What is precocious puberty?
The onset of secondary sexual characteristics before 8 in a female or 9 in a male
OR
The onset of menstruation before 9 in a female
What is significant about particularly early onset of puberty?
The earlier the onset the more likely the chance there is of a pathological cause
What is the difference between central precocious puberty and peripheral prococious puberty?
- Central - This follows concordance and is to do with the hypothalamic/pituatry/gonadal axis
- Peripheral - This may or may not follow concordance and is to do with an input from outside of this axis, eg the adrenals
What hormone profile would you see with a central reason for precocious puberty?
gonadal- Low FSH/LH. High oest/Test
central- High FSH/LH, High Test
What physical examinations would you do?
- BMI
- Tanner score
- Growth Chart
- Skin lesions
What is Mccure Albrights syndrome?
Over production of all endocrine systems causing variable symptoms
Often get short metacarpals (bone dysplasia) and cafe au lait spots (small is neurofibrotoma)
What do cafe au lait spots signify?
If they are large and unilateral with a jagged edge it may be McCure Albrights
If they are small in may be a neurofibrotoma
What investigations would you do?
MRI
bone age
hormone profile
What happens in premature thelarche?
What is normal therlarche?
- Premature growth of breast tissue.
- Normal hormone levels
- Benign condition
Normal thelarche is the growth of breast tissue
What happens in Adrenarche?
- Early release of adrenal androgens causin axillary hair growth, spots, odor but no testicle/breast growth
- 17hydroxyprogesterone is normal ?CAH
- This is normal up to a year before gonadarche
What happens in central precocious puberty, including the investigations
Consonance of puberty before 8 in females and 9 in males
Increase in FSH/LH, sex hormones, bone age
All other investigations are normal
Would would happen to the endometrial strip in central precocious puberty?
It would be developed
What is the treament for central precocious puberty?
GNRH analogues
They stop the release of FSH/LH via negative feedback.
They are given to a more appropriate age for puberty
What is a Hypothalamic Harmotoma and what is a common but unusual symptom?
- A hypothalamic tumour causing an increase in GNRH
- Laughing
What happens in peripheral precocious puberty, including the hormone profile
- Onset may or may not be concordant
- FSH/LH will be low, even when stimulated
What is congenital adrenal hyperplasia and how is it tested?
- This is a lack of 21 hydroxylase meaning that less aldosterone and cortisol are formed, but more sex hormones are formed. It is testing for my measuring levels of 17 hydroxyprogesterone which will be high.
- Salt wasting - no aldosterone so low sodium and dehydration and FTT
- Virilising - non corcordant precocious puberty - virilisation, clitoromegaly, ambiguous genitalia, penis growth, infertility
- Treatment - Fludrocortisone for salt wasting and hydrocortisone low low cortisol
What is the significance of testicle size when investigating precocious puberty?
Both enlarged (high FSH/LH) - Central process
Not enlarged (Low FSH/LH) - peripheral process
one enlarged (Low FSH/LH, high TEST) - ?tumour
