hrp0098fc8.5 | Adrenals and HPA Axis 2 | ESPE2024

Free cortisol and free 21-deoxycortisol in the clinical evaluation of adrenal insufficiency in congenital adrenal hyperplasia

P.H. Adriaansen Bas , Utari Agustini , J. Olthaar Andre , C.B.M. van der Steen Rob , J. Pijnenburg-Kleizen Karijn , Berkenbosch Lizanne , N. Span Paul , C.G.J. Sweep Fred , L. Claahsen-van der Grinten Hedi , E. van Herwaarden Antonius

Background: Some patients with classic congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) survive without glucocorticoid treatment, despite low total cortisol levels. This may be caused by accumulating precursor steroids such as 21-deoxycortisol (21DF), which could contribute to the glucocorticoid activity. In addition, these precursor steroids may decrease the cortisol-protein binding, thereby increasing the free (biologically activ...