[CLMD] Puberty, Disorders of Development, Menstrual Disorders [Moulton] Flashcards

1
Q

The corpus luteum produces copious amounts of which hormone?

A

Progesterone and some estradiol

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2
Q

What does LH vs. FSH stimulate in the ovarian follicle?

A
  • LH stimulates Theca cells to produce androgens (androstenedione and testosterone)
  • FSH stimulates Granulosa cells to convert androgens –> estrogens (E1 and E2)
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3
Q

What is responsible for causing atresia of all but 1 follicle during the follicular phase - leading to selection of the dominant follicle; what does the dominant follicle produce?

A
  • FSH levels progressively cause atresia of all but 1 follicle
  • The dominant follicle produces high levels of estradiol
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4
Q

Diagnosis of menopause is made by looking at levels of what?

A

↑ FSH since ovary is no longer receptive to FSH there is no negative feedback on the anterior pituitary

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5
Q

Which layers of the endometrium remains relatively unchanged during ach cycle and after menstruation provides stem cells for the renewal of the functionalis?

A

Inner portion or basalis

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6
Q

The endometrial linin?g reaches its maximal thickness during which phase?

A

Secretory phase

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7
Q

List 3 medications which can impair coagulation and may be associated with heavy bleeding?

A
  • Warfarin
  • Aspirin
  • Clopidogrel
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8
Q

Define primary amernorrhea.

A
  • No menstruation by 13 y/o WITHOUT secondary sexual development.

OR

  • By the age of 15 y/o WITH secondary sexual characteristics
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9
Q

What is the length of most normal menstrual cycles during the first gynecologic year and how does this change as more cycles occur?

A
  • Often irregular in adolescents, most normal cycles range from 21-45 days
  • By the 3rd year after menarche, majority of cycles are 21-35 days
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10
Q

What is the mean blood loss per menstrual period; how much loss is associated with anemia and what is a normal and abnormal amount of pad changes per day?

A
  • Mean blood loss is 30cc; changing pads 3-6x per day
  • >80cc is assoc. w/ anemia; changin pad q 1-2 hrs is considered excessive
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11
Q

How does obesity vs. a malnourished adolescent affect the onset of puberty?

A
  • Obese children have earlier onset of puberty
  • Malnourished, chronically ill w/ weight loss will have later onset
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12
Q

What is the invariant mean weight an adolescent needs to be or above to start menarche?

A

48 kg (106 lbs.)

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13
Q

Production and differentiation of which adrenal cortex zone are the initial endocrine changes assoc. w/ puberty?

A
  • Adrenal androgen (DHEA, DHEA-S, and androstenedione) production
  • Differentiation by the zona reticularis
  • Causes growth of axillary and pubic hair (adrenarche or pubarche)
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14
Q

Which hormones are required for thelarche (breast development) vs. pubarche/andrenarche (pubic/axillary hair developement)?

A
  • Thelarche requires estrogen
  • Pubarche/adrenarche requires androgens
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15
Q

What are the stages of normal pubertal development from earliest to latest?

A
  • Thelarche
  • Adrenarche
  • Peak Growth/height velocity
  • Menarche

- Mature sexual hair and breasts

*** TAG ME ****

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16
Q

Briefly describe the 5 Tanner stages of breast development.

A
  • Stage 1: preadolescent elevation of papilla only
  • Stage 2: breast bud stage; small mound w/ enlargement of areolar region
  • Stage 3: more enlargin of breast + areola w/o separation of their contours
  • Stage 4: projection of areola and papilla to form secondary mound
  • Stage 5: mature stage; projection of papilla only, recession of areola to general contour of breast
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17
Q

Briefly describe the 5 Tanner stages of pubic hair development.

A
  • Stage 1: absence of pubic hair
  • Stage 2: sparse hair along labia; hair downy w/ slight pigment
  • Stage 3: hair spreads sparsely over jct. of pubes; hair is darker + coarser
  • Stage 4: adult-type hair, there is no spread to medial thigh
  • Stage 5: spread to medial thighs assuming inverted triangle pattern
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18
Q

Precocious puberty is 5x more likely in which gender?

A

Girls

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19
Q

What are the 2 major subgroups of precocious puberty?

A
  1. Heterosexual: development of secondary sex characteristics opposite those anticipated –> virilizing neoplasms, CAH, exposure to exogenous androgens
  2. Isosexual: premature sexual maturation that is appropriate for the phenotype of the affected individual
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20
Q

Which test can be administered clinically to diagnose true isosexual precocious puberty; what are you looking for?

A
  • Administration of exogenous GnRH (stimulation test)
  • Look for resultant ↑ in LH levels consistent w/ older girls who are undergoing normal puberty
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21
Q

If a CNS disorder is suspected to be the cause of true isosexual precocious puberty, what is used to diagnose?

A

MRI of head

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22
Q

How is true isosexual precocious puberty treated pharmacologically; what is the important of treating this condition?

A
  • Use a GnRH agonist (leuprolide acetate) —> will suppress release of FSH and LH
  • If left untreated <50% of girls will not attain an adult height of 5 feet
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23
Q

Which type of precocity results in increased estrogen levels and causes sexual characteristic maturation without activation of H-P-O axis?

A

Pseudoisosexual precocity

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24
Q

What is seen in McCune-Albright syndrome (Polyostotic fibrous dysplasia)?

A
  • Multiple cystic bone defects
  • Café au lait spots (face, neck, shoulder and back)
  • Adrenal hypercortisolism
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25
Q

Which disease is associated with pseudoisosexual precocity due to sex cord tumors that secrete estrogen, GI polyposis, and mucocutaneous pigmentation?

A

Peutz-Jeghers syndrome

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26
Q

Puberty is considered delayed in what 4 situations?

A
  • Secondary sexual characteristics have not appeared by age 13
  • Thelarche has not occurred by age 14
  • No menarche by age of 15-16
  • When menses has not begun 5 years after onset of thelarche
27
Q

Hypergonadotropic hypogonadism (FSH >30 mIU/mL) is a cause of delayed puberty associated with what disease in females?

A

Gonadal dysgenesis (Turner Syndrome)

28
Q

List 6 underlying etiologies responsible for hypogonadotropic hypogonadism (FSH + LH <10 mIU/mL).

A
  • Constitutional (physiologic) delay
  • Kallmann syndrome
  • Anorexia/extreme exercise
  • Pituitary tumors/disorders
  • Hyperprolactinemia
  • Drug useb
29
Q

What are 3 anatomic causes of delayed puberty in girls?

A
  • Mullerian agenesis
  • Imperforate hymen
  • Transverse vaginal septum
30
Q

What is the definition of secondary amenorrhea?

A

Pt w/ prior menses has absent menses for 6 months or more

31
Q

What is the most common cause of primary amenorrhea with absence of secondary sexual characteristics?

A

Constitutional (physiologic) delay

32
Q

After hx and PE of patient with primary amenorrhea what do you check for next and how does this dictate the rest of your work-up?

A
  • Check for secondary sexual characteristics
  • If present then perform ultrasonography of uterus
  • If absent, measure the FSH and LH levels
33
Q

Kallman syndrome is due to mutations of the KAL gene on the x chromosome which prevents what; these patients will often have what sx’s?

A
  • Prevents migration of GnRH neurons into hypothalamus
  • Causes hypogonadotropic hypogonadism
  • Pt’s will often have anosmia or hyposmia (absent orsense of smell)
34
Q

If karyotype of pt with hypogonadotropic hypogonadism comes back with a Y chromosome, what is the next best step?

A

Gonadectomy to prevent malignant neoplastic transformation

35
Q

If a pt has primary amenorrhea with secondary sexual characteristics, but an ultrasound reveals an absent or abnormal uterus, what test is done next and what are you looking for?

A
  • Karyotype analysis
  • 46, XY = Androgen insensitivity syndrome
  • 46, XX = Mullerian agenesis
36
Q

What is the karyotype seen w/ androgen insensitivity syndrome and what are some characteristics seen in females with this disorder?

A
  • 46,XY —> will have male levels of testosterone
  • External female genitalia w/ absent to sparse pubic hair
  • Absent uterus and upper vagina
  • Breast development w/ smaller than normal areola/nipples
37
Q

Tx for androgen insensitivity syndrome (46,XY)?

A
  • Gonadectomy after puberty to avoid neoplasm (gonadoblastoma and dysgerminomas)
  • Can create neovagina by surgical and non-surgical methods
  • +HRT
38
Q

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

A

Mayer-Rokitansky-Kuster-Hauser Syndrome (Mullerian agenesis)

39
Q

Congenital anomalies of the uterus or vagina are often associated with abnormalities of which organ, how should this be assessed?

A
  • Renal abnormalities
  • Assess urinary system w/ an intravenous pyelogram
40
Q

What is the karyotype and characteristics of Mayer-Rokitansky-Kuster-Hauser Syndrome?

A
  • 46,XX —> female range of testosterone
  • Absent uterus and upper vagina; renal abnormalities common
  • Normal ovaries, secondary development, and external genitalia
  • Failure of mullerian ducts to fuse distally
41
Q

What should you suspect in adolescents that present complaining of monthly dysmenorrhea without vaginal bleeding; a vaginal bulge and midline cystic mass?

A

Imperforate hymen

42
Q

What are 2 causes of outflow tract obstruction in pt w/ primary amenorrhea w/ secondary characteristics and a normal uterus on US?

A
  • Imperforate hymen
  • Transverse vaginal septum
43
Q

Although the presentation of sx’s will be similar btw imperforate hymen and transverse vaginal septum, what is one difference?

A

Transverse vaginal septum will NOT have vaginal bulge

44
Q

List 4 labs that should be ordered for patient with secondary amenorrhea?

A
  • Urine hCG (ALWAYS!!!)
  • TSH
  • Prolactin
  • FSH
45
Q

What is the most common sx of hyperprolactinemia?

A

Galactorrhea = spontaneous flow of milk from breast

46
Q

What are 2 causes of hyperprolactinemia (>100 ng/mL)?

A
  • Pituitary adenoma
  • Empty sella syndrome
47
Q

Which test is done clinically in pt with seconary amenorrhea and normal TSH/prolactin; what is a positive and negative test?

A
  • Progesterone challenge test (PCT) —> takes 7-10 days
  • Positive PCT = bleeding –> normogonadotropic hypogonadism; most commonly due to PCOS
  • Negative PCT = no withdrawl bleeding; indicates inadequate estrogenization or an outflow tract abnormality
48
Q

If a progesterone challenge test (PCT) is negative, what are the next tests to do and what does a positive vs. negative result mean?

A
  • Next do an estrogen/progesterone challenge test –> takes 21 days
  • Negative = outflow tract obstruction
  • Positive = indicates abnormality w/ the H-P axis or ovaries
49
Q

If an estrogen/progesterone challenge test is negative, but there is elevated FSH and LH, this indicates the abnormality is where?

A

Ovarian

50
Q

What is the leading cause of female anovulatory infertility?

A

Polycystic ovarian syndrome (PCOS)

51
Q

Diagnosis of PCOS needs to meet 2 of which 3 criteria?

A
  • Oligomenorrhea or amenorrhea
  • Biochemical or clinical signs of hyperandrogenism: LH to FSH (2:1)
  • U/S revealing multiple small cysts beneath cortex of the ovary
52
Q

What is the effect of elevated insulin and androgen levels in pt’s with PCOS?

A

↓ the hepatic prod. of sex hormone binding globulins —> ↑ in circulating testosterone

53
Q

What are some of the treatment options for PCOS?

A
  • Oral contraceptives –> suppress FSH and LH –> ↓ testosterone and estrogen will ↑ SHBG
  • Weight loss
  • Clomiphene citrate can induce ovulation
  • Ovarian diathermy/laser tx
  • Spironolactone and/or electrolysis
  • Metformin = insulin-sensitizing agent
54
Q

If a progesterone challenge test (PCT) is negative and a estrogen (PCT) test is positive, what levels should be checked next?

A

FSH and LH levels

55
Q

Why is acanthosis nigricans a common finding in women with PCOS?

A

Due to the ↑ insulin resistance and hyperinsulinemia

56
Q

Which scale can be used as a scoring system for Hirsutism?

A

Ferrima-Galloway scale

57
Q

In evaluating a female pt with hyperandrogenism what should be suspected if the DHEA-S levels are >7000 ng/mL?

A

Adrenal androgen producing tumor

58
Q

In evaluating a female pt with hyperandrogenism what should be suspected if the total testosterone is >200 ng/dL?

A

Ovarian androgen producing tumor

59
Q

Define polymenorrhea and oligomenorrhea?

A
  • Polymenorrhea = abnormally frequent menses at intervals <21 days
  • Oligomenorrhea = menstrual cycles occuring >35 days but less than 6 months
60
Q

Define menorrhagia, metrorrhagia, and menometrorrhagia.

A
  • Menorrhagia = excessive and/or prolonged bleeding (>80 mL and >7 days) occurring at normal intervals
  • Metrorrhagia = irregular episodes of uterine bleeding
  • Menometrorrhagia = heavy and irregular uterine bleeding
61
Q

What is the PALM-COEIN classification system for abnormal bleeding in reproductive aged women?

A
  • Polyps - Coagulopathy
  • Adenomyosis - Ovulatory dysfunction
  • Leiomyoma - Endometriosis
  • Malignancy and hyperplasia - Iatrogenic
  • Not yet classified
62
Q

What is the treatment for abnormal uterine bleeding when massive?

A
  • Hospitalization and transfusions if hemodynamically unstable
  • 25 mg IV conjugated estrogens then hormonal tx (Mirena)
63
Q

What is the treatment for abnormal uterine bleeding when moderate amount of blood?

A

Combination OCP’s, Mirena

64
Q

Which disorder of coagulation may be associatd with heavy menstrual flow/AUB?

A

Von Willebrand disease