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The urinary steroidome of treated children with classic 21-hydroxylase deficiency
- Source :
- The Journal of steroid biochemistry and molecular biology. 165
- Publication Year :
- 2016
-
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-OHD) on hydrocortisone and fludrocortisone treatment. Daily urinary excretion of glucocorticoid-, 17α-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. 11β-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 11β-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.
- Subjects :
- 0301 basic medicine
Male
endocrine system diseases
Hydrocortisone
Endocrinology, Diabetes and Metabolism
Clinical Biochemistry
urologic and male genital diseases
Biochemistry
Cohort Studies
0302 clinical medicine
Endocrinology
Reference Values
Child
biology
Chemistry
21-Hydroxylase
Child, Preschool
Fludrocortisone
Androgens
Molecular Medicine
Female
Steroids
Glucocorticoid
medicine.drug
medicine.medical_specialty
Adolescent
medicine.drug_class
Urinary system
030209 endocrinology & metabolism
Urinalysis
Androsterone
Gas Chromatography-Mass Spectrometry
03 medical and health sciences
Internal medicine
medicine
Humans
Congenital adrenal hyperplasia
Adrenarche
Molecular Biology
Glucocorticoids
Retrospective Studies
Adrenal Hyperplasia, Congenital
Body Weight
Cell Biology
Androgen
medicine.disease
Body Height
030104 developmental biology
biology.protein
Steroid 21-Hydroxylase
Subjects
Details
- ISSN :
- 18791220
- Volume :
- 165
- Database :
- OpenAIRE
- Journal :
- The Journal of steroid biochemistry and molecular biology
- Accession number :
- edsair.doi.dedup.....7c676c4d02392e87aa270ffbb551508c