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Early and late puberty. Tim Cheetham January 2011. 1. Normal physiology. Adrenal . Gonad . Steroid producing tissues. Adrenal glands Ovaries . Androgen. Oestrogen Androgen. Peripheral tissue. Oestrogen. Do men make oestrogen? Do women make testosterone?.
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Early and late puberty Tim Cheetham January 2011
1. Normal physiology Adrenal Gonad
Steroid producing tissues Adrenal glands Ovaries Androgen Oestrogen Androgen Peripheral tissue Oestrogen
Do men make oestrogen? • Do women make testosterone?
Gn production in boys Gn 2 9 Age
Normal physiology What next?
Adrenarche • Body odour • Greasy hair • Acne • Pubic hair Pre-puberty
cholesterol Adrenal • Adrenarche A C Weak Androgen
cholesterol Adrenal • Adrenarche Weak androgens A C Weak Androgen
7 year old • Body odour • Greasy hair • 2 or 3 pubic hairs Adrenal • Adrenarche Body odour Pubic Hair
What next? pituitary LH, FSH adrenal gonad • Adrenarche Body odour Pubic Hair Girls - Bust development Boys - Testicular enlargement
Puberty ♀: Growth spurt 2 years before boys, at start of clinical puberty Peak height velocity ~12 years Followed by menarche ♂: Growth spurt when puberty already well established (testicular volume ~ 10 mls) Peak height velocity ~14 years
2. ‘Early puberty’ • Bust development in the very young child • Early pubic hair • Precocious puberty
Isolated premature thelarche Gn Bust tissue 2 9 Age
Adrenarche Body odour Pubic Hair Acne
Adrenarche More pronounced or early if: • Obese • SGA • History of PCOS
cholesterol • Adrenarche • CAH • Adrenal tumour Weak androgens A C Body odour Pubic Hair Acne
cholesterol • Adrenarche • CAH • Adrenal tumour Androgens A C Body odour Pubic Hair Acne
Investigations? • Nothing • Morning 17-OHP and testosterone
Obesity • Promotes growth (height) in early life • Associated with an earlier onset of puberty Hence the Paediatricians interest in the short, heavy child
True precocious puberty • Bust development < 8 years in girls • Testicular enlargement < 9 years in boys
Early puberty: Idiopathic – girls CNS lesion – boys LH, FSH Bust development Testicular enlargement
Gonadotrophin independent Bust development Testicular enlargement
TSH - hypothyroidism Bust development Testicular enlargement
Case 1: Jordan Age 20 months Pubic hair ‘Large testes’ Tall Healthy non-consanguinous parents
Examination Height and weight 75th centile Penile length +2 SD Testicular volume 3 mls Pubic hair stage 1
Investigations Time (min)LH (U/L)FSH(IU/L) 0 <1 <1 30 2.1 <1 60 1.4 <1 Urine steroid profile – normal 17 OHP – 1.3 nmol/L Testosterone < 1nmol/l
Jordan 3.2 years Increase in size of genitalia Temper tantrums Testes 4-5 mls Penile length 7 cm PH stage 2 Concerns about gait
Investigations Time (min)LH (U/L)FSH(IU/L) 0 2.9 2.5 30 22.8 4.4 60 19.7 4.4 Testosterone 11.2 nmol/L MRI brain • No intra-cranial abnormality shown. • No mass lesion shown in the pituitary fossa nor in the hypo-thalamic region. • There is a little asymmetry in the lateral ventricles just above the foramen of Monro but there is no structural abnormality to account for this.
Jordan Diagnosis – ‘Idiopathic’ GDPP’ Started on Leuprorelin acetate injections
Jordan – 6 years Ongoing concerns about gait Plan • Neurodevelopmental assessment • Repeat MRI
JH – high signal in the white matter In keeping with perinatal ischaemic injury
Precocious puberty and CNS lesions Abnormal (enhanced) gonadotrophin production can commence at a very early age
3. Delayed puberty ~ 14 years in girls ~ 15 years in boys
Delayed puberty Scenario 1 LH, FSH
Delayed puberty Scenario 1 LH, FSH Causes 1. Late 2. Chronic illness 3. Endocrinopathy eg prolactinoma tumour Gn deficiency
Delayed puberty Scenario 2 LH, FSH
Delayed puberty Scenario 2 LH, FSH • Causes • Ovarian pathology • Abnormal karyotype