Table 4. IEM acute illness protocol template for PCP.

IEM disorders: OA, mitochondrial disorders, GSD, UCD

Common illness/conditions: viral/bacterial infections; surgical procedures

Medical attention (PCP, ER): STAT

STAT laboratory tests: BMP, urinalysis (+/−). CBC, NH3 (ammonia)

STAT IV access: D10 W + 0.5 normal saline at 1.5 times maintenance (per age guidelines)

Feeding: NPO with above IV fluids until hemodynamically stable

Call: local friendly biochemical/IEM specialist, intensive care unit

PCP, primary care physician; IEM, inborn error of metabolism; OA, organic aiduria; GSD, glycogen storage disease; UCD, urea cycle disorders; BMP, basic metabolic panel; CBC, complete blood count; D10W, dextrose solution containing 10% glucose.