Normal and Abnormal Female Puberty - Strawn Flashcards Preview

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Flashcards in Normal and Abnormal Female Puberty - Strawn Deck (24):
1

List 4 major events of normal female puberty in chronological order.

  1. Initiation of the growth spurt is first
    • Note: peak of growth spurt occurs later in puberty
  2. Initiation of breast development & pubic/axillary hair
  3. Menstruation is last

2

What two hormones contribute to initiation of the growth spurt in females?

  1. Estrogen
  2. Growth hormone

3

  1. Breast development requires the presence of which hormone?
  2. Knowing this, what three things must be functional in a female who develops breasts normally?

  1. Estrogen
  2. Presence of normal estrogen levels (typically) implies:
    • Functioning gonad is present
    • Gonad is responsive to FSH/LH
    • Production of FSH/LH implies a functioning hypothalamic-pituitary axis

4

  1. What hormone causes development of pubic/axillary hair in a female?
  2. How & where is this hormone synthesized from its precursor?
  3. What two locations in the female body are responsible for synthesizing the precursor?

  1. DHT
  2. Synthesized from testosterone by 5a-reductase in the hair follicle
  3. Ovaries & Adrenal cortex

5

  1. What hormone drives testosterone production in the ovary?
  2. What hormone drives testosterone production in the adrenal cortex?

  1. LH
  2. ACTH

6

In order for menstruation to occur, there must be what?

What events during development contribute to this?

A functioning outflow tract.

  • Development of intact, functional uterus
  • Functional development of mullerian (paramesonephric) duct system
  • Functional development of distal vagina, fusion & membrane breakdown with Mullerian system

7

Pubertal events can be considered normal in females who are how young?

As early as 7-8 years of age

(Although, the lecture notes indicate that the presence of breasts and pubic hair in a girl prior to 8 years of age warrants evaluation. Any secondary sex characteristics prior to age 6 definitely warrants a thorough evaluation.)

8

  1. At what age does the absence of breasts or pubic hair warrant evaluation?
  2. At what age does the absence of menstruation warrant evaluation?

  1. Age 14
  2. Age 16

9

Breast development is ultimately the result of which hormone?

Knowing this, pathology of what system could cause issues for breast development?

Estrogen

Relies on intact hypothalamic-pituitary-gonadal axis

10

Define precocious puberty.

Occurrence of breasts, pubic/axillary hair, and/or menstruation prior to age 7-8 years.

11

What are the two major categories of precocious puberty?

  1. Gonadotropin independent
    • Pathology originates from outside of a normal hypothalamic-pituitary axis
  2. Gonadotropin dependent
    • Premature activation of the hypothalamic-pituitary-ovarian or -adrenal axis

12

Name ~4 causes of gonadotropin independent precocious puberty.

  • Granulosal, thecal, sertoli, or leydig cell tumors of the ovary
  • Adrenal tumors (benign & malignant)
  • Congenital adrenal hyperplasia
  • McCune Albright syndrome

13

Defects in which enzymes are implicated in congenital adrenal hyperplasia?

  • 21-hydroxylase
  • 11-hydroxylase

14

What are the features of McCune Albright syndrome?

  • café O lait spots
  • polyostotic ("of many bones") fibrous dyplasia
  • autonomous ovarian estrogen production
  • hyperthyroidism

15

What is the underlying pathogenesis of McCune Albright syndrome?

Aberrant activation of GDP system leading to increased enzymatic activity of multiple endocrine organs

16

While gonadotropin dependent precocious puberty is typically idiopathic, what is an important possible source to rule out?

CNS tumors

17

What are the major consequences of precocious puberty if not evaluated and treated?

  • At the least: very short height to due premature growth plate fusion
    • Chronic estrogen and/or androgen exposure
  • At the worst: treatable benign or malignant condition of the brain, adrenal gland, or ovary

18

What bones are typically used to determine the age of the epiphyseal plates?

Epiphyseal plates in the hand

19

Which hormones should be evaluated when the following pubertal events occur precociously?

  1. Breat development
  2. Pubic/axillary hair growth
  3. Menstruation

  1. Estrogen, FSH
  2. Testosterone, LH
  3. Estrogen, FSH, LH

20

A girl presents with Turner's syndrome.

  1. What organ is dysfunctional?
  2. Why?
  3. What hormone would be lacking secondary to the dysfunction?
  4. What pubertal events would be absent?

  1. Ovaries
  2. Lack of two functional X chromosomes - monosomy X, X/Y mosiacism, etc.
  3. Estrogen
  4. Menstruation, breast development (possibly stunted growth as well?)

21

In what age range should you always first rule out pregnancy?

Age 6(!)-60

22

What three imaging modalities are most useful for evaluating female reproductive abnormalities?

  • Ultrasound
  • CT
  • MRI

23

In a woman with a history of monthly menstrual cycles, absence of menstruation for how long warrants an evaluation?

3 consecutive cycles or 6 months

24

Estrogen is protective against what two major diseases?

  • Cardiovascular disease
  • Osteoporosis
  • (probably others, not mentioned)

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