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Hypergonadotropic hypogonadism in men

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lack of androgens under hypergonadotrophic( primary) hypogonadism is caused by testicular dysfunction, as the main body of synthesizing androgens. The insufficiency of sex hormones on the back( Fig. 1), the pituitary gland responds to increased synthesis of hormones, LH and FSH, the so-called gonadotropins. They are responsible for regulating the production of androgens by the testicles, i. E.The more LH and FSH is produced, the more in response to this reaction will be produced androgens and spermatozoa testicles. But since the function of the testicles is disturbed, the hypothalamus produces these hormones "in the dead," so their level is always elevated in the blood of the patient with primary hypogonadism.

There are congenital and acquired primary hypogonadism.

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  • 1 Etiology congenital hypergonadotropic hypogonadism
    • 1.1 Congenital pathology testicular
    • 1.2 Chromosomal pathology
    • 1.3 Other reasons:
  • 2 etiology of acquired hypergonadotropic hypogonadism:
  • 3 Diagnosis and treatment
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Etiology congenital hypergonadotropic hypogonadism

Congenital pathology testicular

Pathology and its frequency Hormone Characteristics Clinical Signs
Anarchism - manifested complete absenceeat the testicles in the scrotum, penis and underdevelopment moshonki. Redkaya pathology occurs in approximately 1: 20,000 testosterone decreased, FSH, LH, high, elevated SHBG syndrome, sexual dysfunction, lack of secondary sexual characteristics, obesity
monorchism - evidentCongenital absence of a single testicle. Frequency of pathology 0.001% In normal function of a single testicle, the testosterone level is within the normal range. In rare cases it may occur reduced testosterone with elevated levels of gonadotropin-releasing hormone In normal operation only egg abnormalities were found
Cryptorchidism - no one and two testicles in the scrotum because of their undescended. With cryptorchidism testicles are present most often in the abdominal or inguinal canal. The most frequent pathology of testicles, occurring in 2-4% of full-term and 15-30% of premature infants, as well as in 1% of boys under 1 year. Depending on the distance of the testicle( testicles) from the scrotum, there may be a violation and preservation of the functions of the synthesis of sex hormones and spermatogenesis. At preservation of functions of testicles, level of testosterone, LG, FSG is within the limits of norm. Violation of spermatogenesis and synthesis of sex hormones due to increased temperature exposure.
Hypoplasia of testicles - underdevelopment of one or two testicles, the sizes of which are significantly deviated from the norm. Often hypoplasia is combined with cryptorchidism. Depending on the form: unilateral hypoplasia - signs of androgen insufficiency, as a rule, no;Bilateral - clear signs of androgen insufficiency. Depending on the form: unilateral hypoplasia - signs of androgen insufficiency, as a rule, no;Bilateral - clear signs of androgen insufficiency. Chromosomal

pathology

Normally genetic set consists of 46 human chromosomes, 44 of which form a so-called somatic 22 couples a 23th pair responsible for sexually individual identity.

Female sexual X chromosome creates a pair of female sexual XX, while the male Y chromosome produces a male XY chromosome pair. Normally, the male karyotype is designated as 46XY, where 46 is the number of chromosomes, XY is the male sex pair. In most cases, violations occur when the male sex pair contains extra female chromosomes( Klinefelter's syndrome).The etiology of these disorders is unknown.

Pathology and its frequency characteristic Hormone Clinical signs
Klinefelter Syndrome - a genetic disease characterized by one or more extra X chromosomes in a female male karyotype XY.It is one of the most common causes of primary hypogonadism. Prevalence: 1: 500-700, or 0.2% of the male population FSH, LH increased, testosterone is reduced Tall, long legs, gynaecomastia, abdominal obesity, infertility, cognitive impairment, symptoms of androgen deficiency in men
Syndrome XX male orThe de la Chapelle syndrome .The patient's karyotype is 46XX instead of 46XY.Despite the female karyotype( 46XX), the patient's phenotype is male, becauseOne or two chromosomes X contain a normal male gene, the SRY gene, responsible for the development of the body in the male type. The prevalence of 4-5 per 100 000. FSH, LH increased, testosterone is reduced Clinical symptoms are similar to symptoms of Klinefelter's syndrome with some differences( lower growth, normal body proportions( in most cases), less a manifestation of disorders of mental faculties).Infertility of the patient is not amenable to treatment
XYY-syndrome .The patient's karyotype is 47XYY instead of 46XY.Was known as the "super-man syndrome".This name was associated with the first studies, when pathology was revealed in many prison inmates and patients in psychiatric hospitals, which indicated a possible cause of increased aggressiveness of the patient. Later, the connection between aggressiveness and-XYY syndrome is completely refuted FSH, LH increased, testosterone may be reduced slightly In most cases, the clinical symptoms of hypogonadism were not found

Other reasons:

  • Noonan syndrome;
  • Aplasia of Leydig cells. The etiology of Acquired

hypergonadotropic hypogonadism:

  • Disease and testicular trauma( orchitis, testicular torsion, testicular infarction);
  • Endocrine diseases( metabolic syndrome and obesity, diabetes mellitus, dysfunction of the thyroid gland and adrenal glands);
  • Diseases of the liver, kidneys, lungs, gastrointestinal tract;
  • Effects of poisons, toxins, medications, alcohol, drugs;
  • Infectious diseases( tuberculosis, syphilis, HIV, parotitis).Diagnosis and treatment

basic form is a diagnostic examination of the patient to determine the severity of primary and secondary sexual characteristics and laboratory analysis, indicating an increased level of gonadotrophic hormones( FSH, LH) and testosterone reduced.

Nebido Treatment of hypergonadotropic hypogonadism is carried out by substitutive preparations of testosterone. One of the standard formulations of the injection form is testosterone undecanoate under the trade name Nebido. Advantages of Nebido over other hormonal drugs is the duration of the action( one injection at 10-14 weeks) and its gradual release into the patient's blood, without abrupt changes in testosterone levels.

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