Evaluation of Adrenomedullary Function in Patients with Congenital Adrenal Hyperplasia

Küçük Resim Yok

Tarih

2009

Dergi Başlığı

Dergi ISSN

Cilt Başlığı

Yayıncı

Karger

Erişim Hakkı

info:eu-repo/semantics/closedAccess

Özet

Background/Aims: 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 11 beta-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 11 beta-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. Conclusion: 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. Copyright (C) 2009 S. Karger AG, Basel

Açıklama

Anahtar Kelimeler

Adrenomedullary Function, Congenital Adrenal Hyperplasia, 21-Hydroxylase, 11 Beta-Hydroxylase, Norepinephrine, 21-Hydroxylase Deficiency, Epinephrine Reserve, Turkish Children, Stress, References, Exercise

Kaynak

Hormone Research

WoS Q Değeri

Q4

Scopus Q Değeri

Q1

Cilt

72

Sayı

6

Künye