Clinical manifestations and survival pattern of Wilson's disease

Background. Wilson's disease usually presents in childhood. Early recogniti on and treatment is crucial to retard the progression of this disease, whic h can be debilitating, if not fatal. We analysed the clinical manifestation s 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 prospec tive follow up, The Kaplan-Meier survival curve and univariate Cox proporti onal 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 sy mptoms was 12 years (range 5-52 years). The log rank test showed a signific ant difference in the mean (SD) survival between patients who presented wit h hepatic [87.36 (15.26) months] and neurological symptoms [114.33 (9.14)mo nths]. Cox proportional hazards analysis showed a hazard ratio of 4.9 for p atients with a hepatic presentation compared to those with neurological pre sentation. Conclusion. The presentation of Wilson's disease is not limited to the paed iatric age group. Patients with a hepatic presentation had a five-fold high er risk of mortality when compared to those with a neurological presentatio n.