[Adrenalectomy as therapy in refractory adrenogenital syndrome]

Monatsschr Kinderheilkd. 1989 Jun;137(6):341-4.
[Article in German]

Abstract

A ten year old girl with congenital adrenal hyperplasia was bilaterally adrenalectomized because even high doses of glucocorticosteroids and mineralocorticosteroids did not sufficiently suppress androgen hypersecretion. Thus, her bone age had advanced over chronological age by three years. The difficulties and started after the institution of phenytoin and clonazepam therapy for epilepsy at the age of three years. The surgical removal of the adrenal glands in such patients appears to be a logical therapeutical approach in such situations, since the organs principally are useless in patients with congenital adrenal hyperplasia, lacking the ability to react with adequate cortisol secretion.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • 17-alpha-Hydroxyprogesterone
  • Adrenal Hyperplasia, Congenital / blood
  • Adrenal Hyperplasia, Congenital / drug therapy
  • Adrenal Hyperplasia, Congenital / surgery*
  • Adrenalectomy*
  • Child
  • Female
  • Follow-Up Studies
  • Humans
  • Hydrocortisone / administration & dosage
  • Hydroxyprogesterones / blood

Substances

  • Hydroxyprogesterones
  • 17-alpha-Hydroxyprogesterone
  • Hydrocortisone