Syndrome of mono- and anorchism

The defect of embryo development arises up after the 20th week of development. Born anorchia is investigation of violation of blood circulationof testicles with actual death of testicles of embryos. In a period after the 20th week of embryo development an urethra is already formed on a masculine type, but the normal forming of phallus and scrotum does not take place. For these patients a scrotum is underdeveloped, testicles are obsolete.

Phenotype and genotype is masculine. In a pubertal period the structure of skeleton is formed in default of the second sexual signs. Table of contents of testosterone in blood very low. In a pubertal period a level ofgonadotropins is in blood та excretion them with urine promoted.

After the offensive of pubertal period the substitute therapy by androgens is recommended.

Cryptorchism

A testicle goes down in a scrotum on 7-th months of pregnancy.Gonadotropine of mother and placentas, which influence on testiculary tissue, induce formation of androgens and necessary both for the process of lowering of testicle and for functional activity of cages of Leidig andspermatogonies in new-born. If a testicle does not go down in a scrotum during the first year of life, this process stays too long to the period of the sexual ripening or a testicle does not go down in general.

At a review absence appears more frequent one testicle and rarer at bilateral cryptorchism – two testicles. At bilateral cryptorchism it is necessary to conduct differential diagnostics from anorchism by test withchorionic gonadotropine and by subsequent determination of level oftestosterone in the whey of blood. A testicle which did not go down can be disposed in an inguinal channel or in an abdominal region.

Cryptorchism is reason of inferior development of testicle, that is why it is necessary as possible before to begin therapy by chorionic gonadotropine (for adults for 1500 МО twice for a week during 6 weeks) which improves circulation of blood in a testicle and lowering of testicle. Therapy of by chorionic gonadotropine is conducted in any age, beginning from multimonthly age, and if she appears uneffective, an operation is recommended (surgical report of testicles).

VIAGRA FOR WOMEN

A drug that failed to fight the blues could be the female answer to the little blue pill Viagra, the lead North American investigator analyzing tests of the drug said Tuesday.

Women who took the drug flibanserin when it was being tested as an anti-depressant said it didn’t help them beat the glums, but did give them "an increase in libido that they liked," said John Thorp, one of the investigators analyzing data from three clinical trials of the drug.

Lack of desire is the most common sexual problem in women aged 30 to 60, just as erectile dysfunction, for which Viagra is one of a choice of treatments, is the most common sexual disorder among men in the same age bracket, Thorp said.

"Men remain interested but can’t act or perform properly and women lose interest," Thorp said.

"So where Viagra and other erectile dysfunction medications work in the blood supply, flibanserin works in the brain," he said.

In the light of the women’s reactions to flibanserin, the German drug company that had first tested the drug as a treatment for depression, Boehringer Ingelheim, several years ago began exploring the possibilities of it being the active ingredient in the female answer to Viagra.

Clinical trials were held in Canada, Europe and the United States to test the drug’s efficacy in raising the level of sexual desire in women.

Nearly 2,000 pre-menopausal women were given flibanserin or a placebo for 24 weeks and asked to report back to researchers or make diary entries on six variables, including the number of satisfactory sexual encounters they had and their level of sexual desire.

The studies found that 100 milligrams a day of flibanserin resulted in "significant improvements" in the two variables.

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PATHOLOGICAL CLIMAX BY WOMEN

A climacteric syndrome is observed in 65-70% women. A premature (early) climax develops earlier than 45 years. A late climax appears after 55 years old.

Select a few variants of climacteric syndrome after the features of clinical displays:

1) typical form;

2) аtypical form;

3) a combined form.

Medical tactic for the different forms of climacteric syndrome differs substantially.

1. Typical form of climacteric syndrome

Most frequent form of climacteric syndrome. More frequent observed for women which carry the protracted mental or physical overloads. Menopause comes in good time, but in postmenopause(in 2-6 months) there are typical climacteric symptoms, the expressed of which grows during 3-6 months, and then slowly diminishes for period 15-20 months.

A major diagnostic criterion is violation of menstrual function. At first the delays of monthly appear on 2-3 months (opsomenorrhea), quite often with the next uterine bleeding. Afterwards there is the proof stopping of menstruations (amenorrhea).

There are unsteady emotionally-psychical violations – nervousness, promoted fatigueability, tearfulness, feeling of fear. Characteristic general amotivational weakness, violation of rhythm of sleep.

Head pain also is a widespread enough complaint. Most characteristic moderately expressed, but practically permanent head pain, feeling of weight in a head. At a mental and physical overstrain, insomnia, overeating before sleep, intoxications brief pains appear in parietal and temporal areas.

Dizziness develops as a result of vegetative-vascular violations, atherosclerosis of vessels of cerebrum.

"Waves" of heat to the head and neck, pathological sweating is leading symptoms of climacteric syndrome. Type "waves" begin from the brief feeling of alarm, "pressure" in breasts and palpitation during a 30-60 sec. Then a face and neck blushes quickly, through 2-3 minutes hyperemia disappears, a face and neck is covered sweat. Frequency and expressed of "waves" is the basic (but not unique) criterion of weight of climacteric syndrome. At the easy form of climacteric syndrome the number of episodes of "waves" is small (to 10 on days), at middle- 10-20 on days, at heavy – more than 20 on days.

Pains are possible in bones and joints of different character and localization. The most characteristic pains are in a spine, large and shallow bones and joints. Оsteoporosis results in squeezing of vertebrae, diminishing of growth.

Obesity is a frequent sign of menopause, result of age-old change of hormonal background and exchange of matters

The symptoms of hypercorticism quite often are observed in an initial period of climax. Glucocorticoid and androgenic function of adrenal cortex rises, possibly, as an adaptation reaction on hypofunction of gonads. In menopause for patients with hypofunction of ovaries, which developed before time, the signs of hyperandrogenia- hirsutism can develop, pigment spot on the skin of face, hands, becoming rough of voice.

Violations of the functional state of щитоподібної gland are possible. There is insignificant гіпофункція of щитоподібної gland in 50% women, in 7% is гіперфункція.

Additional criteria of средньотяжкої form of climacteric syndrome: dizziness, head pain, worsening of memory and common state.

Additional criteria of heavy motion of climacteric syndrome: vegetative-vascular, endocrine, metabolic and trophic violations, disfunction of the репродуктивної system.

2. Аtypical form of climacteric syndrome

An аtypical form develops for women, which carried psychical or physical traumas, heavy diseases (infectious, somatic, gynaecological), operative interferences before, workings in the conditions of action harmful factors, protracted mental and physical overloads.

Characteristic violation of menstrual function (оpsо- or hypomenorrhea, and then proof amenorrhea). In 1-3 months typical climacteric symptoms (violation of rhythm of sleep, crabbiness, tearfulness, worsening of memory, decline of capacity promoted) which are combined with untypical appear after appearance of violation of menstrual cycle: by a general weakness, pains in the area of heart, palpitation, head pain, worsening of ear, sight, constipations or diarrhoea.

There is dryness of skin, fragility of nails, fall and fragility of hairs, appearance of pigmental spots on the skin of face, hands.

Almost all women have multiplying mass of body with the even or regional deposit of fat on a breast, stomach.

The symptoms of delay of liquid, edemata, pains, are possible in bones, joints, sometimes sickly urination.

The state progressively gets worse with the decline of capacity or its complete loss.

For patients with the atypical form of climacteric syndrome can develop the attacks of bronchial asthma, hypercholesterinaemia, hyper- and hypoglycaemia. Hormonal cardiopathy, osteoporosis, osteochondrosis is formed quite often.

Typical development of crises.

Sympato-adrenal crisis. The exchange of matters is reduced. Аsthenic constitution. Crises more frequent develop in the day-time or in the evening. Crisis begins from feeling of alarm, fear of death, chill. Promoted arterial hypertension, tachycardia. A skin is initial cold, pale, extremities are cold. With development of crisis a skin turns pale or blushes. Temperature of 37-39С°. Characteristic spasms of vessels of different localization, which squeeze pains in the area of heart. Head pains each evening, sleep is uneasy. Crabbiness is promoted. A capacity is higher in the evening. Pupils are extended. Dryness of mucous membrane of mouth, appetite reduced. Constipations. Liquid urination. The level of adrenalin in blood is promoted, hypopotassiumaemia. Duration of crisis 20-40 minutes, ends with intensive urination, sharp general weakness. Intervals between crises can have different duration.

Vagoinsular of crises. The exchange of matters is promoted. Constitution of picnic. Crises more frequent at night, in the morning. Skin is heat, rose. Extremities heat, hot. Temperature of 35,5-36С°, sweat is warm, liquid. Reduced arterial blood pressure, bradycardia. Characteristic prickly pains in the area of heart, head pains in the morning. Sharp weakness, episodes of fainting fit. Depression. Sleep is deep. Pupils are narrowed. Capacity higher in the day-time. Salivation. An appetite is promoted. Diarrhoea, nausea. Urination is frequent. The level of adrenalin in blood is reduced, hyperpotassiumaemia.

3. Combined form of climacteric syndrome.

The combined form develops for patients with chronic diseases of cardiac, hepato-billiary systems, by diabetes and other endocrine diseases, allergy.

Dysfunctional climacteric uterine bleeding – one of basic variants of pathological motion of menopause. Observed in 30% women which are in the period ofpre-menopause. The origin of dysfunctional of the uterine bleeding is caused the age-old increase of hypothalamic activity with the increase of gonadotrophic function of hypophysis. In a pre-climacteric period a sensitiveness is to hormonal stimulation in peripheral organs normal or promoted, and as a result the promoted hormonal stimulation there is persistence of follicles in ovaries, in an uterus and pectoral glands are hyperplastic processes.

Dyshormonal climacteric cardiopathy.

Climacteric cardiopathy (myocardial dystrophy) is the atypical form of climacteric syndrome which runs across with pains in the area of heart for as cardialgia and by the defeat of myocardium of non-coronarogenic character.

Typical cardialgia is pains in the area of heart of non-coronal character. Pains are localized in the area of apex or in a precardial area. An irradiation of pain can be absent, to be limited (in the left shoulder-blade) or widespread (the half of thorax engulfs all left). Variable intensity of the removed pain – from the easy aching to very great, unbearable pain. The typical bright emotional colouring of pain syndrome is feeling of the "killed nail", "stuck knife".

The signs of electrocardiographies are expressed moderately, unsteady, the expressed of rejections on ECG does not answer weight of pain syndrome.

Hormonal therapy results in reverse development of symptoms of disease - removal of cardialgia, normalization to the positive dynamics of ECG, normalization of indexes of lipid exchange, decline of secretion of lutropine and follitropine.

Diagnostics of climacteric syndrome by woman.
For diagnostics drawn on hormonal researches.

In development of climax for women select three stages.

I phase - the recurrence of excretion of estrogen is violated, there is relative hyperestrogenia, metabolism of estrogen is violated. The sensitiveness of tissues rises to estrogen, an allergy can develop to own estrogen. Excretion is promoted with urine of 17-КС and 17-ОКС.

II - excretion of estrogen with urine is reduced. Cytological reaction of vaginal stroke of 2-3 degrees. The phenomenon of crystallization of cervical mucus is poorly expressed. Mucus shells of body of uterus at histological research in the stage of atrophy.

III is the ahormonal stage. The secretion of gonadotropins is promoted. Correlation of lutropine / follitropine diminishes to 0,4-0,7 (in reproductive age even 1,0).

Treatment of climacteric syndrome.
Treatment must be complex, such, which sets rational mode of labour and rest, rational feed: hypocalorial ration with limitation of carbohydrates, enriched cellulose. Expedient psychotherapy, physiotherapy and medical physical education.

For the correction of vegetative-vascular violations apply tranquilizers, neuroplegics, antidepressants. The extracts of root of valerian normalize the function of hypothalamus. Effectively preparations which contain alkaloids remove vegetative dysfunction –belloidum, bellataminalum, bellasponum.

In more heavy cases use tranquilizers –elenium, sibazonum, andaxinum, trioxazinum. Treatments begin with small doses (1/2 pills 2-3 times per a day), gradually multiplying a dose to optimum. Course of therapy – 1-2 months.

Heavy symptoms of climacteric neurosis are a testimony for setting of neuroplegics–aminazinum, propazinum, etaperazinum. Apply small doses (1/2 pills 2-3 times per a day) during 2-4 weeks to development of proof effect, then a dose is gradually reduced. At presence of depression use antidepressants, for example, amitriptilinum or azaphenum.

If violations of menstrual function appear in age 40-45, it is expedient to conduct a hormonal correction gestagen preparations with the purpose of proceeding in the reproductive system. Proceeding in hormonal homeostasis allows to normalize and functioning of privy parts, and all organism on the whole. Proceeding in a menstrual cycle in pre-menopause, with appearance of proof violations of menstrual cycle, favourably influences on the state of women: a feel gets better, "waves" are halted, sleep gets better.

Treatments begin from the periodic setting of gestagens during lengthening terms between menstruations. Gestagen preparations assign for the 18-20th day of the induced menstrual cycle at the positive phenomenon of "pupil" (-++) and (+++) during 6-8 days. The courses of treatment by gestagen preparations conduct until menstrual reactions are not halted (6-18 months). Testimonies for the repeated setting of gestagen is a delay of menstruation, the positive "phenomenon of pupil", high kariopicnotic index, is prolonged.

Norkolut (noretisteronum) accept for 1/2-1 pills (5 mg in a pill) daily in 20 minutes after a meal.

Turinal appoint for 2 pills on a day.

Pregnine accept on a 1 pill 3 times per a day sub lingua, or 1 injection of прогестерону for 1 мл of a 1% solution intramuscular daily.

Oxyprogesterone capronatis is the synthetic prolonged analogue of progesterone, enter intramuscular for 1 ml of a 12,5% solution on the 16-18th day of the created cycle.

In the hyperestrogenic phase of climax treatment is recommended by the combined estrogen-gestagen preparations. Application of the combined preparations imitates a normal ovarial cycle: primary estrogenic stimulation of endometrium is with a subsequent progestin-estrogen action. Rigevidonum, non-ovlonum appoint for 1/4-1/6 pills from the 5th day of spontaneous or induced menstruations during a 21th day and by a next interruption on 7 days. A continuous reception is practiced in small doses.

Divina removes the displays of climacteric syndrome and normalizes a lipid exchange. During the first it is 11 days accepted a cycle on a 1 white pill on a day (2mg estradiole), then 10 days on a 1 blue pill (2 mg estradiole and 10 mgmedroxyprogesterone), interruption 7 days.

The second and third phases of climax for women run across on a background of estrogenic insufficiency, in these phases it is recommended to apply estrogen. To the most active estrogenic preparations take estradiole and ethynilestradiole, less active estrone and estriole.

With средньотяжким motion of climacteric syndrome folliculinum is appointed patients for 5000 IU daily or for 10 000 IU in a day intramuscular during 15 days. Then conduct supporting therapy of synestrole for 1 mg (10 000 IU) 2 times per a day (10 days) and for 0,5 mg 1 time per a day (5 days).

The patent combined preparation which contains estrogen is climacterinum. On the course of treatment - 100 drops, accept for a 1 drop 3 times per a day in 1 hour after a meal. It is possible to move 3-4 courses with interruptions on 2-3 months.

Contra-indication is to application of estrogen: disease of liver and kidneys, tumor, mastopathia, vaginal bleeding of unknown etiology, endometriosis.

By a patient in the age 50 and more years, proceeding in a menstrual cycle does not follow. The symptoms of climacteric syndrome are removed the combined setting of estrogen and аndrogens. In one syringe enter intramuscular 0,5 ml of a 0,1% solution of estradiole dipropionatis(0,5 mg) and 2,5 ml of a 1% solution of testosterone propionatis (25 mg). Injections execute 1 time per 5-7 days during 5-7 weeks to the removal of symptoms of climacteric syndrome, then 1 time per a month during 6-12 months.

Аmboseks, combined preparation of estrogen and androgen, enter intramuscular for 1 ml 1 time per a month during 6-12 months.

Contra-indication is to the use of androgen: gout, rheumatoid arthritis, hypertensive illness.

PATHOLOGICAL CLIMAX BY MEN
To the pathological variants of menopause for men take an early climax and climacteric syndrome. The symptoms of neuro-endocrine alteration accumulate on the displays of defeat of vessels atherosclerosis, which for men is formed on 7-10 years before, than for women.

Clinical picture
A hypertensive syndrome appears the unsteady increase of arterial blood pressure, typical transitory hypertension. Systolic pressure which brings an increase over of pulse pressure rises more considerable.

Cardial syndrome appears the attacks of cardial pains, unconnected with the physical loading, which are badly removed by coronarolitic preparations. Pains are removed on a background treatment by androgen.

Vasoasthenic syndrome is characterized the promoted fatigueability and muscular weakness. The attacks of muscular weakness appear suddenly, does not depend on the physical loading, are investigation of violation of regional circulation of blood.

Sexual and urinary dysfunction is related to the decline of tone of urinary bladder, violations of functioning of prostata: disturb indefinite dull pains in the area of urinary bladder and other dysuric phenomena. A libido is stored, but there can be violations of copulative cycle.

Diagnostics
And physiology, and pathological climax is accompanied the decline of formation of androgens in testicles. But a pathological variant is accompanied violation of metabolism of hormones: formation of active androgens (androsterone, dehydroepiandrosterone) goes down, the index of androsterone/dehydroepiandrosterone goes down.

A large value in pathogeny of pathological masculine climax has violation of balance of estrogens. At a physiology climax excretion of active estrogens (estrone, estradiole) goes down for men, prevails nonactive metabolite estriole. At a pathological climax for men a compensate decline of excretion of estriole is absent, active estrogen prevails (estradiole). Hypertensive and vasoasthenic syndromes are accompanied the expressed predominance of active estrogen, increase of relation of estradiole to estriole, diminishing of excretion of estriole.

Treatment
Therapy must be complex, to include psychotherapy, medical gymnastics, physical therapy treatment.

The purpose of hormonal therapy is creation of optimum hormonal background: androgens promote reactivity of spinal centres of erection, proceed in a libido, diminish expressed of vegetative violations. Treatment by androgens must not be continuous - development of atrophy of gonads is possible. Apply complex hormonal preparation of testobromlecitum, that consists methyltestosterone, bromuralum and lecithin. The course of treatment makes 6 weeks: 2 weeks on a 1 pill 3 times per a day under a language after a meal, 2 weeks - on a 1 pill 2 times per a day, 2 weeks - on a 1 pill 1 time per a day. Treatment can be picked up thread in 3-4 months.

Application of one андрогенів is possible. Methyltestosterone appoint on a chart: 1 week - on a 1 pill (5 mg) 2 times per a day sub lingua after a meal, 2 weeks - on a 1 pill 1 time per a day, 2 weeks - for 1/2 pills 2 times per a day. Sometimes appoint intramuscular injections 1% or to a 5% solution of testosterone propionates daily or in a day during 2-3 weeks. For the protracted therapy use the prolonged androgenic preparations - sustanon-250 or omnadrenum for 1 ml intramuscular 1 time per a month during 3-6 months.

DELAY OF SEXUAL DEVELOPMENT

DELAY OF SEXUAL DEVELOPMENT

A delay of sexual development is undeveloping of the second sexual signs and absence or rare irregular menstruations in age 15 and more.

Does not follow to equate the delay of sexual development with primary amenorrhea- absence of menarche in age 16 and more years old. Delay of sexual development - a concept is wider and primary amenorrhea often is its symptom.

Cerebral form of delay of sexual development

A delay of sexual development is polyethiologic pathology. Reasons of delay of sexual development can be cerebral violations of organic character: traumatic, toxic, infectious defeats (encephalitis, epilepsy, tumors of hypothalamic area). This pathology of CNS next to expressed nervous-psychical symptoms can entail the delay of sexual development, if the structures of mediobasal hypothalamus are attracted in a process.

To the cerebral forms belong delay of sexual development at psychoses, neuroses as a result of stresses (conflicts are in family, school, emotional overloads). One of cerebral forms of delay of sexual development is anorexia nevrosa, that a waiver is of meal, which is considered as a neurotic reaction in reply to pubertal changes in an organism. This pathology, as a rule, appears for girls with the burdened psychical heredity, in this connection a careful inspection is shown for a psychiatrist.

Constitutional (genetic) delay of sexual development

A delay of sexual development is not the display of some pathology and has the constitutional, inherited, genetically conditioned character.

Girls time-lagged sexual development differ from coevals not only insufficient development of the second sexual signs and аменореєю but also absence of "feminisation of figure", that by the division of fatty and muscular tissue on a womanish type and changes in the structure of pelvis. For girls time-lagged sexual development anthropometric researches are set a eunuch`s build: lengthening hands and feet, relatively short trunk, diminishing of transversal sizes of pelvis. Grew them usually higher, than for coevals. Chronologic age passes ahead biological (bone) age usually.

Hypoplasia of privy parts appears at gynaecological research (sexual infantilism is expressed). External and internal privy parts for patients time-lagged sexual development in 16-18 years answer the state of organs in age 10-11, that to the first phase of pubertal development. Ovaries are formed correctly, but the normal process of foliculogenesis and ovulation does not take place in them.

An exception is made by girls from hyperprolactinaemia (prolactinoma of hypophysis or functional hyperprolactinaemia). For these patients usually the sign of delay of sexual development is primary amenorrhea in combination with unsharply expressed hypoplasia of uterus. A build and length of body answer for them age-old norms, and pectoral glands are developed correctly.

Ovarial form of delay of sexual development

This form is least explored, obviously, in connection with its rareness. At her it is not set chromosomal pathology and changes of maintenance of sexual chromatine. In the structure of ovaries it is not discovered except for diminishing of follicle vehicle, changes. Ovaries are hypoplastic. Possibly, the infectious diseases of children's or toxic influencing which draw violation of follicle vehicle or innervations of ovaries have the defined value in pathogeny of this pathology, that causes change of their sensitiveness to the hormones of stimulants of hypophysis.

For the clinical picture of delay of sexual development there are the personal intersexual touches of build at hypoplastic ovaries. Without regard to the delay of processes of ossification of epiphysis of tubular bones, length of body of girls does not exceed ordinary age-old norms. The second sexual signs are underdeveloped, appears also hypoplasia of external and internal privy parts. Characteristic primary amenorrhea, however there can be liquid and wretched menstruations.

Patients with the delay of sexual development speak to the doctor, as a rule, in age not early than 17-18-th years. To that time they, their parents and, unfortunately, even doctors wait appearances of signs of sexual development. At the inspection of girls time-lagged sexual development it is necessary to use the followings criteria:

· absence of menarche is in age of 16-th years;

· absence of signs of beginning of the sexual ripening in age 13-14-ти years and more;

· absence of menarche during 3th and anymore from the beginning of appearance of development of pectoral glands;

· disparity of indexes of growth and mass of body to chronologic age.

For patients with the delay of sexual development at suspicion on the cerebral forms of pathology a neurological inspection is needed:

· EEG, which allow to differentiate organic or functional violations of cerebral diencephalons structures;

· sciagraphy of skull and Turkish saddle, computer tomography of the Turkish saddle;

· ultrasonic research of organs of small pelvis is for clarification of sizes of uterus and ovaries;

· at presence of additional indexes is laparoscopia with the biopsy of gonads;

· determination of sexual hormones is in the whey of blood;

· sciagraphy of hands is for determination of bone age.

Treatment of central forms of delay of sexual development is a difficult task. In development of complex treatment must take part neurologist. Except for settings of neurologist, cyclic vitamin therapy, and also substitute hormonal therapy is used at hypoplastic ovaries.

PREMATURE SEXUAL DEVELOPMENT

PREMATURE SEXUAL DEVELOPMENT

Premature sexual development is appearance of the second sexual signs and menstruations for girls under age 8.

1. Isosexual form of premature sexual development - the signs of sexual development, that appear for girls.

2. Heterosexual form of premature sexual development - girls have signs, incident to masculine sexual development.

Isosexual premature sexual development can be true and erroneous.

1.1. Veritable premature sexual development (cerebral form) is the state, when structures which are responsible for adjusting of the reproductive system - hypothalamus and hypophysis are involved in a process.

Veritable premature sexual development for girls can have organic and functional character.

1. Organic cerebral premature sexual development - caused the organic defeat of CNS. At the organic defeat of brain premature sexual development, as a rule, develops after appearance or on a background cerebral and neurological symptomatic. Reasons of cerebral premature sexual development:

1. an asphyxia is in births, maternity trauma, hypotrophy of fruit, gestosis;

2. heavy intoxication and infection during the first year of life;

3. cerebral infections like meningitis, encephalitis;

4. brain tumors.

These reasons result in development of internal hydrocephalia- to the stretch and increase of pressure in the ventricles of cerebrum, pressure on hypothalamus, which forms the bottom of Ш ventricle, is the same carried out.

2. Functional cerebral premature sexual development - caused violations of CNS of functional character after carried in the first years of life (2 - 4 years) of infectious diseases, intoxications.

Cerebral premature sexual development runs across as a complete or incomplete form.

3. The complete form of cerebral premature sexual development is characterized development of the second sexual signs and menstruation.

4. The incomplete form of cerebral premature sexual development is characterized the different degree of development of the second sexual signs in default of menstruations.

An incomplete form of premature sexual development is the first phase of period of the sexual ripening stretched as though in time. Меnarche for girls with the incomplete form of premature sexual development comes in 10 - 11 years.

Constitutional form of veritable premature sexual development.

At this form of premature sexual development it is not succeeded to find out neurological, cerebral pathology. Premature is age the process of the sexual ripening begins in which. A rate and sequence of this process is not violated. The inherited character of constitutional premature sexual development appears.

1.2. Ovarial form of premature sexual development.

Ovarial form of premature sexual development is conditioned the secretion of sexual hormones by tumor tissue of ovaries.

1.2.1. Hormonal active tumors of ovaries.

The first symptom of ovarial premature sexual development usually are menstrual-similar excretions of аcyclic character at the poorly developed second sexual signs. Appearance of menstrual-similar excretions from sexual ways for girls under age 8 induces parents immediately to appeal to the doctor. That is why from the beginning of hormonal secretion to the address to the doctor passes tumor tissue little time and the second sexual signs do not have time to develop.

HETEROSEXUAL PREMATURE SEXUAL DEVELOPMENT

HETEROSEXUAL PREMATURE SEXUAL DEVELOPMENT

Name appearance of signs of the sexual ripening of opposite (masculine) sex heterosexual premature sexual development for girls on the first decade of life.

Adrenogenital syndrome

Most frequent clinical form of heterosexual premature sexual development.

An adrenogenital syndrome is born hyperplasia of adrenal cortex, erroneous womanish hermaphroditism or premature sexual development of girls on a heterosexual type. An adrenogenital syndrome is investigation of a born deficit of the enzymic systems which take part in the synthesis of steroid hormones of adrenal glands.

An adrenogenital syndrome is a genetic defect with the recession way of inheritance, the transmitters of imperfect gene can be and men, and women.

Hyperproducts of androgens in the adrenal cortex at a born adrenogenital syndrome are investigation of mutation of gene and born genetically conditioned deficit of the enzymic system. The synthesis of cortisole is violated - basic glucocorticoid hormone of adrenal cortex, formation of which diminishes.

Depending on character of deficit of the enzymic systems an adrenogenital syndrome divides in 3 forms, a general symptom for them is virilisation.

· An adrenogenital syndrome is with the syndrome of loss of salt: the deficit of 3-β-dehydrogenasa results in the sharp diminishing of formation of cortisole, frequent vomit develops as a result, dehydration of organism with violation of cardiac activity. The symptoms of adrenogenital syndrome with the loss of salt appear at first time after birth.

· An adrenogenital syndrome with hypertension: the deficit of 11-hydroxylasa results in the accumulation of corticosterone hereupon to development of hypertension on a background violation of water and electrolyte exchange. The symptoms of adrenogenital syndrome with hypertension develop in the first decade of life. An adrenogenital syndrome with the loss of salt and hypertension meets rarely. Both these forms violate not only sexual development but also function of cardiac, digestive and other systems of organism. These patients make a general contingent for endocrinologies and paediatricians.

· adrenogenital syndrome, simple virilizing form: the deficit of С-21- hydroxylasa draws multiplying formation of androgens and development of symptoms of hyperandrogenia without the substantial decline of synthesis of cortisole. This form of adrenogenital syndrome is most frequent, not accompanied somatic violations of development. The deficit of С-21- of hydroxylasa, without regard to a born character, can appear in different periods of life; depending on it select born, pubertal, post-pubertal forms.

Diagnostics

An objective diagnostic method is ultrasonic research of adrenal glands and magnetically-resonancetomography of adrenal glands. By an informing test for diagnostics maintenance of 17-ketosteroids (17-КС) and dehydroepiandrosterone is sharply promoted in urine and/or testosterone in blood, which are normalized after conducting of test with glucocorticoid preparations.

Treatment

Treatment of a born adrenogenital syndrome consists in application of glucocorticoid preparations. A dose depends on age, mass of body of child and degree of hyperandrogenia, which expressly correlates with the level of testosterone in blood or 17-КС and dehydroepiandrosterone in urine. Therapy is conducted lasted, because the deficit of the enzymic system has a born character. Abolition of treatment results in getting up of level of andogens in blood and to returning of all signs of virilization.

ABSENCE OF SEXUAL DEVELOPMENT

ABSENCE OF SEXUAL DEVELOPMENT
Absence of sexual development is absence of menarche, absence of development of pectoral glands in age more than 16 years old.

Reason of this pathology is aplasia of gonads or violation of their development at which active hormone-producting tissue of ovaries is absent functionally. Sexual development does not come for girls to which by any testimony the delete of ovaries was conducted under age 8-10 years old. By other reason of absence of sexual development the defect of development of sexual glands, causedby dysgenesia of gonads, is genetically conditioned. Dysgenesia of gonads is rare pathology, with frequency 1 on 10 - 12 000 new-born.

Reason of development of dysgenesia of gonads is chromosomal violations as one loss of Х chromosome or its part. More frequent in all there are four clinical forms of dysgenesia of gonads: typical, or classic (syndromeTurner), effaced, clean and mixed.



Climax by woman and men

Menopause is the appropriate stage of individual development of man, transitional period from maturity to old age, which is accompanied the loss of reproductive function. A climax can be physiology or pathological. Middle ages of offensive of climax – 49-51 year, for women - 45-55 years, for men - 47-60 years. Duration of menopause – 2-5 years. Frequency of pathological motion of menopause is 25-50%.

Climacteric syndrome, or a pathological climax is an aggregate of endocrine-vegetative violations which are formed for some people during climacteric hormonal alteration. A deciding value has a presence of premorbid background - presence of stress factors, psycho-emotional or sexual violations, sharp or chronic diseases of СNS, internal organs, genitals.

Pathogeny of climacteric syndrome consists in the "senescence of hypothalamus", violation of functioning of homeostatic centers, located in hypothalamus and limbic system which results in disfunction of hypophysis and sexual glands.