Clinical manifestations and survival pattern of Wilson's disease.

Background.Wilson'sdiseaseusuallypresentsin childhood. Earlyrecognitionand treatment is crucialto 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 clinicalmanifestations.The survivalstatus of patients was determined by a prospectivefollowup. The Kaplan-Meier survivalcurve and univariate Cox proportional hazards model were used to determine the survivalpattern and riskfor death in Wilson's disease. Results.A total of 60 patientswere studied. The medianage at onset of symptomswas 12 years (range 5-52 years). The log ranktest showeda significantdifferenceinthe mean (SO)survival between patients who presented with hepatic [87.36 (15.26) months] and neurologicalsymptoms [114.33 (9.14) months]. Cox proportional hazardsanalysisshowed a hazard ratio of 4.9 for patients with a hepatic presentation compared to those with neurologicalpresentation. Conclusion. 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.