precocious puberty, delayed puberty

Question 1

what is precocious puberty

Answer 1

onset of secondary sexual characteristics before the age of 8 years in girls ( breast development) and 9 years in boys (testicular enlargment)

Question 2

classificaion

Answer 2

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

Question 3

what is McCune Albright syndrome

Answer 3

Boys: McCune-Albright syndrome, estrogen-secreting leydig cell tumor
Girls: congenital adrenal hyperplasia

Question 4

presentation of precoocious puberty

Answer 4

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

Question 5

what is central precoious puberty

Answer 5

precocious puberty w/ elevated GnRH

Question 6

etio of central precocious (5)

Answer 6

1. Idiopathic (most cases)
2. CNS lesions
3. Pituitary gonadotropin-secreting tumors (rare)
4. Systemic conditions: #tuberous sclerosis, #neurofibromatosis
5. Obesity-related precocious sexual development due to increased levels of leptin in obesity

Question 7

PP of central precocious puberty

Answer 7

premature activation of the hypothalamo-hypophyseal axis → abnormally early initiation of pubertal changes → early development of secondary sexual characteristics and gonadarche

Question 8

dg of central preciosious puberbty

Answer 8

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

Question 9

rx of precoious puberty

Answer 9

GnRH agonist causing super stim desesnsitizing receptors in the pw and reducing fh/lsh secretion

#buserelin
#leuprolidee

Question 10

what is peripheral precocious puberty

Answer 10

precocious puberty without elevated GnRH levels

Question 11

causes of peripheral pre pub

Answer 11

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

Question 12

dg of peripheral pre pub

Answer 12

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

Question 13

rx of pre puberty

Answer 13

surgical removal of hormone producing tumor

no intervention for ovarian cysts

Question 14

obesity related precosioius puberty

Answer 14

increased food intake.
increased blood glucose

hyperinsulenemia and leption

letptin stims GnRH

Question 15

delayed puberty

Answer 15

absent /incomplete development of secondary sex characteristics by the age of
13 in girls
14 in boys or

Question 16

etiology of delayed pub

Answer 16

constititional growth delay (common)

malnutriotion

hypogonadism

Question 17

what is constitutional growth delay

Answer 17

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

Question 18

dg of delayed pub

Answer 18

monitoring

hand xray

basal FH/LSH LEPTIN and TSH

Question 19

rx of delayed puberty

Answer 19

rx underyling disease

monitor measurments every 6 months

gonadal steroids in severe cases