Ten of these cases resulted in mortality, with diagnoses including tyrosinemia type 1 (one case), citrullinemia type 1 (three cases), isovaleric aciduria (one case), carnitine-acylcarnitine translocase deficiency (three cases), very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (one case), and multiple acyl-CoA dehydrogenase deficiency (one case). The gene discussed is SLC25A20; the disease is hyperinsulinemic hypoglycemia, familial, 4.