Pathophysiology and Management of Acquired von Willebrand Syndrome
Autor: | Dominder Kaur, Sarah H. O'Brien |
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Rok vydání: | 2020 |
Předmět: |
Antifibrinolytic
biology business.industry medicine.drug_class medicine.disease Bioinformatics Pathophysiology Acquired von Willebrand syndrome Immune system Von Willebrand factor Coagulation hemic and lymphatic diseases Circulatory system biology.protein Von Willebrand disease Medicine business circulatory and respiratory physiology |
Zdroj: | Pediatric Bleeding Disorders ISBN: 9783030316600 |
Popis: | Acquired von Willebrand disease (AVWD) is a defect of coagulation occurring secondary to other pathologies, leading to abnormal von Willebrand factor (VWF) function. Multiple pathophysiological mechanisms are implicated, and broadly, these are categorized into immune (e.g., antibody-related clearance) and nonimmune causes (e.g., shear-related loss of high molecular weight multimers in complex cardiac anatomy or in patients on circulatory support devices). High index of suspicion is required for correct diagnosis. Evaluation consists of VWF activity and antigen levels, as well as multimer testing. Therapy should be geared toward correcting the underlying issue, as once the offending mechanism is removed, VWF levels go back to normal. VWF replacement and adjunctive therapies can be utilized to control bleeding in the interim. |
Databáze: | OpenAIRE |
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