Copper and Indian childhood cirrhosis.

Abstract

The hypothesis is advanced that copper is aetiologically related to Indian Childhood Cirrhosis (ICC), and thus that ICC may be a treatable and even preventable disorder. A prerequisite for any discussion about ICC is a clear definition of terms, and, in the studies summarised here, the label 1CC was restricted to those children proven to have the histopathological features of ICC described by Nayak and Ramalingaswami. t Data derived from series in which biopsy confirmation of the diagnosis was not obtained must be interpreted with the greatest caution. The findings which have led us to the hypothesis advanced above are as follows : 1. In all cases of ICC, orcein staining of liver sections revealed multiple, coarse, dark-brown granules in most hepatocytes. ~ These had previously been noted, and thought to be breakdown products of degenerating hepatocytes. 3 They are, however, identical to the deposits of copper-associated protein (CAP) seen in other situations of hepatic copper accumulation, such as Wilson's disease or primary biliary cirrhosis in adults. 4 Two important points mu~t be made about orcein staining. Firstly, it is essential to test each batch of the stain with a positive control, and, secondly, alcohol fixation of tissues gives a falsely