Indian childhood cirrhosis is a chronic
liver disease
Liver disease, or hepatic disease, is any of many diseases of the liver. If long-lasting it is termed chronic liver disease. Although the diseases differ in detail, liver diseases often have features in common.
Signs and symptoms
Some of the si ...
of childhood characterised by
cirrhosis
Cirrhosis, also known as liver cirrhosis or hepatic cirrhosis, and end-stage liver disease, is the impaired liver function caused by the formation of scar tissue known as fibrosis due to damage caused by liver disease. Damage causes tissue rep ...
of the liver
associated with the deposition of
copper
Copper is a chemical element with the symbol Cu (from la, cuprum) and atomic number 29. It is a soft, malleable, and ductile metal with very high thermal and electrical conductivity. A freshly exposed surface of pure copper has a pinkis ...
in the
liver
The liver is a major organ only found in vertebrates which performs many essential biological functions such as detoxification of the organism, and the synthesis of proteins and biochemicals necessary for digestion and growth. In humans, it ...
.
It primarily affects children of 1–3 years of age and has a genetic predisposition. It had a very high case fatality in the past, but has eventually become preventable, treatable "with D-penicillamine in the treatment of 85 biopsy proven cases of Indian childhood cirrhosis", according to the ''Indian Journal of Pediatrics''. "The drug significantly (P< 0.002) reduced the serum and hepatic copper content and simultaneously there was improvement in clinical and symptomatic aspects. This therapy was compared with the conventional corticosteroid therapy."
It remains a part of the differential diagnosis of
Wilson's disease
Wilson's disease is a genetic disorder in which excess copper builds up in the body. Symptoms are typically related to the brain and liver. Liver-related symptoms include vomiting, weakness, fluid build up in the abdomen, swelling of the legs, ...
.
See also
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North American Indian childhood cirrhosis
References
Further reading
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Diseases of liver
Hepatology
Pediatrics
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