作者: Tom J. G. Gevers , Joost P. H. Drenth
DOI: 10.1038/NRGASTRO.2012.254
关键词:
摘要: Polycystic liver disease (PLD) is arbitrarily defined as a liver that contains> 20 cysts. The condition is associated with two genetically distinct diseases: as a primary phenotype in isolated polycystic liver disease (PCLD) and as an extrarenal manifestation in autosomal dominant polycystic kidney disease (ADPKD). Processes involved in hepatic cystogenesis include ductal plate malformation with concomitant abnormal fluid secretion, altered cell–matrix interaction and cholangiocyte hyperproliferation. PLD is usually a benign disease, but …