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• W2572462014 abstract "Achar ST, Raju VB, Sriramachari S. J Pediatr 1960;57:744-58Editor's Column: The Mystery of Indian Childhood CirrhosisJelliffe DB. J Pediatr 1960;57:801-2Indian childhood cirrhosis (ICC) or Sen syndrome may go as far back in time as the legendary Indian magic rope trick. Dr BC Sen's original description appeared in the Indian Medical Gazette in 1887.1Sen B.C. Enlargement of liver in children.Indian Medical Gazette. 1887; 22: 338-343Google Scholar Achar et al described 166 cases of cirrhosis with “insidious onset” without “antecedent cause” collected at the Madras Medical College from 1950 to 1958. They detailed the natural history of patients with ICC and found a 44% mortality rate. Serial liver biopsies showed progression to cirrhosis with parenchymal regeneration and a diffuse “basket-like” fibrosis. Asymptomatic siblings had hepatomegaly and biopsies with “hepatic damage.” These data suggested genetic, environmental, or infectious etiologies. Detailed nutritional histories excluded malnutrition as a cause. Canine and chick embryo experiments ruled out infectious etiologies, and normal serum oxidase activity in patients with ICC excluded Wilson disease.A decade later, Kapoor et al2Kapoor S.K. Singh M. Ghai O.P. Study of serum copper and copper oxidase in patients with Indian childhood cirrhosis.Indian J Med Res. 1971; 59: 115-121PubMed Google Scholar reported high levels of serum copper in patients with ICC. Early epidemiological data suggested that a large proportion of index families were using copper vessels to boil milk while breast-feeding was protective.3Chawla V. Chandra R.K. Verma I.C. Ghai O.P. An epidemiologic approach to Indian childhood cirrhosis.Indian Pediatr. 1973; 10: 73-79PubMed Google Scholar Tanner et al4Tanner M.S. Portmann B. Mowat A.P. Williams R. Pandit A.N. Mills C.F. et al.Increased hepatic copper concentration in Indian childhood cirrhosis.Lancet. 1979; 1: 1203-1205Abstract PubMed Scopus (103) Google Scholar later found excess copper binding proteins in the liver by orcein staining. Treatment of ICC has thus focused on copper chelation and dietary exclusion. There has also been a significant decline in the incidence of ICC over the last two decades.5Sriramachari S. Nayak N.C. Indian childhood cirrhosis: several dilemmas resolved.Indian J Med Res. 2008; 128: 93-96PubMed Google Scholar Interestingly, the copper toxicity hypothesis has been recently questioned by a multicenter study wherein use of copper vessels was not found to be different between ICC and control subjects.6Sriramachari S. Indian childhood Cirrhosis (ICC): A Multicentric National Collaborative Study. Indian Council of Medical Research, New Delhi2006Google Scholar Thus, just like the Indian magic rope trick, the mystery behind ICC still remains unsolved. Achar ST, Raju VB, Sriramachari S. J Pediatr 1960;57:744-58 Editor's Column: The Mystery of Indian Childhood Cirrhosis Jelliffe DB. J Pediatr 1960;57:801-2 Indian childhood cirrhosis (ICC) or Sen syndrome may go as far back in time as the legendary Indian magic rope trick. Dr BC Sen's original description appeared in the Indian Medical Gazette in 1887.1Sen B.C. Enlargement of liver in children.Indian Medical Gazette. 1887; 22: 338-343Google Scholar Achar et al described 166 cases of cirrhosis with “insidious onset” without “antecedent cause” collected at the Madras Medical College from 1950 to 1958. They detailed the natural history of patients with ICC and found a 44% mortality rate. Serial liver biopsies showed progression to cirrhosis with parenchymal regeneration and a diffuse “basket-like” fibrosis. Asymptomatic siblings had hepatomegaly and biopsies with “hepatic damage.” These data suggested genetic, environmental, or infectious etiologies. Detailed nutritional histories excluded malnutrition as a cause. Canine and chick embryo experiments ruled out infectious etiologies, and normal serum oxidase activity in patients with ICC excluded Wilson disease. A decade later, Kapoor et al2Kapoor S.K. Singh M. Ghai O.P. Study of serum copper and copper oxidase in patients with Indian childhood cirrhosis.Indian J Med Res. 1971; 59: 115-121PubMed Google Scholar reported high levels of serum copper in patients with ICC. Early epidemiological data suggested that a large proportion of index families were using copper vessels to boil milk while breast-feeding was protective.3Chawla V. Chandra R.K. Verma I.C. Ghai O.P. An epidemiologic approach to Indian childhood cirrhosis.Indian Pediatr. 1973; 10: 73-79PubMed Google Scholar Tanner et al4Tanner M.S. Portmann B. Mowat A.P. Williams R. Pandit A.N. Mills C.F. et al.Increased hepatic copper concentration in Indian childhood cirrhosis.Lancet. 1979; 1: 1203-1205Abstract PubMed Scopus (103) Google Scholar later found excess copper binding proteins in the liver by orcein staining. Treatment of ICC has thus focused on copper chelation and dietary exclusion. There has also been a significant decline in the incidence of ICC over the last two decades.5Sriramachari S. Nayak N.C. Indian childhood cirrhosis: several dilemmas resolved.Indian J Med Res. 2008; 128: 93-96PubMed Google Scholar Interestingly, the copper toxicity hypothesis has been recently questioned by a multicenter study wherein use of copper vessels was not found to be different between ICC and control subjects.6Sriramachari S. Indian childhood Cirrhosis (ICC): A Multicentric National Collaborative Study. Indian Council of Medical Research, New Delhi2006Google Scholar Thus, just like the Indian magic rope trick, the mystery behind ICC still remains unsolved." @default.
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• W2572462014 title "50 Years Ago in The Journal of Pediatrics" @default.
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