Very few people think about the huge role hormones play in our body. The endocrine system is second only to the nervous system in importance, it is responsible for the normal functioning of the whole body, including procreation. With her pathology, diseases such as diabetes, gigantism, hypothyroidism and even hypogonadism.
Hypogonadism - what is it in men?
Hypogonadism syndrome refers to the underdevelopment of the gonads or a decrease in their function. Basically, its pathogenesis is characterized by a decrease in the production of male steroids, signs of infantilism appear, that is, there is a reverse development from a man to a boy, in connection with.
IMPORTANT: the concentration of androgens depends not only on the normal state of the organs directly producing it - but also on endocrine system located in the brain. According to the international classification of diseases of the 10th revision (ICD 10), hypogonadism can be assigned the code E29 if it is associated with testicular dysfunction and E23 if it is a consequence of the pathology of the pituitary gland.
Kinds
There are many disorders that lead to the described symptom complex, however, for convenience, it is classified into 3 main types.
Hypergonadotropic hypogonadism
Caused by damage to the hypothalamic-pituitary system, which dominates the rest of the endocrine glands. That is, in fact, it is responsible for the production of absolutely all hormones in our body, and human viability depends on its work.
The hypothalamus controls the pituitary gland, and the second synthesizes gonadotropins: follicle-stimulating hormone (FSH) and luteinizing (LH) hormone, which regulate all the activity of the reproductive system. With this hypogonadism, there is an excessive or incorrect production of these hormones.
Normogonadotropic hypogonadism
This type differs from the previous one in that the pituitary gland is working normally, but the problem occurs directly in the sex glands. The formation of testosterone in the Leydig cells of the testicles is disrupted, usually this is caused by inflammatory or cicatricial changes.
Hypogonadotropic hypogonadism
It is characterized by a decrease in the secretory function of the dominant endocrine system - the synthesis of gonadotropins in the structures of the brain decreases, which leads to underdevelopment of the testicles and, as a result, a decrease in the production of steroids.
Causes
Many researchers divide the causes of hypogonadism into two large groups.
Congenital (primary):
- complete absence of testicles;
- not lowering them into the scrotum;
- Klinefelter's syndrome (additional X chromosome in males, formula 47, XXY);
- Heller-Nelson syndrome (improper development of Leydig cells in the testes, which leads to a decrease in testosterone production);
- de la Chapelle disease (violation of the divergence of sex chromosomes, as a result of which a person looks like a man, but has a female set of chromosomes 46, XX);
- Morris symptom complex (complete insensitivity of receptors in the body to androgens) and Reifenstein's syndrome (partial insensitivity);
- Jacobs syndrome (47, XYY);
- myotonic dystrophy (a genetic disease characterized by weakness of the muscles of the face and hands, as well as changes in the thyroid, pancreas and gonads);
- Del Castillo disease (during development in the womb, the primary cells of the gonads are not laid);
- underdevelopment of Leydig cells and, as a result, insufficient production of the main male hormone;
- Kallman's syndrome (hereditarily determined decrease in the production of FSH and LH);
- adrenogenital symptom complex (genetic deficiency of the enzyme that forms steroids).
Acquired (secondary):
- castration;
- bilateral atrophy, that is, a decrease in volume and loss of function;
- excess estrogens inhibit the synthesis of FSH due to the "feedback" mechanism;
- orchitis;
- exposure to radiation;
- in the area of the pituitary gland;
- tumors of the hypothalamus, pituitary gland or testicles;
- trauma;
- slowing down the breakdown of estrogens;
- uncontrolled use of drugs (cytostatics);
- hyperprolactinemic symptom complex (which inhibits testosterone secretion);
- with chronic renal failure;
- with cirrhosis of the liver;
- with anorexia nervosa;
- with other endocrinological diseases (, thyrotoxicosis, etc.);
- with injuries lumbar spine with spinal cord injury;
- age-related androgen deficiency in men.
Symptoms and signs
Testosterone and gonadotropins affect not only the reproductive system, but also the musculoskeletal system. Accordingly, if pituitary hypogonadism is congenital or acquired during sexual maturation, then changes in the normal proportions are observed.
A eunuchoid skeleton is formed: tall and long limbs. The bones become thin and brittle. Skeletal muscles are poorly expressed, fat is deposited on the thighs and buttocks (according to female type). There are no changes in the larynx, the voice remains high, like that of a child. Beard, mustache, pubic hair and in the armpits are weakly expressed. The genitals are reduced, the penis is small, there are no folds on the scrotum, and there is no erection. Often observed and gynecomastia (benign breast enlargement in men).
If hypogonadism began to progress in adulthood, then changes are observed mainly in the reproductive system:
- increasing impotence;
- the disappearance of adequate and spontaneous erections;
- reduction of testicles.
Stop growing hair on the face, in the armpits and groin. Weakens, however, does not disappear completely. Very often, such men suffer from depression, neurosis, there is a frequent change of mood. Gynecomastia may also develop.
Diagnosis of hypogonadism in men
The lack of sex hormones is called hypogonadism. In men, this disease is associated with insufficient secretion of androgens, and in women - estrogens. With hypogonadism, the manifestations of the disease primarily relate to the sexual sphere and reproductive abilities. Also, the lack of sex hormones provokes changes in metabolism and functional disorders of various organs and systems.
sex hormones
Sex steroids in adults are formed mainly in the gonads. In women, the source of estrogens is the ovaries, in men, the source of androgens is the testicles.
The activity of sex steroid synthesis is regulated central regions endocrine system. The pituitary gland secretes stimulatory gonadotropins.
These include:
- FSH - follicle stimulating hormone;
- LH is a luteinizing hormone.
Both hormones support normal functioning reproductive system in adults and contribute to its proper development in children.
Follicle-stimulating gonadotropin causes:
- accelerates the maturation of eggs in women;
- triggers spermatogenesis in men.
Luteinizing gonadotropin:
- stimulates the synthesis of estrogens in the ovaries;
- responsible for ovulation (release of a mature egg);
- activates the production of testosterone in the testicles.
The activity of the pituitary gland is subject to the regulation of the hypothalamus. In this department of the endocrine system, releasing hormones are produced for LH and FSH. These substances increase the synthesis of gonadotropins.
The hypothalamus secretes:
- luliberin;
- folliberin.
The first of them stimulates mainly the synthesis of LH, the second - FSH.
Classification of hypogonadism
The lack of sex steroids in the body may be due to damage to the hypothalamus, pituitary gland, ovaries or testicles.
Depending on the level of damage, 3 forms of the disease are distinguished:
- primary hypogonadism;
- secondary hypogonadism;
- tertiary hypogonadism.
The tertiary form of the disease is associated with damage to the hypothalamus. With such a pathology, releasing hormones (luliberin and folliberin) cease to be produced in sufficient quantities.
Secondary hypogonadism is associated with dysfunction of the pituitary gland. At the same time, gonadotropins (LH and FSH) cease to be synthesized.
Primary hypogonadism is a disease associated with the pathology of the gonads. In this form, the testicles (ovaries) cannot respond to the stimulating effects of LH and FSH.
Another classification of androgen and estrogen deficiency:
- hypogonadotropic hypogonadism;
- hypergonadotropic hypogonadism;
- normogonadotropic hypogonadism.
Normogonadotropic hypogonadism is observed in obesity, metabolic syndrome, hyperprolactinemia. According to laboratory diagnostics, in this form of the disease, normal levels of LH and FSH, a decrease in estrogens or androgens are observed.
Hypergonadotropic hypogonadism develops when the testicles (ovaries) are affected. In this case, the pituitary and hypothalamus secrete an increased amount of hormones, trying to activate the synthesis of sex steroids. As a result, an increased concentration of gonadotropins and low level androgens (estrogens).
Hypogonadotropic hypogonadism is manifested by a simultaneous drop in blood tests of the levels of gonadotropins and sex steroids. This form of the disease is observed when the central parts of the endocrine system (pituitary and / or hypothalamus) are affected.
Thus, primary hypogonadism is hypergonadotropic, while secondary and tertiary hypogonadism is hypergonadotropic.
Primary and secondary hypogonadism can be congenital or acquired.
Etiology of hypogonadotropic hypogonadism
Secondary hypogonadism can develop for a variety of reasons.
Congenital forms are associated with:
- Cullman's syndrome (hypogonadism and impaired sense of smell);
- Prader-Willi syndrome (a genetic pathology that combines obesity, hypogonadism and low intelligence);
- Laurence-Moon-Barde-Biedl syndrome (a genetic pathology that combines obesity, hypogonadism, pigmentary retinal degeneration and low intelligence);
- Maddock's syndrome (loss of gonadotropic and adrenocorticotropic functions of the pituitary gland);
- adiposogenital dystrophy (a combination of obesity and hypogonadism);
- idiopathic hypogonadism (cause unknown).
Isolated idiopathic hypogonadism may be associated with adverse effects on the fetus at the time of intrauterine development. Excess chorionic gonadotropin in the mother's blood, dysfunction of the placenta, intoxication, exposure medicines can negatively affect the emerging pituitary and hypothalamus. Subsequently, this can lead to hypogonadotropic hypogonadism. Sometimes there is a sharp decrease in the synthesis of only one of the hormones (LH or FSH).
Acquired secondary hypogonadism can be caused by:
- severe stress;
- lack of nutrition;
- a tumor (malignant or benign);
- encephalitis;
- trauma;
- surgery (for example, removal of a pituitary adenoma);
- irradiation of the head and neck;
- systemic connective tissue diseases;
- vascular diseases of the brain.
Manifestations of the disease
Congenital hypogonadism provokes violations of the formation of the genital organs and the absence of puberty. In boys and girls, the disease manifests itself in different ways.
Congenital hypogonadotropic hypogonadism in men leads to:
- underdevelopment of the genital organs;
- lack of spermatogenesis;
- eunuchoidism;
- gynecomastia;
- deposition of adipose tissue according to the female type;
- lack of secondary sexual characteristics.
For girls:
- the external genital organs are developed correctly;
- secondary sexual characteristics do not develop;
- observed primary amenorrhea and infertility.
In adults, the loss of secretion of LH and FSH can lead to a partial regression of secondary sexual characteristics and the formation of infertility.
Diagnosis of the disease
Secondary hypogonadism may be suspected in children and adults with characteristic reproductive disorders.
To confirm the diagnosis is carried out:
- external examination;
- Ultrasound of the testicles in men;
- Ultrasound of the small pelvis in women;
- blood test for LH and FSH;
- analysis for releasing hormones (luliberin);
- blood test for androgens or estrogens.
In men, sperm can be examined (with an assessment of the number and morphology of gametes). In women, egg maturation is monitored (for example, with ovulation tests).
Secondary hypogonadism is put if a low level of FSH, LH, androgens (estrogens) is detected.
Treatment of hypogonadism
Treatment is carried out by doctors of different specialties: pediatricians, endocrinologists, urologists, gynecologists, reproductologists.
In boys, treatment should begin as soon as the disease is diagnosed. In girls, hypogonadism is corrected from the age of 13–14 (after reaching a bone age of 11–11.5 years).
Hypogonadism in men is corrected with drugs with gonadotropic activity. Therapy with exogenous testosterone does not restore its own spermatogenesis and the synthesis of sex steroids.
The choice of a specific medication to protect against a form of deficiency. The most commonly used preparations of chorionic gonadotropin. This substance is effective in the treatment of LH deficiency or a combined decrease in two gonadotropins. Chorionic gonadotropin is used for idiopathic secondary hypogonadism, Maddock and Cullman syndromes, adiposogenital dystrophy.
If the patient is dominated by FSH deficiency, then he is shown treatment with other drugs - menopausal gonadotropin, serum gonadotropin, pergonal, etc.
In women, treatment is carried out:
- chorionic gonadotropin;
- clomiphene;
- menopausal gonadotropin;
- pergonal;
- estrogen and progesterone.
Clomiphene stimulates the synthesis of gonadotropins in the pituitary gland. Chorionic hormone, menopausal gonadotropin and pergonal replace LH and FSH. Estrogens and progesterone are used with caution. These hormones suppress the pituitary gland. But they successfully replace the natural hormones of the ovaries.
As a rule, hypogonadotropic hypogonadism is associated with underdevelopment of the genital organs and secondary sexual characteristics. Fat and protein metabolism in pathology is also impaired, which causes obesity, cachexia, disorders in the skeletal system and malfunctions of the heart.
Which doctors to contact?
It should be noted that male and female hypogonadotropic hypogonadism is different.
Diagnosis and treatment of the disease is carried out jointly by endocrinologists, gynecologists and gynecologists-endocrinologists, if the patient is a woman, and andrologists, if the patient is a man.
There is a reduced production of sex hormones, which provokes an increase in the level of gonadotropins that stimulate the ovaries.
The analysis shows a high level of hormones that stimulate follicles and lutein, as well as a low level of estrogen. A reduced estrogen level causes atrophy or underdevelopment of the female genital organs, mammary glands, and the absence of menstruation.
If ovarian function was impaired before puberty, then there is a lack of secondary sexual characteristics.
Hypogonadotropic hypogonadism in women in the primary form is noted in the following conditions:
- congenital disorder at the genetic level;
- congenital ovarian hypoplasia;
- infectious processes (syphilis, tuberculosis, mumps, radiation, surgical removal of the ovaries);
- defeat of an autoimmune nature;
- polycystic ovaries.
Secondary hypogonadotropic hypogonadism in women occurs with pathology of the pituitary gland and hypothalamus. It is distinguished by a low content or a complete cessation of the production of gonadotropins that regulate ovarian function. This process is triggered by inflammation in the brain area. Such diseases have a damaging effect and are accompanied by a decrease in the level of exposure of gonadotropins to the ovaries.
How are diseases such as hypogonadotropic hypogonadism in women and pregnancy related? Unfavorable intrauterine development of the fetus can also affect the occurrence of pathology.
Symptoms of the disease in women
Vivid symptoms of the disease in the childbearing period are a violation of menstruation or their absence.
A low level of female hormones leads to underdevelopment of the genital organs, mammary glands, impaired deposition of fatty tissue and poor hair growth.
If the disease is congenital, then secondary sexual characteristics do not appear. Women have a narrow pelvis and flat buttocks.
If the disease occurred before puberty, then the sexual characteristics that appeared remain preserved, but menstruation stops, genital tissues atrophy.
Carrying out diagnostics
With hypogonadism, there is a decrease in estrogen levels and an increase in gonadotropin levels. Through ultrasound, a reduced uterus is detected, osteoporosis and a delay in the formation of the skeleton are diagnosed.
Treatment of pathology in women
How is hypogonadotropic hypogonadism in women eliminated? Treatment involves replacement therapy. Women are prescribed medications, as well as sex hormones (ethinyl estradiol).
In the event of the onset of menstruation, oral contraceptives containing estrogens and gestagens are prescribed, as well as drugs "Trisiston", "Trikvilar".
Means "Klimen", "Trisequens", "Klimonorm" are prescribed to patients after 40 years.
Treatment with hormonal agents is contraindicated in the following conditions:
- oncological tumors in the area of the mammary glands and genital organs;
- diseases of the heart and blood vessels;
- pathology of the kidneys and liver;
- thrombophlebitis.
Prevention
A disease such as hypogonadotropic hypogonadism has a favorable prognosis. Prevention consists in the population and monitoring of pregnant women, as well as in health measures.
Hypogonadism in men is a disease characterized by a decrease in the production of male sex hormones - androgens. The disease is expressed by the underdevelopment of the external and internal genital organs, as well as external sexual characteristics.
There are primary hypogonadism and secondary. Also, the disease is divided into hyper- and hypogonadotropic hypogonadism.
The primary type of the disease is the result of a defect in the testicles themselves, more precisely, a violation of the function of the testicular tissue. The cause of the development of pathology is chromosomal abnormalities.
Secondary hypogonadism develops due to a violation of the structure of the pituitary gland, a decrease in its gonadotropic function, or damage to the hypothalamic centers that regulate the activity of the pituitary gland.
Primary hypogonadism usually occurs at an early age, and is accompanied by a slowdown in mental development. The secondary form of the disease is often due to mental disorders, from which a person can suffer at any age. Primary and secondary hypogonadism are congenital and acquired.
Hypogonadotropic hypogonadism is characterized by reduced secretion of tropic pituitary hormones, hypergonadotropic - increased.
Reasons for the development of hypogonadism
Hypogonadism occurs due to:
- Congenital underdevelopment of the gonads, which is associated with some genetic defects. In this case we are talking about a violation of the tissue structure of the seminiferous tubules, aplasia or testicular dysgenesis (monorchism and anorchism). Often, hypogonadism is provoked by the mother taking some medicines during pregnancy. Also, the disease can contribute to a violation of the descent of the testicles;
- Toxic effects of pesticides, chemotherapy, drugs (hormonal drugs and tetracyclines), alcohol and others;
- Infectious diseases (root orchitis, mumps, deferentitis, epididymitis, vesiculitis, etc.). This group also includes radiation injury as a result of radiation therapy and X-ray exposure;
- All kinds of damage to the testicles: trauma, varicocele, torsion of the spermatic cord, testicular torsion;
- Inflammatory processes, tumors and vascular disorders.
So, primary hypogonadism (hypergonadotropic) is caused by infectious, traumatic and radiation factors. Secondary (hypogonadotropic) hypogonadism is provoked by a wider range of causes: from intrauterine lack of androgens to damage to higher cortical centers that regulate the production of releasing hormones by the hypothalamus.
Symptoms of hypogonadism (primary and secondary)
Manifestations of hypogonadism in men depend on the age at which the disease made itself felt, and the degree of androgen deficiency. If the pathology began to develop even in the prenatal period, then often such a violation leads to the appearance of bisexual external genitalia.
Symptoms of androgen deficiency are expressed in disorders of sexual function: decreased libido, ejaculation and orgasm disorders, erectile dysfunction and reduced ejaculate fertility.
Hypogonadism is also manifested by psycho-emotional and vegetative-vascular disorders. The patient finds himself with hyperemia of the face, upper body and neck, while periodically feeling a feeling of heat, fluctuations in blood pressure, lack of air and dizziness. Increasingly, he is worried about irritability, memory loss, insomnia, fast fatiguability, decreased performance and deterioration in general well-being.
Hypogonadism in men is often accompanied by such somatic symptoms: an increase in adipose tissue, a decrease in muscle mass, gynecomastia, thinning and atrophy of the skin, a decrease in hair on the body and face.
Depending on the cause and form of the disease, the symptoms may be different. So, if the testicles are affected even before puberty, then the patient suffers from the so-called eunuchoid syndrome. A person is distinguished by high disproportionate growth, elongated limbs, underdeveloped shoulder girdle and chest. Skeletal muscles are poorly developed, subcutaneous tissue is distributed as in women. There is practically no hair on the face and body, the voice is high, the larynx is underdeveloped, which indicates underdevelopment secondary sexual characteristics. In hypogonadism in men, the size of the penis is unnaturally small, there are no folds on the scrotum, and the prostate gland cannot be felt mechanically.
In secondary hypogonadism, in addition to the main symptoms of androgen deficiency, obesity and dysfunction of the endocrine glands - the adrenal cortex and the thyroid gland - occur. The patient almost does not feel sexual desire, he has no potency.
With the development of hypogonadism after puberty, the symptoms are less pronounced due to the formation of the genital organs and the musculoskeletal system. However, for this form of the disease, the following symptoms are still characteristic:
- Reduction of the testicles;
- thinning of the skin;
- Obesity by female type;
- Decrease in the density of the hairline of the face and body;
- sexual dysfunction;
- Infertility;
- Vegetative-vascular disorders.
Almost half of male infertility cases are due to hypogonadism.
Diagnosis and treatment of hypogonadism
The following methods are used to detect the disease:
- General examination of the patient;
- Hormonal blood test for testosterone, luteinizing hormone, sex steroid-binding globulin and follicle-stimulating hormone;
- Determination of some pituitary hormones, sex hormones;
- Genetic analysis;
- spermogram;
- MRI of the brain.
Hypogonadism is a chronic disease that is difficult to completely cure. However, there is always an opportunity to reduce symptoms. To begin with, the doctor determines the causes that caused the disease, then prescribes a course of adequate treatment for hypogonadism.
If the disease appeared after puberty, then the main goal of treatment is the prevention of further abnormalities and infertility. Infertility on the background of congenital hypogonadism or that occurred before puberty is incurable.
The choice of therapy depends on the clinical picture of the disease and the severity of symptoms. Treatment of hypogonadism consists in correcting androgen deficiency and normalizing the reproductive system. Properly chosen measures, applied for a long time, are able to resume the growth of secondary sexual characteristics and regulate the production of androgens.
Congenital and acquired forms of the disease with a reserve capacity of the testicles are treated with special stimulating therapy. Boys are prescribed non-hormonal drugs, and men are given small doses of androgens and gonadotropins (hormonal drugs). If there is no reserve capacity, a person will have to use testosterone throughout his life.
Sometimes the treatment of hypogonadism is carried out surgically, although such cases are extremely rare.
It is a serious pathology, which is caused by insufficient formation of sex hormones and poor sperm production.
This disease is manifested in the underdevelopment of the genital organs and secondary sexual characteristics.
At the same time, hypogonadism is distinguished as hypergonadotropic and hypogonadotropic.
The second type of the disease can be combined with insufficient production of FSH and LH.
It also manifests itself with Kalman syndrome.
What it is
Hypogonadotropic hypogonadism is a lesion of the reproductive system and is characterized by a deficiency of gonadotropic hormones or their complete absence. The disease occurs in men in different ways, depending on age and the degree of hormone deficiency.
If hypogonadotropic hypogonadism has developed during pregnancy, then it can manifest itself in the formation of bisexual external genital organs.
Prevalence and significance
Causes and consequences
The causes of this disease have not yet been fully determined. Heredity does not play such a big role in this case.. A decrease in gonadotropins in the blood is sometimes associated with the course of pregnancy in the mother of a sick child and her contact with harmful chemicals.
In some cases, the cause of the disease may be fetal asphyxia or difficult childbirth.
This pathological condition can be congenital or acquired.
Besides, replacement therapy is being developed taking into account the lack of pituitary hormones and production of peripheral hormones. With insufficient thyroid function, L-thyroxine can be prescribed in the daily rate.
With a decrease in ACTH secretion, it is necessary to take glucocorticoids. These include prednisone, cortisone, triamcinolone, and other drugs. One of the drugs is taken several times a day.
In case of violation of the gonadotropic function of the pituitary gland, therapy with the help of androgens is prescribed. In addition, gonadotropic hormones and antiestrogenic agents are prescribed.
The choice of treatment method depends on the age of the patient. It takes into account the importance of maintaining the function of the testicles and provides for the mandatory control of the pituitary or hypothalamus. No less important is the severity of androgen deficiency.
With a severe form of the disease and the unimportance of the sperm-forming function of the testicles, the doctor prescribes testosterone replacement therapy. With complete dysfunction of the testicles, it must be used for life.
At a young age, androgen replacement therapy is performed, which leads to the active development of the external genital organs and sexual characteristics. After their formation, gonadotropic drugs are used that stimulate spermatogenesis.
If androgen deficiency appeared with age, androgen replacement therapy is prescribed. But the most commonly used chorionic gonadotropin. It leads to stimulation of the production of its own testosterone. As a result of the use of this hormone, it is possible to maintain normal testosterone levels and eliminate the symptoms of androgen deficiency.
In addition, menopausal gonadotropin is prescribed, which contains follicle-stimulating and luteinizing hormone in equal amounts. With the help of such drugs, you can stimulate the production of testosterone and sperm. In this disease, drugs are used that stimulate the production of gonadotropic hormones.
Help with hypogonadotropic hypogonadism and physiotherapy, which can strengthen the general condition of the body. In addition to treatment, physical therapy exercises are prescribed.
Surgery
Male hypogonadism in some cases requires surgical treatment. It consists in transplanting a testicle, which is obtained from a donor. Also, a surgical operation is performed with an underdeveloped penis, which allows you to increase its size. If at birth the testicle did not descend into the scrotum, the help of a surgeon is also needed. In this case, a synthetic testicle is used, which is sewn into the scrotum.
Treatment at home
Folk remedies do not allow to cure this disease, therefore they are not prescribed. You can only use recipes that make it possible to improve potency.
