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Congenital primary hypogonadism
Last reviewed: 23.04.2024
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Causes of the congenital primary hypogonadism
The cause of the development of congenital primary hypogonadism is not clear. It is believed that the death of embryonic testes occurs around the 20th week of intrauterine development of the fetus, when the urethra is already formed according to the male type, but there is no normal development of the penis: there are no cavernous bodies, the penis and scrotum are underdeveloped, sometimes the scrotum is absent ("smooth crotch ").
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Diagnostics of the congenital primary hypogonadism
Diagnosis of congenital primary hypogonadism - in the examination of patients, testicles are not detected either in the abdominal cavity or along the inguinal canals. Karyotype 46.XY, sexual chromatin is negative. The level of gonadotropins in the plasma is high, and testosterone is low. The urinary content of 17-CS is significantly reduced.
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Treatment of the congenital primary hypogonadism
Treatment of congenital primary hypogonadism depends on the clinical picture, psychosexual orientation and the patient's response to androgen treatment. With a sudden underdevelopment of the penis, which excludes the possibility of sexual activity, and low sensitivity to androgens, it is sometimes justified to elect a female civil (passport) sex with a feminizing reconstruction of the genitals and a constant substitution therapy with estrogens. With a relatively developed penis and an adequate response to androgen therapy (injections of sustanone-250 per 1 ml every 3-4 weeks intramuscularly or 10% solution of testenate per 1 ml every 10 days), it is recommended that males are kept and replacement therapy with androgens commencing puberty ( from 12-13 years).