Polycystic liver disease: a clinical review
Journal Title: Annals of Hepatology - Year 2012, Vol 11, Issue 6
Abstract
Polycystic liver disease rarely occurs in isolation as part of autosomal dominant polycystic liver disease, but more commonly, it exists as an extra-renal manifestation of autosomal dominant polycystic kidney disease. The pathogenesis of polycystic liver disease involves defects in the primary cilium of the cholangio-cyte, with genetic mutations that impair key proteins integral to the complex functioning of cilia. While most patients are asymptomatic and require no intervention aside from reassurance and genetic counseling, in a minority of patients, polycystic liver disease creates a myriad of symptoms from the compressive effects of enlarged cysts, and can even cause malnutrition and liver decompensation in the severest of cases. In patients with symptomatic disease, a variety of interventional radiology or surgical techniques can be considered, including aspiration with sclerotherapy of a dominant cyst, fenestration, segmental hepaticresection, and even liver transplantation. Although there are no curative medical options for polycystic liver disease, somatostatin analogs hold promise and have shown minimal efficacy in human studies. However, further research is needed to develop more efficacious medical treatments.
Authors and Affiliations
Natasha Chandok
Keywords
Related Articles
- EP ID EP78201
- DOI -
- Views 101
- Downloads 0
Fighting Mortality in the Waiting List: Liver Transplantation in North America, Europe, and Asia
Liver disease is a major cause of mortality worldwide. Liver transplantation (LT) is the most effective treatment for end stage liver disease. Available resources and social circumstances have led to different ways of im...
Portopulmonary hypertension: state of the art
Portopulmonary hypertension is an uncommon but treatable pulmonary vascular consequence of portal hypertension, which can lead to significant morbidity and mortality. Portopulmonary hypertension results from excessive pu...
Most overweight and obese Indian children have nonalcoholic fatty liver disease
Background and rationale. Nonalcoholic fatty liver disease (NAFLD) is the most common cause of pediatric liver disease in western countries. Its prevalence in Indian subcontinent is not well studied. Material and method...
Reply to the letter to the Editor
Kava hepatotoxicity - a clinical review
This review critically analyzes the clinical data of patients with suspected kava hepatotoxicity and suggests recommendations for minimizing risk. Kava is a plant (Piper methysticum) of the pepper family Piperaceae, and...