XIAP and hyperinsulinemic hypoglycemia, familial, 4: In light of the variable manifestation of the phenotype (some patients with IBD have not presented with the classical immunodeficiency phenotypes) the intestinal inflammation in these patients might either be regarded as a manifestation of the immunodeficiency (such as XIAP deficiency, XLP1, or chronic granulomatous disease) or as a form of monogenic IBD.