Decreased 3 alpha-androstanediol glucuronide levels in plasma and random urines in male pseudohermaphroditism caused by 5 alpha-reductase deficiency.

Abstract

Concentrations of 3 alpha-diol glucuronide (3 alpha-diol G) in plasma and/or random urine samples were determined in seven subjects with familial male pseudohermaphroditism (FMP) due to 5 alpha-reductase deficiency (5 alpha-RD). All subjects were natives of an isolated Turkish village with a high incidence of consanguineous marriage. A specific and sensitive antibody to 3 alpha-diol was used for radioimmunoassay of 3 alpha-diol G after hydrolysis and chromatographic purification. The mean plasma 3 alpha-diol G in three subjects (31 ng/dL) was much lower than the normal male concentration (516 +/- 50) (+/- SE) and was even lower than normal female values (119 +/- 10.9 ng/dL). In five subjects, mean urinary 3 alpha-diol G in random urine samples was 7.6 (range 2.1 to 12.7) ng/mg creatinine. This was considerably decreased compared with the mean adult male concentration of 65.4 +/- 9.4 and even lower than normal age-matched nonhirsute female values (19.6 +/- 2.1 ng/mg Cr). To validate the use of 3 alpha-diol G/creatinine ratios in random urine samples, correlations of three consecutive eight-hour samples with 24-hour values were determined in 8 male and 3 female age-matched controls. There was an excellent correlation (r = .95) and the linear regression line (y = 0.51x + 2.58) indicates that the 24-hour excretion of 3 alpha-diol G in microgram/24 h is approximately twice the random urinary concentration in ng/mg Cr.(ABSTRACT TRUNCATED AT 250 WORDS)

• Authors: N H Ertel, S Akgun, E Samojlik, M A Kirschner, J Imperato-McGinley
• Journal: Metabolism: clinical and experimental (Metabolism) Vol. 38 Issue 9 Pg. 817-21 (Sep 1989) ISSN: 0026-0495 [Print] United States
• PMID: 2770532 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Androstanols
• Androstane-3,17-diol
• androstane-3,17-diol glucuronide
• 3-Oxo-5-alpha-Steroid 4-Dehydrogenase

Topics

• 3-Oxo-5-alpha-Steroid 4-Dehydrogenase (deficiency)
• Adolescent
• Adult
• Androstane-3,17-diol (analogs & derivatives, blood, metabolism, urine)
• Androstanols (metabolism)
• Disorders of Sex Development (blood, genetics, metabolism, urine)
• Female
• Humans
• Male
• Middle Aged
• Radioimmunoassay
• Reference Values
• Turkey