title: New Advances in Polycystic Liver Diseases.
abstract: Polycystic liver diseases (PLDs) include a heterogeneous group of congenital disorders inherited as dominant or recessive genetic traits; they are manifested alone or in association with polycystic kidney disease. Ductal plate malformation during embryogenesis and the loss of heterozygosity linked to second-hit mutations may promote the dilatation and/or development of a large number (> 20) of biliary cysts, which are the main cause of morbidity in these patients. Surgical procedures aimed to eliminate symptomatic cysts show short-term beneficial effects, but are not able to block the disease progression. Therefore, liver transplantation is the only curative option. Intense studies on the molecular mechanisms involved in the pathogenesis of PLDs have resulted in different clinical trials, some of them with promising outcomes. Here the authors summarize the key aspects of PLD etiology, pathogenesis, and therapy, highlighting the most recent advances and future research directions.
authors.affiliation: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Santos-Laso
authors.affiliation: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
authors.forename: L
authors.initials: L
authors.lastname: Izquierdo-Sánchez
authors.affiliation: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
authors.forename: P Y
authors.initials: PY
authors.lastname: Lee-Law
authors.affiliation: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
authors.forename: M J
authors.initials: MJ
authors.lastname: Perugorria
authors.affiliation: Department of Gastroenterology, Università Politecnica delle Marche, Ancona, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Marzioni
authors.affiliation: National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, Instituto de Salud Carlos III), Spain.
authors.forename: J J G
authors.initials: JJ
authors.lastname: Marin
authors.affiliation: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
authors.forename: L
authors.initials: L
authors.lastname: Bujanda
authors.affiliation: Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute - Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain.
authors.forename: J M
authors.initials: JM
authors.lastname: Banales
mesh_terms: D003560:Cysts / Q000235:genetics / Q000473:pathology / Q000628:therapy; D018450:Disease Progression; D006801:Humans; D008107:Liver Diseases / Q000235:genetics / Q000473:pathology / Q000628:therapy; D016031:Liver Transplantation; D009154:Mutation; D010641:Phenotype