Article
Primary sclerosing cholangitis is associated with abnormalities in CFTR.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society - 1 Sept 2018
Werlin Steven, Scotet Virginie, Uguen Kevin, Audrezet Marie-Pierre, Cohen Michael, Yaakov Yasmin, Safadi Rifaat, Ilan Yaron, Konikoff Fred, Galun Eitan, Mizrahi Meir, Slae Mordechai, Sayag Shirley, Cohen-Cymberknoh Malena, Wilschanski Michael, Ferec Claude
Abstract excerpt
BACKGROUND: The etiology of primary sclerosing cholangitis (PSC) is unknown. PSC and Cystic Fibrosis related liver disease have common features: chronic inflammation, biliary damage and similar cholangiographic findings. It is unknown whether or not PSC is related to cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. We hypothesize that a sub-group of PSC patients may be a "single-organ"...
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