The four IEBAM disorders that can be treated via oral administration of unconjugated primary bile acids such as CA and CDCA are HSD3B1 deficiency (13), SRD5B1 deficiency (14), and CYP7B1 deficiency (15), which involve abnormal modifications of the cholanic acid backbone, and CYP27A1 deficiency (16), which involves abnormal side chain modifications. Here, AKR1D1 is linked to hyperinsulinemic hypoglycemia, familial, 4.