Hypogonadism-syndrome, accompanied by lack of function of sexual glands and violation of the synthesis of sex hormones. Hypogonadism is usually accompanied byunderdevelopment of external or internal genital organs and secondary sexual characteristics, disorders of lipid and protein metabolism (obese or cachectic patients,changes in the skeletal system, cardiovascular compromise). Diagnosis and therapy ofhypogonadism is the joint work of endocrinologists, obstetricians and Gynecologists-Endocrinologists (among women), andrologists (men). Foundation treatment ofhypogonadism is hormone replacement therapy. If necessary a surgical correction, plastic and prosthetic genitalia.
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Hypogonadism
Classification of hypogonadism in males
Causes and mechanisms of the development of the hypogonadism in males
Symptoms of hypogonadism in males
Diagnosis of hypogonadism in males
Treatment of hypogonadism in males
Symptoms of hypogonadism in women
Diagnosis of hypogonadism in women
Treatment of hypogonadism in women
Hypogonadism-treatment in Moscow
Hypogonadism-syndrome, accompanied by lack of function of sexual glands and violation of the synthesis of sex hormones. Hypogonadism is usually accompanied byunderdevelopment of external or internal genital organs and secondary sexual characteristics, disorders of lipid and protein metabolism (obese or cachectic patients,changes in the skeletal system, cardiovascular compromise).
There are male and female hypogonadism.
Hypogonadism in men
Classification of hypogonadism in males
Hypogonadism is divided into primary and secondary.
Primary hypogonadism is caused by dysfunction of the testicular tissues due to a defect of the testicles. Chromosome disorders can cause testicular hypoplasia or aplazii fabrics thatreflected the lack of androgen secretion or failure for the normal formation of sex organsand secondary sexual characteristics.
The emergence of secondary hypogonadism due to violation of the pituitary gland, decreased its gonadotrophic function or lesion of gipotalamičeskih centers, regulating the activity of the pituitary gland.
Primary Hypogonadism, developing in early childhood is accompanied by mentalinfantilizmom, secondary-mental disorders.
There
are also gipergonadotropnyj and normogonadotropnyj, HYPOGONADOTROPICHYPOGONADISM. Gipergonadotropnyj hypogonadism is the primary defeat of testiculartesticular tissue in combination with elevated gonadotroponah pituitary hormones.HYPOGONADOTROPIC HYPOGONADISM occur in normogonadotropnyj and defeat of the hypothalamic-pituitary system. HYPOGONADOTROPIC HYPOGONADISM is associated with a reduction in the secretion of gonadotropins, resulting in reduced testicular tissue testicularandrogen production. Normogonadotropnyj hypogonadism due giperprolaktinemiei isnormal levels of gonadotropins and reduced testicular testicular function.
Both primary and secondary hypogonadism may be congenital and acquired.
Manifestations of hypogonadism may provide some forms of male infertility (from 40 to60% of all cases of male infertility).
Depending on the age of the development failure of sex hormones are embryonic,dopubertatnaâ (from 0 to 12 years old) and postpubertatnaâ forms of hypogonadism.
Primary (gipergonadotropnyj) congenital hypogonadism occurs:
When anorhizme (aplazii) testicles;
If any omissions (cryptorchidism and ectopia) testicles;
When the true hromatinpoložitel′nom Klajnfel′tera syndrome (combines gipoplaziu testes,gialinoz walls and disgeneziû of seminiferous tubules, Gynecomastia, often accompanied byazoospermia (absence of spermatozoa). testosterone Production is reduced by about 50%.
the syndrome Shereszewski-Turner (chromosomal disease with characteristic physicaldevelopment: nizkoroslost′û and lack of sexual development, rudimentary semennikami);
the sertoli cell syndrome or syndrome del Castillo (testicular hypoplasia with normal or elevated amount of gonadotropins). In this syndrome, patients are not formed sperm are infertile. Physical development is male;
the syndrome of incomplete masculinization-the wrong man's germafroditizme. Reason forreduction of tissue sensitivity to androgens.
Primary hypogonadism experience develops as a result of the effects on the testes of internal or external factors after birth.
Testicular tumors, injuries and early castration is a typical picture-evnuhizma-pervasivehypogonadism;
in the absence of germinativnogo epithelium (the false Klajnfeltera syndrome).Characterized by high growth, evnuhoidnym physique, Gynecomastia, underdevelopedsecondary sexual characteristics, small size of the genitals. The pubertatnomu period in patients developed evnuhoidnye traits in subsequent reduced fertility.
Congenital secondary (HYPOGONADOTROPIC) hypogonadism is when States:
associated with damage to the hypothalamus-a stand-alone form only with the defeat of the reproductive system. Is characterized by a severe deficiency of gonadotropins, there may be a shortage of lûtropina or folitropina;
Kallmena syndrome is characterized by deficiency of gonadotropins, underdevelopment of genitals and secondary sexual characteristics, the reduction or lack of sense of smell(giposmiej or anosmiej). The evnuhoidizm (often in combination with kriptorhizmom), various malformations: cleft of the upper lip, hard palate, shortening bridles, asymmetrical face, language šestipalost′, Gynecomastia, cardio-vascular disorders.
at gipofizarnom nanizme (pituitary dwarfism). There has been a sharp decline in GH,luteinizing, follicle-stimulating adrenocorticotropic hormones and thyroid-stimulating, that isa violation of the testes, the adrenal glands and the thyroid gland. Characterized by a lack of sexual characteristics, a dwarf growth less than 130 cm, infertility.
congenital pangipopituitarizme (kraniofaringiome), caused by congenital brain tumor. It grows, it compresses the pituitary tissue, disrupting its function. Reduced production of gonadotropins and the hormones regulating adrenal function and thyroid. Results in abacklog of physical and sexual development of the child.
Mèddoka syndrome is an extremely rare form of Hypogonadism, caused by insufficientgonadotrophic function adrenokortikotropnoj and the pituitary gland. Is characterized by a gradual increase in gipokortitizma. After passing puberty there is a paucity of sex glands-evnuhoidizm, gipogenitalizm (maturation of sexual organs and secondary sexual characteristics), reduced libido, infertility.
Acquired secondary hypogonadism is when:
adipozogenitalina dystrophy-is obesity and gipogenitalizmom. The insufficiency of thegonadotrophic function of the pituitary. Manifesting with 10-12 years. Hypothalamic-pituitary abnormalities with distinct clinical symptoms are not observed. Characterized byskeletal proportions, usually evnuhoidnye sexual dysfunction and infertility. BecauseDystrophic changes in the heart and vascular hypotension may develop shortness of breath, biliary dyskinesia, flatulence.
Lawrence syndrome-Moon-Bardet-Bidlâ (LMBB), syndrome Prader-Willie. LMBB syndrome isobese, low intelligence, pigmentary retinal dystrophy and polidaktiliej. There iscryptorchidism, testicular hypoplasia, ginekomastia, erectile dysfunction, poor adult face,underarms, pubic area, there may be defects in kidney development. Prader-Willy syndromeunlike LMBB has multiple anomalies ("Gothic" sky, èpikant, etc.), pronounced muscle weakness on the background of reducing androgen and gonadotropins in blood. Bothsyndromes are attributed to functional disorders of the pituitary gland and hypothalamus.
gipofizarnom gipotalamicescom syndrome due to the defeat of the hypothalamic-pituitaryregion by infectious-inflammatory, tumor, brain trauma.
giperprolaktinemičeskom syndrome with infertility and disorders of sexual function, and thechildhood and adolescence, causes a delay in pubertal development and hypogonadism.
Causes and mechanisms of the development of the hypogonadism in males
Androgen deficiency can be caused by hormones produced by reduction or violation of their biosynthesis by testicular or violation of the pathology of the hypothalamic-pituitarydysregulation.
Etiological factors of primary hypogonadism often provide:
congenital deficiency of sex glands, occurs when genetic defects, for example, dysgenesis(the fabric of the structure) of seminiferous tubules; dysgenesis and testicular Aplasia(anorchia, monorchism). In the case of congenital pathology negative role played by the harmful effects on the body of a pregnant. As gipogonadnomu can lead to the violation ofthe omission of the testicles.
toxic (chemotherapy of malignant neoplasms, organic solvents, alcohol, pesticides,nitrofuran, tetracycline antibiotics, hormonal drugs in large doses, etc.)
infectious diseases (measles, mumps, Orchitis, epididymitis, deferentit, vesiculitis)
radiation syndrome (when exposed to x-rays, radiation therapy)
acquired testicular injury-trauma, torsion of the spermatic cord torsion, testicular, varicocele; atrophy and hypoplasia of testis after orhipeksii operations, herniotomy, surgical interventions on organs of scrotum.
Some cases of primary hypogonadism are idiopathic. Modern Endocrinology has insufficient data on the etiology of idiopathic hypogonadism.
When the primary Androgen levels decrease gipogonadizme in blood, the development ofcompensatory reactions the adrenal gland at gipoandrogenizaciû, an increase of production of gonadotropins.
Gipogonadizmu lead to secondary violations of hypothalamic-pituitary dysregulation(inflammation, tumors, vascular disorders, abnormal embryonic development). Development of hypogonadism may cause pituitary adenomas produce growth hormone (whenacromegaly) or adrenocorticotropic hormone (Cushing's disease), prolactinoma, postoperative or Posttraumatic hypothalamic-hypophyseal Portal dysfunction,hemochromatosis, the aging process, causing blood testosterone levels decline in the population.
At the secondary level is low gipogonadizme gonadotropins, leads to reduced secretion of androgens eggs.
A form of male hypogonadism is the reduction of the production of sperm in the normallevel of testosterone, as well as the extremely rare cases of reduction of testosteronewithout reducing the production of sperm.
Symptoms of hypogonadism in males
Clinical manifestations of hypogonadism due to age of the disease and the level ofandrogen deficiency. Violation of the production of androgens in the prenatal period maylead to the development of external genitalia intersex.
If a testicular lesion occurred in boys in the dopubertatnom period, there is a delay sexual development, a typical evnuhoidizm: disproportionate growth associated with delayedossification of epiphyseal (growth) zone, undeveloped chest and shoulder, long limbs,underdeveloped skeletal musculature. There may be development of obesity in female type,true Gynecomastia, gipogenitalisme, which is evident in the small size of the penis, there is no pigmentation and folding of the scrotum, testicular hypoplasia, underdevelopment of the prostate, absence of body hair growth on the face and pubic hair, hypoplasia of the larynx,high voice.
In cases of secondary hypogonadism, obesity is often caused symptoms of hypothyroidismthyroid, adrenal cortex, of pangipopituitarizma, lack of sex drive and potency.
If the reduced function of the testes is developing after puberty, the symptoms ofhypogonadism are weaker. There has been a decrease in the size of the testicles, smalladult face and body fat for women's type, loss of elasticity and thinning of the skin, infertility, decreased sexual function, vegetative-vascular disorders.
Reduced testes observed in almost all cases of male hypogonadism (exception: If the disease started recently). Reducing the size of the testicles are usually closely associatedwith reduced sperm production. The loss of spermoproduciruûŝej function of the testes is developing infertility with the termination of the production of testosterone, decreasedlibido, there is regression of secondary sexual characteristics, erectile dysfunction, there aregeneralized symptoms (reduced muscle strength, fatigue, weakness).
Diagnosis of hypogonadism in males
Based on the complaints of the patient data, medical history, general study status usingAnthropometry, inspection and palpation of the genitals, the evaluation of clinical symptoms of Hypogonadism, the degree of sexual maturity.
According to estimated bone age x-ray. To determine the mineral bone densitometry is saturation. When x-ray the Sella turcica are determined by its size and the presence of a tumor. Assessment of bone age allows time-ossification of the wrist joint and hand accurately enough to determine the beginning of puberty. Early puberty is associated with formation of sesamovidnoj bone in the 1st phalanx metacarpophalangeal joint (approximately 13.5-14 years). Full sexual maturity indicates the appearance of anatomical sinostozov. This feature makes it possible to distinguish between not being spanked or age pubertal. Evaluating bone age you need to take into account the possibility of an earlier (for patients from the southern regions) and late (for patients from Northern) ossification, and that violation of osteogenesis may be due to other factors. With dopubertatnom gipogonadizme lagging behind for several years the "bone age" from the Passport.
Laboratory study semen analysis (sperm count) when gipogonadizme is azo-oroligospermiei; sometimes ejaculate you cannot get.
A measurement of the level of sex and gonadotropins: serum testosterone (total and free),luteinizing and follicle-stimulating hormone, gonadoliberina, and antimûllerovskogo of blood serum hormone, prolactin, estradiol. The content of testosterone in the blood is reduced. At the primary level in blood of gonadotropins gipogonadizme upgraded, with secondary-lowered, sometimes the content is within the normal range. Determination of the level of serum estradiol required when clinically pronounced feminization and secondarygipogonadizme, in the case of testicular tumours or èstragenproduciruûŝih of the adrenal gland.
Level 17-KS (ketosteroidov) in the urine when gipogonadizme may be normal or reduced. In case of Klajnfeltera syndrome shows a chromosome analysis.
Testicular biopsy is rarely gives information for diagnosis and evaluation of prognosis or treatment.
Treatment of hypogonadism in males
Treatment of hypogonadism is strictly individual, and aim to eliminate the causes of the disease. The goal of treatment is to prevent the backlog of sexual development, hereinafter referred to as testicular tissue testicular malignancy and sterility. Treatment ofhypogonadism must be carried out under the supervision of an endocrinologist andurologist.
Treatment of hypogonadism is dependent on its clinical forms of violations in thehypothalamic-pituitary system and sex, concomitant pathologies, the time of occurrence of illness and the age of diagnosis.
Treatment of hypogonadism starts with treating the underlying disease.
Treatment of adult patients is to correct insufficient androgens and sexual dysfunction.Infertility, occurred against a backdrop of innate and incurable hypogonadism who,especially in the case of a low sperm count and azoospermia.
In case of primary congenital and acquired hypogonadism (with stored reserves ofendocrinocytes, in the testicles) is stimulating therapy: boys-negormonal′nymi drugs, and in adult patients-hormonal means (small doses of gonadotropins, androgens). In the absence of a reserve capacity of the testicles is shown as a substitution taking androgens(testosterone) constantly, throughout life.
When the secondary gipogonadizme in children and in adults should be a stimulatinghormone therapy gonadotropins (if necessary, combining them with sex hormones). It is also shown the conditioning therapy, physiotherapy exercises.
Surgical treatment of gipogoandizma is to transplant the eggs, bringing down the eggs inthe case of cryptorchidism, when nedorazvii penis-falloplastike. For cosmetic purposesimplantation of synthetic eggs (in the absence of neopustivšegosâ eggs in the abdominal cavity). Operations are carried out using Microsurgical technique in the control of immuneand hormonal status of the patient and of the transplanted organ.
In the process of a systematic treatment of hypogonadism is decreased androgeninsufficiency: resumes development of secondary sexual characteristics, partly restoredpotency, reduced the severity of associated manifestations (osteoporosis, bone age"backlog", etc.).
Hypogonadism in women
Female hypogonadism is characterized by underdevelopment and hypofunction of sexual glands-ovaries. Primary hypogonadism due to a congenital underdevelopment of the ovaries, or damage them in the newborn period. In the body there is shortage of female sex hormones, which causes increased production of gonadotropins, stimulating the ovariesin the pituitary gland. In the blood serum of high levels of follicle stimulating and luteinizinghormones (gipergonadotropnyj hypogonadism) and low concentration of estrogen.
Lack of estrogen causes the maturation and atroficskie changes of female genitals, breasts, primary amenorrhoea. If the violation occurs in the ovaries, not being spanked or period, the secondary sex characteristics are missing.
Causes of primary gipergonadotropnogo hypogonadism is a congenital genetic disorder(syndrome Shereszewski-Turner) ovarian hypoplasia, congenital, infectious processes(syphilis, tuberculosis, mumps), ionizing radiation (radiation, x-ray), surgical removal of the ovaries, autoimmune ovarian lesion (autoimmune oophoritis), testicular feminizationsyndrome (congenital condition in which the appearance of a man is a woman with a malegenotype), polycystic ovarian syndrome.
The secondary female hypogonadism (HYPOGONADOTROPIC) occurs when the hypothalamic-pituitary pathology, characterized by the lack or cessation of synthesis andsecretion of gonadotropins, governing the function of the ovaries. Develops due toinflammation in the brain (encephalitis, meningitis, arachnoiditis), the damaging effects of brain tumors and stimulating action accompanied by a decrease in gonadotropins on theovarian function.
Symptoms of hypogonadism in women
One of the main symptoms of hypogonadism in child-bearing period-the menstrual cycleand amenorrhea.
Lack of female sex hormones leads to underdevelopment of sexual characteristics: the genitals, breast, violation of women's fat deposition type, poor ovoloseniû. If the disease iscongenital or it arose in early childhood, the secondary sex characteristics are missing.Characterized by narrow hips and flat buttocks. If hypogonadism has puberantnom period,sexual characteristics that have already managed to develop, but menstruation ceases,tissues of female genitalia are exposed to atrophy.
Diagnosis of hypogonadism in women
If there is a noticeable decrease of gipogonadizme estrogen in the blood, increased levelsof gonadotropins (follicle stimulating and luteinizing hormone). Ultrasound shows uterus,reduced in size (hypoplasia), reduced testes. X-ray detects osteoporosis or delay formationof the skeleton.
Treatment of hypogonadism in women
The first female appointed gipogonadizme drug replacement therapy women's sex hormones (etinilestradiolom). In the case of the occurrence of menstrual′nopodobnojreactions, prescribed combined oral contraceptives that contain two types of hormones-estrogens and progestogens: Diane-35, silest, triziston, trikvilar. Women after 40 years appoint clymenus, trisekvens, movalis for rheumatoid arthritis. Hormone replacement therapy is contraindicated in malignant tumors of mammary glands and sexual organs, cardio-vascular diseases, kidney disease, liver disease, thrombophlebitis, etc.
Forecast for life when gipogonadizme is favorable. Prevention of hypogonadism is healtheducation population, watching pregnant women and health care.
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