LCHAD deficiency refers to the loss of 3-hydroxyacyl-CoA dehydrogenase activity alone, whereas mitochondrial trifunctional protein deficiency refers to the loss of 3-hydroxyacyl-CoA dehydrogenase activity and the other 2 long-chain acyl-CoA β-oxidation enzymes activities. This evidence concerns the gene EHHADH and hyperinsulinemic hypoglycemia, familial, 4.