Korean J Pathol.  1987 Dec;21(4):278-284.

Wilson's Disease: Report of a Case with Comprehensive Review of the Previously Reported Cases in Korean Literature

• Lee MK
• Park CI

Abstract

We reported a case of Wilson's disease, which was histologically confirmed by liver biopsy in a 15 year-old boy and made a comprehensive analysis on the sum of 22 cases reported in Korean literature. Although Wilson's disease is still rare, it should be included in the differential diagnosis of chronic liver diseases particularly in children of 5 to 15 years old. For the clinical diagnosis of Wilson's disease, changes in serum ceruloplasmin, plasma copper and urine copper values are most important as well as Kayser-Fleischer ring and family hestory. The serum level of GOT, when elevated, were always higher than GPT. This atypical transaminase profile may be a clue for diagnosis of hepatic diseases by a metabolic derangement. Among various histologic changes of the liver in Wilson's disease, what have diagnostic importance are anisonucleosis, nuclear glycogenosis and Mallory body, all of which may appear in any stages of the disease. In 3 cases only neurologic symptoms such as dysarthria and athetosis were found. Hemolytic anemia was another rare complication.

Keyword

Wilson's disease; Hepatolenticular degeneration; Copper; Cirrhosis; Ceruloplasmin

MeSH Terms

Adolescent
Anemia, Hemolytic
Athetosis
Biopsy
Ceruloplasmin
Child
Copper
Diagnosis
Diagnosis, Differential
Dysarthria
Fibrosis
Glycogen Storage Disease
Hepatolenticular Degeneration*
Humans
Liver
Liver Diseases
Male
Neurologic Manifestations
Plasma
Ceruloplasmin
Copper