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Analysis of the pathology and regulation system in pregnancy-associated thrombotic microangiopathy (TMA)

Research Project

Project/Area Number 23591425
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeMulti-year Fund
Section一般
Research Field Hematology
Research InstitutionNara Medical University

Principal Investigator

FUJIMURA Yoshihiro  奈良県立医科大学, 医学部, 名誉教授 (80118033)

Co-Investigator(Kenkyū-buntansha) MATSUMOTO Masanori  奈良県立医科大学, 輸血部, 教授 (60316081)
SADO Toshiyuki  奈良県立科大学, 産婦人科, 講師 (50275335)
HAYAKAWA Masaki  奈良県立医科大学, 輸血部, 医員 (30516729)
Project Period (FY) 2011-04-28 – 2015-03-31
Project Status Completed (Fiscal Year 2014)
Budget Amount *help
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2013: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2012: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2011: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywords妊娠関連TMA / HELLP症候群 / ADAMTS13 / VEF / 胎盤由来E-NTPDase / VWF / 胎盤由来E-NTPase / 活性型VWF / 補体調節因子 / 妊娠中毒症 / 正常妊娠経過 / 胎盤E-NTPDase
Outline of Final Research Achievements

In this study, we aimed to reveal the pathology of pregnancy-associated thrombotic microangiopathy (TMA). We analyzed von Willebrand factor (VWF), VWF-cleaving specific cleavage protease ADAMTS13 and placenta-derived E-NTPDase by using the plasma samples of normal pregnant women and the patients with pregnancy-induced hypertension (PIH) or HELLP syndrome.
We identified that the patients with HELLP syndrome had the severe deletion of "VWF unbound forms of ADAMTS13 (free ADAMTS13)" in isoelectric focusing. We previously showed that cryosupernatant, which abundantly contains “free ADAMTS13”, inhibited initial phase of platelet aggregation. Thus, we suggested that the development of HELLP syndrome might be associated with the decreased function of inhibiting initial phase of platelet aggregation by “free ADAMTS13”.

Report

(5 results)
  • 2014 Annual Research Report   Final Research Report ( PDF )
  • 2013 Research-status Report
  • 2012 Research-status Report
  • 2011 Research-status Report
  • Research Products

    (84 results)

All 2015 2014 2013 2012 2011 Other

All Journal Article (52 results) (of which Peer Reviewed: 49 results,  Open Access: 9 results,  Acknowledgement Compliant: 4 results) Presentation (28 results) (of which Invited: 16 results) Book (3 results) Remarks (1 results)

  • [Journal Article] A unique case involving a female patient with Upshaw-Schulman syndrome: low titers of antibodies against ADAMTS13 prior to pregnancy disappeared after successful deliver.2015

    • Author(s)
      Ogawa Y, Matsumoto M, Sadakata H, Isonishi A, Kato S, Nojima Y, Fujimura Y.
    • Journal Title

      Transfus Med Hemother.

      Volume: 42 Issue: 1 Pages: 59-63

    • DOI

      10.1159/000370225

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Eculizumab in the treatment of atypical hemolytic uremic syndrome in an infant leads to cessation of peritoneal dialysis and improvement of severe hypertension.2015

    • Author(s)
      Ohta T, Urayama K, Tada Y, Furue T, Imai S, Matsubara K, Ono H, Sakano T, Jinno K, Yoshida Y, Miyata T, Fujimura Y.
    • Journal Title

      Pediatr Nephrol.

      Volume: 30 Issue: 4 Pages: 603-608

    • DOI

      10.1007/s00467-014-2975-4

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Familial C3 glomerulonephritis associated with mutations in the gene for complement factor B.2015

    • Author(s)
      Imamura H, Konomoto T, Tanaka E, Hisano S, Yoshida Y, Fujimura Y, Miyata T, Nunoi H.
    • Journal Title

      Nephrol Dial Transplant.

      Volume: 30 Issue: 5 Pages: 862-864

    • DOI

      10.1093/ndt/gfv054

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Autoimmune-type atypical hemolytic uremic syndrome treated with eculizumab as first-line therapy2015

    • Author(s)
      Hisano M, Ashida A, Nakano E, Suehiro M, Yoshida Y, Matsumoto M, Miyata T, Fujimura Y, Hattori M.
    • Journal Title

      Pediatrics Int.

      Volume: 57 Issue: 2 Pages: 313

    • DOI

      10.1111/ped.12469

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] STEC:O111-HUS complicated by acute encephalopathy in a young girl was successfully treated with a set of hemodiafiltration, steroid pulse, and soluble thrombomodulin, under plasma exchange.2015

    • Author(s)
      Yada N, Fujioka M, Bennett CL, Inoki K, Miki T, Watanabe A, Yoshida T, Hayakawa M, Matsumoto M, Fujimura Y.
    • Journal Title

      Clin Case Rep.

      Volume: 3 Issue: 4 Pages: 208-212

    • DOI

      10.1002/ccr3.196

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] A Novel Quantitative Hemolytic Assay Coupled with Restriction Fragment Length Polymorphisms Analysis Enabled Early Diagnosis of Atypical Hemolytic Uremic Syndrome and Identified Unique Predisposing Mutations in Japan.2015

    • Author(s)
      Yoshida Y, Miyata T, Matsumoto M, Shirotani-Ikejima H, Uchida Y, Ohyama Y, Kokubo T, Fujimura Y.
    • Journal Title

      PLos One.

      Volume: 10 Issue: 5 Pages: e0124655-e0124655

    • DOI

      10.1371/journal.pone.0124655

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] A rapid, fully automatic and highly sensitive ADAMTS13 gold particle immunoassay using a routine biochemistry analyser.2015

    • Author(s)
      Kato S, Tanaka M, Isonishi A, Matsumoto M, Samori T, Fujimura Y.
    • Journal Title

      Brit J Haematol

      Volume: in press Issue: 4 Pages: 655-658

    • DOI

      10.1111/bjh.13425

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Poor-responder to plasma exchange therapy in acquired TTP is associated with ADAMTS13 inhibitor boosting: Visualization of an ADAMTS13-inhibitor complex, and its proteolytic clearance from plasma.2015

    • Author(s)
      Isonishi A, Bennett C.L., Plaimauer B, Scheiflinger F, Matsumoto M, Fujimura Y.
    • Journal Title

      Transfusion

      Volume: in press

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Acknowledgement Compliant
  • [Journal Article] Candidate gene analysis using genomic quantitative PCR: identification of ADAMTS13 large deletions in two patients with Upshaw-Schulman syndrome.2014

    • Author(s)
      Eura Y, Kokame K, Takafuta T, Tanaka R, Kobayashi H, Ishida F, Hisanaga S, Matsumoto M, Fujimura Y, Miyata T.
    • Journal Title

      olecular Genetics & Genomic Medicine.

      Volume: 2 Issue: 3 Pages: 240-244

    • DOI

      10.1002/mgg3.64

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access / Acknowledgement Compliant
  • [Journal Article] Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: Concentration of C3 p.I1157T mutation.2014

    • Author(s)
      Matsumoto T, Fan X, Ishikawa E, Ito M, Amano K, Toyoda H, Komada Y, Ohishi K, Katayama N, Yoshida Y, Matsumoto M, Fujimura Y, Ikejiri M, Wada H, Miyata T.
    • Journal Title

      Int J Hematol.

      Volume: 100 Issue: 5 Pages: 437-442

    • DOI

      10.1007/s12185-014-1655-2

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] Postoperative atypical hemolytic uremic syndrome associated with complement c3 mutation.2014

    • Author(s)
      Matsukuma E, Imamura A, Iwata , Takeuchi T, Yoshida Y, Fujimura Y, Fan X, Miyata T, Kuwahara T.
    • Journal Title

      Case Rep Nephrol.

      Volume: 2014 Pages: 784943-784943

    • DOI

      10.1155/2014/784943

    • Related Report
      2014 Annual Research Report
    • Peer Reviewed / Open Access
  • [Journal Article] A first bout of thrombotic thrombocytopenic purpura triggered by herpes simplex infection in a 45-year-old nulliparous female with Upshaw-Schulman syndrome.2014

    • Author(s)
      Morioka M, Matsumoto M, Saito M, Kokame K, Miyata T, Fujimura Y.
    • Journal Title

      Blood Transfus.

      Volume: 12

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Case of maternal and fetal deaths due to severe congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) during pregnancy.2014

    • Author(s)
      Tanaka H, Tenkumo C, Mori N, Kokame K, Fujimura Y, Hata T.
    • Journal Title

      J Obset Gynaecol Res.

      Volume: 40 Issue: 1 Pages: 247-249

    • DOI

      10.1111/jog.12125

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Response to 'Death during pregnancy: thrombotic thrombocytopenic purpura or septic shock?'.2014

    • Author(s)
      Takuma Y, Fujimura Y, Emoto Y, Kuriu Y, Iino M, Matoba R.
    • Journal Title

      J Obstet Gynaecol Res.

      Volume: 40 Issue: 3 Pages: 887-887

    • DOI

      10.1111/jog.12220

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Diagnostic criteria for atypical hemolytic uremic syndrome proposed by the Joint Committee of the Japanese Society of Nephrology and the Japan Pediatric Society.2014

    • Author(s)
      Sawai T, Nangaku M, Ashida A, Fujimaru R, Hataya H, Hidaka Y, Kaname S, Okada H, Sato W, Yasuda T, Yoshida Y, Fujimura Y, Hattori M, Kagami S.
    • Journal Title

      Clin Exp Nephrol.

      Volume: 18 Issue: 1 Pages: 4-9

    • DOI

      10.1007/s10157-013-0911-8

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Effect of ADAMTS-13 on cerebrovascular microthrombosis and neuronal injury after experimental subarachnoid hemorrhage.2014

    • Author(s)
      Muroi C, Fujioka M, Mishima K, Irie K, Fujimura Y, Nakano T, Fandino J, Keller E, Iwasaki K, Fujiwara M.
    • Journal Title

      J Thromb Haemost.

      Volume: 12(4) Issue: 4 Pages: 505-514

    • DOI

      10.1111/jth.12511

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Drug-induced Thrombotic Thrombocytopenic Purpura Successfully Treated with Recombinant Human Soluble Thrombomodulin2013

    • Author(s)
      Nishijima Y, Hirata H, Himeno A, Kida H, Matsumoto M, Takahashi R, Otani Y, Inoue K, Nagatomo I, Takeda Y, Kijima T, Tachibana I, Fujimura Y, Kumanogoh A.
    • Journal Title

      Internal Medicine

      Volume: 52 Issue: 10 Pages: 1111-1114

    • DOI

      10.2169/internalmedicine.52.9180

    • NAID

      130003365599

    • ISSN
      0918-2918, 1349-7235
    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Ratio of von Willebrand Factor Propeptide to ADAMTS13 Is Associated With Severity of Sepsis.2013

    • Author(s)
      Fukushima H, Nishio K, Asai H, Watanabe T, Seki T, Matsui H, Sugimoto M, Matsumoto M, Fujimura Y, Okuchi K.
    • Journal Title

      Shock.

      Volume: 39 Issue: 5 Pages: 409-414

    • DOI

      10.1097/shk.0b013e3182908ea7

    • Related Report
      2013 Research-status Report 2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Analysis of genetic and predisposing factors in Japanese patients with atypical hemolytic uremic syndrome.2013

    • Author(s)
      Fan X, Yoshida Y, Honda S, et al. (Matsumoto M 4番目)
    • Journal Title

      Mol Immunol.

      Volume: 54 Issue: 2 Pages: 238-246

    • DOI

      10.1016/j.molimm.2012.12.006

    • Related Report
      2013 Research-status Report 2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Ticlopidine-associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR).2013

    • Author(s)
      Bennett CL, Jacob S, Fujimura Y (last author).
    • Journal Title

      Br J Haematol.

      Volume: 161 Issue: 6 Pages: 896-898

    • DOI

      10.1111/bjh.12303

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndorome) on hemodialysis for 19 years: a case report2013

    • Author(s)
      Mise K, Ubara Y, Matsumoto M, Sumida K, Hiramatsu R, Hasegawa E, Yamanouchi M, Hayami N, Suwabe T, Hoshino J, Sawa N, Ohashi K, Kokame K, Miyata T, Fujimura Y, Takaichi K.
    • Journal Title

      BMC Nephrol.

      Volume: 14 Issue: 1 Pages: 156-156

    • DOI

      10.1186/1471-2369-14-156

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Therapeutic Modality of 11 Patients with TTP in a Single Institution in Miyazaki from 2000 to 20112013

    • Author(s)
      Kawano N, Yokota-Ikeda N, Yoshida S, Kuriyama T, Yamashita K, Sugio Y, Makino S, Ono N, Inoue Y, Himeji D, Kodama K, Uezono S, Shimao Y, Ueda A, Matsumoto M, Iino H, Fujimura Y.
    • Journal Title

      Internal Medicine

      Volume: 52 Issue: 17 Pages: 1883-1891

    • DOI

      10.2169/internalmedicine.52.8253

    • NAID

      130003365801

    • ISSN
      0918-2918, 1349-7235
    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Limited renal prophylaxis in regular plasmatherapy for heritable ADAMTS13 deficiency.2013

    • Author(s)
      Doi T, Ohga S, Ito N, Ishimura M, Suga N, Nomura A, Takada H, Matsumoto M, Fujimura Y, Hara T.
    • Journal Title

      Pediatr Blood Cancer.

      Volume: 60 Issue: 9 Pages: 1557-1558

    • DOI

      10.1002/pbc.24553

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment.2013

    • Author(s)
      Hori Y, Hayakawa M, Isonishi A, Soejima K, Matsumoto M, Fujimura Y.
    • Journal Title

      Transfusion.

      Volume: 53 Issue: 12 Pages: 3192-3202

    • DOI

      10.1111/trf.12182

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] ADAMTS13 activity and genetic mutations in Japanese subjects.2013

    • Author(s)
      Toshiyuki Miyata, Koichi Kokame, Masanori Matsumoto, and Yoshihiro Fujimura.
    • Journal Title

      Haemostaselolgie

      Volume: 33 Issue: 02 Pages: 131-137

    • DOI

      10.5482/hamo-12-11-0017

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Hereditary thrombotic thrombocytopenic purpura and the hereditary TTP registry.2013

    • Author(s)
      Mansouri Taleghani M, von Krogh AS, Fujimura Y, George JN, Hrachovinová I, Knöbl PN, Quist-Paulsen P, Schneppenheim R, Lämmle B, Kremer Hovinga JA.
    • Journal Title

      Hamostaseologie.

      Volume: 33 Issue: 02 Pages: 138-143

    • DOI

      10.5482/hamo-13-04-0026

    • Related Report
      2013 Research-status Report
    • Peer Reviewed
  • [Journal Article] Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura.2013

    • Author(s)
      Yamamoto T, Fujimura Y, Emoto Y, Kuriu Y, Iino M, Matoba R.
    • Journal Title

      J obset Gynaecol Res.

      Volume: 39 Issue: 1 Pages: 351-354

    • DOI

      10.1111/j.1447-0756.2012.01941.x

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Drug-induced Thrombotic Thrombocytopenic Purpura Successfully Treated with Recombinant Human Soluble Thrombomodulin.2013

    • Author(s)
      Nishijima Y, Hirata H, Himeno A, Kida H, Matsumoto M, Takahashi R, Otani Y, Inoue K, Nagatomo I, Takeda Y, Kijima T, Tachibana I, Fujimura Y, Kumanogoh A.
    • Journal Title

      Intern Med.

      Volume: 52 Pages: 1111-1114

    • NAID

      130003365599

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Ticlopidine-associated ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in 22 persons in Japan: a report from the Southern Network on Adverse Reactions (SONAR).2013

    • Author(s)
      Bennett CL, Jacob S, Dunn BL, et al. (Fujimura Y, last author)
    • Journal Title

      Br J Haematol.

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] The first bout of TTP triggered by herpes simplex infection in a 45-year-old nonparous female with Upshaw-Schulman syndrome2013

    • Author(s)
      Morioka M, Matsumoto M, Saito M, Kokame K, Miyata T, Fujimura Y.
    • Journal Title

      Blood Transfusion.

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] ADAMTS13 activity and genetic mutations in Japanese subjects2013

    • Author(s)
      Miyata T, Kokame K, Matsumoto M, Fujimura Y.
    • Journal Title

      Hamostaseologie

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
  • [Journal Article] Therapeutic modality of 11 patients with TTP: Experience in a single institution in Miyazaki during 2000-20112013

    • Author(s)
      Kawano N, Yokota-Ikeda N, Sugio Y, Yoshida S, Ono N, Kuriyama T, Yamashita K, Makino S, Inoue Y, Himeji D, Kodama K, Uezono S, Shimao Y, Matsumoto M, Iino H, Fujimura Y
    • Journal Title

      Intern Med.

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: Implications for selection of plasma preparations for TTP treatment2013

    • Author(s)
      Hori Y, Hayakawa M, Isonishi A, Soejima K, Matsumoto M, Fujimura Y
    • Journal Title

      Transfusion

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Limited renal prophylaxis in regular plasmatherapy for heritable ADAMTS13 deficiency2013

    • Author(s)
      Doi T, Ohga S, Ito N, Ishimura M, Suga N, Nomura A, Takada H, Matsumoto M, Fujimura Y, Hara T
    • Journal Title

      Pediatric Blood & Cancer

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Hereditary thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) and the hereditary TTP registry2013

    • Author(s)
      Mansouri M-T,von Krogh A-S, Fujimura Y, George JN, Hrachovinova I, Knӧbl PN, Quist-Paulsen P, Schneppenheim R, Lӓmmle B, Kremer-Hovinga JA
    • Journal Title

      Hamostaseologie

      Volume: 印刷中

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Pediatric idiopathic TTP diagnosed with decreased ADAMTS13 activity.2012

    • Author(s)
      Morishima T, Nomura A, Saida S, Watanabe K, Yagi H, Matsumoto M, Fujimura Y, Heike T, Nakahata T, Adachi S.
    • Journal Title

      Pediatr Int.

      Volume: 54 Issue: 3 Pages: 422-423

    • DOI

      10.1111/j.1442-200x.2011.03467.x

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Identification and recombinant analysis of botrocetin-2, a snake venom cofactor for von Willebrand factor-induced platelet agglutination.2012

    • Author(s)
      Yamamoto-Suzuki Y, Sakurai Y, Fujimura Y, Matsumoto M, Hamako J, Kokubo T, Kitagawa H, Kawsar SM, Fujii Y, Ozeki Y, Matsushita F, Matsui T.
    • Journal Title

      Biochemistry.

      Volume: 51 Pages: 5329-5338

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Identification of epitopes on ADAMTS13 recognized by a panel of monoclonal antibodies with functional or non-functional effects on catalytic activity.2012

    • Author(s)
      Igari A, Nakagawa T, Moriki T, Yamaguchi Y, Matsumoto M, Fujimura Y, Soejima K, Murata M.
    • Journal Title

      Thromb Res.

      Volume: 130 Issue: 3 Pages: e79-e83

    • DOI

      10.1016/j.thromres.2012.06.006

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Cilostazol down-regulates the height of mural platelet thrombi formed under a high-shear rate flow in the absence of ADAMTS13 activity.2012

    • Author(s)
      Yagi H, Yamaguchi N, Shida Y, Hayakawa M, Matsumoto M, Sugimoto M, Wada H, Tsubaki K, Fujimura Y.
    • Journal Title

      Eur J Pharmacol.

      Volume: 691 Issue: 1-3 Pages: 151-155

    • DOI

      10.1016/j.ejphar.2012.07.001

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Paucity of CD34-positive cells and increased expression of high-mobility group box 1 in coronary thrombus with type 2 diabetes mellitus.2012

    • Author(s)
      Yamashita A, Nishihira K, Matsuura Y, Ito T, Kawahara K, Hatakeyama K, Hashiguchi T, Maruyama I, Yagi H, Matsumoto M, Fujimura Y, Kitamura K, Shibata Y, Asada Y.
    • Journal Title

      Atherosclerosis.

      Volume: 224 Issue: 2 Pages: 511-514

    • DOI

      10.1016/j.atherosclerosis.2012.07.027

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] Ticlopidine-, clopidogrel-, and prasugrel-associated thrombotic thrombocytopenic purpura: a 20- year review from the Southern Network on Adverse Reactions (SONAR).2012

    • Author(s)
      Jacob S, Dunn BL, Qureshi ZP, et al. (Mastumoto M 20番目)
    • Journal Title

      Semin Thromb Hemost.

      Volume: 38 Issue: 08 Pages: 845-853

    • DOI

      10.1055/s-0032-1328894

    • Related Report
      2012 Research-status Report
  • [Journal Article] Two newborn-onset patients of Upshaw-Schulman syndrome with distinct subsequent clinical courses2012

    • Author(s)
      Tanabe S, Yagi H, Kimura T, Isonishi A, Kato S, Yoshida Y, Hayakawa M, Matsumoto M, Ohtaki S, Takahashi Y, Fujimura Y.
    • Journal Title

      Int J Hematol.

      Volume: 96 Issue: 6 Pages: 789-797

    • DOI

      10.1007/s12185-012-1221-8

    • NAID

      10031144620

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] A novel Wiskott-Aldrich syndrome protein mutation in an infant with thrombotic thrombocytopenic purpura.2012

    • Author(s)
      Kawasaki Y, Toyoda H, Otsuki S, Iwasa T, Iwamoto S, Azuma E, Itoh-Habe N, Wada H, Fujimura Y, Morio T, Imai K, Mitsuiki N, Ohara O, Komada Y.
    • Journal Title

      Eur J Haematol.

      Volume: 90 Issue: 2 Pages: 164-168

    • DOI

      10.1111/ejh.12057

    • Related Report
      2012 Research-status Report
    • Peer Reviewed
  • [Journal Article] The ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis-in comparison with the Child-Turcotte-Pugh score and the MELD score2012

    • Author(s)
      Takaya H, Uemura M, Fujimura Y, Matsumoto M, Matsuyama T, Kato S, Morioka C, Ishizashi H, Hori Y, Fujimoto M, Tsujimoto T, Kawaratani H, Kurumatani N, FukuiH
    • Journal Title

      Hepatology research

      Volume: 42 Issue: 5 Pages: 459-459

    • DOI

      10.1111/j.1872-034x.2011.00950.x

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency.2012

    • Author(s)
      Yagi H, Matsumoto M, Fujimura Y.
    • Journal Title

      Presse Med.

      Volume: 41 Issue: 3 Pages: e137-e155

    • DOI

      10.1016/j.lpm.2011.10.027

    • Related Report
      2011 Research-status Report
  • [Journal Article] Reduced larger VWF multimers at dawn in OSA plasmas reflect severity of apneic episodes2012

    • Author(s)
      Koyama N, Matsumoto M, Tamaki S, Yoshikawa M, Fujimura Y, Kimura H
    • Journal Title

      Eur Resp J

      Volume: (印刷中) Issue: 3 Pages: 657-664

    • DOI

      10.1183/09031936.00186210

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] The homozygous p.C1024R-ADAMTS13 gene mutation links to a late-on set phenotype of Upshaw-Schulman syndrome in Japan2012

    • Author(s)
      Taguchi F, Yagi H, Matsumoto M, Sadamura S, Isonishi A, Soejima K, Fujimura Y
    • Journal Title

      Thromb Haemost

      Volume: (印刷中) Issue: 05 Pages: 1003-1005

    • DOI

      10.1160/th11-11-0799

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocyt openic purpura in a population from Japan2012

    • Author(s)
      Matsumoto M, Bennett CL, Isonishi A, Qureshi Z, Hori Y, Hayakawa M, Yoshida Y, Yagi H, Fujimura Y
    • Journal Title

      PLoS ONE

      Volume: 7 Issue: 3 Pages: e33029-e33029

    • DOI

      10.1371/journal.pone.0033029

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan2011

    • Author(s)
      Y.Fujimura, 他7名
    • Journal Title

      J Thromb Haemost

      Volume: 9(review) Pages: 283-301

    • DOI

      10.1111/j.1538-7836.2011.04341.x

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] H1N1 Influenza (Swine Flu)-Associated Thrombotic Microangiopathy with a Markedly High Plasma Ratio of von Willebrand Factor to ADAMTS132011

    • Author(s)
      Akiyama R, Komori I, Hiramoto R, Isonishi A, Matsumoto M, Fujimura Y.
    • Journal Title

      Internal Medicine

      Volume: 50 Issue: 6 Pages: 643-647

    • DOI

      10.2169/internalmedicine.50.4620

    • NAID

      130000649901

    • ISSN
      0918-2918, 1349-7235
    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Epitope analysis of autoantibodies to ADAMTS13 in patients with acquired thrombotic thrombocytopenic purpura.2011

    • Author(s)
      Yamaguchi Y, Moriki T, Igari A, Nakagawa T, Wada H, Matsumoto M, Fujimura Y, Murata M.
    • Journal Title

      Thromb Res.

      Volume: 128 Issue: 2 Pages: 169-173

    • DOI

      10.1016/j.thromres.2011.03.010

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Journal Article] Determination of ADAMTS13 and its clinical significance for ADAMTS13 supplementation therapy to improve the survival of patients with decompensated liver cirrhosis2011

    • Author(s)
      Uemura M, Fujimura Y, Ko S, et al.
    • Journal Title

      Int J Hepatol

      Volume: Epub 2011 Jul 18. Pages: 1-12

    • DOI

      10.4061/2011/759047

    • Related Report
      2011 Research-status Report
    • Peer Reviewed
  • [Presentation] TMA:最近の考え方と治療法の選択2015

    • Author(s)
      藤村吉博
    • Organizer
      Nagoya Hematology Forum
    • Place of Presentation
      ヒルトン名古屋(愛知県・名古屋市)
    • Year and Date
      2015-02-06
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] Introduction of a quick assay for ADAMTS13 activity improved a survival of acquired TTP patients who received platelet transfusions.2014

    • Author(s)
      Yoshii Y, Matsumoto M, Kurumatani N, Isonishi A, Uemura M, Hori Y, Hayakawa M, Yagi H, Bennett C.L., Fujimura Y
    • Organizer
      The 56th Annual meeting of American Society of Hematology
    • Place of Presentation
      San Francisco(USA)
    • Year and Date
      2014-12-08
    • Related Report
      2014 Annual Research Report
  • [Presentation] Decreased platelet thrombus size, due to a heightened proteolysis of VWF by ADAMTS13 is quickly restored after valve replacement in aortic stenosis patients.2014

    • Author(s)
      Yagi H, Hayakawa M, Yamaguchi N, Yamashita K, Taniguchi S, Matsumoto M, Tsubaki K, Fujimura Y
    • Organizer
      The 56th Annual meeting of American Society of Hematology
    • Place of Presentation
      San Francisco(USA)
    • Year and Date
      2014-12-07
    • Related Report
      2014 Annual Research Report
  • [Presentation] Registry of congenital thrombotic microangiopathy in Japan.2014

    • Author(s)
      Fujimura Y
    • Organizer
      The 3rd ASEAN Federation of Hematology Congress.Plenary lecture
    • Place of Presentation
      Bangkok(Thailand)
    • Year and Date
      2014-10-23
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] ADAMTS13と血小板輸血-過去、現在、未来2014

    • Author(s)
      藤村吉博
    • Organizer
      第20回 ADAMTS13研究会
    • Place of Presentation
      奈良ホテル(奈良県・奈良市)
    • Year and Date
      2014-09-18
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] TTP, HUS, aHUSの鑑別診断と治療.2014

    • Author(s)
      藤村吉博
    • Organizer
      第59回臨床病理近畿支部例会
    • Place of Presentation
      天理医療大学キャンパス(奈良県・天理市)
    • Year and Date
      2014-06-21
    • Related Report
      2014 Annual Research Report
    • Invited
  • [Presentation] 非典型溶血性尿毒症症候群(aHUS)2013

    • Author(s)
      藤村吉博
    • Organizer
      第75回日本血液学会学術集会
    • Place of Presentation
      ロイトン札幌
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] Phenotype modifiers of ADAMTS 13 deficiency with respect to gender, gene mutations, and inhibitor boosting2012

    • Author(s)
      藤村吉博
    • Organizer
      56. Jahrestagung der Gesellschaft für Thrombose- und Hämostaseforschung (GTH)
    • Place of Presentation
      St. Gallen (Switzerland)
    • Related Report
      2011 Research-status Report
  • [Presentation] Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan2011

    • Author(s)
      藤村吉博
    • Organizer
      XXIII Congress of the International Society on Thrombosis and Haemostasis
    • Place of Presentation
      京都国際会議場(京都府)
    • Related Report
      2011 Research-status Report
  • [Presentation] 止血因子VWFとその特異的切断酵素ADAMTS13の均衡破綻による血栓症のパラダイムシフト2011

    • Author(s)
      藤村吉博
    • Organizer
      奈良県医師会第一回生涯教育講座(招待講演)
    • Place of Presentation
      奈良県医師会館(奈良県)
    • Related Report
      2011 Research-status Report
  • [Presentation] TMAとDIC:血液専門医が認識すべき病態と治療のパラダイムシフト2011

    • Author(s)
      藤村吉博
    • Organizer
      第2回サイト研究会 (招待講演)
    • Place of Presentation
      ホテル阪急インターナショナル前 エコルテホール(大阪府)
    • Related Report
      2011 Research-status Report
  • [Presentation] ADAMTS13とFactor Hに焦点を当てた血栓性微小血管障害症(TMA)の診断と治療2011

    • Author(s)
      藤村吉博
    • Organizer
      第15回 炎症と凝固研究会 (招待講演)
    • Place of Presentation
      三重大学医学部(三重県)
    • Related Report
      2011 Research-status Report
  • [Presentation] 周産期TMA(血栓性血小板減少性紫斑病)の診断と治療2011

    • Author(s)
      藤村吉博
    • Organizer
      平成23年度富山県産科婦人科学会 第4回例 (招待講演)
    • Place of Presentation
      ANAクラウンプラザホテル3階飛鳥の間(富山県)
    • Related Report
      2011 Research-status Report
  • [Presentation] Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan2011

    • Author(s)
      藤村吉博
    • Organizer
      第73回日本血液学会学術集会(JSH2011)
    • Place of Presentation
      名古屋国際会議場(名古屋県)
    • Related Report
      2011 Research-status Report
  • [Presentation] 後天性TTPに対する血漿交換療法

    • Author(s)
      藤村吉博
    • Organizer
      第61回日本輸血・細胞治療学会総会
    • Place of Presentation
      パシフィコ横浜
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] Thrombotic thrombocytopenic purpura(TTP) と Hemolytic uremic syndrome(HUS) ~病態解明と診断のための新たなマーカー~

    • Author(s)
      藤村吉博
    • Organizer
      第35回日本血栓止血学会学術集会
    • Place of Presentation
      山形国際ホテル
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] 非典型溶血性尿毒症症候群 (aHUS): 診断と治療の進歩

    • Author(s)
      藤村吉博
    • Organizer
      第35回日本血栓止血学会学術集会
    • Place of Presentation
      山形国際ホテル
    • Related Report
      2013 Research-status Report
    • Invited
  • [Presentation] A long-term phenotype analysis of 51 patients with Upshaw -Schulman Syndrome in Japan, with special references to pregnancy and renal failure that requires hemodialysis

    • Author(s)
      Fujimura Y, Yoshii Y, Matsumoto M, Isonishi A, Hayakawa M, Yoshida Y, Yagi H, Kokame K, Miyata T.
    • Organizer
      The 55th Annual meeting of American Society of Hematology
    • Place of Presentation
      Ernest N. Morial Convention Center(New Orleans, USA)
    • Related Report
      2013 Research-status Report
  • [Presentation] 均一な集団と考えられるADAMTS13 活性著減(<0.5%)後天性特発性TTP 150 例の解析.

    • Author(s)
      松本雅則,Bennett Charles,石西綾美,Qureshi Zenia,堀勇二,早川正樹,吉田瑶子,八木秀男,藤村吉博.
    • Organizer
      第34回日本血栓止血学会学術集会.
    • Place of Presentation
      ハイアットリージェンシー(東京)
    • Related Report
      2012 Research-status Report
  • [Presentation] TMA:診断と治療の新しい展開

    • Author(s)
      藤村吉博
    • Organizer
      第21回 群馬血液疾患研究会
    • Place of Presentation
      群馬ロイヤルホテル(群馬)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] ADAMTS13-VWFの均衡破綻による血栓性微小血管障害症

    • Author(s)
      藤村吉博
    • Organizer
      第3回 Global and New Insight into HSCT研究会
    • Place of Presentation
      ウエスティン都ホテル京都(京都)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] 本邦でのaHUS患者のdiagnostic pathwayと登録状況

    • Author(s)
      藤村吉博
    • Organizer
      第49回補体シンポジウム
    • Place of Presentation
      大阪府立成人病センター(大阪)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] TMA:診断と治療の新しい展開

    • Author(s)
      藤村吉博
    • Organizer
      第19回岐阜血液研究会
    • Place of Presentation
      岐阜グランドホテル(岐阜)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] Establishing a TTP diagnostic network in Japan -Experience at Nara-

    • Author(s)
      Yoshihiro Fujimura
    • Organizer
      International and ANZ Steering Committee for APMAT Research Network
    • Place of Presentation
      Melbourne Convention Center(オーストラリア)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] 本邦におけるTMA解析状況

    • Author(s)
      藤村吉博
    • Organizer
      第7回日本血栓止血学会学術標準化委員会シンポジウム
    • Place of Presentation
      東医健保会館(東京)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] TMA病態に対する新鮮凍結血漿由来ADAMTS13の治療効果

    • Author(s)
      藤村吉博
    • Organizer
      石川県合同輸血療法委員会
    • Place of Presentation
      石川県地場産業振興センター(石川)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Presentation] Unusually large VWF multimers appearing in patient plasmas of HSCT predispose TMA:Management with plasma ADAMTS13 and recombinant soluble thrombomodulin

    • Author(s)
      Hayakawa M, Matsumoto M, Fujimura Y
    • Organizer
      Jahrestagung der Gesellschaft fur Thrombose- und Hamostaseforschung (GTH)
    • Place of Presentation
      ICM-Internationales Congress Center Munchen(ドイツ)
    • Related Report
      2012 Research-status Report
  • [Presentation] ADAMTS13: Biology and Upshaw-Schulman Syndrome

    • Author(s)
      Yoshihiro Fujimura
    • Organizer
      23rd Annual Hemophilia Research Study Update Meeting(HRSU)
    • Place of Presentation
      THE GRAND HOTEL MALAHIDE(アイルランド)
    • Related Report
      2012 Research-status Report
    • Invited
  • [Book] ADAMTS13 book2015

    • Author(s)
      Fujimura Y, Kokame K, Yagi H, Isonishi A, Matsumoto M, Miyata T
    • Publisher
      Springer (edited by George Rodgers)
    • Related Report
      2014 Annual Research Report
  • [Book] Annual Review 血液2013

    • Author(s)
      松本雅則、藤村吉博
    • Total Pages
      8
    • Publisher
      中外医学社
    • Related Report
      2012 Research-status Report
  • [Book] 新版日本輸血・細胞治療学会認定医制度指定カリキュラム2012

    • Author(s)
      藤村吉博
    • Total Pages
      3
    • Publisher
      日本輸血・細胞治療学会
    • Related Report
      2012 Research-status Report
  • [Remarks] 難病情報センター「血栓性血小板減少性紫斑病(TTP)」

    • URL

      http://www.nanbyou.or.jp/entry/87

    • Related Report
      2012 Research-status Report

URL: 

Published: 2011-08-05   Modified: 2019-07-29  

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