C27-bile acids can be detected in plasma and urine from patients with a ZSD as well as in DBP, AMACR, SCPx and ABCD3 deficiency (Ferdinandusse and Houten 2006; Ferdinandusse et al 2009, 2015; Clayton 2011). The gene discussed is DBP; the disease is hyperinsulinemic hypoglycemia, familial, 4.