%0 Journal Article
%T Nocturnal Dexamethasone versus Hydrocortisone for the Treatment of Children with Congenital Adrenal Hyperplasia
%A Andrew Dauber
%A Henry A Feldman
%A Joseph A Majzoub
%J International Journal of Pediatric Endocrinology
%D 2010
%I BioMed Central
%R 10.1155/2010/347636
%X Congenital adrenal hyperplasia results from a defect in the adrenal steroid biosynthetic pathway leading to hypocortisolemia, resultant elevated ACTH levels and hyperandrogenism. In theory, glucocorticoid replacement should restore normocortisolemia, thus inhibiting excess ACTH secretion and correcting the hyperandrogenic state. In reality, however, adequate adrenal androgen suppression is difficult to achieve as, even in the face of ACTH levels in the normal range, adrenal steroid precursors proximal to the enzymatic defect will still accumulate and maintain the hyperandrogenic state [1]. Supraphysiologic doses of glucocorticoids may be needed to suppress excess androgen production at the risk of impaired linear growth [2, 3]. On the other hand, suboptimal adrenal androgen suppression with inadequate hydrocortisone therapy may accelerate linear growth, resulting in premature closure of the epiphyses and an impaired final adult height [4]. There is no consensus as to the optimal treatment or monitoring regimen for congenital adrenal hyperplasia.Our study is an open-labeled pilot study comparing the efficacy of nocturnal dexamethasone versus standard hydrocortisone therapy in the control of the hypothalamic-pituitary-adrenal axis in prepubertal subjects with classic salt-wasting congenital adrenal hyperplasia. We hypothesize that the administration of a long-acting glucocorticoid, dexamethasone, being given at night will more effectively suppress the pituitary gland diurnal secretion of ACTH, most of which occurs during the night. There is much debate in the literature as to the dose equivalency between hydrocortisone and dexamethasone with ratios ranging from 17£¿:£¿1 to 80£¿:£¿1 [5¨C8]. As our study was intended as an initial proof of principle pilot study, we chose a dose equivalency of 50£¿:£¿1 in order to ensure our ability to detect a difference in hormonal profiles on the two medication regimens, prior to exploring the lowest effective dose and potential toxicities o
%U http://www.ijpeonline.com/content/2010/1/347636