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References in periodicals archive ?
Two of the 11 cases were excluded after being identified as the congenital form of TTP (Upshaw-Schulman syndrome) with ADAMTS13 activity levels of 0% and 7% in combination with negative ADAMTS13 inhibitor assays.
(4,22,23) Clinical criteria remain inadequate for definitive diagnosis of TTP in children and are incapable of differentiating diseases with overlapping presentations such as atypical hemolytic uremic syndrome and Upshaw-Schulman syndrome. (24) The treatments of atypical hemolytic uremic syndrome, UpshawSchulman syndrome, and A-TTP (eculizumab, (25) plasma infusion, and TPE, respectively) are significantly different, requiring rapid results from an ADAMTS13 activity assay to aid in differentiating these diagnoses in the neonatal and pediatric populations.