Figure 1.

Recommended endocrine surveillance for presymptomatic infants and children with ALD. Infants identified through NBS should have an early morning ACTH and cortisol level at the time of diagnosis to screen for glucocorticoid deficiency, which usually precedes mineralocorticoid deficiency (10, 53). Any ACTH level of > 100 pg/mL and/or any morning cortisol level < 5 ug/dL is considered abnormal and warrants further diagnostic evaluation (53). *In the case of a stimulated peak cortisol level of < 18 μg/dL, stress dose steroids should be started, but daily glucocorticoid replacement can be considered as clinically indicated. Abbreviation: PRA, plasma renin activity.