MONDAY, Dec. 3 (HealthDay News) -- A new classification system for disorders presenting with diffuse lung disease in infants provides new information on disease frequency, clinical settings and outcome, researchers report in the Dec. 1 issue of the American Journal of Respiratory and Critical Care Medicine.
Gail H. Deutsch, M.D., from Cincinnati Children's Hospital Medical Center in Ohio, and colleagues developed consensus terminology and diagnostic criteria for disorders presenting with diffuse lung disease in infants and described the diseases, clinical features and outcomes for children who undergo lung biopsy. They used data from 187 cases from 11 centers in North America on children under 2 years old who underwent lung biopsy.
The researchers were able to categorize 88 percent of cases, of which 60 percent were disorders that were more common in infancy such as primary developmental and lung growth abnormalities. Lung growth disorders were under-recognized, while surfactant mutations had characteristic pathologic features. High mortality was associated with disorders such as pulmonary hypertension, while mortality was low for disorders such as pulmonary interstitial glycogenosis.
"This retrospective cohort study identifies a diverse spectrum of lung disorders, largely unique to young children," Deutsch and colleagues conclude. "Application of a classification scheme grouped clinically distinct patients with variable age of biopsy and mortality."
Abstract
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