Primary hypogonadism is a syndrome caused by the direct effect of the pathological process on ovarian function, as a result of which a sharp decrease in estrogen secretion occurs.
Etiology. Primary ovarian failure may occur as a result of congenital disorders of sexual differentiation (gonadal dysgenesis), damage to the ovaries by an infectious process (epidemic parotitis, syphilis, tuberculosis, etc.), surgical removal of the ovaries, etc.
Pathogenesis. The basis of the pathogenesis of primary hypogonadism is a decrease in estrogen production, resulting in atrophic changes in the genital organs, mammary glands, primary amenorrhea, etc. When you turn off ovarian function before puberty, secondary sexual characteristics do not develop.
Pathological anatomy. Histologically, ovarian biopsy reveals proliferation of connective tissue, desolation of follicles, etc.
Clinic. With damage to the ovaries in children, secondary sexual characteristics do not develop or are weakly expressed (underdevelopment of the mammary glands, poor pubic hair growth and under the arms). The uterus, vagina, ovaries remain underdeveloped, primary amenorrhea is noted. The body has eunuchoid proportions, a narrow pelvis, and flat buttocks. When a disease develops in adults, there is no skeletal imbalance. Underdevelopment of the genitals is less pronounced. Secondary amenorrhea occurs, various manifestations of vegetative neurosis are often noted.
Laboratory data. In the blood, the level of gonadotropic hormones is increased, and estrogen is significantly reduced. Urinary excretion of gonadotropic hormones is increased, and estro genes are sharply reduced.
Radiodiagnosis. On pneumopelviogram revealed a sharp hypoplasia of the uterus and ovaries. When X-ray logical study of the osteo-articular system, there is a delay in the maturation of the skeleton, osteoporosis (usually in the bones of the wrist joint and the spine). When craniography, hyperpneumatization of the sinus of the main bone, small size of the Turkish saddle and “juvenilization” (straightening) of its back are often found.
Diagnosis and differential diagnosis. The diagnosis is made on the basis of anamnesis, clinic and laboratory data. Primary hypogonadism is differentiated from secondarily about hypogonadism. In contrast to the primary, secondary hypogonadism occurs as a result of the defeat of the hypothalamic-pituitary system and is characterized by a decrease in the level of gonadotropic hormones in the blood and a decrease in their excretion with urine.
Forecast. The prognosis for life is favorable. Full recovery does not occur. Patients remain barren.
Treatment. To stimulate physical development in prepubertal age, anabolic steroids are prescribed: oral methandrostenolone (nerobol) at 0.1-0.15 mg per 1 kg of body weight or intramuscularly retabolil at 1 mg per 1 kg of body weight 1 time per month, etc. e. Treatment with anabolic steroids is carried out for a long time, intermittently (2-3 months with monthly breaks) for many years.
It should be remembered that the use of anabolic steroids can cause virilization (clitoral enlargement). From the age of 15, estrogen therapy is prescribed. In order to soften the epiphyseal growth zones, estrogenic drugs are administered in the initial period in small doses (no higher than 5000 IU per day in terms of folliculin). Estrogen preparations (synesterol, diethylstilbestrol, estradiol dipropionate, etc.) are administered first constantly until menstrual-like bleeding occurs, after which they are prescribed intermittently, mimicking the normal menstrual cycle. When the proliferative vaginal smear type is proliferative, estrogenic drugs are combined with pregnin or progesterone (estrogen therapy is administered every 15–16 days every month, and 10 mg 3 times a day with preggnin or progesterone 1 ml 0.5 % solution inside the muscular or subcutaneous). After the growth zones are closed, treatment with female sex hormones is performed in conventional therapeutic oses. The use of synthetic progestins (infekundin, biseurin) gives a good effect.