Adrenogenital syndrome

Adrenogenital syndrome (adrenal virilism) is a syndrome in which an excessive amount of adrenal androgen causes virilization.

The diagnosis is clinical, confirmed by elevated levels of androgens with and without suppression of dexamethasone; To determine the cause of the cause, it may be necessary to visualize the adrenal glands with a biopsy when revealing volumetric formation. Treatment of adrenogenital syndrome depends on the cause.

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Causes of the adrenogenital syndrome

The cause of adrenogenital syndrome may be an androgen-secreting tumor of the adrenal glands or adrenal hyperplasia. Sometimes a tumor secretes an excess of androgens and cortisol, which leads to the development of Cushing's syndrome with suppression of ACTH secretion and atrophy of the contralateral adrenal gland.

Hyperplasia of the adrenal glands is usually congenital; late virilizing adrenal hyperplasia is a congenital variant. Both are caused by a violation of hydroxylation of cortisol precursors, whereby they are accumulated and used for the production of androgens. With late virilizing hyperplasia, the defect is partial, so clinically the disease may not appear until adulthood.

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Symptoms of the adrenogenital syndrome

Effects depend on the sex and age of the patient at the time of onset of the disease, more visible in women. Symptoms and symptoms include hirsutism (mild cases may be the only sign), baldness, acne, voice change (coarsening). Perhaps increased libido. Children in prepubertate can accelerate growth. Boys in prepubertate have early sexual maturation. Girls can develop amenorrhea, atrophy of the uterus, clitoral hypertrophy, breast reduction, masculinization.

In adult men, an excess of androgen adrenal glands can suppress the function of the sex glands and cause infertility. Ectopic adrenal tissue in the testicles can increase and simulate the tumor.

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Diagnostics of the adrenogenital syndrome

Adrenogenital syndrome is suspected on the basis of clinical data, although moderate hirsutism and virilization with hypomenorrhea and elevated plasma testosterone level can also be observed in polycystic ovary syndrome (Stein-Leventhal). The diagnosis of adrenal virilization is confirmed by an increased level of androgens of the adrenal glands. Adrenal hyperplasia in the urine increased dehydroepiandrosterone (DHEA) and its sulfate (DHEAS), the excretion of pregnanthriol is often increased, the level of free cortisol is lowered. Levels of DHEA, DHEAS, 17 hydroxyprogesterone, testosterone and androstenedione in plasma can be increased. The level of 17 hydroxyprogesterone is more than 30 nmol / L 30 minutes after the administration of 0.25 mg of ACTG cozintropine is intramuscularly characteristic for the most common form of adrenal hyperplasia.

Virilizing tumors are excluded if dexamethasone at a dose of 0.5 mg orally every 6 hours for 48 hours suppresses excessive production of androgens. If suppression does not occur, CT and MRI of the adrenal glands and ultrasound of the ovaries should be performed to find the tumor.

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Treatment of the adrenogenital syndrome

The recommended treatment for adrenal hyperplasia is the administration of dexamethasone by mouth at 0.5-1 mg at bedtime, but even at such small doses, signs of Cushing's syndrome may develop. Alternatively, you can use cortisone (25 mg once a day) or prednisolone (5-10 mg once a day). Although adrenogenital syndrome and its symptoms disappear, hirsutism and alopecia are slow, the voice may remain rough, fertility may be compromised.

When tumors, adrenalectomy is necessary. Patients with cortisol-secreting tumors need the appointment of hydrocortisone before and after the surgery, since non-humoral areas of the cortex will be atrophied and suppressed.