, 705
Vascular cell adhesion molecule-1 (VCAM-1),
105
Vascular compliance, diuretics and, 589
Vascular compression, 1123
Vascular endothelial growth factor (VEGF),
18–19
Vascular endothelium, as source of locally
active mediators, 567
Vascular engorgement, in hydrocarbon
aspiration, 626
Vascular forces, 582
Vascular markings, preservation of, 148
Vascular permeability, 582
increased, in fluid-exchanging vessels, 584
Vascular rings, 312–313, 615–619, 616f–617f
clinical presentation of, 619
diagnosis of, 619
treatment of, 619, 619f–620f
Vascular trees, resin cast of, 64f
Vascular tumors, isolation, 1079
Vascularization, 35–36
Vasculitis, antineutrophil cytoplasmic
antibody associated, 895–897
treatment of, 897
Vasculitis syndromes, pulmonary involvement
and, 867–869
Vasoconstriction, pathways responsible for, in
pediatric pulmonary arterial
hypertension, 566–567, 566f
Vasoconstrictive agents, 567
Vasodilator testing, response to, 571
Vasoproliferative substances, release of, 567
VATS. see Video-assisted thoracoscopic surgery
VC. see Vital capacity
VCD. see Vocal cord dysfunction
VCD syndrome, 691–692
VEGF. see Vascular endothelial growth factor
Veno-occlusive disease, 937
Venous thromboemboli (VTE), following
solid-organ transplantation, 933–934
Ventilated patients, paralysis in, 613
Ventilation
alveolar, 86
for asthma, 720
distribution of, 79f, 81
mechanics and, of breathing, 73
modalities of, 610–612
support, CPAP (usage), 273
via tracheostomy, 387
Ventilation associated pneumonia (VAP), 554
Ventilation index, oxygenation (contrast), 609
Ventilation/perfusion (V/Q)
isotope study, 172f
mismatch, 17–18, 385
relationship, 89
scanning
for pulmonary embolism, 890–891
usage, 320–321
for, increase in, 609
Ventilator-dependent children, 382–383
Ventilator-induced lung injury (VILI), 372
Ventilators, dependency, HRQoL measure, 240
Ventilatory assistance, measure of, 609
Ventilatory drive, normalcy, 1147–1148
Ventilatory response, abnormalities,
1140–1141
Ventilatory support
ancillary equipment, 389–391
augmentation, 383
devices, usage, 282
options for, 387–389
quality of life, 393
survival, 392–393
weaning from, 392
Ventricular dysfunction, in human
immunodeficiency virus infected children,
979–980
Ventricular ectopic beats, 21
Venturi effect, 406
Venturi jet injector, 136
Very late antigen-4 (VLA-4), 105
Very long chain acyl-CoA dehydrogenase
(VLCAD), 1139
Video-assisted thoracoscopic surgery (VATS),
436
systematic review, 436
Videofluoroscopic swallow study, 1100, 1110,
1110f, 1110t
fiberoptic endoscopic evaluation of
swallowing versus, 1100t
VILI. see Ventilator-induced lung injury
Vinca alkaloids (vinblastine and vindesine),
880
Viral gene transfer agents, 806
Viral infection
in human immunodeficiency virus–infected
children and adolescents, 972–973,
973f
in pediatric lung transplantation, 986
Index 1201
Viral laryngotracheobronchitis, 406–409
clinical features of, 408–409
dosing regimen for, 410–411
epidemiology of, 406–407
etiology of, 407
formulation for, 410–411
management of, 409–411
mild, 408
moderate, 408
other treatments for sever cases of, 411
pathology of, 407–408
prevention, 411
prognosis and further evaluation of,
411–412
route of administration for, 410–411
sever, 408–409
Viral pathogens, 924–927
Viral pneumonia, respiratory syncytial
virus-positive nasopharyngeal aspirate,
presence, 431f
Viral shedding, bronchiolitis and, 421
Virtual bronchoscopy, 159–161, 160f–161f
Virulence-activated gene 8, in Bordetella
pertussis, 530t
Visceral larva migrans, 535
Visceral pleura, 1007
covering, 63
Visceral toxocariasis (VT), 535
Visual analysis, 143
Vital capacity (VC), 76
reduction, 283–284
Vitamin D deficiency, asthma and, 694
VLCAD. see Very long chain acyl-CoA
dehydrogenase
VM-26, 880
VMD. see Volume median diameter
Vocal cord, syndromes of dysfunction of,
asthma and, 726
Vocal cord dysfunction (VCD), measures,
234
recommendation, 234
Vocal cord paralysis, 121, 306–309,
1121–1122
hemangiomas, 307–309
laryngoceles, 307
saccular cysts, 307, 307f
Voice
evaluations, 1119
mitigating factors, 1119–1120
optimization, 1120
patient selection, 1120
Voice-generated vibrations, 8–9
Volume control ventilation, 549, 549f
Volume median diameter (VMD), 261
Volume of carbon dioxide (V̇ CO2), volume of
oxygen (V̇ O2) relationship with, 98
Volume of oxygen (V̇ O2), relationship with
volume of carbon dioxide (V̇ CO2), 98
Volume-rendering technique, 159, 160f
Volutrauma, BPD development, 372
von Recklinghausen disease, 1085
von Willebrand factor, 68
Voriconazole
for invasive aspergillosis, 517–518
for pulmonary mycoses, 507–508
VT. see Visceral toxocariasis
VZV. see Varicella-zoster virus
W
Waking, neural circuitry, 1143
Wasserman gears, 214f
Water lily sign, 539
Weaning, mechanical ventilation in, 554
Weather, changes in, asthma and, 692
Weibel-Palade bodies, 68
1202 Index
Weight gain, gastroesophageal reflux disease
and, 1114
WES. see Whole exome sequencing
Westley scale, 409
“Wet lung”, 1067
WGS. see Whole genome sequencing
Wheezes, 11, 13–14, 677
in asthma, 648–649
clinical patterns of, 679
differential diagnosis of, 17b
in Down syndrome, 993t, 998–999
findings in, 18f
parental reporting of, 677–678
pitch of, 17
Wheezing, 16–17, 686–721
associated with hyperreactive airway
disease, 17
causes of, in older children, 687b
in children, preventive strategy for, 681
defined, 686
infections, association, 429
inspiratory, 690–691
natural history and prognosis of, 689–690
Wheezing (Continued) Y
pathology of, 686–687, 687f–688f Young adults
pathophysiology of, 687–688 chronic illness, independence (inability),
patterns, in asthma, 648–649 241–242
physical examination of, 691–692, 692f chronic respiratory disorder, transition, 241
prevalence of, 649f confidentiality, establishment, 243
Whole exome sequencing (WES), 43, 45t rarer conditions, ongoing care
Whole genome sequencing (WGS), 43, (identification), 245
45t Youth Quality of Life Instrument, 232
Whole lung lavage (bronchopulmonary
lavage), usage of, 842
Williams-Campbell syndrome, 65–66 Z
WNT2/2b, in lung morphogenesis, 26–27 Zafirlukast, 709
Work, of breathing, 80–81 Zanamivir, for influenza, 463
Worrisome signs, 408–409 Zileuton, 709
Z-scores, 176
Zygomycota, 522
X
Xenobiotics, cytochrome P-450-dependent
detoxification of, 65
Xerostomia, 872
X-linked CGD, carriers of, 913
Xpert, 490