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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
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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
