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Medical Treatment of Classic and Nonclassic Congenital Adrenal Hyperplasia

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Hormonal and Genetic Basis of Sexual Differentiation Disorders and Hot Topics in Endocrinology: Proceedings of the 2nd World Conference

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 707))

Abstract

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.

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References

  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. 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).

    Article  CAS  Google Scholar 

  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).

    Article  PubMed  CAS  Google Scholar 

  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. 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).

    Article  PubMed  CAS  Google Scholar 

  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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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).

    Article  PubMed  CAS  Google Scholar 

  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. 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).

    Article  PubMed  CAS  Google Scholar 

  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 

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Authors and Affiliations

  1. Division of Pediatric Endocrinology, Cohen Children’s Medical Center of New York, New Hyde Park, NY, 11040, USA

    Phyllis W. Speiser

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  1. Phyllis W. Speiser
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Correspondence to Phyllis W. Speiser .

Editor information

Editors and Affiliations

  1. Mount Sinai School of Medicine, One Gustave L. Levy place box, New York, 10029, USA

    Maria I. New

  2. Florida International University, 11200 S.W. 8th Street, Miami, 33199, Florida, USA

    Joe Leigh Simpson

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Speiser, P.W. (2011). Medical Treatment of Classic and Nonclassic Congenital Adrenal Hyperplasia. In: New, M., Simpson, J. (eds) Hormonal and Genetic Basis of Sexual Differentiation Disorders and Hot Topics in Endocrinology: Proceedings of the 2nd World Conference. Advances in Experimental Medicine and Biology, vol 707. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-8002-1_9

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