PMID- 21790682
OWN - NLM
STAT- MEDLINE
DCOM- 20111223
LR  - 20181201
IS  - 1365-2036 (Electronic)
IS  - 0269-2813 (Linking)
VI  - 34
IP  - 7
DP  - 2011 Oct
TI  - Systematic review: the pathophysiology and management of polycystic liver 
      disease.
PG  - 702-13
LID - 10.1111/j.1365-2036.2011.04783.x [doi]
AB  - BACKGROUND: Polycystic liver diseases (PCLD) represent a group of genetic 
      disorders in which cysts occur solely in the liver, or together with renal cysts. 
      Most of the patients with PCLD are asymptomatic, however, in some patients, 
      expansion of liver cysts causes invalidating abdominal symptoms. AIM: To provide 
      a systemic review on the pathophysiology and management of PCLD. METHODS: A 
      PubMed search was undertaken to identify relevant literature using search terms 
      including polycystic liver disease, pathophysiology, surgical and medical 
      management. RESULTS: The most common complication in patients with PCLD is 
      extensive hepatomegaly, which may lead to malnutrition and can be lethal. 
      Conservative surgical approaches are only partially effective and do not change 
      the natural course of the disease. Liver transplantation has been successfully 
      performed in PCLD, however, in an era of organ shortage, medical management needs 
      to be evaluated. A better understanding of the pathophysiology and the 
      availability of animal models have already identified promising drugs. 
      Abnormalities in cholangiocyte proliferation/apoptosis and enhanced fluid 
      secretion are key factors in the pathophysiology. It has been demonstrated in 
      rodents and in humans that somatostatin analogues diminish liver volume. The role 
      of the inhibitors of the mammalian target of rapamycin (mTOR) in the management 
      of PCLD is still under investigation. CONCLUSIONS: The exact pathophysiology of 
      polycystic liver disease still remains unclear. In symptomatic patients, none of 
      the currently available surgical options except liver transplantation have been 
      shown to change the natural course of the disease. The use of somatostatin 
      analogues has been shown to diminish liver volume.
CI  - (c) 2011 Blackwell Publishing Ltd.
FAU - Temmerman, F
AU  - Temmerman F
AD  - Department of Hepatology, UZ Gasthuisberg, K.U. Leuven, Leuven, Belgium.
FAU - Missiaen, L
AU  - Missiaen L
FAU - Bammens, B
AU  - Bammens B
FAU - Laleman, W
AU  - Laleman W
FAU - Cassiman, D
AU  - Cassiman D
FAU - Verslype, C
AU  - Verslype C
FAU - van Pelt, J
AU  - van Pelt J
FAU - Nevens, F
AU  - Nevens F
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Review
PT  - Systematic Review
DEP - 20110726
PL  - England
TA  - Aliment Pharmacol Ther
JT  - Alimentary pharmacology & therapeutics
JID - 8707234
RN  - Polycystic liver disease
SB  - IM
MH  - Animals
MH  - Cysts/genetics/*physiopathology/*therapy
MH  - Gene Expression Regulation
MH  - Humans
MH  - Liver Diseases/genetics/*physiopathology/*therapy
MH  - Liver Transplantation
MH  - Randomized Controlled Trials as Topic
EDAT- 2011/07/28 06:00
MHDA- 2011/12/24 06:00
CRDT- 2011/07/28 06:00
PHST- 2011/07/28 06:00 [entrez]
PHST- 2011/07/28 06:00 [pubmed]
PHST- 2011/12/24 06:00 [medline]
AID - 10.1111/j.1365-2036.2011.04783.x [doi]
PST - ppublish
SO  - Aliment Pharmacol Ther. 2011 Oct;34(7):702-13. doi: 
      10.1111/j.1365-2036.2011.04783.x. Epub 2011 Jul 26.