Immunochemical studies in Wilson's disease

The present study deals with a total of 28 cases of Wilson's disease, 50 normal individuals alongwith siblings and parents of eight cases. Male predominance (18 out of 28 cases), a median age of 11 years and universal presence of Kayser-Fleischer (K. F.) ring marked the cardinal features. Furthermore, 11 patients had hepatic-neural presentation while two had only the K. F. Ring without clinical abnormality. Single Radial Immunodiffusion (SRID) as the absolute quantitative procedure revealed a profound deficiency of ceruloplasmin with the levels ranging anywhere between 0.5 mg/dI to 23 mg/dI amongst the patients of Wilson's disease. The data from siblings and parents also revealed deficiency in 15 out of 23 serum samples when subjected to disc electrophoresis-benzidine screening procedure.