Pertaining to outcomes, 3 TMA patients died (3% of patients with severe ADAMTS13 deficiency vs 25% without), 14 patients exacerbated and 6 relapsed at 6 months after the acute event, all from the patient group with ADAMTS13 activity below 10% (Table 4). The gene discussed is ADAMTS13; the disease is hyperinsulinemic hypoglycemia, familial, 4.