Fatal relapse of thrombotic thrombocytopaenic purpura after cardiac surgery in patient with congenital absence of vWF-cleaving protease activity.
نویسندگان
چکیده
conventional assays of serum creatinine in the future and the MDRD formula has recently been re-calibrated to IDMStraceable serum creatinine values [4]. Until there is widespread adoption of IDMS creatinine analysis, we believe the four-variable MDRD formula represents an improvement compared with serum creatinine alone for detection of chronic kidney disease. As with any first order screening test, there is a trade-off of higher sensitivity for lower specificity. Thus, whilst the MDRD GFR is imperfect, we would prefer to choose a relative over-estimation of lowGFR, rather than risk missing a significant number of patients who do have established CKD, but who would be overlooked when using conventional biochemical parameters.
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and markers of haemolysis are not routinely ordered in our intensive care unit; this fact, together with the not unusual fall of platelets in patients after cardiac surgery, resulted in delayed recognition of TTP relapse. This case clearly demonstrates that every postoperative decrease in the number of platelets in a patient with TTP should be considered as a relapse and calls for intensive med...
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Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 is associated with the development of thrombotic thrombocytopenic purpura (TTP). A case of congenital TTP that was previously reported to have normal ADAMTS13 activity was analyzed at the molecular level. Reanalysis of plasma VWF cleaving protease activity using a different assay revealed that the patient had less than 0.1 U/L...
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In patients with thrombotic thrombocytopenic purpura (TTP), excessive intravascular platelet aggregation has been associated with appearance in plasma of unusually large von Willebrand factor (vWF) multimers. These extremely adhesive vWF multimers may arise due to deficiency of a "depolymerase" cleaving vWF to smaller molecular forms, either by reducing the interdimeric disulfide bridges or by ...
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The present case report described a patient with thrombotic thrombocytopenic purpura (TTP), who presented to the China-Japan Union Hospital of Jilin University (Changchun, China), and the changes in plasma von Willebrand factor (vWF) and vWF cleaving protease (vWF-cp) observed during treatment. Computed tomography and diffusion-weighted magnetic resonance (MR) imaging of the brain, cerebral MR ...
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Retrospective studies of patients with thrombotic microangiopathies (TMAs) have shown that a deficient activity of von Willebrand factor (vWF)-cleaving protease is involved in thrombotic thrombocytopenic purpura (TTP) but not in the hemolytic-uremic syndrome (HUS). To further analyze the relevance of this enzymatic activity in TMA diagnosis, a 20-month multicenter study of vWF-cleaving protease...
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ورودعنوان ژورنال:
- Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
دوره 21 6 شماره
صفحات -
تاریخ انتشار 2006