II. Epidemiology

  1. Incidence: 1% of women

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

VIII. Labs

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

IX. 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

X. 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)
      1. Consider combined Hormonal Contraception instead of Estrogen Replacement to prevent pregnancy
    2. Otherwise pregnancy is possible with donor implantation

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Ontology: Ovarian Failure, Premature (C0085215)

Definition (MEDLINEPLUS)

Premature ovarian failure (POF) is when a woman's ovaries stop working before she is 40. POF used to be called premature menopause. However, POF is not the same as menopause. Some women with POF still have occasional periods. Premature menopause is when periods stop before age of 40. This can be natural or caused by surgery, chemotherapy or radiation.

Missed periods are usually the first sign of POF. Later symptoms may be similar to those of natural menopause.

Most women with POF cannot get pregnant naturally. Fertility treatments help a few women; others use donor eggs to have children. There is no treatment that will restore normal ovarian function. However, many health care providers suggest taking hormones until age 50.

NIH: National Institute of Child Health and Human Development

Definition (MSH) Cessation of ovarian function after MENARCHE but before the age of 40, without or with OVARIAN FOLLICLE depletion. It is characterized by the presence of OLIGOMENORRHEA or AMENORRHEA, elevated GONADOTROPINS, and low ESTRADIOL levels. It is a state of female HYPERGONADOTROPIC HYPOGONADISM. Etiologies include genetic defects, autoimmune processes, chemotherapy, radiation, and infections.
Concepts Disease or Syndrome (T047)
MSH D016649
SnomedCT 237788002
Dutch vroegtijdig eierstokfalen, Functieverlies, ovarium, prematuur, Ovarium, prematuur functieverlies
German vorzeitiges Nachlassen der ovariellen Funktion, Ovarieller Funktionsausfall, prämaturer
Italian Insufficienza ovarica prematura, Insufficienza ovarica precoce
Portuguese Insuficiência prematura do ovário, Insuficiência Ovariana Primária, Falência Ovariana Prematura, Falência Prematura do Ovário, Síndrome do Ovário Resistente às Gonadotrofinas, Insuficiência Prematura do Ovário
Spanish Insuficiencia ovárica prematura, Insuficiencia Ovárica Primaria, Fallo Ovárico Prematuro, Síndrome del Ovario Resistente a Gonadotrofinas, Insuficiencia Ovárica Prematura, insuficiencia ovárica prematura (trastorno), insuficiencia ovárica prematura
Japanese 早期卵巣機能不全, ソウキランソウキノウフゼン, 早期卵巣不全, 未熟卵巣不全, 卵巣不全-早期, 卵巣不全-未熟, 難治性卵巣症候群, 性腺刺激ホルモン難治性卵巣症候群
Swedish Ovarialsvikt, prematur
English Ovarian Failure, Premature, POF - Premat ovarian failure, Insufficiency, Primary Ovarian, Ovarian Insufficiency, Primary, Primary Ovarian Insufficiency, premature ovarian failure, failure ovarian premature, Primary Ovarian Insufficiency [Disease/Finding], Ovarian failure, premature, POF, primary ovarian insufficiency, Premature ovarian failure, POF - Premature ovarian failure, Premature ovarian failure (disorder), Premature Ovarian Failure
Finnish Munasarjojen ennenaikainen vajaatoiminta
Russian GONADOTROPIN-REZISTENTNYI OVARIAL'NYI SINDROM, IAICHNIKA NEDOSTATOCHNOST' PREZHDEVREMENNAIA, IAICHNIKA REZISTENTNOGO SINDROM, ЯИЧНИКА НЕДОСТАТОЧНОСТЬ ПРЕЖДЕВРЕМЕННАЯ, PERVICHNAIA IAICHNIKOVAIA NEDOSTATOCHNOST', ПЕРВИЧНАЯ ЯИЧНИКОВАЯ НЕДОСТАТОЧНОСТЬ, ГОНАДОТРОПИН-РЕЗИСТЕНТНЫЙ ОВАРИАЛЬНЫЙ СИНДРОМ, ЯИЧНИКА РЕЗИСТЕНТНОГО СИНДРОМ
Czech Předčasné ovariální selhání, primární ovariální insuficience, ovariální činnost - vyhasnutí předčasné
French Défaillance ovarienne prématurée, Insuffisance ovarienne primaire, Insuffisance ovarienne précoce, Insuffisance ovarienne primitive, Défaillance ovarienne précoce, Insuffisance ovarienne prématurée, IOP (Insuffisance Ovarienne Prématurée), IOP (Insuffisance Ovarienne Primitive)
Polish Niewydolność jajników przedwczesna, Niedomoga jajników przedwczesna, Zespół opornego jajnika, Zespół oporności jajnika na gonadotropinę
Hungarian Korai ovarialis elégtelenség
Norwegian Primær ovarialsvikt, Prematur ovariesvikt, Primær ovariesvikt, Prematur ovarialsvikt