Dexamethasone therapy for isosexual precocious pseudopuberty caused by generalized glucocorticoid resistance.

Abstract

Generalized glucocorticoid resistance presents with clinical features secondary to excess production of mineralocorticoids and adrenal androgens. It is our hypothesis that these clinical and biochemical features will respond to glucocorticoid therapy. We tested this hypothesis in a boy with generalized glucocorticoid resistance and increased adrenal androgens. Dexamethasone was administered from age 7 6/12 yr until the onset of true puberty at 11 0/12 yr. Serum concentrations of cortisol and adrenal androgens decreased to the normal or near normal range. The accelerated precocity improved. Secondary sex characteristics did not progress; the difference between bone age and chronological age decreased from 3 1/2 yr to 2 yr, and the difference between height age and bone age decreased from 2 yr to 1/2 yr. We conclude that dexamethasone is effective and safe therapy for the sexual precocity of generalized glucocorticoid resistance.

• Authors: C D Malchoff, G Reardon, E C Javier, A D Rogol, P McDermott, D L Loriaux, D M Malchoff
• Journal: The Journal of clinical endocrinology and metabolism (J Clin Endocrinol Metab) Vol. 79 Issue 6 Pg. 1632-6 (Dec 1994) ISSN: 0021-972X [Print] United States
• PMID: 7989467 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Androgens
• Glucocorticoids
• Dexamethasone
• Hydrocortisone

Topics

• Age Determination by Skeleton
• Androgens (blood)
• Body Height
• Child
• Dexamethasone (administration & dosage, therapeutic use)
• Drug Resistance
• Glucocorticoids
• Humans
• Hydrocortisone (blood)
• Male
• Puberty, Precocious (drug therapy, etiology, physiopathology)