SLEs may be observed in carbamoyl phosphate synthetase I (CPS1) deficiency, ornithine transcarbamylase (OTC) deficiency and citrullinemia [3,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65,66]. The gene discussed is OTC; the disease is hyperinsulinemic hypoglycemia, familial, 4.