Indian Childhood Cirrhosis

A fatal disease of early childhood onset with familial tendencies first described in the middle classes of rural India, which occurs elsewhere in the tropics and subtropics
Lab Increased immunoglobulins, increased serum and hepatic copper
Pathogenesis Idiopathic, cooking in copper cookware
References in periodicals archive ?
The misleading mutations include a variant thought to cause a liver disease, known as North American Indian childhood cirrhosis, when kids inherit two copies of the variant.
Indian Childhood Cirrhosis was last reported in the 90s
Indian Childhood Cirrhosis ( ICC), a chronic liver disease common to the Indian subcontinent, can prove fatal for children between one and three years of age.
Indian childhood cirrhosis (ICC) is a chronic liver disease in 1-3 yr old children, unique to the Indian subcontinent.
Incidentally, the current eponym 'Indian childhood cirrhosis', was actually introduced in 1957 by Jelliffe, et al (7) and it acquired long lease subsequently following Achar et al (8).
(1.) Indian childhood cirrhosis (ICC): A Multicentric National Collaborative Study, S.Sriramachari, Editor-in-Chief.
Although hyalin bodies were first described in alcoholic liver disease, they are also present in nonalcoholic steatohepatitis,[14] Indian childhood cirrhosis, and Wilson's disease, and in the livers of patients receiving amiodarone therapy.
The century-old Indian Childhood Cirrhosis (ICC) worldwide is known as a disease unique to India.
(1.) MNCS Report on Indian Childhood Cirrhosis, October 2006 Publ.
Early introduction of Copper-contaminated animal milk feeds as a possible cause of Indian Childhood Cirrhosis. Lancet 1983; 2 : 992-9.
However, several studies have demonstrated with serial liver biopsies that fibrosis may decrease with time in some cirrhotic livers.[1] This partial regression has been documented in diseases in which progression can be arrested by therapy, as in alcoholism, hemochromatosis, Wilson disease, Indian childhood cirrhosis, autoimmune hepatitis, and chronic hepatitis C virus.
Reversal of Indian childhood cirrhosis by D-penicillamine therapy.

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