With successful treatment, the prognosis with adiposogenital dystrophy is favorable, the development of the disease can be stopped.
Adipozohenitalna dystrophy syndrome (Pehkrantsa-Babinski-Fröhlich) - a neuroendocrine disease manifested metabolic disorders (obesity) and the function of sexual glands (hypogonadism) due to lesions of the hypothalamic-pituitary system.
Causes of Adiposogenital Dystrophy
- Pathologically leaking pregnancy in the mother (intoxication, toxoplasmosis and other infections);
- Bacterial and viral encephalitis and meningitis;
- Brain tumors.
Manifestations of adiposogenital dystrophy
Despite the fact that the disease occurs in childhood, it is most often diagnosed in puberty, paying attention to the overweight child.
The disease is equally inclined to both boys and girls. It is noted typical body type: long limbs disproportionate high growth, combined with the weakness of ligament-muscular system (flat feet, posture, myopia, etc.) and abdominal obesity, but intellectual development is usually not disturbed.
The boys may experience an increase breasts (gynecomastia) and the small size of the external genitalia. It is often noted neopuschennya testicles into the scrotum (cryptorchidism) and delayed sexual development (hypogonadism), including weak expression of secondary sexual characteristics (body hair on pubis, armpits may be missing mustache and beard), infertility.
In girls, there may be a delay and even a complete absence of the menstrual cycle, and genital organs lag behind in development, infertility develops. The skin is often dry and thin.
On the internal organs there are disturbances in the form of dystrophic changes in the myocardium, propensity to hypotension: hypomotor dyskinesia of the gall bladder, constipation, low blood pressure, etc.
Diagnosis of adiposogenital dystrophy
In addition to the very characteristic symptoms and appearance of patients, there is a decrease in the level of hormones in the blood: testosterone, estrogens, lutropin, folitropin.
Conduct genetic (chromosomal) research and examination to exclude other causes of adiposogenital dystrophy (diabetes mellitus, thyroid disease, heart disease, etc.).
To exclude the tumor process and other diseases of the brain, X-rays of the skull and Turkish saddle, MRI of the brain are performed. The ultrasound of the pelvic organs and consultations of related specialists are shown.
Treatment of adiposogenital dystrophy
If the primary cause of the disease that caused the disorder in the hypothalamic-pituitary system is found, the treatment of the underlying disease (if it is a tumor lesion, then operative and radiotherapy, chemotherapy) is performed.
Also nominate nootropics - drugs that improve metabolic processes in the nervous system.
Weight adjustment (diet, exercise therapy, massage, physiotherapy procedures, etc.) is carried out.
Conduct correction of hormonal condition. As a rule, this is done by an endocrinologist in conjunction with a gynecologist, andrologists (urologist), a psychotherapist (a medical psychologist). From the period of puberty, substitution therapy is performed by male or female sex hormones.