Hermansky–Pudlak syndrome pulmonary fibrosis: a rare inherited interstitial lung disease

Pulmonary fibrosis is a progressive interstitial lung disease of unknown aetiology with a poor prognosis. Studying genetic diseases associated with pulmonary fibrosis provides insights into the pathogenesis of the disease. Hermansky–Pudlak syndrome (HPS), a rare autosomal recessive disorder characte...

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Bibliographic Details
Main Authors: Tadafumi Yokoyama, Bernadette R. Gochuico
Format: Article
Language:English
Published: European Respiratory Society 2021-02-01
Series:European Respiratory Review
Online Access:http://err.ersjournals.com/content/30/159/200193.full