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      Acquired von Willebrand Syndrome Complicating Nephrotic Syndrome: A Case of a Patient With Membranous Nephropathy


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          Acquired von Willebrand syndrome (AVWS) is a rare clinical entity presenting with heterogeneous hemorrhagic manifestations, although some subsets of patients with AVWS may be asymptomatic until they are exposed to major trauma, an invasive procedure, or surgery. We herein report one such case in a 73-year-old male patient with nephrotic syndrome with a prolonged active partial thromboplastin time. We initially did not deal with this distinct abnormal clotting profile seriously, but persistent bleeding after a retroperitoneoscopic-assisted renal biopsy that allowed us to ascribe his nephrotic syndrome to membranous nephropathy fortuitously led to the discovery of concurrent AVWS. We feel that an accurate and prompt diagnosis as well as awareness of the disease remain a challenge for physicians and therefore strongly recommend the further accumulation of experiences similar to our own in a prospective manner. This report underscores the pitfalls associated with determining the bleeding risk, including an insufficient assessment and improper weighting of an abnormal clotting profile prior to the invasive procedure. Several management concerns that emerged in the current case are also discussed.

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          Paucity of studies to support that abnormal coagulation test results predict bleeding in the setting of invasive procedures: an evidence-based review.

          The literature was systematically reviewed to determine whether a prolonged prothrombin time or elevated international normalized ratio predicts bleeding during invasive diagnostic procedures. MEDLINE and CENTRAL were searched through August 2004, with no language restriction, and reference lists were reviewed. For inclusion, articles must have reported on bleeding in more than five patients with abnormal test results undergoing diagnostic procedures. One trial and 24 observational studies were included. In 2 studies of bronchoscopy, the bleeding rates were similar among those with normal and abnormal tests, with wide confidence intervals (CIs) around the risk differences. During central vein cannulation (3 studies), bleeding rates among those with abnormal tests was unlikely to exceed 2.3 percent. The largest of 3 studies of arteriography found equivalent bleeding rates in patients with and without abnormal tests (risk difference, 0%; 95% CI, -3% to 2%). In the 3 studies of liver biopsy with plugging, bleeding rates were 0, 4, and 5 percent with the upper bounds of the CI as high as 17 percent. In the largest study of transjugular biopsy, the bleeding rate was 1.5 percent (95% CI, 0.3%-4%) in patients with abnormal tests. The highest bleeding rate in the 3 studies of percutaneous liver biopsy was 5.3 percent (95% CI, 1%-13%), similar to the rate in patients with normal test results. There is insufficient evidence to conclude that abnormal test results predict bleeding. Randomized controlled trials should be performed to provide stronger evidence for clinical decision making regarding preprocedure transfusion.
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            High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: results from a large retrospective cohort study.

            No data are available on the absolute risk of either venous thromboembolism (VTE) or arterial thromboembolism (ATE) in patients with nephrotic syndrome. Reported risks are based on multiple case reports and small studies with mostly short-term follow-up. We assessed the absolute risk of VTE and ATE in a large, single-center, retrospective cohort study and attempted to identify predictive factors in these patients. A total of 298 consecutive patients with nephrotic syndrome (59% men; mean age, 42+/-18 years) were enrolled. Mean follow-up was 10+/-9 years. Nephrotic syndrome was defined by proteinuria > or =3.5 g/d, and patients were classified according to underlying histological lesions accounting for nephrotic syndrome. Objectively verified symptomatic thromboembolic events were the primary study outcome. Annual incidences of VTE and ATE were 1.02% (95% confidence interval, 0.68 to 1.46) and 1.48% (95% confidence interval, 1.07 to 1.99), respectively. Over the first 6 months of follow-up, these rates were 9.85% and 5.52%, respectively. Proteinuria and serum albumin levels tended to be related to VTE; however, only the predictive value of the ratio of proteinuria to serum albumin was significant (hazard ratio, 5.6; 95% confidence interval, 1.2 to 26.2; P=0.03). In contrast, neither the degree of proteinuria nor serum albumin levels were related to ATE. Sex, age, hypertension, diabetes, smoking, prior ATE, and estimated glomerular filtration rate predicted ATE (P< or =0.02). This study verifies high absolute risks of symptomatic VTE and ATE that were remarkably elevated within the first 6 months. Whereas the ratio of proteinuria to serum albumin predicted VTE, estimated glomerular filtration rate and multiple classic risk factors for atherosclerosis were predictors of ATE.
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              How I treat the acquired von Willebrand syndrome.

              The acquired von Willebrand syndrome (AVWS) is a bleeding disorder that is frequently unrecognized or is misdiagnosed as von Willebrand disease. AVWS is characterized by structural or functional defects of von Willebrand factor (VWF) that are secondary to autoimmune, lymphoproliferative or myeloproliferative, malignant, cardiovascular, or other disorders. VWF abnormalities in these disorders can result from (1) antibody-mediated clearance or functional interference, (2) adsorption to surfaces of transformed cells or platelets, or (3) increased shear stress and subsequent proteolysis. Diagnosis can be challenging as no single test is usually sufficient to prove or exclude AVWS. Furthermore, there are no evidence-based guidelines for management. Treatments of the underlying medical condition, including chemo/radiotherapy, surgery, or immunosuppressants can result in remission of AVWS, but is not always feasible and successful. Because of the heterogeneous mechanisms of AVWS, more than one therapeutic approach is often required to treat acute bleeds and for prophylaxis during invasive procedures; the treatment options include, but are not limited to, desmopressin, VWF-containing concentrates, intravenous immunoglobulin, plasmapheresis or recombinant factor VIIa. Here, we review the management of AVWS with an overview on the currently available evidence and additional considerations for typical treatment situations.

                Author and article information

                Clin Med Insights Case Rep
                Clin Med Insights Case Rep
                Clinical Medicine Insights. Case Reports
                SAGE Publications (Sage UK: London, England )
                13 March 2018
                : 11
                : 1179547618763371
                [1 ]Division of Nephrology, Department of Internal Medicine, Jichi Medical University, Shimotsuke, Japan
                [2 ]Division of Renal Surgery and Transplantation, Department of Urology, Jichi Medical University, Shimotsuke, Japan
                Author notes
                [*]Tetsu Akimoto, Division of Nephrology, Department of Internal Medicine, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke-Shi 329-0498, Tochigi, Japan. Email: tetsu-a@ 123456jichi.ac.jp
                10.1177_1179547618763371 ICR-0044108
                © The Author(s) 2018

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                : 18 December 2017
                : 14 February 2018
                Case Report
                Custom metadata
                January-December 2018

                acquired von willebrand syndrome,membranous nephropathy,retroperitoneoscopic-assisted renal biopsy,prophylactic anticoagulation,atrial fibrillation


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