We observed the inhibitor of ADAMTS13 in 83% of patients with severe to moderate ADAMTS13 deficiency, but its inhibitory activity was in a marginal zone between 0.5 and 1.0 BU/mL in most cases except in cases of a TTP patient (2.0 BU/mL) and a patient with severe ADAMTS13 deficiency (3.0 BU/mL) [30]. The gene discussed is ADAMTS13; the disease is hyperinsulinemic hypoglycemia, familial, 4.