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Gastroenterol Hepatol. 1995 Aug-Sep;18(7):375-8.

[Chronic asymptomatic intrahepatic cholestasis associated with Turner's syndrome].

[Article in Spanish]

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  • 1Servicio de Aparato Digestivo, Hospital Universitario Virgen de la Victoria, Málaga.

Abstract

Turner syndrome or the gonadal dysgenesis syndrome which is monosomic because of the lack of an X chromosome (45 X) is associated to a greater incidence of autoimmune, particularly thyroidal, disorders and inflammatory intestinal disease, but is rarely associated to hepatic disorders. A female patient with chronic asymptomatic intrahepatic cholestasis which, to our knowledge, is the first reported in Spain, is herein presented. The 40-year old patient with a 45 X karyotype, feminine phenotype was accidently found to have a chronic alteration in the hepatic profile. Hepatic biochemical tests revealed AST 59 U/L, ALT 90 U/L, GGT 201 U/L and alkaline phosphatase 320 U/L. Hepatic echography was normal. Percutaneous liver biopsy was performed demonstrating minimum changes consisting of sinusoidal dilatation and pigment accumulation in the hepatocyte biliary pole. Treatment with ursodeoxycholic acid 15 mg/kg/day was administered showing a marked decrease in the laboratory parameters during follow up. Different hypothesis which may explain the association between chronic asymptomatic intrahepatic cholestasis and Turner syndrome are discussed.

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PMID:
7553275
[PubMed - indexed for MEDLINE]
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