HETEROSEXUAL PREMATURE SEXUAL DEVELOPMENT

HETEROSEXUAL PREMATURE SEXUAL DEVELOPMENT

Name appearance of signs of the sexual ripening of opposite (masculine) sex heterosexual premature sexual development for girls on the first decade of life.

Adrenogenital syndrome

Most frequent clinical form of heterosexual premature sexual development.

An adrenogenital syndrome is born hyperplasia of adrenal cortex, erroneous womanish hermaphroditism or premature sexual development of girls on a heterosexual type. An adrenogenital syndrome is investigation of a born deficit of the enzymic systems which take part in the synthesis of steroid hormones of adrenal glands.

An adrenogenital syndrome is a genetic defect with the recession way of inheritance, the transmitters of imperfect gene can be and men, and women.

Hyperproducts of androgens in the adrenal cortex at a born adrenogenital syndrome are investigation of mutation of gene and born genetically conditioned deficit of the enzymic system. The synthesis of cortisole is violated - basic glucocorticoid hormone of adrenal cortex, formation of which diminishes.

Depending on character of deficit of the enzymic systems an adrenogenital syndrome divides in 3 forms, a general symptom for them is virilisation.

· An adrenogenital syndrome is with the syndrome of loss of salt: the deficit of 3-β-dehydrogenasa results in the sharp diminishing of formation of cortisole, frequent vomit develops as a result, dehydration of organism with violation of cardiac activity. The symptoms of adrenogenital syndrome with the loss of salt appear at first time after birth.

· An adrenogenital syndrome with hypertension: the deficit of 11-hydroxylasa results in the accumulation of corticosterone hereupon to development of hypertension on a background violation of water and electrolyte exchange. The symptoms of adrenogenital syndrome with hypertension develop in the first decade of life. An adrenogenital syndrome with the loss of salt and hypertension meets rarely. Both these forms violate not only sexual development but also function of cardiac, digestive and other systems of organism. These patients make a general contingent for endocrinologies and paediatricians.

· adrenogenital syndrome, simple virilizing form: the deficit of С-21- hydroxylasa draws multiplying formation of androgens and development of symptoms of hyperandrogenia without the substantial decline of synthesis of cortisole. This form of adrenogenital syndrome is most frequent, not accompanied somatic violations of development. The deficit of С-21- of hydroxylasa, without regard to a born character, can appear in different periods of life; depending on it select born, pubertal, post-pubertal forms.

Diagnostics

An objective diagnostic method is ultrasonic research of adrenal glands and magnetically-resonancetomography of adrenal glands. By an informing test for diagnostics maintenance of 17-ketosteroids (17-КС) and dehydroepiandrosterone is sharply promoted in urine and/or testosterone in blood, which are normalized after conducting of test with glucocorticoid preparations.

Treatment

Treatment of a born adrenogenital syndrome consists in application of glucocorticoid preparations. A dose depends on age, mass of body of child and degree of hyperandrogenia, which expressly correlates with the level of testosterone in blood or 17-КС and dehydroepiandrosterone in urine. Therapy is conducted lasted, because the deficit of the enzymic system has a born character. Abolition of treatment results in getting up of level of andogens in blood and to returning of all signs of virilization.