Adrenogenital syndrome. Differentiated approach to treatment selection

Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder leading to impaired corticosteroid synthesis due to the deficiency of one of the steroidogenic enzymes. The most common mutation in the CYP21A gene is 21-hydroxylase deficiency. Insufficient activity of this enzyme results in excessive synthesis of androgens. The main problem is the selection of glucocorticoid replacement therapy to maintain eucorticism and suppression of adrenocorticotropic hormone (ACTH) levels to eliminate hyperandrogenism. However, it is not always possible to select an adequate dose of glucocorticoids, leaving the patient in either a state of hyperandrogenism or iatrogenic Cushing’s syndrome. In women, hyperandrogenism often leads to amenorrhea and anovulatory infertility. In severe cases, when no effect can be achieved with conservative treatment in adult patients, bilateral adrenalectomy is considered. Like any surgical intervention, in addition to certain advantages, it entails a high risk of acute adrenal insufficiency, hypoglycemic states, and late complications in the form of testicular tumors and ACTH-secreting pituitary adenomas. This article presents a description of two clinical cases of patients with virilizing and salt-wasting forms of CAH. The aim of these cases was to draw the attention of practitioners to the necessity of individual assessment of potential benefits and harms of bilateral adrenalectomy, as well as to emphasize the feasibility of adrenalectomy in benign nodular adrenal hyperplasia. Key words: congenital adrenal hyperplasia, salt-wasting form, virilizing form, 21-hydroxylase deficiency, bilateral adrenalectomy, clinical case