Genetics of Indian childhood cirrhosis.

Indian Childhood Cirrhosis (ICC) is a unique syndrome with characteristic clinical, epidemiological and histopathological features which is a major cause of mortality in India in children 1 to 4 years of age. The aetiopathogenesis of this invariably fatal disease is still obscure. Various theories of its aetiopathogenesis include genetic, viral, metabolic, toxic, autoimmune or a combination of factors. The present article deals with a brief review of literature to elucidate the possible genetic mechanisms involved. In earlier reports autosomal recessive (AR) mode of inheritance was suggested. A familial susceptibility, geographic limitation to the Indian sub-continent and some unknown environmental factors strongly suggest the multifactorial inheritance as the most likely genetic mechanism involved.