Analysis of glucocorticoid metabolites in the neonatal period: catabolism of cortisone acetate by an infant with 21-hydroxylase deficiency.

Abstract

The urinary excretion of 27 individual 17-hydroxycorticosteroids by a female infant with 21-hydroxylase deficiency was studied using gas chromatography on an open-tubular column and combined gas chromatography-mass spectrometry. Samples were analysed from days 1, 3 and 4 of life, before cortisone acetate treatment commenced on day 6, and on days 9 and 192, during treatment. The pattern of metabolites before and after treatment showed similarities with extensive reductions at positions 3 and 20 of the steroid nucleus and hydroxylations at positions 1 and 6. The following new glucocorticoid metabolites were identified: 1beta,3alpha,17alpha,21-tetrahydroxy-5alpha(andbeta)-pregnane-11,20-dione; 3alpha,6xi,17alpha,21-tetrahydroxy-5xi-pregnane-11,20-dione (2 epimers); 1beta,3alpha,17alpha,20alpha(andbeta),21-pentahydroxy-5beta-pregnan-11-one, and 3alpha,6xi,17alpha,20alpha (andbeta),21-pentahydroxy-5beta-pregnan-11-one. These steroids comprised 50% of the total metabolites on day 9, but only 20% on day 192. They have also been detected in the urine of normal neonates.

• Authors: N F Taylor, D H Curnow, C H Shackleton
• Journal: Clinica chimica acta; international journal of clinical chemistry (Clin Chim Acta) Vol. 85 Issue 3 Pg. 219-29 (May 02 1978) ISSN: 0009-8981 [Print] Netherlands
• PMID: 306899 (Publication Type: Case Reports, Journal Article)

Chemical References

• 17-Hydroxycorticosteroids
• Steroid Hydroxylases
• Cortisone

Topics

• 17-Hydroxycorticosteroids (urine)
• Adrenal Hyperplasia, Congenital
• Chromatography, Gas
• Cortisone (analogs & derivatives, metabolism, therapeutic use)
• Female
• Humans
• Infant, Newborn
• Mass Spectrometry
• Steroid Hydroxylases (deficiency)