Article
Pulmonary function and high-resolution CT findings in patients with an inherited form of pulmonary fibrosis, Hermansky-Pudlak syndrome, due to mutations in HPS-1.
Chest - 1 Jan 2000
Brantly M, Avila N A, Shotelersuk V, Lucero C, Huizing M, Gahl W A
Abstract excerpt
OBJECTIVE: To describe and correlate pulmonary function and high-resolution CT (HRCT) scan scores in individuals with a high risk for development of pulmonary fibrosis, ie, Hermansky-Pudlak syndrome (HPS) patients with mutations in the HPS-1 gene. DESIGN: Cross-sectional analysis of consecutive, eligible patients. PATIENTS: Thirty-eight HPS inpatients at the National Institutes of Health Clinical Center with...
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