Low-Dose Dexamethasone Therapy from Infancy of Virilizing Congenital Adrenal Hyperplasia

Rivkees, Scott A.; Stephenson, Kerry

doi:10.1155/2009/274682

Cited by 11 publications

(6 citation statements)

References 10 publications

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“…These relative dose equivalencies have been reaffirmed in clinical studies [6–9], and when prednisone and hydrocortisone are used at the higher potency equivalency ratios, children can be effectively treated with these long-acting glucocorticoids [6–8]. …”

Section: Introductionmentioning

confidence: 91%

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Dexamethasone Therapy of Congenital Adrenal Hyperplasia and the Myth of the “Growth Toxic” Glucocorticoid

Rivkees

2010

International Journal of Pediatric Endocrinology

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The use of long-acting glucocorticoids in the treatment of individuals with Congenital Adrenal Hyperplasia (CAH) has been greeted with controversy. Avoidance of dexamethasone therapy is in part due to the mistaken assumptions that dexamethasone is 30-fold more potent than hydrocortisone in suppressing adrenal activity, resulting in the overtreatment and the “growth toxic” label. However, as shown more than 50 years ago, dexamethasone is 80- to 100-fold (or greater) more potent than hydrocortisone in suppressing adrenal androgen production. When children are treated with low doses of dexamethasone once daily in the morning (0.15–0.3 mg/m2/day of dexamethasone versus 10–25 mg/m2/day of hydrocortisone), studies involving infants and children show that normal growth and skeletal maturation can be achieved, along with appropriate suppression of adrenal androgen secretion. Due to its high potency, the potential for overtreatment remains high with dexamethasone. Thus, it is imperative that dexamethasone-treated children be closely monitored.

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Section: Introductionmentioning

confidence: 91%

“…Following studies of dexamethasone in older children, we applied dexamethasone to the treatment of infants with CAH [8]. Three boys and 5 girls were identified as having CAH by newborn screening and treated with dexamethasone from birth until about 7 years of age, when data were analyzed.…”

Section: Treatment Of Cah With Dexamethasone Beginning In Infancymentioning

confidence: 99%

Dexamethasone Therapy of Congenital Adrenal Hyperplasia and the Myth of the “Growth Toxic” Glucocorticoid

Rivkees

2010

International Journal of Pediatric Endocrinology

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“…Treatment with dexamethasone in children with simple virilizing CAH was reported by Rivkees et al with promising results (7-8). The average daily morning dose in the first study was 0.27 mg/m 2 /day.…”

Section: Discussionmentioning

confidence: 97%

“…To the best of our knowledge, this is the first case series describing children with nonclassical CAH who were treated with ultralow-dose dexamethasone. Treatment with dexamethasone in children with simple virilizing CAH was reported by Rivkees et al with promising results ( 7 - 8 ). The average daily morning dose in the first study was 0.27 mg/m 2 /day.…”

Section: Discussionmentioning

confidence: 97%

Ultralow-dose Dexamethasone to Preserve Endogenous Cortisol Stress Response in Nonclassical Congenital Adrenal Hyperplasia: a New Promising Treatment

Kaay

Akker

2014

Int J Endocrinol Metab

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Introduction:Nonclassical congenital adrenal hyperplasia (CAH) is characterized by sufficient cortisol and aldosterone production at the cost of androgen overproduction. Hydrocortisone or dexamethasone in supraphysiological doses are current treatment; however, their downside is suppression of endogenous cortisol production resulting in corticosteroid dependency. We aimed to treat children with nonclassical CAH with a ultralow-dose dexamethasone to normalize androgen levels, without a detrimental effect on endogenous cortisol production.Case Presentation:We recruited five patients diagnosed with nonclassical CAH on the basis of clinical presentation, biochemical analyses, and genetic testing. Anthropometric as well as biochemical parameters and bone age were measured on a regular basis. During treatment, an adrenocorticotropin (ACTH) stimulation test was performed. Outcome measures were normalization of androgens and deceleration of the bone age advancement with sufficient endogenous cortisol response. Androgen levels were normalized in all patients resulting in a deceleration of the bone age advancement. Cortisol stress response remained normal in four out of five patients. Only one patient needed hydrocortisone stress dosing.Conclusions:According to this case series, it seems that ultralow-dose dexamethasone in treatment of nonclassical CAH would be a promising novel treatment strategy. The advantage of this treatment strategy is that adverse effects of hyperandrogenism can be reversed while preserving the endogenous cortisol stress response.

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“…Their patients had predicted adult heights that were comparable to target mid-parental heights with normal levels of adrenal hormones. Recently, Rivkees and Stephenson [22] reported on their experience in treating young children with dexamethasone starting in infancy. These children had normal growth patterns.…”

Section: Discussionmentioning

confidence: 99%

Nocturnal Dexamethasone versus Hydrocortisone for the Treatment of Children with Congenital Adrenal Hyperplasia

Dauber

Feldman

Majzoub

2010

International Journal of Pediatric Endocrinology

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Classic congenital adrenal hyperplasia affects approximately 1 in 15,000 children. Current treatment strategies using multiple daily doses of hydrocortisone lead to suboptimal outcomes. We tested the hypothesis that nocturnal administration of dexamethasone will suppress the hypothalamic-pituitary-adrenal axis more effectively than standard hydrocortisone treatment by blocking the inherent diurnal secretion of ACTH. We performed a pilot study of five prepubertal patients comparing CAH control during two 24-hour hospitalizations, one on hydrocortisone and the other on dexamethasone. The patterns of adrenal suppression differed markedly between hydrocortisone and nocturnal dexamethasone, with significant suppression of the morning rise in ACTH, 17-hydroxyprogesterone, and androstenedione while on dexamethasone. On hydrocortisone therapy, there is a marked variation in ACTH and adrenal hormones depending on time of day and timing of hydrocortisone administration. Longer-term studies are needed to investigate the lowest effective dose and potential toxicity of nocturnal dexamethasone to determine its utility as a therapy for CAH.

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Low-Dose Dexamethasone Therapy from Infancy of Virilizing Congenital Adrenal Hyperplasia

Cited by 11 publications

References 10 publications

Dexamethasone Therapy of Congenital Adrenal Hyperplasia and the Myth of the “Growth Toxic” Glucocorticoid

Dexamethasone Therapy of Congenital Adrenal Hyperplasia and the Myth of the “Growth Toxic” Glucocorticoid

Ultralow-dose Dexamethasone to Preserve Endogenous Cortisol Stress Response in Nonclassical Congenital Adrenal Hyperplasia: a New Promising Treatment

Nocturnal Dexamethasone versus Hydrocortisone for the Treatment of Children with Congenital Adrenal Hyperplasia

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