Pediatric interstitial lung disease revisited

Pediatr Pulmonol. 2004 Nov;38(5):369-78. doi: 10.1002/ppul.20114.


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.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Bronchoalveolar Lavage
  • Child
  • Diagnosis, Differential
  • Humans
  • Lung Diseases, Interstitial / diagnosis*
  • Lung Diseases, Interstitial / genetics
  • Lung Diseases, Interstitial / physiopathology*
  • Lung Diseases, Interstitial / therapy
  • Mutation
  • Prognosis
  • Pulmonary Surfactants / pharmacology*
  • Respiratory Function Tests
  • Tomography, X-Ray Computed


  • Pulmonary Surfactants