Acquired von Willebrand Syndrome in Pediatric Extracorporeal Membrane Oxygenation Patients: A Single Institution’s Experience*

Conclusions: Acquired von Willebrand syndrome is a common but under recognized disorder in pediatric extracorporeal membrane oxygenation patients. The clinical implications of this disorder on bleeding and its potential treatments are still unclear.
Source: Pediatric Critical Care Medicine - Category: Pediatrics Tags: Extracorporeal Support Source Type: research

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Condition:   Bleeding Interventions:   Drug: Wilate;   Other: Placebo Sponsor:   University Hospital, Basel, Switzerland Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Abstract Platelets play a major role in primary hemostasis, where activated platelets form plugs to stop hemorrhaging in response to vessel injuries. Defects in any step of the platelet activation process can cause a variety of platelet dysfunction conditions associated with bleeding. To make an accurate diagnosis, constitutional platelet dysfunction (CPDF) should be considered once von Willebrand disease and drug intake are ruled out. CPDF may be associated with thrombocytopenia or a genetic syndrome. CPDF diagnosis is complex, as no single test enables the analysis of all aspects of platelet function. Furthermor...
Source: Hamostaseologie - Category: Hematology Authors: Tags: Hamostaseologie Source Type: research
AbstractAdvancements in the design and functionality of continuous flow left ventricular assist devices (CF-LVADs), as well as a limited number of donor hearts, have resulted in an increased utilization of this therapy among advanced heart failure (HF) patients. Despite these advancements, gastrointestinal bleeding (GIB) remains a common complication after CF-LVAD implantation. The mechanism of GIB in these patients is complex and includes a combination of angiodysplasia, platelet dysfunction, acquired von Willebrand disease, and a variety of patient-specific factors including advanced age and history of GIB. Several pharm...
Source: Heart Failure Reviews - Category: Cardiology Source Type: research
DiscussionAVWS secondary to cardiovascular diseases occurs from a selective loss of the largest multimers of VWF due to high shear forces in the blood circulation. Although it is established that stenotic valvular lesions are associated with AVWS, there have only been rare reports of regurgitant lesions leading to AVWS. We successfully treated this patient with perioperative supplementation of VWF and factor VIII.
Source: Journal of Cardiac Surgery - Category: Cardiovascular & Thoracic Surgery Authors: Tags: CASE REPORT Source Type: research
Abstract Von Willebrand disease (VWD) is the most common inherited bleeding disorder and is mainly caused by dominant-negative mutations in the multimeric protein von Willebrand factor (VWF). These mutations may either result in quantitative or qualitative defects in VWF. VWF is an endothelial protein that is secreted to the circulation upon endothelial activation. Once secreted, VWF multimers bind platelets and chaperone coagulation factor VIII in the circulation. Treatment of VWD focuses on increasing VWF plasma levels, but production and secretion of mutant VWF remain uninterrupted. Presence of circulating muta...
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: Thromb Haemost Source Type: research
Semin Thromb Hemost DOI: 10.1055/s-0040-1714202There is increasing evidence that portal hypertension plays a major role in bleeding risk during orthotopic liver transplantation (OLT). We investigated the association between preoperative blood levels of von Willebrand factor (VWF) and soluble CD163 (sCD163), which are established markers of portal hypertension, and blood loss and transfusion requirements during OLT. We measured levels of VWF and sCD163 in preoperative serum samples of 168 adult patients undergoing a primary OLT between 1998 and 2012. Preoperative levels of VWF and sCD163 correlated with the model of end-sta...
Source: Seminars in Thrombosis and Hemostasis - Category: Hematology Authors: Tags: Review Article Source Type: research
CONCLUSION:  A PK model was developed, describing VWF activity versus time profile after desmopressin administration in patients with VWD or low VWF. Interpatient variability in response was quantified and partially explained. This model is a starting point toward more accurate prediction of desmopressin dosing effects in VWD. PMID: 32746466 [PubMed - as supplied by publisher]
Source: Thrombosis and Haemostasis - Category: Hematology Authors: Tags: Thromb Haemost Source Type: research
The deficiency or abnormal activity of von Willebrand factor, a multi-adhesive protein which binds platelets to exposed subendothelium and carries factor VIII in circulation, is responsible for von Willebrand disease, the most frequent inherited bleeding disorder. Clinical symptoms are characterized by mucous membrane and soft tissue bleeding, bleeding after surgery and rarely joint and gastrointestinal bleeding. Intriguingly, also factor VIII, the protein deficient in hemophilia A, may be variably reduced because VWF stabilizes it into circulation.
Source: Thrombosis Research - Category: Hematology Authors: Tags: Review Article Source Type: research
To review the use of Von Willebrand Factor (VWF) concentrate for treatment of acquired Von Willebrand syndrome (VWS)-related bleeding in adult extracorporeal membrane oxygenation (ECMO) patients and determine if it was associated with improved VWF laboratory parameters.
Source: Journal of Cardiothoracic and Vascular Anesthesia - Category: Anesthesiology Authors: Tags: Original Article Source Type: research
Hemophilia A is an X-linked bleeding disorder caused by a deficiency of factor VIII. Depending on the factor VIII activity in patient's plasma, we can have three different forms of hemophilia A: mild (5–40 IU/dl), moderate (1–5 IU/dl) and severe (
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: CASE REPORTS Source Type: research
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