Abstract
We report a disease entity which occured in certain areas of West Germany and which was not diagnosed correctly in the past. At present, we are aware of 21 patients (pts) in 15 unrelated families with a total of 45 children. 11 pts died between 8 and 13 months of age, and 6 survived with liver cirrhosis. In the remaining 4 transaminases were initially elevated and turned to normal with correction of drinking water. All families lived in rural areas known for acid ground water. Drinking water was obtained from the own farm's wells with low pH (5.0 - 6.2) and was highly contaminated after passage through copper pipes. All pts were exposed to copper beginning shortly after birth; none of them was breast fed and all received milk formula prepared with the tap water. None of the 24 healthy siblings were exposed during the first three months of life. All pts developed a micronodular liver cirrhosis which resembled the “Indian Childhood Cirrhosis” and which was accompanied by significant copper accumulation in the liver. Common clinical symptoms included: enlargement of liver and spleen, anemia, bleeding disorders, muscular hypotonia. We believe that this disease might occur in other parts of the world if the environmental conditions are similar (acid drinking water and copper piping).
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Eife, R., Müller-Höcker, J., Wei, M. et al. FAMILIAL' INFANTILE LIVER CIRRHOSIS AND HEMOLYTIC ANEMIA DUE TO CHRONIC COPPER INTOXICATION VIA TAP WATER FROM COPPER PIPES. Pediatr Res 26, 525 (1989). https://doi.org/10.1203/00006450-198911000-00153
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DOI: https://doi.org/10.1203/00006450-198911000-00153