%0 Journal Article
%T Blood Pressure and Left Ventricular Characteristics in Young Patients with Classical Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency
%A Graziamaria Ubertini
%A Carla Bizzarri
%A Armando Grossi
%A Fabrizio Gimigliano
%A Lucilla Rav¨¤
%A Danilo Fintini
%A Marco Cappa
%J International Journal of Pediatric Endocrinology
%D 2010
%I BioMed Central
%R 10.1155/2009/383610
%X The 21 hydroxylase deficiency is the most common enzyme defect causing congenital adrenal hyperplasia (CAH). It is distinguished by the severity of the enzyme defect, in classical and non classical form. Approximately 70% individuals with classical CAH are affected by the more severe salt-wasting (SW) form, characterized by androgen hyper secretion and impaired synthesis of both glucocorticoids (GCs) and mineralcorticoids (MCs). About 20%¨C30% patients are affected by the milder simple virilizing (SV) form, where the excessive androgen production results in virilization, but the synthesis of MCs is sufficient to avoid salt wasting crises. The non classical form is characterized by later and milder signs of androgen excess as precocious pubarche [1].The treatment of CAH is based on GCs therapy, in order to suppress the androgen production trough the inhibition of CRH and consequently ACTH. The therapy must be balanced to avoid poor linear growth, obesity and hypertension.Several factors may interfere with the physiological control of BP in patients affected by CAH due to 21 hydroxilase deficiency: (a) over treatment with GCs and MCs; (b) elevated GCs levels that affect the MCs receptor (MR); (c) impaired adrenomedullary function, with a negative impact on sympathetic control of BP; (d) obesity itself [2¨C5].An alteration in endothelial function has been demonstrated in GCs excess. Dexamethasone induces overproduction of reactive oxygen species causing dysregulation of the endothelial function. A reduction of nitric oxide levels, with a reduction of arteriolar vasodilatation has been demonstrated in GCs-induced hypertension [6].MR binds progesterone, deoxycorticosterone (DOC), corticosterone, cortisol and aldosterone with a similar high affinity, but only DOC and aldosterone are full agonists of the MR. Corticosterone and cortisol have lower transcriptional activity on the MR, and elevated levels of these steroids are necessary to affect the MR. Hypertension in Cushing
%U http://www.ijpeonline.com/content/2009/1/383610