Premium
Medical and surgical treatment options for polycystic liver disease 1
Author(s) -
Drenth Joost P.H.,
Chrispijn Melissa,
Nagorney David M.,
Kamath Patrick S.,
Torres Vicente E.
Publication year - 2010
Publication title -
hepatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 5.488
H-Index - 361
eISSN - 1527-3350
pISSN - 0270-9139
DOI - 10.1002/hep.24036
Subject(s) - medicine , general surgery , intensive care medicine
A hepatic cyst is a fluid-filled, epithelial lined cavity which varies in size from a few milliliters to several liters. Unlike single cysts, polycystic liver, which is arbitrarily defined when >20 cysts are present, is a rare condition and is part of the phenotype of two inherited disorders. In autosomal dominant polycystic kidney disease (ADPKD), patients have polycystic kidneys and may eventually develop polycystic liver disease (PLD). In autosomal dominant polycystic liver disease (PCLD), multiple hepatic cysts are the primary presentation, whereas polycystic kidneys are absent. Traditionally, treatment consists of physical removal or emptying of cysts by a range of invasive techniques. However, there has been considerable progress in the development of new medical modalities over the last few years. Therefore, it is timely to review recent advances focused on promising novel therapies for this disease.