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...
Main Authors: | , |
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Format: | Article |
Language: | English |
Published: |
European Respiratory Society
2021-02-01
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Series: | European Respiratory Review |
Online Access: | http://err.ersjournals.com/content/30/159/200193.full |