Puberty, Disorders of Development, and Menstrual Disorders (Moulton) PART 2

Question 1

__1__ precocious puberty is characterized by development of secondary sexual characteristics opposite those of anticipated phenotypic sex.

__2__ precocious puberty is characterized by premature sexual maturation that is appropriate for the phenotype of the affected individual.

Answer 1

1) Heterosexual

2) Isosexual

Question 2

What virilizing tumor can cause hetersexual precocious puberty, is exceedingly rare in childhood, and usually originates in the ovaries?

What do they secrete?

Answer 2

1) Sertoli-Leydig cell

2) Androgens

Question 3

1) Hetersexual precocious puberty can lead to what enzyme deficiency?
2) What can cause this
3) What does the enzyme deficiency lead to

Answer 3

1) 21-hydroxylase
2) Congenital adrenal Hyperplasia
3) Excess androgen production

Question 4

1) How can you diagnose True isosexual precocious puberty?

2) What will be increased as a result?

Answer 4

1) Administer exogenous GnRH

2) LH (consistent with older girls undergoing normal puberty)

Question 5

What is the treatment for true isosexual precocious puberty?

This suppresses the pituitary release of?

Answer 5

1) GnRH agonist (leuprolide acetate)

2) FSH and LH

Question 6

1) What does Pseudoisosexual precocity result in?

2) What does this cause?

Answer 6

1) Increased Estrogen

2) Sexual characteristics WITHOUT HPO axis activation

Question 7

What are the 2 causes of pseudoisosexual precocity?

A cause of pseudoisosexual precocity is ____, which presents as multiple cystic bone defects, café au lait spots, and adrenal hypercortisolism.

A cause of pseudoisosexual precocity is ____, which is associated with a sex cord tumor that secretes estrogen, gastrointestinal polyposis, and mucocutaneous pigmentation.

Answer 7

1) McCune-Albright syndrome (Polyostotic fibrous dysplasia)

2) Peutz-Jeghers syndrome

Question 8

Primary Amenorrhea Causes: Hypogonadotropic Hypogonadism

1) What are causes?
2) What is the mutation in?
3) what chromoosome
4) What happens?

Answer 8

1) Anorexia nervosa + Kallmann Syndrome
2) Mutation of KAL gene
3) X chromosome
4) No migration of GnRH to Hypothalamus

Question 9

What condition can cause primary amenorrhea due to hypergonadotropic hypogonadism?

What is this the most common form of?

Answer 9

1) Turner syndrome (45 XO)

2) Gonadal Dysgenesis

Question 10

What are some findings associated with Turner’s syndrome?

Answer 10

1) Webbing of the neck
2) Broad flat chest
3) Short stature
4) Rudimentary streaked ovaries
5) Coarctation of the aorta

WSB RC

Question 11

What conditions can cause primary amenorrhea with breast development?

Answer 11

1) Androgen insensitivity syndrome (AIS)

2) Mullerian agenesis (most common)

Question 12

Androgen Insensitivity Syndrome

1. What karyotype?
   2) What levels are increased?
   3) How is External female genitalia
   4) What is missing?

Testes form in the abdominal wall and secrete normal amounts of __3__ hormones causing no uterus to form.

Answer 12

1) 46XY
2) Testosterone
3) Normal
4) Pubic hair and Uterus
5) Anti Mullerian

Question 13

__1__ is the most common cause of primary amenorrhea in women with normal breast development.

2) What karyotype
3) What testosterone levels
4) What associated issue?
5) What is missing?
6) What is the issue with this

Answer 13

1) Mullerian agenesis (Meyer-Rokitansky-Kuster-Hauser syndrome)
2) 46XX
3) Normal
4) Renal Anomalies
5) Absent Uterus
6) Failure of paramesonephric duct to fuse

Question 14

What should you suspect in adolescents if a

• Vaginal bulge and midline cystic mass are found
• Monthly dysmenorrhea without vaginal bleeding?

What if they present with similar symptoms but do not have a vaginal bulge?

Answer 14

1) Imperforate hymen

2) Transverse vaginal septum

Question 15

In the diagnostic evaluation of a patient with secondary amenorrhea, what labs do you want to draw?

Answer 15

1) TSH
2) Urine hCG
3) Prolactin

PUT

Question 16

Galactorrhea is the most common symptom of?

Answer 16

Hyperprolactinemia

Question 17

When prolactin levels are really high (> 100ng/mL) why would you want to order a head MRI?

Answer 17

Evaluate for

Prolactinoma (pituitary adenoma)
Empty sella syndrome

Question 18

1) What are Normogonadotropic amenorrhea with Hyperandrogenism Causes
2) What is the enzyme deficiency for one of them leading to increased level of what
3) What is most common?

Answer 18

1) Adrenal: Non classical Congenital Adrenal Hyperplasia, Cushing, Androgen Secreting Tumor

Ovarian: PCOS (most common)

2) Non classical Congenital Adrenal Hyperplasia: 21-Hydroxylase deficiency leading to increased 17-hydroxyprogesterone

Question 19

When working up a diagnosis for secondary amenorrhea, a negative estrogen/progesterone challenge test indicates?

A positive test estrogen/progesterone challenge test with elevated FSH & LH (hypergonadotropic hypogonadism) indicates?

A positive test estrogen/progesterone challenge test with normal or low FSH & LH (hypogonadotropic hypogonadism) indicates?

Answer 19

1) Outflow tract obstruction
2) Abnormality the ovaries
3) Abnormality with the hypothalamic-pituitary axis

Question 20

What are some anatomic causes of secondary amenorrhea?

Answer 20

1) Asherman syndrome

2) Cervical stenosis

Question 21

Amenorrhea can occur in __1__ syndrome which presents as central obesity, moon-like face, buffalo hump, and HTN.

__2__ levels are elevated.

Answer 21

1) Cushing’s

2) Cortisol

Question 22

What is the leading cause of female anovulatory infertility?

What can this Lead to that is bad?

Answer 22

Polycystic ovarian syndrome (PCOS)

Endometrial Cancer

Question 23

What sensitivity does PCOS have?

What does this lead to?

What else is increased?

What is FSH: LH Ratio?

Answer 23

1) Insulin
2) Insulin Hypersecretion
3) Testosterone and Androgens
4) 2:1

Question 24

The presentation of an obese female patient, anovulation, hirsutism, and acanthosis nigricans points to?

Answer 24

PCOS

Question 25

What are treatment options for PCOS?

Answer 25

1) Weight loss
2) OCPs
3) Clomiphene citrate (induce ovulation)
4) Spironolactone (competes for testosterone-binding sites)

WOCS

Question 26

24 hr free urinary cortisol or an overnight dexamethasone suppression test can be ordered to rule out?

Answer 26

Cushing syndrome

Question 27

DHEA-S levels in excess of 7000ng/ml you should suspect?

Total testosterone > 200ng/dl you should suspect?

Answer 27

1) Adrenal androgen producing tumor

2) Ovarian androgen producing tumor

Question 28

__1__ is characterized by no menstruation by 13 y/o without secondary sexual development OR by the age of 15 years with secondary sexual characteristics.

__2__ is characterized by the absence of menses for 6 months or more.

__3__ is characterized by abnormally frequent menses at intervals at < 21 days.

__4__ is characterized by excessive and/or prolonged bleeding (>80mL and > 7 days) occurring at normal intervals.

__5__ is characterized irregular episodes of uterine bleeding

__6__ is characterized heavy and irregular uterine bleeding

__7__ is characterized scant bleeding at ovulation for 1 or 2 days

__8__ is characterized menstrual cycles occurring >35 days but less then 6 months.

Answer 28

1) Primary Amenorrhea
2) Secondary Amenorrhea
3) Polymenorrhea
4) Menorrhagia (hypermenorrhea)
5) Metrorrhagia
6) Menometrorrhagia
7) Intermenstrual bleeding
8) Oligomenorrhea

Question 29

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

Answer 29

Structural:

1) Polyp
2) Adenomyosis
3) Leiomyoma
4) Malignancy and Hyperplasia

Nonstructural:

1) Coagulopathy
2) Ovulatory Dysfunction
3) Endometrial
4) Iatrogenic
5) Not yet classified

Question 30

Coagulopathies such as Von Willebrand disease are associated with ___.

Ovulatory Dysfunction such as PCOS are associated with ____ menses.

What are some iatrogenic causes of abnormal bleeding?

Answer 30

1) Heavy flow
2) Unpredictable
3) IUD and exogenous hormones