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Updated: Jul 27, 2026

Establishment and Validation of a Rat Model of Pulmonary Arterial Hypertension Associated with Pulmonary Fibrosis
Published on: May 23, 2025
Diffuse pulmonary fibrosis and the Hermansky-Pudlak syndrome: clinical course and postmortem findings
S P Reynolds1, B H Davies, A R Gibbs
1Asthma and Allergy Unit, Sully Hospital, Penarth, South Glamorgan.
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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