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Primary adrenal insufficiency
Guidelines
Key sources
The following summarized guidelines for the evaluation and management of primary adrenal insufficiency are prepared by our editorial team based on guidelines from the Endocrine Society (ES 2016).
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Screening and diagnosis
Indications for testing: as per ES 2016 guidelines, obtain diagnostic testing to exclude PAI in acutely ill patients with otherwise unexplained symptoms or signs suggestive of PAI (volume depletion, hypotension, hyponatremia, hyperkalemia, fever, abdominal pain, hyperpigmentation or, especially in children, hypoglycemia).
B
Diagnostic investigations
Corticotropin stimulation test: as per ES 2016 guidelines, perform a corticotropin stimulation test in patients with clinical symptoms or signs suggesting PAI, when the patient's condition and circumstance allow.
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Morning cortisol
Plasma ACTH
Plasma renin and aldosterone
Evaluation for underlying etiology
Medical management
Corticosteroid replacement: as per ES 2016 guidelines, administer corticosteroid therapy to all patients with confirmed PAI.
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Mineralocorticoid replacement
DHEA replacement
Management of adrenal crisis
Nonpharmacologic interventions
Specific circumstances
Pregnant patients: as per ES 2016 guidelines, consider using hydrocortisone over cortisone acetate, prednisolone, or prednisone, in pregnant women with PAI.
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Pediatric patients
Patient education
Genetic counseling: as per ES 2016 guidelines, consider genetic counseling for patients with PAI due to monogenic disorders.
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Stress dose corticosteroids
Corticosteroid injection kits
Preventative measures
Follow-up and surveillance
Clinical follow-up: as per ES 2016 guidelines, consider referring adults and children with PAI to be seen by an endocrinologist or a healthcare provider with endocrine expertise at least annually. Infants should be seen at least every 3 to 4 months.
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Monitoring of corticosteroid replacement
Monitoring of mineralocorticoid replacement
Monitoring of DHEA replacement
Periodic screening for autoimmune diseases
Quality improvement
Medical alert bracelet: as per ES 2016 guidelines, equip all patients with a steroid emergency card and medical alert identification to alert health personnel regarding the need for increased corticosteroid doses in order to avert or treat adrenal crisis, and regarding the need for immediate parenteral steroid treatment in the event of an emergency.
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