In addition, Chron’s-like enteropathy, a feature shared by many other IEI (i.e., Cytotoxic T-Lymphocyte Antigen 4 (CTLA-4) haploinsufficiency, immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX), Lipopolysaccharide-Responsive, Beige-Like Anchor Protein (LRBA) deficiency) [30,31], was reported in one of our patients and initially responded to first-line treatment [32]. This evidence concerns the gene CTLA4 and hyperinsulinemic hypoglycemia, familial, 4.