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Diagnosis and management of polycystic liver disease.
MedLine Citation:
PMID:  23296249     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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 can cause debilitating abdominal symptoms in some patients. The main risk factors for growth of liver cysts are female sex, exogenous oestrogen use and multiple pregnancies. Ultrasonography is very useful for achieving a correct diagnosis of a polycystic liver and to differentiate between ADPKD and PCLD. Current radiological and surgical therapies for symptomatic patients include aspiration-sclerotherapy, fenestration, segmental hepatic resection and liver transplantation. Medical therapies that interact with regulatory mechanisms controlling expansion and growth of liver cysts are under investigation. Somatostatin analogues are promising; several clinical trials have shown that these drugs can reduce the volume of polycystic livers. The purpose of this Review is to provide an update on the diagnosis and management of PLD with a focus on literature published in the past 4 years.
Authors:
Tom J G Gevers; Joost P H Drenth
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2013-1-08
Journal Detail:
Title:  Nature reviews. Gastroenterology & hepatology     Volume:  -     ISSN:  1759-5053     ISO Abbreviation:  Nat Rev Gastroenterol Hepatol     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-1-8     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101500079     Medline TA:  Nat Rev Gastroenterol Hepatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Department of Gastroenterology and Hepatology, Radboud University Nijmegen Medical Centre, Nijmegen, P. O. Box 9101, Code 455, 6500 HB Nijmegen, The Netherlands.
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