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Primary adrenal insufficiency

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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Guidelines

1.Screening and diagnosis

Indications for testing: 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).
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2.Diagnostic investigations

Corticotropin stimulation test: 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

3.Medical management

Corticosteroid replacement: administer corticosteroid therapy to all patients with confirmed PAI.
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  • Mineralocorticoid replacement

  • DHEA replacement

  • Management of adrenal crisis

4.Nonpharmacologic interventions

Salt intake: advise all patients with confirmed aldosterone deficiency not to restrict their salt intake.
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5.Specific circumstances

Pregnant patients: consider using hydrocortisone over cortisone acetate, prednisolone, or prednisone, in pregnant women with PAI.
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  • Pediatric patients

6.Patient education

Genetic counseling: consider genetic counseling for patients with PAI due to monogenic disorders.
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  • Stress dose corticosteroids

  • Corticosteroid injection kits

7.Preventative measures

Prevention of adrenal crisis: consider adjusting corticosteroid dose according to severity of illness or magnitude of the stressor to prevent adrenal crisis.
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8.Follow-up and surveillance

Serial clinical assessment: 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

9.Quality improvement

Medical alert bracelet: 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.