title: Diagnosis and monitoring of therapy of the various enzymatic defects causing congenital adrenal hyperplasia by semiautomatic capillary gas-liquid chromatography.
abstract: A semiautomatic capillary gas-liquid chromatographic method for the determination of urinary steroids has been developed. Trimethylsilylenol ethers were used as steroid derivatives instead of the more common methoxime trimethylsilyl ethers. The diagnosis of the various enzymatic defects causing congenital adrenal hyperplasia can be made using the characteristic pattern of urinary steroid chromatograms. Furthermore, the method presented can be used routinely to monitor therapeutic control in congenital adrenal hyperplasia. Reference data for patients of different age groups under good therapeutic control are presented.
mesh_terms: D015096:3-Hydroxysteroid Dehydrogenases / Q000172:deficiency; D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis*; D000328:Adult; D001322:Autoanalysis / Q000379:methods; D002648:Child; D002675:Child, Preschool; D002849:Chromatography, Gas / Q000379:methods; D003350:Cortodoxone / Q000031:analogs & derivatives / Q000652:urine; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011279:Pregnanetriol / Q000652:urine; D011283:Pregnenes / Q000652:urine
chemical_list: D011283:Pregnenes; D011279:Pregnanetriol; C009412:tetrahydro-11-deoxycortisol; C026087:5-pregnene-3 beta,17 alpha,20 alpha-triol; D015096:3-Hydroxysteroid Dehydrogenases; D003350:Cortodoxone