DEXAMETHASONE THERAPY FOR ISOSEXUAL PRECOCIOUS PSEUDOPUBERTY CAUSED BY GENERALIZED GLUCOCORTICOID RESISTANCE

Generalized glucocorticoid resistance presents with clinical features secondary to excess production of mineralocorticoids and adrenal andro gens. It is our hypothesis that these clinical and biochemical feature s will respond to glucocorticoid therapy. We tested this hypothesis in a boy with generalized glucocorticoid resistance and increased adrena l androgens. Dexamethasone was administered from age 76/12 yr until th e onset of true puberty at 110/12 yr. Serum concentrations of cortisol and adrenal androgens decreased to the normal or near normal range. T he accelerated precocity improved. Secondary sex characteristics did n ot progress; the difference between bone age and chronological age dec reased from 31/2 yr to 2 yr, and the difference between height age and bone age decreased from 2 yr to 1/2 yr. We conclude that dexamethason e is effective and safe therapy for the sexual precocity of generalize d glucocorticoid resistance.