Clinical management and outcomes of patients with Hermansky-Pudlak syndrome pulmonary fibrosis evaluated for lung transplantation

Pulmonary fibrosis is a progressive, fatal manifestation of Hermansky-Pudlak syndrome (HPS). Some patients with advanced HPS pulmonary fibrosis undergo lung transplantation despite their disease-associated bleeding tendency; others die while awaiting donor organs. The objective of this study is to d...

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Detalhes bibliográficos
Publicado no:PLoS One
Main Authors: El-Chemaly, Souheil, O’Brien, Kevin J., Nathan, Steven D., Weinhouse, Gerald L., Goldberg, Hilary J., Connors, Jean M., Cui, Ye, Astor, Todd L., Camp, Philip C., Rosas, Ivan O., Lemma, Merte, Speransky, Vladislav, Merideth, Melissa A., Gahl, William A., Gochuico, Bernadette R.
Formato: Artigo
Idioma:Inglês
Publicado em: Public Library of Science 2018
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5856338/
https://ncbi.nlm.nih.gov/pubmed/29547626
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0194193
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