17 alpha-hydroxylase deficiency medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]
Overview
The mainstay of therapy for 17 alpha-hydroxylase deficiency is glucocorticoid therapy. Spironolactone and estrogen may also be used.
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
- ↑ 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.