Hermansky-Pudlak Syndrome: An unusual pattern of pulmonary fibrosis

Hermansky-Pudlak Syndrome: An unusual pattern of pulmonary fibrosis

Hermansky-Pudlak Syndrome is a rare genetic cause of pulmonary fibrosis, associated with albinism, nystagmus, and a bleeding diathesis. Histologically, Hermansky-Pudlak Syndrome Pulmonary Fibrosis (HPS-PF) typically resembles usual interstitial pneumonia (UIP), however radiologically this is not alw...

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Bibliographic Details
Main Authors: Matthew Donnan, Samantha Ellis, Ian Glaspole
Format: Article
Language:English
Published: Elsevier 2024-01-01
Series:Respiratory Medicine Case Reports
Subjects:
Hermansky-Pudlak Syndrome
Pulmonary fibrosis
Genetics
Computed tomography
Online Access:http://www.sciencedirect.com/science/article/pii/S2213007124001461

Internet

http://www.sciencedirect.com/science/article/pii/S2213007124001461

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