9
Management and Prevention of Adrenal Crisis in Patients with PAI
Î ES recommends that patients with suspected adrenal crisis should
be treated with an immediate parenteral injection of 100 mg
(50 mg/m
2
for children) hydrocortisone, followed by appropriate
fluid resuscitation and 200 mg (50–100 mg/m
2
for children) of
hydrocortisone/24 hours (via continuous IV therapy or 6 hourly
injection); age- and body surface-appropriate dosing is required in
children (see Table 3). (1|⊕⊕⊕
)
Î If hydrocortisone is unavailable, ES suggests prednisolone as an
alternative. Dexamethasone is the least preferred alternative and
should be given only if no other glucocorticoid is available. (2|⊕⊕
)
Î For the prevention of adrenal crisis, ES suggests adjusting
glucocorticoid dose according to severity of illness or magnitude of
the stressor. (2|⊕⊕
)
Î ES suggests patient education concerning glucocorticoid adjustments
in stressful events and adrenal crisis prevention strategies including
parenteral self- or lay-administration of emergency glucocorticoids. (U)
Î ES recommends that all patients should be equipped with a steroid
emergency card and medical alert identification to inform health
personnel of the need for increased glucocorticoid doses to avert
or treat adrenal crisis and the need of immediate parenteral steroid
treatment in the event of an emergency. (U)
Î ES recommends that every patient should be equipped with a
glucocorticoid injection kit for emergency use and be educated on how
to use it. (U)
Additional Monitoring Requirement
Î ES suggests that adults and children with PAI be seen by an
endocrinologist or a healthcare provider with endocrine expertise at
least annually. Infants should be seen at least every 3–4 months. (U)
Î ES suggests that PAI patients be evaluated annually for symptoms and
signs of over- and under-replacement. (U)
Î ES suggests periodic screening for autoimmune diseases known to be
more prevalent in PAI patients in whom autoimmune origin of PAI has
not been excluded. The optimal frequency of screening is unknown
but can be done annually. These conditions include thyroid disease,
diabetes mellitus, premature ovarian failure, celiac disease, and
autoimmune gastritis with vitamin B12 deficiency. (2|⊕⊕
)
Î ES suggests patient education about increasing the dosage of
glucocorticoids during intercurrent illness, fever, and stress. This
education includes identification of precipitating symptoms and signs
and how to act in impending adrenal crisis. (U)
Î ES suggests genetic counseling for patients with PAI due to monogenic
disorders. (U)
