The importance of estrogen replacement in young women with Turner syndrome.
BACKGROUND
Most girls with Turner syndrome (TS) need estrogen replacement treatment (ERT) to induce and maintain feminization and prevent osteoporosis. There is abundant information on ERT use in postmenopausal women, but there is little information on this issue in women with TS. We aimed to determine the level of ERT use in women with TS living in the United States and assess the effects of ERT adherence vs. nonadherence on bone mineral density (BMD).


METHODS
Fifty women with TS aged 30-59 years had ERT history obtained by structured interviews and BMD assessed at the lumbar spine by dual x-ray absorptiometry (DXA) and quantitative computed tomography (QCT).


RESULTS
Thirty-four of the 50 women with TS had received ERT according to current recommendations, and the rest did not either because of physician failure to prescribe (5 of 50) or because of nonadherence to prescribed ERT (11 of 50). The mean duration of ERT was 25 +/- 2 years for the standard of care group vs. 8 +/- 2 years for the others (p < 0.0001). The major factor promoting adherence to ERT was education on the importance of ERT for bone health (p < 0.001). As expected, lumbar spine BMD was significantly reduced in women not taking ERT according to current guidelines (e.g., a reduction of 20% by QCT, p < 0.001) with 6 of 16 of these women having osteoporosis and 3 of 16 having vertebral compression fractures compared with 0 of 34 in the ERT adherent group.


CONCLUSIONS
Approximately 70% of women with TS in this sample of highly educated women in the United States are taking ERT as currently recommended and appear to be protected from osteoporosis of the spine, whereas those women using ERT less than 75% of the time are at grave risk for osteoporosis. In a time of new reservations about postmenopausal ERT, it is important to emphasize to young women with TS and their caregivers that ERT is critical for bone health.
Hanton
Lori
L
Developmental Endocrinology Branch, National Institute of Child Health, National Institutes of Health, Bethesda, Maryland20892, USA.
Axelrod
Lauren
L
Bakalov
Vladimir
V
Bondy
Carolyn A
CA
D015502:Absorptiometry, Photon; D000328:Adult; D015519:Bone Density / Q000187:drug effects*; D015914:Estrogen Replacement Therapy; D004967:Estrogens / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008159:Lumbar Vertebrae / Q000000981:diagnostic imaging / Q000187:drug effects; D008875:Middle Aged; D010024:Osteoporosis / Q000517:prevention & control*; D010353:Patient Education as Topic / Q000379:methods; D011322:Primary Prevention / Q000379:methods; D012307:Risk Factors; D011795:Surveys and Questionnaires; D013997:Time Factors; D016896:Treatment Outcome; D014424:Turner Syndrome / Q000188:drug therapy* / Q000503:physiopathology*; D014481:United States; D016387:Women's Health
D004967:Estrogens