4.1 Review

Management of congenital adrenal hyperplasia in childhood

Journal

Publisher

LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MED.0b013e32835a1a1b

Keywords

childhood; congenital adrenal hyperplasia; NCAH; treatment

Funding

  1. SC CTSI, Keck School of Medicine of USC, NIH [KL2RR031991]
  2. Genentech, Inc.
  3. Novo Nordisk

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Purpose of review Congenital adrenal hyperplasia (CAH) can present management challenges to the pediatric clinician. Glucocorticoid replacement remains the cornerstone of treatment; however, there are new formulations and delivery mechanisms being studied. Clinicians continue to discuss the optimal treatment of patients from the prenatal stage, through infancy to adulthood. As well, the role of genetics in the clinical care of patients with CAH, and screening for complications, remain topics of discussion. This review will highlight advances made in the past year, as they pertain to the management of pediatric patients with CAH. Recent findings This article covers recent studies pertaining to optimal medication regimens, including prenatal dexamethasone treatment; medication delivery; monitoring of hormonal control; and the role of genotyping and genetics in the management of children with CAH. Summary Much remains to be learned about the optimal management of children with CAH, including fludrocortisone replacement in simple-virilizing patients, frequency of specific monitoring strategies (e.g., electrolytes, bone age), catecholamine status, stress-dosing in nonclassical adrenal hyperplasia, and early screening for complications or metabolic sequelae. Further randomized and prospective studies are needed to address these issues.

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