Adiposogenital dystrophy or syndrome Pehkrantsa-Babinski-Fröhlich is a neuroendocrine disorder characterized by obesity of the hypothalamic-pituitary origin and dysfunction of the sexual glands (hypogonadism).
Adiposogenital dystrophy caused by damage to the nuclei of the hypothalamus, which are responsible for the regulation of appetite and hypothalamic nuclei, in which the synthesis of hormones GnRH, stimulates the pituitary hormones follicle stimulating hormone and lyutropina. In the absence or reduced number of follicle stimulating hormone stimulation and lyutropina hormone gonads does not occur and, ultimately, there is insufficiency of sexual glands.
Causes of Adiposogenital dystrophy may serve as:
deferred infections of the brain - viral and bacterial meningitis, encephalitis
By the destruction of the nuclei of the hypothalamus may cause intrauterine infection and intoxication, birth injuries of the head, carried by the mother during pregnancy, toxoplasmosis. Sometimes lead to the defeat of the hypothalamus of brain tumors in this area.
Adiposogenital dystrophy occurs in childhood, but is most often seen during puberty, in adolescence, when it should happen puberty. Equally likely to get sick boys and girls. The earliest sign of disease is overweight.
In boys, there is glandular enlargement (gynecomastia) can be reduced to the size of the testicles and penis. Often occurs in boys cryptorchidism (undescended testicle into the scrotum). In adolescence, puberty is delayed. Male and female secondary sexual characteristics are formed poorly. The boys lean body hair on the pubes and armpits, often a lack of growth of beard and mustache. Scanty hair growth persists into adulthood. Sexual organs are underdeveloped.
In girls, the vulva are lagging behind in development, menstruation delayed (late menarche) or not at all arise.
In both sexes disrupted development of the skeleton, which is formed by evnuhoidnomu type: tall, long limbs, the scope of which exceeds the growth of a few centimeters.
There is a deficiency of ligament-joint system: pathologically joints mobile, pererazognuty is formed flat. Patients marked obesity. Pokozhno fat is stored mostly in the abdomen, thighs, chest and face. The skin of patients thin and dry.
Internal organs can also be changed:
lead to abnormalities in the gastro-intestinal tract
violations of the outflow of bile
from cardiovascular degenerative changes occur in cardiac muscle - myocardial appears.
Intellectual development of patients in most cases, normal, appropriate to the age and education.
Diagnosis Adiposogenital dystrophy set on the basis of specific complaints, the appearance of the patients show a decrease in blood concentration:
follicle stimulating hormone
Treatment of Adiposogenital dystrophy
First of all, be appointed, if possible treatment of the underlying disease, which led to the defeat of the hypothalamus.
In the process of tumor assigned radiotherapy or surgery.
Is the treatment of inflammatory diseases:
In addition, prescribers, improving the condition of the tissues of the brain:
To limit the weight assigned to a diet with reduced calorie foods, reduced fat and carbohydrates.
Necessarily assigned to physical therapy with reasonable exercise.
Boys are assigned to hormone replacement therapy with human chorionic gonadotropin from the age of 12 years. This allows us to reach puberty. And at the age of 15-16 years are assigned male sex hormones:
Girls in their teens to be appointed within three months of the female sex hormones - estrogen. Then we treat complex estrogen-progestin therapy.
If you make use of counseling hypophyseal syndrome and diet is a good state of patients for a lifetime.