Evaluation of adrenomedullary function in patients with congenital adrenal hyperplasia.
OBJECTIVE
Congenital adrenal hyperplasia (CAH) is characterized by adrenal insufficiency with or without salt wasting. It is also accompanied by adrenomedullary hypofunction. The aim of the present study was to investigate adrenomedullary function in patients with CAH due to 21-hydroxylase and 11beta-hydroxylase deficiencies and in age-matched normal subjects.


METHODS
We measured plasma catecholamines (epinephrine and norepinephrine) and urine metanephrine in 44 patients with CAH, 32 due to 21-hydroxylase deficiency (17 patients with the salt-wasting form and 15 patients with the simple virilizing form), and 12 due to 11beta-hydroxylase deficiency, and in 25 healthy controls.


RESULTS
Plasma epinephrine and urine metanephrine levels were significantly higher in the controls than in patients with CAH (p = 0.02 and p < 0.001, respectively). Plasma norepinephrine levels were significantly lower in the controls than in patients with CAH (p < 0.001). Interestingly, patients with the salt-wasting form had lower norepinephrine levels in comparison to the other subgroups of CAH.


CONCLUSIONS
Despite the fact that CAH patients have insufficient epinephrine secretion, these patients have the ability to increase compensatory norepinephrine. However, this increase is much lower in patients with the salt-wasting form. These findings need to be confirmed by other studies.
Department of Pediatrics, Faculty of Medicine, Trakya University, Edirne, Turkey. ftutunculer@yahoo.com
Filiz
F
Tutunculer
Nurcin
N
Saka
Selda Can
SC
Arkaya
Semra
S
Abbasoglu
Firdevs
F
Bas
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000235:genetics / Q000503:physiopathology*; D000313:Adrenal Medulla / Q000503:physiopathology*; D000328:Adult; D016022:Case-Control Studies; D002648:Child; D004837:Epinephrine / Q000097:blood; D005260:Female; D006801:Humans; D008297:Male; D008676:Metanephrine / Q000652:urine; D009638:Norepinephrine / Q000097:blood; D010641:Phenotype; D012964:Sodium / Q000652:urine; D013252:Steroid 11-beta-Hydroxylase / Q000235:genetics; D013255:Steroid 21-Hydroxylase / Q000235:genetics
D008676:Metanephrine; D012964:Sodium; D013255:Steroid 21-Hydroxylase; D013252:Steroid 11-beta-Hydroxylase; D009638:Norepinephrine; D004837:Epinephrine