Polycystic liver diseases

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Polycystic liver diseases.

Polycystic liver diseases (PCLDs) are genetic disorders with heterogeneous etiologies and a range of phenotypic presentations. PCLD exhibits both autosomal or recessive dominant pattern of inheritance and is characterized by the progressive development of multiple cysts, isolated or associated with polycystic kidney disease, that appear more extensive in women. Cholangiocytes have primary cilia...

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Proliferation-Independent Initiation of Biliary Cysts in Polycystic Liver Diseases

Biliary cysts in adult patients affected by polycystic liver disease are lined by cholangiocytes that proliferate, suggesting that initiation of cyst formation depends on proliferation. Here, we challenge this view by analyzing cyst-lining cell proliferation and differentiation in Cpk mouse embryos and in livers from human fetuses affected by Autosomal Recessive Polycystic Kidney Disease (ARPKD...

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Isolated polycystic liver disease.

Isolated polycystic liver disease (PCLD) is an autosomal dominant disease with genetic and clinical heterogeneity. Apart from liver cysts, it exhibits few extrahepatic manifestations, and the majority of patients with this condition are asymptomatic or subclinical. However, a small fraction of these patients develop acute liver cyst-related complications and/or massive cystic liver enlargement,...

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Polycystic liver disease.

A 54-year-old man had an asymptomatic elevation of cytolysis-related enzymes, GGT, and alkaline phosphatase, detected at a routine laboratory study. The patient reported no alcohol abuse, no history of liver disease, and no constitutional syndrome. Physical examination showed a good general status, normal colored skin and mucosas, and no stigmata of chronic liver disease. The abdomen was soft, ...

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Polycystic liver disease.

A 57-year-old woman with a past history of serous ovarian cystadenoma was admitted to hospital complaining of progressive abdominal bloating for the last six months. Physical examination revealed an increase in abdominal perimeter. The rest of the physical examination was normal. Alkaline phosphatase and gamma-glutamyl-transpeptidase were marginally raised, while the rest of the liver function ...

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ژورنال

عنوان ژورنال: Digestive and Liver Disease

سال: 2010

ISSN: 1590-8658

DOI: 10.1016/j.dld.2010.01.006