Abstract
An Arab female child presented with rapidly progressive liver disease, with apparent onset in late infancy and death at 15 months. Microscopy showed panacinar hepatitis, portal and pericellular fibrosis, and diffuse Mallory bodies in the absence of steatosis or significant cholestasis. Hepatic copper concentration was moderately elevated. Known causes of early childhood cirrhosis were excluded. This case meets most of the established criteria of Indian childhood cirrhosis, yet is unusual in its occurrence in a child of Arab ancestry and in having a moderate degree of hepatocellular copper overload.
Similar content being viewed by others
References
Adamson M, Reiner B, Olson JL, Goodman Z, Plotnick L, Bernardini I, Gahl WA (1992) ICC in an American child Gastroenterology 102:1771–1777
Anthony PP, Ishak KG, Nayak NC, Poulsen HE, Scheuer PJ, Sobin LH (1977) The morphology of cirrhosis: definition nomenclature, and classification. Bulletin of the World Health Organization 155:521–524
Lefkowitch JH, Honig C, King M, Hagstrom JWC (1982) Hepatic copper overload and features of Indian childhood cirrhosis in an American sibship. N Engl J Med 307:271–277
Maggiore G, De Giacomo C, Sessa F, Burgio GR (1987) Idiopathic hepatic copper toxicosis in an Italian child. J Pediatr Gastroenerol Nutr 6(6): 980–983
Mehrotra R, Pandey RK, Nath P (1981) Hepatic copper in Indian childhood cirrhosis. Histopathology 5:659–665
Muller-Hocker J, Weiss MM, Meyer U, Schramel P, Wiebecke B, Belohradsky BH, Hubner G (1987) Fatal copper liver storage disease in German infant resembling Indian childhood cirrhosis. Virchows Arch [A] 411:379–385
Muller-Hocker J, Meyer U, Wiebecke B, Hubner G, Eife R, Kellner M, Schramel P (1988) Copper storage disease of the liver and chronic dietary copper intoxication in two further German infants mimicking Indian childhood cirrhosis. Pathol Res Pract 183(1): 39–45
Nayak NC (1987) Indian childhood cirrhosis. In: MacSween RNM, Anthony PP, Scheuer PJ (eds). Pathology of the liver. Churchill Livingstone, New York, pp. 358–360
Roy S, Ramalingaswami V, Nayak NC (1971) An ultrastructural study of the liver in Indian childhood cirrhosis with particular reference to the structure of cytoplasmic hyaline. Gut 12:693–701
Tanner MS, Portman B, Mowat AP, Williams R, Pandit AM, Mills CF, Bremner I (1979) Increased hepatic copper concentration in Indian childhood cirrhosis. Lancet II: 1203–1205
Walker-Smith JA, Blomfield J (1973) Wilson's disease or chronic copper poisoning? Arch Dis Child 49:476–479
Yokoo H, Minick OT, Batti F, Kent G (1972) Morphologic variants of alcoholic hyaline. Am J Pathol 69:25–40
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Aljajeh, I.A., Ajrawi, T., Mughal, S. et al. Indian childhood cirrhosis — like liver disease in an Arab child. A brief report. Vichows Archiv A Pathol Anat 424, 225–227 (1994). https://doi.org/10.1007/BF00193504
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00193504