Indian childhood cirrhosis explained

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]

It remains a part of the differential diagnosis of Wilson's disease.^[7]

See also

• North American Indian childhood cirrhosis^[8]

Further reading

• Paton. A. Indian childhood cirrhosis. Br Med J (Clin Res Ed). October 17, 1981. 283. 6298. 1006. 1507261. 10.1136/bmj.283.6298.1006. 6794739.
• Raju. VB. Sundaravalli. N. Sriramachari. S. 30670129. Indian childhood cirrhosis: clinical features, prognosis and treatment.. Indian Journal of Pediatrics. Nov–Dec 1980. 47. 389. 537–41. 10.1007/BF02822546. 7262970.
• 1996. Bavdekar. AR. Bhave. SA. Pradhan. AM. Pandit. AN. Tanner. MS. Long term survival in Indian childhood cirrhosis treated with D-penicillamine. 74. 1. 32–35. Archives of Disease in Childhood. 8660042. 1511595. 10.1136/adc.74.1.32.

• Nayak N.C. . Chitale A.R. . 2013. Indian childhood cirrhosis (ICC) & ICC-like diseases: The changing scenario of facts versus notions . Indian J. Med. Res.. 137. 6. 1029–42. 3734708 . 23852284.

Notes and References

1. Sriramachari . S . Nayak . NC . Indian childhood cirrhosis: several dilemmas resolved. . The Indian Journal of Medical Research . August 2008 . 128 . 2 . 93–6 . 19001668.
2. Tanner. MS. Role of copper in Indian childhood cirrhosis.. The American Journal of Clinical Nutrition. May 1998. 67. 5 Suppl. 1074S–1081S. 9587155. 10.1093/ajcn/67.5.1074S. free.
3. Pandit. A. Bhave. S. Present interpretation of the role of copper in Indian childhood cirrhosis.. The American Journal of Clinical Nutrition. May 1996. 63. 5. 830S–5S. 8615370. 10.1093/ajcn/63.5.830. free.
4. Nayak . NC . Chitale . AR . Indian childhood cirrhosis (ICC) & ICC-like diseases: the changing scenario of facts versus notions. . The Indian Journal of Medical Research . June 2013 . 137 . 6 . 1029–42 . 23852284. 3734708 .
5. Nayak . NC . Visalakshi . S . Singh . M . Chawla . V . Chandra . RK . Ramalingaswami . V . Indian childhood cirrhosis--a re-evaluation of its pathomorphologic features and their significance in the light of clinical data and natural history of the disease. . The Indian Journal of Medical Research . February 1972 . 60 . 2 . 246–59 . 5064312.
6. Tomar . B. S. . Saxena . S. . Prakash . P. . Tomar . S. . Verma . C. . 1983 . D-penicillamine in the treatment of Indian childhood cirrhosis--a preliminary report . Indian Journal of Pediatrics . 50 . 407 . 613–618 . 10.1007/BF02957727 . 0019-5456 . 6680110. 26817281 .
7. Hermann . Wieland . Classification and differential diagnosis of Wilson's disease . Annals of Translational Medicine . April 2019 . 7 . S2 . S63 . 10.21037/atm.2019.02.07. 31179300 . 6531651 . free .
8. Richter. A. Mitchell. GA. Rasquin. A. [North American Indian childhood cirrhosis (NAIC)].. Médecine/Sciences . Nov 2007. 23. 11. 1002–7. 18021715. 10.1051/medsci/200723111002. free.