Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.
case_report · Level V
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- Record sourced from PubMed, PMID 8016804.
- Also identified by PMC identifier 474969.
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Abstract
The Hermansky-Pudlak syndrome consists of albinism, platelet function defect, pigment laden macrophases and, on occasions, pulmonary fibrosis. The clinical course and postmortem findings of a patient with pulmonary fibrosis which mimicked cryptogenic fibrosing alveolitis are reported. Histological examination revealed a chronic inflammatory infiltrate of pigment laden microphages.
Medical subject headings
- Albinism, Oculocutaneous
- Pulmonary Fibrosis