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[Adrenalectomy as therapy in refractory adrenogenital syndrome].

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.
AuthorsK E von Mühlendahl, W G Sippell
JournalMonatsschrift Kinderheilkunde : Organ der Deutschen Gesellschaft fur Kinderheilkunde (Monatsschr Kinderheilkd) Vol. 137 Issue 6 Pg. 341-4 (Jun 1989) ISSN: 0026-9298 [Print] Germany
Vernacular TitleAdrenalektomie als Therapie bei schwer einstellbarem adrenogenitalen syndrom.
PMID2761548 (Publication Type: Case Reports, English Abstract, Journal Article)
Chemical References
  • Hydroxyprogesterones
  • 17-alpha-Hydroxyprogesterone
  • Hydrocortisone
Topics
  • 17-alpha-Hydroxyprogesterone
  • Adrenal Hyperplasia, Congenital (blood, drug therapy, surgery)
  • Adrenalectomy
  • Child
  • Female
  • Follow-Up Studies
  • Humans
  • Hydrocortisone (administration & dosage)
  • Hydroxyprogesterones (blood)

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