8
Treatment
Dehydroepiandrosterone Replacement
Î ES suggests a trial of dehydroepiandrosterone (DHEA) replacement
in women with PAI and low libido, depressive symptoms, and/or
low energy levels despite otherwise optimized glucocorticoid and
mineralocorticoid replacement. (2|⊕⊕
)
Î ES suggests an initial period of 6 months of DHEA replacement. If the
patient does not report a sustained, beneficial effect of replacement
after 6 months, the DHEA should be discontinued. (2|⊕⊕
)
Î ES suggests monitoring DHEA replacement by measuring morning
serum DHEA sulfate (DHEAS) levels (aiming at the mid-normal range)
before the intake of the daily DHEA replacement dose. (2|⊕⊕
)
Treatment During Pregnancy
Î ES suggests that pregnant patients with PAI be monitored for clinical
symptoms and signs of glucocorticoid over- and under-replacement
(eg, normal weight gain, fatigue, postural hypotension or hypertension,
hyperglycemia), with at least one review per trimester. (U)
Î ES suggests that, based on the individual clinical course, an increase
in hydrocortisone dose should be implemented, in particular during
the third trimester. (U)
Î In pregnant women with PAI, ES suggests using hydrocortisone
over cortisone acetate, prednisolone, or prednisone (2|⊕⊕
)
and recommends against using dexamethasone because it is not
inactivated in the placenta. (1|⊕⊕
)
Î ES recommends hydrocortisone stress dosing during the active phase
of labor, similar to that used in major surgical stress. (1|⊕⊕
)
Treatment and Monitoring During Childhood
Î In children with PAI, ES suggests treatment with hydrocortisone in three
or four divided doses (total starting daily dose of 8 mg/m
2
body surface
area) over other types of glucocorticoid replacement therapies, with
doses adjusted according to individual need. (2|⊕⊕
)
Î In children with PAI, ES suggests avoiding synthetic, long-acting
glucocorticoids (eg, prednisolone, dexamethasone). (2|⊕⊕
)
Î ES suggests monitoring glucocorticoid replacement by clinical
assessment, including growth velocity, body weight, blood pressure,
and energy levels. (US)
Î In children with PAI and confirmed aldosterone deficiency, ES
recommends treatment with fludrocortisone (starting dosage, 100
μg/d). For infants, ES recommends sodium chloride supplements in
the newborn period and up to the age of 12 months. (1|⊕⊕
)
