Indian childhood cirrhosis – i am still around

Abstract

<h3>Background</h3> Indian childhood cirrhosis (ICC) is unique to the Indian subcontinent and has fascinated the scientific community for decades. ICC is said to be disappearing from India but scattered reports of ICC-like cirrhosis are appearing in the West. <h3>Materials and methods</h3> Fifteen cases of ICC were diagnosed amongst 181 pediatric autopsies with various hepatic disorders. <h3>Results</h3> ICC constituted 21.4% of the metabolic disorders and 8.2% of all the causes of hepatic diseases. They ranged in age from 8 months to 2.5 years. Of these 15 cases, 9 were of classical ICC and 6 were of atypical ICC. Coronal slices of the liver were bile stained and revealed micronodules with a sharp leafy margin. Microscopically the cases of classical ICC showed micronodular cirrhosis, diffuse degeneration of hepatocytes with absence of regenerative nodules, abundant Mallory's hyaline, predominantly neutrophilic infiltrate, extensive pericellular fibrosis and excess of Cu-binding proteins by Orcein stain. There were 6 cases of atypical ICC, all which showed presence of regenerative nodules and steatosis in 4 cases. <h3>Conclusion</h3> ICC is very much prevalent in the Indian subcontinent, although reports say that it has nearly disappeared.