Primary amenorrhea is the cessation of menstruation in patients which are characterized by one of the following features:

Amenorrhea (absence of menstruation) may be primary or secondary. Primary amenorrhea is the cessation of menstruation in patients which are characterized by one of the following characteristics: 16 years old or 2 years after the onset of puberty girls aged about 14 years, at which puberty (eg growth spurt development. of secondary sexual characteristics) is not used has patients who at the age of 13 years no menstrual bleeding and no signs of the onset of puberty yet (z. B. breast development) should be tested for primary amenorrhea. A Secondary amenorrhea is the absence of menses, after it had been ever present. Normally, patients should be evaluated for secondary amenorrhea, when the menses for ? 3 months failed or ? 3 atypical cycles have occurred since a cycle time is unusual between menarche and perimenopause> 90 days. Pathophysiology The hypothalamus secretes (GnRH) usually in phases gonadotropin-releasing hormone. GnRH stimulates the pituitary gland to the formation of gonadotropins to (follicle stimulating hormone [FSH] and luteinizing hormone [LH]; Reproductive Endocrinology the woman’s menstrual cycle), which are released into the bloodstream. The gonadotropins stimulate the ovaries to the formation of estrogen (primarily estradiol), androgens (mainly testosterone) and progesterone to. These hormones cause: FSH stimulates the developing oocytes nearby tissue to convert testosterone to estradiol. Estrogen stimulates the endometrium to proliferate. LH promotes when it is increasingly present during the menstrual cycle produces the maturation of the dominant oocyte, the release of the egg and the formation of the corpus luteum, the progesterone. Progesterone brings about a change of the endometrium, which is thereby prepared to rich glands and for implantation (decidualization of the endometrium). If no pregnancy, estrogen and progesterone production decreases, the endometrium builds up and becomes exfoliated during menses. In a typical cycle occurs 14 days after ovulation to menses. Are parts disturbed this system, there is an ovarian dysfunction; the cycle of gonadotropin-stimulated estrogen production and the cyclic endometrial are disturbed, resulting in anovulatory amenorrhea, and it may be that the menstrual absent. Usually is a amenorrhea, especially ondere the secondary, anovulatory. However, it can cause amenorrhea even with normal ovulation, as they at anatomical genital changes (eg. As congenital obstruction of the outflow path, intrauterine adhesions [Asherman’s syndrome]) occurs, prevent normal menses despite normal hormonal stimulation. Etiology A amenorrhea is usually classified as anovulatory (see table: causes of anovulatory amenorrhea) ovulatory (see table: causes of ovulatory amenorrhea) Each type has many causes, but the most common causes of amenorrhea include pregnancy (the most common cause in women childbearing age) Constitutional delay of puberty Functional hypothalamic anovulation (eg., by excessive exercise, eating disorders or stress) ingestion or abuse of drugs (eg. as oral contraceptives, Depoprogesteron, antidepressants, antipsychotics) breastfeeding Polycystic ovary syndrome, the endometrium through contraceptives are thinner, which occasionally has amenorrhea result; usually the menstrual periods begin again about 3 months after discontinuation of oral contraceptives. Antidepressants and antipsychotics can increase prolactin levels, which the breasts are stimulated to milk production and amenorrhea may be caused. Some diseases can lead to ovulatory or anovulatory amenorrhea. Congenital anatomic changes have only one primary amenorrhea result. Diseases that are responsible for secondary amenorrhea, may also cause a primary amenorrhea. Anovulatory amenorrhea The most common causes (see Table: Causes of anovulatory amenorrhea) involve a disturbance of the axis hypothalamus-pituitary-ovarian. These include Hypothalamic dysfunction (inbesonderse functional hypothalamic anovulation) pituitary dysfunction Primary ovarian failure (premature ovarian failure) Endocrine disorders that lead to an androgen (especially polycystic ovarian syndrome) Although anovulatory amenorrhea is usually secondary, but it can be primary, even if it never to ovulation occurs, eg. As a result of a genetic disease. Is it ever come to ovulation, puberty and development of secondary sexual characteristics do not run normally. Genetic disorders that are associated with a Y chromosome, increase the risk of a germ cell carcinoma. Causes of anovulatory amenorrhea cause Examples hypothalamic dysfunction, organic Genetic diseases (for. Example, congenital gonadotropin-releasing hormone deficiency, GnRH receptor gene mutations that lead to decreased FSH and estradiol levels as well as increased levels of LH, Prader-Willi syndrome) Infiltrative diseases of the hypothalamus (z. B. Langerhans cell granulomatosis, lymphoma, sarcoidosis, tuberculosis) irradiation of the hypothalamus traumatic brain tumors of the hypothalamus hypothalamic dysfunction, cachexia functional Chronic diseases , In particular respiratory, gastrointestinal, hematological, renal or hepatic diseases (e.g.. As Crohn’s disease, cystic fibrosis, sickle cell anemia, thalassemia major) slimming drug abuse (eg. As alcohol, cocaine, marijuana, or opioids) eating disorders (z. B. anorexia, bulimia) Physical exercise, excessive HIV infection immunodeficiency Psychiatric disorders (eg . as stress, depression, obsessive-compulsive disorder, schizophrenia) Psychoactive drug malnutrition pituitary dysfunction aneurysms pituitary hyperprolactinemia * idiopathic hypogonadism infiltrative disorders of the pituitary (z. B. hemochromatosis, Langerhans cell granulomatosis, sarcoidosis, tuberculosis) Isolated gonadotropin deficiency Kallmann syndrome (hypogonadotropic hypogonadism (with anosmia) Postpartum Hypophysennekrose Sheehan’s syndrome) head injury brain tumors (eg. as meningioma, craniopharyngioma, glioma) Tu Moren the pituitary gland (eg. B. microadenoma) dysfunction of the ovaries autoimmune diseases (eg. As autoimmune oophoritis in myasthenia gravis, thyroiditis or vitiligo) chemotherapy (z. B. high doses of alkylating agents) Genetic alterations, including chromosomal abnormalities (eg. As congenital thymic aplasia, Fragile X Syndrome , Turner’s syndrome [45, X], idiopathic accelerated ovarian follicular atresia) gonadal dysgenesis (incomplete development of the ovary, occasionally, as a result of genetic diseases) (radiation of the pelvis metabolic diseases (for. example, Addison’s disease, diabetes mellitus, galactosemia) Viral infections e.g. . B. mumps) Other endocrine dysfunction androgen insensitivity (testicular feminization) Congenital adrenal Virilism (congenital adrenal hyperplasia, z. For example, by 17-hydroxylase or 17,20-Lyasemangel) or adulthood onset adrenal Cushing’s syndrome virilism † †, ‡ drug-induced virilization (z. B. by androgens, anti-depressants, danazol or high dose progestins) † hyperthyroidism hypothyroidism obesity (the excessive extraglandular estrogen production result has) polycystic ovary syndrome † Real Hermaphroditismus † androgen-producing tumors z. (mostly of the ovaries or adrenal glands) † estrogen or human chorionic gonadotropin-producing tumors (gestational trophoblastic) * hyperprolactinemia (as a result of other disorders as hypothyroidism , use of some drugs) can also lead to amenorrhea. † Women with these conditions may have virilization or unclear sexual characteristics. ‡ virilization can occur as a result of an adrenal tumor in Cushing’s syndrome. Ovulatory amenorrhea The most common causes (see Table: causes of ovulatory amenorrhea) are chromosomal abnormalities Congenital genital anomalies that impede blood flow during menses causes of ovulatory amenorrhea cause Examples Congenital abnormalities of the reproductive system cervical stenosis (rare) Hymen Imperforate pseudohermaphrodism Vaginal cross septum vaginal and uterine aplasia ( z. B. Müller agenesis) Acquired uterine anomalies Ashe rman syndrome Endometriumtuberkulose Obstructive fibroids and polyps patients with obstructive abnormalities usually have normal hormonal function. An obstruction can be a result of a Hämatokolpos (a collection of menstrual blood in the vagina which can cause a swelling of the vagina), or a hematometra (a collection of menses in the uterus, which can cause an expansion of the uterus, a resistance or a protrusion of the cervix) be. Because ovarian function is normal, and the external genitalia and the other secondary sexual characteristics are normally developed. Some congenital anomalies (eg. As those that underlie a vaginal aplasia or a vaginal septum) may be associated with abnormalities of the urinary tract and skeleton. Some acquired anatomical changes, such as scarring of the endometrium after surgery in postpartum hemorrhage or infection (Asherman’s syndrome) have an ovulatory amenorrhea result. Clarification girls should be investigated if Until the age of 13 years no signs of puberty (z. B. breast development, growth spurt) are present. By the age of 14 years no pubic hair has occurred. Menarche up to 16 years or 2 years is not occurred after puberty (development of secondary sexual characteristics). In women of childbearing age after the cessation of menses, a pregnancy test is required. They are tested for amenorrhea, if you are not pregnant and for ? 3 months had no menses or ? 3 atypical cycles have occurred. You have <9 menses per year. You have a sudden change in their menstrual pattern. History to history of the current disease include whether the menarche ever took place, how old the patients were (important for distinguishing between primary and secondary amenorrhea) and if at menarche. Whether the period always been regularly is When was the last normal menstrual period occurred How long and difficult menstruation is whether patients cyclic breast tenderness and mood swings have when they reach a certain size and the milestone of development, including the age at thelarche (development of breasts in puberty) in reviewing the organ systems symptoms should be included, pointing to a cause, including galactorrhea, headache and visual field defects: pituitary disorders fatigue, weight gain and cold intolerance: hypothyroidism palpitations, nervousness, tremors and heat intolerance: hyperthyroidism acne, hirsutism and deepening of the voice, androgen excess in patients with secondary amenorrhea, hot flashes, vaginal Dryness, sleep disturbances, fragility and decreased libido: Estrogen deficiency In order to clarify whether ovulation has occurred, patients with primary amenorrhea are informed of developments in their puberty interviewed (eg. As breast development, growth spurt, presence of axillary and pubic hair). Anamnestic include risk factors for Functional hypothalamic anovulation such as stress, chronic disease, new drugs and a recent change in weight, diet or exercise intensity in patients with secondary amenorrhea, Asherman's syndrome (for example, D & C, endometrial ablation, endometritis. , obstetric injury, uterine surgery) the history of drug intake should include specific questions on the use of drugs such as the following:. drugs that affect dopamine have (eg antihypertensives, antipsychotics, opioids, tricyclic antidepressants) cytostatic drugs (eg. can cause. busulfan, chlorambucil, cyclophosphamide) sex hormones, virilization (z. B. androgens, estrogens, high-dose progestins, anabolic steroids OTC) contraceptives, in particular the last use Systemic Kortikosteroi de OTC products and nutritional supplements, some of which contain bovine hormones or other medicines interact to family history should be the detection of the amount of family members, all cases of delayed puberty and genetic disorders include including fragile X Syndrom.Körperliche investigation To study the vital signs be considered and the physical constitution and the physique, including height, weight and body mass index (BMI). The secondary sexual characteristics are evaluated and beurteit breast development and pubic hair development after Tanner. If axillary and pubic hair are present, Adrenarche has taken place. the Brustsekretion is tested at the seated patient, by applying to all portions of pressure is applied with a movement from the base to the nipple out. A galactorrhea (breast milk secretion without reference to childbirth) can be observed; they can be distinguished from other Sekretionsarten by the detection of fat particles in the liquid with a stereomicroscope. By a pelvic examination can determine anatomical disorders of the genitals; a vorgewölbes hymen may be a result of a Hämatokolpos, which is evidence of genital obstruction. The findings from the gynecological examination may also provide evidence of an estrogen deficiency. Thus, a thin, pale vaginal mucosa without wrinkles and a p-value> 6.0 in women after puberty displays an estrogen deficiency. If the cervical mucus spinnability (stringy, stretchy consistency), this has generally indicate adequate amount of estrogen. During the general examination of virilization attention is paid to signs, including hirsutism, receding hairline, acne, deep voice, increased muscle mass, Klitoromegalie (clitoral enlargement) and defeminization (decrease of the previously normal secondary sexual characteristics such as a reduction in breast size and vaginal atrophy). The hypertrichosis (excessive hair growth on limbs, head and back), which often occurs in some families, separated from the real hirsutism, associated with dense hair of the upper lip, chin and between the breasts. On skin discoloration (. Eg yellowing in jaundice or Carotinämie, black spots in acanthosis nigricans) taken werden.Warnzeichen The following findings are of particular importance: Delayed puberty virilization visual field defects excluded Impaired sense of smell interpretation of the findings Pregnancy should not be based on the medical history become; a pregnancy test is required. Tips and risks occurs amenorrhea, pregnancy testing should be made independent of the sexual history and information on menstruation in girls with secondary sexual characteristics and in women of childbearing age. In primary amenorrhea normally developed secondary sexual characteristics are usually for a normal hormonal activity; the amenorrhea is ovulatory usually and typically due to a congenital obstruction of the genital tract. A primary amenorrhea with abnormal secondary sexual characteristics is usually anovulatory (z. B. due to a genetic disorder). The clinical results are possible in secondary amenorrhea to the underlying disorder point (see Table: findings that indicate the cause of an amenorrhea): galactorrhea has hyperprolactinemia out (for example, pituitary dysfunction, use of some drugs.); also occur visual field defects and headache, a pituitary tumor should be considered. Symptoms and signs of estrogen deficiency (eg. As hot flushes, night sweats, vaginal dryness or atrophy) indicate a premature ovarian failure (premature failure of the ovaries) or functional hypothalamic anovulation (z. B. due to excessive sport, low body weight and low body fat ) out. Virilization may be indicative of an androgen (e. As polycystic ovary syndrome, androgenproduzierender tumor, Cushing’s syndrome, taking certain drugs). Patients with a high body mass index (BMI) and / or acanthosis nigricans probably have polycystic ovary syndrome. Findings that indicate the causes of amenorrhea finding Similar findings Possible cause use of some drugs affecting dopamine drugs (acting in the regulation of prolactin secretion with): antihypertensives (. Eg methyldopa, reserpine, verapamil) antipsychotics 2nd generation ( z. B. molindone, olanzapine, risperidone) antipsychotics, conventional (z. B. haloperidol, phenothiazine, pimozide) cocaine estrogens Gastrointestinal drugs (e.g., as cimetidine, metoclopramide) Hallucinogens, opioids (eg. Codeine, morphine) Tricyclic antidepressants (eg. As clomipramine, desipramine) galactorrhea hyperprolactinemia hormones and drugs that affect the estrogen-androgen balance (z. B. androgens, anti-depressants, danazol, high-dose progestins) virilization drug-induced virilization Physical appearance High body mass index (eg> 30 kg / m2.) – estrogen excess Polycystic ovary syndrome virilization Low body mass index (eg. B. <18.5 kg / m2) Risk factors such as chronic disease, dietary measures or eating disorder Functional hypothalamic anovulation hypothermia, bradycardia, hypotension Functional hypothalamic anovulation in anorexia nervosa or starvation Reduced gag reflex, soft palate lesions, subconjunctival hemorrhage Functional hypothalamic anovulation in bulimia nervosa with frequent vomiting dwarfism primary amenorrhea, wrinkly neck, widely spaced mammillae Turner syndrome Skin lesions Warm, moist skin tachycardia, tremor hyperthyroidism Rough, thickened skin, loss of eyebrows bradycardia, delayed tendon reflexes, weight gain, constipation hypothyroidism acne virilization androgen by polycystic ovary syndrome Androgenproduzierender tumor Cushing's syndrome Adrenal Virilism Ar zneimittel (eg. B. androgens, anti-depressants, danazol, high-dose progestins) Striae moon face, buffalo hump, truncal obesity, thin extremities, virilization, hypertension, Cushing's syndrome, acanthosis nigricans obesity, virilization polycystic ovary syndrome Vitiligo or hyperpigmentation of the palm of orthostatic hypotension Addison's disease on estrogen or Androgenveränderungen indicative Be general discoveries symptoms of estrogen deficiency (eg. As hot flushes, night sweats, particularly vaginal dryness or atrophy) risk factors such as oophorectomy, chemotherapy, or pelvic radiation Primary ovarian failure functional hypothalamic anovulation pituitary tumors hirsutism with virilization -. Androgen by polycystic ovary syndrome Androgenproduzierenden tumor Cushing's disease Adrenal virilism drug (e.g., androgens , antidepressants, danazol, high-dose progestins) Primary amenorrhea androgen by True hermaphroditism pseudohermaphrodism Androgenproduzierender tumor Adrenal Virilism gonadal dysgenesis Genetic disease Enlarged ovaries androgen excess by 17-hydroxylase Polycystic ovary syndrome Androgenproduzierender ovarian tumor changes in the breast and genitals galactorrhoea - hyperprolactinemia Nocturnal Headache eg, visual field defects pituitary None or incomplete development of the breast (and of secondary sexual characteristics) Normal Adrenarche Primary anovulatory amenorrhea missing by isolated ovarian failure Adrenarche Primary anovulatory amenorrhea by hypothalamic-pituitary dysfunction Missing Adrenarche with limited olfactory Kallmann syndrome Delayed development of the breast and the secondary Gesc hlechtsmerkmale Delayed menarche family history of constitutional delay of growth and puberty normal breast development and secondary sexual characteristics with primary amenorrhea cycle-dependent abdominal pain, swelling of the vagina, expansion of the uterus obstruction in the genital area Unclear sexual characteristics - True hermaphroditism pseudohermaphrodism virilization labia fusion, clitoral enlargement at birth- androgen exposure in the first trimester, may reference to congenital adrenal Virilism True hermaphroditism Drug-induced virilization clitoral enlargement after birth virilization Androgenproduzierenden tumor (usually ovarian cancer) Adrenal Virilism taking anabolic steroids Normal external genitalia (with incompletely developed secondary sexual characteristics occasionally breast development, but minimal pubic hair ) obvious lack of cervix and uterus androgen insensitivity Vergrößerung der Ovarien (bilateral) Zeichen eines Östrogenmangels Primäre Ovarialinsuffizienz durch Autoimmun-Oophoritis Virilisierung 17-Hydroxylasemangel Polyzystisches Ovarialsyndrom Läsionen Pelvine Resistenz (unilateral) Pelvine Schmerzen Beckentumoren Tests Anamnese und körperliche Untersuchung unterstützen direkte Tests. Bei Mädchen mit sekundären Geschlechtsmerkmalen sollte ein Schwangerschaftstest durchgeführt werden, um eine Schwangerschaft und ein gestationsbedingter Trophoblasttumor als Ursache der Amenorrhö auszuschließen. Nach Ausbleiben der Menses sollte bei Frauen im gebärfähigen Alter ein Schwangerschaftstest gemacht werden. Die Herangehensweise bei einer primären Amenorrhö ( Untersuchung der primären Amenorrhöa.) unterscheidet sich von der bei einer sekundären Amenorrhö ( Untersuchung der sekundären Amenorrhö.), auch wenn keine besonderen allgemeinen Richtlinien oder Algorithmen allgemein akzepti

Health Life Media Team

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