Cirrhosis Indian childhood

Tanner, M., Kantarjian, A., Bhave, S. and Pandit, A. (1983). Early introduction of copper-contaminated animal feeds as a possible cause of Indian childhood cirrhosis. Lancet, 332, 992-995. 

The immunological disorders of Indian childhood cirrhosis and of tropical splenomegaly syndrome, where there are disturbances of the immunoglobulin synthesis as well as defects of cellular immune responses, may be the results of a combination of infections and perhaps certain types of unusual autoimmune phenomena—or even to vitamin deficiencies. 

This section is confined to the disturbance of the serum immunoglobulins in an unusual type of liver disease Indian childhood cirrhosis or infantile cirrhosis is one of the major causes of mortality in Indian children and the disease is uniformly fatal. Its etiology is unknown, but recent studies strongly suggest that there is a disturbed immunological process, probably involving both the humoral and cellular aspects of the immune response. 

C6. Chandra, R. K., Immunological picture in Indian childhood cirrhosis. Lancet i, 537-540 (1970). 

Rl. Ramakumar, L., and Gupata, S. M., Haemagglutination. Reaction in Indian childhood cirrhosis indicating viral aetiology. Arch. Dis. Childhood 46, 390-392 (1972). 

Non-Indian childhood cirrhosis This similar condition, found in other countries, is indistinguishable from Indian childhood cirrhosis. Therefore, it is also called copper-related liver disease. A genetic defect is probably involved. Any correlation with increased copper ingestion is unlikely. (398)... 

Bavdekar, A.R., Bhave, S.A., Pradhan, AJM., Pandit, A.N., Tanner, M.S. Long term survival in Indian childhood cirrhosis treated with D-penicillamine. Arch. Dis. Childh. 1996 74 32-35... 

M.S. Long term survival in Indian childhood cirrhosis treated with D-penicillamine. Arch. Dis. Childh. 1996 74 32-35... 

Tanner MS. Role of copper in Indian childhood cirrhosis. Am J Clin Nutr 1998 67 10748-8IS. 

Idiopathic (Indian) childhood cirrhosis is a disorder of progressive liver failure in early childhood, with marked accumulation of hepatic copper usually resulting in death from liver failure. Although the etiology of this disorder in unknown, in all such cases the serum ceruloplasmin is elevated, suggesting a defect in biliary copper excretion beyond the point of entry into the secretor pathway. Originally described in infants... 

Extremely high concentrations of hepatic copper are seen in Indian childhood cirrhosis, a progressive, fatal disease of infants of... 

With the exception of genetically impaired individuals with Menkes disease and Wilson s disease, individuals who suffer from various grossly inadequate diets, diarrhea and severe malnutrition, and the patients who suffer from primary biliary cirrhosis, and cholestatic syndromes of Indian childhood cirrhosis, the development of copper deficiency and toxicity is not a significant risk for man. 

Indian childhood cirrhosis is a familial and probably a genetically determined disease with a high accumulation of copper in liver. 

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