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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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Detalhes bibliográficos
Main Authors: O’Brien, Kevin, Troendle, James, Gochuico, Bernadette R., Markello, Thomas C., Salas, Jose, Cardona, Hilda, Yao, Jianhua, Bernardini, Isa, Hess, Richard, Gahl, William A.
Formato: Artigo
Idioma:Inglês
Publicado em: 2011
Assuntos:
Acesso em linha: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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