DIFFUSE PULMONARY FIBROSIS AND THE HERMANSKY-PUDLAK SYNDROME - CLINICAL COURSE AND POSTMORTEM FINDINGS

Authors
REYNOLDS SP DAVIES BH GIBBS AR
Citation
Sp. Reynolds et al., DIFFUSE PULMONARY FIBROSIS AND THE HERMANSKY-PUDLAK SYNDROME - CLINICAL COURSE AND POSTMORTEM FINDINGS, Thorax, 49(6), 1994, pp. 617-618
Citations number
10
Categorie Soggetti
Respiratory System
Journal title
Thorax → ACNP
ISSN journal
00406376
Volume
49
Issue
6
Year of publication
1994
Pages
617 - 618
Database
ISI
SICI code
0040-6376(1994)49:6<617:DPFATH>2.0.ZU;2-4
Abstract
The Hermansky-Pudlak syndrome consists of albinism, platelet function defect, pigment laden macrophases and, on occasions, pulmonary fibrosi s. The clinical course and postmortem findings of a patient with pulmo nary fibrosis which mimicked cryptogenic fibrosing alveolitis are repo rted. Histological examination revealed a chronic inflammatory infiltr ate of pigment laden microphages.