[11beta-hydroxylase deficiency: improvement of final height with growth hormone and gonadotropin-releasing hormone analog]

An Pediatr (Barc). 2007 Aug;67(2):153-6.
[Article in Spanish]

Abstract

Steroid 11beta-hydroxylase deficiency is the second most common cause of congenital adrenal hyperplasia. 11beta-hydroxylase intervenes in cortisol synthesis and its deficiency leads to accumulation of adrenal androgens--producing prenatal virilization and, subsequently, hyperandrogenism--as well as 11-deoxycorticosterone, leading to the development of hypertension. We describe a 7-year-old girl who was referred for pubarche and accelerated skeletal maturation due to 11beta-hydroxylase deficiency. Because the patient's predicted height was below her target height, the combination of gonadotropin-releasing hormone analog and growth hormone was added to oral glucocorticoid therapy. With this therapeutic strategy, the patient's predicted height improved significantly and the girl reached a final height in agreement with her target height at the age of 13 years and 6 months.

Publication types

  • Case Reports
  • Comparative Study
  • English Abstract

MeSH terms

  • Administration, Oral
  • Adrenal Hyperplasia, Congenital / diagnosis
  • Adrenal Hyperplasia, Congenital / drug therapy*
  • Age Factors
  • Body Height
  • Child
  • Drug Therapy, Combination
  • Female
  • Follow-Up Studies
  • Glucocorticoids / administration & dosage
  • Glucocorticoids / therapeutic use*
  • Gonadotropin-Releasing Hormone / administration & dosage
  • Gonadotropin-Releasing Hormone / analogs & derivatives*
  • Gonadotropin-Releasing Hormone / therapeutic use*
  • Growth Hormone / administration & dosage
  • Growth Hormone / therapeutic use*
  • Humans
  • Prognosis
  • Steroid 11-beta-Hydroxylase* / metabolism
  • Time Factors
  • Treatment Outcome

Substances

  • Glucocorticoids
  • Gonadotropin-Releasing Hormone
  • Growth Hormone
  • Steroid 11-beta-Hydroxylase