Adrenogenital syndrome in children
Adrenogenital syndrome is characterized by abnormalities in the functioning and structure of the genital organs. The exact etiology is not installed. But it is considered that the deviation occurs as a result of excessive production of androgens by the adrenal cortex. Pathology can be caused by tumors (adenocarcinoma, adenoma) or congenital hyperplasia of these glands. The hereditary factor is also given considerable value.
Adrenogenital syndrome: symptoms
Pathology occurs in both boys and girls. Her symptoms depend on the time of onset and sex of the sick child. It also affects which form of adrenogenital syndrome takes place. And they are distinguished by three: simple (viril), hypertensive and solter. Each of them manifests itself either in the postnatal or in the prepubertal period. Adrenogenital syndrome in girls is expressed in the viril form immediately at birth. This is manifested in a false hermaphroditism. The clitoris is too enlarged and the opening of the urethra opens right at its base. Big labia resemble a split scrotum.The changes are sometimes pronounced so dramatically that it is difficult to determine the sex of the child. At the age of three to six years, the hair on the back, legs and pubic grows intensely, physical development is enhanced. The girl becomes like a boy and looks. Soon its sexual development ceases completely. The mammary glands are small. Menstruation in girls is either absent, or very scarce, irregular. Adrenogenital syndrome in boys develops from two to three years of age. Together with enhanced physical development, the penis increases, excessive hair growth is observed, sometimes erections appear. But the testicles with all this are infantile and in the future do not develop at all. Both girls and boys grow intensively, but despite this they remain low and stocky, since growth zones in the pituitary gland close early.
Adrenogenital syndrome: diagnosis
It is not so hard to install. It is confirmed by hirsutism, the anomalous structure of the external genital organs, which is not age-appropriate physical development. Urine has a high level of 17-ketosteroids. Difficulties arise, as a rule, not in diagnosis, but in clarifying the cause of this syndrome.Anomalies in sex discrimination and indications of similar abnormalities in previous generations indicate congenital hyperplasia. If virilization has developed after the birth of a child, then the presence of a tumor can be ascertained using computed tomography. You also need to carefully differentiate congenital adrenogenital syndrome with premature sexual development, having cerebral-pituitary origin. In the latter case, testicular biopsy reveals the presence of deferent tubules with the active formation (under the influence of hormones) of male germ cells. In girls, too, premature development is possible. It often occurs in granulocystic ovarian tumors. In this pathology, premature development is of the female type, and in case of adrenogenital syndrome, it is of the male type. This is the main difference between them.
Adrenogenital syndrome: treatment
If the pathology arose against the background of congenital adrenal hyperplasia, corticosteroid drugs are prescribed. Their constant minimum dose does not adversely affect. But at the same time they are well suppress the function of the glands.Begin treatment with large doses, which support from ten days to a month. Longer use can adversely affect the growth processes of the baby. After reaching the effect of the dose is reduced to supporting, focusing on the level of 17-ketosteroids in the urine. The treatment is carried out under strict medical supervision.