Is Indian childhood cirrhosis an extinct disease now?--An observational study

Indian childhood cirrhosis (ICC), an unique liver disease that has been endemic in most parts of India and advocated by some to be caused by hepatotoxic effect of excess dietary copper is generally believed now to have virtually disappeared from the country. In the face of this the authors report here five cases of ICC encountered in one children hospital over the last 10 y period.

Cases histologically categorized as ICC were initially picked up from the records of the department of Pathology. Their clinical, investigational and follow up information retrieved from hospital data base along with pathologic features of liver biopsies were reviewed in detail.

The age range of the three male and two female children were from 1 ½ to 12 y and on clinical and investigational features all 5 cases were labeled as non-Wilsonian liver disease of uncertain etiology. Histopathologic findings in each case however, was characteristic of accepted established phase of ICC. Three of the five children died in hospital while the other two left the hospital and were lost to follow up. None of the children had exposure to excess dietary copper.

Cases of ICC continue to occur in Andhra Pradesh and very likely in other parts of India. Established and non-typical cases are possibly being missed because of no histologic confirmation and unawareness of the protean manifestation and natural history of this disease. Dietary copper overload is unlikely to play a causal role in ICC.