Primary amenorrhoea

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Primary amenorrhoea

  1. 1. PRIMARY AMENORRHOEA Prof. M.C.Bansal MBBS., MS., FICOG., MICOG. Founder Principal & Controller, Jhalawar Medical College & Hospital Jjalawar. MGMC & Hospital , sitapura ., Jaipur
  2. 2. DIFFERENTIAL DIAGNOSIS OF PRIMARY AMENORRHOEAA. Anatomic abnormalities of the genital outflow tract1. Müllerian dysgenesis (Rokitansky–Küster–Hauser syndrome)2. Distal genital tract obstruction a. Imperforate hymen b. Transverse vaginal septumB. Hypergonadotropic (follicle–stimulating hormone >30mIU/mL) hypogonadism (gonadal “failure”)1. Gonadal dysgenesis with stigmata of Turner syndrome2. Pure gonadal dysgenesis a. 46,XX b. 46,XY3. Early gonadal “failure” with apparent normal ovariandevelopment
  3. 3. • C. Hypogonadotropic (luteinizing hormone and follicle–stimulating hormone <10 mIU/mL) hypogonadism• 1. Constitutional delay• 2. Isolated gonadotropin deficiency • a. Associated with midline defects (Kallmann syndrome) • b. Independent of associated disorders • c. Prader–Labhart–Willi syndrome • d. Laurence–Moon–Bardet–Biedl syndrome • e. Many other rare syndromes• 3. Associated with multiple hormone deficiencies• 4. Neoplasms of the hypothalamic–pituitary area • a. Craniopharyngiomas • b. Pituitary adenomas • c. Other
  4. 4. • 5. Infiltrative processes (Langerhans cell–type histiocytosis)• 6. After irradiation of the central nervous system• 7. Severe chronic illnesses with malnutrition• 8. Anorexia nervosa and related disorders• 9. Severe hypothalamic amenorrhea (rare)• 10. Antidopaminergic and gonadotropin–releasing hormone–inhibiting drugs(especially psychotropic agents, opiates)
  5. 5. • 11. Primary hypothyroidism• 12. Cushing syndrome• 13. Use of chemotherapeutic (especially alkylating) agents• II. Asynchronous pubertal development• A. Complete androgen insensitivity syndrome (testicular feminization)• B. Incomplete androgen insensitivity syndrome
  6. 6. DISCUSSION
  7. 7. Central signals Peripheral HYPOTH signals GABA, ALAMUSNPY,GLUTAMATE Leptin, Ghrelin Kisspeptin- GPR54 system GnRH ANT. PITUITARY FSH LH
  8. 8. FSH LH OVARY GRAN THE Aromatisation Androgen of androgens production ESTRADIOLINHIBIN Follicular growth Mid cycle LH peak
  9. 9. • WHO divides patients into groups based on endogenous oestrogen production, follicle-stimulating hormone (FSH) levels, prolactin levels, and hypothalamic-pituitary dysfunction.• This classification is a guide that eliminates several diagnoses based on initial information. However, further work-up is still required.• Group I: low oestrogen, low FSH, and no hypothalamic- pituitary pathology, leading to a diagnosis of hypogonadotrophic hypogonadism.• Group II: normal oestrogen, normal FSH, and normal prolactin, leading to a diagnosis of polycystic ovary syndrome.• Group III: low oestrogen and high FSH, leading to a diagnosis of gonadal failure.
  10. 10. APPROACH TO A CASE OF PRIMARY AMENORRHOEA HISTORY & CLINICAL EXAM ASYNCHRONOUS IMMATURE MATURE SECONDARY DEVELOPMENT SECONDARY SEXUAL SEXUAL BREAST > PUBIC HAIR CHARACTERISTICS CHARACTERISTICS ANDROGEN FSH , PROLACTIN DISTAL GENITAL INSENSITIVITY TRACT OBSTRUCTION, MULLERIAN AGENESIS
  11. 11. FSH , PROLACTIN HIGH FSH LOW OR NORMAL FSH HIGH PROLACTIN KARYOTYPE PITUITARY FUNCTION CHECK T4, TSH TESTING SELLAR X-RAY NORMAL HIGH TSHNORMAL ABNORMAL NORMAL ABNORMAL TSH MRI OR CT• 46, XX • 45,XX OR •CONSTITUIONAL GONADAL 46,XY DELAY HYPOTHYROIDISM DYSGENE • MOSAIC •ISOLATED SIS GONADAL GONADOTROPIN• PREMATU DYSGENES DEFICIENCY RE IS •MALNUTRITION • HYPOPITUITARISM OVARIAN •CHRONIC • CNS TUMOR FAILURE ILLNESS

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