ESPE Abstracts

Introduction: Estrogen treatment is crucial for girls with TS to induce puberty. To mimic normal physical development, initiation of treatment should begin at 11–12 years of age if gonadotropins are elevated, or AMH is low. Different estrogen preparations can be used for this purpose, however only IM depot estradiol is available in Egypt.

Aim: To evaluate progression of puberty and uterine development using IM depot estradiol in girls with TS.

Methods: This study was conducted on 40 girls with Turner syndrome around the age 11-12 years old, on GH therapy, with high gonadotropins and low AMH and inhibin B. All were prepubertal. Twenty-six patients (65%) had 45 X0 karyotype, 14 patients (35%) had mosaic karyotype: 5 patients with 46Xi, 4 patients with 45 X0/ 46 XX, 3 patients with 45X0/ 46Xi, 1 patient with 46 XX/ 46 Xi and 1 patient with 45 X0/ 47 XXX. Average duration of GH therapy was 2 to 5 years before recruitment, with an average dose of 0.12 U/kg/day. IM depot estradiol was used for pubertal induction at a monthly intramuscular dose of 0.2 mg and the dose increased at 6-month intervals to 0.4, 0.6 and 0.8 mg, before introduction of progestin. They are followed up clinically for signs of puberty at 6 months and 1 year after estrogen therapy. Also 40 age and sex matched healthy controls were included for serum AMH and Inhibin B values. Uterine and ovarian ultrasound was done at the start of the study before induction of puberty and after one year of pubertal induction.

Results: After 6 months of IM depot estradiol, 37.5 % developed breast stage 2, and after 1 year 77.5% had B2, 22.5% had B3 and one of them had menarche once. After 1 year of depot estradiol, there is a statistically significant increase in uterine dimensions by ultrasonography (total body, cervical and transverse lengths). There is also a statistically significant increase in endometrial thickening and AP diameter. Also the study shows statistically significant increase in the growth velocity after adding the minimal dose IM depot estradiol with the GH therapy.

Conclusion: IM estradiol depot resulted in improved breast development and significant increase in uterine and ovarian size at 12 months of therapy in TS girls. Timely initiation of low doses of E2 is crucial to preserve growth potential.