Salivary morning androstenedione and 17α-OH progesterone levels in childhood and puberty in patients with classic congenital adrenal hyperplasia

Monique J.M. de Groot; Karijn J. Pijnenburg-Kleizen; Chris M.G. Thomas; Fred C.G.J. Sweep; Nike M.M.L. Stikkelbroeck; Barto J. Otten; Hedi L. Claahsen-van der Grinten

doi:10.1515/cclm-2014-0375

Article

Salivary morning androstenedione and 17α-OH progesterone levels in childhood and puberty in patients with classic congenital adrenal hyperplasia

Monique J.M. de Groot , Karijn J. Pijnenburg-Kleizen , Chris M.G. Thomas , Fred C.G.J. Sweep , Nike M.M.L. Stikkelbroeck , Barto J. Otten and Hedi L. Claahsen-van der Grinten

Published/Copyright: October 6, 2014

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From the journal Clinical Chemistry and Laboratory Medicine (CCLM) Volume 53 Issue 3

Abstract

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.

Keywords: congenital adrenal hyperplasia; 21-hydroxylase deficiency; monitoring; salivary androstenedione; salivary 17α-OHprogesterone

Corresponding author: Dr. Hedi L. Claahsen-van der Grinten, Department of Paediatric Endocrinology, Radboud University Medical Centre, Geert Grooteplein 10, 6525 GA Nijmegen, The Netherlands, Phone: +31 24 3614430, E-mail : Hedi.Claahsen@radboudumc.nl

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Received: 2014-4-6

Accepted: 2014-9-1

Published Online: 2014-10-6

Published in Print: 2015-2-1

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Articles in the same Issue

https://doi.org/10.1515/cclm-2014-0375

Keywords for this article

congenital adrenal hyperplasia; 21-hydroxylase deficiency; monitoring; salivary androstenedione; salivary 17α-OHprogesterone