Continuous subcutaneous hydrocortisone infusion (CSHI) in a young adolescent with congenital adrenal hyperplasia (CAH)

J Pediatr Endocrinol Metab. 2011;24(7-8):561-3. doi: 10.1515/jpem.2011.226.

Abstract

Congenital adrenal hyperplasia (CAH) is an autosomal recessive enzymatic defect caused by mutations or deletions of the cytochrome P450 21-hydroxylase CYP21 gene. Oral therapy with glucocorticoids and mineralcorticoids is administered to prevent adrenal crisis and to control hyperandrogenism. During puberty this type of therapy is difficult to manage owing to physiological and hormonal changes and poor compliance. We describe a case of a pubertal boy affected by CAH, in whom continuous subcutaneous infusion of hydrocortisone led to improved metabolic control and compliance.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adolescent Behavior / psychology
  • Adrenal Hyperplasia, Congenital / drug therapy*
  • Adrenal Hyperplasia, Congenital / physiopathology
  • Adrenal Hyperplasia, Congenital / psychology
  • Anti-Inflammatory Agents / administration & dosage*
  • Anti-Inflammatory Agents / therapeutic use
  • Drug Delivery Systems
  • Glycosuria / etiology
  • Glycosuria / prevention & control
  • Humans
  • Hydrocortisone / administration & dosage*
  • Hydrocortisone / therapeutic use
  • Infusions, Subcutaneous
  • Male
  • Medication Adherence / psychology
  • Puberty, Precocious / etiology
  • Treatment Outcome

Substances

  • Anti-Inflammatory Agents
  • Hydrocortisone

Supplementary concepts

  • Congenital adrenal hyperplasia due to 21 hydroxylase deficiency