Adrenomedullary function in patients with nonclassic congenital adrenal hyperplasia

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • S. Verma - , Eunice Kennedy Shriver National Institute of Child Health and Human Development, United States Public Health Service (Author)
  • L. Green-Golan - , National Institutes of Health (NIH) (Author)
  • C. Vanryzin - , National Institutes of Health (NIH) (Author)
  • B. Drinkard - , National Institutes of Health (NIH) (Author)
  • S. P. Mehta - , National Institutes of Health (NIH) (Author)
  • M. Weise - , Federal Institute for Drugs and Medical Devices (BfArM) (Author)
  • G. Eisenhofer - , Institute of Clinical Chemistry and Laboratory Medicine, Department of Internal Medicine III (Author)
  • D. P. Merke - , Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health (NIH), United States Public Health Service (Author)

Abstract

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is classified into three types based on disease severity: classic salt-wasting, classic simple virilizing, and nonclassic. Adrenomedullary dysplasia and epinephrine deficiency have been described in classic CAH, resulting in glucose dysregulation. Our objective was to investigate adrenomedullary function in nonclassic CAH and to evaluate adrenomedullary function according to disease severity. Adrenomedullary function was evaluated in response to a standardized cycle ergonometer test in 23 CAH patients (14 females, age 938 years; 6 salt-wasting, 7 simple virilizing, 5 nonclassic receiving glucocorticoid treatment, 5 nonclassic not receiving glucocorticoid), and 14 controls (7 females, age 1238 years). Epinephrine, glucose, and cortisol were measured at baseline and peak exercise. CAH patients and controls were similar in age and anthropometric measures. Patients with nonclassic CAH who were not receiving glucocorticoid and controls experienced the expected stress-induced rise in epinephrine, glucose, and cortisol. Compared to controls, patients with all types of CAH receiving glucocorticoid had impaired exercise-induced changes in epinephrine (salt-wasting: p=0.01;simple virilizing: p=0.01; nonclassic: p=0.03), and cortisol (salt-wasting: p=0.004; simple virilizing: p=0.006; nonclassic: p=0.03). Salt-wasting patients displayed the most significant impairment, including impairment in glucose response relative to controls (p=0.03). Hydrocortisone dose was negatively correlated with epinephrine response (r=0.58; p=0.007) and glucose response (r=0.60; p=0.002). The present study demonstrates that untreated patients with nonclassic CAH have normal adrenomedullary function. The degree of epinephrine deficiency in patients with CAH is associated with the severity of adrenocortical dysfunction, as well as glucocorticoid therapy.

Details

Original languageEnglish
Pages (from-to)607-612
Number of pages6
JournalHormone and metabolic research
Volume42
Issue number8
Publication statusPublished - 2010
Peer-reviewedYes

External IDs

PubMed 20446239

Keywords

Sustainable Development Goals

Keywords

  • adrenal cortex, adrenal medulla, epinephrine, glucocorticoids