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Practical approach to amenorrhea warda

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this is a simple practical approach to the diagnosis of a case of amenorrhea for the general gynecologist.

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Practical approach to amenorrhea warda

  1. 1. Practical Approach to Amenorrhea Osama M Warda, MD Professor of Obstetrics & Gynecology Mansoura University-Egypt Osama Warda 1
  2. 2. Definition Osama Warda 2 Primary amenorrhea is diagnosed if; (1) the absence of menstruation by the age of 14 years in the absence of growth or development of secondary sex characters, or (2) No menstruation by the age of 16 with or without growth or development of secondary sex characters.
  3. 3. Definition Secondary amenorrhea: is the absence of menstruation for 6 months or more or for a period of time equivalent to that of previous 3 consecutive cycles in a woman who was previously menstruating. Osama Warda 3
  4. 4. Background Development of the breast and feminine constitution depends on E2 secreted by the granulosa cells of the developing ovarian follicles. E2 exerts its action via estrogen receptors in the target organ. The uterus (and most genital tract) develops from the mullerian ducts in the female fetus with 46xx karyotype. The external male genitalia develps in 46xy embryo under the effect of dihydrotestosterone exerting its action on its specific androgen receptors in the target organs. Osama Warda 4
  5. 5. CLINICAL APPROACH TO A CASE OF PRIMARY AMENORRHEA The patient is one of four: 1. Breast absent- uterus present group. 2. Breast developed- uterus absent group. 3. Breast absent- uterus absent group. 4. Breast developed- uterus present group. Osama Warda 5
  6. 6. 1- BREAST ABSENT- UTERUS PRESENT Osama Warda 6 -BREASTABSENT- UTERUSPRESENT (i.e.NoE2) [SERUMFSH-ESTIMATION] -BREASTABSENT- UTERUSPRESENT (i.e.NoE2) [SERUMFSH-ESTIMATION] HIGH SERUM FSH HYPERGONADOTROPIC HYPOGONADISM [DEFECT IN OVARY] KARYOTYPING IS DONE: HIGH SERUM FSH HYPERGONADOTROPIC HYPOGONADISM [DEFECT IN OVARY] KARYOTYPING IS DONE: NORMAL KARYOTYPE [46XX] GONADAL DYSGENESIS NORMAL KARYOTYPE [46XX] GONADAL DYSGENESIS ABNORMAL KARYOTYPE [ 45XO] TURNER'S SYNDROME ABNORMAL KARYOTYPE [ 45XO] TURNER'S SYNDROME LOW SERUM FSH HYPOGONADOTROPIC HYPOGONADISM DEFECT IN HYPOTHALAMUS OR PITUITARY LOW SERUM FSH HYPOGONADOTROPIC HYPOGONADISM DEFECT IN HYPOTHALAMUS OR PITUITARY HYPOTHALAMIC: -KALLMAN SYNDROME -FROLICH SYNDROME - LAURANCE-MOON-BIEDLE - POSTPILL AMENORRHEA HYPOTHALAMIC: -KALLMAN SYNDROME -FROLICH SYNDROME - LAURANCE-MOON-BIEDLE - POSTPILL AMENORRHEA PITUITARY: -CHIARI-FROMMEL SYNDROME -DELCASTELLO SYNDROME -LEVI-LORIAN SYNDROME PITUITARY: -CHIARI-FROMMEL SYNDROME -DELCASTELLO SYNDROME -LEVI-LORIAN SYNDROME
  7. 7. PRIMARY AMENORRHEA Osama Warda 7 L-M-B- syndrome
  8. 8. 2- BREAST DEVELOPED- UTERUS ABSENT Only in 2 cases; androgen insensitivity syndrome (testicular feminization), and Müllerian agenesis: Osama Warda 8 Item Androgen insensitivity Mullerian agenesis 1- Axillary & pubic hair Absent Present 2- Serum testosterone Male level Female level 3- Karyotyping * 46XY (most important) 46XX 4- Gonads Testes (mostly inguinal) Normal ovaries 5- Fertility Impossible Possible via surrogate uterus (she will be the genetic mother) 6- Gonadectomy Indicated before 25 years age (protect against malignancy) Contraindicated 7- Inheritance X-linked Not heriditary
  9. 9. 3-BREAST ABSENT- UTERUS ABSENT - This is a very rare condition. - Karyotype is 46XY ( i.e. genitically males )in all. - It is due to enzymatic deficiency such as: [a]. 17, 20 desmolase deficiency, [b]. 17 alpha- hydroxylase deficiency in 46XY individuals, and [c]. Agonadism. - Those patients do not respond to exogenous estrogen replacement to help development of secondary sex characters such as breast development, hence the feminine constitution. Osama Warda 9
  10. 10. 4- BREAST DEVELOPED- UTERUS DEVELOPED Osama Warda 10 4.BREASTDEVELOPED- UTERUSPRESENT [LOCALGYNECOLOGICAL EXAMINATIONISDONE] NO OUTFLOW TRACT OBSTRUCTION [SERUM FSH, LH, & PRL ] 1. PITUITARY ADENOMA [↑ PRL] 2. PCOS [LH/FSH ratio > 3] 3. RESISTANT OVARY SYNDROME [ ↑ FSH] 4. CONSTITUTIONAL DELAY OUTFLOW TRACT OBSTRUCTION CRYPTOMENORRHEA [Imperforate hymen or transverse vaginal septum or cervical atresia]
  11. 11. Osama Warda 11 Imprforate hymen PCOS
  12. 12. APPROACH TO A CASE OF SECONDARY AMENORRHEA The 1st step is to exclude pregnancy The second step ( if not pregnant) is to evaluate Prolactin and TSH>>>>> If both revealed normal, test the endometrial responsiveness by progestin withdrawal test….. Osama Warda 12
  13. 13. HCG testing negative (no pregnancy) High PRL : - Prolactinoma - Drug induced - Thyroid disease High TSH hypothyroidism Normal TSH & PRL: Proceed to progestin challenge test Request for serum PRL and TSH Osama Warda 13 NEXTSLIDE SECONDARY AMENORRHEA
  14. 14. Progestin challenge test Negative withdrawal bleeding Request FSH Low FSH: Give cyclic E& P High FSH= Ovarian failure Positive withdrawal bleeding - PCOS - Idiopathic anovulation - If virilization (request testosterone, DHEA-S, 17OHP- +VE BLEEDING= HYPOGONADOTROPIC - -VE BLEED= ASHERMAN Osama Warda 14 Secondary Amenorrhea
  15. 15. Thank You Osama Warda 15

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