DOI: 10.36347/sjmcr.2019.v07i07.007

Pages: 425-427

Wilson's disease was described for the first time by the neurologist S.A.K as an autosomal recessive genetic disorder characterized by a toxic accumulation of copper in the body, mainly in the liver, central nervous system and cornea. As early as 1956, patients benefit from an effective treatment, D-penicillamine, copper chelating agent limiting the consequences of the disease. A retrospective study of 10 cases of Wilson's disease followed on the department of pediatric over a period of 12 years having various clinical, biochemical and radiological features. Wilson’s disease is an inherited metabolic disorder. Early diagnosis and appropriate management help to prevent the systemic complications. It also points out the need to suspect Wilson’s disease in any young patient presented with the unexplained liver disease.