Clinical manifestations and survival pattern of Wilson's disease.
BACKGROUND
Wilson's disease usually presents in childhood. Early recognition and treatment is crucial to retard the progression of this disease, which can be debilitating, if not fatal. We analysed the clinical manifestations and survival pattern of patients admitted at our centre from 1993 to 1996.


METHODS
Hospital records of patients were reviewed to obtain the clinical manifestations. The survival status of patients was determined by a prospective follow up. The Kaplan-Meier survival curve and univariate Cox proportional hazards model were used to determine the survival pattern and risk for death in Wilson's disease.


RESULTS
A total of 60 patients were studied. The median age at onset of symptoms was 12 years (range 5-52 years). The log rank test showed a significant difference in the mean (SD) survival between patients who presented with hepatic [87.36 (15.26) months] and neurological symptoms [114.33 (9.14) months]. Cox proportional hazards analysis showed a hazard ratio of 4.9 for patients with a hepatic presentation compared to those with neurological presentation.


CONCLUSIONS
The presentation of Wilson's disease is not limited to the paediatric age group. Patients with a hepatic presentation had a five-fold higher risk of mortality when compared to those with a neurological presentation.
Richard
V S
VS
Christian Medical College, Vellore 632004, Tamil Nadu, India.
Harris
V K
VK
Shankar
V
V
Loganathan
G
G
Chandy
G M
GM
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D005260:Female; D006527:Hepatolenticular Degeneration / Q000188:drug therapy / Q000401:mortality* / Q000503:physiopathology*; D006801:Humans; D007194:India / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D015996:Survival Rate