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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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Detalles Bibliográficos
Autores principales: Yokoyama, Tadafumi, Gochuico, Bernadette R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: European Respiratory Society 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9488956/
https://www.ncbi.nlm.nih.gov/pubmed/33536261
http://dx.doi.org/10.1183/16000617.0193-2020