Several diseases were associated with periodic fever syndrome, such as cryopyrin-associated periodic syndromes (caused by NLRP3), familial Mediterranean fever (caused by MEFV), tumor necrosis factor receptor-associated periodic syndrome (caused by TNFRSF1A), and mevalonate kinase deficiency (caused by MVK) (26). Here, MVK is linked to hyperinsulinemic hypoglycemia, familial, 4.