Liver abnormalities in Turner syndrome

We evaluated whether hepatic abnormalities represent a specific feature in girls with Turner syndrome (TS) or whether they are related to an increased susceptibility to hormonal therapies and/or other factors. Alanine aminotransferase, aspartate aminotransferase and gamma-glutamyl transferase were m...

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Bibliographic Details
Published in:European journal of pediatrics 1999-08, Vol.158 (8), p.618-623
Main Authors: SALERNO, M, DI MAIO, S, GASPARINI, N, RIZZO, M, FERRI, P, VAJRO, P
Format: Article
Language:English
Subjects:
Age
Alanine Transaminase - blood
Aspartate Aminotransferases - blood
Biological and medical sciences
Child, Preschool
Chromosome aberrations
Enzymes
Genetic disorders
Growth hormones
Hepatitis
Humans
Infant
Liver - enzymology
Liver diseases
Liver Diseases - etiology
Liver Function Tests
Medical genetics
Medical sciences
Obesity
Overweight
Patients
Retrospective Studies
Thyroid gland
Transferases - blood
Turner Syndrome - complications
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Summary:We evaluated whether hepatic abnormalities represent a specific feature in girls with Turner syndrome (TS) or whether they are related to an increased susceptibility to hormonal therapies and/or other factors. Alanine aminotransferase, aspartate aminotransferase and gamma-glutamyl transferase were monitored in 70 patients with TS for a mean period of 7.6+/-4.2 years. An increase in serum liver enzymes was observed in 14 out of 70 girls (20%) at a mean age of 12.7 years; it was present at entry before hormonal therapy in 3 girls and developed thereafter during the follow up in the other 11. The increase in serum liver enzymes was never observed before the age of 7 years. In the majority of cases (10/14) it was drug related: in 50% the liver abnormalities were transient and self-limiting, in the remaining cases they required interruption of hormonal therapy. Hepatotoxicity was more frequently observed in girls treated with oestrogens or oxandrolone than in those treated with growth hormone. In a small number of cases, liver disease was either auto-immunity-related (2/14), or cryptogenic (1/14) with a benign and self-limiting course. Obesity was a frequent finding, but it played a likely pivotal role only in one patient. Hepatic abnormalities are relatively frequent in Turner syndrome and surveillance of liver function should be included in the management of these patients independent of initiation of hormonal treatment.
ISSN:0340-6199
1432-1076
DOI:10.1007/s004310051163