Prolonged remission of severe Cushing syndrome without adrenalectomy in an infant with McCune-Albright syndrome

J Pediatr. 2008 Jun;152(6):882-4, 884.e1-4. doi: 10.1016/j.jpeds.2008.01.037.

Abstract

A 4 month-old girl presented with severe Cushing syndrome caused by McCune-Albright syndrome. After undergoing 19 months of pharmacologic suppression of cortisol production, she has been in clinical remission for more than 6 years. Adrenalectomy may be avoidable even in severe cases of Cushing syndrome associated with McCune-Albright syndrome.

Publication types

  • Case Reports

MeSH terms

  • Adrenalectomy
  • Adrenergic Agents / therapeutic use*
  • Aminoglutethimide / therapeutic use*
  • Child
  • Cushing Syndrome / drug therapy*
  • Cushing Syndrome / etiology*
  • Enzyme Inhibitors / therapeutic use*
  • Female
  • Fibrous Dysplasia, Polyostotic / complications*
  • Follow-Up Studies
  • Humans
  • Infant
  • Metyrapone / therapeutic use*
  • Remission Induction
  • Severity of Illness Index
  • Time Factors

Substances

  • Adrenergic Agents
  • Enzyme Inhibitors
  • Aminoglutethimide
  • Metyrapone