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Original Article
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Korean J Med. 2008;75(1):81-87.
- 증례 : 담관결석과 동반된 윌슨씨병 1예
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정윤화.장정원.지정선.김병욱.최 황.최규용.맹이소, , , , , ,
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- A case of Wilson’s disease associated with cholelithiasis
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Yun Hwa Jung, Jeong Won Jang, Jeong Seon Ji, Byung Wook Kim, Whang Choi, Gyu Yong Choi, I So Maeng
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경희대학교 의과대학 순환기내과학교실
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- Abstract
- Wilson’s disease is a rare autosomal recessive metabolic disease. The ATB7B gene mutation results in a defect of biliary copper excretion and subsequent accumulation of copper in the liver, brain, and sclera.
The usual clinical signs of Wilson’s disease include hepatitis, liver cirrhosis, movement disorder, or a Kayser-Fleisher ring in the sclera, but patients occasionally present with hepatic failure or hemolytic anemia. Under such metabolic conditions, free copper induce chronic hemolysis with oxidative damage via free radical production, and chronic hemolysis, in turn, can cause secondary pigment bililary stone formation. Herein we report a case of Wilson’s disease associated with cholelithiasis in a young female. (Korean J Med 75:82-87, 2008)
Key Words : Wilson’s disease; Hemolysis; Cholelithiasis
Keywords :Wilson’s disease, Hemolysis, Cholelithiasis
