Family consanguinity was reported in 11 patients overall (13.9%; in 2 patients with OTC Deficiency, 4 patients with CPS1 Deficiency, 4 patients with NAGS Deficiency and 1 patient with ORNT1 Deficiency) (Table 1). The gene discussed is SLC25A15; the disease is hyperinsulinemic hypoglycemia, familial, 4.