<prompt> Congenital wilson;s disease is characterized byKF ring is present at bihMay present as acute hepatitisDecreased Urinary copper excretionDecreased hepatic copper concentration  <response>: Wilson disease is an inborn error of metabolism due to toxic accumulation of copper in liver,brain,cornea and other tissues.Manifestations are more likely to be hepatic in early childhood neurological in adolescents or adults.The spectrum of hepatic manifestations include all forms of acute or chronic liver disease,i.e.,;viral hepatitis; like illness,acute liver failure,chronic hepatitis,poal hypeension,cirrhosis or asymptomatic hepatomegaly.KF Kayser-Fleischerrings are most common in children with neurological96 than hepatic60 Wilson disease. Wilson disease is characterised by decreased serum ceruloplasmin increased urinary copper excretion with increased hepatic copper stores. Reference:Essential pediatrics-Ghai,8th edition,page no:320,321 