Pirfenidone for the treatment of Hermansky-Pudlak Syndrome pulmonary fibrosis

Hermansky-Pudlak syndrome (HPS) type is a rare disorder of oculocutaneous albinism, platelet dysfunction, and in some subtypes, fatal pulmonary fibrosis. There is no effective treatment for the pulmonary fibrosis except lung transplantation, but an initial trial using pirfenidone, an anti-fibrotic a...

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Autors principals: O’Brien, Kevin, Troendle, James, Gochuico, Bernadette R., Markello, Thomas C., Salas, Jose, Cardona, Hilda, Yao, Jianhua, Bernardini, Isa, Hess, Richard, Gahl, William A.
Format: Artigo
Idioma:Inglês
Publicat: 2011
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Article
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC3656407/
https://ncbi.nlm.nih.gov/pubmed/21420888
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2011.02.003
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