Adrenogenital syndrome - a hereditary disease, transmitted in an autosomal recessive manner.
There are three basic forms of the disease:
virilnuyu (simple), associated with a mild 21-hydroxylase deficiency
form with the loss of salt - develops in a deep 21-hydroxylase deficiency
hypertension - due to lack of 11 - beta - hydroxylase.
When virilnoy form girls have the phenomenon of intrauterine virilization: enlarged clitoris like penis, labia take the form of a split scrotum. There is a common urogenital opening - false female hermaphroditism. In 2-3 years these girls is a premature sexual development on a false heterosexual type, accelerated growth, masculinization, early closure of the epiphyseal lines and short.
Boys have a false premature sexual development is on izoseksualnomu type. Other symptoms are the same as in girls. In stressful situations, these patients may develop symptoms of adrenal insufficiency.
When the form c loss of salt from the first days of life, not later than the third week, symptoms of salt loss: poor weight gain, vomiting, drowsiness, tachycardia, impaired ability to drink, dehydration. Girls - hermaphrodite genitalia in boys sometimes penis enlargement.
In hypertensive form, except for virilization symptoms are cardiovascular disorders caused by prolonged hypertension. At an early age may be hypertensive crises with hemorrhage into the brain. Forecast Prospect form of serious hypertension, with virilnoy form and shape with the loss of salt in proper treatment, prognosis is favorable. In all forms of adrenogenital syndrome spend replacement therapy with glucocorticoids overwhelming, moreover, the form of loss of salt and form prescribed virilnoy mineralocorticoids.
