21. Puberty Disorders of Development

Question 1

In the anterior lobe of the pituitary, one can find FSH and LH which are synthesized and stored in cells called gonadotrophs

Answer 1

MEOW

Question 2

Ovarian cycle is divided into follicular phase which begins with onset on menstruation and culminated in the preovulatory surge of LH and Luteal phase which begins with the onset of preovulatory surge of LH and ends with the first days of?

Answer 2

Menses

Question 3

Decreasing estradiol and progesterone from regressing corpus luteum initiate an increase in FSH by a negative feedback mechanism, which stimulates follicular growth and estradiol secretion… major characteristic of follicular growth and estradiol secretion is explained by?

Answer 3

FSH and LH

Question 4

LH stimulates theca cells to produce androgens (testosterone and androstenedione) and FSH stimulates the granulosa cells to covert the androgens to?

Answer 4

Estrogens E1/E2

Question 5

During Luteal phase, LH and FSH are suppressed via negative feedback due to elevated estradiol and progesterone, in no conception occurs, E/P decline due to regressing corpus luteum near the end of luteal phase, FSH will then rise to initiate new?

Answer 5

Follicular Growth

Question 6

GnRH (Gonadotropin-Releasing Hormone) in the hypothalamus (arcuate nucleus) is responsible for synthesis and release of LH and FSH, it does so by reaching the anterior pituitary and stimulating its synthesis and release, what enhances hypothalamic release of GnRH?

Answer 6

Estradiol - so induces midcycle LH surge

Question 7

During follicular development, ovary secretes minimal progesterone, most of it comes from conversion of adrenal pregnenolone, prior to ovulation the unruptured luteinizing graafian follicle begins to produce inc. progesterone. secretion of progesterone by the corpus luteum reaches a max at how many days after ovulation and returns to normal before mesntruation?

Answer 7

5-7 days (same time as estrogen)

Question 8

Endometrium divided into 2 zones: outer functionalis- sprial arteries (sloughed off), and inner basalis- unchanged during each cycle containing basal arteries… Endometrial stages are menstrual phase, proliferative phase (estrogenic) and the?

Answer 8

secretory phase (progestational)

Question 9

Menstrual phase is the only portion of the cycle that is vizualized, first day is cycle day 1, sloughing off functionalis layer, proliferative phase- endometrial growth due to estrogen stimulation- inc in length of spiral arteries and numerous mitoses, glands are straight, folowing ovulation, progesterone secretion by corpus luteum stimulate gland cells to secrete mucous and gylocen, what phase?

Answer 9

secretory phase

Question 10

In secretory phase, stroma is edmatous, mitosis are rare, spiral arteries continue to extend into endometrium, glands are tortuous, endometrial lining is max thickness at 18mm (should be less than 4mm in postmeno women), no conception by day 23, corpus luteum regresses and secretion of what declines?

Answer 10

estradial and progesterone

1 day prior to menstruation: constriction of spiral arteries causing ischemia of endometrium

Question 11

Menarche is 12.43 years, 10% at 11.11 years, 90% at 13.75 years, occurs 2-3 years after thelarche at tanner stage IV, what is it called when there is no menstruation by 13 years old WITHOUT secondary sexual development or by the age of 15 WITH secondary sexual characteristics?

Answer 11

Primary Amenorrhea

Question 12

First year of cycls are usually 21-45 days (avg34days), by 3rd year most are between 21-35 days, first year flow is less than 7 days and the amount of tampons used per day should be between 3 and?

Answer 12

6

Question 13

Median blood loss per period is 30cc, greater than 80cc is assoc with anemia-changing pad q1-2 hours is excessive especially if bleeding lasts longer than 7days. Puberty is usually at 12.4 years and depends on race, urban, and obese/malnutrioned will be early?

Answer 13

Obese have earlier onset
Malnutrioned later onset

(106lbs mean weight of menarche)

Question 14

Around 11, there is a loss of sensistivity by the gonadostat to the negative feedback of sex steroids, in combo with the intrinsic loss of CNS inhibition of hypothalamic GnRH release. increase in GnRH promotes ovarian follicular maturation and sex steroid production which elads to development of ?

Answer 14

secondary sexual characteristics

Question 15

Thelarche is the first sign of puberty (requires estrogen), unliateral is normal, Adrenarche (pubarche) (requires androgens) is the growth of pubic hair/axillary hair development, peak height velocity occurs 2 years earlier than men and then menarche occurs which requires PULSATILE GnRH from hypo, FSH and LH from pit, estrogen and progesterone from ovaries and a normal?

Answer 15

outflow tract

all these happen earlier in AA and earliest in hispanic

Question 16

match the following Tanner stages for breast growth…
Stage 1
Stage 2
Stage 3
Stage 4
Stage5
preadolescent: elevation of papilla only
mature: projection of papilla only, resulting from recession of areola to the general contour of breast
Futher enlargment of breast and areola without separation of their contours
breast bud- elevationof breast and papilla as a small mound w enlargement of areolar region
projection of areola and papilla to form a secondary mound above the level of the breast

Answer 16

Stage 1 preadolescent: elevation of papilla only
Stage 2 breast bud- elevationof breast and papilla as a small mound w enlargement of areolar region
Stage 3 Futher enlargment of breast and areola without separation of their contours
Stage 4 projection of areola and papilla to form a secondary mound above the level of the breast
Stage5 mature: projection of papilla only, resulting from recession of areola to the general contour of breast

Question 17

Stage 1
Stage 2
Stage 3
Stage 4
Stage 5
absence of hair
adult type hair with spread to medial thighs assuming inverted triangle pattern
adult type hair, no spread to medial surface of thighs
hair spreads sparesly over juncction of pubes; darker and coarser
sparse hair along labia, hair downy with slight pigment

Answer 17

Stage 1 absence of hair
Stage 2 sparse hair along labia, hair downy with slight pigment
Stage 3 hair spreads sparesly over juncction of pubes; darker and coarser
Stage 4 adult type hair, no spread to medial surface of thighs
Stage 5 adult type hair with spread to medial thighs assuming inverted triangle pattern

Question 18

Precocious puberty occurs at 8 for girls and 9 for boys and can be caused by heterosexual- development of opposite sexual characteristics (virulizing tumors, CAD) or isosexual- development of?

Answer 18

sexual maturation that is appropriate for pphenotype of affected individual (tumors, trauma, infectious)

Question 19

Heterosexual precocity is due to andorgen secreting neoplasms such as sertoli leydig cells or adrenals, What is MC result from defect of the adrenal enzymes 21-hydroxylase leading to excessive androgen production, present with birth of female w ambiguous genitalia w progressive virilization and short adult status?

Answer 19

Congenital Adrenal Hyperplasia CAD

Question 20

True isosexual precocity means premature activation of normal process while pseuoisosexual precocity is caused by estrogen producing tumors. True - 75% idiopathic, dx with admin of GnRH see rise in LH (tx with GnRH agonist) Pseudo is due to McCune Albright Syndrome and?

Answer 20

Puetz Jegher syndrome

Question 21

McCune Albright Syndrome involves somatic mutation during embryogenesis which causes them to function independent of normal sitmulating hormones, cystic bone defects, cafe au lati spots (face neck shoulder and back) and will see adrenal hypercortisolism. In puetz jegher associated with sex cord tumor that secretes estrogen and GI polyps with what skin lesions?

Answer 21

Mucocutaneous pigmentation

Question 22

Amenorrhea is absence of menses primary listed above, secondary is patient with prior menses has absent menses for 6 months or more

Answer 22

meow

Question 23

Hypogonadotropic Hypogonadism (anorexia/Kallman)… Kallman syndrome is mutation of KAL gene on x chr that prevents migration of GnRH neurons into hypothalamus, patients have anosmia or hyposmia (cant smell)– these are diagnosed via low FSH LH and possible

Answer 23

MRI

primary ammenorrhea with absence of secondary sexual characteristics

Question 24

Hypergonadotropic Hypogonadism (chr, inj to ovariy, chemo, radiation) test via MRI of brain, FSH, karyotype, progesterone and prolactin, find LH/FSH high, think TURNER SYNDROME 45XO: MC form of female gonadal dysgenesis, no signs of secondary sex characters, mosaicisim, see webbing neck, broad flat chest, short stature, streaked ovaries, no puberty and what of the heart?

Answer 24

Coarctation of the Heart

primary ammenorrhea with absence of secondary sexual characteristics

Question 25

primary ammenorrhea WITH secondary sexual characteristic: with breast development and mullerian anomalies 2 categories: androgen insensitivity syndrome (AIS) and mullerian agenesis. What has 46XY karyotype, male level of testosterone, defect in adrogen receptor, tests are in abdominal wall, no uterus, external female genetalia w no pubes, has some breast development?

Answer 25

Androgen Insensitivity Syndrome AIS

Question 26

Mullerian dysgenesis is primary amenorrhea, breast development, testosterone aprop for females and 46XX, 1) imperforate hymen 2) absence of normal uterus = mullerian agenesis = meyer rokitansky kuster hauser syndrome- failure ducts to fuse, absent uterus w tubes and ovaries- with renal abnls… normal secondary devel and external female genetalia, absent uterus/upper vagina, normal ovaries, mC primary cause of primary amenorrhea in women with normal what development?

Answer 26

BREAST DEVELOPMENT

Question 27

Androgen insensitivity: (46XY) normal breasts, no sexual hair, normal looking female genetalia, absent uterus/upper vagina, tx with gonadectomy after puberty to avoid neoplasm, create neovagina, and what is elevated ***?

Answer 27

MALE RANGE TESTOSTERONE LEVELS

Question 28

Secondary amenorrhea (6 months with absence menstruation) can be due to hypothyroidism (abnl TSH), or abnormally high prolactin level= hyperprolactinemia which has a MC sx of galactorrhea - get MRI of head, might see microadenoma- monitor since slow growing, pituiatry macroadenoma (>10mm) may be treated with what? (never srugery cause always b9 and grow back)

Answer 28

Dopamine agonists

***Pituitary Adenoma (Micro/macro) is MCC of hyperprolactinemia >100ng/ml

Question 29

What is a common occular symptom associated with large pituitary adenomas?

Answer 29

Bitemporal Hemianopia (cant see on either side laterally)

Question 30

Secondary amenorrhea due to normogonadotropic hypogonism- test with progesterone challenge test PCT if normal TSH and prolactin… Positive PCT = positive bleeding which points you to what MC diagnosis?
NOTE: if negative PCT (no bleeding) = outflow tract abnl

Answer 30

PCOS

outflow tract abl= asherman syndrome/cervical stenosis

Question 31

PCOS (normogonadotropic amenorrhea with hyperandrogenism) is the leading cause of female anovulatory infertility… Have insuling sensitivity= hyperinsulinemia, + elevated androgen levels leads to hepatic production of sex hormone binding globulins leads to INC testosterone, dx need 2/3: oligo/amenorrhea + LH:FSH of 2:1 and/or US revealing?

Answer 31

Multiple cysts beneath cortex of the ovary

**high LH low FSH

Question 32

Tx for PCOS includes weight loss, OCP which supresses FSH and LH, Clomiphene citrate to induce ovulation, spironolactone or metformin (tx DM)

Answer 32

Meow

Question 33

If there is a negative progestogen challenge test (no bleeding) then do estrogen/progesten challenge test, if estrogen pos and progesten negative, check FSH/LH, if both are elevated then Hypergonadotropic Hypogonadism which is commonly due to postmeno ovairan failure or premature ovarian?

Answer 33

failure

Question 34

(secondary amenorrhea) If there is a negative progestogen challenge test (no bleeding) then do estrogen/progesten challenge test, if estrogen pos and progesten negative, check FSH/LH, if both are LOW the perform MRI for pit tumor, normal MRI means hypogonadotropic hypogonadism due to anorexia, malnurtrition, too much exercise or?

Answer 34

chronic illness (liver dz/DM)

Question 35

24hr free urinary cortisol or overnight dexamethasone suppression test to rule out cushing, prolactin and TSH to rule out hyperprolactinemia/thyroid, if DHEA-S levels are high >7000 suspect adrenal androgen producing tumor, if testosterone is >200- suspect ovarian?

Answer 35

androgen producing tumor

Question 36

Polymenorrhea: abnl freq menses at intervals <21 days
Menorrhagia: excessive/prolonged bleeding >80ml
Metrorrhagia: irregular episodes of bleeding
Menometrorrhagia: heavy/irregular uterine bleeding
Oligomenorrhea: menstrual cycles occuring at greater than how many days, but less than 6 months?

Answer 36

greater than 35 days between menses but less than 6m

Question 37

Dysfunctional Uterine Bleeding DUB is defined as abnormal uterine bleeding that cannot be attributed to medications, blood issues, systemic disease, trauma, organic conditions- usually caused by abberations in th HPO axis resulting in?

Answer 37

Anovulation

Question 38

AUB Tx:
Massive bleeding: hosp + transfusion: 25mg IV conjugated estrogens then hormonal treatment
Moderate: OCP + Mirena
Unresponsive to conservative therapy (meds): D and C, polypectomy, myomectomy, endometrial ablation or?

Answer 38

Hysterectomy