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Summary
Etiology
Pathophysiology
Clinical features
Diagnostics
Treatment
Approach
Glucocorticoids
Mineralocorticoids
Glucocorticoids
Steroid replacement
Glucocorticoids [1]
Agents
[1]
Hydrocortisone
[1]
Cortisone acetate
[1]
Prednisolone
Considerations
The total daily replacement dose should be given in divided doses, with the highest dose given in the
morning to mimic diurnal fluctuations.
Educate patients about increasing their glucocorticoid dose according to outpatient sick day rules.
Mineralocorticoids [1]
Considerations
A temporary increase may be advised if increased sweating is anticipated (e.g., in the summer)
Monitor for adequate replacement and consider a dose reduction if hypertension develops. [1]
Edema
Androgens [1]
Consider treatment in anatomically female patients with low libido, depressive symptoms, and low
energy levels.
[1]
Agent: DHEA
Considerations
Results of studies investigating the positive effects of DHEA have been mixed and data on long-term
outcomes is lacking. [1]
Infiltration of the adrenal glands: Treat malignancy, e.g., via tumor resection.
Patients who do not require hospitalization can be taught self-treatment plans, known as sick day rules.
If the dose of glucocorticoids is not increased during periods of stress, the patient may
develop an adrenal crisis!
Adhere to any preexisting protocol/care plan by the patient's endocrinologist. The following
recommendations can apply to all patients on adrenosuppressive doses of glucocorticoids.
Febrile illness: Double the usual oral dose until recovery; consider IV route if the patient is vomiting.
[31]
Critically ill patients: IV hydrocortisone
Perioperative patients
Minor and moderate procedures : usual daily steroid dose PLUS stress-dose hydrocortisone
followed by a return to the usual steroid dose [29]
Major procedure : usual daily steroid dose PLUS stress-dose hydrocortisone followed by
steroid taper as needed [29]
The following rules should be taught to patients with primary adrenal insufficiency so that they can self-
administer glucocorticoids in times of illness/physiological stress:
Sick day rule one: circumstances requiring double the routine oral glucocorticoid dose
Fever
Severe illness
Trauma
Perioperative period
Adrenal crisis is an acute, severe glucocorticoid deficiency that requires immediate emergency
treatment.
Precipitating factors for adrenal crisis
Other infections
Perioperative period
Psychological stress
Hypotension, shock
Fever
Vomiting, diarrhea
Diagnosis [12][19][32]
Based on clinical suspicion: Maintain a low threshold for diagnosis in at-risk patients , especially
those with shock refractory to fluids and/or vasopressors. [33]
Hypoglycemia
[34]
Normal anion gap metabolic acidosis
ACTH stimulation test: diagnostic confirmation once the patient has been stabilized (see
“Diagnostics”)
Management [1][19]
Consider adding mineralocorticoid replacement, e.g., fludrocortisone (off-label) for the following:
[31]
Fluid resuscitation
Further management should be guided by clinical response (see “Intravenous fluid therapy” and “
Shock”).
The 5 S’s of adrenal crisis treatment are Salt (0.9% saline), Sugar (50% dextrose), Steroids
(100 mg hydrocortisone IV once, then 200 mg over 24 hours), Support (normal saline to
correct hypotension and electrolyte abnormalities), and Search (for the underlying
disorder).
Monitor vitals, blood glucose, and urine output every 1–2 hours.
Definition: a set of conditions characterized by autoimmune disease that causes multiple endocrine
deficiencies, which affect the hormone-producing (endocrine) glands
Types
Most commonly
Hypoparathyroidism
Type 2 (APS-2, Schmidt syndrome): defined by the occurrence of primary adrenal insufficiency with
thyroid autoimmune disease and/or type 1 diabetes mellitus [38]
Most commonly
References:[39][40]
References
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