Will the real Indian childhood cirrhosis please stand up?1

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Indian childhood cirrhosis (ICC), so named to reflect its ethnic origins, was found in 4 Caucasian siblings in the United States who died during the period 1958-1962. Since these children had not been exposed to copper (either endogenous or exogenous) and had no Indian ancestry, the disease at that time was classified as “cryptogenic cirrhosis.” Many years later, pathologic review suggested that the true diagnosis should have been ICC. Hence, Indian childhood cirrhosis is probably a misnomer. Because of the large influx of immigrants from the Indian subcontinent, Western pathologists should become more familiar with the true histologic and gross picture of ICC. The authors differentiate cryptogenic cirrhosis and cirrhosis of Indian childhood from ICC (which is not a true cirrhosis) with illustrative gross specimens and photomicrographs. Historical background and a comprehensive review of the literature are offered.



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