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AMENORRHEA

Criseline D. Tolentino, MD, DPOGS, FPSSTD

MENSTRUAL CYCLE (REVIEW)

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o Gonadotropin control of the ovarian and endometrial cycles.
o The ovarian-endometrial cycle has been structured as a 28-day cycle.
o The follicular phase (days 1-14) is characterized by rising levels of estrogen, thickening of the
endometrium, and selection of the dominant “ovulatory” follicle.
o During the luteal phase (days 14-21), the corpus luteum (CL) produces estrogen which prepare the
endometrium for implantation.
o If implantation occurs, the developing blastocysts will begin to produce hCG and rescue the CL, thus
maintaining progesterone production.

HYPOTHALAMUS-PITUTARY-OVARIAN AXIS

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NORMAL FEMALE PUBERTAL DEVELOPMENT

PRIMARY AMENORRHEA
 Absence of menses (IN WOMEN) by 16 years of age in the presence of normal secondary sexual
characteristics.
 Absence of menses (IN WOMEN) by 14 years of age when there is no visible secondary sexual
characteristics.

SECONDARY AMENORRHEA
 Absence of menstruation for three normal menstrual cycles of six months after menarche.

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EVALUATION OF PRIMARY AMENORRHEA

History and PE completed For a patient with primary amenorrhea

Secondary sexual characteristic present

No Yes

Measure FSH and LH levels perform UTZ of uterus

FSH and LH <5 IU/L FSH >20 IU/L uterus absent/abnormal uterus present/Normal
LH >40 IU/L

Hypognadotropic hypergonadotropic karyotype analysis outflow obstruction


Hypogonadism hypogonadism

karyotype analysis
46XY 46XX No Yes

46XY 46XX evaluate for imperforate hymen


Secondary or transverse
Amenorrhea vaginal septum
Androgen mullerian
Insensitivity agenesis
syndrome

premature turner’s
ovarian syndrome
failure

HYPERGONADOTROPIC HYPOGONADISM (GONADAL FAILURE)


 Elevated levels of FSH and LH that result from decreased negative feedback mechanism.
1. Gonadal Dysgenesis
 Turner’s Syndrome (45X)
o Is the most common abnormality causing gnadal failure and primary amenorrhea
2. Partial deletions of X Chr
3. Mosaicism (45X, 46XY)
4. Pure Gonadal Dysgenesis (46XX, 46XY)
5. 17 £ hydroxylase with 46XX

HYPOGONADOTROPIC HYPOGONADISM
 When the hypothalamus fails to secrete adequate amounts of GnRH
 When the pituitary disorder associated with inadequate production or release of pituitary gonadotropins
is present.
1. Physiology delay
o Most common manifestation
o Delayed reactivation of the GnRH pulse generation
2. Kallman’s Syndrome
o 2nd most common
o Insufficient pulsatile secretion of GnRH
3. Central Nervous System Tumor
o Most common is craniopharyngioma

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TREATMENT
 Cyclic estrogen and progesterone therapy
 0.625 mg/day of Estradiol
 Progestin (MPA 5-10mg)
 Should be added for the first 12 days of the month
 If 17 £ hydroxylase deficiency is confirmed, corticosteroid replacement is instituted as well as estrogen
and progestin

MAYER ROKITANSKY-KUSTER-HAUSSER SYNDROME


o 2nd most frequent cause of primary amenorrhea
o Absence of the uterus (cervix) and/ or vagina

ANDROGEN INSENSITIVITY
o Phenotypically female, genotypically male (XY)
o Defect in the androgen receptor gene located in the X Chr
o Anti mullerian hormone is present
o Testes, rather tha ovaries, arepresent in the abdomen

GUIDELINES OR PROGESTERONE AND ESTROGEN/ PROGESTOGEN CHALLENGE TEST


Drug Dosing Duration
Progestogen Challenge Test
MPA (Provera) 10 mg PO, OD 7-10 days
Norethindrone (Aygestin) 5 mg PO, OD 7-10 days
progesterone 200 parenteral, OD Single dose
Progesterone micronized 400 mg PO, OD 7-10 days
Progesterone micronized gel (4-8%) Intravaginally every other day Six applications
Estrogen / Progesterone Challege test
Estrogen / Progesterone (Prevnarin) 1.25 mg PO, OD 21 days
or
Estrdiol (Estrace) 2 mg PO, OD 21 days
followed by
Progestational agent As noted above

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EVALUATION OF SECONDARY AMENORRHEA

Patient presenting with secondary amenorrhea, negative pregnancy test

Check TSH and Prolactin levels

Both normal normal prolactinlevels normal TSH


Abnormal TSHlevels abnormal Prolactin levels

Progestogen thyroid disease PRL ≤100 ng/ml PRL >100 ng/ml


Challenge test 100 mcg/L

Withdrawal bleed no withdrawal bleed consider other causes MRI to evaluate


prolactinoma
Normogonadotropic estrogen/progestogen
Hypogonadism challenge test negative MRI

Withdrawal bleed no withdrawal bleed consider other


causes
Check FSH and LH outflow obstruction

FSH >20 IU/L FSH and LH <5 IU/L


LH >40 IU/L

Hypergonadotropic perform MRI to evaluate


Hypogonadism for pituitary tumor

Normal MRI, hypogonadotropic


Hypogonadism

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