Salivary morning androstenedione and 17α-OH progesterone levels in childhood and puberty in patients with classic congenital adrenal hyperplasia
Open accessAbstract
BACKGROUND: Treatment of congenital adrenal hyperplasia due to 21-hydroxylase deficiency can be monitored by salivary androstenedione (A-dione) and 17α-hydroxyprogesterone (17OHP) levels. There are no objective criteria for setting relevant target values or data on changes of 17OHP and A-dione during monitoring. METHODS: We evaluated A-dione and 17OHP levels in nearly 2000 salivary samples collected during long-term treatment of 84 paediatric patients with classic 21-hydroxylase deficiency. RESULTS: A-dione and 17OHP levels and its ratio 17OHP/A-dione remained constant from 4 to 11 years with no sex-related differences. During puberty, A-dione and 17OHP levels both increased, starting at earlier age in girls than in boys. The ratio 17OHP/A-dione declined. Normalised A-dione concomitant with elevated 17OHP [1.43 nmol/L (0.46-4.41) during prepuberty; 2.36 nmol/L (0.63-8.89) for boys and 1.99 nmol/L (0.32-6.98) for girls during puberty] could be obtained with overall median glucocorticoid doses of 11-15 mg/m2/day. A-dione levels above the upper reference limit (URL), suggesting undertreatment, coincided with 17OHP levels ≥10 times URL. The percentage of A-dione levels above URL was 16% at ages 4-8 years, but increased to 31% for girls at 16 years and 46% for boys at 17 years. CONCLUSIONS: Normalised A-dione consistent with 17OHP three times URL during prepuberty and normalised A-dione consistent with 4-6 times URL during puberty could be obtained by moderate glucocorticoid dosages. A constant 17OHP/A-dione ratio during prepuberty suggested absence of adrenarche. During puberty, a higher percentage of samples met the criteria for undertreatment, especially of boys.
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Current insights into monitoring of congenital adrenal hyperplasia
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- Authors
- de Groot, M. J. M., Pijnenburg-Kleizen, K. J., Thomas, C. M. G., Sweep, F. C. G. J., Stikkelbroeck, N. M. M. L., Otten, B. J., & Claahsen-van der Grinten, H. L.
- Affiliations
- Radboud University Medical Center (NL)
- Venue
- Clinical Chemistry and Laboratory Medicine (CCLM)
- Publisher
- Walter de Gruyter GmbH
- Published
- 2015-01-01
- Volume
- 53
- Issue
- 3
- Pages
- 461-8
- ISSN
- 1434-6621 (ISSN-L)
- 1437-4331
- OpenAlex
- W1995260983
- Cited by
- 18
- References
- 13
- Citations by year
- 2026: 1 · 2023: 3 · 2022: 3 · 2021: 3 · 2019: 4 · 2018: 3 · 2017: 1
- FWCI
- 0.14
- Type
- journal article
- Language
- en
- Open access
- green
- hdl.handle.net
- Locations
- Clinical Chemistry and Laboratory Medicine (CCLM) (publishedVersion)
- PubMed (publishedVersion)
- Radboud Repository (Radboud University) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sexual Differentiation and Disorders
- Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
- Health Sciences › Medicine › Reproductive Medicine › Ovarian function and disorders
- Keywords
- classic congenital adrenal hyperplasia, 21-hydroxylase deficiency, 17-hydroxyprogesterone, glucocorticoid dosing, treatment monitoring, puberty, adrenarche, childhood, reference values
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Androstenedione, Child, Child, Preschool, Female, Glucocorticoids, Humans, Male, Puberty, Retrospective Studies, Saliva, 17-alpha-Hydroxyprogesterone
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