8/46 patients who had prominent fevers and/or systemic inflammation had testing for monogenic autoinflammation: mevalonate kinase deficiency (7/8), cryopyrin-associated periodic fever (4/8), familial Mediterranean fever (8/8) and NOD2 (1/8) mutations. The gene discussed is NOD2; the disease is hyperinsulinemic hypoglycemia, familial, 4.