TY - CHAP M1 - Book, Section TI - Adrenal Insufficiency A1 - White, Perrin C. A1 - Sarafoglou, Kyriakie A2 - Sarafoglou, Kyriakie A2 - Hoffmann, Georg F. A2 - Roth, Karl S. PY - 2017 T2 - Pediatric Endocrinology and Inborn Errors of Metabolism, 2e AB - Primary adrenal insufficiency (PAI) results from destruction or dysfunction of the adrenal cortex; 50% to 70% of cases presenting in infancy or childhood represent specific enzymatic defects in corticosteroid biosynthesis, collectively termed congenital adrenal hyperplasia (CAH; see Chapter 29). CAH occurs in 1:16,000 newborns in most populations, corresponding to an incidence of ~1/300,000 if counting all children younger than 18 years. Therefore the incidence of other causes of adrenal insufficiency must be similar to or slightly less than this. Approximately half of the remaining non-CAH cases are caused by autoimmune adrenalitis (Table 28-1). We begin this section with a general discussion of primary adrenal insufficiency, and then consider specific etiologies. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/04/19 UR - accesspediatrics.mhmedical.com/content.aspx?aid=1140319277 ER -