Wilson's disease: clinical spectrum of liver disease

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism,characterized by copper accumulation in the liver and brain. This rare entity, which has a broad clinical spectrum, is often difficult to diagnose and should therefore always be suspected in patients with liver d...

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Published inGastroenterología y hepatología Vol. 36; no. 2; p. 86
Main Authors Ochoa Palominos, Alejandra, Ibáñez Samaniego, Luis, Catalina Rodríguez, María-Vega, Pajares Díaz, José, Clemente Ricote, Gerardo
Format Journal Article
LanguageSpanish
Published Spain 01.02.2013
Subjects
Adult
Copper
Female
Hepatolenticular Degeneration - diagnosis
Humans
Liver Diseases - diagnosis
Middle Aged
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Summary:Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism,characterized by copper accumulation in the liver and brain. This rare entity, which has a broad clinical spectrum, is often difficult to diagnose and should therefore always be suspected in patients with liver disease of unclear cause. We describe two types of manifestation of liver disease in two patients; the first developed fulminant hepatic failure requiring urgent liver transplantation and the second showed advanced chronic liver disease and received standard medical treatment. The objective of this clinical observation is to analyze the diagnosis of Wilson's disease in two patients with distinct onset, illustrating the broad clinical spectrum of the disease, and its treatment.
ISSN:0210-5705
DOI:10.1016/j.gastrohep.2012.07.009