Loss of Fibrocystin Promotes Interleukin-8-Dependent Proliferation and CTGF Production of Biliary Epithelium

Congenital hepatic fibrosis (CHF) is a rare genetic liver disease (1/20,000 births) characterized by ductal plate malformation during bile duct development and progressive hepatic fibrosis, and is often associated with autosomal recessive polycystic kidney disease.[1] Liver transplantation is necessary for the treatment of progressive CHF patients with a severe phenotype. There are several pathological evidences to indicate that the mechanism of fibrosis in CHF is quite different from liver cirrhosis due to chronic hepatitis: hepatic fibrosis in CHF patients lacks necroinflammatory changes of hepatocytes and the activation of hepatic stellate cells.

Source: Journal of Hepatology - March 17, 2019 Category: Gastroenterology Authors: Tomoyuki Tsunoda, Sei Kakinuma, Masato Miyoshi, Akihide Kamiya, Shun Kaneko, Ayako Sato, Jun Tsuchiya, Sayuri Nitta, Fukiko Kawai-Kitahata, Miyako Murakawa, Yasuhiro Itsui, Mina Nakagawa, Seishin Azuma, Tsuyoshi Sogo, Haruki Komatsu, Ryutaro Mukouchi, Aya Source Type: research