The clinical experience of Chinese patients with Wilson's disease.
OBJECTIVE
Wilson's disease has protean presentations. Most patients manifested as either hepatic or neurologic symptoms and signs initially. No study has investigated the predictor of presentation and differences in laboratory tests and survival between patients presented with hepatic or neurological disease so far.


METHODS
Forty-one patients with Wilson's disease were diagnosed between 1981 and 2002 at our hospital. Their clinical features at presentation and laboratory findings were retrospectively collected. Survival was calculated using the Kaplan-Meier analysis.


RESULTS
Twelve patients initially presented with hepatic symptoms including chronic hepatitis, complications of cirrhosis, and fulminant hepatic failure. Twenty-nine patients presented with neurological symptoms including tremor, dysarthria, ataxia, and psychiatric diseases. No specific factor identified could predict the initial presentations. Compared to patients with neurological symptoms, patients with hepatic disease had significantly higher serum levels of aminotransferase, ceruloplasmin, copper, and 24-hour urine copper excretion. There was no significant difference in survival between these two groups. Among the patients who died, the most common causes of death were fulminant hepatic failure and complications of cirrhosis.


CONCLUSIONS
Our results suggested that the overall survival did not differ significantly according to the initial presentations. There was no predictor found that could foretell the clinical course of Wilson's disease.
Pan
Jen-Jung
JJ
Division of Gastroenterology, Department of Medicine, Taipei Veterans General Hospital and National Yang-Ming University School of Medicine, Taipei, Taiwan, Republic of China.
Chu
Chi-Jen
CJ
Chang
Full-Young
FY
Lee
Shou-Dong
SD
D000293:Adolescent; D000328:Adult; D000410:Alanine Transaminase / Q000097:blood; D001219:Aspartate Aminotransferases / Q000097:blood; D002570:Ceruloplasmin / Q000378:metabolism; D002648:Child; D002681:China; D003300:Copper / Q000097:blood / Q000652:urine; D005260:Female; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000378:metabolism* / Q000401:mortality; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D012189:Retrospective Studies; D015996:Survival Rate
D003300:Copper; D002570:Ceruloplasmin; D001219:Aspartate Aminotransferases; D000410:Alanine Transaminase