Adrenomedullary function in patients with nonclassic congenital adrenal hyperplasia

Publikation: Beitrag in FachzeitschriftForschungsartikelBeigetragenBegutachtung

Beitragende

  • S. Verma - , Eunice Kennedy Shriver National Institute of Child Health and Human Development, United States Public Health Service (Autor:in)
  • L. Green-Golan - , National Institutes of Health (NIH) (Autor:in)
  • C. Vanryzin - , National Institutes of Health (NIH) (Autor:in)
  • B. Drinkard - , National Institutes of Health (NIH) (Autor:in)
  • S. P. Mehta - , National Institutes of Health (NIH) (Autor:in)
  • M. Weise - , Bundesinstitut für Arzneimittel und Medizinprodukte (BfArM) (Autor:in)
  • G. Eisenhofer - , Institut für Klinische Chemie und Laboratoriumsmedizin, Medizinische Klinik und Poliklinik III (Autor:in)
  • D. P. Merke - , Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health (NIH), United States Public Health Service (Autor:in)

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

OriginalspracheEnglisch
Seiten (von - bis)607-612
Seitenumfang6
FachzeitschriftHormone and metabolic research
Jahrgang42
Ausgabenummer8
PublikationsstatusVeröffentlicht - 2010
Peer-Review-StatusJa

Externe IDs

PubMed 20446239

Schlagworte

Ziele für nachhaltige Entwicklung

Schlagwörter

  • adrenal cortex, adrenal medulla, epinephrine, glucocorticoids