Indian childhood cirrhosis is a liver disease caused by the toxic effects of copper overload that occurs in genetically susceptible infants and children. Although initially thought to only occur in Indian children, this disease has been documented in other ethnic groups, as well and is characterized by excess copper (with a higher level than those detectable in Wilson's disease) and Orcein stain positive copper binding protein. An autoimmune mechanism has also been postulated.
Despite the fact that both Wilson's disease and Indian childhood cirrhosis are characterized by hepatic dysfunction induced by copper exposure, their clinical and histological traits do not overlap.
Indian childhood cirrhosis may become symptomatic between the ages of 6 months and 5 years, more commonly after the child is one to two years old . Disease onset is often gradual, with nonspecific signs such as malaise, irritability, sleeplessness, lack of appetite, constipation or diarrhea, fever and abdominal distension due to hepatosplenomegaly, representing pre-cirrhotic findings . These abnormalities, including an enlarged liver, may have multiple causes in children, therefore an early diagnosis is difficult to establish. In severe cases, a hepatic bruit may be noticed. The ponderal curve becomes flat. Abdominal palpation reveals a hard liver with a sharp inferior border localized 3 to 5 centimeters under the inferior rib cage border. Splenomegaly appears after the liver has become enlarged. By the time jaundice appears, the liver function is severely compromised and events like hepatocellular failure, ascites, bleeding due to esophageal varices leading to anemia, bacterial infection and death occur rapidly. In the preterminal phase, symptoms of cirrhosis become overt and include confusion due to hepatic encephalopathy, palmar erythema, spider nevi localized on the upper torso, portal hypertension and hepatic coma. At this point, a specific garlic odor may also be present, also a sign of severe hepatic dysfunction. Dyspnea and cyanosis on exertion are probably a consequence of severe anemia.
Blood workup should include standard hepatic function tests. Alanine transaminase and gamma glutamyl transpeptidase will be increased. A coagulation panel composed of bleeding and clotting time and prothrombin time should be obtained in order to further assess hepatic function and to prevent catastrophic bleeding following a liver biopsy. Ceruloplasmin and copper levels are within normal limits at first  . Cupriuresis following d-penicillamine will be high.
Liver biopsy shows extensive hepatocyte damage, with liver cells appearing vacuolated, necrotic or ballooned. Histologic examination further describes the presence of inflammatory cells  and extensive pericellular intralobular fibrosis with poor regeneration, leading to a so- called "micro-micro nodular" cirrhosis  . Hyaline inclusions, similar to those found in Wilson's disease or alcoholic liver disease have also been described. Fat deposits are small. However, there is one typical histological aspect of this disease, namely the presence of granules that color dark brown with orcein staining due to the fact that they consist of a copper-associated protein .