The clanfelter syndrome develops only in boys and is associated with the addition of excess X-chromosome. The main symptoms of the disease are manifestations of eunuchoidism, a decrease in testosterone concentration and a decrease in the size of the testicles.
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The concept of clanfelther syndrome
Chaninfelter syndrome or dysgenezia of seed tubules (violation of the development of seed tubules) was first described in 1942 by Klinfelter as a combination of eunuchoidism, gynecomastia (increase in the chest), small testicles, lack of selection of sperm and enhanced secretion of follicularity immuling hormone.
The clanfelter syndrome is caused by a congenital anomaly of sex chromosomes, in which a patient has one extra x-chromosome, less often for extra x-chromosoma there are several. Normally, a normal set of genital chromosomes in men is described as xy.
With the clanfelter syndrome in the intrauterine period, the development of the testicles occurs normally and the newborn child does not differ from other children almost until adolescence. During the period of puberty, the sizes of the testicles do not increase, as it happens in the norm, and decrease. Eggs become more dense. They are replaced by the normal tissue tissue with fibrous heavy eggs, the number of cells producing men's sex hormones decrease sharply.
The main manifestations of the disease
Hypogonadism occurs (failure of the function of the germ). The growth of bones in length due to lack of androgens does not stop and develop «Eornchoid» Body proportions with long limbs. Hair growth scanty, on the pubic hair growth in the female type. Sexual member of usually normal sizes or can be somewhat reduced, small eggs, flabby.
Sexual function, the erection is reduced, the amount of ejaculate (semen released) is small, the orgasm is lowered. In patients in infertility. Some patients with clanfelter syndrome meet mental disorders. Patients often avoid medical care and argue that they are completely healthy. They have antisocial behavior.
Chaninfelter syndrome can be combined with other systemic diseases, sugar diabetes, thyroid disease, chronic nonspecific lung diseases.
Diagnosis and treatment of disease
The diagnosis of clinfell syndrome is set on the basis of the characteristic complaints and the appearance of patients, the absence of testicles during palpation and ultrasound examination. In the blood of patients reduced the level of testosterone. Confirms the diagnosis of a genetic study.
Treatment of the disease is aimed at restoring the level of men's hormones in the body. For this purpose, replacement therapy androgen are carried out - male sex hormones (Sustanon, Testanat). Initially, large doses are prescribed when the positive effect is reached, the dose is reduced. Infertility in such patients is incurable.
