Due to the heterogeneity of the individual IEMs, it cannot be determined from the results whether it is the restrictive therapy, as in patients with PKU, or the potential for metabolic emergencies, as in patients with Medium-chain-acyl-CoA-dehydrogenase-deficiency, that restricts parents the most. Here, ACADM is linked to hyperinsulinemic hypoglycemia, familial, 4.