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 aminotr ansferase, aspartate aminotransferase and gamma-glutamyl transferase were m onitored 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 II. T he increase in serum liver enzymes was never observed before the age of 7 y ears. In the majority of cases (10/14) it was drug related: in 50% the live r abnormalities were transient and self-limiting, in the remaining cases th ey required interruption of hormonal therapy.. Hepatotoxicity was more freq uently observed in girls treated with oestrogens or oxandrolone than in tho se treated with growth hormone. In a small number of cases, liver disease w as 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. Conclusion 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.