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
Summary
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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