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Case Report

Wilson’s Disease (Coomb Negative Hemolytic Anaemia in Acute Liver Failure)

Sunil N. Mhaske , Sunil N. Mhaske

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Indian Journal of Genetics and Molecular Research 1(2):p 67-70, July - December 2012. | DOI:

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Abstract

Wilson’s disease or hepatolenticular degeneration is an autosomal recessive genetic disorder in which copper accumulates in tissues; manifesting as neurological or psychiatric symptoms and liver disease. This has varied range of manifestations like asymptomatic hepatomegaly, subacute or chronic hepatitis, acute hepatic failure etc. Because of this there is delay in diagnosis and initiation of treatment are common, even in patients with positive family history. There is no consensus regarding therapeutic protocols since the use of penicillamine, once a ‘gold standard’ for treatment, has been debated by experts. Mortality and morbidity of this potentially treatable disease and nonavailability of medications to the poor patients remain a major area of concern. 

Keywords: Wilson’s disease; Acute hepatic failure; K.F. ring; Coomb negative hemolytic anaemia.

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