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ea0063gp187 | Adrenal and Neuroendocrine - Clinical | ECE2019

Primary adrenal insufficiency in children: results from a large nationwide cohort

Improda Nicola , Esposito Andrea , Capalbo Donatella , Cappa Marco , Ferro Giusy , Balsamo Antonio , Baronio Federico , Russo Gianni , Lascio Alessandra Di , Greggio Nella Augusta , Tosetto Ilaria , Valenzise Mariella , Wasniewska Malgorzata , Maghnie Mohamad , Radetti Giorgio , Longhi Silvia , Betterle Corrado , Salerno Mariacarolina

Background: Primary Adrenal Insufficiency (PAI) is a rare life-threatening disorder. Data on etiology and outcome of PAI in childhood are scanty, with the exception of Congenital Adrenal Hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD). The aim of our study is to evaluate etiology, morbidity and long-term outcome of PAI in a large cohort of children and characterize clinical presentation in subjects with PAI not due to 21OHD CAH.Material and me...