뇌하수체 호르몬의 분비 이상을 보인 Wilson 씨 병

Wilson`s disease is a hereditary disorder in hepa- tic excretion of copper that results in toxic accumulations of the metal in liver, brain and other organs. Though there have been some reports of Wilson's disease with hepatic, neurologic, psychiatric symptoms, or hemolytic anemia, a case of Wilson's disease with abnormal secretion of pituitary hormones is, to our best knowledge, the first report in Korea. We report a case of Wilson`s disease with abnormal secretion of pituirary hormones, chronic active hepatitis with feature of early cirrhosis and hemolytic anemia in 18-year-old man showing delayed puberty. And also we describe a case of successful desensitization to penicillamine in a penicillamine hypersensitivity.