As a rule, hypogonadotropic hypogonadism is associated with underdevelopment of the genitals and secondary sexual characteristics. The pathology of fat and protein metabolism 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 endocrinolong gynecologists, 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 are indicated.
How is the disease classified?
Hypogonadism can be primary and secondary. The primary form is provoked by a violation of the functionality of the testicular tissue due to a defect in the testicles. Disorders at the chromosomal level can provoke aplasia or hypoplasia of testicular tissue, manifested in the absence of androgen secretion or insufficient production of them for the full formation of the genitals 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 the primary and secondary forms can be congenital or acquired. Pathology can contribute to infertility in men in 40-60% of cases.
Causes of the disease in men
A low amount of androgens can be caused by a decrease in the amount of hormones produced or by the pathological condition of the testicles themselves, impaired function of the hypothalamus and pituitary gland.
The etiology of the primary manifestation of the disease can be attributed to:
- congenital underdevelopment of the sex glands, which occurs with defects of a genetic nature,
- testicular aplasia.
Predisposing factors include:
- testicular prolapse;
- exposure to toxic substances;
- conducting chemotherapy;
- the effect of organic solvents, nitrofurans, pesticides, alcohol, tetracycline, hormone-based drugs in high dosage, etc .;
- diseases of an infectious nature (mumps, measles, orchitis, vesiculitis);
- the presence of radiation sickness;
- acquired pathology of the testicles;
- twisting of the spermatic cord;
- testicular inversion;
- atrophic process after surgery;
- hernia excision;
- surgical operation in the scrotum.
With primary hypogonadism, a drop in the level of androgens in the blood is observed. A compensatory reaction of the adrenal glands develops, gonadotropin production increases.
Violations of the hypothalamus and pituitary (inflammatory processes, neoplasms, impaired vascular function, fetal developmental pathology) lead to the secondary form.
Promote the development of secondary hypogonadism can:
- pituitary adenoma that produces growth hormone;
- 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, a decrease in gonadotropins is noted, leading to a decrease in the production of androgens by the testes.
One form of disease that affects men is a decrease in sperm production with a normal testosterone count. Very rarely there is a decrease in testosterone levels at 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 the boy’s testicles are affected before puberty, then typical eunuchoidism is formed. The skeleton becomes disproportionately large. This is due to the delay in ossification in the growth zone. The humeral region and chest also lag behind in development, the limbs become long, the skeletal muscles are poorly developed.
Female type obesity, gynecomastia, hypogenitalism can be noted, which is manifested in a small penis, lack of folds in the scrotum, testicular hypoplasia, underdevelopment of the prostate gland, lack of hair in the pubic region, underdevelopment of the larynx, and high voice timbre.
With the secondary manifestation of the disease, a large patient weight, increased adrenal cortex function, and thyroid dysfunction are often noted.
If the function of the testicles is reduced after the puberty, then the symptoms of such a pathology as hypogonadotropic hypogonadism are less pronounced.
The following phenomena are noted:
- testicular contraction;
- slight hairiness of the face and body;
- loss of skin elasticity and thinning;
- decreased sexual function;
- vegetative disturbances.
A decrease in testicular size is always associated with reduced sperm production. This causes infertility, a regression of secondary sexual characteristics occurs, muscle weakness, asthenia are noted.
Diagnosis of hypogonadism in men
Diagnosis is through anthropometry, examination and palpation of the genitals, assessment of the clinical symptoms of puberty.
An X-ray examination will help evaluate bone age. Densitometry is used to determine bone mineral saturation. With an x-ray of the Turkish saddle, its size and the presence of neoplasms are determined.
Evaluation of bone age allows the timing of ossification of the joint of the hand and wrist to determine when puberty began. In this case, it is necessary to 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 a violation of osteogenesis can be caused by other factors.
Research in the field of sperm laboratory allows us to talk about such conditions as azo - or oligospermia.
The content of such hormones is indicated:
- sexual gonadotropins;
- total and free testosterone;
- luteinizing hormone;
- gonadoliberin;
- anti-Muller hormone;
- prolactin;
- estradiol.
In the primary form of the disease, the rate of gonadotropins in the blood is increased, and in the secondary, it is reduced. Sometimes their level is in the range of the norm.
The determination of estradiol in blood serum is necessary with clinically pronounced feminization and with the secondary manifestation of the disease, in the case of the presence of tumors in the testicles producing estrogen, or tumors in the adrenal gland.
Urinary ketosteroids may be normal or decreased. If Klinefelter syndrome is suspected, chromosome analysis is indicated.
Testicular biopsy is not able to provide information for proper diagnosis.
Treatment
Treatment of hypogonadotropic hypogonadism is aimed at eliminating the main cause of the pathology. The goal of therapy is 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 endocrinologist.
How is hypogonadotropic hypogonadism in men eliminated? Treatment depends on a number of factors:
- clinical form of pathology;
- the severity of the disruption of the hypothalamus, pituitary gland and reproductive system;
- the presence of parallel existing pathologies;
- time of occurrence of the disease;
- age of the patient.
Treatment of adult patients consists in correcting the level of androgens and eliminating sexual dysfunction. Infertility that occurs with a congenital form of hypogonadism cannot be treated.
In the case of the primary congenital form of the anomaly or acquired disease with the preservation of endocrinocytes in the testes, stimulant drugs are used. Boys are treated with non-hormonal drugs, and adult patients are treated with hormone-based drugs (androgens and gonadotropins in small doses).
In the absence of testicular reserve function, replacement therapy with androgens and testosterone is indicated. Reception of hormones is carried out throughout life.
In the secondary form of the disease in children and adults, it is necessary to use hormone therapy with gonadotropins. If necessary, they are combined with sex hormones.
General strengthening treatment and physical education are also indicated.
The operation for the disease consists in an ovary transplant with cryptorchidism, with underdevelopment of the penis, plastic surgery is used. For cosmetic purposes, they resort to testicular implantation on a synthetic basis (in the absence of an undescended testicle in the abdominal cavity).
Surgical intervention is used using microsurgical techniques along with monitoring the state of the immune system, hormone levels, and also the implanted organ.
In the process of systematic therapy, androgen deficiency decreases, the development of secondary sexual characteristics resumes, the potency is partially restored, the manifestations of osteoporosis and lagging of bone age are reduced.
How is the disease in women?
Hypogonadotropic hypogonadism in women is characterized by underdevelopment and increased function of the reproductive glands of the ovaries. The primary form is due to congenital underdevelopment of the ovaries or their damage during the neonatal period.
There is a decreased production of sex hormones, which provokes an increase in the level of gonadotropins that stimulate the ovaries.
The analysis shows a high rate of hormones that stimulate follicles and lutein, as well as a low level of estrogen content. A decreased estrogen index 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 primary form is observed in the following conditions:
- a congenital disorder at the genetic level;
- congenital ovarian hypoplasia;
- infectious processes (syphilis, tuberculosis, mumps, radiation, surgical removal of the ovaries);
- autoimmune damage;
- testicular feminization syndrome ;
- polycystic ovary.
Secondary hypogonadotropic hypogonadism in women occurs with the pathology of the pituitary 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 provoked by inflammation in the brain. Such diseases have a damaging effect and are accompanied by a decrease in the level of exposure of the gonadotropins to the ovaries.
How are diseases such as hypogonadotropic hypogonadism in women and pregnancy related? Adverse 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.
Low levels of female hormones lead to underdevelopment of the genitals, mammary glands, impaired deposition of fatty tissue and poor hair growth.
If the disease is congenital in nature, then secondary sexual characteristics do not appear. In women, there is a narrow pelvis and flat buttocks.
If the disease arose before puberty, then the sexual characteristics that appeared remain preserved, but the menstrual period ceases, the genital tissues atrophy.
Diagnostics
With hypogonadism, there is a decrease in estrogen levels and an increase in gonadotropins. By means of ultrasound, a uterus reduced in size is detected, osteopoporosis 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 case of menstruation, oral contraceptives containing estrogens and gestagens, as well as Triziston and Trikvilar preparations are prescribed.
Means "Klimen", "Trisekvens", "Klimonorm" are prescribed to patients after 40 years.
Hormonal treatment is contraindicated in the following conditions:
- oncological tumors in the area of mammary glands and genitals;
- heart and vascular diseases;
- pathology of the kidneys and liver;
- thrombophlebitis.
Prevention
A disease such as hypogonadotropic hypogonadism has a favorable prognosis. Prevention consists in public health education and monitoring of pregnant women, as well as in measures to protect health.