ADAMTS13 and hyperinsulinemic hypoglycemia, familial, 4: The original diagnostic score we established, based on the differences we identified between patients with ADAMTS13 deficiency and those with a detectable ADAMTS13 activity (≥20%) (ie, serum creatinine level, platelet count and, to a lesser extent, ANA positivity) may therefore rapidly identify patients with acquired, immune-mediated ADAMTS13 dysfunction.