Journalartikel

Jahr der Veröffentlichung: 2017

Seiten: 396-406

Zeitschrift: The Journal of Steroid Biochemistry and Molecular Biology

Bandnummer: 165

ISSN: 0960-0760

eISSN: 1879-1220

DOI Link: https://doi.org/10.1016/j.jsbmb.2016.08.006

Verlag: Elsevier

Abstract: 

Monitoring treatment of children with classic congenital adrenal hyperplasia (CAH) is difficult and biochemical targets are not well defined.

We retrospectively analysed 576 daily urinary steroid hormone metabolite profiles determined by gas chromatography-mass spectrometry of 150 children aged 3.0-17.9 years with classic 21-hydroxylase deficiency (21-0HD) on hydrocortisone and fludrocortisone treatment.

Daily urinary excretion of glucocorticoid-, 17 alpha-hydroxyprogesterone (17-OHP)-, and androgen metabolites as well as growth and weight gain are presented. Children with classic CAH exhibited increased height velocity during prepubertal age, which was then followed by diminished growth velocity during pubertal age until final height was reached. Final height was clearly below the population mean. 11 beta-Hydroxyandrosterone was the dominant urinary adrenal-derived androgen metabolite in CAH children. Adrenarche is blunted in children with CAH under hydrocortisone treatment and androgen metabolites except 11 beta-hydroxyandrosterone were suppressed. Cortisol metabolite excretion reflected supraphysiological hydrocortisone treatment dosage, which resulted in higher body-mass-indices in children with CAH.

Reference values of daily urinary steroid metabolite excretions of treated children with CAH allow the clinician to adequately classify the individual patient regarding the androgen-, 17-OHP-, and glucocorticoid status in the context of the underlying disorder. Additionally, urinary 21-OHD-specific reference ranges will be important for research studies in children with CAH. (C) 2016 Elsevier Ltd. All rights reserved.

Harvard-Zitierstil: Kamrath, C., Wettstaedt, L., Boettcher, C., Hartmann, M. and Wudy, S. (2017) The urinary steroidome of treated children with classic 21-hydroxylase deficiency, The Journal of Steroid Biochemistry and Molecular Biology, 165, pp. 396-406. https://doi.org/10.1016/j.jsbmb.2016.08.006

APA-Zitierstil: Kamrath, C., Wettstaedt, L., Boettcher, C., Hartmann, M., & Wudy, S. (2017). The urinary steroidome of treated children with classic 21-hydroxylase deficiency. The Journal of Steroid Biochemistry and Molecular Biology. 165, 396-406. https://doi.org/10.1016/j.jsbmb.2016.08.006