11β-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

Treatment for 11β-hydroxylase deficiency in children is administration of glucocorticoids. The response to therapy should be monitored by laboratory tests and clinical findings. The treatment option in women is spironolactone. If pregnancy is not desired, spironolactone plus oral contraceptive pills can be combined with replacement doses of hydrocortisone. In adult males, replacement doses of hydrocortisone should be administered to avoid the development of adrenal rest tumors.

Medical Therapy

• Treatment for 11β-hydroxylase deficiency in children is glucocorticoids as followings:
  • 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

• The response to therapy should be monitored by:
  • Laboratory tests such as serum DHEAS, potassium levels, and 11-deoxycortisol.
  • Clinical findings such virilization, growth velocity, and bone age.
• Glucocorticoid-related side effects such as bruising, weight gain, and glucose intolerance should be monitored.

• Genital malformations in females may require surgical correction with one or more surgeries and vaginal dilation.

• In women with 11β-hydroxylase deficiency, androgen excess and hypertension are indications for treatment. The treatment option in this groups:
  • Preferred regimen (1): Spironolactone 25 to 200 mg/day PO
  • If pregnancy is not desired, oral contraceptive pills can be combined with hydrocortisone.

• In adult males, replacement doses of hydrocortisone should be administered to avoid the development of adrenal rest tumors.