Pathophysiology of Cystic Fibrosis Liver Disease: a channelopathy leading to alterations in innate immunity and in microbiota.

08:00 EDT 7th May 2019 | BioPortfolio

Summary of "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. Cystic fibrosis associated liver disease (CFLD) is a common non-pulmonary cause of mortality in CF and accounts for about 2.5-5% of overall CF mortality. The peak of the disease is in the pediatric population but a second wave of liver disease in CF adults has been reported in the last decade in association with an increase in the life expectancy of these patients. New drugs are available to correct the basic defect in CF but their efficacy in CFLD is not known. CFTR, expressed in the apical membrane of cholangiocytes, is a major determinant for bile secretion and CFLD has been classically considered a channelopathy. However, the recent findings of CFTR as regulator of epithelial innate immunity and the possible influence of the intestinal disease with an altered microbiota on the liver complication have open new mechanistic insight on the pathogenesis of CFLD. This review will give an overview of the current understanding of the pathophysiology of the disease and will discuss potential target for intervention.


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This article was published in the following journal.

Name: Cellular and molecular gastroenterology and hepatology
ISSN: 2352-345X


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