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Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace’s Law?

Department of Preclinical Research, Angion Biomedica Corp., Uniondale, NY 11553, USA
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Med. Sci. 2019, 7(4), 55; https://doi.org/10.3390/medsci7040055
Received: 23 December 2018 / Revised: 25 March 2019 / Accepted: 27 March 2019 / Published: 1 April 2019
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Abstract

Caroli syndrome, characterized by saccular dilatation of intrahepatic ducts and congenital hepatic fibrosis, is without therapy in part due to its ultra-rare prevalence and the apparent lack of availability of a suitable experimental model. While the PCK rat has long been used as a model of fibropolycystic kidney disease, hepatobiliary biophysics in this animal model is incompletely characterized. Compared to age-matched, wild-type controls, the PCK rat demonstrated severe hepatomegaly and large saccular dilated intrahepatic ducts. Nevertheless, hepatic density was greater in the PCK rat, likely due to severe duct wall sclerosis accompanied by scarring across the hepatic parenchyma. Extracellular matrix accumulation appeared proportional to duct cross-sectional area and liver volume and appeared compensatory in nature. The PCK rat livers exhibited both cholangiocarcinoma and hepatocellular carcinoma coincident with areas of increased extracellular matrix deposition. Together, these data suggest that the PCK rat model mimics at least in part the spectrum of hepatobiliary pathology observed in Caroli syndrome and highlights the attendant risk associated with this disease. View Full-Text
Keywords: Caroli syndrome; congenital hepatic fibrosis; intrahepatic bile duct; scar; hepatocellular carcinoma; cholangiocarcinoma Caroli syndrome; congenital hepatic fibrosis; intrahepatic bile duct; scar; hepatocellular carcinoma; cholangiocarcinoma
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This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited (CC BY 4.0).
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Chavre, B.M.; Jiang, K.; St. Surin, L.G.; Bissoondial, T.; Zhou, P.; Li, J.; Gadhiya, S.V.; Goldberg, I.D.; Narayan, P. Remodeling of Intrahepatic Ducts in a Model of Caroli Syndrome: Is Scar Carcinoma a Consequence of Laplace’s Law? Med. Sci. 2019, 7, 55.

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