The traditional view of nonclassic congenital adrenal hyperplasia is that affected individuals are not glucocorticoid-deficient. The results of a French study now cast doubts on this assumption, however, and raise important issues for classification and management of patients with this common genetic disorder.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 12 print issues and online access
$209.00 per year
only $17.42 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
References
Bidet, M. et al. Clinical and molecular characterization of a cohort of 161 unrelated women with nonclassical congenital adrenal hyperplasia due to 21-hydroxylase deficiency and 330 family members. J. Clin. Endocrinol. Metab. 94, 1570–1578 (2009).
White, P. C. & Speiser, P. W. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr. Rev. 21, 245–291 (2000).
Azziz, R. et al. Androgen excess in women: experience with over 1000 consecutive patients. J. Clin. Endocrinol. Metab. 89, 453–462 (2004).
New, M. I. Extensive clinical experience: nonclassical 21-hydroxylase deficiency. J. Clin. Endocrinol. Metab. 91, 4205–4214 (2006).
Merke, D. P. & Bornstein, S. R. Congenital adrenal hyperplasia. Lancet 365, 2125–2136 (2005).
Krone, N., Dhir, V., Ivison, H. E. & Arlt, W. Congenital adrenal hyperplasia and P450 oxidoreductase deficiency. Clin. Endocrinol. (Oxf.) 66, 162–172 (2007).
Agha, A., Tomlinson, J. W., Clark, P. M., Holder, G. & Stewart, P. M. The long-term predictive accuracy of the short Synacthen (corticotropin) stimulation test for assessment of the hypothalamic–pituitary–adrenal axis. J. Clin. Endocrinol. Metab. 91, 43–47 (2006).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The authors declare no competing financial interests.
Rights and permissions
About this article
Cite this article
Krone, N., Stewart, P. An overlooked cause of glucocorticoid deficiency?. Nat Rev Endocrinol 5, 362–363 (2009). https://doi.org/10.1038/nrendo.2009.107
Issue Date:
DOI: https://doi.org/10.1038/nrendo.2009.107