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Pathophysiology of Cystic Fibrosis Liver Disease: A Channelopathy Leading to Alterations in Innate Immunity and in Microbiota

Cystic fibrosis (CF) is a monogenic disease caused by mutation of Cftr. CF-associated liver disease (CFLD) is a common nonpulmonary cause of mortality in CF and accounts for approximately 2.5%–5% of overall CF mortality. The peak of the disease is in the pediatric population, but a second wave of li...

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Detalhes bibliográficos
Publicado no:Cell Mol Gastroenterol Hepatol
Main Authors: Fiorotto, Romina, Strazzabosco, Mario
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier 2019
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6664222/
https://ncbi.nlm.nih.gov/pubmed/31075352
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.jcmgh.2019.04.013
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