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Amenorrhea

Amenorrhea. Dr. Maysa’ Khadra A/Professor in Reproductive Endocrinology and Infertility Dept of Obstetrics and Gynaecology University of Jordan; Jordan University Hospital. Amenorrhea definition. Primary:

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Amenorrhea

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  1. Amenorrhea Dr. Maysa’ Khadra A/Professor in Reproductive Endocrinology and Infertility Dept of Obstetrics and Gynaecology University of Jordan; Jordan University Hospital

  2. Amenorrhea definition • Primary: • No period by age 14 in the absence of growth or development of secondary sexual characteristics. or • No period by age 16 regardless of the presence of normal growth and development with the appearance of secondary sexual characteristics.

  3. Amenorrhea definition • Secondary: • In a woman who is menstruating , it is the absence of periods for a length of time equivalent to a total of at least three previous cycle intervals or six months of amenorrhea • Oligomenorrhea is defined as an interval of more than 35 days between periods

  4. differential diagnosis of amenorrhoea • Disorders of outflow • Disorders of the ovary • Disorder of the anterior pituitary • Disorders of the central nervous system

  5. Disorders of outflow • Asherman’s syndrome • Mullerian Anomlies • Cervical stenosis • Imperforate hymen • Transverse vaginal septum • Mullerian agenesis • Vaginal and uterine aplasia- Rokitansky Kuster Hauser syndrome • Androgen insensitivity

  6. Cyclical abdominal pain • urinary retention 37-60% • back pain • Constipation • distended bluish membrane at the introitus

  7. 46XY karyotype, X-linked recessive • mutations in AR, the gene for the human Androgen Receptor, located at Xq11-12 • Undervirilization and/or infertility in XY persons • undescended testis in abdomen or inguinal canals • No uterus or vagina-AMH • no prostate or internal male gentalia • No pubic or axillary hair • normal breast development • 25% chance of developing benign testicular tumors and a 4-9% chance of malignancy.

  8. Disorders of the ovary • Streak ovaries • Turner’s syndrome xo or mosaicism • XY Gonadal dysgenesis • Resistant ovary syndrome • Premature ovarian failure • Polycystic ovarian syndrome • hormone secreting ovarian tumors

  9. Genetic mosaicism (46XX/45XO) is most often implicated, nondisjunction (45XO) and partial monosomy (46XX ) • XO : primary amenorrhea • Mosaic: premature ovarian failure, • streak gonads and infertility • Horse shoe kidneys-HTN • Hypothyroidism- Hashimoto’s • risk of gonadoblastoma (45 XO/46 XY)

  10. Gonadal dysgenesis • It is characterized by a progressive loss of primordial germ cells on the developing gonads of an embryo. This loss leads to extremely hypoplastic and disfunctioning gonads mainly composed of fibrous tissue, hence the name streak gonads. • absence of both Mullerian inhibiting factor and testosterone • regression of the Wolffian ducts due to absence of testosterone • The absence of Mullerian inhibiting factor will allow the Mullerian ducts to differentiate into the oviducts and uterus • individual will be genotypically male (i.e., XY), yet will possess female-like internal and external reproductive characteristics

  11. Gonadal dysgenesis • pure gonadal dysgenesis, 46, XX • Swyer syndrome: pure gonadal dysgenesis, 46, XY type of hypogonadismin which no functional gonads are present to induce puberty in an externally female person whose karyotype is then found to be XY. The gonads are found to be partially non-functional streaks • Mutation of the SRY gene: When such a gene is defective, the indifferent gonads fail to differentiate into testes in an XY (genetically male) fetus • absence of breasts ( streak gonads), and the presence of a uterus and pubic hair (adrenals) • High risk of malignancy esp. gonadoblastoma, gonads should be removed as cancer may develop in infancy

  12. Disorders of the anterior pituitary • Non- functional adenomas • Prolactin secreting adenomas • Empty sella syndrome or compression of pituitary stalk • Sheehan’s syndrome • Galactosemia: Fewer oogonia result as a direct result of the toxic effect of galactose metabolites on germ cell migration to the genital the genital ridge

  13. Disorders of the central nervous system • Hypothalamic amenorrhea • weight loss, anorexia, bulimia • Excessive exercise-induced amenorrhea (decreased body fat and elevated endorphins and CRH) • Inherited genetic defects- Kallman’s syndrome

  14. Primary amenorrhea- causes • Chromosomal abnormalities (45%) • Physiological delay (20%) • Mullerian agenesis (15%) • imperforate hymen or vaginal septum (5%) • Hypothalamic GRH deficiency (5%) • anorexia nervosa (2%) • Hypopituitarism (2%) • Prolactinoma, hypothyroidism, craniopharyngima, adrenal disease ( < 1%)

  15. Primary amenorrhea- 2 questions approach • Has puberty occurred ? • “No”, then “normal stature or short? • “yes”, then Feminizing or Virilizing

  16. Has puberty occurred ? • NO • Normal to tall GnRH Deficiency Pure FSH deficiency FSH low Pure gonadal dysgenesis FSH high

  17. Has puberty occurred ? • NO • Short= something else is going on • Hypopituitarism • Turner’s • Hypothyroidism FSH slightly high, TSH high

  18. Has puberty occurred ? • Yes • Virilizing • 46 xx • 46 xy Virlizing female intersex Congenital adrenal hyperplasia • Virlizing male intersex • Partial androgen insensitivity syndrome • Leydig cell hypoplasia

  19. Has puberty occurred ? • Yes • Feminizing 46,xx 46,xy Idiopathic delay Mullerian agenesis Serum testosterone Complete androgen insensitivity syndrome Serum testosterone

  20. Presentation with primary amenorrhea : feminizing • Uterovaginal agenesis • low transverse vaginal septum • Complete androgen insensitivity • All the entities of secondary amenorrhea: • Pregnancy • Central • Ovarian failure • Uterine • Ovarian or adrenal tumor • metabolic

  21. Diagnostic evaluation • When to investigate? • No menarche by 15.5-16 years • Investigate earlier if : • Galactorrhea • Short stature • Dysmorphic features • Virilisation • Abnormal pubertal development • Out of keeping with family history of menarche • Symptoms or signs of hypothalamic-pituitary disease • Parent/adolescent concerned

  22. Investigations • Thorough history and physical exam • Estrogen • FSH/LH level • High-ovarian failure(turner’s,chemo, autoimmune) • Low or normal-hypothal-pit axis, constitutional • Thyroid function tests • Karyotype • Bone age • Prolactin • Testosterone, DHEAS, 17-oh Prog, androstenedione • Pelvic US, imaging • “routine bloods” looking for chronic illness

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