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ER II Exam I > Lecture 6: Puberty, Disorders of Development, Menstrual Disorders I & II > Flashcards

Lecture 6: Puberty, Disorders of Development, Menstrual Disorders I & II Flashcards

1
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

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2
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
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3
Q

What are 3 anatomic causes of delayed puberty in girls?

A
  • Mullerian agenesis
  • Imperforate hymen
  • Transverse vaginal septum
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4
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
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5
Q

What is the definition of secondary amenorrhea?

A

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

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6
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

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7
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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8
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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9
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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10
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)
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11
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

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12
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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13
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
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14
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

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15
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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16
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
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17
Q

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

A
  • Warfarin
  • Aspirin
  • Clopidogrel
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18
Q

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

A

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

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19
Q

The corpus luteum produces copious amounts of which hormone?

A

Progesterone and some estradiol

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20
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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21
Q

What is the leading cause of female anovulatory infertility?

A

Polycystic ovarian syndrome (PCOS)

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22
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

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23
Q

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

A
  • Urine hCG (ALWAYS!!!)
  • TSH
  • Prolactin
  • FSH
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24
Q

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

A

Von Willebrand disease

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25
Briefly describe the 5 Tanner stages of pubic hair development.
- **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**
26
Which test can be administered clinically to diagnose true isosexual precocious puberty; what are you looking for?
- **Administration** of **exogenous** **GnRH** (stimulation test) - Look for **resultant** ↑ in **LH levels** consistent w/ older girls who are undergoing normal puberty
27
What are 2 causes of hyperprolactinemia (\>100 ng/mL)?
- Pituitary adenoma - Empty sella syndrome
28
Hypergonadotropic hypogonadism (FSH \>30 mIU/mL) is a cause of delayed puberty associated with what disease in females?
**Gonadal dysgenesis** **(Turner Syndrome)**
29
Tx for androgen insensitivity syndrome (46,XY)?
- **Gonadectomy** after puberty to avoid neoplasm (gonadoblastoma and dysgerminomas) - Can create **neovagina** by surgical and non-surgical methods - **+HRT**
30
Congenital anomalies of the uterus or vagina are often associated with abnormalities of which organ, how should this be assessed?
- **Renal** abnormalities - Assess **urinary system** w/ an **intravenous pyelogram**
31
Diagnosis of menopause is made by looking at levels of what?
**↑ FSH** since ovary is no longer receptive to FSH there is no negative feedback on the **anterior pituitary**
32
What is the invariant mean weight an adolescent needs to be or above to start menarche?
**48 kg (106 lbs.)**
33
What is the most common sx of hyperprolactinemia?
**Galactorrhea** = spontaneous flow of milk from breast
34
Diagnosis of PCOS needs to meet 2 of which 3 criteria?
- **Oligomenorrhea** or **amenorrhea** - **Biochemical** or **clinical signs** of **hyper**androgenism: **LH to FSH (2:1)** - **U/S** revealing multiple small cysts beneath cortex of the ovary
35
Define menorrhagia, metrorrhagia, and menometrorrhagia.
- **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
36
What is the PALM-COEIN classification system for abnormal bleeding in reproductive aged women?
- **P**olyps - **C**oagulopathy - **A**denomyosis - **O**vulatory dysfunction - **L**eiomyoma - **E**ndometriosis - **M**alignancy and hyperplasia - **I**atrogenic - **N**ot yet classified
37
Production and differentiation of which adrenal cortex zone are the initial endocrine changes assoc. w/ puberty?
- Adrenal **androgen** (DHEA, DHEA-S, and androstenedione) production - **Differentiation** by the **zona reticularis** - Causes growth of **axillary** and **pubic hair** (**adrenarche** or **pubarche**)
38
The endometrial linin?g reaches its maximal thickness during which phase?
**Secretory phase**
39
What is the most common cause of primary amenorrhea with absence of secondary sexual characteristics?
Constitutional (physiologic) delay
40
If karyotype of pt with hypogonadotropic hypogonadism comes back with a Y chromosome, what is the next best step?
**Gonadectomy** to _prevent_ malignant neoplastic transformation
41
Why is acanthosis nigricans a common finding in women with PCOS?
Due to the ↑ **insulin resistance** and **hyperinsulinemia**
42
What is the karyotype and characteristics of Mayer-Rokitansky-Kuster-Hauser Syndrome?
- **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**
43
What are some of the treatment options for PCOS?
- **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
44
Puberty is considered delayed in what 4 situations?
- 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
45
Define primary amernorrhea.
- **No** menstruation by **13 y/o** *_WITHOUT_* secondary sexual development. **OR** - By the age of **15** **y/o** *_WITH_* secondary sexual characteristics
46
Which layers of the endometrium remains relatively unchanged during ach cycle and after menstruation provides stem cells for the renewal of the functionalis?
**Inner** portion or **basalis**
47
Define polymenorrhea and oligomenorrhea?
- **Polymenorrhea** = **abnormally** frequent menses at intervals **\<21 days** - **Oligomenorrhea** = menstrual cycles occuring **\>35 days** but less than **6 months**
48
What is the karyotype seen w/ androgen insensitivity syndrome and what are some characteristics seen in females with this disorder?
- **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
49
What should you suspect in adolescents that present complaining of monthly dysmenorrhea without vaginal bleeding; a vaginal bulge and midline cystic mass?
**Imperforate hymen**
50
Which disease is associated with pseudoisosexual precocity due to sex cord tumors that secrete estrogen, GI polyposis, and mucocutaneous pigmentation?
Peutz-Jeghers syndrome
51
How does obesity vs. a malnourished adolescent affect the onset of puberty?
- **Obese** children have **earlier** onset of puberty - **Malnourished**, chronically ill w/ **weight loss** will have **later** onset
52
Which type of precocity results in increased estrogen levels and causes sexual characteristic maturation **without** activation of H-P-O axis?
**Pseudoisosexual precocity**
53
What is the treatment for abnormal uterine bleeding when moderate amount of blood?
Combination OCP's, **Mirena**
54
Which scale can be used as a scoring system for Hirsutism?
**Ferrima-Galloway** scale
55
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?
- **Karyotype analysis** - **46, XY** = **Androgen insensitivity syndrome** - **46, XX** = **Mullerian agenesis**
56
What is the length of most normal menstrual cycles during the first gynecologic year and how does this change as more cycles occur?
- 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**
57
Which test is done clinically in pt with seconary amenorrhea and normal TSH/prolactin; what is a positive and negative test?
- **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
58
Precocious puberty is 5x more likely in which gender?
Girls
59
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?
- **Prevents** migration of **GnRH** neurons into **hypothalamus** - Causes **hypogonadotropic hypogonadism** - Pt's will often have **anosmia** or **hyposmia (absent or** ↓ **sense of smell)**
60
Which hormones are required for thelarche (breast development) vs. pubarche/andrenarche (pubic/axillary hair developement)?
- **Thelarche** requires **estrogen** - **Pubarche/adrenarche** requires **androgens**
61
If an estrogen/progesterone challenge test is negative, but there is elevated FSH and LH, this indicates the abnormality is where?
**Ovarian**
62
What does LH vs. FSH stimulate in the ovarian follicle?
- **LH** stimulates **Theca** cells to produce **androgens** (androstenedione and testosterone) - **FSH** stimulates **Granulosa** cells to convert **androgens --\> estrogens (E1 and E2)**
63
Briefly describe the 5 Tanner stages of breast development.
- **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
64
If a CNS disorder is suspected to be the cause of true isosexual precocious puberty, what is used to diagnose?
**MRI** of **head**

ER II Exam I (26 decks)

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