Schramel P, Müller-Höcker J, Meyer U, Weiss M, Eife R
Gesellschaft für Strahlen- und Umweltforschung mbH, Institut für Okologische Chemie, Neuherberg, FR Germany.
J Trace Elem Electrolytes Health Dis. 1988 Jun;2(2):85-9.
Three of the four children of two unrelated German families fell ill in the first year of life with severe hepatopathy leading to death in two of the children so far, after a progressive clinical course and severe hepatic failure. Laboratory and morphological investigations revealed a high concentration of copper in the liver and to a lesser degree in the kidneys and other organs. The liver architecture was severely altered by micronodular cirrhosis with toxic liver cell damage similar to that found in Indian childhood cirrhosis. Epidemiologically, copper intoxication of the drinking water was verified. The drinking water was obtained from wells via copper pipes. The copper content of the drinking water went as high as 3400 micrograms/l in the two families while water taken directly from the well showed normal content but had lowered pH (6.2). Both parents were clinically healthy, as was an older son who had not been exposed to copper intoxication during the first nine months of his life. Therefore, copper intoxication during the perinatal period appears to be a prerequisite for manifestation of the disease.
两个不相关的德国家庭的四个孩子中有三个在出生后的第一年就患上了严重的肝病，其中两个孩子在经历了渐进性的临床病程和严重的肝衰竭后，目前已经死亡。实验室和形态学检查发现肝脏中铜的浓度很高，肾脏和其他器官中的铜浓度较低。肝脏结构因小结节性肝硬化而严重改变，伴有毒性肝细胞损伤，类似于印度儿童肝硬化。从流行病学角度证实了饮用水铜中毒。饮用水是通过铜管从水井中获取的。这两个家庭的饮用水中铜含量高达3400微克/升，而直接从水井中取出的水铜含量正常，但pH值较低（6.2）。父母双方临床健康，一个年长的儿子在其生命的前九个月未接触过铜中毒，因此，围产期铜中毒似乎是该疾病表现的一个先决条件。
