central genesis. Central form of amenorrhea

Amenorrhea (with Latin- from a negation and Greek men - month, rheo - flow) absence of menstruation in girls aged 15 years or for 6 months or more in previously menstruating women of reproductive age. is not an independent diagnosis, but a symptom of certain disorders in the body: physiological, biochemical, genetic, or even emotional and psychological

AMENORRHEA PRIMARY - never had menstruation at the age of 16 years and older, with and without impaired development of secondary sexual characteristics. It is less common, 10% in the structure of amenorrhea. SECONDARY - the absence of menstruation after their presence, against the background of the development of secondary sexual characteristics

PHYSIOLOGICAL AMENORRHEA - in girls before puberty, - during pregnancy and lactation, - in postmenopause. FALSE - cyclic processes in the hypothalamus-pituitary-ovaries-uterus system occur normally, and external excretion menstrual blood does not occur due to violation of the outflow (atresia of the vagina, cervical canal, hymen) Hematocolpos, hematometra, hematosalpinx, " acute abdomen» . Surgical treatment (dissection of the hymen or expansion of the cervical canal).

AMENORRHEA PATHOLOGICAL (TRUE) no menstruation and cyclical changes in system G-G-Z-M Not an independent disease Symptom of gynecological or extragenital pathology IATROGENIC after hysterectomy and total oophorectomy while taking medications (gonadotropin agonists, antiestrogens) after radiation exposure to chemotherapy

CLASSIFICATION OF AMENORRHEA Depending on the level of damage (link) of the neuro endocrine system Central genesis (hypothalamic-pituitary) Ovarian Uterine Caused by pathology of the adrenal glands Caused by pathology thyroid gland

CLASSIFICATION OF AMENORRHEA The defeat of each of the levels of regulation of menstrual function can be of different genesis (character) Functional Organic congenital pathology

PRIMARY AMENORRHEA OF CENTRAL GENESIS 1. Hypothalamic genesis - hypothalamic hypogonadism: Colman's syndrome; Pehkrantz-Babinski-Frelich syndrome (adiposogenital dystrophy); Hand-Schuller-Christian disease; Laurence-Moon-Barde-Bill syndrome.

PRIMARY AMENORRHEA OF THE CENTRAL GENESIS Colman's syndrome The development of this syndrome is caused by a mutation of the Kail gene. It is inherited in an autosomal dominant manner. Congenital or sporadically occurring gene defects lead to isolated hypothalamic insufficiency of Gn. RG and damage to the olfactory center. Delayed maturation of the skeleton, lack of secondary sexual characteristics, hypoplasia of the internal genital organs, primary infertility. Anosmia is observed.

PRIMARY AMENORRHEA OF CENTRAL GENESIS Pehkrantz-Babinski-Frelich syndrome (adiposogenital dystrophy) Develops as a result of a traumatic or neoplastic lesion of the hypothalamus in the prepubertal period. The ventromedial and parabasal nuclei of the hypothalamus are affected. There is a delay in growth and sexual development, hypothalamic obesity. The deposition of fat in the abdomen, face, mammary glands, which gives the girl a feminine appearance. At severe forms ah, by the age of 18, obesity does not disappear, underdevelopment of the genital organs (narrow vagina, small uterus) is detected, secondary sexual characteristics are poorly expressed.

PRIMARY AMENORRHEA OF CENTRAL GENESIS Hand-Schuller-Christian disease A genetically determined disease with an autosomal recessive type of inheritance, the development of which is based on damage to the hypothalamic-pituitary region. Manifested by nanism, sexual infantilism, exophthalmos, diabetes insipidus, xanthomatosis, enlarged lymph nodes, skeletal changes.

PRIMARY AMENORRHEA OF CENTRAL GENESIS Lawrence-Moon-Barde-Biedl Syndrome Hereditary diencephalic-reticular degeneration caused by multiple gene defects is often familial. The nuclei of the hypothalamus are exposed degenerative changes, the number of ganglion cells decreases and glia grows in their place. Clinic: hypogonadism, retinitis pigmentosa, obesity, mental retardation, multiple congenital malformations, growth retardation, mental retardation, decreased vision, hearing loss.

PRIMARY AMENORRHEA OF CENTRAL GENESIS 2. Pituitary genesis - hypogonadotropic hypogonadism: pituitary dwarfism; gigantism; pituitary eunuchoidism.

PRIMARY AMENORRHEA OF THE CENTRAL GENESIS Pituitary dwarfism Defeat of the adenohypophysis in childhood. The disease is characterized by insufficient production of all tropic pituitary hormones with a predominant deficiency of growth hormone. There is a delay in growth and sexual development. Growth at adult woman does not exceed 120 cm, the proportions of the body are preserved, mental development is not disturbed, the genitals are sharply underdeveloped.

PRIMARY AMENORRHEA OF THE CENTRAL GENESIS Gigantism Hyperproduction of growth hormone by the pituitary gland and relative insufficiency of gonadotropic hormones. An acidophilic pituitary adenoma or, more rarely, an infectious process that develops in childhood, before puberty, leads to increased production of growth hormone. High growth, preserved body proportions, secondary sexual characteristics are not developed enough.

PRIMARY AMENORRHEA OF THE CENTRAL GENESIS Pituitary eunuchoidism Hyperostosis of the back of the Turkish saddle with a decrease in the volume of the pituitary gland. The decrease in the level of gonadotropins in the blood is due to a decrease in the mass of hormone-producing pituitary tissue. Eunuchoid physique: excessive fat deposition in the neck, mammary glands, abdomen, pelvis, hips, buttocks; underdevelopment of the mammary glands, labia, vagina, uterus, lack of hair growth on the pubis and in the armpits; paleness and dryness of the skin. The size of the uterus and ovaries correspond to the age of 2-7 years.

DIAGNOSIS OF AMENORRHEA I OF CENTRAL GENESIS Molecular cytogenetic method Detection of a defect in the Kail gene - Colman syndrome, Hand-Schuller-Christian disease, multiple gene defects - Laurence-Moon-Barde-Biedl syndrome - diencephalic-reticular degeneration. Hormonal studies ↓ estrogen, ↓ progesterone, ↓ FSH, ↓ LH - always positive gonadotropin test - always, ↓ STH, ↓ TSH - Hand-Schüller-Christian disease, ↓ TSH - ↓ Laurence-Moon-Barde-Biedl syndrome, ↓ STH - pituitary dwarfism, STG - gigantism X-ray of the skull Hyperostosis of the back of the Turkish saddle - with pituitary eunuchoidism. Expansion of the entrance, deepening of the bottom, increase in size and destruction of the Turkish saddle - with gigantism. Definition of fields of view

PRINCIPLES OF TREATMENT OF AMENORRHEA I OF CENTRAL GENESIS In tumors - X-ray exposure of the pituitary gland, in the absence of effect - surgical treatment. 2. Correction of lipid metabolism disorders. Diet with restriction of fats and carbohydrates, exercise therapy. 3. Hormone therapy with sex steroids from 12-13 years old. Gonadotropins Cyclic therapy with estrogens and gestagens Corded oral contraceptives Stimulation of ovulation with clostilbegit 4. Component hormone replacement therapy: thyroidin 0.05-0.2 g per day for 5 days with intervals of 2-3 days; analogues of growth hormone in case of its insufficiency. one.

PRIMARY AMENORRHEA 4. UTERINE FORM Rokitansky-Kyustner syndrome Uterus agenesis. 5. FALSE AMENORRHEA Congenital malformations of the external and internal genital organs. 6. AMENORRHEA IN DISEASES OF THE ADRENAL AND THYROID GLANDS: Congenital adrenogenital syndrome; congenital hypothyroidism.

ROKITANSKY-KUSTNER SYNDROME congenital disease- malformation: the absence of the uterus and vagina, with the normal development of the gonads. It is caused by a violation of the organogenesis of the internal genital organs during intrauterine development. The phenotype is female. Sexual development is normal, secondary sexual characteristics are formed correctly and in a timely manner. Diagnosis: gynecological examination - absence of the vagina and uterus, ultrasound, laparoscopy - absence of the uterus. Sexual function can be restored after performing plastic surgery- the formation of the vagina from the skin flap or intestines. Reproductive function cannot be restored. The birth of a child is possible with surrogate motherhood.

FALSE AMENORRHEA Occurs due to congenital malformations of the external and internal genital organs: vaginal aplasia (congenital obstruction of the vagina due to the absence of part or all of the organ), vaginal atresia (presence of a septum in the vagina less than 2 cm long), hymen atresia (infection of the hymen). The clinic manifests itself with the onset of menstrual function and is characterized by the formation of hematocolpos and hematometers. With the onset of menarche, acute, cyclic pains in the abdomen appear, with hematocolpos - aching, with hematometer - spastic. Diagnosis: probing the vagina - determining the depth of the lower part of the vagina; Ultrasound of the genitourinary system. Treatment: cruciform dissection of the hymen with its atresia; performing plastic surgery for aplasia and atresia of the vagina.

SECONDARY AMENORRHEA OF CENTRAL GENESIS 1. Hypothalamic genesis: psychogenic; with anorexia nervosa; with a lack of body weight; at false pregnancy; with excessive physical activity; neuroexchange-endocrine syndrome; Morgagni-Stewart-Morel syndrome.

PSYCHOGENIC AMENORRHEA Stress amenorrhea Occurs after emotional and mental trauma (acute and chronic). In this case, large doses of ACTH, endorphins and neurotransmitters are released, which leads to a decrease in the synthesis and release of gonadoliberins and, accordingly, gonadotropins. Clinic Asthenoneurotic syndrome Astheno-depressive syndrome Asthenohypochondriac syndrome

PSYCHOGENIC AMENORRHEA Autonomic functions are disturbed (anorexia, weight loss, sleep disorders in the form of early morning awakenings, decreased libido, heart pain, tachycardia, blood pressure fluctuations, constipation, dry skin and mucous membranes). Diagnosis: consultation of a psychoneurologist, hormonal studies (decrease in the level of LH and FSH, estradiol in the blood, a test with gonadotropin is positive). Treatment consists in the normalization of the regime of work and rest, the elimination of neuropsychic overload, stress. Apply sedatives, tranquilizers as prescribed by a psychoneurologist.

Anorexia nervosa It is observed in young women and adolescent girls with an unstable nervous system, after severe mental trauma, mental stress, due to self-treatment with hunger, with uncontrolled use of drugs that reduce appetite. There is an inhibition of cyclic secretion of Gn. RG. Clinic: there is an aversion to food, weakness, exhaustion, a decrease in basal metabolism. Hypotension, hypothermia. Treatment: high-calorie, fractional nutrition; vitamin therapy (vitamins A, C, group B); neurotropic medicines(seduxen); psychotherapy. If after 3-4 months menstruation is not restored, prescribe cyclic hormone therapy.

AMENORRHEA II WITH DEFICIENCY OF BODY WEIGHT Develops in girls and young women who follow a diet poor in proteins. A sharp decrease in body weight by 10-15% leads to amenorrhea, since adipose tissue is the site of extragonadal estrogen synthesis. With a decrease in body weight to 46 kg, the reaction of the pituitary gland to the introduction of gonadotropins sharply decreases or disappears. Treatment: normalization of nutrition, the use of enzyme preparations (creon, mezim, unenzyme, festal), vitamin therapy (vitamins B, C, E), valerian infusion, psychotherapy.

AMENORRHEA II WITH EXCESSIVE PHYSICAL ACTIVITY More often develops in girls who are actively involved in sports. With significant physical exertion, large doses of ACTH, endorphins and neurotransmitters are released, which leads to a decrease in the synthesis and release of gonadoliberins and, accordingly, gonadotropins. Initially, hypomenstrual syndrome develops, then amenorrhea. Treatment consists in reducing the intensity and frequency of physical activity.

PRINCIPLES OF THE SURVEY I. Complaints. II. Anamnesis (general, gynecological, genealogical). III. General examination with an emphasis on the following typobiological features: height, body weight, the nature of the deposition of adipose tissue in cases of obesity, body type, the presence or absence of somatic anomalies and stigmas, the condition of the mammary glands, the condition of the skin and its appendages. IV. Special gynecological examination. V. Exclusion of pregnancy (with amenorrhea): gynecological examination, ultrasound, immunological tests, control examination after 2-3 weeks.

SURVEY PRINCIPLES VI. Clinical and laboratory studies: 1. General clinical tests ( general analysis urine, blood, biochemical analysis blood, coagulogram, blood glucose, RW, Hbs. Ag, HIV). 2. Analysis of secretions for flora. 3. A smear for oncocytology. 4. Tests of functional diagnostics of ovarian activity: measurement of basal temperature; hormonal colpocytology; study of the phenomenon of mucus arborization. 5. Determination of the level of FSH, LH, testosterone, estrogens, progesterone and TSH in the blood. 6. Determination of the level of prolactin in the blood. 7. Urinalysis for level 17-KC.

PRINCIPLES OF THE SURVEY VII. Instrumental Methods examinations: 1. X-ray of the Turkish saddle. 2. Determination of fields of view. 3. Ultrasound examination (ultrasound). 4. Diagnostic curettage of the uterine cavity. 5. Hysteroscopy. 6. Laparoscopy. VIII. Diagnosis of the level of hormonal disorders - phased hormonal tests

Develops with primary dysfunction of the ovary. It can be primary - congenital or secondary, which appeared at a later age. The disease is based on the complete or partial absence of ovarian function. Congenital forms of primary amenorrhea are most often genetically determined and are the result of chromosomal abnormalities in the sex chromosome system. Of these, amenorrhea is the most common in gonadal dysgenesis. Congenital pathology of the gonads due to anomalies in the system of sex chromosomes or some adverse effects in the early antenatal period Gonadal dysgenesis manifests itself in the form of Shereshevsky-Turner syndrome. "pure" and "mixed" forms Shereshevsky-Turner syndrome develops in utero.

Diagnosis is usually based on clinical signs diseases Patients are characterized by low growth (not higher than 135-145 cm). Birth weight is also usually low - 2000-2300g at full-term pregnancy. On examination, a short neck is revealed, often with pterygoid folds running from the shoulder to the ears, broad shoulders, a barrel-shaped chest, widely spaced nipples of the mammary glands. The outer corners of the eyes are lowered (Mongoloid incision of the eyes), the third eyelid is often found, sometimes ptosis. The palate is high, the voice is nasal. Often, congenital malformations of the cardiovascular system (coarctation of the aorta, failure of the ductus arteriosus), kidneys and urinary system (horseshoe-shaped kidney, bifurcation of the ureters) are detected. mental development not violated, female sexual orientation. In the pubertal period, secondary sexual characteristics are poorly expressed. During a gynecological examination, pronounced signs of sexual infantilism attract attention. Pneumopelviography or ultrasound reveals aplasia or severe hypoplasia of the ovaries, the uterus is also sharply hypoplastic, sometimes it looks like a strand. genetic studies confirming a chromosomal abnormality: chromatin-positive cells are absent or their content is reduced in mosaicism. Dermatoglyphic examination shows an increase in the atd angle up to 55-60 degrees. Hormonal examination: a sharp increase in the content of follitropin and luteotropin in the blood. Excretion of estrogens is sharply reduced. Indicators of functional diagnostic tests: constantly low basal temperature, pupil and fern phenomena are absent, cervical index - 1-2 points. In the vaginal smear, up to 50% of parabasal cells are determined.

Treatment before puberty should be aimed at promoting growth. Prescribe anabolic steroids After 15-17 years, you can carry out replacement therapy estrogen. After that, they switch to cyclic hormonal therapy with estrogens and gestagens. what causes cyclic uterine bleeding.

"Pure" form of gonadal dysgenesis. The disease is pathogenetically identical to the Shereshevsky-Turner syndrome, but differs from it in the absence or slight severity of somatic anomalies. Diagnosis. Usually patients are tall or medium height, with a female phenotype. When analyzing morphograms, an intersex physique is determined with an increase in the circumference of the chest and a moderate decrease in the transverse dimensions of the body. There is a eunuchoid type of constitution with an increase in the length of the limbs and a decrease in the transverse dimensions of the body. There are no somatic anomalies. A gynecological examination reveals scant sexual hair growth, pronounced sexual infantilism (underdevelopment of the external genitalia, vagina and uterus). Ultrasound, laparoscopy or pneumopelviography reveals an infantile uterus, ovaries in the form of strands or sharply hypoplastic, sometimes absent (agonism).

In a genetic study, chromatin-positive cells are not detected or their content is normal (with a 46 XY karyotype), the content of hormones is the same as in Shereshevsky-Turner syndrome. Since there is no growth retardation in patients with a "pure form" of dysgenesis, anabolic hormones are not used.

"Mixed form" of gonadal dysgenesis. One of the forms of hermaphroditism (intersexualism). It is characterized by an indeterminate phenotype with an intersex structure of the external genital organs. In place of the gonads, on the one hand, an undifferentiated strand, on the other, a dysgenetic testicle

Diagnosis Growth retardation is common, as in Shereshevsky-Turner syndrome, although growth may be normal. Since at the birth of children with this pathology, the external genitalia are intersex, the passport sex is defined as both female and male. During puberty with the activation of testicular function, the phenotype approaches the male, clitoral hypertrophy increases, male-type sexual hair growth occurs, hypertrichosis, the voice timbre changes. Morphograms of the male type. Somatic anomalies are often absent, sometimes there is a lag in growth. In this contingent of patients, the gonads are prone to malignancy, so a particularly thorough examination is necessary. The dysgenetic testicle may be located in the rudimentary scrotum, in place of the ovary, in the inguinal canal; when a tumor occurs, the symptoms of virilization are especially pronounced. Gynecological examination reveals male-type sexual hair growth, hypertrophic clitoris, vagina and hypotrophic uterus, often a urogenital sinus. Hormonal studies: 17-KS excretion is increased to 40-60 µmol / day (normal for men) , 17-OKS- within normal limits. The excretion of follitropin and luteotropin is sharply increased - up to 20-30 and 300-500 IU / day, respectively, the content of follitropin and luteotropin in the blood is also increased - up to 20 and 30 mcg "l, respectively. Estrogen excretion is sharply reduced - up to 5-15 nmol / day

The results of tests, functional diagnostics are the same as in Shereshevsky-Turner syndrome. Treatment. In the prepubertal period, castration with plastic surgery of the genital organs. If a tumor is suspected at any age, a laparotomy is necessary; in case of a neoplasm, both the tumor and the gonad are removed. After surgery, hormonal therapy is carried out aimed at enhancing feminization: Testicular feminization is a false male hermaphroditism. It is characterized by varying degrees of feminization in individuals with a genetic male sex, 46 XY karyotype. Diagnosis. With complete syndrome (Morris syndrome) - typically female morphograms, normally developed mammary glands and external genitalia in the presence of primary amenorrhea. Sexual hair growth is absent or insignificant. With an incomplete syndrome - an increase in the clitoris, morphograms according to the male type, the mammary glands are absent. inguinal hernia, in which the testicles are palpated, sometimes they are in the labia majora, less often in abdominal cavity During a gynecological examination, a vagina (sometimes rudimentary) is revealed, ending blindly, the uterus is absent. An x-ray examination determines aplasia of the uterus. The determination of sex chromatin has the greatest diagnostic value: the percentage of chromatin-positive cells is not more than 5, 20-70% of the cells contain bodies of Y - chromatin (the norm for men).

Gnpohormonal amenorrhea, not associated with the pathology of sex chromosomes - If the hormone-producing ovarian tissue is damaged in childhood, primary amenorrhea occurs, accompanied by defects in somatic development. When the ovaries are affected during the reproductive period, secondary amenorrhea occurs without somatic pathology.

Amenorrhea with ovarian involvement in childhood (eunuchoidism). Pathology occurs when the ovaries are destroyed due to the tuberculous process in the small pelvis, typhoid, and also after surgical removal of the ovaries, X-ray therapy. Diagnostics. Pronounced sexual infantilism with disproportionate development: high growth with a predominance of longitudinal body dimensions - a narrow chest, long limbs, narrow pelvis. The mammary glands are not developed (Ma1), sometimes there is excessive deposition of fat in the abdomen, thighs and mammary glands. Sexual hair growth is poorly expressed (AX 1 and PI). Gynecological examination reveals underdevelopment and weak pigmentation of the labia majora and labia minora, a narrow vagina. The uterus is small, round, the cervix is ​​long. On ultrasound and pneumopelviogram, hypoplasia of the ovaries and uterus is determined. hormonal studies of the function of the pituitary gland, (the content of follitropin and luteotropin in the blood and urine is sharply increased) Indicators. Functional diagnostic tests: single-phase basal temperature, absence of pupil and fern phenomenon, cervical index 1-3 points. In the vaginal smear - parabasal cells< 70-80%). Экскреция эстрогенов снижена, прегнандиол не определяется, содержание эстрадиола и прогестерона в крови также резко снижено Лечение рассчитано на стимуляцию развития половых органов, молочных же­лез и замедление роста (первый этап), а затем - на появление циклических кро­вотечений (второй этап). Гормональную терапию проводят под контролем на­сыщенности организма.

Amenorrhea with ovarian damage during the reproductive period. Hypofunction of the ovaries is most often associated with chronic infectious processes.

Diagnostics. Depending on the severity of the course of the disease, there are two degrees of amenorrhea:

/ light and moderate- relatively little pronounced ovarian failure, in which amenorrhea is secondary, the uterus is of normal size, the endometrium is functioning;

// - severe - deep ovarian failure, observed with amenorrhea of ​​a longer duration, when the uterus is significantly reduced in size, dense, the endometrium does not function. Amenorrhea II degree is more common in people over the age of 30 years.

When establishing the diagnosis of amenorrhea, data on past infectious and other diseases, harmful factors, as well as previous menstrual function are of decisive importance. Hormonal studies. The results depend on the degree of hypostrogenia. With a pronounced pathology, a decrease in the excretion of estrogens in the blood and in the urine is observed. The results of functional tests indicate estrogen deficiency varying degrees- single-phase basal temperature, absence or mild (+) pupil and fern phenomena, CI decrease to 0-10% and (or) the presence of parabasal cells in the vaginal smear Histological examination of the endometrial scraping indicates the complete absence of progesterone exposure: the glands are straight, with a round cross section. Treatment begins with the elimination of possible intoxication, adverse working and living conditions. Carry out hormonal cyclic therapy (3-6 months).

uterine AMENORRHEA It develops with an unchanged function of the hypothalamus - pituitary gland - ovaries as a result of a pathological process in the endometrium. If the destruction of the endometrium occurred before the onset of puberty, amenorrhea is primary; if after - secondary.

Diagnostics. Normal somatic and sexual development Amenorrhea occurs after tuberculous or gonorrheal endometritis, intrauterine infusions of iodine, curettage of the uterus, after childbirth or abortion. Diagnostic value has hysterosalpingography and hysteroscopy. basal temperature is biphasic, pupil and fern phenomena appear within a month (3 points), cervical index ranges from 7 to 12 points Excretion of hormones and their blood levels are within normal limits or slightly reduced. Great importance for differential diagnosis with Other forms of amenorrhea, hormonal tests have results: in the uterine form of amenorrhea, the test with progesterone is negative (no bleeding after the administration of progesterone) The combined test with estrogens and progesterone is also negative (there is no bleeding after the administration of both drugs). Both tests are positive for ovarian hypofunction. Treatment depends on the etiological factor that caused the pathology. In the presence of intrauterine adhesions (Asherman's syndrome) that occur after repeated curettage or the introduction of cauterizing substances into the uterine cavity, the treatment consists in the destruction of adhesions with the obligatory introduction of polyvinyl protectors into the uterine cavity for a period of 3-4 weeks. After the operation, diathermy is prescribed for the lower abdomen and cyclic hormonal therapy.

Amenorrhea of ​​central origin includes dysfunctions of both the cerebral cortex and subcortical structures (hypothalamic-pituitary amenorrhea). Violations of the hypothalamic-pituitary system can be functional, organic and the result of congenital pathology.

Amenorrhea of ​​central origin is more often functional and, as a rule, occurs as a result of exposure to adverse environmental factors. The mechanisms of the disorder are realized through the neurosecretory structures of the brain that regulate the tonic and cyclic secretion of gonadotropins. Under the influence of stress, there is an excessive release of endogenous opioids that reduce the formation of dopamine, as well as a decrease in the formation and release of GnRH, which can lead to amenorrhea. With minor violations, the number of anovulatory cycles increases, and luteal phase insufficiency appears.

Most often, the emergence of central forms of amenorrhea is preceded by mental trauma, neuroinfection, intoxication, stress, complicated pregnancy and childbirth. Amenorrhea is observed in every 3rd patient with schizophrenia and manic-depressive psychosis, especially during an exacerbation. Psychological stress and childhood experiences are important infectious diseases. Physical overload associated with significant emotional and volitional stress can cause amenorrhea with mental, asthenoneurotic, asthenodepressive or asthenohypochondriac disorders. Menstruation stops suddenly. Along with amenorrhea, irritability, tearfulness, headache, memory impairment, working capacity, and sleep disturbance are observed. During the war, as a result of forced starvation, women dramatically lost weight, which led to a violation in the hypothalamic-pituitary region and to the so-called wartime amenorrhea. Psycho-emotional stress also contributed to this.

Functional disorders hypothalamic-pituitary system lead to the development anorexia nervosa, Itsenko-Cushing's disease, gigantism, functional hyperprolactinemia. Causes of functional disorders of the hypothalamic-pituitary system:

Chronic psychogenic stress;

Chronic infections (frequent tonsillitis) and especially neuroinfections;

endocrine diseases;

Taking drugs that deplete dopamine stores in the central nervous system (reserpine, opioids, monoamine oxidase inhibitors) and affect the secretion and metabolism of dopamine (haloperidol, metoclopramide).

Anatomical disorders of the hypothalamic-pituitary structures, leading to Sheehan's syndrome and hyperprolactinemia, are as follows:

Hormonally active pituitary tumors: prolactinoma, mixed prolactin- and ACTH-secreting pituitary adenomas;

Damage to the pituitary stalk due to trauma or surgical intervention, exposure to radiation;

Necrosis of the pituitary gland tissue, thrombosis of the pituitary vessels.

Congenital pathology of the hypothalamic-pituitary system can lead to adiposogenital dystrophy.

Regardless of the causes of damage to the hypothalamic-pituitary region, there is a violation of the production of hypothalamic GnRH, which leads to a change in the secretion of FSH, LH, ACTH, STH, TSH and prolactin. In this case, the cyclicity of their secretion may be disturbed. When the hormone-forming function of the pituitary gland changes, various syndromes arise. A decrease in the secretion of FSH and LH leads to a violation of the development of follicles and, consequently, insufficient production of estrogens by the ovaries. Secondary hypoestrogenism, as a rule, is accompanied by hyperandrogenism, which, in turn, contributes to the emergence of a viril syndrome, moderately pronounced in hypothalamic-pituitary disorders.

Since the pituitary gland is also responsible for metabolic processes, with damage to the hypothalamic-pituitary region, patients are distinguished by a characteristic appearance: obesity, moon face, fatty apron, striae on the abdomen and thighs, however, excessive thinness with mild secondary sexual characteristics is also possible. Obesity and severe weight loss as a result of disorders of the hypothalamic-pituitary region exacerbate the manifestations of hormonal dysfunction.

to amenorrhea anorexia nervosa leads to a sharp decrease in the secretion of gonadotropins. This is often observed with an insistent desire to lose weight and a rapid decrease in body weight by 15% or more. This pathology is common among teenage girls who exhaust themselves with diet and physical activity, and can be the beginning of a mental illness. The absence of menstruation is one of the first signs of the onset of the disease, which leads girls to a gynecologist. On examination, there is a sharp decrease in subcutaneous adipose tissue with female type physique. Secondary sexual characteristics are developed normally. Gynecological examination reveals moderate hypoplasia of the external and internal genital organs. Continued weight loss can lead to bradycardia, hypotension, and hypothermia. In the future, irritability, aggressiveness, cachexia with total loss appetite and aversion to food. The hypoestrogenic state, along with a lack of nutrition, predisposes patients to osteoporosis.

Syndrome (disease) Itsenko-Cushing characterized by increased production of corticoliberin by the hypothalamus. This causes activation of the adrenocorticotropic function of the anterior pituitary due to hyperplasia of basophilic cells and, as a result, hypertrophy and hyperfunction of the adrenal glands, excessive production of glucocorticosteroids and androgens. The consequence of such hormonal disorders is hypercortisolism, which leads to hypokalemic acidosis, increased gluconeogenesis processes, an increase in blood sugar and, as a result, to steroid diabetes. The disease is observed at any age. In children, Itsenko-Cushing's disease is accompanied by virilization of varying severity, in adults, amenorrhea is observed at the onset of the disease, later signs of virilization appear. Characterized by disproportionate obesity with the deposition of subcutaneous fatty tissue on the face, neck, upper body. In patients, the face is round, cyanotic red.

The skin is dry, atrophic, with a marble pattern and areas of pigmentation and acne. Crimson-red stripes of stretching on the chest, abdomen, thighs.

Gigantism also becomes a consequence of hyperplasia of eosinophilic cells of the pituitary gland with increased production of somatotropic and lactogenic hormones. With hyperproduction of growth hormone, growth is excessively high, relatively proportional or disproportionate. An excessive increase in height is usually noticed in the prepubertal and pubertal periods, over a number of years. Over time, acromegaloid enlargement of facial features may develop. From the very beginning of the disease, hypogonadism, primary amenorrhea, or early cessation of menstruation are noted.

To Sheehan's syndrome lead to structural changes in the pituitary gland due to massive postpartum or post-abortion bleeding. At the same time, necrotic changes and intravascular thrombosis in the pituitary gland are detected. Pituitary ischemia also contributes to the physiological decrease in ACTH release in the postpartum period. Intravascular thrombosis also leads to changes in the liver, kidneys, and brain structures. The severity of the clinical manifestations of Sheehan's syndrome depends on the size and localization of the pituitary lesion and, accordingly, the insufficiency of its gonadotropic, thyrotropic, adrenocorticotropic functions. The disease is often accompanied by a clinical picture of hypofunction of the thyroid gland or vegetovascular dystonia of the hypotonic type (headache, fatigue, chilliness). A decrease in the hormonal function of the ovaries is manifested by oligomenorrhea, anovulatory infertility. The symptomatology of the total hypofunction of the pituitary gland is due to a pronounced insufficiency of gonadotropic, thyrotropic and adrenocorticotropic functions: persistent amenorrhea, hypotrophy of the genital organs and mammary glands, baldness, memory loss, weakness, adynamia, weight loss.

When collecting an anamnesis, the connection between the onset of the disease and complicated childbirth or abortion is clarified. You can clarify the diagnosis by lowering the blood levels of gonadotropins, TSH, ACTH, as well as estradiol, cortisol, T 3 and T 4.

Hyperprolactinemia. The occurrence of amenorrhea of ​​the hypothalamic-pituitary genesis is often accompanied by excessive secretion of prolactin - hyperprolactinemia. Prolactin is the only anterior pituitary hormone whose secretion is constantly suppressed by the hypothalamus and increases sharply after the release of the pituitary gland from hypothalamic control. Physiological hyperprolactinemia is observed during pregnancy and lactation, in almost healthy women during sleep, after physical exertion, as well as during stress. Hyperprolactinemia is possible due to damage to intrauterine receptors with frequent curettage of the mucous membrane of the uterine body, manual examination of the walls of the uterus after childbirth.

Etiology and pathogenesis. The cause of hyperprolactinemia can be both anatomical and functional disorders in the hypothalamus-pituitary system. In addition, the production of prolactin is affected by:

Estrogens, estrogen-containing oral contraceptives;

Drugs that affect the secretion and metabolism of dopamine (haloperidol, metoclopramide, sulpiride);

Drugs that deplete dopamine stores in the central nervous system (reserpine, opioids, monoamine oxidase inhibitors);

Stimulants of the serotonergic system (hallucinogens, amphetamines);

Hypothyroidism.

The pathogenesis of hyperprolactinemia is a violation of the tonic dopaminergic inhibitory control of prolactin secretion caused by dysfunction of the hypothalamus. Of the endogenous prolactin-inhibiting substances, dopamine is the most important. A decrease in its content in the hypothalamus leads to a decrease in the level of prolactin inhibitory factor and an increase in the amount of circulating prolactin. Continuous stimulation of prolactin secretion leads to hyperplasia of prolactotrophs, and then micro- and macroadenoma of the pituitary gland can form.

In 30-40% of women with hyperprolactinemia, the level of adrenal androgens, DHEA and DHEA-S, is elevated. Hyperandrogenism in hyperprolactinemia is explained by the common hypothalamic regulation of prolactin and ACTH secreting functions of the pituitary gland. In addition, prolactin receptors have been found in the reticular zone of the adrenal cortex.

The mechanism of reproductive dysfunction against the background of hyperprolactinemia is as follows. In the hypothalamus, under the influence of prolactin, the synthesis and release of GnRH and, accordingly, LH and FSH decrease. In the ovaries, prolactin inhibits gonadotropin-dependent steroid synthesis, reduces the sensitivity of the ovaries to exogenous gonadotropins.

Clinical symptoms. Hyperprolactinemia is manifested by disorders menstrual cycle according to the type of hypo-, oligo-, opso- and amenorrhea, as well as infertility.

In women with hyperprolactinemia, galactorrhea is often observed, and it does not always correlate with the level of prolactin. So, galactorrhea is possible even at its normal level, which is associated with hypersensitivity of prolactin receptors in the mammary gland.

The so-called asymptomatic hyperprolactinemia is distinguished, in which the level of biologically active prolactin is increased. About 50% of women with hyperprolactinemia report headache and dizziness, a transient increase in blood pressure.

Diagnostics hyperprolactinemia includes the study of the general and gynecological history, a detailed general therapeutic examination. special attention deserves the state of the endocrine system, mainly the thyroid gland and the adrenal cortex.

An increase in the level of prolactin in the peripheral blood plasma is one of the confirmations of hyperprolactinemia. The ratio of the content of gonadotropic and sex hormones is also important. For the differential diagnosis of hyperprolactinemia, and in particular its transient form, it is necessary to repeatedly determine prolactin over time. Most informative functional tests with dopamine agonist - bromocriptine (Parlodel ♠) and dopamine antagonist - metoclopramide (Cerucal ♠). Functional hyperprolactinemia is not accompanied by

changes in the Turkish saddle on the radiograph, with CT and MRI in the case of an increase in the level of prolactin to 2000 mIU / l.

To exclude anatomical changes in the pituitary gland, an X-ray examination of the skull is performed in order to identify changes in the area of ​​the Turkish saddle. With pituitary macroadenoma, the size of the Turkish saddle is increased, its bottom is 2-3-contour, there are signs of sclerosis of the Turkish saddle. The level of prolactin in macroadenoma exceeds 5000 mIU / l. With pituitary macroadenoma, amenorrhea and galactorrhea are noted. Diagnosis of pituitary microadenoma is possible using CT or MRI. The level of prolactin in microadenoma ranges from 2500 to 10,000 mIU / l.

Treatment hyperprolactinemia is carried out taking into account its form. Dopamine agonists are widely used to treat functional hyperprolactinemia. Treatment begins with the appointment of 1 / 2 tablets of bromocriptine per day with meals, then the dose is increased every two days by 1 / 2 tablets, bringing up to 3-4 tablets per day under the control of blood prolactin levels and basal temperature. When restoring ovulatory menstrual cycles, the dose is reduced to 1 tablet per day; such treatment is carried out for 6-8 months. Fertility is restored in 75-90% of cases. In case of insufficiency of the 2nd phase of the cycle, you can additionally prescribe clomiphene from the 5th to the 9th day of the menstrual cycle, which stimulates ovulation. To drugs latest generation in the treatment of hyperprolactinemia are quinagolide (norprolac ♠) and cabergoline (dostinex ♠) (1 mg per week for 3-4 weeks). These are long-acting drugs with minimal side effects.

With pituitary microadenoma, therapy is also carried out with bromocriptine or its analogues. At long-term treatment develop dystrophic changes in a tumor decreases until it disappears completely. Pregnancy during treatment in patients with pituitary microadenoma proceeds safely. During pregnancy, the supervision of a neurologist and an ophthalmologist is mandatory.

Macroadenoma of the pituitary gland is an indication for surgical treatment, which is performed by a neurosurgeon, or for radiation therapy.

Adiposogenital dystrophy is a consequence of congenital pathology of the hypothalamic-pituitary region. The disease is accompanied by progressive obesity as a result of a violation of the central regulation of satiety due to damage to the paraventricular nuclei of the hypothalamus. A decrease in the gonadotropic function of the pituitary gland causes the underdevelopment of the reproductive system (hypogonadism). Infectious processes in the pituitary region and pituitary adenoma with hyperplasia of eosinophilic pituitary cells can lead to hyperproduction of growth hormone and excessively high growth (relatively proportional or disproportionate gigantism).

Ovarian amenorrhea

Ovarian forms of amenorrhea are caused by functional, organic changes and congenital pathology of the ovaries. Most common cause functional and morphological disorders on

ovarian level of regulation of the menstrual cycle is polycystic ovary syndrome(PCOS). A decrease or depletion of the hormonal function of the ovaries is noted with resistant ovary syndrome(SRY) and ovarian failure syndrome(SIA). Organic changes in the ovaries, accompanied by menstrual dysfunction, are due to hormonally active ovarian tumors(See "Tumours of the ovaries").

PCOS - pathology of the structure and function of the ovaries with a very diverse clinical picture, the most constant component of which is anovulation. PCOS consists of significant morphological changes in the ovaries. This is a smooth and dense albuginea, proliferation of connective tissue, an increase in the number of cystic-modified follicles in the absence of a dominant follicle. Polycystic ovaries are enlarged (>9 cm 3 ) as a result of proliferation of connective tissue, the albuginea is pearly white. When cut, the cortical layer resembles a honeycomb, since the follicles are of different diameters.

PCOS is accompanied by chronic anovulation, infertility, often metabolic disorders, decreased glucose tolerance, as well as hyperandrogenism and, consequently, virilization. Excessively high production of androgens contributes to the growth of interstitial tissue

with PCOS.

According to the results of numerous hormonal and clinical studies, primary (Stein-Leventhal syndrome, described in 1935) and secondary polycystic ovaries are distinguished, the latter developing with adrenal hyperandrogenism, hyperprolactinemia, neuroexchange-endocrine syndromes.

Most convenient for use in clinical practice proposed by M.L. Crimean classification, including three forms:

A typical form, accompanied predominantly by ovarian hyperandrogenism, is primary polycystic ovaries;

Combined or mixed form with both ovarian and suprarenal hyperandrogenism;

Central form with hyperandrogenism and severe dysfunction of the central sections reproductive system with a predominance of secondary polycystic ovaries.

Etiology and pathogenesis. The etiology and pathogenesis depend on the form of PCOS. In the 1960s, pathogenesis typical shape PCOS (Stein-Leventhal syndrome) has been associated with a genetically determined deficiency of ovarian enzymes that blocks the conversion of androgens to estrogens. However, later it was shown that the activity of granulosa cells depends on FSH. Violation of the process of aromatization of androgens into estrogens leads to the accumulation of testosterone (active androgen) and a decrease in the level of estrogen in the ovaries. As a result, the cyclic secretion of gonadotropins is disrupted by the feedback mechanism, which, in turn, leads to hyperplasia of the stroma and theca cells of the ovaries, excessive or increased production of androgens. Androgens are partially converted to estrone, and some of the estrone is converted to estradiol. However, this is not enough for

occurrence of preovulatory and luteal peaks. The menstrual cycle becomes monophasic.

In pathogenesis mixed (form PCOS can be triggered by primary dysfunction of the adrenal cortex or a transient excess of adrenal androgens during the adrenarche period. In peripheral tissues, androgens are partially converted into estrogens. Upon reaching the critical body weight, the peripheral conversion of androgens in adipose tissue increases. This is accompanied by an increase in the synthesis of LH in the pituitary gland and a violation of the ratio of LH / FSH, which leads to hyperplasia of the theca cells and ovarian stroma. These structures synthesize androgens in excess. Hyperandrogenism inhibits follicular maturation, leads to anovulation, and further suppresses FSH secretion. Thus the vicious circle closes.

Participation of brain structures in development central form PCOS is confirmed by a chronological relationship between the onset of the disease and a stressful state (the onset of sexual activity, mental trauma, childbirth, abortion). CNS dysfunction may result from acute or chronic infection or intoxication. At the same time, the synthesis and release of endogenous opioids increase, which disrupts the dopaminergic regulation of GnRH secretion, leads to an increase in the basal level of LH secretion, a relative decrease in FSH production, and impaired folliculogenesis. An increase in LH release in PCOS is due to both a primary violation of GnRH synthesis and chronic anovulation; these effects are mutually potentiated.

Modern representation about the pathogenesis of PCOS, in addition to disorders of the hypothalamic-pituitary complex, ovaries and adrenal glands, includes metabolic disorders and autoparacrine factors regulating steroidogenesis in the ovaries. Metabolic disorders are associated with the insulin-glucose system, since insulin is involved in the production of ovarian androgens. Obesity does not play a decisive role in the pathogenesis of PCOS, however, as a result of hyperinsulinemia and insulin resistance, existing endocrine disorders are exacerbated. In patients with obesity and insulin resistance, chronic hyperinsulinemia stimulates the formation of insulin-like growth factor-1 (IGF-1). The latter, through specific receptors, increases the formation of androgens in the cells and interstitial tissue of the ovaries. In addition, insulin is able to inhibit the formation of sex hormone-binding globulins in the liver, as a result of which the free biologically active fraction of testosterone increases in the blood.

According to the existing hypothesis, the stimulating effect of insulin on the synthesis of androgens in the ovary is due to a genetic predisposition.

PCOS develops in women with normal body weight. In their blood, the level of growth hormone is increased, which causes the formation of IPFR-1 in granulosa cells and enhances the formation of ovarian androgens. The study of hormone biosynthesis in granulosa cells of polycystic ovaries showed

It was shown that luteinized cells lose their ability to synthesize progesterone. This is one of the possible mechanisms of anovulation in patients.

Clinical symptoms. The clinical manifestations of PCOS are very different, but the main ones in all forms of PCOS are hypo-, opso-, oligo- and amenorrhea. Violations of folliculogenesis lead to the development of anovulatory primary and secondary infertility.

In the typical form of PCOS, menstrual irregularities begin with menarche. In the mixed form of PCOS, late menarche is combined with a violation of the menstrual cycle in the future by the type of secondary amenorrhea. In the reproductive age, chronic anovulation and infertility, more often primary, are observed. In the central form of PCOS, menarche is normal, but the menstrual cycle is unstable. Subsequently, this leads to hypo-, opso-, oligo- or amenorrhea. Violations of the reproductive function are in miscarriage at short terms and secondary infertility. In addition to menstrual dysfunction, dysfunction of the hypothalamic-pituitary system is noted. The onset of the disease may be associated with stress, adenovirus infection, brain injury.

main reason When patients of young age go to the doctor, excessive hair growth becomes, the frequency of which in PCOS is, according to different authors, from 50 to 100%. Hirsutism in typical PCOS develops gradually from the menarche period. There is excessive hair growth on the upper lip, chin, along the white line of the abdomen. Pronounced hirsutism and hypertrichosis are not typical for this form of PCOS, but with a mixed form, hirsutism is observed in all patients. Areas of excessive hair growth - inner and outer thighs, white line of the abdomen, upper lip, shins. Hair growth starts at or before menarche. In the central form of PCOS, hirsutism is detected in 90% of patients, occurs 3-5 years after menstrual dysfunction, already against the background of obesity, and is more pronounced in reproductive age. In these patients, dystrophic changes can be noticed: stretch marks on the chest, abdomen, thighs, brittle nails and hair.

The clinical picture of PCOS is largely determined by general metabolic disorders - such as dyslipidemia, carbohydrate metabolism disorders, and an increased risk of developing genital hyperplastic processes. These violations can lead to early development atherosclerotic changes in blood vessels, hypertension, coronary disease hearts. In 50% of patients with typical PCOS with adolescence there is an increased body weight with a uniform distribution of subcutaneous adipose tissue. Obesity is rare in mixed PCOS. In the central form, the leading complaint is overweight. Obesity reaches II-III degree; adipose tissue is localized mainly on the shoulder girdle, lower abdomen and thighs.

Diagnostics PCOS should begin with a thorough history and physical examination. The formation of PCOS begins with puberty

period and is accompanied by a violation of the formation of menstrual function. Primary polycystic ovaries cause irregular menstruation from puberty, which distinguishes them from secondary polycystic ovaries.

The clinical criterion for the diagnosis of PCOS is hirsutism (in 69% of patients), which appears simultaneously with the onset of puberty. The severity of other clinical manifestations of hyperandrogenism is different. With the progression of symptoms of virilization (hypertrophy of the clitoris, defeminization of the figure, lowering the timbre of the voice), it is necessary to exclude hormonally active tumors of the ovaries and adrenal glands; this is usually atypical for PCOS.

The main methods for diagnosing PCOS include echography of the pelvic organs, the study of blood plasma hormones, laparoscopy with biopsy and histological examination ovarian tissue.

Bilateral enlargement of the ovaries is pathognomonic for PCOS, often with a hypoplastic uterus, which is clearly defined by echography. The echoscopic picture of the ovaries with transvaginal ultrasound (Fig. 9.1) shows an increase in the volume of the ovaries by more than 9 cm 3 (on average 16-20 cm 3), hyperplastic stroma, more than 10 atretic follicles located along the periphery under a thickened capsule.

The hormonal criteria for the diagnosis of PCOS include an LH/FSH ratio of more than 2.5-3. However, in recent years it has been shown that normal level gonadotropic hormones does not exclude the diagnosis of PCOS. So, the level of DHEA and DHEA-S is normal in the typical form and elevated in the presence of the adrenal component (mixed form of PCOS). In the central form of PCOS, the LH/FSH ratio is the same as in typical PCOS, but the history and clinical symptoms allow verification of the diagnosis.

Rice. 9.1. Polycystic ovary syndrome. ultrasound

An obligatory stage in the examination of patients with PCOS is the diagnosis of metabolic disorders: hyperinsulinemia and insulin resistance. BMI over 25 kg/m 2 and dyslipidemia indicate hyperinsulinemia and insulin resistance.

A typical laparoscopic picture of the ovaries in PCOS: increased size (up to 5-6 cm in length and 4 cm in width), smoothed, thickened, pearly whitish capsule. The absence of translucent small follicular cysts and ovulation stigmas indicates a pronounced thickness of the ovarian capsule, which sometimes makes biopsy difficult (Fig. 9.2).

Treatment. The sequence of therapeutic measures in patients with PCOS depends on the complaints, clinical manifestations and age of the patient. Since the main reason for seeking medical attention in patients of reproductive age is infertility, the goal of treatment is the restoration of menstrual and reproductive functions at the same time, the prevention of hyperplastic processes in target organs and the correction of the prevailing symptom complex. For this purpose, conservative and surgical methods of treatment are used.

With obesity, the first stage of treatment (regardless of the form of the disease) is the normalization of body weight. However, curative fasting is contraindicated; largest healing effect gives a combination of diet therapy with physiotherapy measures - massage, physical therapy, acupuncture. Weight loss leads to the normalization of the endocrine blood profile, a decrease in the level of insulin and androgens, and the restoration of regular menstruation. In PCOS of central origin, the use of drugs that correct neurotransmitter metabolism (phenytoin - diphenin *), beclamid - chloracone *) is pathogenetically justified. It is possible to prescribe orlistat, which selectively inhibits lipid metabolism, or subitramine, which blocks the saturation center.

The next stage of treatment is the stimulation of ovulation. Stimulation begins with the use of clomiphene, which has an antiestrogenic effect by blocking estradiol receptors. After discontinuation of the drug, the gonadotropic function returns to normal. Clomiphene does not directly stimulate

Rice. 9.2. Biopsy of the ovary. Laparoscopy

ovaries, but causes ovulation due to short-term normalization of the hypothalamic-pituitary-ovarian system. The drug is prescribed 100 mg from the 5th to the 10th day of the menstrual cycle. Treatment with clomiphene restores ovulation in 48-80% of patients, pregnancy occurs in 20-46%. With resistance to clomiphene, ovulation stimulation can be carried out with gonadotropic drugs (pergonal ♠, humegon ♠) according to individual schemes. However, stimulation of ovulation, especially when elevated level insulin and obesity, increases the risk of hyperstimulation syndrome or may lead to ovarian unresponsiveness.

Treatment of women who do not plan pregnancy is aimed at restoring the menstrual cycle, treating hirsutism and preventing long-term effects PCOS, impairing the quality of life. For this purpose, combined oral contraceptives (COCs) are used, which reduce the level of androgens, normalize the menstrual cycle and help prevent endometrial hyperplastic processes. In patients with PCOS and impaired fat metabolism, COCs should be combined with drug therapy insulin resistance. The combination of COCs with antiandrogens potentiates a decrease in androgen secretion. Antiandrogens block androgen receptors in the target tissue and suppress gonadotropic secretion. The use of drugs with antiandrogenic properties (Diana-35 *) has significantly expanded the therapeutic options for PCOS. The antiandrogenic effect of Diane-35 ♠ can be enhanced by the additional administration of cyproterone (Androkur ♠) at 25-50 mg from the 5th to the 15th day of the menstrual cycle. Duration of treatment - from 6 months to 2 years or more.

Spironolactone (veroshpiron ♠) has an antiandrogenic effect, blocking peripheral receptors and androgen synthesis in the adrenal glands and ovaries. Its long-term use at a dose of 100 mg / day reduces hirsutism. However drug treatment hirsutism is not always effective.

Surgical treatments for PCOS are often performed using endoscopic access. Surgery normalizes gona-dotropic secretion by reducing the volume of androgen-secreting tissues of polycystic ovaries. As a result, the level of extragonadal estrogens decreases, which increases the sensitivity of the pituitary gland to GnRH. To surgical methods PCOS correction includes wedge resection, thermocauterization (Fig. 9.3), thermovaporization and decapsulation of polycystic ovaries. Surgical treatment is most effective in the typical form of PCOS.

The ineffectiveness of wedge-shaped resection of the ovaries in some patients indicates combined adrenal-ovarian hyperandrogenism.

The incidence of endometrial hyperplastic processes and the risk of endometrial cancer in patients with PCOS, especially in typical and central forms, determine the active management tactics (hysteroscopy with separate diagnostic curettage) even in the absence of complaints. Timely diagnosis and treatment of such patients are endometrial cancer prevention measures.

Rice. 9.3. Ovary after cauterization. Laparoscopy

Resistant ovary syndrome. Rarely, ovarian failure may be due to resistant ovarian syndrome (ROS; Savage's syndrome). In women younger than 35 years, amenorrhea, infertility, micro- and macroscopically unchanged ovaries are noted with a high level of gonadotropins. Secondary sexual characteristics are developed normally. The causes of SOS have not been studied; the autoimmune nature of this pathology is assumed. It is known that hypergonadotropic amenorrhea can be combined with autoimmune diseases: Hashimoto's disease, myasthenia gravis, alopecia, thrombocytopenic purpura, autoimmune hemolytic anemia. Ovarian resistance to high levels of gonadotropins may be due to an abnormal FSH molecule or lack of biological activity of the hormone. A large role is given to intraovarian factors involved in the regulation of ovarian function. There is evidence of the influence of iatrogenic factors - radiotherapy, cytotoxic drugs, immunosuppressants, ovarian surgery. The development of resistant ovaries can be facilitated by damage to the ovarian tissue in tuberculosis, mumps, and sarcoidosis.

Clinical symptoms and diagnosis. Most patients associate the onset of the disease with stress, severe viral infections. The first menstruation, as a rule, occurs in a timely manner, and after 5-10 years amenorrhea develops, but 84% of patients subsequently have episodic menstruation. Pregnancy and childbirth are observed in 5% of patients. Patients with EDS have a correct physique, satisfactory nutrition, and well-developed secondary sexual characteristics. Periodically, they feel flushes of heat to the head. When examined according to tests of functional diagnostics, they reveal signs of ovarian hypofunction: thinning of the mucous membranes of the vulva and vagina, a weakly positive "pupil" phenomenon, low rates KPI (from 0 to 25%).

During gynecological examination, echography, laparoscopy, the uterus and ovaries are somewhat reduced. Most authors believe that the diagnosis of EOC can only be made after laparoscopy and ovarian biopsy, followed by a histological examination, in which

primordial and preantral follicles are formed. With laparoscopy, translucent follicles are visible in the ovaries.

Hormonal studies indicate a high level of FSH and LH in the blood plasma. The level of prolactin is normal.

Hormonal tests are of great diagnostic value. A decrease in the level of FSH with the introduction of estrogens and an increase in the level of FSH and LH in response to the administration of luliberin indicate the preservation of the feedback mechanism between the hypothalamic-pituitary system and sex steroids.

Treatment. The treatment of SOC presents great challenges. In the treatment of gonadotropins received conflicting data. Some authors noted an increase in follicles and menstrual-like discharge against the background of the introduction of FSH and LH, others - only the growth of follicles (empty follicles) without an increase in blood estrogen levels.

The appointment of estrogens is based on the blockade of endogenous gonadotropins and the subsequent rebound effect (reflection effect). In addition, estrogens increase the number of gonadotropic receptors in the ovaries and, possibly, thus increase the response of follicles to endogenous gonadotropins. Restoration of the generative function is possible only with the help of assisted reproductive technologies (IVF of a donor egg).

An integral part of the work of a neurologist is the consultation of patients referred by gynecologists from antenatal clinics in connection with their examination regarding their amenorrhea and / or infertility (arising in connection with / without connection with amenorrhea). This article discusses possible reasons amenorrhea associated with damage to the nervous system, and therefore requiring mandatory participation and / or control in the examination and treatment of such patients by a neurologist and / or psychiatrist.

Consider the definition of "amenorrhea" and the possible causes of its occurrence.

Amenorrhea - is the absence of menstruation for 6 months or more.

CLASSIFICATION OF AMENOREA:
TRUE - these are disorders in which there are no cyclic processes in the reproductive system of a woman; true amenorrhea is divided into physiological and pathological:
- physiological amenorrhea - this is the absence of menstruation during puberty, during pregnancy, during breastfeeding (lactation) and in old age;
- pathological amenorrhea - occurs with various diseases of the female reproductive system: lesions of the central nervous system and hormonal disorders (hypothalamus, ovaries, pituitary and adrenal glands), acute and chronic infectious diseases (tuberculosis, adenovirus infection, septic conditions), severe intoxication (poisoning by heavy metals, alcohol, household poisons), metabolic disorders (malnutrition, anemia, intense physical activity); in turn, pathological amenorrhea can be primary and secondary:
- - primary pathological amenorrhea- the absence of menstruation in a woman who has reached the age of 16, or in a woman who has never had them;
- - secondary pathological amenorrhea- the absence of menstruation for 3 or more terms of the normal menstrual cycle in a woman who previously menstruated;
FALSE - this is a condition in which cyclic processes in the reproductive system of a woman occur, however, there is no external bleeding due to psychogenic causes (false or imaginary pregnancy, emotional stress), mechanical obstacles (infection of the hymen, vagina; infection of the cervical canal (cervical canal ); septa and adhesions in the uterine cavity); false amenorrhea occurs due to a violation of the development of the female genital organs and after reactive changes in the mucous membrane of the uterine cavity (endometritis, endocervicitis, frequent curettage of the walls of the uterine cavity, including during medical abortions).

There is also the following classification of pathological amenorrhea:
by severity :
I degree (mild) - the duration is not more than 1 year, there are no complaints, the uterus is slightly enlarged, according to the probe - from 5 to 7.5 cm;
II degree (moderate) - the duration of amenorrhea is from 1 to 3 years. Vegetovascular disorders appear (in 50% of women). The uterus is enlarged in size, the uterine cavity along the probe is from 3.5 to 5.5 cm;
III degree (severe) - duration more than 3 years, severe clinical manifestations, practically refractory to therapy;
by time of occurrence : primary amenorrhea (menses never occurred); secondary amenorrhea (develops after a period of normal menstruation (as a result of abortion, inflammatory, tumor processes, etc.);
due to the occurrence : amenorrhea of ​​central origin (occurs due to changes in the cerebral cortex, hypothalamus, pituitary gland); amenorrhea of ​​peripheral origin (due to changes in the adrenal glands, thyroid gland, ovaries, uterus);
depending on the level of pathology : hypothalamic; pituitary; ovarian; uterine; adrenal; amenorrhea due to thyroid disease.

Amenorrhea may occur with long-term use of certain medicines (hormonal, chemotherapeutic, psychotropic, drugs). Phenothiazine derivatives, tricyclic antidepressants, and many centrally acting antihypertensive drugs such as reserpine and methyldopa cause amenorrhea through their action on hypothalamic function. Chemotherapy drugs such as cyclophosphamide can cause ovarian failure and therefore amenorrhea. Low estrogen oral contraceptives can lead to amenorrhea due to inadequate stimulation of the endometrium.

AMENORRHEA OF CENTRAL GENESIS INCLUDES:
amenorrhea due to dysfunction of the cerebral cortex ,
amenorrhea due to damage to subcortical structures (hypothalamic-pituitary amenorrhea); disorders of the hypothalamic-pituitary system can be:
(1) - functional: chronic psychogenic stress, diet violation, chronic infections(frequent sore throats) and especially neuroinfections, endocrine diseases, taking drugs that deplete dopamine reserves in the central nervous system (reserpine, opioids, monoamine oxidase inhibitors) and affect the secretion and metabolism of dopamine (haloperidol, metoclopramide);
(2) - organic (anatomical);
(3) - due to congenital pathology.

!!! In most cases of amenorrhea of ​​central origin, signs organic damage the hypothalamus and pituitary gland are absent, however, in such conditions in the anamnesis, it is possible to detect indications of a skull injury or the presence of infectious diseases occurring with high temperature. When determining the level of gonadotropins in the blood and excreting them in the urine, a decrease in their secretion is often detected. However, in some cases, the concentration of gonadotropins in the blood may be normal.

REGARDLESS OF THE CAUSES OF IMPAIRMENT OF THE HYPOTHALAMO-PITUITARY REGION there is a violation of the production of hypothalamic gonadotropin-releasing hormone, which leads to a change in the secretion of FSH, LH, ACTH, growth hormone, TSH and prolactin. In this case, the cyclicity of their secretion may be disturbed. In violation of the hormone-forming function of the pituitary gland, various syndromes occur. A decrease in the secretion of FSH and LH leads to a violation of the development of follicles and, consequently, insufficient production of estrogens by the ovaries. Secondary hypoestrogenism, as a rule, is accompanied by hyperandrogenism, which in turn contributes to the emergence of a viril syndrome, moderately pronounced in hypothalamic-pituitary disorders.

(1) Amenorrhea of ​​central origin is more often functional (functional hypothalamic amenorrhea) and, as a rule, occurs as a result of exposure to adverse environmental factors and a decrease in the quality of life. Most often, the emergence of central forms of amenorrhea is preceded by mental trauma, neuroinfection, intoxication, stress, complicated pregnancy and childbirth.

Psychological stress and infectious diseases transferred in childhood are important. Physical overload associated with significant emotional and volitional stress can cause amenorrhea with mental, asthenoneurotic, asthenodepressive or asthenohypochondriac disorders. Menstruation stops suddenly. Along with amenorrhea, irritability, tearfulness, headache, memory impairment, working capacity, and sleep disturbance are observed. During the war, as a result of forced starvation, women dramatically lost weight, which led to a violation in the hypothalamic-pituitary region and to the so-called wartime amenorrhea. Psycho-emotional stress also contributed to this.

Amenorrhea is observed in every third patient with schizophrenia and manic-depressive psychosis especially during an exacerbation.

MECHANISMS OF MENSTRUAL FUNCTION DISTURBANCE are realized through the neurosecretory structures of the brain that regulate the tonic and cyclic secretion of gonadotropins. Under the influence of stress, there is an excessive release of endogenous opioids that reduce the formation of dopamine, as well as a decrease in the formation and release of GnRH, which can lead to amenorrhea. With minor violations, the number of anovulatory cycles increases, and luteal phase insufficiency appears.

Functional disorders of the hypothalamic-pituitary system lead to the development of Itsenko-Cushing's disease, gigantism, functional hyperprolactinemia, anorexia nervosa. Conditions such as pseudo-pregnancy and anorexia nervosa represent an extreme degree of negative impact of mental status on hypothalamic-pituitary function.

Amenorrhea in anorexia nervosa leads to a sharp decrease in the secretion of gonadotropins. This is often observed with an insistent desire to lose weight and a rapid decrease in body weight by 15% or more. This pathology is common among teenage girls who exhaust themselves with diet and physical activity, and can be the beginning of a mental illness. The absence of menstruation is one of the very first signs of the onset of the disease, which leads girls to a gynecologist. On examination, there is a sharp decrease in subcutaneous adipose tissue in the female body type. Secondary sexual characteristics are developed normally. Gynecological examination reveals moderate hypoplasia of the external and internal genital organs. Continued weight loss can lead to bradycardia, hypotension, and hypothermia. In the future, irritability, aggressiveness, cachexia with a complete loss of appetite and aversion to food appear. The hypoestrogenic state, along with a lack of nutrition, predisposes patients to osteoporosis.

The most common form of functional hypothalamic amenorrhea is psychogenic amenorrhea . With prolonged emotional stress, the development of disadaptation and subclinical depression with concomitant disorders in the neuroendocrine system is possible. There is an activation of the hypothalamic-pituitary-adrenal system (HPAS), which manifests itself in smoothing the daily rhythm of adrenocorticotropic hormone (ACTH) secretion. HHA has various, mainly inhibitory, effects on the reproductive system. The diagnosis of psychogenic amenorrhea, as a rule, presents great difficulties. It is often impossible to separate the impact of such damaging factors as stress, weight loss, increased physical activity.

Somewhat less common is another form of functional hypothalamic amenorrhea - food amenorrhea . Recently, cases of reproductive dysfunction associated with the observance of various diets and the desire to lose weight ("hungry amenorrhea") have become more frequent. At the same time, the amount of food consumed is an important factor affecting the state of the reproductive system. Loss of more than 15% of ideal body weight is inevitably associated with the development of amenorrhea. Hypothalamic amenorrhea, which occurs with weight loss, is a natural form of reversible contraception. !!! Recent studies show that nutritional hypothalamic amenorrhea can occur not only in case of weight loss, but also with an unbalanced diet. Thus, when examining women with idiopathic functional hypothalamic amenorrhea and normal body weight, significant shifts in their diet were revealed in the direction of increasing fiber intake and reducing the fat content in food. The pathophysiological mechanism for the formation of functional hypothalamic amenorrhea in malnutrition is unknown. Often, the restoration of weight and / or body composition in patients with functional hypothalamic amenorrhea of ​​nutritional origin is accompanied by the restoration of the menstrual cycle. In some patients, after the normalization of fat reserves, a stable long-term idiopathic amenorrhea persists, which is not amenable to treatment.

Functional hypothalamic amenorrhea is much less common in the form of load amenorrhea . In recent years, the benefits of various kinds of physical activity for health and life extension have been promoted. The positive effects of these activities are undeniable, especially in relation to the delay in the age-related increase in lipid and insulin levels in the blood serum, prevention cardiovascular diseases and bone demineralization. It has been shown that, on the one hand, with moderate exercise in postmenopausal women, the total calcium content in the body and bone density increase. However, on the other hand, fast growth The popularity of physical culture and sports in recent decades has also revealed the adverse effects of excessive physical activity on reproductive function. Women who exercise intensively various types sports, very often develop functional hypomenstrual syndrome and osteoporosis. At the same time, the nature of physical activity and the age at which it began play a significant role. If classes begin before menarche, as is usually the case with gymnasts and ballerinas, then menarche is delayed by about 3 years, and then secondary amenorrhea often develops. The sport also has an impact on menstrual dysfunction. Amenorrhea is more common (50-60%) among female athletes involved in intensive running and among ballerinas than among women involved in swimming, regardless of the intensity of training (12%). The severity of these disorders depends on the duration and intensity of sports training. Determining the role of physical activity in the mechanism of reproductive dysfunction is very difficult. Pathogenetic mechanisms load amenorrhea is not entirely clear. Apparently, a significant role in this process is played by a negative energy balance, which leads to a decrease in the level of leptin in the blood and, as a result, to a decrease in the activity of the GnRH oscillator. Changes in steroid metabolism in this pathology are also noted, especially an increase in the intracerebral content of catechol estrogens, leading to a change in the catecholaminergic tone in the brain.

Wide use contraceptive drugs recently led to an increase in the number of patients suffering from functional hypothalamic amenorrhea. Statistical studies show that about 5% of women develop functional hypothalamic amenorrhea after stopping oral contraceptives. The causes of hypothalamic amenorrhea after taking contraceptives are unknown. It is possible that the stimulating effect of estrogens on the transcription of serotonin receptor mRNA in the brain plays a certain role in this. Serotonin is known to have an inhibitory effect on the secretion of gonadotropin-releasing hormone by the hypothalamus.

!!! THE DIAGNOSIS OF "FUNCTIONAL HYPOTHALAMIC AMENORRHEA" CAN BE MADE ONLY AFTER COMPLETE EXAMINATION OF THE PATIENT . The main approach to diagnosis is the method of exclusion. Based on anamnestic data and clinical examination, you should make sure that the following factors are absent:
medications (psychotropic drugs, antihypertensives, hormonal drugs, sleeping pills);
severe somatic or mental pathology;
aplasia of the endometrium or uterine synechia (Asherman's syndrome);
organic neoplasms and pathological changes hypothalamic-pituitary region;
endocrine-metabolic disorders, additionally confirmed by the results of hormonal studies.

!!! Confirmation of the diagnosis of "functional hypothalamic amenorrhea", according to hormonal screening, is a normal level of prolactin, FSH, TSH, androgens and a normal or reduced level of LH in the patient's blood.

(2) Anatomical disorders of the hypothalamic-pituitary structures leading to the development of amenorrhea (for example, due to the development of hyperprolactinemia or Shien's syndrome), are as follows:
hormone-active pituitary tumors: prolactinoma, mixed prolactin- and ACTH-secreting pituitary adenomas;
damage to the hypothalamus and pituitary stalk as a result of: tumors (for example, craniopharyngioma), trauma (trauma to the base of the skull, hemorrhage), basal meningitis, granuloma, reticulosis, surgery, radiation exposure, infectious-allergic and neuro-reflex lesions of the hypothalamic region in chronic tonsillitis;
necrosis of the pituitary gland due to thrombosis of the pituitary gland vessels or massive postpartum or post-abortion bleeding.

Elevated levels of prolactin in the blood are observed in 15-20% of women suffering from amenorrhea, and may be accompanied by pituitary tumors of any size. However, cases are described when the tumor could not be detected. According to various authors, pituitary tumors occur in 25-75% of patients with hyperprolactinemia and amenorrhea. Other diseases that cause amenorrhea through dysfunction of the hypothalamic-pituitary system or direct damage to the pituitary gland include postpartum pituitary necrosis, craniopharyngioma, and empty sella syndrome.

See the article about the "empty Turkish saddle" syndrome. EMPTY TURKISH SADDLE SYNDROME in the section "neurology and neurosurgery" of the medical portal site.

Amenorrhea can be caused by a number of other disorders of the hypothalamus. For example, systemic diseases such as sarcoidosis can affect the function of the hypothalamus and lead to amenorrhea.

(3) Congenital pathology of the hypothalamic-pituitary system can lead to adipose-genital dystrophy. Any congenital (genetic) abnormalities and disorders in the chromosomes can cause amenorrhea. You should be aware of the syndrome of isolated hypogonadotropic hypogonadism or Kalman syndrome, which is characterized by a decrease in the levels of both FSH and LH. This disease is accompanied by anosmia, the inability to smell. In isolated hypogonadism syndrome, the hypothalamus does not produce GnRH (gonadotropin-releasing hormone), although its other functions are normal. This pathology can be hereditary or sporadic and is caused by a violation in the embryonic period of the migration of GnRH-producing neurons from the olfactory zone to their localization in the hypothalamus.

Since the pituitary gland is also responsible for metabolic processes, with damage to the hypothalamic-pituitary region, patients have a characteristic appearance. They have obesity, a moon-shaped face, a fatty apron, striae on the abdomen and thighs, but there may also be excessive thinness with mild secondary sexual characteristics. Obesity and severe weight loss as a result of disorders of the hypothalamic-pituitary region exacerbate the manifestations of hormonal dysfunction.

Amenorrhea is a disorder of menstrual function in which women of childbearing age (years) do not have menstruation for six months or more. Amenorrhea, as a rule, is not an independent disease, but is a symptom of genetic, biochemical, physiological, psycho-emotional disorders in the body.

WHO classification Group I - hypothalamic-pituitary insufficiency ( low level gonadotropins and estrogens, the level of prolactin is not elevated, the tumor in the hypothalamic-pituitary region is not detected) Group II - hypothalamic-pituitary dysfunction (normal levels of gonadotropins, prolactin and estrogens). LH:FSH ˃ 2.5 III group- ovarian insufficiency (high level of gonadotropins, estrogens are not determined) Group IV - congenital or acquired disorders of the genital tract (negative estrogen test) Group V - hypserprolactinemia in the presence of a tumor in the hypothalamic-pituitary region Group VI - hypserprolactinemia without damage to the hypothalamic-pituitary region Group VII - amenorrhea with a tumor of the hypothalamic-pituitary region (low levels of gonadotropins, estrogens, in the hypothalamic-pituitary region)

Hypogonadotropic amenorrhea Hypogonadotropic amenorrhea develops secondary and refers to one of the most severe forms of amenorrhea and is observed in the following conditions: - acute and chronic psycho-emotional stress, as well as excessive physical exertion, when there is an increased release of β-endorphins and neurotransmitters of the endogenous peptide class, leading to a decrease in the formation of dopamine and a decrease in the secretion of gonadoliberins and gonadotropins; - Cancellation of hormonal oral contraceptives; - treatment with phenothiazine derivatives, reserpine and ganglioblockers, which disrupt the balance of dopamine and norepinephrine in the hypothalamus; - anorexia nervosa, leading to cachexia and inhibition of the functions of the hypothalamus;

Kallman's syndrome, when irreversible defects in the synthesis of gonadotropins develop; - Sheehan's syndrome (postpartum hypopituitarism), which is characterized by ischemia and necrosis of the pituitary gland, developed as a result of large blood loss or septic disease and accompanied by insufficiency of gonadotropic function; - acidophilic adenoma of the adenohypophysis, suppressing the gonadotropic function of the pituitary gland by excessive secretion of growth hormone; - basophilic adenoma of the adenohypophysis, which produces an excessive amount of ACTH and oxycorticosteroids, while the secretion of gonadotropins is at a low level; - chromophobic adenoma of the pituitary gland, destroying the walls of the Turkish saddle and squeezing the surrounding tissues; - Hypoor hypserfunction of the thyroid gland or adrenal glands.

"Psychogenic" amenorrhea "Psychogenic" amenorrhea is also characterized by general weakness, fatigue, headache, sweating, memory impairment, tearfulness, irritability or apathy, weight loss; Anorexia nervosa Anorexia nervosa is accompanied by lack of appetite, cachexia, hypothermia, arterial hypotension, bradycardia, acrocyanosis, dry skin, general weakness, atony of the stomach, constipation, while the mentality of patients is not disturbed, physical activity is preserved; Chiari-Frommel syndrome Chiari-Frommel syndrome, which occurs during treatment with phenothiazine derivatives, reserpine and ganglion blockers, is characterized by the addition of galactorrhea, can be combined with obesity, mental disorders;

Syndromes of Kallman, Sheehen, acidophilic, basophilic, chromophobic adenomas of the adenohypophysis Kallmann's syndrome (Kallmann) is a congenital form of hypogonadotropic amenorrhea, the cause of which is a violation of impulse secretion of gonadoliberin in the hypothalamus. Sheehen's syndrome is manifested by agalactia, loss of pubic and axillary hair, weakness, dizziness, fainting, arterial hypotension, myxedema, weight loss, dryness, pallor and loss of turgor skin, memory impairment, apathy, hypothermia, bradycardia, weakness, anorexia, nausea, vomiting, constipation, decreased libido, anemia, brittle nails. Acidophilic adenoma of the adenohypophysis leads to gigantism or acromegaly (an increase in height, a disproportionate increase in the bones of the face and limbs), rapid fatigue, masculinization phenomena, headaches, decreased mental abilities, visual impairment, coarsening of the voice, thickening and roughening of the skin, the formation of its folds on the skull. Basophilic adenoma of the adenohypophysis causes the development of Itsenko-Cushing's disease, manifested by obesity, headache, irritability, decreased libido, impaired memory and sleep, thirst, polyuria, often hirsutism, pallor and dry skin, moon-shaped face, red acne, arterial hypsertonia, skin hyperpigmentation. Chromophobic pituitary adenoma is accompanied by obesity, visual impairment, etc. Hypothyroidism is characterized by galactorrhea and hypsercholesterolemia.

Normogonadotropic amenorrhea Normogonadotropic amenorrhea can be both primary and secondary, is a symptom of congenital and acquired anomalies of the uterus and genital tract, as well as diseases accompanied by hypseandrogenism, while the ovaries secrete a normal amount of estrogens and progesterone, and feedback from the pituitary gland provides normal the level of gonadotropins. Congenital anomalies of the uterus and genital tract lead to it: - congenital malformation in the form of aplasia of the uterus and vagina (Rokitansky-Kuster-Hauser syndrome, Rokitansky-Kuster-Houser) occurs as a result of regression of the Müllerian ducts; - congenital malformation in the form of fusion (atresia) of the hymen and the transverse septum of the vagina, due to the pathological course of the antenatal period and characterized by the absence of natural outflow during menstrual blood loss.

And acquired: - Asherman's syndrome (Asherman), arising from complete or partial obliteration of the uterus with intrauterine synechia, caused by trauma to the endo- and myometrium due to excessive curettage of the uterus during abortion or after childbirth, complicated by endometritis, as well as cryodestruction or the introduction of a solution into the uterine cavity iodine; - tuberculous endometritis, causing pronounced cicatricial changes in the endometrium with the formation of synechiae and obl iteration of the uterine cavity; - tumors of the adrenal glands (adenoma or carcinoma), synthesizing a large number of androgens with or without excess cortisol; - congenital adrenal hypserplasia - adrenogenital syndrome (AGS) develops as a result of insufficiency of 21-hydroxylase and 11-hydroxylase in the synthesis of cortisol, as a result of which ACTH production increases, hypserandrogenism develops; - sclerocystic ovaries (Stein-Leventhal syndrome, Stein-Leventhal), caused by congenital or acquired insufficiency of 19-hydroxylase and 3-aldehyderogenase enzyme systems, which ensure the conversion of androgens into estrogens during the synthesis of the latter. The ovary secretes an excessive amount of androgenic steroids (androstenedione and other androgens), which prevent the maturation of follicles and promote anovulation, the cyclic release of gonadotropins by the pituitary gland is disrupted, and constant stimulation of ovarian gonadotropins leads to cystic atresia of the follicles, hypserplasia of the theca cells and ovarian stroma (sites of androgen secretion), dystrophic processes in granuloma cells, thickening of the protein membrane.

Clinic Rokitansky-Küster-Hauser syndrome is characterized by amenorrhea in summer against the background of normal somatic development and pronounced secondary sexual characteristics, the absence or shortening (up to 2 cm) of the vagina, the absence or presence of a uterus in the form of a flat cord with normal ovarian sizes. Infection of the hymen or the transverse septum of the vagina is detected with the onset of puberty, when, against the background of normal physical and sexual development, pain in the lower abdomen, frequent urination, difficulty urinating and defecation due to the lack of a natural outflow of menstrual blood with the formation of hematocolpos, hematometers and hematosalpinx. Asherman's syndrome and tuberculous lesions of the uterus are manifested by amenorrhea and infertility in the absence of obvious pathological changes. Tumors of the adrenal glands are manifested by a rapidly developing and pronounced virial syndrome (hirsutism, obesity, arterial hypsertonia, osteoporosis (OP), stretch marks on the skin, lowering of the voice, reduction of the mammary glands, etc.). Congenital adrenal hypserplasia is usually manifested by pseudohermaphroditism, accelerated physical and sexual development according to the male type, accelerated bone age, virilization of the genital organs, and hypserpigmentation of the skin. Stein-Leventhal syndrome is clinically characterized by amenorrhea, infertility, hirsutism, ovarian enlargement, oily skin, acne, and sometimes obesity.

Hypergonadotropic amenorrhea Occurs primarily, includes gonadal, chromosomal and genetic defects that disrupt the hormonal feedback mechanism, are necessary to suppress the secretion of gonadotropins. 1. Chromosomal abnormalities: - gonadal dysgenesis (Shereshevsky-Turner syndrome) is caused by chromosomal abnormalities. The karyotype is 45XO or mosaicism. 2. Diseases not accompanied by chromosomal abnormalities: - Resistant ovary syndrome is explained by a defect in membrane receptors for gonadotropins. Karyotype - 46XX; - testicular feminization syndrome develops due to a defect in cytosolic testosterone receptors and, accordingly, resistance of target tissues to androgens. Karyotype - 46XY.

Clinic Shereshevsky-Turner syndrome: anomalies in the development of the ovaries (small strands), hypoplasia of the genital organs, absence of mammary glands, short stature, arched palate, short neck, presence of pterygoid folds on the neck, low location auricles, barrel-shaped chest, defects in the development of the cardiovascular, bone, urinary and other systems. Resistant ovary syndrome is manifested by the cessation of menstruation and infertility before the age of 35 years. Testicular feminization syndrome (Morris syndrome, Morris) is detected during puberty; the child looks like a girl, with pronounced mammary glands, but there is no hair on the pubis and in the armpits. The external genital organs are female, the labia are underdeveloped, the vagina is underdeveloped and ends in the form of a blind sac, the testicles can be located in the inguinal canal or large labia. Instead of the uterus and fallopian tubes, there are hypoplastic male ducts.

Examination of patients with amenorrhea A. History taking I. Complaints. II. Anamnesis (general, gynecological, genealogical). III. General examination with an emphasis on the following typobiological features: height, body weight, the nature of the deposition of adipose tissue in cases of obesity, body type, the presence or absence of somatic anomalies and stigmas, the condition of the mammary glands, the condition of the skin and its appendages.

Treatment group I - hypothalamic-pituitary insufficiency (low levels of gonadotropins and estrogens, prolactin levels are not elevated, a tumor in the hypothalamic-pituitary region is not detected) To regulate the menstrual cycle: cyclic hormone therapy - Cyclo-Proginova, Femoston 2/10, Klimonorm When planning pregnancy, stimulation of ovulation with gonadotropins is indicated

Treatment group II - hypothalamic-pituitary dysfunction (normal levels of gonadotropins, prolactin and estrogen). LH:FSH ˃ 2.5 To regulate the menstrual cycle gestagens: duphaston, norkolut, utrozhestan F, puregon, menogon, humegon)

Treatment group III - ovarian failure (high levels of gonadotropins, estrogens are not detected). To regulate the menstrual cycle - cyclic hormone therapy: Femoston 2/10, Cyclo-Proginova, Klimonorm When planning pregnancy: - egg donation with subsequent transfer to the uterine cavity