Adrenal androgens, production

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. 

Reduced sperm count and motility and inhibition of the secretory function of the testicles during glucocorticoid treatment have been reported and discussed in relation to the suppression of adrenal androgen production. These reports still await confirmation. 

Surgery was subsequently performed to correct the labial fusion and clitoral hypertrophy. The child has developed normally and is being closely monitored for hormone replacement dose requirements to avoid either iatrogenic Cushing s syndrome from excess glucocorticoid or premature epiphyseal closure and short stature from unsuppressed adrenal androgen production. 

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. ... 

Measurements of DHEA or its sulfated conjugate, DHEA-S, in serum and plasma are important to investigations of adrenal androgen production, such as the assessment of hyperplasia, adrenal tumors, adrenarche, delayed puberty, or hirsutism, DHEA-S in circulation originates primarily from the adrenal glands, although in men some may be derived from the testes none is produced by the ovaries. DHEA is secreted almost entirely by the adrenal glands. 

The bulk of the urinary 17-KSs consists of andro-sterone, epiandrosterone, etiocholanolone, DHEA, 11-keto-and lip-hydroxyandrosterone, and 11-keto- and 11(3-hydroxyetiocholanolone. DHEA and 11-oxygenated 17-KSs are produced only by the adrenal glands, whereas the others also arise from precursors (androstenedione and testosterone) elaborated by the gonads. Thus the main purpose of measuring these steroid metabolites is to assess adrenal androgen production. 

Rainey, W. E., Carr, B. R., Sasano, H., Suzuki, T., and Mason, J. I. (2002). Dissecting human adrenal androgen production. Trends Endocrinol Metab 13, 234-239. 

Because Vera s symptoms began in adult life, her genetically determined adrenal hyperplasia is referred to as a nonclassic or atypical form of the disorder. A more severe enzyme deficiency leads to the classic disease, which is associated with excessive fetal adrenal androgen production in utero and, therefore, manifests itself at birth, often with ambiguous external genitalia and virihzing features in the female neonate. 

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