All issues > Volume 31(11); 1988

Original Article

J Korean Pediatr Soc. 1988;31(11):1502-1508. Published online November 30, 1988.

A Case of Wilson Disease.

Cheol Soo Dan¹, Sang Hoon Lee¹, Woo Yeong Chung¹, Soon Yong Lee¹, Jong Eun Joo², Hye Jae Cho²

¹Department of Pediatrics, Inje Medical College, Ptisan Paik Hospital, Korea
²Department of Pathology, Seoul Paik Hospital

Received: March 16, 1988;  Accepted: June 2, 1988.

Abstract

We have experienced a case of Wilson disease manifesting chronic hepatitis. The patient, 10-year-old boy, has shown elevated SGOT and SGPT levels since 5 years ago, but no other specific symptoms of Wilson disease. His younger sister died of fulminant hepatitis (absence of hepatitis A or B markers) at the age of eight. The diagnosis was based on the characteristic laboratory data and the histologic findings of liver tissue by the light and electron microscopy. We have treated the patients with D-penicillamine and pyridoxine and the blood lerels of SGOT and SGPT have been decreased down to normal level and the urinary copper excretion in 24 hours has been decreased. We presented this case and reviewed related literatures briefly.

Keywords :Wilson disease, Chronic hepatitis