Disease entailing liver disease noted as once prevalent in Indigenous American communities
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][1]
It remains a part of the differential diagnosis of Wilson's disease.[7]
^ abSriramachari, S; Nayak, NC (August 2008). "Indian childhood cirrhosis: several dilemmas resolved". The Indian Journal of Medical Research. 128 (2): 93–6. PMID 19001668.
^Tanner, MS (May 1998). "Role of copper in Indian childhood cirrhosis". The American Journal of Clinical Nutrition. 67 (5 Suppl): 1074S–1081S. doi:10.1093/ajcn/67.5.1074S. PMID 9587155.
^Pandit, A; Bhave, S (May 1996). "Present interpretation of the role of copper in Indian childhood cirrhosis". The American Journal of Clinical Nutrition. 63 (5): 830S–5S. doi:10.1093/ajcn/63.5.830. PMID 8615370.
^Nayak, NC; Chitale, AR (June 2013). "Indian childhood cirrhosis (ICC) & ICC-like diseases: the changing scenario of facts versus notions". The Indian Journal of Medical Research. 137 (6): 1029–42. PMC 3734708. PMID 23852284.
^Nayak, NC; Visalakshi, S; Singh, M; Chawla, V; Chandra, RK; Ramalingaswami, V (February 1972). "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. 60 (2): 246–59. PMID 5064312.
^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. doi:10.1007/BF02957727. ISSN 0019-5456. PMID 6680110. S2CID 26817281.
^Hermann, Wieland (April 2019). "Classification and differential diagnosis of Wilson's disease". Annals of Translational Medicine. 7 (S2): S63. doi:10.21037/atm.2019.02.07. PMC 6531651. PMID 31179300.
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