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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...
| Autores principales: | , |
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
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European Respiratory Society
2021
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| 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 |