Modified release and conventional glucocorticoids and diurnal androgen excretion in congenital adrenal hyperplasia
Open accessAbstract
Context: The classic androgen synthesis pathway proceeds via dehydroepiandrosterone, androstenedione, and testosterone to 5α-dihydrotestosterone. However, 5α-dihydrotestosterone synthesis can also be achieved by an alternative pathway originating from 17α-hydroxyprogesterone (17OHP), which accumulates in congenital adrenal hyperplasia (CAH). Similarly, recent work has highlighted androstenedione-derived 11-oxygenated 19-carbon steroids as active androgens, and in CAH, androstenedione is generated directly from 17OHP. The exact contribution of alternative pathway activity to androgen excess in CAH and its response to glucocorticoid (GC) therapy is unknown. Objective: We sought to quantify classic and alternative pathway-mediated androgen synthesis in CAH, their diurnal variation, and their response to conventional GC therapy and modified-release hydrocortisone. Methods: We used urinary steroid metabolome profiling by gas chromatography-mass spectrometry for 24-hour steroid excretion analysis, studying the impact of conventional GCs (hydrocortisone, prednisolone, and dexamethasone) in 55 adults with CAH and 60 controls. We studied diurnal variation in steroid excretion by comparing 8-hourly collections (23:00-7:00, 7:00-15:00, and 15:00-23:00) in 16 patients with CAH taking conventional GCs and during 6 months of treatment with modified-release hydrocortisone, Chronocort. Results: Patients with CAH taking conventional GCs showed low excretion of classic pathway androgen metabolites but excess excretion of the alternative pathway signature metabolites 3α,5α-17-hydroxypregnanolone and 11β-hydroxyandrosterone. Chronocort reduced 17OHP and alternative pathway metabolite excretion to near-normal levels more consistently than other GC preparations. Conclusions: Alternative pathway-mediated androgen synthesis significantly contributes to androgen excess in CAH. Chronocort therapy appears superior to conventional GC therapy in controlling androgen synthesis via alternative pathways through attenuation of their major substrate, 17OHP.
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In summary, androgens are generated in CAH patients via all three major pathways.
“In summary, androgens are generated in CAH patients via all three major pathways (132).”
From Congenital Adrenal Hyperplasia—Current Insights in Pathophysiology, Diagnostics, and Management (2022) · cites this paper as [132]
11-oxygenated C19 steroids have been shown to be increased in CAH due to 2OH deficiency.
“11-oxygenated C19 steroids have been shown to be increased in CAH due to 2OH deficiency 130,131”
From Congenital adrenal hyperplasia (2017) · cites this paper as [130]
Exaggerated activity of both backdoor and 11-oxygenated C19 pathways persists in treated patients, even if the classic androgen pathway activity is downregulated.
“exaggerated activity of both backdoor and 11-oxygenated C19 pathways persists in treated patients, even if the classic androgen pathway activity is downregulated.130”
From Congenital adrenal hyperplasia (2017) · cites this paper as [130]
Urinary 11-oxygenated ETIO is predominantly a product of glucocorticoid metabolism, while urinary 11-oxygenated AST results mainly from 11-oxygenated androgens.
“While urinary 11-oxygenated ETIO is predominantly a product of glucocorticoid metabolism, urinary 11-oxygenated AST results mainly from 11-oxygenated androgens (Jones et al., 2016, Shackleton et al., 2008).”
From Intracrine androgen biosynthesis, metabolism and action revisited (2018) · cites this paper as Jones et al., 2016
Urinary 11-oxygenated AST and 11-oxygenated ETIO originate from androgen and glucocorticoid metabolism, however in different ratios.
“Urinary 11-oxygenated AST and 11-oxygenated ETIO originate from androgen and glucocorticoid metabolism, however in different ratios.”
From Intracrine androgen biosynthesis, metabolism and action revisited (2018) · cites this paper as Jones et al., 2016
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Self-citationEndocrine Care During Adolescence into Young Adulthood
Gleeson, H. K. (2019) · Wiley eBooks · doi:10.1002/9781119152712.ch19
Gleeson, H. K., & Wright, R. J. (2022) · doi:10.1093/med/9780198870197.003.0190
Betterle, C., Presotto, F., & Zanchetta, R. (2019) · Elsevier eBooks · doi:10.1016/b978-0-12-812102-3.00042-7
Management of Adult Patients With Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency
Grouthier, V., Dulon, J., Bachelot, A., & Touraine, P. A. (2018) · Elsevier eBooks · doi:10.1016/b978-0-12-801238-3.66140-8
Adrenal Multidisipliner Güncel Yaklaşım
Özen, F. Z., Özer, M., Özsoy, Ç., Binnetoğlu, E., Alparslan, A. Ş., Budak, E. S., Kök, E. V., Çetinkaya, G. S., Durmuş, M. E., Köker, G., et al. (2024) · Akademisyen Kitabevi eBooks · doi:10.37609/akya.2992
Betterle, C., Sabbadin, C., & Presotto, F. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-23947-2.00056-4
Management of adult patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency
Grouthier, V., Dulon, J., Bachelot, A., & Touraine, P. A. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-13825-6.00132-1
Steroid replacement in adrenal insufficiency
Blair, J., Debono, M., & Ross, R. J. M. (2024) · Elsevier eBooks · doi:10.1016/b978-0-443-13825-6.00147-3
Self-citationChong, H., Pi, Y., Zhang, Y., Li, Y., Xing, Y., & Zhang, H. (2025) · BMC Pediatrics · doi:10.1186/s12887-025-05643-3
Praktisches Management des Adrenogenitalen Syndroms
Wörther, H. (2025) · Journal für Endokrinologie Diabetologie und Stoffwechsel · doi:10.1007/s41969-025-00269-3
Congenital adrenal hyperplasia
Vorontsova, M. V., Кокорина, Т. С., Nuralieva, N. F., Yukina, M. Y., Troshina, E., Mel'nichenko, G. A., & Mokrysheva, N. G. (2026) · Problems of Endocrinology · doi:10.14341/probl13763
Reproductive and fertility challenges in adolescents with congenital adrenal hyperplasia
Tschaidse, L., Auer, M. K., Nowotny, H. F., & Reisch, N. (2026) · Frontiers in Adolescent Medicine · doi:10.3389/fradm.2026.1878713
Self-citationSzklarz, M., Wołos-Kłosowicz, K., Szulc, J., Górny, J. M., & Matuszewski, W. (2026) · Middle East Fertility Society Journal · doi:10.1186/s43043-026-00364-8
- Authors
- Jones, C. M., Mallappa, A., Reisch, N., Nikolaou, N., Krone, N., Hughes, B. A., O’Neil, D. M., Whitaker, M. J., Tomlinson, J. W., Storbeck, K.-H., Merke, D. P., Ross, R. J., & Arlt, W.
- Affiliations
- University of Birmingham (GB)
- Eunice Kennedy Shriver National Institute of Child Health and Human Development (US)
- Ludwig-Maximilians-Universität München (DE)
- University of Oxford (GB)
- Diurnal (United Kingdom) (GB)
- Stellenbosch University (ZA)
- Corresponding author
- Arlt, Wiebke
- ORCID
- Venue
- The Journal of Clinical Endocrinology & Metabolism
- Publisher
- The Endocrine Society
- Published
- 2016-11-15
- Volume
- 102
- Issue
- 6
- Pages
- jc.2016-2855
- ISSN
- 0021-972X (ISSN-L)
- 1945-7197
- PMC
- PMC5470768
- OpenAlex
- W2556069495
- Cited by
- 74
- References
- 27
- Citations by year
- 2026: 4 · 2025: 6 · 2024: 12 · 2023: 6 · 2022: 7 · 2021: 3 · 2020: 8 · 2019: 8 · 2018: 13 · 2017: 6 · 2016: 1
- FWCI
- 4.85
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- cc-by
- Locations
- The Journal of Clinical Endocrinology & Metabolism (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- White Rose Research Online (University of Leeds, The University of Sheffield, University of York) (acceptedVersion)
- University of Birmingham Research Portal (University of Birmingham) (submittedVersion, cc-by)
- Spiral (Imperial College London) (submittedVersion, cc-by)
- Funders
- National Institute for Health and Care Research (ACF-2015-02-004)
- Royal Society
- Academy of Medical Sciences (NAF004\1002)
- European Commission (281654)
- National Institutes of Health
- Medical Research Council (MR/P011462/1, 0900567)
- 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
- congenital adrenal hyperplasia, modified-release hydrocortisone, glucocorticoid therapy, 17-hydroxyprogesterone, hyperandrogenism, prednisolone, dexamethasone
- MeSH terms
- Adolescent, Adrenal Hyperplasia, Congenital, Adult, Androgens, Androsterone, Circadian Rhythm, Cortodoxone, Delayed-Action Preparations, Dexamethasone, Female, Glucocorticoids, Humans, Hydrocortisone, 17-alpha-Hydroxypregnenolone, Male, Gas Chromatography-Mass Spectrometry, Middle Aged, Prednisolone, Pregnanetriol, Young Adult
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