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Korean J Gastroenterol. 1999 Mar;33(3):437-442. Korean. Case Report.
Kim EJ , Kim C , Chung IK , Kim HS , Park SH , Lee MH , Kim SJ , Kim DJ , Nam KR .
Abstract

Wilson' s disease is an autosomal inherited disorder of copper metabolism; impaired copper excretion into the bile leads to copper accumulation in liver, brain and other organ. Hepatocellular carcinoma (HCC) has rarely been reported as a sequele of Wilson' s disease. It has been shown experimentally that copper has an inhibitory effect on the factors responsible for HCC development in rats, and the low prevalance of HCC in case with Wilson' s disease may result from hepatic copper deposition which has been postulated to have a protective effect against hepatic oncogenesis. The clinica features of these cases are characterized by relatively advanced age at diagnosis, the presence of cirrhosis and a male predominance. It is suggested that hepatic copper content in the patient with Wilson' s disease who was treated with penicillamine and subsequently developed HCC may be decreased toward a nonprotective range in response to penicillamine therapy. We report the one case of patient with Wilson' s disease combined with HCC.

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