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1 日本血栓止血学会サイトお役立ちリンク集 日本血栓止血学会サイトに掲載しているおすすめコンテンツのリンクをご紹介します 診療ガイドライン 研修医のお役立ち論文コンテンツ 用語集 本編は次ページより掲載しております
2 特集 : 長鎖分子と血栓止血 2016; 27 3 : 循環器疾患随伴後天性フォンウィルブランド症候群の臨床的インパクト Clinical impact of acquired von Willebrand syndrome associated with cardiovascular diseases Hisanori HORIUCHI, Masanori MATSUMOTO, Koichi KOKAME 要約 : VWF AVWS AVWS AVWS AVWS Key words: von Willebrand factor, shear stress, multimer, Heyde s syndrome, left ventricular assist device (LVAD) 1. はじめに VWF ADAMTS VWF VWF Tel: Fax: hisanori.horiuchi.e8@tohoku.ac.jp acquired von Willebrand syndrome; AVWS percutaneous cardiopulmonary support; PCPS left ventricular assist device; LVAD AVWS 4 2. 合併する疾患とその推定症例数 対処法など 1) 大動脈弁狭窄症 閉塞性肥大型心筋症 Heyde 5
3 317 1 AVWS VWF ADAMTS AVWS AVWS Heyde transcatheter aortic valve implantation; TAVI 6 TAVI VWF 7 VWF large multimer index 2 50 mmhg AVWS 8 AVWS angiodysplasia 2 hypertrophic obstructive cardiomyopathy; HOCM 100 mmhg HOCM AVWS 9 2) 肺高血圧症 AVWS 3 AVWS AVWS AVWS AVWS AVWS mmhg 4 v v 2 m/ 100 mmhg 5 m/ AVWS 3 AVWS 10 AVWS 27 3
4 318 2 VWF large multimer index 8 A VWF large multimer index VWF densitometry large VWF VWF VWF large multimer index 100 VWF B VWF large multimer index AVWS 3) 僧帽弁閉鎖不全 53 VWF AVWS ) 先天性心疾患 11 AVWS ) 機械的補助循環 ECMO PCPS ECMO AVWS 13 AVWS LVAD 2011 bridging LVAD LVAD QOL safety/surveillance-analysis/0009.html LVAD LVAD LVAD LVAD
5 319 LVAD 1 LVAD AVWS 14, 15 LVAD 1 16 LVAD AVWS VWF Sakatsume et al, LVAD AVWS 診断法の問題点とその解決策 AVWS VWF 2000 AVWS AVWS VWF VWF SDS- VWF 1.2 VWF VWF HRP X LAS 4000 VWF VWF large multimer index VWF large multimer index 2 index 100 VWF 4. 消化管血管異形成の形成メカニズム AVWS angiodysplasia AVWS 27 3
6 320 3 The AVeC Study LVAD VEGF VWF 18 VWF 5. おわりに AVWS VWF AVWS acquired von Willebrand syndrome coexisting with cardiovascular diseases study The AVeC Study 3 AVWS 謝辞 VWF The AVeC Study COI 1 Matsumoto M, Kawaguchi S, Ishizashi H, Yagi H, Iida J,
7 321 Sakaki T, Fujimura Y: Platelets treated with ticlopidine are less reactive to unusually large von Willebrand factor multimers than are those treated with aspirin under high shear stress. Pathophysiol Haemost Thromb 34: 35 40, Loscalzo J: From clinical observation to mechanism Heyde s syndrome. N Engl J Med 367: , Veyradier A, Nishikubo T, Humbert M, Wolf M, Sitbon O, Simonneau G, Girma JP, Meyer D: Improvement of von Willebrand factor proteolysis after prostacyclin infusion in severe pulmonary arterial hypertension. Circulation 102: , Suarez J, Patel CB, Felker GM, Becker R, Hernandez AF, Rogers JG: Mechanisms of bleeding and approach to patients with axial-flow left ventricular assist devices. Circ Heart Fail 4: , Heyde EC: Gastrointestinal bleeding in aortic stenosis. N Engl J Med 259: , Spangenberg T, Budde U, Schewel D, Frerker C, Thielsen T, Kuck KH, Schäfer U: Treatment of acquired von Willebrand syndrome in aortic stenosis with transcatheter aortic valve replacement. JACC Cardiovasc Interv 8: , Yamashita K, Yagi H, Hayakawa M, Abe T, Hayata Y, Yamaguchi N, Sugimoto M, Fujimura Y, Matsumoto M, Taniguchi S: Rapid restoration of thrombus formation and high-molecular-weight von Willebrand factor multimers in patients with severe aortic stenosis after valve replacement J Atheroscler Thromb in press, Tamura T, Horiuchi H, Imai M, Tada T, Shiomi H, Kuroda M, Nishimura S, Takahashi Y, Yoshikawa Y, Tsujimura A, Amano M, Hayama Y, Imamura S, Onishi N, Tamaki Y, Enomoto S, Miyake M, Kondo H, Kaitani K, Izumi C, Kimura T, Nakagawa Y: Unexpectedly high prevalence of acquired von Willebrand syndrome in patients with severe aortic stenosis as evaluated with a novel large multimer index. J Atheroscler Thromb 22: , Pruthi RK: Hypertrophic obstructive cardiomyopathy, acquired von Willebrand syndrome, and gastrointestinal bleed- ing. Mayo Clin Proc 86: , Koyama N, Matsumoto M, Tamaki S, Yoshikawa M, Fujimura Y, Kimura H: Reduced larger von Willebrand factor multimers at dawn in OSA plasmas reflect severity of apnoeic episodes. Eur Respir J 40: , Blackshear JL, Wysokinska EM, Safford RE, Thomas CS, Shapiro BP, Ung S, Stark ME, Parikh P, Johns GS, Chen D: Shear stress-associated acquired von Willebrand syndrome in patients with mitral regurgitation. J Thromb Haemost 12: , Onimoe G, Grooms L, Perdue K, Ruymann F: Acquired von Willebrand Syndrome in congenital heart disease: does it promote an increased bleeding risk? Br J Haematol 155: , Kalbhenn J, Schmidt R, Nakamura L, Schelling J, Rosenfelder S, Zieger B: Early diagnosis of acquired von Willebrand Syndrome (AVWS) is elementary for clinical practice in patients treated with ECMO therapy. J Atheroscler Thromb 22: , Slaughter MS: Hematologic effects of continuous flow left ventricular assist devices. J Cardiovasc Translat Res 3: , Draper KV, Huang RJ, Gerson LB: GI bleeding in patients with continuous-flow left ventricular assist devices: a systematic review and meta-analysis. Gastrointest Endosc 80: e1, Sakatsume K, Akiyama M, Saito K, Kawamoto S, Horiuchi H, Saiki Y: Intractable bleeding tendency due to acquired von Willebrand syndrome after Jarvik 2000 implant. J Artif Organs in press, Meyer AL, Malehsa D, Budde U, Bara C, Haverich A, Strueber M: Acquired von Willebrand syndrome in patients with a centrifugal or axial continuous flow left ventricular assist device. JACC Heart Fail 2: , Randi AM, Laffan MA, Starke RD: Von Willebrand factor, angiodysplasia and angiogenesis. Mediterr J Hematol Infect Dis 5: e ,
日本血栓止血学会サイトお役立ちリンク集 日本血栓止血学会サイトに掲載しているおすすめコンテンツのリンクをご紹介します 診療ガイドライン 研修医のお役立ち論文コンテンツ 用語集 本編は次ページより掲載しております
日本血栓止血学会サイトお役立ちリンク集 日本血栓止血学会サイトに掲載しているおすすめコンテンツのリンクをご紹介します 診療ガイドライン 研修医のお役立ち論文コンテンツ 用語集 本編は次ページより掲載しております トピックス 2016; 27 5 : 553-556 補体関連 TMA( 非定型 HUS) Complement-mediated TMA (atypical HUS) Masanori MATSUMOTO
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特集 : 自己抗体による出血 血栓 : 基礎と臨床の現状 2018; 29 3 : 273-280 後天性フォンヴィレブランド症候群 Acquired von Willebrand Syndrome (AvWS) Nobuaki SUZUKI 要約 :von Willebrand Factor VWF Acquired von Willebrand Syndrome AvWS 1968 SLE 50
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