Primary Adrenal Insufficiency

Primary Adrenal Insufficiency

• • Infants, selected children and adults → 17-OH-Progesterone
• • All > 6 months age → 21-OH-Antibody

17-OH-Progesterone

• • Positive (+)^a → CAH
• • Negative (-) → Genetic Syndromes (Rare CAH, AHC) - Negative (-) → Idiopathic PAI

21-OH-Antibody

• • Positive (+) → Autoimmune AI; Consider APS-1, APS-2
• • Negative (-) → CT Adrenals: - Negative (-) → Genetic Syndromes (Rare CAH, AHC) - Positive (+) → Infiltrative Disease, Adrenal Hemorrhage, Infections, Malignant Tumors
• • Negative (-)^b → VLCFA (Males): - Positive (+) → Adrenoleukodystrophy