Early childhood cirrhoses (ECC) in Germany between 1982 and 1994 with special consideration of copper etiology

Abstract

In a multicentric retrospective clinical study with 16 pediatric centres we identified 103 cases of histologically confirmed early childhood cirrhosis (ECC) in Germany for the years 1984-1994. The most prominent diagnoses were congenital bile duct anomalies (47.5%), inborn metabolic disorders (17.5%) and unclear etiologies (17.5%). Chronic and excessive intake of copper might be discussed as an etiological factor in 8 other cases. 5 of these were proven to have coincided with very high hepatic copper contents and copper plumbing/acid well water. Their connection with copper exposure must be considered as probable, whereas 3 others were only suspected copper cases, mainly due to reliable exclusion of other etiologies. High corrosivity (base capacity) values and copper levels in the water for infants formula of 9-26.4 mg/L were determined in those probable cases for which exposure conditions could be exactly reproduced. Additional reports on copper associated ECC, either Indian Childhood Cirrhosis (ICC) from outside India or so-called Idiopathic Copper Toxicosis ( ICT ), originate from Austria, Australia, Germany, Ireland, USA.