Abstract

GUERRA MONTERO, Luis; ORTEGA ALVAREZ, Félix; SUMIRE UMERES, Julia  and  COK GARCIA, Jaime. Wilson disease: liver form . Rev. gastroenterol. Perú [online]. 2015, vol.35, n.4, pp.361-365. ISSN 1022-5129.

Wilson disease (WD) is a disorder of copper metabolism that is inherited as an autosomal recessive, which produces toxic copper accumulation mainly in the liver and brain, in general has two ways presentation, liver at early ages and neurological in later ages. We present the case of a female patient of 21 years diagnosed of WD in liver cirrhosis that started with an edematous ascites without any neurological symptoms despite the age. Their laboratory studies showed decrease in serum ceruloplasmin and high cupruria within 24 hours of the disease , characteristic data of WD. Although WD is not a common disease should be suspected in all chronic liver disease of unknown etiology with negative viral markers and autoimmunity with or without neurological manifestations as soon as posible and starting treatment with copper chelating mainly leads to a substantial improvement the prognosis of these patients.

Keywords : Wilson Disease; Liver cirrhosis; Ceruloplasmin.

        · abstract in Spanish     · text in Spanish     · Spanish ( pdf )