Virilism, adrenal

In adrenogenital syndrome and adrenal virilism, an attempt may be made to suppress excess adrenal androgen secretion by inhibiting pituitary corticotropin production by means of prednisolone or dexamethasone. Suppression of androgen production is effective if there is adrenal hyperplasia, but not if an adrenal tumour is present. Hairiness, which women especially dislike in themselves, is often unaffected even though good suppression is achieved, and menstruation recommences. 

J2. Jailer, J. W., Gold, J. J., Vande Wiele, R., and Lieberman, S. 17a-Hydroxy-progesterone and 21-desoxyhydrocortisone their metabolism and possible role in congenital adrenal virilism. J. Clin. Invest. 34, 1639-1646 (1955). 

Cushing s disease and adrenal carcinomas cause adrenal androgen hypersecretion in high enough concentrations to result in signs of androgen excess (such as acne, menstrual irregularities, and hirsutism) and cause virilization in women.4... 

Hypersecretion from the adrenal cortex leads to condition known as Cushing s syndrome which leads to feminism in males, which is the tendency to develop female sex characters and in females virilism develops, which is the tendency to develop male sex characters such as excess growth of hair on chest and pubic region, increase and darkening of facial hair, atrophy of mammary glands (breasts) and cessation of menstrual cycle (amenorrhoea). 

The THEiTHF + 5aTHF ratio is commonly greater than 15 in these disorders, the opposite to AME syndrome. While the ratio of saturated steroids demonstrates a high 11-oxo l 1/1-hydroxy ratio, this is not the case for steroids retaining a - -4-ene structure. The F E ratio is normal or elevated (i.e., > 1 Table 5.3.9). Steroid profile analysis also typically reveals an elevated excretion of DHEA and other androgen metabolites. Clearly the excessive ACTH production resultant from an apparent cortisol deficiency is responsible for the elevated adrenal androgen production, which in turn is responsible for female virilization and other manifestations of polycystic ovary syndrome. 

A similar conclusion has been drawn by New and Levine [53] and may help to explain the known clinical features of the 21-hydroxylase defect of congenital adrenal hyperplasia, i.e., the existence of the simple virilizing form and the salt-losing type. It has been suggested that the 21-hydroxylase activity is impaired in the ZF for both 17-hydroxy- and 17-deoxycorticosteroid pathways, so that 11-deoxycorti-sol levels (and also cortisol levels) are decreased (Fig. 7), and the build-up of excess... 

Fig. 7. Adrenal steroidogenesis in the simple virilizing and salt-wasting forms of congenital adrenal hyperplasia (from Ref. 53, with permission).

G-12) (G-13) 21-bydroxylase deficiency. This is the most common hereditary enzyme defect in steroid biosynthesis. There is decreased glucocorticoid and mineralocorticoid production, as the enzyme is common to both pathways. This leads to increased ACTH production, which in turn causes adrenal hyperplasia and increased pregnenolone production. This results in increased androgen production and virilization. Therapy consists in administering glucocorticoids. This decreases ACTH production by negative feedback. 

Apart from unrecognized Addison s disease, previous ablative adrenal surgery and virilizing hyperplasia are causes of primary adrenal failure. Hypopituitarism and previous hypophysectomy are examples of secondary adrenal failure, but secondary adrenal unresponsiveness due to prior treatment with corticosteroids is much more common and is liable to prevent patients from responding normally to even minor degrees of stress (J5). 

Patients with virilizing hyperplasia of the adrenal have a relative cortisol deficiency due to a block in normal synthesis, and this deficiency may be detected only during the demands of a surgical procedure. 

A deficiency of ll -hydroxylase is the second most common form of CAH, with an incidence of 1 per 100,000 births, and is associated with manifestations of virilization, elevated concentrations of plasma androstenedione and DHEA-S, and hypertension. The mineralocorticoid-induced hypertension is caused by an elevation of DOC 11-deoxycortisol concentrations are markedly raised in subjects with this enzyme defect,The major distinguishing characteristic of this disorder from 21-hydroxylase deficiency, besides the elevated plasma concentrations of 11-deoxycortisol, is hypertension elicited by the salt retention caused by increased concentrations of DOC, A deficiency of 3 -hydroxysteroid dehydrogenase-isomerase has been reported and leads to an elevation in the ratio of 17a-hydroxypregnenolone to that of 17a-hydroxyprogesterone and to an increased ratio of DHEA to androstenedione. In severe forms of this rare disorder, female infants have pseudohermaphroditism, and male infants present with incomplete masculinization. Patients with this disorder usually present in early infancy with complete adrenal insufficiency including salt wasting. A late-onset form has also been reported in patients with premature pubarche with hirsutism, acne, and menstrual irregularities. The... 

Nonfunctioning Adrenocortical Tumors Approximately 2% of the population have an adrenal tumor most of these tumors are nonfunctioning and are sometimes called incidentalomas. They are found when CT scans of the abdomen are performed that can easily detect tumors 1 cm in diameter or 5 g in weight. No virilizing tumors smaller than 1 cm in diameter have been reported. Carcinomas are usually more than 30 g in weight. 

Late-onset adrenal hyperplasia Cushing s syndrome Virilizing adrenal tumors Adrenocorticoid insufficiency Thyroid disorders Hypothyroidism Hyperthyroidism Pituitary disorders... 

Virilization is characterized by clitoral hypertrophy, deepening of the voice, temporal hair recession, baldness, increased libido, decreased body fat, and menstrual irregularities or amenorrhea. Hirsutism is usually associated with normal or slightly elevated serum androgens, whereas virilization is associated with marked increases in ovarian or adrenal androgen production. ... 

T is most commonly measured in serum, and it is one of the hormones most commonly measured in serum, T circulates in plasma non-specifically hound to albumin, specifically bound to sex hormone binding globulin and unbound (free). Clinicians use serum T measurements to diagnose and monitor various disorders such as hypogonadism (androgen deficiency), testicular dysfunction, hirsutism, virilization, alopecia, prostate disease, adrenal hyperplasia and aging, in addition to anomalies caused by exposure to chemicals or hormone disrupters. 

Virilism results from the excessive secretion of androgens from the adrenal gland and is usually seen as hirsutism in... 

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