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First Description of “Indian Childhood Cirrhosis” in a Non‐Indian Infant in Europe

Identifieur interne : 00E052 ( Main/Exploration ); précédent : 00E051; suivant : 00E053

First Description of “Indian Childhood Cirrhosis” in a Non‐Indian Infant in Europe

Auteurs : M. Weiss [Allemagne] ; J. Muller-Hocker [Allemagne] ; B. Wiebecke [Allemagne] ; B. H. Belohradsky

Source :

RBID : ISTEX:422D506F56C3ADDD2E8C0013AD17EFA7142DD244

Abstract

ABSTRACT. The first female child of healthy German parents, breast‐fed for 5 weeks, developed progressive abdominal distension due to hepatosplenomegaly at the age of 7 months and died from acute liver failure 3 months later. Histology showed destruction of liver architecture with extreme hepatocellular copper storage, characteristic of “Indian childhood cirrhosis” (ICC). The family received drinking water from a well via copper pipes. The copper level of the water was extremely elevated (430 and 5 510 μg/1) so that exogenous copper intake during the first months of life may have induced the fatal liver disease, probably in association with an unproven genetic disposition. This is the first description of a case of ICC in a member of a non‐Indian family in Europe.

Url:
DOI: 10.1111/j.1651-2227.1989.tb10908.x


Affiliations:


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Le document en format XML

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<div type="abstract">ABSTRACT. The first female child of healthy German parents, breast‐fed for 5 weeks, developed progressive abdominal distension due to hepatosplenomegaly at the age of 7 months and died from acute liver failure 3 months later. Histology showed destruction of liver architecture with extreme hepatocellular copper storage, characteristic of “Indian childhood cirrhosis” (ICC). The family received drinking water from a well via copper pipes. The copper level of the water was extremely elevated (430 and 5 510 μg/1) so that exogenous copper intake during the first months of life may have induced the fatal liver disease, probably in association with an unproven genetic disposition. This is the first description of a case of ICC in a member of a non‐Indian family in Europe.</div>
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