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Patients with Classic Congenital Adrenal Hyperplasia Have Decreased Epinephrine Reserve and Defective Glucose Elevation in Response to High-Intensity Exercise

Pediatric and Reproductive Endocrinology Branch (S.L.M., E.R., E.C., G.P.C., D.P.M.), Developmental Endocrinology Branch (M.W., J.A.Y.), National Institute of Child Health and Human Development, The Warren Grant Magnuson Clinical Center (B.D., D.P.M.), and Clinical Neurocardiology Section, National Institute of Neurological Disorders and Stroke (M.H., G.E.), National Institutes of Health, Bethesda, Maryland 20892

Address all correspondence and requests for reprints to: Deborah P. Merke, M.D., National Institutes of Health Clinical Center, Building 10, Room 13S260, 10 Center Drive MSC 1932, Bethesda, Maryland 20892-1932. E-mail: dmerke{at}nih.gov.

Classic congenital hyperplasia (CAH) is characterized by impaired adrenocortical function with a decrease in cortisol and aldosterone secretion and an increase in androgen secretion. Adrenomedullary function is also compromised due to developmental defects in the formation of the adrenal medulla, leading to decreased production of epinephrine. To examine the response to a natural stressful stimulus in patients with classic CAH, we studied hormonal, metabolic, and cardiorespiratory parameters in response to a standardized high-intensity exercise protocol in nine adolescent patients with CAH and nine healthy controls matched for gender, age, and percent body fat. The same relative workload was applied, based on individual maximal aerobic capacity, and all patients received their usual glucocorticoid and mineralocorticoid replacement. When compared with their normal counterparts, patients with CAH had significantly lower epinephrine levels both at baseline and at peak exercise (P < 0.01), whereas norepinephrine levels did not differ. Blood glucose concentrations were similar at baseline, but the normal exercise-induced rise observed in the healthy controls was significantly blunted in the CAH patients (P < 0.01). Peak heart rate was also lower in CAH patients than healthy controls (P < 0.05). As expected, the normal exercise-induced increase in cortisol was not observed in patients with CAH. No significant differences were found in serum levels of insulin, glucagon, GH, lactate and free fatty acids, blood pressure, or ability to sustain exercise between the two groups. Patients with CAH replaced with glucocorticoids have decreased adrenomedullary reserve and impaired exercise-induced changes in glucose but normal short-term high-intensity exercise performance. Whether the combination of epinephrine and cortisol deficiency poses a risk for hypoglycemia and/or decreased endurance during long-term physical stress has to be determined.

J.A.Y. and D.P.M. are Commissioned Officers in the United States Public Health Service.

Abbreviations: CAH, Congenital adrenal hyperplasia; E, epinephrine; FFA, free fatty acid; NE, norepinephrine; RPE, rating of perceived exertion; VO₂, oxygen uptake; VO_2max, maximal aerobic capacity.

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