Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings. |
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| Authors: | S P Reynolds B H Davies A R Gibbs |
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| Affiliation: | Asthma and Allergy Unit, Sully Hospital, Penarth, South Glamorgan. |
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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. |
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