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Indian Childhood Cirrhosis and Tyrolean Childhood Cirrhosis

Disorders of a Copper Transport Gene?

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Copper Transport and Its Disorders

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 448))

Abstract

Indian Childhood Cirrhosis (ICC) (Tanner 1997) and Tyrolean Childhood Cirrhosis (TCC) (Müller et al 1996) have both now virtually disappeared. In ICC, and putatively in TCC, greatly increased levels of ingested dietary copper in infancy were associated with greatly increased hepatic copper concentrations and, hence, cirrhosis. Cessation of this feeding habit has been associated with disappearance of the disease in each case.

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References

  • Adamson M, Reiner B, Olson JL, Goodman Z, Plotnick L, Bernardini I, Gahl WA. (1992) Indian childhood cirrhosis in an American child. Gastroenterology 102: 1771–1777

    PubMed  CAS  Google Scholar 

  • Aljajeh IA, Mughal S, Al-Tahou B, Ajrawl T, Ismail EA, Nayak NC (1994) Indian childhood cirrhosis — like liver disease in an Arab child. A brief report. Virchows Archiv 424: 225–227

    Article  PubMed  CAS  Google Scholar 

  • Baker A, Gormally S, Saxena R, Baldwin D, Drumm B, Bonham J, Portmann B, Mowat AP (1995). Copper-associated liver disease in childhood. J Hepatol 23: 538–543

    Article  PubMed  CAS  Google Scholar 

  • Bartok I, Szabo L, Horvath E, Ormos J (1971) Juvenile Cirrhose mit hochgradiger kupferspeicherung in der leber. Acta Hepato-Splenologica 18: 119–128

    PubMed  CAS  Google Scholar 

  • Bavdekar AR, Bhave SA, Pradhan AM, Pandit AN, Tanner MS. (1996) Long term survival in Indian childhood cirrhosis treated with D-penicillamine. Archives of Disease in Childhood 74: 32–35

    Article  PubMed  CAS  Google Scholar 

  • Bent S, Böhm K (1995) Copper-induced liver cirrhosis in a 13-month old boy Gesundheitswesen 57:667–669

    PubMed  CAS  Google Scholar 

  • Bhave SA, Pandit AN, Singh S, Walia BNS, Tanner MS. (1992) The prevention of Indian Childhood Cirrhosis. Annals of Tropical Paediatrics 12: 23–30

    PubMed  CAS  Google Scholar 

  • Bunton TE, Baksi SM, George SG, Frazier JM. (1987) Abnormal hepatic copper storage in a teleost fish (Morone americana) Vet Pathol; 24: 515–524

    PubMed  CAS  Google Scholar 

  • Culotta VC, Klomp LWJ, Strain J, Ruby Leah B. Casareno, Krems B, Gittin JD (1997). The Copper Chaperone for Superoxide Dismutase. J Biol Chem; 272: 23469–23472

    Article  PubMed  CAS  Google Scholar 

  • Glerum, D. M., Shtanko, A., and Tzagoloff, A. (1996a) J. Biol. Chem. 34, 20531–20535

    Google Scholar 

  • Glerum, D. M., Shtanko, A., and Tzagoloff, A. (1996b) J. Biol. Chem. 271, 14504–14509

    Article  PubMed  CAS  Google Scholar 

  • Gogl H, (1947) Pathologisch-anatomische Untersuchungen über Leberzirrhose bei Sauglingen und Kleinkindern (infantile Leberzirrhose) mit endemischer Haufung. In Priesel R, ed: Wiener Beitrage zur Kinderheilkunde, vol 1. Wien: Wilhelm Maudrich.

    Google Scholar 

  • Hahn SH, Brantly ML, Oliver C., Adamson M, Kaier SG, Gahl WA. (1994) Metallothionein synthesis and degradation in Indian childhood cirrhosis fibroblasts. Pediatric Research,; 35: 197–204

    Article  PubMed  CAS  Google Scholar 

  • Hahn SH, Tanner MS, Danks DM, Gahl WA. (1995) Normal metallothionein synthesis in fibroblasts obtained from children with Indian childhood cirrhosis or copper-associated childhood cirrhosis. Biochemical and Molecular Medicine 54: 142–145

    Article  PubMed  CAS  Google Scholar 

  • Harris ZL, Takahashi Y, Miyajima H, Serizawa M, MacGillivray RT, Gitlin JD (1995) Aceruloplasminemia: molecular characterization of this disorder of iron metabolism. Proc Natl Acad Sci U S A 92:2539–2543

    Article  PubMed  CAS  Google Scholar 

  • Horslen SP, Tanner MS, Lyon TDB, Fell GS, Lowry MF (1994) Copper associated childhood cirrhosis. Gut 1994; 35 1497–1500.

    Article  PubMed  CAS  Google Scholar 

  • Klass HJ, Kelly JK, Warnes TW (1980) Indian childhood cirrhosis in the United Kingdom. Gut 21: 344–350

    Article  PubMed  CAS  Google Scholar 

  • Klomp, L. W. J., Lin, S. J., Yuan, D., Klausner, R. D., Culotta, V. C., and Gitlin, J. D. (1997) Identification and functional expression of HAH1, a novel human gene involved in copper homeostasis J. Biol. Chem. 272, 9221–9226.

    Article  PubMed  CAS  Google Scholar 

  • Lefkowitch JH, Honig CL, King ME, Hagstrom JWC (1982) Hepatic coper overload and features of Indian childhood cirrhosis in an American sibship. New Engl J Med 307:271–277

    Article  PubMed  CAS  Google Scholar 

  • Lim CT, Choo KE. (1979) Wilson’s disease — in a 2 year old. J Singapore Paediatric Society. 21: 99–102

    CAS  Google Scholar 

  • Lin, S. J., Pufahl, R., Dancis, A., O’Halloran, T. V., and Culotta, V.C. (1997) J. Biol. Chem. 272, 9215–9220

    Article  PubMed  CAS  Google Scholar 

  • Maggiore G, de Giacomo C., Sessa F, Burgio GR. (1987) Idiopathic hepatic toxicosis in a child. J Ped Gastroenterol Nutr 6:980–983

    Article  CAS  Google Scholar 

  • Muller-Hocker J, Weiss M, Meyer J et al (1987). Fatal copper storage disease of the liver in a German infant resembling Indian childhood cirrhosis. Virchows Arch A; 411: 379–385.

    Article  CAS  Google Scholar 

  • Muller-Hocker J, Meyer U, Wiebecke B, et al. (1988) Copper storage disease of the liver and chronic dietary copper intoxication in two further German infants mimicking Indian Childhood Cirrhosis. Path Res Pract; 183: 39–45.

    Article  PubMed  CAS  Google Scholar 

  • Muller T, Feichtinger H, Berger H, Muller W. (1996) Endemic Tyrolean infantile cirrhosis: An ecogenetic disorder. Lancet; 347: 877–880

    Article  PubMed  CAS  Google Scholar 

  • Nayak NC, Ramalingaswami V. (1975) Indian childhood cirrhosis. Clin Gastroenterol;4: 333–349.

    PubMed  CAS  Google Scholar 

  • O’Donohue JW, Reid MA, Varghese A, Portmann B, Williams R. (1993) Micronodular cirrhosis and acute liver failure due to chronic copper self-intoxication. Eur J Gastroenterol Hepatol; 5: 561–562

    Google Scholar 

  • Portmann B, Tanner MS, Mowat AP, Williams R. (1978) Orcein positive liver deposits in Indian childhood cirrhosis. Lancet; i: 1338–1340

    Article  Google Scholar 

  • Pradhan AM, Bhave SA, Joshi VV, Bavdekar AR, Pandit AN, Tanner MS (1995). Reversal of Indian Childhood Cirrhosis by D-penicillamine therapy. J Ped Gastroenterol Nutr;20: 28–35

    Article  CAS  Google Scholar 

  • Price LA, Walker NI, Clague AE, Pullen ID, Smits SJ, Ong TH, Patrick M (1996) Chronic copper toxicosis presenting as liver failure in an Australian child Pathology 28:316–320

    Article  PubMed  CAS  Google Scholar 

  • Schramel P, Muller-Hocker J, Meyer U, Weiss M, Eife R. (1988) Nutritional copper intoxication in three German infants with severe liver cell damage (features of Indian Childhood Cirrhosis). J Trace Elem Electrolytes Health Dis; 2: 85–89.

    PubMed  CAS  Google Scholar 

  • Summer K (1994) Metallothionein und die chronische Toxizitat von kupfer. (Metallothionein and copper toxicity). In: Institut fur wasser-, boden-, und lufthygiene. Kupfer und fruhkindliche Leberzirrhose. (Copper and Childhood Liver Cirrhosis). Dieter, H.H. and Seffner, W. editors. Umwelt Bundes Amt. Berlin.; 38 38–42

    Google Scholar 

  • Tanner (1997) The role of copper in Indian childhood cirrhosis Amer J Clin Nutr 1997 in press

    Google Scholar 

  • Takahashi Y, Miyajima H, Shirabe S, Nagataki S, Suenaga A, Gitlin JD (1996) Characterization of a nonsense mutation in the ceruloplasmin gene resulting in diabetes and neurodegenerative disease. Hum Mol Genet 5:81–84

    Article  PubMed  CAS  Google Scholar 

  • Toyokuni S, Okada S, Hamazaki S, Fujioka M, Li JL, Midorikawa O. (1989) Cirrhosis of the liver induced by cupric nitrilotriacetate in Wistar rats. An experimental model of copper toxicosis. Amer J Path; 134: 1263–1274

    PubMed  CAS  Google Scholar 

  • Walker-Smith J, Blomfield J. (1973) Wilson’s disease or chronic copper poisoning? Arch Dis Child; 48: 476–479

    Article  PubMed  CAS  Google Scholar 

  • Weiss M, Muller-Hocker J, Wiebecke B, Belohradsky BH. (1989) First description of “Indian Childhood Cirrhosis” in a non-Indian infant in Europe. Acta Paediatr Scand; 78: 152–156

    Article  PubMed  CAS  Google Scholar 

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Author information

Authors and Affiliations

  1. University of Sheffield, Children’s Hospital, Western Bank, Sheffield, S10 2TH, UK

    M. S. Tanner

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  1. M. S. Tanner
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Editors and Affiliations

  1. University of Salerno, Salerno, Italy

    Arturo Leone

  2. The Murdoch Institute for Research into Birth Defects, Royal Children’s Hospital, Parkville, Australia

    Julian F. B. Mercer

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© 1999 Springer Science+Business Media New York

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Tanner, M.S. (1999). Indian Childhood Cirrhosis and Tyrolean Childhood Cirrhosis. In: Leone, A., Mercer, J.F.B. (eds) Copper Transport and Its Disorders. Advances in Experimental Medicine and Biology, vol 448. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-4859-1_11

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  • DOI: https://doi.org/10.1007/978-1-4615-4859-1_11

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-7204-2

  • Online ISBN: 978-1-4615-4859-1

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