Both P1 and P2 suffered from CMC, as reported in five of the seven patients with AR NIK deficiency and one patient with AR partial IKK-α deficiency, but not in patients with AD inborn errors of NF-κB2 (6, 9, 18, 19, 40, 46, 47). The gene discussed is CHUK; the disease is hyperinsulinemic hypoglycemia, familial, 4.