Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings.
AUTOR(ES)
Reynolds, S P
RESUMO
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.
ACESSO AO ARTIGO
http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=474969Documentos Relacionados
- Diffuse pulmonary fibrosis and Hermansky-Pudlak syndrome.
- Albinism with haemorrhagic diathesis: Hermansky-Pudlak syndrome.
- Alveolar Macrophage Dysregulation in Hermansky-Pudlak Syndrome Type 1
- Pigment, platelets, and Hermansky–Pudlak in human and mouse
- The mouse pale ear (ep) mutation is the homologue of human Hermansky–Pudlak syndrome