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CLINICAL GUIDELINES FOR  EVALUATION AND MANAGEMENT OF AMENORRHEA Dr. JEHAD YOUSEF  FICS, FRCOG ALHAYAT ART CENTER AMMAN – JORDAN
Definitions ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
CNS-Hypothalamus-Pituitary Ovary-uterus Interaction Neural control Chemical control Dopamine (-) Norepiniphrine (+) Endorphines (-) Hypothalamus Gn-RH Ant. pituitary FSH, LH Ovaries  Uterus Progesterone Estrogen Menses – ± ?
Pathophysiology  of  Amenorrhea   ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Euestrogenic Anovulatory Amenorrhea ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Hypoestrogenic Anovulatory Amenorrhea ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
AMENORRHOEA  AN  APPROACH  FOR  DIAGNOSIS ,[object Object],[object Object],[object Object],Exclude Pregnancy Exclude Cryptomenorrhea
Cryptomenorrhea Outflow obstruction to menstrual blood -  Imperforate hymen - Transverse Vaginal septum with functioning uterus - Isolated Vaginal agenesis with functioning uterus - Isolated Cervical agenesis   with functioning uterus ,[object Object],[object Object],[object Object],[object Object],[object Object]
Imperforate hymen
Once Pregnancy and cryptomenorrhoea are excluded: The patient is a bioassay for Endocrine abnormalities   Four categories of patients are identified  1.  Amenorrhoea with absent or poor secondary sex Characters 2.  Amenorrhoea with normal 2ry sex characters 3.  Amenorrhoea with signs of androgen  excess 4.  Amenorrhoea with absent uterus and vagina
AMENORRHOEA Absent or poor secondary sex Characteristics FSH Serum level   Low / normal  High Hypogonadotropic hypogonadim  Gonadal dysgenesis
AMENORRHOEA Normal secondary sex Characteristics   ,[object Object],[object Object],[object Object],+ Bleeding No bleeing   Prolactin    TSH Further Work-up (Endocrinologist) - Mild hypothalamic dysfunction  - PCO (  LH/FSH) Review FSH result And history  (next slide)
FSH  Low / normal High  Hypothalamic-pituitary Failure  Ovarian failure  If < 25 yrs or primary amenorrhea    karyoptype   If < 35 yrs   R/O  autoimmune disease  ??  Ovarian biopsy head CT- scan or MRI - Severe hypothalamic dysfunction - Intracranial pathology
Amenorrhoea  Utero-vaginal absence Karyotype  46- XX Mullerian  Agenesis (MRKH syndrome) Androgen Insenitivity (TSF syndrome)  .  Gonadal regressioon . Testocular  enzyme  defenciecy .  Leydig cell agenisis 46- XY Normal breasts & sexual hair  Normal breasts & absent sexual hair Absent breasts & sexual hair
Normal FSH, LH; -ve bleeding history is suggestive of amenorrhea trumatica ,[object Object],[object Object],[object Object],[object Object],[object Object],Some  will prescribe a cycle  of  Estrogen and  Progesterone challenge Before HSG or Hysterescopy
Asherman’s  syndrome
Amenorrhoea Signs of androgen excess Testosterone, DHEAS, FSH, and LH DHEAS 500-700 mug/dL DHEAS >700 mug/dL TEST. >200 ng/dL  Serum 17-OH Progesterone level Late CAH   Adrenal  hyperfunction  U/S ? MRI or CT   Ovarian Or adrenal tumor Lower elevations    PCOS  (High LH / FSH)
Amenorrhoea PRIMARY AMENORRHOEA . Ovarian failure  36% . Hypogonadotrophic  34% Hypogonadism. . PCOS  17% . Congenital lesions (other than dysgenesis)  4% . Hypopituitarism  3% . Hyperprolactinaemia  3% . Weight related  3% SECONDARY AMENORRHOEA . Polycystic ovary syndrome  30% . Premature ovarian failure  29% . Weight related amenorrhoea  19% . Hyperprolactinaemia  14% . Exercise related amenorrhoea  2% . Hypopituitarism  2%
Gonadal dysgeneis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Gonadal dysgenesis XO/XY 46- XX(Pure) 46-XY (Swyer) XX/XO  or abnormal  X XO Karyotype ± Nil  ± Classical  Somatic stigmata  Short  Tall  -  Short  - Normal  Short  Hight  -  Streak  - Testes Streak  Streak  Streak  Gonad  Ambiguous  Female  Female  Female  Phenotype Mixed Dysgenesis  True  gonadal  Dysgenesis  Turner Variant  Classic  Turner’s
Turner ’s syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Mosaic (46-XX / 45-XO)  ( Classic 45-XO)  Turner ’s syndrome
Ovarian dysgenesis
None-dysgenesis ovarian failure ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Premature ovarian failure ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Polycystic ovary syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object]
Polycystic ovary syndrome
Hypogonadotrophic Hypogonadism ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Constitutional pubertal delay ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Sheehan ’s syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Weight-related amenorrhoea Anorexia Nervosa ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Exercise-associated amenorrhoea ,[object Object],[object Object],[object Object]
Contraception related amenorrhea ,[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Late onset congenital adrenal hyperplasia
Cushing’s syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Utero-vaginal Agenisis   Mayer-Rokitansky-Kuster-Hauser syndrome ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Androgen insensitivity Testicular feminization syndrome   ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
General Principles of management of Amenorrhoea ,[object Object],[object Object],[object Object],[object Object],[object Object]
Hormonal treatment Primary Amenorrhea with absent secondary sexual characteristics ,[object Object],[object Object],[object Object],[object Object],[object Object]
Summary  ,[object Object],[object Object],[object Object]
THANK YOU FOR  YOUR  ATTENTION  Dr. Jehad Yousef F.I.C.S, F.R.C.O.G

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amenorrhea

  • 1. CLINICAL GUIDELINES FOR EVALUATION AND MANAGEMENT OF AMENORRHEA Dr. JEHAD YOUSEF FICS, FRCOG ALHAYAT ART CENTER AMMAN – JORDAN
  • 2.
  • 3. CNS-Hypothalamus-Pituitary Ovary-uterus Interaction Neural control Chemical control Dopamine (-) Norepiniphrine (+) Endorphines (-) Hypothalamus Gn-RH Ant. pituitary FSH, LH Ovaries Uterus Progesterone Estrogen Menses – ± ?
  • 4.
  • 5.
  • 6.
  • 7.
  • 8.
  • 10. Once Pregnancy and cryptomenorrhoea are excluded: The patient is a bioassay for Endocrine abnormalities Four categories of patients are identified 1. Amenorrhoea with absent or poor secondary sex Characters 2. Amenorrhoea with normal 2ry sex characters 3. Amenorrhoea with signs of androgen excess 4. Amenorrhoea with absent uterus and vagina
  • 11. AMENORRHOEA Absent or poor secondary sex Characteristics FSH Serum level Low / normal High Hypogonadotropic hypogonadim Gonadal dysgenesis
  • 12.
  • 13. FSH Low / normal High Hypothalamic-pituitary Failure Ovarian failure  If < 25 yrs or primary amenorrhea  karyoptype  If < 35 yrs  R/O autoimmune disease ?? Ovarian biopsy head CT- scan or MRI - Severe hypothalamic dysfunction - Intracranial pathology
  • 14. Amenorrhoea Utero-vaginal absence Karyotype 46- XX Mullerian Agenesis (MRKH syndrome) Androgen Insenitivity (TSF syndrome) . Gonadal regressioon . Testocular enzyme defenciecy . Leydig cell agenisis 46- XY Normal breasts & sexual hair Normal breasts & absent sexual hair Absent breasts & sexual hair
  • 15.
  • 17. Amenorrhoea Signs of androgen excess Testosterone, DHEAS, FSH, and LH DHEAS 500-700 mug/dL DHEAS >700 mug/dL TEST. >200 ng/dL  Serum 17-OH Progesterone level Late CAH Adrenal hyperfunction U/S ? MRI or CT Ovarian Or adrenal tumor Lower elevations  PCOS (High LH / FSH)
  • 18. Amenorrhoea PRIMARY AMENORRHOEA . Ovarian failure 36% . Hypogonadotrophic 34% Hypogonadism. . PCOS 17% . Congenital lesions (other than dysgenesis) 4% . Hypopituitarism 3% . Hyperprolactinaemia 3% . Weight related 3% SECONDARY AMENORRHOEA . Polycystic ovary syndrome 30% . Premature ovarian failure 29% . Weight related amenorrhoea 19% . Hyperprolactinaemia 14% . Exercise related amenorrhoea 2% . Hypopituitarism 2%
  • 19.
  • 20. Gonadal dysgenesis XO/XY 46- XX(Pure) 46-XY (Swyer) XX/XO or abnormal X XO Karyotype ± Nil ± Classical Somatic stigmata Short Tall - Short - Normal Short Hight - Streak - Testes Streak Streak Streak Gonad Ambiguous Female Female Female Phenotype Mixed Dysgenesis True gonadal Dysgenesis Turner Variant Classic Turner’s
  • 21.
  • 22. Mosaic (46-XX / 45-XO) ( Classic 45-XO) Turner ’s syndrome
  • 24.
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  • 40.
  • 41. THANK YOU FOR YOUR ATTENTION Dr. Jehad Yousef F.I.C.S, F.R.C.O.G