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Semin Liver Dis 2017; 37(01): 045-055
DOI: 10.1055/s-0036-1597817
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

New Advances in Polycystic Liver Diseases

A. Santos-Laso   *
1   Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
,
L. Izquierdo-Sánchez   *
1   Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
,
P. Y. Lee-Law
1   Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
2   National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, Instituto de Salud Carlos III), Spain
3   Department of Gastroenterology and Hepatology, Radboud University Medical Centre, Nijmegen, The Netherlands
,
M. J. Perugorria
1   Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
2   National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, Instituto de Salud Carlos III), Spain
4   IKERBASQUE, Basque Foundation for Science, Bilbao, Spain
,
M. Marzioni
5   Department of Gastroenterology, Università Politecnica delle Marche, Ancona, Italy
,
J. J. G. Marin
2   National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, Instituto de Salud Carlos III), Spain
6   Experimental Hepatology and Drug Targeting (HEVEFARM), Biomedical Research Institute of Salamanca (IBSAL), University of Salamanca, Salamanca, Spain
,
L. Bujanda
1   Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
2   National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, Instituto de Salud Carlos III), Spain
4   IKERBASQUE, Basque Foundation for Science, Bilbao, Spain
,
J. M. Banales
1   Department of Liver and Gastrointestinal Diseases, Biodonostia Health Research Institute – Donostia University Hospital, University of the Basque Country (UPV/EHU), San Sebastian, Spain
2   National Institute for the Study of Liver and Gastrointestinal Diseases (CIBERehd, Instituto de Salud Carlos III), Spain
4   IKERBASQUE, Basque Foundation for Science, Bilbao, Spain
› Author Affiliations
Further Information

Publication History

Publication Date:
15 February 2017 (online)

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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.

Keywords

polycystic liver diseases - hepatic cystogenesis - etiology - pathogenesis - therapy

* Both authors contributed equally to this work.


 

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