NORMAL RANGE40-130% ASSOCIATED TESTINGADAMTS13 Gene Sequencing; aHUS Genetic Panel; TMA-Complete Genetic Panel REFERENCES 1. Moake, MD. Thrombotic microangiopathies. NEJM. 2002;347(8):589-600. 2. Tsai, HM. Autoimmune Thrombotic Microangiopathy: Advances in Pathogenesis, Diagnosis, and Management...
Normal range for VWF activity, 50% to 187%. LL, lower limit of normal; UL, upper limit of normal. Cost-saving potential This study used a multimodal cost analysis, as indicated in the Methods section and the legend to supplemental Table 2.13, 14 A global analysis summarizes the cost-...
Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient ...
Dotted red lines denote the upper and lower limit of the local reference range, with green shaded area falling within normal limits. Data are presented as median and the IQR. Comparisons between groups were assessed by the Mann-Whitney U test. Correlations between plasma VWF:Ag levels and (E...
Total reaction volumes of 100 µL containing ADAMTS13-BirA* or BirA* (100 nM) or no protein (PBS—phosphate-buffered saline: 137 mM NaCl, 2.7 mM KCl, 10 mM Na2HPO4, and 1.8 mM KH2PO4), containing 90 µL citrated pooled normal plasma (PNP), ATP (1 mM / hour) and biotin (...
(a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 ADAMTS13 mutations resulting in familial TTP have been reported. Considerable progress has also been realized toward understanding the role of ADAMTS13 in normal hemostasis, as well as the mechanisms by which...
Mixing plasma ofTTP patients with normal blood (1:3) yielded a 1.6 to 2-fold increase of platelet adhesion under flow compared to mixing normal plasma with normal blood, at shear rate range of 1,800–2,500s1. Maximal increase of platelet adhesion was observed under 2,050s1. Under these...
protease)andfactorH.Testresultsforantibodiesagainstbothproteinswerepositive.Whileplasmaexchange therapywascontinued,rituximabwasgivenonceweeklyforfourconsecutiveweeks.Afterthelastdose, thrombocytesandactivitiesofADAMTS-13andfactorHincreasedintothenormalrange.Ourpatientimproved ...
In transplant-associated TMA, 8/16 patients not responding to first-line treatment received eculizumab due to elevated C5b-9 levels (median 353 ng/ml, range 281–1252 ng/ml) and achieved TMA resolution. In conclusion, our real-world data confirm that ADAMTS13, complement testing, and...
The domain interfaces in MDTCS are considerable, which provides mechanistic insight into how this long-range structural cross-talk may be manifest. Download: Download high-res image (597KB) Download: Download full-size image Figure 7. Mode of action of ADAMTS13. (A) Under normal circumstances,...