Primary Ovarian Failure

(Premature menopause, Hypergonadotropic hypogonadism, premature ovarian failure, premature failure of the ovaries)

In primary ovarian failure of women <40 years, the ovaries do not pour out enough eggs and produce despite high Gonadotropinkonzentrationen (particularly of follicle stimulating hormone [FSH]) sex hormones only in insufficient quantities. Diagnostically FSH and estradiol levels are measured. As a rule, the treatment consists of a combined estrogen-progestin therapy.

In primary ovarian failure, the ovaries work in women who are <40, normal. This disease is called premature ovarian failure or premature menopause; However, these terms are misleading because women do not always have no menstruation with primary ovarian failure and their ovaries do not always stop function correctly. Thus, not always, a diagnosis of primary ovarian failure means that pregnancy is impossible. This disorder does not mean that a woman ages prematurely; it just means that their ovaries no longer function normally.

In primary ovarian failure of women <40 years, the ovaries do not pour out enough eggs and produce despite high Gonadotropinkonzentrationen (particularly of follicle stimulating hormone [FSH]) sex hormones only in insufficient quantities. Diagnostically FSH and estradiol levels are measured. As a rule, the treatment consists of a combined estrogen-progestin therapy. In primary ovarian failure, the ovaries work in women who are <40, normal. This disease is called premature ovarian failure or premature menopause; However, these terms are misleading because women do not always have no menstruation with primary ovarian failure and their ovaries do not always stop function correctly. Thus, not always, a diagnosis of primary ovarian failure means that pregnancy is impossible. This disorder does not mean that a woman ages prematurely; it just means that their ovaries no longer function normally. In primary ovarian failure, the ovaries Stop the release of eggs or give them this only temporarily free Stop the production of the hormones estrogen, progesterone, and testosterone or produce it they have only temporarily etiology Premature ovarian failure and premature ovarian failure different causes see table : Common causes of primary ovarian failure), including the following: the number of ovarian follicles at birth is reduced. The follicular atresia is accelerated. The follicles are dysfunctional (as in autoimmune ovarian dysfunction does). Certain genetic disorders are. Genetic disorders which may be associated with a Y-chromosome cause a primary ovarian failure. These diseases, which usually show up until the age of 35 years, increase the risk of ovarian germ cell tumor. Common causes of primary ovarian failure cause Examples enzyme defects galactosemia 17?-hydroxylase 17,20-Lyasemangel Genetic defects Premature ovarian follicular atresia (idiopathic) Certain autosomal defects FMR1Prämutation (Fragile X Syndrome) gonadal dysgenesis (as a result of genetic defects z. B. Turner's syndrome [ 45, X], pure [46 XX or 46 XY] or mixed gonadal dysgenesis) idiopathic hypogonadotropic hypogonadism Kallmann syndrome myotonic dystrophy Reduced germ cell number trisomy X with or without chromosomal Mosaicism immune disorders Autoimmune disorders (most common thyroiditis, Addison's disease, hypoparathyroidism, diabetes mellitus, myasthenia gravis, vitiligo, pernicious anemia, candidiasis of the skin and mucous membranes) Congenital thymic Insulated ovarian sarcoidosis Other causes Addison's disease adrenal insufficiency cytotoxic drugs (especially alkylating agents) Cigarette smoking Diabetes radiation gonadal Surgical removal of the gonads or the adnexa viral infections (eg. B. Mumps) symptoms and discomfort in women with occult or biochemically primary ovarian failure (s. U. Under classification) an unexplained infertility can be the only symptom. Women with ovarian failure or premature ovarian failure overt primary usually have amenorrhea or irregular menstrual periods and often symptoms of estrogen deficiency (eg. As osteoporosis, atrophic vaginitis, decreased libido). The ovaries are usually small and barely palpable, but occasionally larger, especially if an immune disease is the cause. Sometimes, patients also show evidence of the causal disease (z. B. dysmorphia at Turner's syndrome, mental retardation, dysmorphic and autism in Fragile X syndrome, rarely orthostatic hypotension, hyperpigmentation and reduced underarm and pubic hair in adrenal insufficiency). Under estrogen therapy, the risk of dementia, Parkinson's disease and coronary heart disease is increased. If primary ovarian failure is caused by an autoimmune disease, women have a risk of potentially life-threatening primary adrenal insufficiency. Diagnosis FSH and estradiol thyroid levels, fasting blood glucose, electrolytes and creatinine Sometimes genetic testing primary is made the diagnosis premature ovarian failure in women <40 years with unexplained infertility, menstrual disorders or signs of estrogen deficiency. A pregnancy test is performed about 2-4 weeks and the serum levels of FSH and estradiol were determined weekly; high FSH levels (> 20 mI.E./ml, but usually> 30 mI.E./ml) at low Estradiolwerten (usually <20 pg / ml) confirm the presence of ovarian failure. Further tests are based on the suspected cause. Since the Anti-Müllerian hormone is produced only in small follicles, was trying to diagnose a reduced ovarian reserve with the blood levels of this hormone. Normal values ??are from 1.5 to 4.0 ng / ml. A very low level pointed to decreased ovarian reserve. Genetic counseling and testing FMR1 premutation are indicated if the family history has resulted in a primary ovarian failure, or mental retardation, tremor or ataxia. The karyotype is determined in women <35 years with proven ovarian failure or ovarian failure. If karyotype is normal, or when an autoimmune cause is suspected, tests on serum and anti-adrenal 21-hydroxylase antibodies (adrenal autoantibodies). If an autoimmune cause is suspected, tests for autoimmune hypothyroidism are carried out; they comprise the measurement of thyroid stimulating hormone (TSH), thyroxine (T4) and antithyroid peroxidase and thyroglobulin antibodies. Bone density is determined in women with symptoms of estrogen deficiency. A Ovarialbiopsie is not indicated. Classification Primary ovarian failure can be divided according to the clinic and serum FSH levels: occult primary ovarian failure: Unexplained infertility and normal FSH levels in serum Biochemical primary ovarian failure: Unexplained infertility and increased FSH levels in serum manifests primary ovarian failure: irregular menstrual cycle, and increased FSH levels in serum Premature ovarian failure: Irregular or occasional bleeding for years, possible pregnancy and increased FSH levels in serum Premature menopause: amenorrhea, permanent infertility, complete depletion of the primordial follicle treatment estrogen / progesterone therapy women without children can request to age of about 51 years a cyclic estrogen-progestin therapy (combination of hormonal therapy), provided that such hormones are not contraindicated; therapy relieves symptoms of estrogen deficiency, supports the preservation of bone density and may help prevent coronary heart disease, Parkinson's disease and dementia. In women with infertility, in vitro fertilization is donor eggs plus exogenous supply of estrogen and a progestogen a way that will enable the endometrium in a position to accommodate the transferred embryo. The age of the donor of the egg is more important than the age of the recipient. This method is relatively successful, but some women get pregnant diagnosed with primary ovarian failure without in-vitro fertilization. So far there is no treatment to increase ovulation or restore fertility in women with primary ovarian failure. Other options for women who desire a pregnancy include cryopreservation of ovarian tissue, ova and embryos and embryo or egg donation. These techniques can during ovarian failure are used, particularly in cancer patients before or. Neonatal and adult ovaries have a small number of "oogonial" stem cells that grow stable for months and can produce mature oocytes in vitro; these cells can be used to develop treatments for infertility in the future. To prevent osteoporosis, women should with primary ovarian failure an adequate amount of calcium and vitamin D (with the diet and / or supplements) consume. In women with a Y chromosome laparotomy or laparoscopy with removal of all Gonadengewebes is necessary because the risk of germ cell tumors of the ovary is increased. Summary suspicion of premature ovarian failure is in women with unexplained menstrual disorders, infertility or signs of estrogen deficiency. The diagnosis is (increased, usually> 30 mI.E./ml) by determination of FSH and estradiol (reduced, typically <20 pg / ml) asked. Unless contraindicated, then a cyclic estrogen-progesterone treatment up to the age of about 51 years to maintain bone density and relieve the symptoms and complications of estrogen deficiency.

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