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Master the board step 3

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<strong>Master</strong> <strong>the</strong> Boards: USMLE Step 3<br />

Hypothalamic-Pituitary Failure<br />

This is <strong>the</strong> diagnosis when <strong>the</strong>re are no sexual characteristics but <strong>the</strong> uterus is<br />

normal on ultrasound and FSH levels are low. It may be due to stress, excessive<br />

exercise, or anorexia nervosa. Kallmann syndrome is <strong>the</strong> likely diagnosis<br />

when <strong>the</strong> case also describes anosmia (hypothalamus doesn’t produce GnRH).<br />

CNS imaging (CT head) will rule out a brain tumor.<br />

Management involves estrogen and progesterone replacement for development<br />

of secondary sexual characteristics.<br />

Secondary Amenorrhea<br />

This is diagnosed when one of <strong>the</strong> following conditions presents:<br />

··<br />

Regular menses are replaced by an absence of menses for 3 months.<br />

··<br />

Irregular menses are replaced by an absence of menses for 6 months.<br />

Steps in <strong>the</strong> Workup of Secondary Amenorrheap<br />

Steps in <strong>the</strong> Workup<br />

1. Pregnancy Test<br />

(ß-hCG)<br />

2. Thyrotropin (TSH) (rule<br />

out hypothyroidism)<br />

3. Prolactin (rule out<br />

elevation)<br />

4. Progesterone<br />

Challenge Test (PCT)<br />

5. Estrogen–Progesterone<br />

Challenge Test (EPCT)<br />

Next Step in Management<br />

An elevated TRH in primary hypothyroidism → ↑ prolactin.<br />

Treat hypothyroidism with thyroid replacement for rapid restoration of menstruation<br />

If elevated:<br />

1) Review medications: Antipsychotics and antidepressants have antidopamine side effect<br />

→ ↑ prolactin<br />

2) CT or MRI of head to rule out pituitary tumor<br />

– Tumor < 1 cm: give bromocriptine (dopamine agonist)<br />

– Tumor > 1 cm: treat surgically<br />

3) If <strong>the</strong> cause of elevated prolactin is idiopathic, treat with bromocriptine.<br />

• Positive PCT: Any withdrawal bleeding is diagnostic of anovulation.<br />

– Treatment: Cyclic progesterone to prevent endometrial hyperplasia. Clomiphene<br />

ovulation induction is done if pregnancy is desired.<br />

• Negative PCT: Inadequate estrogen or outflow tract obstruction<br />

• 3 weeks of oral estrogen followed by 1 week of progesterone<br />

• Positive EPCT: Any withdrawal bleeding is diagnostic of inadequate estrogen. Next <strong>step</strong> is<br />

to get an FSH level.<br />

– ↑ FSH is ovarian failure. Y chromosome mosaicism may be <strong>the</strong> cause if patient is < 25<br />

years. Order a karyotype for confirmation.<br />

– ↓ FSH is hypothalamic–pituitary insufficiency. Order a brain CT/MRI to rule out<br />

a tumor. Give estrogen-replacement <strong>the</strong>rapy to prevent osteoporosis and cyclic<br />

progestins to prevent endometrial hyperplasia.<br />

• Negative EPCT: Diagnostic of an outflow tract obstruction or endometrial scarring<br />

(e.g., Asherman syndrome). Order a hysterosalpingogram to identify <strong>the</strong> lesion.<br />

Management: Adhesion lysis followed by estrogen stimulation of <strong>the</strong> endometrium. An<br />

inflatable stent prevents re-adhesion of <strong>the</strong> uterine walls.<br />

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