Download - Induction of puberty in adult endocrinology
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Induction of Puberty in Adult Endocrinology:What do I need to know?
Kate Lissett
Torbay Hospital
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Induction of puberty in adults
• Who• When• How• Hazards
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Who
• Which doctor?• Which patient?
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• How many of you have been involved in taking patients through puberty.........
• Can anyone give a brief case history......
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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.
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• 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
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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 )
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What is normal
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Stages of Pubertal Development
Pubertal Sequence
Accelerated growth→breast development→adrenarche→menarche
• requires 4.5 years (range 1.5 to 6 years)
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Signs of Puberty: Tanner Staging
Stage 1 (Prepubertal)
Elevation of papilla only. No pubic hair.
Stage 2 Elevation of breast and papilla as small mound, areola diameter enlarged. Median age: 9.8
Sparse, long, pigmented hair, along labia majora. Median age: 10.5
Stage 3 Further enlargement without separation of breast and areola. Median age: 11.2
Dark, course, curled hair sparsely spread over mons. Median age: 11.4
Stage 4 Secondary mound of areola and papilla above the breast. Median age: 12.1
Adult type hair, abundant but limited to mons. Median age: 12
Stage 5 Recession of areola to contour of breast. Median age: 14.6
Adult type spread in quantity and distribution. Median age: 13.7
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Tanner Staging
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How....
• What estrogen• At what dose• How would you monitor
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Be aware
• Emotional /psychological as well as physical impact
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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
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Men
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Do you have any case histories
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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
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What are the aims of treatment
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What options do you have in this man to induce puberty?
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• 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.
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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.
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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%
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• 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.