II. Epidemiology

  1. Incidence: 1%

III. Pathophysiology

  1. Follicle depletion or dysfunction resulting in impaired ovarian function

IV. Causes

  1. Idiopathic (>90%)
  2. Chromosomal Abnormality
    1. X-Chromosome abnormality (e.g. Turner Syndrome)
    2. Y-Chromosome presence abnormality
  3. Infection (e.g. Mumps)
  4. Autoimmune Disease directed at Ovary
  5. Empty Sella Syndrome
  6. Chemotherapy or radiation exposure related

V. Associated Conditions

  1. FMR1 Gene premutation
    1. Associated with risk of Fragile X Syndrome in children
  2. Thyroid autoimmune disorders
    1. Consider testing for Thyroid autoantibodies
  3. Adrenal autoimmune disorders
    1. Consider testing for adrenal autoantibodies
  4. Turner syndrome
    1. May present only as Short Stature and Amenorrhea
    2. Obtain karyotype from women with Short Stature and Primary Ovarian Insufficiency
      1. Multi-system implications of Turner Syndrome require surveillance and highlight the importance of testing

VI. Diagnosis

  1. Follicle Stimulating Hormone in menopausal range on 2 consecutive tests, one month apart AND
  2. Woman younger than age 40 years old with Amenorrhea or oligomenorrhea

VII. Labs

  1. See Secondary Amenorrhea
  2. Follicle Stimulating Hormone
    1. Used as part of diagnosis above

VIII. Labs: As Indicated for secondary cause

  1. Fasting Serum Glucose
  2. Calcium
  3. Phosphorus
  4. Total protein
  5. Albumin to globulin ratio
  6. Complete Blood Count (CBC)
  7. Erythrocyte Sedimentation Rate (ESR)
  8. Rheumatoid Factor (RF)
  9. Antinuclear Antibody (ANA)
  10. Thyroid Stimulating Hormone (TSH)
  11. Morning Serum Cortisol Level
  12. Consider Karyotype

IX. Management

  1. Osteoporosis Prevention
    1. Calcium Supplementation 1200 mg daily
    2. Vitamin D supplementation 800 IU daily (or more)
  2. Estrogen Replacement Therapy
  3. Pregnancy
    1. Risk of variable fertility (10% have spontaneous resolution and risk of pregnancy)
    2. Otherwise pregnancy is possible with donor implantation

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