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Primary Adrenal Insufficiency

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106 Commerce Street, Suite 105 Lake Mary, FL 32746 TEL: 407.878.7606 • FAX: 407.878.7611 Order additional copies at GuidelineCentral.com Copyright © 2016 All rights reserved Disclaimer is Guideline attempts to define principles of practice that should produce high-quality patient care. It is applicable to specialists, primary care, and providers at all levels. is Guideline should not be considered exclusive of other methods of care reasonably directed at obtaining the same results. e ultimate judgment concerning the propriety of any course of conduct must be made by the clinician aer consideration of each individual patient situation. Neither IGC, the medical associations, nor the authors endorse any product or service associated with the distributor of this clinical reference tool. ESPAI16102a Abbreviations 11β-HSD2, 11β-hydroxysteroid dehydrogenase type 2; AHC, adrenal hypoplasia congenita; AI, adrenal insufficiency; APECED, autoimmune polyendocrinopathy- candidiasis-ectodermal dystrophy; CMV, cytomegalovirus; CTLA-4, cytotoxic T lymphocyte- associated protein 4; CYP, cytochrome P; D10W, 10% dextrose solution; D25W, 25% dextrose solution; ES, Endocrine Society; HSD, hydroxysteroid dehydrogenase; IMAGe, intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenital, genital abnormalities; MC2R, melanocortin 2 receptor gene; MCM4, minichromosome maintenance-deficient 4; MRAP, melanocortin receptor accessory protein; NNT, nicotinamide nucleotide transhydrogenase; StAR, steroidogenic acute regulatory protein; TXNRD2, thioredoxin reductase; VLCFA, very long chain fatty acid Source Bornstein SR, Allolio B, Arlt W, Barthel A, Don-Wauchope A, Hammer GD, Husebye ES, Merke DP, Murad MH, Stratakis CA, Torpy DJ. Diagnosis and Treatment of Primary Adrenal Insufficiency: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2016;101(2):364-89. Grading System Strength of Recommendation 1 = strong 2 = weak U = ungraded Quality of Evidence ⊕⊕⊕⊕ = high ⊕⊕⊕ = moderate ⊕⊕ = low ⊕ = very low

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