RT Book, Section
A1 White, Perrin C.
A1 Sarafoglou, Kyriakie
A2 Sarafoglou, Kyriakie
A2 Hoffmann, Georg F.
A2 Roth, Karl S.
SR Print(0)
ID 1140319277
T1 Adrenal Insufficiency
T2 Pediatric Endocrinology and Inborn Errors of Metabolism, 2e
YR 2017
FD 2017
PB McGraw-Hill Education
PP New York, NY
SN 9780071773140
LK accesspediatrics.mhmedical.com/content.aspx?aid=1140319277
RD 2024/04/24
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.