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 11beta-hydroxylase deficiency in children is glucocorticoids such as hydrocortisone. The response to therapy should be monitored by laboratory tests and clinical findings. 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 adult women with 11beta-hydroxylase deficiency androgen excess and hypertension remain indications for treatment. The treatment option in this groups is spironolactone.If pregnancy is not desired, spironolactone plus an oral contraception pill 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 11beta-hydroxylase deficiency in children is glucocorticoids such as:
  • Hydrocortisone (10 to 25 mg/m2)
  • Prednisolone (0.1 mg/kg)
  • Dexamethasone (up to 0.5 mg/day).

• 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 skeletal maturation (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 adult women with 11beta-hydroxylase deficiency androgen excess and hypertension remain indications for treatment. The treatment option in this groups:
  • Spironolactone, 25 to 200 mg/day
  • If pregnancy is not desired, spironolactone plus an oral contraception pill 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.