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 should I 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.
The basis of treatment is hormone therapy. If necessary, surgery, plastic surgery is indicated.
How is disease classified?
Hypogonadism can be primary or secondary. The primary form is provoked by a violation of the functionality of the testicular tissue due to a defect in the testicles. Violations at the chromosomal level can provoke aplasia or hypoplasia of the testicular tissue, manifested in the absence of androgen secretion or insufficiency of their production for the full formationsexual organs and secondary sexual characteristics.
Hypogonadotropic hypogonadism in boys is expressed in mental infantilism.
The secondary form is caused by a violation of the structure of the pituitary gland, a decrease in its gonadotropic function or damage to the centers of the hypothalamus, which affect the pituitary gland and regulate its activity. The disease is expressed in mental disorders.
Both primary and secondary forms can be congenital or acquired. Pathology can contribute to infertility in men in 40-60% of cases.
Causes of disease in men
Low androgens can be caused by a decrease in the amount of hormones produced or by the pathological condition of the testicles themselves, dysfunction of the hypothalamus and pituitary gland.
To the etiology of the primary manifestation of the disease can be attributed:
- congenital underdevelopment of the gonads, which occurs with genetic defects,
- testicular aplasia.
Predisposing factors include:
- violation of testicular descent;
- exposure to toxic substances;
- administering chemotherapy;
- exposure to organic-based solvents, nitrofurans, pesticides, alcohol, tetracycline, high-dose hormone-based drugs, etc.;
- diseases of an infectious nature (mumps, measles, orchitis, vesiculitis);
- presence of radiation sickness;
- acquired testicular disease;
- twistspermatic cord;
- testicular torsion;
- atrophic process after surgery;
- hernia excision;
- scrotal surgery.
In primary hypogonadism, there is a drop in the level of androgens in the blood. A compensatory reaction of the adrenal glands develops, the production of gonadotropins increases.
Disorders of the hypothalamus and pituitary gland lead to the secondary form (inflammatory processes, neoplasms, disruption of blood vessels, pathology of intrauterine development of the fetus).
Contribute to the development of secondary hypogonadism can:
- growth hormone pituitary adenoma;
- adrenocorticotropic hormone (Cushing's disease);
- prolactinoma;
- dysfunction of the pituitary or hypothalamus after surgery;
- the aging process, which provokes a decrease in testosterone in the blood.
In the secondary form, there is a decrease in gonadotropins, leading to a decrease in the production of androgens by the testicles.
One form of disease that affects men is a decrease in sperm production with a normal testosterone level. It is very rare to see a decrease in testosterone levels with normal sperm levels.
Symptoms of the disease in men
Hypogonadotropic hypogonadism in men has its own clinical manifestations. They are due to the age of the patient, as well as the level of androgen deficiency.
If a boy's testicles are affectedbefore puberty, then a typical eunuchoidism is formed. The skeleton becomes disproportionately large. This is due to the delay in ossification in the growth zone. The shoulder region and chest also lag behind in development, the limbs become long, the skeletal muscles are poorly developed.
Female-type obesity, gynecomastia, hypogenitalism may be noted, which manifests itself in a small penis size, absence of folds in the scrotum, testicular hypoplasia, underdevelopment of the prostate gland, absence of pubic hair, underdevelopment of the larynx, high timbre of the voice.
With a secondary manifestation of the disease, a large patient weight, increased function of the adrenal cortex, and disruption of the thyroid gland are often noted.
If testicular function is reduced after puberty, then the symptoms of such a pathology as hypogonadotropic hypogonadism are less pronounced.
The following phenomena are noted:
- testicular reduction;
- small hair on the face and body;
- loss of skin elasticity and thinning;
- reduced sexual function;
- vegetative disorders.
Reducing the size of the testicles is always associated with reduced sperm production. This causes infertility, regression of secondary sexual characteristics occurs, muscle weakness, asthenia is noted.
Diagnosis of hypogonadism in men
Diagnosis is carried out by anthropometry, examination and palpation of the genitals, assessment of clinical symptoms of the degree of puberty.
X-ray examination will help estimate bone age. Densitometry is used to determine the saturation of bones with minerals. An x-ray of the Turkish saddle determines its size and the presence of neoplasms.
Estimating bone age allows you to determine by the timing of ossification of the joint of the hand and wrist when puberty began. This should take into account the possibility of earlier (for patients born in the south) and later (for patients born in the north) ossification, as well as the fact that other factors can also cause impaired osteogenesis.
Research in the sperm laboratory suggests conditions such as azo- or oligospermia.
The content of such hormones is indicated:
- sex gonadotropins;
- total and free testosterone;
- luteinizing hormone;
- gonadoliberin;
- anti-Müllerian hormone;
- prolactin;
- estradiol.
In the primary form of the disease, the level of gonadotropins in the blood is increased, and in the secondary form it is lowered. Sometimes their level is in the redistribution of the norm.
Determination of estradiol in blood serum is necessary for clinically pronounced feminization and secondary manifestation of the disease, in the presence of tumors in the testicles that produce estrogen, or tumors in the adrenal glands.
Urine ketosteroids may be normal or low. If Klinefelter's syndrome is suspected, a chromosome analysis is indicated.
Testicular biopsy is notable to provide information for correct diagnosis.
Treatment
Treatment of hypogonadotropic hypogonadism is aimed at eliminating the main cause that caused the pathology. The goal of therapy lies in preventive measures that contribute to the normalization of sexual development, the subsequent restoration of testicular tissue of the testicles and the elimination of infertility. The therapy is carried out under the supervision of a urologist and an endocrinologist.
How is hypogonadotropic hypogonadism in men eliminated? Treatment depends on a number of factors:
- clinical form of pathology;
- severity of disruption of the hypothalamus, pituitary gland and reproductive system;
- presence of parallel existing pathologies;
- time of onset of disease;
- age of the patient.
Treatment of adult patients is to correct the level of androgens and eliminate sexual dysfunction. Infertility caused by congenital hypogonadism cannot be treated.
In the case of a primary congenital anomaly or an acquired disease, with the preservation of endocrinocytes in the testicles, stimulant drugs are used. Boys are treated with non-hormonal drugs, while adult patients are treated with hormonal drugs (androgens and gonadotropins in small doses).
In the absence of reserve testicular function, replacement therapy with androgens and testosterone is indicated. Hormone intake is carried out throughout life.
In the secondary form of the disease in children andadults need to use hormone therapy with gonadotropins. If necessary, they are combined with sex hormones.
Fortifying treatment and physical education are also shown.
The operation for the disease consists in transplanting the ovary with cryptorchidism, with the underdevelopment of the penis, plastic surgery is used. For cosmetic purposes, they resort to implantation of a testicle on a synthetic basis (in the absence of an undescended testicle in the abdominal cavity).
Surgery is used using microsurgical techniques along with control of the immune system, hormone levels, and the implanted organ.
In the course of systematic therapy, androgen deficiency decreases, the development of secondary sexual characteristics resumes, potency is partially restored, manifestations of osteoporosis and bone age lag decrease.
How the disease progresses in women
Hypogonadotropic hypogonadism in women is characterized by underdevelopment and increased function of the gonads of the ovaries. The primary form is due to congenital underdevelopment of the ovaries or damage during the neonatal period.
There is a decrease in the 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, lack ofmenses.
If the function of the ovaries was impaired before puberty, then there is a lack of secondary sexual characteristics.
Hypogonadotropic hypogonadism in women in its primary form is noted under 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;
- testicular feminization syndrome;
- 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 effect of gonadotropins on the ovaries.
How are such diseases 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
Strong symptoms of a disease in the childbearing period are a violation of menstruation or their absence.
Low levels of female hormones lead to underdevelopment of the genitals, mammary glands, impaireddeposition 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.
Diagnostics
In hypogonadism, there is a decrease in estrogen levels and an increase in gonadotropin levels. By means of ultrasound, a reduced uterus is detected, osteoporosis and delayed skeletal formation are diagnosed.
Treatment of pathology in women
How is hypogonadotropic hypogonadism in women treated? Treatment involves replacement therapy. Women are prescribed medication, as well as sex hormones (ethinyl estradiol).
In the event of menstruation, oral contraceptives containing estrogens and progestogens, as well as drugs "Trisiston", "Trikvilar" are prescribed.
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 hypogonadotropichypogonadism, has a favorable prognosis. Prevention consists of public he alth education and monitoring of pregnant women, as well as he alth promotion activities.