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Best Pract Res Clin Gastroenterol. 2010 Oct;24(5):573-84. doi: 10.1016/j.bpg.2010.08.007.

Congenital fibrocystic liver diseases.

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Department of Gastroenterology and Hepatology(455), Radboud University Nijmegen Medical Center, Geert Grooteplein-Zuid 10, 6525 GA Nijmegen, The Netherlands.


Fibrocystic diseases affecting the liver and often also other organs like the kidneys are a clinically and genetically heterogeneous group of disorders that may present in utero or remain clinically silent into late adulthood. During recent years, substantial progress has been made in unravelling the aetiology with primary cilia playing a central pathogenic role in many if not all of these diseases. The fibrocystogenic process shares some common features including proliferation and dilatation of epithelial bile ducts with concomitant abnormal apoptosis, fluid secretion and extracellular matrix deposition. In this review, we summarise clinical and diagnostic aspects, mechanisms of hepatic cystogenesis, and recent knowledge on potential therapies for these conditions.

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