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Wilson氏 病

A Case of Wilson´s Disease

최신의학 1967년 10권 9호 p.78 ~ 82
허윤,
소속 상세정보
허윤 (  ) - 부산대학교 의과대학 내과학교실

Abstract


This is a case of Wilson´s disease found in a 16 year old Korean boy. In this report the author described the clinical course, the laboratory findings including serum ceruloplasmin activity, the morphological feature of the liver obtained by the Menghini needle, and the result of treatment.
The case had no family history. suggestive of hepatolenticular degeneration, but the patient sought medical libation because he Lad known that he had a gradually developed weakness, loss of body weight and brownish green discoloration of the skin.
The liver was not enlarged, but the spleen was palpated 3 finger breadth below the left costal margin. Dilatation of the superficial veins around the umbilicus was found. There was no sign of ascites or esophageal varices. The Kayser-Fleischer ring, a greenish-yellow discoloration at the limbus of the cornea, was demonstrated with the naked eye and with a slit lamp without difficulty. The patient presented signs of an extrapyramidal neurologic disturbance with tremor, forced smiling, and difficulty with swallowing and phonation. The liver function tests showed a marked hepatocellular damage. The serum copper was 147 micrograms per 100 ml. and the serum ceruloplasmin activity was 11 units with the control value of 396 units. The microscopic feature of the liver stained with hematoxyiin-eosin showed a characteristic finding of liver cirrhosis of portal type. The treatment employed in this case consisted of a symptomatic therapy and BAL administration. The result was unfavourable.

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