All issues > Volume 32(11); 1989

Original Article

J Korean Pediatr Soc. 1989;32(11):1496-1502. Published online November 30, 1989.

A clinical studies on Wilson's disease.

Won Kyu Lee¹, Ki Sup Chung¹, Chang Jun Coe¹

¹Depatment of Pediatrics, College of Medicine, YonSei University, Seoul, Korea

Received: August 22, 1989;  Accepted: August 22, 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.

Keywords :Wilson’s disease, ceruloplasmin, Kayser-Fleischer’s ring