What to Do When Initial Pubertal Induction Falls Short in a Girl Aged 11–12 with Turner Syndrome and Elevated FSH
This protocol covers the next clinical step for girls aged 11–12 with Turner syndrome who have hypergonadotropic hypogonadism, persistently elevated FSH, and no spontaneous thelarche — specifically when the goals of the initial pubertal induction treatment have not been reached.
Clinical Scenario
- Girl aged 11–12 years with Turner syndrome
- Hypergonadotropic hypogonadism confirmed
- FSH elevated on at least two sequential measurements
- No spontaneous thelarche
Estrogen replacement initiation is recommended when FSH is elevated on at least two sequential measurements in this age group.
Previous Line — Goals Not Yet Achieved
The initial therapy, low-dose 17β-estradiol, aims for progressive breast development and achievement of adult serum E2 concentrations. This next-line protocol is indicated when those targets — progressive breast development and adequate serum E2 levels at full adult replacement — have not been fully reached.
Next-Line Approach (Partial Overview)
At this stage, the protocol involves continuing the estrogen regimen and introducing a cyclic progestogen. The specific agents, sequencing, and full dosing details are set out in the complete protocol.
References
DOI: 10.1093/ejendo/lvae050
- We recommend initiation of low dose estrogen replacement between 11 and 12 years of age, if FSH is elevated on at least two sequential measurements.
- We recommend adding cyclic progesterone once breakthrough bleeding occurs (mostly this will be after about 18-24 months of unopposed estrogen exposure but this can occur later based on pubertal stage, serum E2 and uterine growth, endometrial thickness, and estrogen dose). The preferred option is micronized progesterone 200 mg for 10-12 days per month.
- When available, micronized progesterone at 200 mg for 12 days per month is the preferred progestin, with dydrogesterone at 10 mg for 12 days as second choice.
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