In one study, the presenting features and clinical outcomes of 16 patients with idiopathic TTP-HUS who had severe ADAMTS13 deficiency were variable and not distinctively different from the 32 patients with idiopathic TTP-HUS who did not have ADAMTS13 deficiency [25]. This evidence concerns the gene ADAMTS13 and hyperinsulinemic hypoglycemia, familial, 4.