Prolongation of growth by treatment of 11-hydroxylase deficiency with depot-leuprolide, growth hormone, hydrocortisone

Laura J. Chalmers, Luis Casas, Maria I. New, Piers R. Blackett

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

A 4-10/12 year-old boy presented with tall stature and advanced secondary sexual characteristics. His bone age was 13 years giving him a height prediction of 147 cm. An initial 11-deoxycortisol level of 13,770 ng/dl and associated hypertension suggested the diagnosis of 11-hydroxylase deficiency, which was confirmed by dexamethasone suppression and genotyping. Treatment strategy was based on the premise that known hypothalamic priming resulting in early pubertal development could be averted by delaying puberty with leuprolide; also that effects of hydrocortisone and leuprolide on attenuating growth could be counteracted by growth hormone. The combined treatment resulted in a final height at age 12 years which was 25.4 cm greater than predicted, and bone density above average. We conclude that delaying puberty until an appropriate age, offsetting growth suppression, and improving bone mineralization can be effectively achieved using glucocorticoids, leuprolide and growth hormone in patients with 11-hydroxylase deficiency.

Original languageEnglish
Pages (from-to)1251-1255
Number of pages5
JournalJournal of Pediatric Endocrinology and Metabolism
Volume19
Issue number10
DOIs
StatePublished - Oct 2006

Keywords

  • 11-hydroxylase deficiency
  • Growth
  • Growth hormone
  • Leuprolide
  • Treatment

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