Precocious Puberty

Question 1

What is precocious puberty?

Answer 1

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

Question 2

What is significant about particularly early onset of puberty?

Answer 2

The earlier the onset the more likely the chance there is of a pathological cause

Question 3

What is the difference between central precocious puberty and peripheral prococious puberty?

Answer 3

• 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

Question 4

What hormone profile would you see with a central reason for precocious puberty?

Answer 4

gonadal- Low FSH/LH. High oest/Test

central- High FSH/LH, High Test

Question 5

What physical examinations would you do?

Answer 5

• BMI
• Tanner score
• Growth Chart
• Skin lesions

Question 6

What is Mccure Albrights syndrome?

Answer 6

Over production of all endocrine systems causing variable symptoms

Often get short metacarpals (bone dysplasia) and cafe au lait spots (small is neurofibrotoma)

Question 7

What do cafe au lait spots signify?

Answer 7

If they are large and unilateral with a jagged edge it may be McCure Albrights

If they are small in may be a neurofibrotoma

Question 8

What investigations would you do?

Answer 8

MRI

bone age

hormone profile

Question 9

What happens in premature thelarche?

What is normal therlarche?

Answer 9

• Premature growth of breast tissue.
• Normal hormone levels
• Benign condition

Normal thelarche is the growth of breast tissue

Question 10

What happens in Adrenarche?

Answer 10

• 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

Question 11

What happens in central precocious puberty, including the investigations

Answer 11

Consonance of puberty before 8 in females and 9 in males

Increase in FSH/LH, sex hormones, bone age

All other investigations are normal

Question 12

Would would happen to the endometrial strip in central precocious puberty?

Answer 12

It would be developed

Question 13

What is the treament for central precocious puberty?

Answer 13

GNRH analogues

They stop the release of FSH/LH via negative feedback.

They are given to a more appropriate age for puberty

Question 14

What is a Hypothalamic Harmotoma and what is a common but unusual symptom?

Answer 14

• A hypothalamic tumour causing an increase in GNRH
• Laughing

Question 15

What happens in peripheral precocious puberty, including the hormone profile

Answer 15

• Onset may or may not be concordant
• FSH/LH will be low, even when stimulated

Question 16

What is congenital adrenal hyperplasia and how is it tested?

Answer 16

• 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

Question 17

What is the significance of testicle size when investigating precocious puberty?

Answer 17

Both enlarged (high FSH/LH) - Central process

Not enlarged (Low FSH/LH) - peripheral process

one enlarged (Low FSH/LH, high TEST) - ?tumour