First description of "Indian childhood cirrhosis" in a non-Indian infant in Europe.
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 5510 micrograms/l) 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.
Weiss
M
M
Department of Paediatrics, University of München, FRG.
Müller-Höcker
J
J
Wiebecke
B
B
Belohradsky
B H
BH
D003300:Copper / Q000008:administration & dosage / Q000378:metabolism; D005260:Female; D005860:Germany, West; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008103:Liver Cirrhosis / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D011379:Prognosis; D014881:Water Supply / Q000032:analysis
D003300:Copper