Adrenal hyperplasia testosterone

Several en2yme defects will cause a deficiency of testosterone production during fetal development. These include 20a-hydroxylase (cholesterol 20,22-desmolase) deficiency, which prevents conversion of cholesterol to pregnenolone and results in congenital lipoid adrenal hyperplasia, so named because the adrenals are large and filled with lipoid... 

Congenital adrenal hyperplasia 21-Hydroxylase with and without salt loss High 17-08 excretion High 3/S-hydroxy-A steroid excretion (infants) High pregnanetriol excretion High testosterone excretion -1- Normal or low Normal or low... 

D5. Degenhart, H. J., Visser, H. K. A., Wilmink, R., and Frankena, L., Production and excretion of testosterone in children with congenital adrenal hyperplasia and precocious puberty. Acta Endocrinol. Suppl. 100, 51 (1965). 

F. 34.23. Synthesis of the steroid hormones. The rings of the precursor, cholesterol, are lettered. Dihydrotestosterone is produced from testosterone by reduction of the carbon-carbon double bond in ring A. Structural changes between the precursor and final hormone are noted in blue. DHEA = dehydroepiandrosterone. The dashed lines indicate alternative pathways to the major pathways indicated. The starred enzymes are those that may be defective in the condition congenital adrenal hyperplasia. 

Side chain splitting in the absence of or after hydrox-ylation in position 11 results in an overall increase in 17-ketosteroids, some of which are biologically active (androstenedione, 11 jS-hydroxyandrostene-dione, dehydroepiandrosterone) and are responsible for the virilizing symptoms. Whether or not testosterone is formed in excess in patients with the adrenogenital syndrome is not certain. High levels of testosterone glucuronide have been found in some patients with adrenal hyperplasia. 

Hypergonadism, This can result from congenital adrenal hyperplasia or an interstitial cell tumour of the testis. Plasma testosterone and urinary 17-oxosteroid excretion are raised in both conditions. 

The first step in the catabolism of testosterone according to Fig. 10 should be the oxidation of the 17/8-hydroxyl group to the ketone to form A -androstene-3,17-dione. Although this substance has been found in the urine of a man with adrenal hyperplasia, normally the turnover of this compound must be very rapid, since it is not present in normal urine and is not found even after the administration of large amounts of testosterone. However, the administration of isotopic, as well as nonisotopic, A -androstene-3,17-dione gave rise to the same metabolites (androsterone, XLVIII, and etiocholanolone, XLIX) as testosterone therefore, A -androstene-3,17-dione could be an intermediate in the biochemical transformation of the testicular hormone. 

Urinary glucuronides of testosterone and dihydrotestosterone from patients with benign prostate hyperplasia were analysed using on-line SPE-LC coupled to ion-trap MS operated in negative-ion ESI-MS [72]. Because DHEA, excreted by the adrenal gland, is converted into testosterone and subsequently into dihydrotestosterone, it can be involved in the growth of prostate cancer. In order to study this in more detail, an LC-MS method was developed for the simultaneous determination of... 

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