[Chronic asymptomatic intrahepatic cholestasis associated with Turner's syndrome].
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.
Andrade
R J
RJ
Servicio de Aparato Digestivo, Hospital Universitario Virgen de la Victoria, Málaga.
Alcántara
R
R
Fraile
J M
JM
Lazo
M D
MD
Llamas
A
A
Carmona
C
C
Franquelo
E
E
D000328:Adult; D002756:Cholagogues and Choleretics / Q000008:administration & dosage / Q000627:therapeutic use; D002780:Cholestasis, Intrahepatic / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D013997:Time Factors; D014424:Turner Syndrome / Q000150:complications*; D014580:Ursodeoxycholic Acid / Q000008:administration & dosage / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid