precocious puberty, delayed puberty Flashcards
what is precocious puberty
onset of secondary sexual characteristics before the age of 8 years in girls ( breast development) and 9 years in boys (testicular enlargment)
classificaion
Boys: McCune-Albright syndrome or estrogen-secreting leydig cell tumor
central (true precosious)
-d/2 early releases of GnRH
peripheral
-independent of GnRH
isosexual
-prematrue sexual development for the app gender
heterosexual
-premature sex deveopment for opposite gender
what is McCune Albright syndrome
Boys: McCune-Albright syndrome, estrogen-secreting leydig cell tumor
Girls: congenital adrenal hyperplasia
presentation of precoocious puberty
early development of all pubertal features in CENTRAL w/ normal order
can be isolated presentation of one of the stages of puberty e.g isolated premature menarhce, adrenarche
accelereted growth
sx of associated diseases e.g. cafe au lait spots in McCube Albright syndrome
what is central precoious puberty
precocious puberty w/ elevated GnRH
etio of central precocious (5)
- Idiopathic (most cases)
- CNS lesions
- Pituitary gonadotropin-secreting tumors (rare)
- Systemic conditions: #tuberous sclerosis, #neurofibromatosis
- Obesity-related precocious sexual development due to increased levels of leptin in obesity
PP of central precocious puberty
premature activation of the hypothalamo-hypophyseal axis → abnormally early initiation of pubertal changes → early development of secondary sexual characteristics and gonadarche
dg of central preciosious puberbty
LAB -increased LH/FSH -GOLD: GnRH stim test #LH/FSH increase after iv gnrh admin -increased testosterone/ estradiol
IMAGING
- mri/ct to visualise ct tumores
- xray of elft hand to check for accelerated skeletal growth
rx of precoious puberty
GnRH agonist causing super stim desesnsitizing receptors in the pw and reducing fh/lsh secretion
#buserelin #leuprolidee
what is peripheral precocious puberty
precocious puberty without elevated GnRH levels
causes of peripheral pre pub
increased androgen production
- ovarian cyst most common
- congenital adrenal hyperplasia
- leydig-cell tummor
increased estrogen production
- hcg germcell tumors(GRANULOSA CELL TUMOR)
- Mcune albright sx
primary hypothyroidism
obesity related d/2 hyperinsulinemia
dg of peripheral pre pub
LAB
- increased testosterone. estogen
- reduced FSH/LH & GnRH d/2 suppression of gnrh by test/estrogen
- test TSH for HYOTHYROIDISM
- test BHCG
IMAGING
-US of pelvis/ abdomen/ abdomen to check for tumors
rx of pre puberty
surgical removal of hormone producing tumor
no intervention for ovarian cysts
obesity related precosioius puberty
increased food intake.
increased blood glucose
hyperinsulenemia and leption
letptin stims GnRH
delayed puberty
absent /incomplete development of secondary sex characteristics by the age of
13 in girls
14 in boys or
etiology of delayed pub
constititional growth delay (common)
malnutriotion
hypogonadism
what is constitutional growth delay
temporary delay in growth and onset of puberty that is not caused by any pathological process
usually genetic
dg by xray showing the age of a bone thats younger than the aage of the pt
no rx needed
dg of delayed pub
monitoring
hand xray
basal FH/LSH LEPTIN and TSH
rx of delayed puberty
rx underyling disease
monitor measurments every 6 months
gonadal steroids in severe cases
