Medical Treatment of Classic and Nonclassic Congenital Adrenal Hyperplasia

  • Phyllis W. SpeiserEmail author
Conference paper
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 707)


The main goals of medical therapy for congenital adrenal hyperplasia (CAH) are (1) to replace deficient cortisol with a suitable glucocorticoid (GC), (2) to reduce ACTH oversecretion and thereby prevent excessive androgen secretion, and (3) to replace deficient aldosterone with suitable mineralocorticoid (MC) and sodium supplements. Appropriate steroid treatment prevents adrenal crisis and virilization, allowing normal growth and development. A secondary goal is to preserve reproductive potential.


Congenital Adrenal Hyperplasia Adult Height Central Precocious Puberty Adrenal Crisis Congenital Adrenal Hyperplasia Patient 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Bonfig, W., Bechtold, S., Schmidt, H., Knorr, D., & Schwarz, H. P., “Reduced final height outcome in congenital adrenal hyperplasia under prednisone treatment: Deceleration of growth velocity during puberty,” J. Clin. Endocrinol. Metab. 92, 1635–1639 (2007).Google Scholar
  2. 2.
    Punthakee, Z., Legault, L., & Polychronakos, C., “Prednisolone in the treatment of adrenal insufficiency: A re-evaluation of relative potency,” J. Pediatrics 143(3), 402–405 (2003).CrossRefGoogle Scholar
  3. 3.
    Rivkees, S. A. & Crawford, J. D., “Dexamethasone treatment of virilizing congenital adrenal hyperplasia: The ability to achieve normal growth,” Pediatrics 106(4), 767–773 (2000).PubMedCrossRefGoogle Scholar
  4. 4.
    Merke, D. P., Cho, D., Anton Calis, K., Keil, M. F., & Chrousos, G. P., “Hydrocortisone suspension and hydrocortisone tablets are not bioequivalent in the treatment of children with congenital adrenal hyperplasia,” J. Clin. Endocrinol. Metab. 86(1), 441–445 (2001).Google Scholar
  5. 5.
    German, A., et al., “Control of childhood congenital adrenal hyperplasia and sleep activity and quality with morning or evening glucocorticoid therapy,” J. Clin. Endocrinol. Metab. 93(12), 4707–4710 (2008).PubMedCrossRefGoogle Scholar
  6. 6.
    Bonfig, W., et al., “Hydrocortisone dosing during puberty in patients with classical congenital adrenal hyperplasia: An evidence based recommendation,” J. Clin. Endocrinol. Metab. 94 3882–3888 (2009).Google Scholar
  7. 7.
    Speiser, P. W., et al., “Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: An endocrine society clinical practice guideline,” J. Clin. Endocrinol. Metab. 95, 4133–4160 (2010).Google Scholar
  8. 8.
    Charmandari, E., Hindmarsh, P. C., Johnston, A., & Brook, C. G., “Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: Alterations in cortisol pharmacokinetics at puberty,” J. Clin. Endocrinol. Metab. 86(6), 2701–2708 (2001).Google Scholar
  9. 9.
    Nimkarn, S., Lin-Su, K., Berglind, N., Wilson, R. C., & New, M. I., “Aldosterone-to-renin ratio as a marker for disease severity in 21-hydroxylase deficiency congenital adrenal hyperplasia,” J. Clin. Endocrinol. Metab. 92(1), 137–142 (2007).Google Scholar
  10. 10.
    Muthusamy K., et al., “Adult height outcomes in patients with congenital adrenal hyperplasia: A systematic review and meta-analysis,” J. Clin. Endocrinol. Metab. 95, 4161–4172 (2010).Google Scholar
  11. 11.
    Gomes, L. G., et al., “Extraadrenal 21-hydroxylation by CYP2C19 and CYP3A4: Effect on 21-hydroxylase deficiency,” J. Clin. Endocrinol. Metab. 94, 89–95 (2010).Google Scholar
  12. 12.
    Speiser, P. W., Agdere, L., Ueshiba, H., White, P. C., & New, M. I., “Aldosterone synthesis in salt-wasting congenital adrenal hyperplasia with complete absence of adrenal 21-hydroxylase,” N. Engl. J. Med. 324(3), 145–149 (1991).Google Scholar
  13. 13.
    Weise, M., et al., “Stress dose of hydrocortisone is not beneficial in patients with classic congenital adrenal hyperplasia undergoing short-term, high-intensity exercise,” J. Clin. Endocrinol. Metab. 89(8), 3679–3684 (2004).Google Scholar
  14. 14.
    Manoli, I., Kanaka-Gantenbein, C., Voutetakis, A., Maniati-Christidi, M., & Dacou-Voutetakis, C., “Early growth, pubertal development, body mass index and final height of patients with congenital adrenal hyperplasia: Factors influencing the outcome,” Clin. Endocrinol (Oxf). 57(5), 669–676 (2002).Google Scholar
  15. 15.
    Rasat, R., Espiner, E. A., & Abbott, G. D., “Growth patterns and outcomes in congenital adrenal hyperplasia; effect of chronic treatment regimens,” N. Z. Med. J. 108(1005), 311–314 (1995).Google Scholar
  16. 16.
    Weintrob, N., Dickerman, Z., Sprecher, E., Galatzer, A., & Pertzelan, A., “Non-classical 21-hydroxylase deficiency in infancy and childhood: The effect of time of initiation of therapy on puberty and final height,” Eur. J. Endocrinol. 136(2), 188–195 (1997).Google Scholar
  17. 17.
    Laue, L., et al., “A preliminary study of flutamide, testolactone, and reduced hydrocortisone dose in the treatment of congenital adrenal hyperplasia,” J. Clin. Endocrinol. Metab. 81(10), 3535–3539 (1996).Google Scholar
  18. 18.
    Merke, D. P., et al., “Flutamide, testolactone, and reduced hydrocortisone dose maintain normal growth velocity and bone maturation despite elevated androgen levels in children with congenital adrenal hyperplasia,” J. Clin. Endocrinol. Metab. 85(3), 1114–1120 (2000).PubMedCrossRefGoogle Scholar
  19. 19.
    Quintos, J. B., Vogiatzi, M. G., Harbison, M. D., & New, M. I., “Growth hormone therapy alone or in combination with gonadotropin- releasing hormone analog therapy to improve the height deficit in children with congenital adrenal hyperplasia,” J. Clin. Endocrinol. Metab. 86(4), 1511–1517 (2001).Google Scholar
  20. 20.
    Lin-Su, K., et al., “Treatment with growth hormone and luteinizing hormone releasing hormone analog improves final adult height in children with congenital adrenal hyperplasia,” J. Clin. Endocrinol. Metab. 90(6), 3318–3325 (2005).PubMedCrossRefGoogle Scholar
  21. 21.
    Dacou-Voutetakis, C. and Karidis, N., “Congenital adrenal hyperplasia complicated by central precocious puberty: Treatment with LHRH-agonist analogue,” Ann. N. Y. Acad. Sci. 687, 250–254 (1993).Google Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Division of Pediatric EndocrinologyCohen Children’s Medical Center of New YorkNew Hyde ParkUSA

Personalised recommendations