ESPE2024 Poster Category 1 Growth and Syndromes 2 (10 abstracts)
1University of Nottingham, Nottingham, United Kingdom. 2Department of Paediatrics, university of Maiduguri, Borno State, Nigeria. 3Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom
Background: Pubertal induction with incremental doses of oestrogen replacement is an important component of care offered to hypogonadal patients with Turner Syndrome (TS). Low dose oral ethinylestradiol (EE) has been extensively used in the UK but natural 17-β oestradiol (more physiological, cheaper and easily monitored in blood) is becoming increasingly popular. We undertook this updated review, following on from previous work, to compare the efficacy and acceptability of oral (EE) and patch (Evorel) oestrogen preparations used in our centre.
Subjects & Method: A retrospective audit was undertaken analysing the clinical records of all girls with TS who started pubertal induction 2008-2024, excluding those yet to start progestogens (n = 34). Data is mean+/-SD.
Result: Pubertal induction was started at 13.0±1.9years (Q1:11.5; Q3:14.6) and progestogen introduced at 16.0±1.8 years (Q1:14.9; Q3:17.2); duration of unopposed oestrogen action was 3.0±1.1years. Fourteen patients used patches twelve patients used EE and 8 used a combination. Where recorded, 18 were in Tanner stage 1, 6 in stage 2, while 3 were in stage 3 before induction. At introduction of progestogen, 22 were in stage 3 and the rest in stage 4. Height SDS (RCPCH Growth Charts for TS) was 1.5±0.9 at pubertal induction and 1.8±0.9 at completion. Height SDS change during induction was 0.3±0.7. There was no significant difference between oestrogen regimens in height SDS change (oral: 0.3±0.9, patches: 0.3±0.4, P = ns). BMI SDS at induction of oestrogen treatment was 0.9±1.2, at completion 1.2±1.1, with a BMI SDS change of 0.3±0.7. Eleven (32.3%) had pelvic USS before pubertal induction, of which there was a normal prepubertal uterus in 10. Six had a pelvic USS at the end of puberty; 5 had normal sized post-pubertal uterus and 1 remained infantile. Nineteen (55.9%) patients had DEXA at transition. The overall mean z-score for lumbar bone density was -0.8 (Q1:-1.8; Q3:-0.1). The mean whole body density less head was -0.8 (Q1:-1.6; Q3:-0.3). 3 had low bone mineral density (BMD) at the lumbar spine. 2 of these presented with short stature and delayed puberty at 13 and 16 years respectively; 2 used patches and the other used a combination. BMD score was not significantly different between oestrogen regimens (P = ns).
Conclusion: Induction of puberty with oral or patch oestrogen appears to be equally effective in girls with TS. USS and DEXA scans were not consistently undertaken.