Pediatric interstitial lung disease revisited.
The spectrum of pediatric interstitial lung disease (PILD) includes a diverse group of rare disorders characterized by diffuse infiltrates and disordered gas exchange. Children with these conditions typically present with tachypnea, crackles, and hypoxemia. Recent advances have been made in the identification of different types of PILD that are unique to infancy. More exciting has been the discovery of genetic abnormalities of surfactant function, now described in both children and adults. A systematic evaluation of the child presenting with diffuse infiltrates of unknown etiology is essential to the diagnosis. Most often, lung biopsy is required. Current treatment options remain less than satisfactory, and morbidity and mortality remain considerable.
Fan
Leland L
LL
Pediatric Pulmonary Section, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston 77030-2399, USA. llfan@texaschildrenshospital.org
Deterding
Robin R
RR
Langston
Claire
C
D001706:Biopsy; D018893:Bronchoalveolar Lavage; D002648:Child; D003937:Diagnosis, Differential; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology* / Q000628:therapy; D009154:Mutation; D011379:Prognosis; D011663:Pulmonary Surfactants / Q000494:pharmacology*; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed
D011663:Pulmonary Surfactants