MATTE — LEVENTALYA SINDROM (J. F. Stein, an amer. gynecologist, sort. in 1887; M of L. Leventhal, amer. gynecologist, sort. in 1901; synonym: a syndrome of sclerocystic ovaries, a disease of sclerocystic ovaries, a disease of polycystic ovaries, a polycystosis of ovaries, polycystic ovaries) — the syndrome which is characterized by bilateral increase and sklerokistoznsh changes of ovaries and shown various disturbances of a menstrual cycle against the background of anovulation, infertility, excess pilosis, obesity.
In 1915 Ya. K. Hachkuruzov, and in 1928 S. K. Forest observed a similar disease and offered its operational * treatment. In 1935. Matte and Levental in details described a wedge, and patomorfo l. a picture of the syndrome which is characterized by an ovarian giiyerandrogeniya.
In a crust, time under the name of a syndrome of Matte — Levental is combined by the states, various on a pathogeny, which are shown hyper-androgeniyey and sclerocystic or polycystic changes of ovaries.
Etiology and pathogeny. The etiology is not known. The role of genetic factors, and also pathogenic (hormonal or infectious) impacts in the embryonal period on a hypothalamus or ovaries of a fruit is supposed. Mental injuries, stressful situations, change of a klimatogeografichesky zone, especially in the pubertal period, can promote development Sh — JI. page.
According to pathogenetic features allocate three main forms of a disease: typical (described by Matte and Leventa-l), mixed and central. The typiform of Sh. — Hp is characterized by a giperandrogeniya of ovarian genesis. Primary genetically caused enzymatic defect in ovaries at the level of 19-hydroxylase and (or) 3 is the cornerstone of its development (Z-ol-degidro-geiazy therefore biosynthesis of steroids in ovaries is broken and the level of androgens in an organism increases. At the mixed form the giperandrogeniya of an adrenal and ovarian origin is observed that is caused by primary dysfunction of adrenal glands (see) with secondary involvement in patol. process of ovaries (see), formation in them sclerocystic changes and hypersecretion them androgens (see). Development of the central (gipotalamo-pituitary) form Sh. — JI. the page is connected with primary disorder of function z and on tala of an amo-ga pof izar but y systems (see) owing to its inborn defect, acute or persistent infection, intoxication in the prepubertatny or pubertal period and disturbance of secretion of gonadotropic hormones (see) ii of monoamines, especially dopamine (see Catecholamines) and serotonin (see).
In formation of III. — Hp of hypo-tala the amo-pituitary system plays a role of a releaser (at the central form) and (or) supporting (at other forms of a syndrome). For III. — Hp increase in level of secretion of luteinizing hormone (see), and a ratio of content of luteinizing hormone and follicle-stimulating hormone is characteristic (see), normal equal 0,5 and providing growth and maturing of follicles, at Sh. — Hp increases to 1 and more. At similar disturbance of secretion of gonadotropic hormones there is an inadequate stimulation of function of ovaries, disturbance of development in them of follicles, decrease in quantity of the ripening follicles, disturbance of synthesis of steroid hormones (see) that leads to anovulation (see. And novulyatorny cycle). In ovaries there is a hyperplasia of marrow, teka-to summer to and hidusny cells, in to-rykh androgens are much produced. In an organism the quantity of androgens (testosterone, androstendion) increases, the level of the estrogen (see) synthesized from predecessors of androgens increases. Between secretion of androgens, estrogen, about l of actin, dopamine and serotonin there is a difficult interrelation. Dopamine influences cyclic secretion of the luteinizing hormone (LH) and follicle-stimulating hormone (FSG) of a hypophysis, affecting directly lyuli-Berinum (see. Hypothalamic neurohormones) of I, also suppresses release of prolactin (see). Serotonin has preferential oppressing effect on release of gonadotropic hormones of a hypophysis that leads to blockade of an ovulation (see), and also stimulates products of prolactin. Thus, deficit of monoamines (dopamine, serotonin) or disturbance of their metabolism in structures of c. N of page play an essential role in Sh.'s pathogeny — Hp.
Pathological anatomy. Ovaries are increased, grayish color, smooth, dense; on a section cystous cavities (atretic follicles) are visible. The considerable thickening of a white, dystrophic and atrophic changes of follicles, reduction of number primordialny are microscopically noted and increase in quantity of cystous and atretic follicles, lack of mature follicles and yellow bodies, come to light follicles of various degree of a maturity. Sclerous changes in cortical, marrow and vessels of ovaries, a hyperplasia of cells of an internal cover of follicles are expressed. In each of ovaries typical for Sh. — Hp of change can be heterogeneous. At Sh. — Hp the sizes of a uterus is slightly less than norm, in an endometria various changes — from an atrophy before hyperplastic processes are noted.
Clinical picture. The syndrome quite often proceeds in the beginning is hidden and is clinically shown at the age of 16 — 30 years, is frequent only under certain conditions (persistent infections, operative measures, the beginning of sex life, etc.). Sometimes symptoms appear only after pregnancy and childbirth.
The main symptoms are: infertility (see); the disturbances of a menstrual cycle (see) which are shown either lengthening of duration of periods (polymenorrhea) or its shortening (oligomenorrhea) or lack of periods (see the Amenorrhea), are more rare bleedings during periods (menometrorrhagia), a hirsutism (see), obesity (see). Are observed also (more often at the central form of a disease) vegeto-vascular, trophic and exchange frustration, sometimes — frustration of mentality in the form of a depression (see. Depressive syndromes), aggression.
For a typiform of III. — Hp timely approach of the first periods, an irregularity of periods, about l a yoke silt and an amenorrhea is more characteristic, is more rare — acyclic bleedings (see. Uterine bleedings), primary infertility. The hirsutism and obesity are moderately expressed. External genitals are developed correctly, the uterus of the usual sizes, ovaries are increased. By tests of functional diagnosis signs of anovulation — the single-phase basal temperature, a positive symptom of «pupil», etc. come to light (see. Anovulatory cycle). L G maintenance in blood is increased in 1V2 — 5 of time, FSG — in limits, norms or below it, the ratio of LG and FSG is equal 1V2 — 5. Secretion of prolactin, dopamine and serotonin does not differ from norm. Excretion of 17 ketosteroids (see) and estrogen — within norm or exceeds it a little. Level of androsterone, testosterone (see), etiocholanolone is higher than norm. Test with stimulation of ovaries a chorionic gonadotrophin against the background of braking of function of adrenal glands dexamethasone causes increase in level of androgens in an organism.
The mixed form Sh. — Hp is characterized by later (in
16 — 18 years) approach of the first periods, disturbances of a menstrual cycle on type of a secondary amenorrhea (is more rare — oligomenorrheas), primary infertility. The hirsutism is considerably expressed; the constitution approaches intersexual; the hypodermic fatty layer is developed moderately and evenly. Mammary glands and external genitals are often a little underdeveloped, the clitoris is slightly increased. The uterus, as a rule, is less than norm, ovaries are increased. By tests of functional diagnosis (see Ovaries) reveal anovulation and a low oestrogenic saturation (see the Vagina). At a half of patients change of tolerance to glucose is noted, 1/3 patients have an increase in the sizes of the Turkish saddle, osteoporosis of bones of a skull, change of EEG. Giperandrogeniya is more expressed — increase in excretion 17-ketosteroi-do.v, androsterone, etiocholanolone, a degidroepiandrosteron is noted. Excretion of estrogen with urine is lowered. Increase in maintenance of LG in a blood plasma is less expressed, the maintenance of FSG is reduced, L G ratio and FSG about 3,2; content of prolactin within norm, the content in blood of dopamine and serotonin is increased.
For the central form LLI. — Hp are characteristic later (in 16 — 20 years) approach of the first periods, disturbance of a menstrual cycle but to type of an oligomenorrhea or an amenorrhea, is more rare — acyclic bleedings, infertility, and also vegeto-vascular, exchange and trophic frustration. At patients the excessive adiposity on a breast, a stomach, hips is noted. The hypoplasia of external genitals, moderate increase in ovaries, reduction of the sizes of a uterus is observed. On EEG find the changes testimonial of involvement in patol. process of diencephalic area. L G maintenance fluctuates in wide limits (from small increase to considerable), the maintenance of FSG within norm, a ratio of these hormones several times exceeds normal indicators. Level of prolactin in limits or is higher than norm, the content of dopamine in an organism is reduced, serotonin — exceeds norm. Giperandrogeniya is less expressed. Excretion of 17-keto-steroids and a degidroepiandrosterona within norm or is a little raised, excretion of estrogen is lowered.
Current and weight patol. process are defined by expressiveness of fermental disturbances in ovaries, extent of dystrophic changes in their follicular device, disturbances in the central corrective systems, compensatory opportunities of an organism.
Development of hyperplastic processes in an endometria, a thicket at the central form of a disease is characteristic of Sh. — Hp (adenomatous polyps of an endometria and endometrial cancer register in 14 — 30% of cases). In cases of approach of pregnancy the last often is complicated by not incubation (see Nevynashivany pregnancies), late toxicosis (see Toxicoses of pregnant women), pre-natal death of a fruit (see. Pre-natal death), weakness of patrimonial activity (see Childbirth) and bleeding in labor.
The diagnosis is based on data of the anamnesis, a wedge, a picture, data of survey, biochemical blood analyses and urine and a tool research.
During the collecting the anamnesis it is necessary to pay attention to disturbances of reproductive function at relatives, advanced age of parents, to the diseases transferred mother of the patient during pregnancy, such as epidemic parotitis, measles, rubella, and also aggravations hron. diseases, napr, rheumatism, tuberculosis, etc.; reception of pharmaceuticals, in particular synthetic estrogen-gestagennykh of drugs, androgens, defective or a hyponutrient, impact of ionizing radiation and other adverse factors during pregnancy.
At survey increase both, seldom one ovary is, as a rule, noted; in early stages of a disease ovaries can be the normal sizes or are even a little reduced — small sclerocystic ovaries. Extent of increase in ovaries is defined by a pelvigrafiya (see) and ultrasonic scanning (see. Ultrasonic diagnosis). Valuable diagnostic method is the laparoscopy (see Peritoneosko-Pius) with an obligatory biopsy (see) ovaries. At diagnosis of Sh. — Hp define amount of gonadotropic hormones in a blood plasma, L G ratio and FSG, content of testosterone and other androgens (d4-androstendion, dihydrotestosterone — DEA-sulfa-ta), and also prolactin in blood, the nature of fatty and carbohydrate metabolism, carry out rentgenol. a research of a skull and the Turkish saddle (see Kraniografiya), an electroencephalography (see). Definition in urine 17 of ketosteroids has smaller diagnostic value since they are not active androgens. For Sh.'s diagnosis — Hp and specifications of a source giperandro-geniuses use functional trials with dexamethasone (see. Dexamethasonal test), progestinakhm and Prednisolonum, AKTG and chorionic gonadotrophin.
Differential diagnosis of TIT. — JI. villages carry out with adreno-geni-taljnym a syndrome (see), Itsenko's disease — Cushing (see Itsenko — Cushing a disease), a hyper thecosis (see Lipidokletochny tumors), androgenoprodutsiruyushchy tumors of ovaries (see), functional or organic lesions of hypo-talamo-pituitary system (see), with a genital tuberculosis (see Tuberculosis extra pulmonary). Rapid growth at children's age, primary amenorrhea, men's type of a constitution, the hirsutism much developed, the expressed hypertrophy of a clitoris, increase in level 17 of ketosteroids, positive test with dexamethasone is characteristic of an adrenogenital syndrome. At an adrenogenital syndrome of tumoral genesis consider bystry progressing of virilescence, existence of arterial hypertension, exchange disturbances, and also these oksisuprarenografiya or a computer tomography (see the Tomography computer).
At Itsenko's disease — Cushing increase in the AKTG level, cortisol in blood, oksikortikoste-roid in blood and urine is noted.
The hyper thecosis is characterized by more expressed hirsutism, a hyperplasia and a luteinization of a stroma of an ovary, accumulation in it of lipids, lack of cystous and atretic follicles and thickenings of a white; treatment by Clomifencitratum at giperteko ze unlike Sh. — Hp is inefficient.
At an androgenoprodutsiruyushchy tumor of an ovary damage, as a rule, of one ovary, a hypotrophy of mammary glands and a uterus is noted; consider also the data obtained at a laparoscopy.
At organic or functional lesions of gipotalamo-pituitary system disbolism (obesity), vegeto-vascular frustration, disturbance of tolerance to glucose, changes in area of the Turkish saddle, increase in secretion of a cortisone and its derivatives are noted.
The genital tuberculosis is confirmed by data of the anamnesis, gi-sterosalpingog raffias (see Metro-salpingografiya), laparoscopies.
Treatment in initial stages, and also at an easy current of Sh. — Hp conservative. Apply synthetic progestins, Clomifencitratum, gonadotrophins at the mixed form of a syndrome Clomifencitratum appoint in combination with Prednisolonum or dexamethasone. At the central form for the purpose of recovery of functions of hypo-tala of amo-pituitary system appoint also sedatives, synthetic estrogen-gestagennye drugs in small doses (discontinuous courses), carry out a dietotherapy. At the expressed hirsutism apply veroshpiron, anti-androgens.
At absence within 6 — 12 months of a positive take from conservative therapy operational treatment is shown (an exception patients with the central form Sh. — Hp make). The delay of an operative measure is inexpedient in connection with progressing of dystrophic processes in ovaries, and also danger of development of tumoral processes in an endometria. Patients it is better to operate aged up to 20 years; at the same time recovery of a menstrual cycle is noted in 90 — 95% of cases, approach of pregnancy — in 60 — 65% of cases. If operation is made during from 21 to 35 years, these figures are respectively equal to 70 and 40%. Before operation irrespective of a form III. — Hp should make a scraping of a mucous membrane of a uterus (see the Scraping) with the subsequent gistol. a research of the received material for an exception of a precancerous state or endometrial cancer.
The expanded wedge-shaped resection of both ovaries is most widespread (see the Ovary, operation), sometimes make their partial demedullyation (removal of a medulla of an ovary). Delete 2/3 more often — 3/4 volumes of tissue of ovaries, apply a sub-total resection with the maximum removal of a giperplazirovanny stroma and a shchazheniye of cortical substance recently. Assume that the effect of operation is caused by reduction of volume of steroidoprodutsiruyushchy tissue of ovaries therefore there is a decrease in level of androgens in an organism, and also reflex impact of the resected ovaries on function of a hypothalamus and gonadotropic function of a hypophysis. Results of a wedge-shaped resection of ovaries depend on a condition of the follicular device of ovaries: quantities of primordialny follicles, existence of the ripening follicles, extents of dystrophic changes in follicles, and also from completeness of removal of the changed fabric. After operation for patients carry out strict observation. In case of lack of effect of operation with the purpose vosstanovleniye of menstrual and reproductive function appoint replacement therapy (estrogen, progesterone), for the purpose of stimulation of an ovulation — gonadotrophins, Clomifencitratum, at insufficiency of a secretory phase of a cycle (in the second phase of a cycle) — synthetic progestins, at the mixed form Sh. — Hp — Prednisolonum or dexamethasone in combination with Clomifencitratum. At Sh.'s combination — Hp with other endocrine diseases carry out complex treatment. Patients with III. — Hp need systematic observation in connection with danger of development of endometrial cancer at them.
The forecast in respect of recovery of menstrual and reproductive function at timely begun and correctly carried out treatment is favorable.
Prevention consists in an exception of harmful effects during pregnancy, in the prevention of stressful situations and diseases at children's age p the pubertal period. Early treatment of a giperandrogeniya of ovarian and adrenal genesis, and also correction of disturbances of functions of gipotalamo-pituitary system is necessary.
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