study on clinical profile and therapeutic aspect of Wilson’s disease

Abstract

Introduction: Wilson’s Disease is an autosomal recessive disease. It is characterized by degenerative changes in the brain, liver and Kayser-Fleischer Rings in the Cornea. It is observed with the prevalence of approximately 1:3000 among all ethnic groups. Aims & Objectives: To study the clinical profile and therapeutic aspects of Wilson’s Disease. Material and Methods: It was a prospective observational study conducted in the department of Hepatology of SCB Medical College & Hospital, in Cuttack city of Odisha. Patients were followed up after 6 month and response to treatment was observed. Results: Most patients were in age group 11-15 years (43%), male, female ratio being 2:1. 64% patients had hepatic presentations, 11% patients had neurological presentations and 25% of patients had both hepatic & neurological presentations. The hepatic presentations were jaundice in 79% of cases followed by Ascites and pedal edema in 75% of cases each, 29% patients were having UGI bleeding. Among the 28 patients 68% presented with KF Rings, 16% patients had child A, 28% had Child B and 56% patients had Child C cirrhosis. Conclusion: The commonest presentation of Wilson’s disease was Chronic Liver Disease.