Amenorrhea

How do you diagnose secondary amenorrhea?

1) ALways begin with a BhCG assay to rule out pregnancy. 2) Then the questions asked should focus on hypothyroidism (e.g., lethargy, weight gain, cold intolerance), hyperprolactinemia (e.g., nipple discharge, usually bilateral), and hyperandrogenism (e.g., recent changes in hirsutism, acne, or virilism) 3) TSH and prolactin levels should then be checked to rule out hypothyroidism and hyperprolactinemia.

What common anatomic causes of secondary amenorrhea?

Ashermann syndrome - presence of intrauterine synechiae or adhesions, usually seondary to intrauterine surgery (dulation and curretage, myomectomy, cesarean delivery, endometritis) or infection Cervical stenosis - usually caused by scarring of the cervical os secondary to surgical or obstetric trauma

What inhibits prolactin release naturally? Drugs? What stimulates prolactin release naturally? Drugs?

Dopamine/ Serotonin and TRH/ Dopamine antagonists (Haldol, Reglan, phenothiazine), TCAs, estrogen, MAO inhibitors, and opiates

Why do adolescents after their first menarche have anovulatory cycles?

HPO axis is not mature

The differential diagnosis of primary amenorrhea includes outflow tract obstruction, end organ disorders, and central regulatory disorders. Discuss central regulatory disorders.

Hypothalamic Disorders Kallmann syndrome - a form of hypogonadotropic hypogonadism, which involves the congenital absence of GnRH and is commonly associated with anosmia. In Kallmann syndrome, the normal migration of the GnRH neurons are disrupted in their travel from the olfactory placode to the hypothalamus, and the olfactory bulbs also do not form, leading to this combination of hypogonadotropic hypogonadism and anosmia. Pituitary Disorders - Pituitary dysfunction is usually secondary to hypothalamic dysfunction. IT may be caused by tumors, infiltration of the pituitary gland, or infarcts of the pituitary.

Any patient with an elevated prolactin level should have what study done?

Imaging study to rule out prolactinoma

How can you differentiate between a hypothalamic-pituitary disorder and ovarian failure?

Measure of FSH and LH Hypothalamic-pituitary disorder (low/normla FSH and LH levels) Ovarian failure (high FSH and LH levels)

Uterus is dependent on? Breast development is dependent on . Pubic hair is dependent on?

No MIF estradiol secretion by the ovaries androgen

The differential diagnosis of primary amenorrhea includes outflow tract obstruction, end-organ disorders, and central regulatory disorders. Discuess outflow tract obstruction.

Outflow tract obstruction Imperforate hymen - failure of hymen to canalize, remaining a solid membrane across the vaginal inroitus. It does not allow egress of menstrual flow -> pelvic or abdominal pain. Transverse vaginal septum - failure of the Mullerian derived upper vagina to fuse with the urogenital sinus-derived lower vagina -> cyclic pelvic pain End-organ disorders Vaginal agenesis (Mayer-Rokitansky-Kuster-Hauser) - Mullerian agenesis or dysgensis Testicular feminization - androgen insensitivity syndrome - dysfunction or absence of the testoerone receptor that leads to a phenotypical female with 46 XY chromosomes. Because these patients have testes, Mullerian inhibitory factor is secreted early in development, and these patients therefore have an absence of all Mullerian derived structures. The testes may be undescended or may have migrated down to the labia majora. THere is an absence of pubic and axillary hair. Usually estrogen is produced, and these patients develop breatss but present with primary amenorrhea because they have no uterus.

The differential diagnosis of primary amenorrhea includes outflow tract obstruction, end organ disorders, and central regulatory disorders. Discuss end-organ disorders.

Ovarian failure - results in low levels of estradiol but elevated levels of gonadotropins termed hypergonadotropic hypogonadism.

What is the most common cause of secondary amenorrhea?

Pregnancy

Withdrawal bleeding after the progesterone challenge indicates what? Absence of withdrawal bleeding in response to progesterone alone leads to what test?

Presence of estrogen and an adequate outflow tract. In this case, amenorrhea is usually secondary to anovulation, which can be caused by a variety of endocrine disorders that alter pituitary/gonadal feedback such as polycystic ovaries, tumors of the ovary and adrenals, Cushing syndrome, thyroid disorders, and adult onset adrenal hyperplasia. Evaluation with estrogen and progesterone administration. If there is still no menstrual bleeding, an outflow tract disorder suh as Asherman syndrome or cervical stenosis is suspected. If menstrual bleeding does occur in response to estrogen and progesterone administration, this suggests an intact and functional uterus without adequate endogenous estrogen stimulation.

The differential diagnosis of primary amenorrhea includes outflow tract obstruction, end organ disorders, and central regulatory disorders. Discuss end-organ disorders.

Primary Ovarian failure - results in low levels of estradiol but elevated levels of gonadotropins termed hypergonadotropic hypogonadism. Types include - Savage syndrome - failure of the ovaries to respond to LH and FSH secondary to a receptor defect. Turner syndrome (45, XO) - ovaries undergo rapid atresia that by puberty they are usually no primordial oocytes. Gonadal Agensesis with 46, XY chromosomes - defect in 17 a hydoxylase or 17, 20 desmolase - leads to absent testerone production. However, MIF will still be produced; hence there will be no female internal reproductive organs. These patients will be otherwise phenotypically female, usually without breast development. Swyer syndrome - Because testes never develop, MIF is not released and these patients have both internal and external female genitalia. However, eithout estrogen they will not develop breasts.

What is primary amenorrhea? What is secondary amenorrhea?

The absence of menarche (first menses) by age 16 or no menstruation by 4 years after thelarche (the onset of breast development). Absence of menses for three menstrual cycles or a total of 6 months in women who have previosuly had normal menstruation.

What is amenorrhea? How is classified?

The absence of menses - is classified as either primary or secondary.

What is secondary amenorrhea?

The absence of menses for more than 6 months or for the equivalent of three menstrual cycles in a woman who previously had mentrual cycles.

What are the stages of puberty?

Thelarche Adrenarche Growth spurt Menarche

How can tranvserve vaginal septum be differentiated from imperforate hymen?

Transverse vaginal septum has a hymenal ring below the septum whereas imperforate hymen does not.

How do you diagnose primary amenorrhea?

Uterus present -> Patent vagina -> breasts present -> progesterone challe. -> Disruption of hypothalamic-pituitary axis, hypothalamic, pituitary, or ovarian pathogenesis similar to that of secondary amenorrhea, congenital abnormalities of the genital tract Uterus present -> Patent vagina -> No breasts -> gonal failure/agenesis in 46, XX Uterus present -> Non patent vagina -> imperforate hymen, transverse vaginal septum, vaginal agenesis Uterus absent -> breasts present -> karyotype -> enzyme deficiences in testosterone synthesis, testicular feminization, mullerian agenesis or MRKH Uterus absent -> breasts absent -> gonadal agenesis in 46, XY

How is PCOS diagnosed?

When women meet two of three of the following: oligo or anovulation, clinical or laboratory evidence of hyperandrogenism, and polycystic ovaries on ultrasound.

What are other causes of secondary amenorrhea besides anatomic causes?

ovarian failure - ovariant orsion, surgery, infection, radiation, or chemotherapy PCOS - Excessive hair growth (hirsutism) and male pattern hair loss as well as acne. Hyperprolacinemia - excess prolactin leads to amenorrhea and galactorrhea Disruption of the hypothalamic-pituitary axis - causes hypogonadtotropic hypogonadism. Common causes include stress, exercise, anorexia nervosa, and weight loss.