(Congenital adrenal hyperplasia)
Adrenal Virilism is a syndrome in which the increased release of adrenal androgens causes virilization. The diagnosis is made clinically and confirmed by elevated androgen levels with and without Supprimierbarkeit by dexamethasone. To determine the cause of it requires the use of an imaging method of the adrenal glands. Treatment depends on the cause.
(See illustration of the adrenal function.)
Adrenal Virilism is a syndrome in which the increased release of adrenal androgens causes virilization. The diagnosis is made clinically and confirmed by elevated androgen levels with and without Supprimierbarkeit by dexamethasone. To determine the cause of it requires the use of an imaging method of the adrenal glands. Treatment depends on the cause. (See illustration of the adrenal function.) Adrenal Virilism caused by Androgenproduzierender tumor adrenal hyperplasia malignant adrenal tumors can secrete (or all three) excessive androgens, cortisol or mineralocorticoids, resulting in Cushing’s syndrome with suppression of adrenocorticotropic hormone (ACTH) secretion and atrophy of the contralateral adrenal gland leads. A hyperplasia of the adrenal glands is normally inherited; The adrenal hyperplasia, delayed virilization is a variant of congenital adrenal hyperplasia. Both are caused by a Hydroxylierungsdefekt of Kortisolvorläufern; The Kortisolvorläufer accumulate and be as it were redirected to androgen synthesis. The defect is incomplete, so it may come only in young adulthood to clinical symptoms in the adrenal hyperplasia virilization delayed. Symptoms and signs Symptoms vary depending on the patient’s sex and age at onset and can be seen more clearly in women than in men. In female infants with congenital adrenal hyperplasia may lead to a merger of labioskrotalen fold and a hypertrophy of the clitoris, which is similar to the male external genitalia and thus presents itself as a disorder of sexual differentiation. In prepubertal children, growth may accelerate. Without treatment it comes to premature epiphyseal closure with short stature. Ill prepubertal boys show premature sexual maturation. Adult female patients may show amenorrhea, uterine atrophy, clitoral hypertrophy, reduced breast size, acne, hirsutism, voice deepening, alopecia, increased libido and increase muscle mass. In adult men, excessive Androgen can suppress gonadal function and cause infertility. Ectopic adrenal tissue in the testes can stimulate tumor growth and enlarge them. Other diagnostic testosterone adrenal androgens (dehydroepiandrosterone [DHEAS] androstenedione) dexamethasone suppression clinically found Imaging method on the adrenal 17-hydroxyprogesterone A suspected diagnosis of adrenal virilism is in the first place. However, also occur in polycystic ovary syndrome (Stein-Leventhal syndrome); a low-grade hirsutism, a Virilism with hypomenorrhea and increased plasma testosterone values. Adrenal virilism is confirmed by the detection of elevated levels of adrenal androgens. In the adrenal hyperplasia, the values ??for dehydroepiandrosterone (DHEA) and its sulfate are (DHEAS) increased urinary excretion of pregnanetriol is often also increased, and the free cortisol in urine is normal or reduced. Plasma levels of DHEA, DHEAS, 17-hydroxyprogesterone, testosterone and androstenedione may be increased. A 17-hydroxyprogesterone levels> 30 nmol / L (1,000 ng / dL) 30 minutes after the i.m. Administration of 0.25 mg Cosyntropin (synthetic ACTH) makes the diagnosis of the most common form of adrenal hyperplasia very likely. A virilizing tumor can then be excluded if the excessive androgen production by the p.o. administration of 0.5 mg dexamethasone every 6 can be suppressed h 48 h. If the excessive androgen production is not suppressed, a search tumor using a CT or MRI of the adrenals and an ultrasound of the ovaries should be performed. Therapy to be administered orally at glucocorticoids hyperplasia removal of tumors, glucocorticoids are used at a adrenal hyperplasia, generally oral hydrocortisone 10 mg after rising, at noon and 5 mg 5 mg in the afternoon. Alternatively, 0.5-1 mg dexamethasone p.o. be given at night, but even this low doses can cause symptoms of Cushing’s syndrome. Most effective in suppressing ACTH secretion is to administer the dose at bedtime, but this can cause insomnia. may also be used cortisol 25 mg of 1-times daily or prednisone 5-10 mg of 1-times daily. Although most symptoms of Virilism regress of hirsutism and baldness may not disappear slow and deep voice. Fertility can continue to be limited. Tumors require adrenalectomy. In patients with cortisol-secreting tumors preoperatively hydrocortisone should be administered because the contralateral adrenal cortex may be atrophic and suppressed. Conclusion Adrenal Virilism arises because of an androgen-secreting tumor of the adrenal glands or because of hyperplasia. Virilization does become more noticeable in women, men can be infertile due to suppressed gonadal function. Dehydroepiandrosterone in urine and plasma (DHEA) and its sulfate (DHEAS) and often Plasma testosterone are elevated ACTH Stimulationstest- and / or dexamethasone suppression tests can be performed. Hyperplasia is treated with dexamethasone; Tumors require adrenalectomy.