Puberty and Disorders of Development Flashcards
Describe the follicular and luteal phases
Follicular: begins with onset of menses and ends in preovulatory surge of LH
Luteal: begins with onset of the preovulatory LH surge and ends first day of menses
What leads to an increase in FSH by negative inhibition?
Decreasing levels of estradiol and progesterone from the regressing corpus luteum
What is the “2 cell theory of ovarian follicular development and estrogen production”?
LH stimulates the theca cells - production of androgens
FSH stimulates the granulosa cells - converts androgens to estrogens
What causes the fall of LH and FSH in the luteal phase?
What happens if conception does not happen?
Negative feedbcak by estrogen and progesterone
Progesterone and estrogen decline and result in regression of corpus luteum. FSH will rise and initiate new follicular growth.
When do estrogens increase in the ovarian cycle? (2)
Approx. 1 week prior to ovulation (1 day before LH surge)
Again 6-7 days after ovulation, then returns to baseline before menses
What are the 2 sources of progesterone in the ovarian cycle?
Peripheral conversion of precursors
Graafian follicle (corpus luteum about 5-7 days after ovulation)
*ovary only secretes a little bit
What happens in the functionalis and basalis layers of the endometrium?
Functionalis: undergoes changes in mentruation and has spiral aa.
Basalis: remains unchanged and provides stem cells and contains basal aa.
What is the only phase of the menstrual cycle that is seen externally?
Mentrual phase
Median age of menarche
How close to telarche?
12.43 y/o
2-3 years post telarche at Tanner stage IV
Primary amenorrhea
No menstruation by 13 y/o without secondary sex characteristics OR by age 15 with secondary characteristics
How long is the first menstrual cycle?
How long are they in the first year?
Menstrual flow length?
How many tampons?
Mean volume of blood?
34 days
21-45 days
7 days or less
3-6 pads/day
30cc (>80cc is associated w/ anemia, especially if > 7 days)
Mean onset of puberty:
12.4 y/o (10-16 y/o)
How is puberty inhibited the prepubertal stage? (2)
- Gonadostat sensitivity to the negative feedback of low circulating estrogen
- Intrinsic CNS inhibition of GnRH secretion
What causes growth of axillary and pubic hair (adrenarche or pubarche)?
Androgens
Stages of normal pubertal development
TAPME
Thelarche (breast): first signs, UL development is normal, requires estrogen
Adrenarche/pubarche: occurs 2 years earlier in female and 1 year prior to onset of menses
Menses: requires GnRH from hypothalamus, FSH and LH from pituitary, estrogen and progesterone from ovaries
Tanner staging in pubic hair (5)
- Preadolescent: absence
- Sparse hair along labia; downy with slights pigment
- Hair spreads over junction of pubes; hair is coarser or darker
- Adult-type hair; no spread to medial thighs
- Adult-type with spread to medial thighs; inverted triangle pattern
What constitutes precocious puberty?
75% of time it is…
Development of any sign of secondary sex characteristics prior to 2.5 SDs earlier than expected age
Idiopathic
Heterosexual precocious puberty =
Causes (3)
Development of secondary sex characteristics in the opposite gender.
Virulization (Sertoli-Leydig tumors, etc.)
Congenital adrenal hyperplasia (CAD)
Exposure to exogenous angrogens
Isosexual precocious puberty =
Cause
Premature sexual maturation appropriate for the phenotype.
Constitutional and organic brain disease: tumors, trauma, infection
Enzyme associated with CAD =
21-hydroxylase, which leads to excess androgen production
True vs. Pseudoisosexual precocity
True: arises from premature activation of normal development by HPO axis
Pseudoisosexual: exposure to estrogens independent of HPO axis (estrogen producing tumors)
How is true isosexual precocious puberty diagnosed?
Treatment?
Give exogenous GnRH (GnRH stimulation test) and see a resulatnt rise in LH consistent with girls in puberty
GnRH agonist, which suppresses release of FSH and LH
Puberty is considered delayed when… (4)
Secondary sexual characteristics have not appeared by age 13
If thelarche has not occurred by 14 y/o
No menarche by 15-16 y/o
Menses has not happened by 5 years after onset of thelarche
3 categories for delayed puberty
Hypergonadotropic hypogonadism (FSH > 30): Turner’s syndrome
Hypogonadotropic hypogonadism (FSH+LH < 10): all else
Anatomic causes: Mullerian agenesis, imperforate hymen, transverse vaginal septum
Secondary amenorrhea
Patient with prior menses has been absent for 6 mo. or more
3 diseases/processes with primary amenorrhea with breast development and Mullerian anomalies
Androgen insensitivity syndrome (AIS): 46XY, male levels of testosterone, defect in androgen receptor, testes in abdominal wall.
Mullerian agensis (MRKH syndrome): primary amenorrhea, breast development, female levels of testosterone and 46XX karyotype.
Outflow tract obstruction: normal uterus, but imperforate hymen or transverse vaginal septum
What 2 hormones are normal and abnormal in thyroid disease associated with secondary amenorrhea?
Mild hypothyroidism is associated with…
NL prolactin
ABN TSH
Hypermenorrhea or oligomnorrhea (not amenorrhea) - treatment should restore menses
Abnormal prolactin levels can lead to…
Most common symptom?
Secondary amenorrhea
Galactorrhea
What is done to test for secondary amenorrhea if TSH and prolactin levels are WNL?
Progesterone challenge test (PCT)
+ test = bleeding (PCOS most common)
- test = no bleeding
What are 2 anatomic causes of secondary amenorrhea?
Asherman syndrome: scarring/contractures of uterus
Cervical stenosis: narrowing of the cervix
What is a common associated with PCOS?
Insulin sensitivity, leading to insuling hypersecretion
How is PCOS diagnosed? (2 of 3)
Need 2 of 3:
- Oligomenorrhea or amenorrhea
- Biochemical or clinical signs of hyperandrogenism (LH to FSH 2:1)
- US revealing cysts beneath cortex of ovary
2 major treatments of PCOS
Weight loss
OCs - suppresses FSH/LH which decreases overproduction of androgens in the ovary. Estrogen stimulates SHBG.
Hisutism =
Virulization =
Hisutism = excess terminal hair in a male pattern distribution
Virulization = masculinization of female associated structures with an increase in circulating testosterone
Polymenorrhea
Menorrhagia
Metrorrhagia
Menometroraggia
Oligomenorrhea
Polymenorrhea - abnormally frequent menses < 21 days
Menorrhagia - excessive and/or prolonged bleeding at normal intervals (>80mL and >7 days)
Metrorrhagia - irregular episodes of uterine bleeding
Menometroraggia - heavy and irregular uterine bleeding
Oligomenorrhea - menstrual cycles occurring > 35 days but less than 6 mo.
What is PALM COEIN?
Acronym for DUB causes.
PALM = structural causes Polyp Adenomyosis Leiomyosis Malignancy and hyperplasia
COEIN = nonstructural cases Coagulopathy (von Willebrand disease) Ovulatory dysfunction (PCOS) Endometrial (infection) Iatrogenic (IUD, IUS, exogenous hormones) Not yet classified
Lab tests to evaluate if there is abnormal uterine bleeding (5)
Pregnancy test CBC Targeted screening for bleeding disorders TSH Chlamydia trachomatis
AUB treatment if:
Massive bleeding
Moderate bleeding
If unresponsive to conservative therapy
Massive bleeding - hospitalization and transfusions if unstable; 25 mg conjugated estrogens, then hormonal treatment
Moderate bleeding - combination OCPs
If unresponsive to conservative therapy - D and C, polypectomy, myomectomy, endometrial ablation, hysterectomy
