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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
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Pediatric interstitial lung disease revisited

Leland L Fan1, Robin R Deterding, Claire Langston

  • 1Pediatric Pulmonary Section, Department of Pediatrics, Baylor College of Medicine, Texas Children's Hospital, Houston 77030-2399, USA. llfan@texaschildrenshospital.org

Pediatric Pulmonology
|September 18, 2004
PubMed

Insights

Pediatric interstitial lung disease (PILD) encompasses rare disorders affecting children, often requiring lung biopsy for diagnosis. Advances include identifying infant-specific PILD and genetic causes of surfactant dysfunction.

Area of Science:

  • Pulmonology
  • Genetics
  • Pediatrics

Background:

  • Pediatric interstitial lung disease (PILD) is a group of rare, heterogeneous lung disorders in children.
  • PILD presents with diffuse infiltrates, impaired gas exchange, tachypnea, crackles, and hypoxemia.

Purpose of the Study:

  • To review the spectrum of pediatric interstitial lung disease (PILD).
  • To highlight recent advances in PILD diagnosis and understanding, including genetic factors.

Main Methods:

  • Systematic evaluation of children with diffuse infiltrates of unknown etiology.
  • Review of recent literature on PILD, including genetic abnormalities of surfactant function.

Main Results:

  • PILD comprises diverse rare disorders, with specific types unique to infancy identified.
  • Genetic abnormalities affecting surfactant function are increasingly recognized in PILD.
  • Lung biopsy is frequently necessary for diagnosis.

Conclusions:

  • Diagnosis of PILD requires systematic evaluation, often including lung biopsy.
  • Despite advances, PILD treatment options are limited, with significant morbidity and mortality.
  • Further research into genetic causes and novel therapies for PILD is warranted.

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