2 - Gynae - Menstrual Cycle and its disorders - Precocious puberty + Ambiguous development and Intersex Flashcards
define precocious pub?
when is growth spurt? effect on final height? trt aims?
menstruation <10y/other 2’ SCs <8
early - final height reduced
arrest sexual dev and allow normal growth
precocious puberty - mainstay of treatment? this medication causes what? what are the two general types of causes?
GnRH agonists
-inhibit sex hormone secretion and cause regression of 2’ SCs and cessation of menstruation
central causes and ovarian/adrenal causes
central causes of precocious puberty
incr GnRH secretion:
-meningitis, encephalitis, CNS tumours, hydrocephaly, hypothyroidism
-may all prevent normal prepubertal inhibition of GnRH release
Ovarian/adrenal causes of precocious puberty - and mgmt?
incr oestrogen secretion:
- hormone prod tumours of ovary/adrenals
- mccune albright syndrome
remove tumour
Or
cyproterone acetate (antiandrogenic progestogen)
Ambiguous dev and intersex - 2 main reasons genitalia may be ambiguous?
incr androgen fx in a genetic female
red androgen fx in a genetic male
Incr androgen fx in genetic female - what is recessively inherited? what is defective? what does this cause?
CAH
defective cortisol prod
ACTH excess > incr androgen prod
Incr androgen fx in genetic female - when might they present? and with what? trt?
at birth - ambig genitalia
puberty - enlarged clit and amenorrhea
trt - cortisol and mineralocorticoid replacement
Red androgen fx in genetic male - male has cell receptor insensitivity - name? what happens to androgen? how does individual appear?
appears female
androgen insensitivity syndrome
they convert peripherally to oestrogens
Red androgen fx in genetic male - only diagnosed when..? what is absent and what is present? mgmt?
when presx with amenorrhea
uterus absent and rudimentary testes present
remove testes due to Ca risk
oestrogen HRT started
