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Original Report| Volume 32, ISSUE 5, P530-534, October 2019

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The Efficacy of Long-Term Estrogen Replacement Therapy in Turner Syndrome Women with Premature Ovarian Insufficiency

      Abstract

      Study Objective

      To evaluate the efficacy of long-term estrogen replacement therapy (ERT) in uterine development and bone mineral density (BMD) of Turner syndrome (TS) women with premature ovarian insufficiency (POI).

      Design and Setting

      Retrospective study.

      Participants and Interventions

      Thirty-seven TS women grouped according to ovarian function status: TS women with POI (n = 32), aged 11-26 years, and those with intact ovarian function (IOF; n = 5), aged 13-17 years. TS women with POI underwent ERT.

      Main Outcome Measures

      Changes in uterine length, anterior-posterior (AP) fundal diameter of the uterus, and BMD were assessed. Statistical methods included Mann-Whitney U test and paired t test.

      Results

      In TS women with POI, uterine length, AP fundal diameter, and BMD significantly increased after ERT (P < .001). TS women with POI were subdivided into classic (n = 11) and variant (n = 21) types, and there were no significant differences in uterine development and BMD according to types of chromosome. After receiving ERT, AP fundal diameter was significantly longer in classic TS women (P = .034) compared with those with variant type.

      Conclusion

      Long-term ERT increased uterine length (before: 4.4 cm; after: 7.2 cm) and AP fundal diameter (before: 0.9 cm; after: 2.4 cm), and improved BMD in TS women with POI. After ERT, in TS women with POI, uterine length, BMD at lumbar 2-4 and femoral neck were similar to those of TS women with IOF. Therefore, TS women with POI can catch up to those with IOF by receiving ERT.

      Key Words

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      References

        • Ford C.E.
        • Jones K.W.
        • Polani P.E.
        • et al.
        A sex-chromosome anomaly in a case of gonadal dysgenesis (Turner’s syndrome).
        Lancet. 1959; 1: 711
        • Gravholt C.H.
        Epidemiological, endocrine and metabolic features in Turner syndrome.
        Eur J Endocrinol. 2004; 151: 657
        • Birkebaek N.H.
        • Cruger D.
        • Hansen J.
        • et al.
        Fertility and pregnancy outcome in Danish women with Turner syndrome.
        Clin Genet. 2002; 61: 35
        • Bondy C.A.
        • Turner Syndrome Study Group
        Care of girls and women with Turner syndrome: a guideline of the Turner Syndrome Study Group.
        J Clin Endocrinol Metab. 2007; 92: 10
        • Bodri D.
        • Vernaeve V.
        • Figueras F.
        • et al.
        Oocyte donation in patients with Turner’s syndrome: a successful technique but with an accompanying high risk of hypertensive disorders during pregnancy.
        Hum Reprod. 2006; 21: 829
        • Hovatta O.
        Pregnancies in women with Turner’s syndrome.
        Ann Med. 1999; 31: 106
        • Khastgir G.
        • Abdalla H.
        • Thomas A.
        • et al.
        Oocyte donation in Turner’s syndrome: an analysis of the factors affecting the outcome.
        Hum Reprod. 1997; 12: 279
        • Gravholt C.H.
        • Lauridsen A.L.
        • Brixen K.
        • et al.
        Marked disproportionality in bone size and mineral, and distinct abnormalities in bone markers and calcitropic hormones in adult turner syndrome: a cross-sectional study.
        J Clin Endocrinol Metab. 2002; 87: 2798
        • Gravholt C.H.
        • Juul S.
        • Naeraa R.W.
        • et al.
        Morbidity in Turner syndrome.
        J Clin Epidemiol. 1998; 51: 147
        • Kim H.J.
        • Lee D.Y.
        • Yoon B.K.
        • et al.
        Uterine development after estrogen replacement therapy in women with different etiologies of primary hypogonadism.
        J Pediatr Adolesc Gynecol. 2016; 29: 344
        • Kim N.Y.
        • Lee D.Y.
        • Kim M.J.
        • et al.
        Estrogen requirements in girls with Turner syndrome; how low is enough for initiating puberty and uterine development?.
        Gynecol Endocrinol. 2012; 28: 130
        • Cleemann L.
        • Hjerrild B.E.
        • Lauridsen A.L.
        • et al.
        Long-term hormone replacement therapy preserves bone mineral density in Turner syndrome.
        Eur J Endocrinol. 2009; 161: 251
        • Bakalov V.K.
        • Chen M.L.
        • Baron J.
        • et al.
        Bone mineral density and fractures in Turner syndrome.
        Am J Med. 2003; 115: 259
        • Yi K.H.
        • Hwang J.S.
        • Kim E.Y.
        • et al.
        Reference values for bone mineral density according to age with body size adjustment in Korean children and adolescents.
        J Bone Miner Metab. 2014; 32: 281
        • Kang M.J.
        • Hong H.S.
        • Chung S.J.
        • et al.
        Body composition and bone density reference data for Korean children, adolescents, and young adults according to age and sex: results of the 2009-2010 Korean National Health and Nutrition Examination Survey (KNHANES).
        J Bone Miner Metab. 2016; 34: 429
        • Snajderova M.
        • Mardesic T.
        • Lebl J.
        • et al.
        The uterine length in women with Turner syndrome reflects the postmenarcheal daily estrogen dose.
        Horm Res. 2003; 60: 198
        • Nakamura T.
        • Tsuburai T.
        • Tokinaga A.
        • et al.
        Efficacy of estrogen replacement therapy (ERT) on uterine growth and acquisition of bone mass in patients with Turner syndrome.
        Endocr J. 2015; 62: 965
        • Gawlik A.M.
        • Hankus M.
        • Szeliga K.
        • et al.
        Late-onset puberty induction by transdermal estrogen in Turner syndrome girls-a longitudinal study.
        Front Endocrinol (Lausanne). 2018; 9: 23
        • Folsom L.J.
        • Slaven J.E.
        • Nabhan Z.M.
        • et al.
        Characterization of spontaneous and induced puberty in girls with Turner syndrome.
        Endocr Pract. 2017; 23: 768
        • Pasquino A.M.
        • Passeri F.
        • Pucarelli I.
        • et al.
        Spontaneous pubertal development in Turner’s syndrome. Italian Study Group for Turner’s Syndrome.
        J Clin Endocrinol Metab. 1997; 82: 1810
        • Tarani L.
        • Lampariello S.
        • Raguso G.
        • et al.
        Pregnancy in patients with Turner’s syndrome: six new cases and review of literature.
        Gynecol Endocrinol. 1998; 12: 83
        • Hawkins L.K.
        • Correia K.F.
        • Srouji S.S.
        • et al.
        Uterine length and fertility outcomes: a cohort study in the IVF population.
        Hum Reprod. 2013; 28: 3000
        • Griffin I.J.
        • Cole T.J.
        • Duncan K.A.
        • et al.
        Pelvic ultrasound measurements in normal girls.
        Acta Paediatr. 1995; 84: 536
        • Haber H.P.
        • Ranke M.B.
        Pelvic ultrasonography in Turner syndrome: standards for uterine and ovarian volume.
        J Ultrasound Med. 1999; 18: 271
        • Carrascosa A.
        • Gussinye M.
        • Terradas P.
        • et al.
        Spontaneous, but not induced, puberty permits adequate bone mass acquisition in adolescent Turner syndrome patients.
        J Bone Miner Res. 2000; 15: 2005
        • Nadeem M.
        • Roche E.F.
        Bone mineral density in Turner’s syndrome and the influence of pubertal development.
        Acta Paediatr. 2014; 103: e38
        • Bakalov V.K.
        • Bondy C.A.
        Fracture risk and bone mineral density in Turner syndrome.
        Rev Endocr Metab Disord. 2008; 9: 145
        • Mora S.
        • Weber G.
        • Guarneri M.P.
        • et al.
        Effect of estrogen replacement therapy on bone mineral content in girls with Turner syndrome.
        Obstet Gynecol. 1992; 79: 747
        • Lanes R.
        • Gunczler P.
        • Esaa S.
        • et al.
        Decreased bone mass despite long-term estrogen replacement therapy in young women with Turner’s syndrome and previously normal bone density.
        Fertil Steril. 1999; 72: 896
        • Benetti-Pinto C.L.
        • Bedone A.
        • Magna L.A.
        • et al.
        Factors associated with the reduction of bone density in patients with gonadal dysgenesis.
        Fertil Steril. 2002; 77: 571
        • Kodama M.
        • Komura H.
        • Kodama T.
        • et al.
        Estrogen therapy initiated at an early age increases bone mineral density in Turner syndrome patients.
        Endocr J. 2012; 59: 153
        • Doerr H.G.
        • Bettendorf M.
        • Hauffa B.P.
        • et al.
        Uterine size in women with Turner syndrome after induction of puberty with estrogens and long-term growth hormone therapy: results of the German IGLU Follow-up Study 2001.
        Hum Reprod. 2005; 20: 1418
        • Hanew K.
        • Tanaka T.
        • Horikawa R.
        • et al.
        Prevalence of diverse complications and its association with karyotypes in Japanese adult women with Turner syndrome-a questionnaire survey by the Foundation for Growth Science.
        Endocr J. 2018; 65: 509