Wilson's disease: 35 years' experience.
Thirty-seven Chinese patients fulfilling the criteria for Wilson's disease seen during a 35-year period were reviewed. Males and females were equally affected. Twenty-two patients were symptomatic and 15 asymptomatic; most of them presented before the third decade. Thirty-one per cent of the relatives screened showed evidence of disease, and parents were rarely affected (13 per cent). Half of the adult symptomatic females presented with primary amenorrhoea. Liver laboratory tests were abnormal in only 50 per cent of patients, with gamma-glutamyltranspeptidase being the most sensitive index. Renal disease was infrequent. Serum caeruloplasmin level was the single biochemical parameter of prognostic significance (p = 0.0001). Seventy per cent of the symptomatic patients showed an improvement after treatment with penicillamine.
Lau
J Y
JY
Department of Medicine, University of Hong Kong, Queen Mary Hospital.
Lai
C L
CL
Wu
P C
PC
Pan
H Y
HY
Lin
H J
HJ
Todd
D
D
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000469:Alkaline Phosphatase / Q000097:blood; D002570:Ceruloplasmin / Q000032:analysis; D002648:Child; D002681:China; D005260:Female; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000208:ethnology* / Q000235:genetics / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male
D002570:Ceruloplasmin; D000469:Alkaline Phosphatase