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A clinical studies on Wilson's disease.

Journal of the Korean Pediatric Society 1989;32(11):1496-1502.
Published online November 30, 1989.
A clinical studies on Wilson's disease.
Won Kyu Lee, Ki Sup Chung, Chang Jun Coe
Depatment of Pediatrics, College of Medicine, YonSei University, Seoul, Korea
Wilson병에 관한 임상적 고찰
이완규, 정기섭, 고창준
연세대학교 의과대학 소아과학교실
Received: 22 August 1989   • Accepted: 22 August 1989
Abstract
The 12 cases of Wilson disease younger than the age of 18 years were observed at Severance hospital between Jan. 1980 and July 1988 and we obtained following results. 1) The mean age was 14 years old and male to female ratio was 2:1 2) The younger the patient, the clinical symptoms predominantly manifested hepatic dysfunction and the older patient manifested neurologic symptoms. 3) Kayser-Fleischer’s ring was the most commonly noticed clinical finding. Neurologic symptomes were dystonia, dysarthria in order of frequency and hepatic symptoms were hepatomegly and splenomegaly, etc. 4) Mild elevation of SGOT were observed in 4 cases and serum ceruloplasmin levels were below 14 µ1/dL in 10 cases out of 12 and remaining 2 cases were diagnosed by serum copper and 24hr urine copper excretion. Marked elevation of 24hr urine copper excretion were observed in 10 cases out of 12.
Key Words: Wilson’s disease, ceruloplasmin, Kayser-Fleischer’s ring


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