ESPE Yearbook of Paediatric Endocrinology

Brief Summary: This study investigated daily glucose patterns in young children with classic congenital adrenal hyperplasia (CAH) and their relation with hormonal circadian rhythm.

Comment: The aim of treatment in classical CAH is to provide adequate glucocorticoid and – when necessary – mineralocorticoid replacement to prevent adrenal crises and suppress excess adrenal androgen production. Glucocorticoids regulate glucose homeostasis, and patients with classical CAH are at risk of severe hypoglycemia during stress and infections (1). However, mimicking the physiologic, circadian rhythm in cortisol secretion is impossible due to the pharmacokinetics of typical, immediate-release, hydrocortisone (HC) tablets (2). Hence, children receiving HC for CAH may be exposed to hypoglycemic periods during the day.

This study used subcutaneous continuous glucose monitoring (CGM) to assess the prevalence of hypoglycemia in young children with CAH (n=11, 1-6 years) and to identify any relationship with hormonal circadian rhythm. Cortisol and ACTH samples were collected for a 24-hour inpatient admission. Children with CAH were more often exposed to asymptomatic hypoglycemia during the night and morning than age-matched healthy controls (3). Daily cortisol patterns paralleled CGM levels. As prolonged and frequent exposure to hypoglycemia may adversely affect neurodevelopment, these findings suggest CGM may be a supportive tool in the clinical management of young children with CAH, to guide HC dose adjustments.

References: 1. Odenwald B, Nennstiel-Ratzel U, Dörr H, Schmidt H, Wildner M, Bonfig W. Children with classic congenital adrenal hyperplasia experience salt loss and hypoglycemia: evaluation of adrenal crises during the first 6 years of life. Eur J Endocrinol. 2016; 174(2): 177-186.2. Auer M, Nordenström A, Lajic S, Reisch N. Congenital adrenal hyperplasia. Lancet. 2023; 401(10372): 227-244.3. DuBose S, Kanapka L, Bradfield B, Sooy M, Beck R, Steck A. Continuous glucose monitoring profiles in healthy, nondiabetic young children. J Endocr Soc. 2022; 6(6): bvac060.