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Clinical spectrum of chronic interstitial lung disease in children
L L Fan1, A L Mullen, S M Brugman
1Division of Pediatric Pulmonology, National Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado 80206.
Insights
Pediatric interstitial lung disease (ILD) presents a diverse range of rare conditions in children, often leading to restrictive lung disease and hypoxemia. Diagnosis frequently requires invasive procedures, and treatment outcomes for these complex ILD cases are inconsistent.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Interstitial Lung Disease
Background:
- Interstitial lung disease (ILD) in children is a complex group of rare disorders.
- It presents with varied clinical manifestations and significant morbidity.
Purpose of the Study:
- To delineate the clinical spectrum of pediatric interstitial lung disease.
- To analyze diagnostic approaches and treatment outcomes in children with ILD.
Main Methods:
- Retrospective review of 48 pediatric patients diagnosed with ILD over a 12-year period.
- Analysis of clinical findings, diagnostic procedures (including open lung biopsy), and treatment responses.
Main Results:
- Most patients presented with restrictive lung disease and hypoxemia.
- Growth failure and pulmonary hypertension were common comorbidities.
- Open lung biopsy yielded specific diagnoses in 70% of cases, but many remained uncertain.
- Treatment responses to corticosteroids, bronchodilators, and chloroquine were inconsistent.
- High rates of morbidity and mortality were observed, with 6 deaths in the study cohort.
Conclusions:
- Pediatric ILD is a heterogeneous group of rare disorders with significant clinical challenges.
- Accurate diagnosis often necessitates invasive methods, and therapeutic strategies require further investigation.
- The condition is associated with substantial mortality, particularly within the first year of evaluation.
Abstract:
To describe the clinical spectrum of interstitial lung disease in children, we reviewed our experience with 48 patients during a 12-year period. Most patients initially had typical findings of restrictive lung disease and hypoxemia. Growth failure or pulmonary hypertension or both were found in more than one third. Specific diagnosis, made in 35 patients (70%), most often required invasive studies, particularly open lung biopsy. Although the diagnostic yield from open lung biopsy was high, the diagnosis of many patients remained uncertain. Many different disorders were encountered. The response to corticosteroids, bronchodilators, and chloroquine was inconsistent. Six patients died, five within 1 year after the initial evaluation. The spectrum of pediatric interstitial lung disease includes a large, heterogeneous group of rare disorders associated with high morbidity and mortality rates.
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