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- Resistant ovary syndrome
- Delayed menarche
- Hyperprolactinemia ( see other
Testicular feminization syndromegenetic males 46,XY with normal female external genitalia (raised as girls) Mechanism: resistance of target tissues to androgens Findings: external genitals female by default / no internal female organs (vagina ends in blind pouch) / endogenous E stimulates normal breast development at puberty Diagnosis: usu. when menarche fails to occur or testes felt in abdomen Treatment: remove testes, which have malignant potential / give supplemental E to maintain female secondary sex characteristics Resistant ovary syndromeovaries cannot respond to gonadotropins (can result from autoimmune destruction of ovaries or other conditions) Hypogonadotropic hypogonadismPanhypopituitarism causes primary or secondary amenorrhea (depending on time of onset) / from destructive lesions in HPA regions Isolated gonadotropin deficiency usu. from defective GnRH production, Kallman’s is a specific form associated with anosmia) Delayed menarcheConsider if no periods by age 16 / sometimes delay runs in family / one can prescribe E for 6 months or so even prior to making diagnosis in order to assist sexual/psychological development Hypothalamic amenorrhea (psychogenic, functional, and idiopathic) most common nonphysiologic secondary amenorrhea / LH/FSH is low or normal Treatment: if GnRH is infused in pulsatile fashion, this may correct all problems Malnutrition/Anorexia Menstruation often ceases below critical body weight Exercise (common in serious athletes) ? Hypothyroidism “Post-pill amenorrhea” delay > 6 months since stopping pill / only occurs in 1% / must r/o other causes Primary ovarian failure (premature menopause) similar to normal menopause but < 40 yrs / some cases have auto-Ab’s ovarian function declines, estrogen levels decrease with compensatory ↑LH/FSH
may inhibit normal menstrual cycles with excessive E Hyperprolactinemia (see other)excess prolactin is a very common cause of secondary amenorrhea Androgen excess syndromesPolycystic Ovary Syndrome (PCOS or Stein-Leventhal) chronic lack of ovulation, androgen excess, obesity / unknown etiology 3%-7% of reproductive-age women
ovary excess androgenic steroids (esp. androstenedione, converted to estrone) increased estrone has (+) feedback on LH and (-) on FSH increased LH hyperplasia of ovarian theca/stroma increased androgen production lack of follicle maturation from decreased FSH and ovarian androstenedione Note: obesity may increase sex hormone levels by decreasing SHBG and increases peripheral conversion of androstenedione Pathology: ovaries enlarged with thickened capsules and many small follicular cysts Theories: HPA axis, with constant LH vs. excess ovarian secretion of androgen vs. adrenal abnormalities Presentation: infertility and menstrual abnormalities (amenorrhea or oligomenorrhea) from chronic anovulation / prolonged, noncyclic, unopposed E may cause functional bleeding and increase risk of endometrial Ca androgen excess oily skin, acne, hirsutism obesity in 40% insulin resistance // 30% of girls with PCOS have glucose intolerance; 10% chance of diabetes Labs: ↑ LH to FSH ratio (~2) (LH elevated while FSH low-normal) testosterone and androstenedione usually elevated (adrenal androgens, DHEA and DHEA-S, are found less often) estrone usually high, estradiol normal Treatment of androgen excess: use trial and error vs. testing for suppression of androgen levels / usually takes 3-6 months to see improvement in hirsutism oral contraceptives (E-P) decrease androgen levels by negative feedback on LH and increased hepatic production of SHBG (although sometimes not high enough levels) spironolactone decreases ovarian and adrenal synthesis of androgens and inhibits androgen binding to receptors in hair follicles and other target tissues / 100 mg QD or BID often effective steroids decrease adrenal androgen production by suppressing ACTH (may also lower ovarian androgen secretion, although the mechanism is unknown / 0.5 mg dexamethasone q HS (because ACTH peaks in early morning) metformin (effective in ½, mechanism thought to be related to finding that hyperinsulinemia synergizes with LH to increase thecal androgen production and decrease sex hormone-binding globulin) / treatment with metformin can lead to clinical improvement, even in adolescents without overt diabetes mellitus Infertility Clomiphene citrate stimulates LH/FSH production / given 5th to 9th day following P-induced menstruation / 80% effective Human menopausal gonadotropin has both FSH and LH bioactivity / injected daily until increasing serum E and u/s confirm follicle maturation / hCG then injected to induce ovulation / this therapy increases risk of multiple gestation GnRH (given IV or SC in pulses q 90-120 mins may induce ovulation without causing ovarian hyperstimulation MPA to prevent endometrial CA / 10 mg MPA for 10 days q 1-3 months Download 3.95 Mb. Share with your friends:
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