Aside from a positive newborn screen, other expected supportive laboratory findings in patients with CPT1A deficiency include hypoketotic hypoglycemia, elevated AST/ALT levels (2–10 times the upper limit of normal), hyperammonemia, elevated total serum carnitine, and elevated C6–12 species on urine organic acids [8]. Here, CPT1A is linked to hyperinsulinemic hypoglycemia, familial, 4.