Health Dictionary
Reliable, easy-to-read patient information on common medical conditions — written in plain language, free of jargon, and grounded in current evidence. Browse the most frequent diagnoses, understand what they mean for everyday life, and learn which steps and questions help you have a focused conversation with your doctor.
17 alpha-hydroxylase deficiency medical therapy
Introduction
Overview
Overview
The mainstay of therapy for 17 alpha-hydroxylase deficiency is glucocorticoid therapy. Spironolactone and estrogen may also be used.
Medical Therapy
Medical Therapy
The goal of therapy is: [1][2][3]
- Correct the effects of mineralocorticoid excess
- Prevent glucocorticoid deficiency
- Restore desired secondary sexual characteristics
- Treatment for 17 alpha-hydroxylase deficiency is by the use of glucocorticoids such as:
- Preferred regimen (1): Hydrocortisone 10 to 25 mg/m2 body surface area/day PO.
- Preferred regimen (2): Prednisolone 0.1 mg/kg/day PO.
- Preferred regimen (3): Dexamethasone up to 0.5 mg/day PO.
- As all the patients are clinically female, spironolactone is the drug of choice to block the mineralocorticoid receptor:
- Preferred regimen (1): Spironolactone 25 to 200 mg/day PO.
- Treatment should be monitored by:
- At the time of expected puberty, estrogen replacement should be started.
References
References
- ↑ El-Maouche D, Arlt W, Merke DP (2017). “Congenital adrenal hyperplasia”. Lancet. doi:10.1016/S0140-6736(17)31431-9. PMID 28576284.
- ↑ Merke DP, Poppas DP (2013). “Management of adolescents with congenital adrenal hyperplasia”. Lancet Diabetes Endocrinol. 1 (4): 341–52. doi:10.1016/S2213-8587(13)70138-4. PMC 4163910. PMID 24622419.
- ↑ Hughes IA (1988). “Management of congenital adrenal hyperplasia”. Arch Dis Child. 63 (11): 1399–404. PMC 1779155. PMID 3060026.
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]