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Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases

World Journal of Gastroenterology 2013年第35期19卷 5775-5786页

作者： Bassam Abu-Wasel Caolan Walsh Valerie Keough Michele Molinari department of Surgery Dalhousie University department of radiolgoy Dalhousie University department of Surgery and Community Health and Epidemiology Dalhousie University

Polycystic liver diseases(PLD)represent a group of genetic disorders in which cysts occur in the liver(autosomal dominant polycystic liver disease)or in combination with cysts in the kidneys(autosomal dominant polycystic kidney disease).Regardless of the genetic mutations,the natural history of these disorders is *** natural history of PLD is characterized by a continuous increase in the volume and the number of *** genders are affected;however,women have a higher *** patients with PLD are asymptomatic and can be managed *** symptoms can affect 20%of patients who develop massive hepatomegaly with compression of the surrounding ***,patients with PLD suffer from acutecomplications caused by the torsion of hepatic cysts,intraluminal cystic hemorrhage and *** most common methods for the diagnosis of PLD are cross sectional imaging *** ultrasound and computerized tomography are the two most frequently used *** resonance imaging is more sensitive and specific,and it is a valuable test for patients with intravenous contrast allergies or renal *** treatment modalities are available to physicians caring for these *** treatment has been *** sclerotherapy,transarterial embolization,cyst fenestration,hepatic resection and liver transplantation are indicated to specific groups of patients and have to be tailored according to the extent of *** review outlines the current knowledge of the pathophysiology,clinical course,diagnosis and treatment strategies of PLD.

关键词： Polycystic liver disease Hepatic Epidemiolgy Classification Therapy Genetic

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