Adrenogenital syndrome

Adrenogenital syndrome - a pathological condition caused by congenital virilizing adrenal hyperplasia, accompanied usually lack in the body gluco-corticoid and androgen excess.

Patients with adrenogenital syndrome at birth are usually characterized by large mass and body length, but in the 11 - 12 years of bone growth zones in these children are closed, and patients remain stunted with exaggerated masculine type figure: shoulders much wider pelvis, a large body, the limbs are short. Early, sometimes even in the period from 3 to 7 years, girls show signs virilization: lowered his voice, facial and body hair appear, growing male pattern; boys with adrenogenital syndrome in this age group say the so-called false early puberty. Sometimes patients have periods of sharp increase in body temperature, which explains the release into the blood excess of one of the adrenal androgens - etioholanolona.

The most frequently develops virilnaya form adrenogenital syndrome (90% of patients), in which the adrenal glands produce excessive amounts of androgens and the production of glucocorticoids and mineral ralokortikoidov corresponds to the lower limit of normal. In severe forms of embodiment virilnoy syndrome in a fetus with a genetic female sex under the influence of adrenal androgens incorrectly formed external genitalia, acquire resemblance to the male. Error in determining the sex of children entails serious health and social consequences. In the case of lung flow virilnoy forms syndrome girl born with a well-formed external genitalia, and virilization symptoms occur at puberty.

When solteryayuschey form adrenogenital syndrome produced by excess adrenal androgens combined with significantly reduced formation of glucocorticoids and mineralocorticoids. Clinically solteryayuschaya form syndrome, signs of virilization, as well as symptoms of adrenal insufficiency - nausea, vomiting, diarrhea, decreased blood pressure. In the absence of proper treatment of these children die within the first months of life, with symptoms of adrenal insufficiency are often mistakenly regarded as a manifestation of dyspepsia or pilorospazme.

In hypertensive form of adrenogenital syndrome in an excess of adrenal androgens and synthesize, and mineralocorticoids. This form of syndrome is characterized by a combination of symptoms of virilization with early emerging resistant hypertension, resulting in the absence of proper treatment of cerebrovascular accidents and renal failure.

All forms of adrenogenital syndrome can develop in a child with a male genetic sex. The diagnosis in this case is very complicated. Clinically the disease is characterized by symptoms of premature puberty false: the penis increases in size, appears early in the pubic and body hair in the armpits, but the testicles remain small. Growth of patients remains low. Depending on the shape syndrome clinical picture supplemented or symptoms of adrenal insufficiency, or hypertension, or periodic increase in body temperature.

The diagnosis was based on the results of clinical examination and radiographic examination, determination of sex chromatin, the detection of increased urinary excretion of 17-ketosteroids. All infants with an incorrect structure external genitalia should be shown endocrinologist, if the external genitals resemble the male, but the testes are not detected on 5-8-th day after the birth of the child should be examined by sex chromatin and, if possible, urinary excretion of 17 -ketosteroids. Compliance with these requirements will ensure early treatment of patients with adrenogenital syndrome, which will make possible a harmonious physical and sexual development of the child.

Treatment of all forms syndrome is replacement therapy with glucocorticoids, in the form of additional solteryayuschey appoint mineralocorticoids and sodium chloride in hypertensive form of sodium chloride in the diet restricted. In some cases, produce plastic operations on the external genitals.

When virilnoy form syndrome on the background of hormone replacement therapy is the feminization of the child with a genetic female sex, so if birth sex was defined incorrectly, and restore the true sex, but if due to late treatment to the doctor reconstruction of the true sex is no longer possible, after castration and surgical correction was added to the male sex hormones - androgens.

Forecast solteryayuschey forms of adrenogenital syndrome without treatment is poor hypertensive shape - very serious. With proper treatment, which should be conducted under the supervision of an endocrinologist, patients with adrenogenital syndrome retain ability to work.