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 audit 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 2008-2017, excluding those yet to start progestogens (n=27). Data is mean+/-SD.
Result: Pubertal induction was started at 13.1±1.8years and progestogen introduced at 16.1±1.9years; duration of unopposed oestrogen action was 2.8±0.8years. Eleven (40.7%) patients used oral EE, 10 (37.0%) patches and 6 (22.2%) changed from one form to the other. Where recorded, 15(62.5%) were in Tanner stage 1, 7(29.2%) in stage 2, while 2 (8.3%) were in stage 3 before induction. At introduction of progestogen, 19(82.6%) were in stage 3 and the rest in stage 4.
Height SDS (UK-WHO reference) was -2.3±1.0 at pubertal induction and -1.9±1.0 at completion. Height SDS change during induction was 0.5±1.0. There was no significant difference between oestrogen regimens in height SDS change (oral: 0.4±1.0, patches: 0.8±1.1, P=0.4).
Nine (33.3%) had pelvic USS before pubertal induction, of which there was a normal prepubertal uterus in 8 and normal ovaries in 1. Six (21.4%) had a pelvic USS at the end of puberty; 5 had normal sized post-pubertal uterus and 1 remained infantile.
Raised ALT (≥35iu/l) with no clinical symptoms of liver disease was seen both pre- and post- puberty (2/26 and 5/25 respectively). The 2 girls with pre-pubertal raised ALT remained so after puberty. Of the 3 additional girls with deranged LFT, 2(66.7%) used EE and 1(33.3%) used patch, (P=1.000).
Seventeen (63.0%) patients had DEXA at transition, 2 had low bone mineral density (BMD). Both presented at 13 and 16 years with short stature and delayed puberty; both used patches. BMD status was not significantly different between oestrogen regimens (P=0.5).
Conclusion: Induction of puberty with oral or patch oestrogen appears to be equally effective in girls with TS. One third of girls who started on patches switched to an oral preparation. Uterine imaging was not consistently undertaken.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology