Wilson's disease: 2 cases in the pediatric population with varying degrees of hepatic involvement

Abstract

Wilson's disease is an autosomal recessive disorder. The inability to conjugate copper with apoceruloplasmin, due to mutation of the ATP7B protein, causes copper accumulation at the level of hepatocytes and basal ganglia. Secondary to these deposits, patients develop hepatic and neurological alterations. This pathology has a global distribution; however, no cases have been reported in the literature in the Republic of Panama. Two cases of Wilson's disease are presented in pediatric patients from Panama City, Panama, with different degrees of hepatic involvement.