However, since patient 3bINP-036 did not have the typical biochemical profile of holocarboxylase synthetase deficiency at diagnosis, which consists of elevated C5-OH, a urine organic acid profile with elevated lactic acid, 3-OH isovaleric, 3-OH propionic, 3-MCC, methylcitric acid, and tiglylglycine [35], further studies are warranted to confirm or discard this disease. This evidence concerns the gene HLCS and hyperinsulinemic hypoglycemia, familial, 4.