hrp0089p1-p005 | Adrenals and HPA Axis P1 | ESPE2018

Elevated Concentrations of Adrenal Steroid Precursors with Glucocorticoid Activity Might Prevent Addisonian Crisis in Untreated Patients with Classic Congenital Adrenal Hyperplasia

Engels Manon , Pijnenburg-Kleizen Karijn , Utari Agustini , Faradz Sultana , Heuvel Joop , Herwaarden Teun van , Span Paul , Sweep Fred , Grinten Hedi Claahsen-van der

Introduction: Congenital adrenal hyperplasia (CAH) is most often caused by 21-hydroxylase deficiency (21OHD: 95%) or by 11-hydroxylase deficiency (11OHD). Classic CAH results in impaired cortisol production and consequently elevated ACTH concentrations leading to chronic adrenal stimulation with strongly elevated adrenal steroid precursors before the enzymatic defect. In contrast to other forms of adrenal insufficiency, some untreated classic CAH patients seem to have less cli...