Indian_childhood_cirrhosis
Indian childhood cirrhosis
Disease entailing liver disease noted as once prevalent in Indigenous American communities
Indian childhood cirrhosis is a chronic liver disease of childhood characterised by cirrhosis of the liver[1] associated with the deposition of copper in the liver.[2][3][4] It primarily affects children of 1–3 years of age and has a genetic predisposition. It had a very high case fatality in the past,[5] but has eventually become preventable, treatable "with D-penicillamine in the treatment of 85 biopsy proven cases of Indian childhood cirrhosis", according to the Indian Journal of Pediatrics. "The drug significantly (P< 0.002) reduced the serum and hepatic copper content and simultaneously there was improvement in clinical and symptomatic aspects. This therapy was compared with the conventional corticosteroid therapy."[6][1]
It remains a part of the differential diagnosis of Wilson's disease.[7]