Wednesday, 3-1-Puberty & disorders of development and Menstrual disorders (Moulton) Flashcards
1) as the corpus luteum dies, ___ levels fall –> 2) The pituitary responds to falling levels of #1 by increasing __ secretion –> 3) FSH recruits a cohort of large antral follicles to enter rapid growth phase. Follicles secrete large amounts of __ –> 4) The substances secreted by the follicles in #3 negatively feedback on FSH –> 5) declining FSH levels progressively cause atresia of all but 1 follicle-leading to selection of dominant follicle, which produces high levels of ___ –> 6) High estrogen has positive feedback on gonadotropes and leads to a __ surge –> 7) the surge in #6 induces meiotic maturation, ovulation, and luteinization. The corpus luteum produces high amounts of ___. –> 8) High levels of the substances produced by the corpus luteum in #7 feedback on __ to return to basal levels. –> 9) the __ progressively becomes less sensitive to basal LH and dies if levels of LH-like activity (i.e., hCG) do not increase
1) Estrogen and progesterone
2) FSH
3) Estrogen and inhibin
5) Estrogen
6) LH (and some FSH)
7) Progesterone, along with Estrogen and inhibin
8) LH and FSH
9) corpus luteum
which endometrial zone sloughs off at menstruation and contains spiral arteries?
functionalis (outer portion)
median age of menarche? when does it usually occur after thelarche (breast budding)? which tanner stage?
12.43
occurs within 2-3 yrs after thelarche
at tanner stage IV, rare before stage III
__-no menstruation by 13 years old without secondary sexual development OR by the age of 15 yrs with secodnary sexual characteristics
Primary amenorrhea
typical menstrual cycle interval (in days) in young females?
21-45 days
Mean blood loss per menstrual cycle is __ cc.
Greater than __ cc blood loss has been associated with anemia
30
80 –> changing a pad q1-2 hrs considered excessive esp if bleeding >7 days
rises in adrenal androgens in late childhood (8-11) causes growth of ___
axillary and pubic hair (adrenarche or pubarche)
between age 8-11 –> increase [DHEA] and [androstenedione]
__ is known as breast development and 1st physical sign of puberty. It requires estrogen
thelarche
pubarche/adrenarche requires ___
androgens
which demographic experiences the earliest age of thelarche? adrenarche? menarche?
AA’s start the earliest, followed by hispanics, then whites in all 3 categories
this tanner stage of breast development is preadolescent, elevation of papilla only
1
this tanner stage of breast development is the breast bud stage, elevation of breast and papilla as a small mound with enlargement of areolar region
2
this tanner stage of breast development is further enlargement of breast and areola without separation of their contours
3
this tanner stage of breast development is a projection of areola and papilla to form a secondary mound above level of the breast
4
this tanner stage of breast development is the mature stage where you have projection of the papilla only, resulting from recession of the areola to the general countour of the breast
5
this tanner stage of pubarche is preadolescent, absence of pubic hair
1
this tanner stage of pubarche is sparse hair along labia, hair downy with slight pigment
2
this tanner stage of pubarche is hair spreading sparsely over junction of the pubes, hair is darker and coarser
3
this tanner stage of pubarche is adult-type hair, no spread to medial surface of thighs
4
this tanner stage of pubarche is adult-type hair with spread to medial thighs assuming an inverted triangle pattern
5
this heterosexual precocity disorder is exceedingly rare in childhood, usually originate in the ovaries (sertoli-leydig cell) or adrenals. It is dx by physical and radiologic exams and tx with surgical removal. It is characterized by development of secondary sex characteristics OPPOSITE those of anticipated sex
androgen-secreting neoplasms
this heterosexual precocity disorder is most commonly from defect in 21-hydroxylase leading to excessive androgen production. There is a classical and non-classical form
Congenital Adrenal Hyperplasia
what is the term for premature sexual maturation that is appropriate for the phenotype of the affected individual?
isosexual precocious puberty
true–> arises from premature activation of normal process of puebrtal development involving the HPO axis
pseudoisosexual–> increase in estrogen and cause sexual characteristic maturation WITHOUT activation of HPO axis
how do you dx true isosexual precocious puberty?
how do you tx?
Dx with administrtion of exogenous GnRH and see resultant rise in LH levels consistent with older girls who are undergoing normal puberty
Dx with MRI of head
Tx with GnRH agonist (Leuprolide) –> suppresses release of FSH and LH
this pseudoisosexual precocity disorder involves a somatic mutation during embryogenesis, causes multiple cyctic bone defects, cafe au lait spots (face, neck, shoulder and back), and adrenal hypercortisolism
McCune-albright syndrome (Polyostotic fibrous dysplasia)
this pseudoisosexual precocity disorder is associated with a sex cord tumor that secrretes estrogen, GI polyposis and mucocutaneous pigmentation
Peutz-Jeghers syndrome
regarding delayed puberty, considered delayed when:
secondary sex characteristics have not appeared by age __
if thelarche has not occured by age __
no menarche by age of __
when menses has not begun __ years after onset of thelarche
13
14
15-16
5
regarding delayed puberty, hypergonadotropic hypogonadism is known as ___
turner syndrome (gonadal dysgenesis)
FSH > 30
regarding delayed puberty, Kallman syndrome, anorexia/extreme exercise, pituitary tumors/disorders, hyperprolactinemia, and drug use make up this category of “-gonadism”
Hypogonadotropic hypogonadism
FSH + LH <10
Name the disorder based on the exam findings:
Hx and PE for pt with primary amenorrhea –> No secondary sex characteristics present –> FSH and LH < 5 IU per L –> ?
Hypogonadotropic Hypogonadism
slide 56
this form of hypogonadtropic hypogonadism is d/t mutation of KAL gene on X chr, pts have anosmia or hyposmia
Kallman syndrome
Name the disorder based on exam findings:
Hx and PE for pt with primary amenorrhea –> No secondary sex characteristics present –> FSH > 20 IU per L and LH >40 IU per L –> 46XX –> ?
45XO –>
Hypergonadotropic hypogonadism
46xx– premature ovarian failure
45XO–> turners
this is the most common form of female gonadal dysgenesis, majority show no signs of secondary sex characteristics. get webbing of neck, broad flat chest like a shield, wide-spaced nipples, short, streak ovaries, absent or incomplete development of puberty, coarctation of aorta
Turners –> 45XO
name the disorder based on exam findings:
Hx and PE for pt with primary amenorrhea –> Secondary sex characteristics ARE PRESENT –> US of uterus reveals abnormal or absent uterus –> 46 XY –> ?
46XX –> ?
46XY–> androgen insensitivity syndrome
46XX–> Mullerian agenesis
this primary amenorrhea disorder with secondary sex characteritics has a 46XY karyotype, male levels of testosterone, defect in androgen receptor, and testes in abdominal wall. No uterus is formed, have external female genitalia, w/absent to sparse pubic hair
Androgen Insensitivity Syndrome (AIS)
this primary amenorrhea disorder with secondary sex characteristics is characterized by primary amenorrhea, breast development, levels of testosterone consistent with females and a 46XX karyotype. Can have an imperforate hymen or transverse septum or absence of normal uterus (mullerian agenesis AKA Meyer-Rokitansky-Kuster-Hauser Law firm)
Mullerian dysgenesis or agenesis
__ is the most common cause of primary amenorrhea in women with normal breast development
Mayer-Rokitansky-kuster-hauser syndrome (mullerian agenesis)
name the disorder based on exam findings:
hx and PE for pt with primary amenorrhea –> secondary sex characteristics ARE PRESENT –> Uterus is present or normal on US –> There IS an outflow obstruction –> ?
Imperforate hymen or transverse vaginal septum
correct imperforate hymen –> hymenectomy
correct transverse vag septum –> surgery
__ is defined as absence of menstruation x6 months
secondary amenorrhea
hx & PE findings in secondayr amenorrhea?
Labs?
hx and PE: weight changes, strenuous exercise, dietary habits, concomitant ilness, abnormal facial hair, galactorrhea, dyspareunia, hot flashes and/or night sweats
labs: urine hCG, TSH, prolactin, FSH
pt with secondary amenorrhea who has normal prolactin and abnormal TSH?
thyroid disease
pt with secondary amenorrhea who has really high prolactin (>100) should be evaluated for ___.
if high prolactin but <100, how do you evaluate?
really high (>100)=evaluate for prolactinoma with head MRI –> empty sella syndrome, pituitary adenoma
high (<100)=if MRI neg., consider other causes
tx focus for pt with secondary amenorrhea who has microadenomas found on MRI?
manage infertility, galactorrhea, and breast discomfort
consider dopamine agonist (bromocriptine, cabergoline)
tx focus for pt with secondary amenorrhea who has macroadenomas found on MRI?
dopamine agonists
transsphrenoidal resection or craniotomy (most avoid surgery 1st)
name the disorder based on exam findings:
pt with secondary amenorrhea –> normal TSH and prolactin –> withdrawal bleed after progesterone challenge test –> ?
what if no withdrawal bleeding after progesterone challenge test?
Normogonadotropic hypogonadism –> PCOS MOST COMMON
negative PCT –> indicate inadequate estrogenization or outflow tract abnormality
which ovarian disorder is likely to cause a normogonadotropic amenorrhea with hyperandrogenism?
PCOS
__ is the leading cause of female anovulatory infertility. 60-70% of these pts have insulin sensitivity, have elevated insulin and androgen levels which reduce the hepatic production of SHBG –> leading to increase in circulating testosterone
PCOS
how do you dx PCOS?
need 2 of the 3
- oligomenorrhea or amenorrhea
- BCHEM or clinical signs of hyperandrogenism–> LH:FSH of 2:1
- US revealing multiple small cysts beneath cortex of ovary
tx for PCOS?
- weight loss
- OCs
- clomiphene citrate
- ovarian diathermy/laser tx
- spironolactone
- insulin sensitizing agents-biguanides (metformin)
name the disorder based on exam findings:
pt with secondary amenorrhea –> normal TSH and prolactin –> PCT negative, Estrogen PCT positive (withdrawal bleeding) –> FSH > 20 IU and LH >40 IU –> ?
what are causes of this disorder?
Hypergonadotropic hypogonadism
causeds: ovarian injury from surgery, pelvic radiation, chemo, carrier status of fragile X, autoimmune, and mumps
name the disorder based on exam findings:
pt with secondary amenorrhea –> normal TSH and prolactin –> PCT positive (withdrawal bleed) –> FSH and LH <5 IU –> Normal MRI –> ?
what are causes of this disorder?
Hypogonadotropic hypogonadism
causes: anorexia or bullemia nervosa, chronic illness, cranial radiation, excessive exercise, malnutrition/excessive weight loss, Sheehan’s syndrome
__-abnormally frequent menses at intervals <21 days
polymenorrhea
__-excessive and/or prolonged bleeding (>80 mL and >7 days) occurring at normal intervals
menorrhagia (hypermenorhea)
__-irregular episodes of uterine bleeding
metrorrhagia
___-heavy and irregular uterine bleeding
menometrorrhagia
__-scant bleeding at ovulation for 1 or 2 days
intermenstrual bleeding
__-menstrual cycles occuring > 35 days but less than 6 months
oligomenorrhea
what are structural causes of abnormal bleeding in reproductive-aged women?
PALM Polyp Adenomyosis Leiomyoma Malignancy and hyperplasia
what are nonstructural causes of abnormal bleeding in repro-aged women?
COEIN
Coagulopathy–> associated w/heavy flow (VW disease)
Ovulatory dysfunction –> PCOS
Endometrial–> infx
Iatrogenic–> IUD, IUS, exogenous hormones
Not yet classified
what available tissue sampling methods can be used for dx evaluation of AUB?
office endometrial biopsy
hysteroscopy directed endometrial sampling
AUB tx for massive bleeding?
AUB tx for moderate bleeding?
AUB tx unresponsive to conservative tx (meds)?
massive –> hospitalization and transfusions if hemodynamically unstable; 25 mg IV conjugated estrogens then hormonal tx
moderate –> combo OCPs, mirena
unresponsive –> D&C, polypectomy, myomectomy, endometrial ablation, hysterectomy
