Abstract
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.
MeSH terms
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Adult
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Bile Duct Neoplasms / complications
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Bile Duct Neoplasms / diagnosis
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Bile Ducts, Intrahepatic
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Biopsy
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Cholagogues and Choleretics / therapeutic use
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Cholangiocarcinoma / complications
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Cholangiocarcinoma / diagnosis
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Cholangiopancreatography, Endoscopic Retrograde
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Cholestasis / diagnosis
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Cholestasis / drug therapy
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Cholestasis / etiology*
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Chronic Disease
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Diagnosis, Differential
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Fatal Outcome
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Female
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Humans
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Laparoscopy
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Middle Aged
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Turner Syndrome / complications*
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Turner Syndrome / diagnosis
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Ursodeoxycholic Acid / therapeutic use
Substances
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Cholagogues and Choleretics
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Ursodeoxycholic Acid