Wilson's Disease: The Presenting Symptoms

Abstract

Twenty proven and five probable cases of Wilson''s disease have been studied to ascertain the commonest presenting symptom of the disease. In 11 this was jaundice or hepatosplenomegaly, including four of the five probable cases of the disease all of whom died of hepatic failure before the onset of neurological symptoms. All except one of these patients presented between the ages of 6 and 14. Eleven patients presented with symptoms attributable to the nervous system. Their ages ranged between 12 and 32 years. One patient presented with metabolic bone disease and two (Cases 3 and 21) had clinical or biochemical evidence of hepatic disease, but have remained symptom free to the time of writing. The possible diagnosis of Wilson''s disease should be considered in all children with prolonged jaundice or obscure hepatospleno-megaly.