Research Study for Children With Salt Wasting Congenital Adrenal Hyperplasia

Summary

The purpose of this study is to develop a more physiological approach to the management of children and adolescents with salt wasting Congenital Adrenal Hyperplasia. We will administer the glucocorticosteroid via insulin infusion pump to see whether this treatment will improve the serum hormone concentrations.

Timeline

Start

2007-01

Primary completion

2008-09

Completion

2008-09

Publications

• Background Esteban NV, Loughlin T, Yergey AL, Zawadzki JK, Booth JD, Winterer JC, Loriaux DL. Daily cortisol production rate in man determined by stable isotope dilution/mass spectrometry. J Clin Endocrinol Metab. 1991 Jan;72(1):39-45. doi: 10.1210/jcem-72-1-39.
• Background Kerrigan JR, Veldhuis JD, Leyo SA, Iranmanesh A, Rogol AD. Estimation of daily cortisol production and clearance rates in normal pubertal males by deconvolution analysis. J Clin Endocrinol Metab. 1993 Jun;76(6):1505-10. doi: 10.1210/jcem.76.6.8501158.
• Background Speiser PW. Toward better treatment of congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 1999 Sep;51(3):273-4. doi: 10.1046/j.1365-2265.1999.00780.x. No abstract available.
• Background Cutler GB Jr, Laue L. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. N Engl J Med. 1990 Dec 27;323(26):1806-13. doi: 10.1056/NEJM199012273232605. No abstract available.
• Background Winterer J, Chrousos GP, Loriaux DL, Cutler GB Jr. Effect of hydrocortisone dose schedule on adrenal steroid secretion in congenital adrenal hyperplasia. J Pediatr. 1985 Jan;106(1):137-42. doi: 10.1016/s0022-3476(85)80486-8.
• Background Wallace WH, Crowne EC, Shalet SM, Moore C, Gibson S, Littley MD, White A. Episodic ACTH and cortisol secretion in normal children. Clin Endocrinol (Oxf). 1991 Mar;34(3):215-21. doi: 10.1111/j.1365-2265.1991.tb00297.x.
• Background Merza Z, Rostami-Hodjegan A, Memmott A, Doane A, Ibbotson V, Newell-Price J, Tucker GT, Ross RJ. Circadian hydrocortisone infusions in patients with adrenal insufficiency and congenital adrenal hyperplasia. Clin Endocrinol (Oxf). 2006 Jul;65(1):45-50. doi: 10.1111/j.1365-2265.2006.02544.x.
• Background Lukert BP. Editorial: glucocorticoid replacement--how much is enough? J Clin Endocrinol Metab. 2006 Mar;91(3):793-4. doi: 10.1210/jc.2005-2737. No abstract available.
• Background Claude J.Migeon. Can the Long Range Results of the Treatment of Congenital Adrenal Hyperplasia be improved? JCEM 1996 Vol 81, No 9 3187-3189
• Background Laue L, Merke DP, Jones JV, Barnes KM, Hill S, Cutler GB Jr. A preliminary study of flutamide, testolactone, and reduced hydrocortisone dose in the treatment of congenital adrenal hyperplasia. J Clin Endocrinol Metab. 1996 Oct;81(10):3535-9. doi: 10.1210/jcem.81.10.8855797.
• Background Mah PM, Jenkins RC, Rostami-Hodjegan A, Newell-Price J, Doane A, Ibbotson V, Tucker GT, Ross RJ. Weight-related dosing, timing and monitoring hydrocortisone replacement therapy in patients with adrenal insufficiency. Clin Endocrinol (Oxf). 2004 Sep;61(3):367-75. doi: 10.1111/j.1365-2265.2004.02106.x.
• Background Sheila K.Gunn et al Subcutaneous Hydrocortisone Delivery mimics Physiologic Cortisol Concentrations, Poster, Endocrine Society Meeting 2000

Drugs

Indications