Induction of puberty in adult endocrinologyPresentation Transcript
Induction of Puberty in Adult Endocrinology:What do I need to know? Kate Lissett Torbay Hospital
Induction of puberty in adults Who When How Hazards
Who Which doctor? Which patient?
How many of you have been involved in taking patients through puberty......... Can anyone give a brief case history......
AM 18.5 years Attending Cardiology for regular follow up of VSD, PMH IUGR. Referred 18 months ago with primary amenorrhoea, but failed to make /attend appointments due to embarrassment. Also complains of lack of breast development.
On examination: Very limited axillary and pubic hair, breasts stage 2 External genitalia normal, P.V. not performed Trans abdominal ultrasound; v small uterus, ovaries could not be identified. Bloods LH 80, FSH 60, estradiol 44 Karyotype repeated (had been done at birth) normal
CA 33 years Presented on acute medical take with severe heart failure. Investigations go on to reveal severe AR and dilated aortic root. Clinical evidence suggestive of previously undiagnosed Turners syndrome. Goes on to have composite aortic valve and root replacement. Now attending clinic, with a view to further endocrine management (NB karyotype mosaic, 46 X with an isodicentric Y (dominant cell line) and 45 XO )
What is normal
Stages of Pubertal Development Pubertal Sequence Accelerated growth->breast development->adrenarche->menarche requires 4.5 years (range 1.5 to 6 years)
Signs of Puberty: Tanner Staging
How.... What estrogen At what dose How would you monitor
Be aware Emotional /psychological as well as physical impact
Difficulties/Risks Drug availability – prescribe in secondary care Prescription of “adult dose” estrogens by non specialist May have long term impact on breast /uterine development Psychological implications
Do you have any case histories
PC 23 years Referred by GP , as couple have primary infertility Patient denies any symptoms or signs On examination, eunachoidal phenotype, 5’10’’, prepubertal Further investigation suggests hypogonadotrophic hypogonadism
What are the aims of treatment
What options do you have in this man to induce puberty?
GHRH pump – not something I’ve used but available at some centres 200-500 IU (c.f. Adult doses of 1250–5000) hCG – will normalize testosterone levels and induced testicular growth. Cost and need for regular injections means this is not usual option, but may be appropriate in some circumstances.
Induction of puberty using testosterone esters Increasing dose schedule every 6 months: 50 mg monthly i.m. Increased approximately every 6 months Adult dose Sustanon 250 per 3–4 weeks Imperfect – first pass metabolism and profile over month not good. Gel seem an ideal alternative – but I am not aware of specialist centres using as yet..... Inducing puberty with testosterone rather than hCG does not appear adversely affect fertility.
And for fertility Most patients with IHH and KS require a combination of hCG and FSH to stimulate sperm production. The starting dose for hCG is 1000 IU, and FSH is 75-150 IU alternate days Dosage adjusted based on trough T level, testicular growth, sperm production, and avoidance of adverse effects (monitored every 3 months) Gynecomastia common - 30%
Monitor every 3 months until an adequate level of replacement is documented. Pregnancy has occurred with counts as low as 2.5 X 106, but 20-40 X 106/mL produces higher pregnancy rates. Median time to induction of spermatogenesis is 6-8 months – may take 2 years. Remember to cryopreserve sperm should fertility be achieved. Safer and cheaper to than IVF for couple.