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. 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.

Unusual association of Kikuchi's disease and dengue virus infection evolving into systemic lupus erythematosus.

Kikuchi's disease is a histologically alarming self-limiting condition typically affecting the lymph nodes of young females. A 13-year-old girl was presented with fever, skin rash and cervical lymphadenopathy. On examination she was found febrile, mild pallor was present and she had lymphadenopathy. Liver was palpable. Cervical lymph node biopsy showed histiocytic necrotising lymphadenitis (Kikuchi's disease). Dengue virus serology for IgG blot showed evidence of seroconversion in serial samples. She was treated with antibiotics and fluconazole and cyclosporin A. During hospitalisation she developed retinal vasculitis. She was reviewed after one month and showed rashes of subacute cutaneous lupus erythematosus. This case can be described to be a triggering event by dengue viral infection causing abnormal immune response leading initially to Kikuchi's disease and later on to systemic lupus erythematosus.