Wednesday, 3-1-Puberty & disorders of development and Menstrual disorders (Moulton)

Question 1

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

Answer 1

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

Question 2

which endometrial zone sloughs off at menstruation and contains spiral arteries?

Answer 2

functionalis (outer portion)

Question 3

median age of menarche? when does it usually occur after thelarche (breast budding)? which tanner stage?

Answer 3

12.43

occurs within 2-3 yrs after thelarche

at tanner stage IV, rare before stage III

Question 4

__-no menstruation by 13 years old without secondary sexual development OR by the age of 15 yrs with secodnary sexual characteristics

Answer 4

Primary amenorrhea

Question 5

typical menstrual cycle interval (in days) in young females?

Answer 5

21-45 days

Question 6

Mean blood loss per menstrual cycle is __ cc.

Greater than __ cc blood loss has been associated with anemia

Answer 6

30

80 –> changing a pad q1-2 hrs considered excessive esp if bleeding >7 days

Question 7

rises in adrenal androgens in late childhood (8-11) causes growth of ___

Answer 7

axillary and pubic hair (adrenarche or pubarche)

between age 8-11 –> increase [DHEA] and [androstenedione]

Question 8

__ is known as breast development and 1st physical sign of puberty. It requires estrogen

Answer 8

thelarche

Question 9

pubarche/adrenarche requires ___

Answer 9

androgens

Question 10

which demographic experiences the earliest age of thelarche? adrenarche? menarche?

Answer 10

AA’s start the earliest, followed by hispanics, then whites in all 3 categories

Question 11

this tanner stage of breast development is preadolescent, elevation of papilla only

Answer 11

1

Question 12

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

Answer 12

2

Question 13

this tanner stage of breast development is further enlargement of breast and areola without separation of their contours

Answer 13

3

Question 14

this tanner stage of breast development is a projection of areola and papilla to form a secondary mound above level of the breast

Answer 14

4

Question 15

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

Answer 15

5

Question 16

this tanner stage of pubarche is preadolescent, absence of pubic hair

Answer 16

1

Question 17

this tanner stage of pubarche is sparse hair along labia, hair downy with slight pigment

Answer 17

2

Question 18

this tanner stage of pubarche is hair spreading sparsely over junction of the pubes, hair is darker and coarser

Answer 18

3

Question 19

this tanner stage of pubarche is adult-type hair, no spread to medial surface of thighs

Answer 19

4

Question 20

this tanner stage of pubarche is adult-type hair with spread to medial thighs assuming an inverted triangle pattern

Answer 20

5

Question 21

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

Answer 21

androgen-secreting neoplasms

Question 22

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

Answer 22

Congenital Adrenal Hyperplasia

Question 23

what is the term for premature sexual maturation that is appropriate for the phenotype of the affected individual?

Answer 23

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

Question 24

how do you dx true isosexual precocious puberty?

how do you tx?

Answer 24

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

Question 25

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

Answer 25

McCune-albright syndrome (Polyostotic fibrous dysplasia)

Question 26

this pseudoisosexual precocity disorder is associated with a sex cord tumor that secrretes estrogen, GI polyposis and mucocutaneous pigmentation

Answer 26

Peutz-Jeghers syndrome

Question 27

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

Answer 27

13 14 15-16 5

Question 28

regarding delayed puberty, hypergonadotropic hypogonadism is known as ___

Answer 28

turner syndrome (gonadal dysgenesis) FSH > 30

Question 29

regarding delayed puberty, Kallman syndrome, anorexia/extreme exercise, pituitary tumors/disorders, hyperprolactinemia, and drug use make up this category of "-gonadism"

Answer 29

Hypogonadotropic hypogonadism FSH + LH <10

Question 30

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 --> ?

Answer 30

Hypogonadotropic Hypogonadism slide 56

Question 31

this form of hypogonadtropic hypogonadism is d/t mutation of KAL gene on X chr, pts have anosmia or hyposmia

Answer 31

Kallman syndrome

Question 32

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 -->

Answer 32

Hypergonadotropic hypogonadism 46xx-- premature ovarian failure 45XO--> turners

Question 33

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

Answer 33

Turners --> 45XO

Question 34

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 --> ?

Answer 34

46XY--> androgen insensitivity syndrome 46XX--> Mullerian agenesis

Question 35

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

Answer 35

Androgen Insensitivity Syndrome (AIS)

Question 36

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)

Answer 36

Mullerian dysgenesis or agenesis

Question 37

__ is the most common cause of primary amenorrhea in women with normal breast development

Answer 37

Mayer-Rokitansky-kuster-hauser syndrome (mullerian agenesis)

Question 38

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 --> ?

Answer 38

Imperforate hymen or transverse vaginal septum correct imperforate hymen --> hymenectomy correct transverse vag septum --> surgery

Question 39

__ is defined as absence of menstruation x6 months

Answer 39

secondary amenorrhea

Question 40

hx & PE findings in secondayr amenorrhea? Labs?

Answer 40

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

Question 41

pt with secondary amenorrhea who has normal prolactin and abnormal TSH?

Answer 41

thyroid disease

Question 42

pt with secondary amenorrhea who has really high prolactin (>100) should be evaluated for ___. if high prolactin but <100, how do you evaluate?

Answer 42

really high (>100)=evaluate for prolactinoma with head MRI --> empty sella syndrome, pituitary adenoma high (<100)=if MRI neg., consider other causes

Question 43

tx focus for pt with secondary amenorrhea who has microadenomas found on MRI?

Answer 43

manage infertility, galactorrhea, and breast discomfort | consider dopamine agonist (bromocriptine, cabergoline)

Question 44

tx focus for pt with secondary amenorrhea who has macroadenomas found on MRI?

Answer 44

dopamine agonists | transsphrenoidal resection or craniotomy (most avoid surgery 1st)

Question 45

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?

Answer 45

Normogonadotropic hypogonadism --> **PCOS MOST COMMON** negative PCT --> indicate inadequate estrogenization or outflow tract abnormality

Question 46

which ovarian disorder is likely to cause a normogonadotropic amenorrhea with hyperandrogenism?

Answer 46

PCOS

Question 47

__ 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

Answer 47

PCOS

Question 48

how do you dx PCOS?

Answer 48

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

Question 49

tx for PCOS?

Answer 49

- weight loss - OCs - clomiphene citrate - ovarian diathermy/laser tx - spironolactone - insulin sensitizing agents-biguanides (metformin)

Question 50

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?

Answer 50

Hypergonadotropic hypogonadism causeds: ovarian injury from surgery, pelvic radiation, chemo, carrier status of fragile X, autoimmune, and mumps

Question 51

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?

Answer 51

Hypogonadotropic hypogonadism causes: anorexia or bullemia nervosa, chronic illness, cranial radiation, excessive exercise, malnutrition/excessive weight loss, Sheehan's syndrome

Question 52

__-abnormally frequent menses at intervals <21 days

Answer 52

polymenorrhea

Question 53

__-excessive and/or prolonged bleeding (>80 mL and >7 days) occurring at normal intervals

Answer 53

menorrhagia (hypermenorhea)

Question 54

__-irregular episodes of uterine bleeding

Answer 54

metrorrhagia

Question 55

___-heavy and irregular uterine bleeding

Answer 55

menometrorrhagia

Question 56

__-scant bleeding at ovulation for 1 or 2 days

Answer 56

intermenstrual bleeding

Question 57

__-menstrual cycles occuring > 35 days but less than 6 months

Answer 57

oligomenorrhea

Question 58

what are structural causes of abnormal bleeding in reproductive-aged women?

Answer 58

``` PALM Polyp Adenomyosis Leiomyoma Malignancy and hyperplasia ```

Question 59

what are nonstructural causes of abnormal bleeding in repro-aged women?

Answer 59

COEIN Coagulopathy--> associated w/heavy flow (VW disease) Ovulatory dysfunction --> PCOS Endometrial--> infx Iatrogenic--> IUD, IUS, exogenous hormones Not yet classified

Question 60

what available tissue sampling methods can be used for dx evaluation of AUB?

Answer 60

office endometrial biopsy | hysteroscopy directed endometrial sampling

Question 61

AUB tx for massive bleeding? AUB tx for moderate bleeding? AUB tx unresponsive to conservative tx (meds)?

Answer 61

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