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J Pediatr. 2014 Aug;165(2):280-4. doi: 10.1016/j.jpeds.2014.04.011. Epub 2014 May 23.

Reduced activity of 11β-hydroxylase accounts for elevated 17α-hydroxyprogesterone in preterms.

Author information

1
Division of Pediatric Endocrinology and Diabetology, Steroid Research and Mass Spectrometry Unit, Center of Child and Adolescent Medicine, Justus Liebig University, Giessen, Germany. Electronic address: Clemens.kamrath@uni-giessen.de.
2
Division of Pediatric Endocrinology and Diabetology, Steroid Research and Mass Spectrometry Unit, Center of Child and Adolescent Medicine, Justus Liebig University, Giessen, Germany.

Abstract

OBJECTIVE:

To characterize the urinary steroid metabolome of neonates and infants born either at term or preterm.

STUDY DESIGN:

We retrospectively analyzed urinary steroid hormone metabolites determined by gas chromatography-mass spectrometry of 78 neonates and infants born at term and 83 neonates and infants born preterm (median 34 weeks of gestational age). The subjects' 11β-hydroxylase and 21-hydroxylase activities were assessed on the basis of urinary metabolite substrate-to-product ratios.

RESULTS:

Preterm neonates and infants had elevated urinary concentrations of 17α-hydroxyprogesterone (17OHP) metabolites (P<.001) but lower urinary concentrations of the 21-deoxycortisol metabolite pregnanetriolone (PTO) (P<.01). One reason was lower 11β-hydroxylase activity in preterms. We could demonstrate a correlation between low 11β-hydroxylase activity and high urinary concentrations of 17OHP metabolites (r=0.51, P<.001) but low urinary concentrations of the 21-deoxycortisol metabolite PTO (r=-0.24, P=.03) in preterms.

CONCLUSIONS:

Low 11β-hydroxylase activity may explain increased 17OHP but decreased 21-deoxycortisol metabolite excretion in preterms. Our analysis clarifies, first, why preterms have higher 17OHP levels and thus higher rates of false-positive screening results for congenital adrenal hyperplasia than do term infants, and, second, why 21-deoxycortisol or its urinary metabolite PTO is more specific than 17OHP for the diagnosis of 21-hydroxylase deficiency.

PMID:
24862381
DOI:
10.1016/j.jpeds.2014.04.011
[Indexed for MEDLINE]

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