Precocious Puberty Flashcards
definition of precocious puberty
premature appearance of secondary sexual characteristics;
girls <8 years
boys <9 years
- Hypothalamic (GnRH) independent (__)
- True precocious puberty (__) is GnRH dependent
- Hypothalamic (GnRH) independent (Peripheral)
- True precocious puberty (Central) GnRH dependent
variations of normal puberty
- Premature thelarche
- Premature adrenarche
what is premature thelarche
breast development in girls under 3 years, due to transient ovarian cyst.
how does bone age get affected by premature thalrche
bone age= chronological age. no significant advancemnet
how is vaginal growth affected by premature thelarche
there is no growth of labia minora
premature adrenarche definition
premature adrenal androgen secretion – usually due to pubic hair , NO CLITOROMEGALY OR breast enlargement
in terms of physical exam how could you tell between premature adrenarche and thelarche
thelarche is breast development in girls <2
adrnarche is not characterized by breast development or clitoromegaly. its more about pubic hair
outline causes of GnRH independent causes precocious puberty
if its gnrh independent, the problem is at the level of the gonads
Familial Male Limited Gonadotropin Independent Precocious Puberty
How does bone age change in Congenital Adrenal Hyperplasia
• Bone age >> Chronological age
key dermatologic feature of mcCUne Albright syndrome. What happens to sexual characteristics in this disease?
Cafe au lait lesions. Sexual precocity occurs.
- may have polyostotic fibrous dysplasia
endocrinopathies associated with mcCune albright syndrome
pituitary adenomas
curshing syndrome
hyperthyroidism
Causes of GnRH dependent (central) precocious puberty
- idiopathic
- CNS tumors
- Meningitis
- trauma
- hydrocephalus
Idiopathic precocious puberty– how does it affect bone age?
bone age >>CA.
neurogenic precocious puberty is often because of a history of underlying __ ____.
How does it affect bone age?
there is a history of underlying CNS insult. BA>>>CA
t/f in most instances of precocious puberty, bone age is > chronological age
true.
most of the time they are tall as children and then they have a short adult stature
investigations to consider in a kid presenting as taller than their age and looking liek theyre going through puberty way too young.
A kid presents with precocious puberty symptoms, you do a bone age and it’s normal. what do you do?
observe and reassess in 3-6 months. plus do other tests like adrenal androgen test, serum beta HCG, GnRH stim test etc.
You do a gnRH stim test on a kid going through what you think is precocious puberty:
- what if the GnRH stim test produces a blunted response (modest LH rise)?
- what if GnRH stim test produces a significant LH rise?
modest/blunted LH rise: not going through actual puberty. the CNS inhibition of hypothalamic-pituitary axis is still intact and is blockin the exogenous GnRh stimulation from producing pituitary hormone.
- it is GONADOTROPIN INDEPENDENT– the problem is at the level of the pituitayr or the gonads itself.
significant LH rise: it is a central issue. it is gonadotropin dependent and a sign of “true precocious puberty”– it could be due to an organic CNS lesion or idiopathic
general management for GnRH independent andGnRH dependent prevovious puberty
You are seeing a 16 year old boy with anosmia and
delayed puberty. You order a GnRH stimulation test
(giving iv GNRH and measuring LH and FSH). You
would expect to see:
• A. A normal rise in the LH
- B. A rise in the LH initially followed by a drop
- C. A drop in LH followed by a rise
- D. No rise in the LH
D. Gonadotropin deficiency. Produces dubnormal LH response to HnRH stimulation
• You are seeing a 16 year old girl with a history
chemotherapy for treatment of leukemia as a young
child. She reports no pubertal development at all.
You would expect to see:
• A. Low estradiol levels
• B. Tanner stage 2 breast development •
C. Elevated LH and FSH
D. Both A and C
D– A and C
• You are seeing a 3 year old boy with a history of
familial testotoxicosis and signs of precocious
puberty. You would expect to see:
• A. Elevated testosterone levels
- B. Tanner stage 1 pubic hair
- C. GnRH- dependent precocious puberty
- D. Both A and C
A.
testotoxicosis is not a central issue.– it is considered a GnRH independent precocious puberty.
You are seeing a 4 year old girl with an onset of breast
development and pubic hair. A GnRH test shows
pubertal levels of LH and FSH. Her MRI of the head is
normal. You opt to start therapy with a GnRH analogue.
One year after therapy, you would expect:
• A. Elevated estradiol levels
- B. Progression of her bone age
- C. Flat LH and FSH after GnRH analogue therapy
- D. Both A and C
C. giving consistent lupron inhibits the pulsatality so reduces LH and FASH
