Chronic cholestasis associated with Turner's syndrome.
Liver abnormalities seem a very rare association in Turner's syndrome, only reported in a few anecdotal cases. We report the clinical and morphological findings in 3 patients who presented with cholestasis. One of them had a definite diagnosis of primary sclerosing cholangitis, in whom the natural history was complicated by the development of two distinct tumors. A review of the few cases described in the literature and our own case reports support the hypothesis that liver disease in Turner's syndrome may have a variety of expressions including cholestasis.
Floreani
A
A
Department of Surgical and Gastroenterological Sciences, University of Padova, Italy. aflor@uxi.unipd.it
Molaro
M
M
Baragiotta
A
A
Naccarato
R
R
D000328:Adult; D001650:Bile Duct Neoplasms / Q000150:complications / Q000175:diagnosis; D001653:Bile Ducts, Intrahepatic; D001706:Biopsy; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D018281:Cholangiocarcinoma / Q000150:complications / Q000175:diagnosis; D002760:Cholangiopancreatography, Endoscopic Retrograde; D002779:Cholestasis / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D002908:Chronic Disease; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D010535:Laparoscopy; D008875:Middle Aged; D014424:Turner Syndrome / Q000150:complications* / Q000175:diagnosis; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D002756:Cholagogues and Choleretics; D014580:Ursodeoxycholic Acid