Despite the registration of Cholbam® for this broad spectrum of indications in the U.S., the European variant of Cholbam®—Kolbam®—was only granted EU market authorization for three specific SEDs: sterol 27-hydroxylase deficiency (cerebrotendinous xanthomatosis, CTX), alpha-methylacyl-CoA racemase (AMACR) deficiency, and cholesterol 7-alpha-hydroxylase (CYP7A1) defect [24]. This evidence concerns the gene CYP7A1 and hyperinsulinemic hypoglycemia, familial, 4.