Yoshihiro Fujimura

Summary

Affiliation: Nara Medical University
Country: Japan

Publications

  1. pmc Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) on hemodialysis for 19 years: a case report
    Koki Mise
    Nephrology Center, Toranomon Hospital, Tokyo, Japan
    BMC Nephrol 14:156. 2013
  2. doi request reprint Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan
    Y Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan
    J Thromb Haemost 9:283-301. 2011
  3. ncbi request reprint Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008
    Yoshihiro Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan
    Intern Med 49:7-15. 2010
  4. doi request reprint Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients
    Yoshihiro Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan
    Br J Haematol 144:742-54. 2009
  5. doi request reprint Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Thromb Haemost 99:1019-29. 2008
  6. ncbi request reprint Potential role of ADAMTS13 in the progression of alcoholic hepatitis
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, 634 8522, Japan
    Curr Drug Abuse Rev 1:188-96. 2008
  7. ncbi request reprint Increased von Willebrand factor over decreased ADAMTS13 activity may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis
    Tomomi Matsuyama
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Alcohol Clin Exp Res 31:S27-35. 2007
  8. doi request reprint Plasma ADAMTS13 activity parallels the APACHE II score, reflecting an early prognostic indicator for patients with severe acute pancreatitis
    Chie Morioka
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Scand J Gastroenterol 43:1387-96. 2008
  9. doi request reprint Potential role of enhanced cytokinemia and plasma inhibitor on the decreased activity of plasma ADAMTS13 in patients with alcoholic hepatitis: relationship to endotoxemia
    Masatoshi Ishikawa
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Alcohol Clin Exp Res 34:S25-33. 2010
  10. ncbi request reprint Quantitative Western blot analysis of plasma ADAMTS13 antigen in patients with Upshaw-Schulman syndrome
    Hiromichi Ishizashi
    Department of Health Science, Nara Medical University, Nara 634 8522, Japan
    Thromb Res 120:381-6. 2007

Collaborators

Detail Information

Publications36

  1. pmc Long term follow up of congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) on hemodialysis for 19 years: a case report
    Koki Mise
    Nephrology Center, Toranomon Hospital, Tokyo, Japan
    BMC Nephrol 14:156. 2013
    ..In particular, little is known about the long-term prognosis of patients with childhood-onset congenital TTP...
  2. doi request reprint Natural history of Upshaw-Schulman syndrome based on ADAMTS13 gene analysis in Japan
    Y Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan
    J Thromb Haemost 9:283-301. 2011
    ..Our results indicate that 'the late-onset phenotype' of USS is formed with ethnic specificity...
  3. ncbi request reprint Registry of 919 patients with thrombotic microangiopathies across Japan: database of Nara Medical University during 1998-2008
    Yoshihiro Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan
    Intern Med 49:7-15. 2010
    ..Severe deficiency of plasma ADAMTS13 activity (ADAMTS13: AC) is more specific for TTP, but not for HUS. Since 1998, our laboratory has functioned as a nationwide referral center for TMAs by analyzing ADAMTS13...
  4. doi request reprint Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients
    Yoshihiro Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan
    Br J Haematol 144:742-54. 2009
    ..Only the R193W mutation has been previously reported. These observations emphasize the importance of measuring ADAMTS13 activity in the evaluation of thrombocytopenia during childhood and pregnancy...
  5. doi request reprint Comprehensive analysis of ADAMTS13 in patients with liver cirrhosis
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Thromb Haemost 99:1019-29. 2008
    ..An imbalance between the decreased ADAMTS13:AC and its increased substrate may reflect the predisposing state for platelet thrombi formation in patients with advanced LC...
  6. ncbi request reprint Potential role of ADAMTS13 in the progression of alcoholic hepatitis
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, 634 8522, Japan
    Curr Drug Abuse Rev 1:188-96. 2008
    ..The determination of ADAMTS13:AC and its substrate will give us new insights into the pathophysiology of acute alcoholic liver injury and help to elucidate additional therapeutic strategies for this disease...
  7. ncbi request reprint Increased von Willebrand factor over decreased ADAMTS13 activity may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis
    Tomomi Matsuyama
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Alcohol Clin Exp Res 31:S27-35. 2007
    ..We investigated the relationship of ADAMTS13 and von Willebrand factor antigen (VWF:Ag) with the clinical features of AH and SAH...
  8. doi request reprint Plasma ADAMTS13 activity parallels the APACHE II score, reflecting an early prognostic indicator for patients with severe acute pancreatitis
    Chie Morioka
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Scand J Gastroenterol 43:1387-96. 2008
    ..The purpose of the study was to investigate the potential role of ADAMTS13:AC in the severity of SAP...
  9. doi request reprint Potential role of enhanced cytokinemia and plasma inhibitor on the decreased activity of plasma ADAMTS13 in patients with alcoholic hepatitis: relationship to endotoxemia
    Masatoshi Ishikawa
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Nara, Japan
    Alcohol Clin Exp Res 34:S25-33. 2010
    ..The aim of this study was to explore the potential mechanism to reduce the activity of plasma ADAMTS13...
  10. ncbi request reprint Quantitative Western blot analysis of plasma ADAMTS13 antigen in patients with Upshaw-Schulman syndrome
    Hiromichi Ishizashi
    Department of Health Science, Nara Medical University, Nara 634 8522, Japan
    Thromb Res 120:381-6. 2007
  11. doi request reprint Proteolytic fragmentation and sugar chains of plasma ADAMTS13 purified by a conformation-dependent monoclonal antibody
    Hisahide Hiura
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan
    J Biochem 148:403-11. 2010
    ....
  12. doi request reprint Acute myocardial infarction as a systemic prothrombotic condition evidenced by increased von Willebrand factor protein over ADAMTS13 activity in coronary and systemic circulation
    Manabu Horii
    First Department of Internal Medicine, Nara Medical University, 840 Shijo Cho, Kashihara, 634 8521, Japan
    Heart Vessels 23:301-7. 2008
    ....
  13. doi request reprint Ratio of von Willebrand factor propeptide to ADAMTS13 is associated with severity of sepsis
    Hidetada Fukushima
    Department of Emergency and Critical Care Medicine, Nara Medical University, Kashihara, Nara, Japan
    Shock 39:409-14. 2013
    ....
  14. ncbi request reprint ADAMTS13 activity decreases after hepatectomy, reflecting a postoperative liver dysfunction
    Eiji Okano
    Department of Surgery, Nara Medical University, Kashihara, Nara, Japan
    Hepatogastroenterology 57:316-20. 2010
    ..This study evaluated changes of plasma ADAMTS13 activity and its clinical relevance in patients with hepatectomy...
  15. ncbi request reprint Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome
    Masanori Matsumoto
    Department of Blood Transfusion Medicine, Nara Medical University, Shijyo cho 840, Kashihara City, Nara 634 8522, Japan
    Blood 103:1305-10. 2004
    ..Molecular models of the metalloprotease, fifth domain of thrombospondin 1 (Tsp1-5), and Tsp1-8 domains of ADAMTS13 suggest that the missense mutations could cause structural defects in the mutants...
  16. doi request reprint Pivotal role of ADAMTS13 function in liver diseases
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, 840 Shijo Cho, Kashihara Nara, 634 8522, Japan
    Int J Hematol 91:20-9. 2010
    ..These results promise to bring further understanding of the pathophysiology of liver diseases, and offer new insight for development of therapeutic strategies...
  17. ncbi request reprint ADAMTS13 activity may predict the cumulative survival of patients with liver cirrhosis in comparison with the Child-Turcotte-Pugh score and the Model for End-Stage Liver Disease score
    Hiroaki Takaya
    Third Department of Internal Medicine Departments of Blood Transfusion Medicine Community Health and Epidemiology, Nara Medical University, Kashihara, Nara, Japan
    Hepatol Res 42:459-72. 2012
    ..This activity may be a useful prognostic marker that is equal or superior to the CTP score and the MELD score to predict not only the short-term prognosis but also the long-term survival of the cirrhotic patients...
  18. ncbi request reprint Functional imaging of shear-dependent activity of ADAMTS13 in regulating mural thrombus growth under whole blood flow conditions
    Yasuaki Shida
    Department of Pediatrics, Nara Medical University, Kashihara, Nara, Japan
    Blood 111:1295-8. 2008
    ..Our results identify an exquisitely specific regulatory mechanism that prevents arterial occlusion under high shear rate conditions during mural thrombogenesis...
  19. ncbi request reprint The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome
    Masanori Matsumoto
    Department of Blood Transfusion Medicine, Nara Medical University, Japan
    Semin Hematol 41:68-74. 2004
    ..The former patients were associated with "idiopathic" TTP, drugs, and pregnancy, and the latter patients with malignancy and stem cell transplantation. Patients with autoimmune disease-associated TTP fit into both groups...
  20. doi request reprint Heterogeneous pathogenic processes of thrombotic microangiopathies in patients with connective tissue diseases
    Tomomi Matsuyama
    Department of Blood Transfusion Medicine, Nara Medical University, Shijyo cho 840, Kashihara City, Nara, 634 8522, Japan
    Thromb Haemost 102:371-8. 2009
    ..Classifying CTD-TMAs by ADAMTS13 activity may be useful in predicting the clinical course and therapeutic outcomes, as patients with moderately reduced activity are likely to have more prominent renal impairment and poor prognoses...
  21. ncbi request reprint Novel monoclonal antibody-based enzyme immunoassay for determining plasma levels of ADAMTS13 activity
    Seiji Kato
    Department of Blood Transfusion Medicine, Nara Medical University, Nara, Japan
    Transfusion 46:1444-52. 2006
    ..The determination of plasma levels of ADAMTS13 activity is a prerequisite for a differential diagnosis of thrombotic microangiopathies. Here, a unique and highly sensitive enzyme immunoassay (EIA) of ADAMTS13 activity is described...
  22. ncbi request reprint Plasma levels of ADAMTS13 antigen determined with an enzyme immunoassay using a neutralizing monoclonal antibody parallel ADAMTS13 activity LevEls
    Hideo Yagi
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan
    Int J Hematol 85:403-7. 2007
    ..This ELISA may be useful for the rapid determination of ADAMTS13:AG. Further investigations of this antigen would be helpful in advancing the understanding of the pathogenesis of congenital and acquired TTP...
  23. doi request reprint ADAMTS13 unbound to larger von Willebrand factor multimers in cryosupernatant: implications for selection of plasma preparations for thrombotic thrombocytopenic purpura treatment
    Yuji Hori
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan The Chemo Sero Therapeutic Research Institute, Kikuchi, Kumamoto, Japan
    Transfusion 53:3192-202. 2013
    ..Treatment involves plasma exchange (PE). Both fresh-frozen plasma (FFP) and cryosupernatant (CSP) are used, but it remains to be determined which is more effective...
  24. doi request reprint Reduced larger von Willebrand factor multimers at dawn in OSA plasmas reflect severity of apnoeic episodes
    Noriko Koyama
    Second Department of Internal Medicine, Nara Medical University, 840 Shijo Cho, Kashihara, Nara, 634 8522, Japan
    Eur Respir J 40:657-64. 2012
    ..Severe OSA may contribute to an arterial pro-thrombotic state...
  25. doi request reprint Paradigm shift of childhood thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency
    Hideo Yagi
    Nara Medical University, Department of Blood Transfusion Medicine, Nara 634 8522, Japan
    Presse Med 41:e137-55. 2012
    ..This study focused on these 58 patients (41 USS and 17 acquired TTP) who were diagnosed with a severe deficiency in ADAMTS13 activity during childhood, causing a paradigm shift in our concept of TTP...
  26. ncbi request reprint Decreased activity of plasma ADAMTS13 may contribute to the development of liver disturbance and multiorgan failure in patients with alcoholic hepatitis
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, Kashihara, Japan
    Alcohol Clin Exp Res 29:264S-71S. 2005
    ..The aim of this study was to explore the potential role of ADAMTS13 on the development of liver disturbance and multiorgan failure in AH...
  27. ncbi request reprint Plasma ADAMTS13 activity may predict early adverse events in living donor liver transplantation: observations in 3 cases
    Saiho Ko
    Department of Surgery, Nara Medical University, Kashihara, Nara, Japan
    Liver Transpl 12:859-69. 2006
    ..Our findings not only suggest a novel mechanism for thrombocytopenia, but also provide a useful tool for diagnosis of graft dysfunction in the early stage after transplantation...
  28. pmc Determination of ADAMTS13 and Its Clinical Significance for ADAMTS13 Supplementation Therapy to Improve the Survival of Patients with Decompensated Liver Cirrhosis
    Masahito Uemura
    Third Department of Internal Medicine, Nara Medical University, 840 Shijo Cho, Kashihara, Nara 634 8522, Japan
    Int J Hepatol 2011:759047. 2011
    ..Such treatments may improve the survival of patients with decompensated LC...
  29. pmc Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan
    Masanori Matsumoto
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan
    PLoS ONE 7:e33029. 2012
    ..Additional studies exploring these findings are needed...
  30. ncbi request reprint Von Willebrand factor-cleaving protease and Upshaw-Schulman syndrome
    Yoshihiro Fujimura
    Department of Blood Transfusion Medicine, Nara Medical University, Kashihara City, Japan
    Int J Hematol 75:25-34. 2002
    ....
  31. ncbi request reprint Mural thrombus generation in type 2A and 2B von Willebrand disease under flow conditions
    Mitsuhiko Sugimoto
    Department of Pediatrics, Nara Medical University, Kashihara, Japan
    Blood 101:915-20. 2003
    ....
  32. ncbi request reprint Successful treatment of primitive neuroectodermal tumor-associated microangiopathy with multiple bone metastases
    Toru Morishita
    Department of Orthopedic Surgery, National Hospital Organization Nara Medical Center, Nara, Japan
    Jpn J Clin Oncol 37:66-9. 2007
    ..This strategy should be confirmed in further cases...
  33. ncbi request reprint Localization of ADAMTS13 to the stellate cells of human liver
    Masahito Uemura
    Third Department of Internal Medicine, Department of Anatomy, Nara Medical University, 840 Shijo Cho, Kashihara City, Nara, 634 8522, Japan
    Blood 106:922-4. 2005
    ..These results suggest that HSCs may be major cells producing ADAMTS13 in human liver...
  34. doi request reprint Human placental ectonucleoside triphosphate diphosphohydrolase gene transfer via gelatin-coated stents prevents in-stent thrombosis
    Yasuhiro Takemoto
    First Department of Internal Medicine, Nara Medical University, 840 Shijo Cho, Kashihara, Nara 634 8522, Japan
    Arterioscler Thromb Vasc Biol 29:857-62. 2009
    ..We tested the hypothesis that local delivery of human placental E-NTPDase (pE-NTPDase) gene into injured arteries via gene-eluting stent could prevent subacute in-stent thrombosis...
  35. ncbi request reprint [Shear stress-induced platelet aggregation in children with minimal change nephrotic syndrome]
    Ken Takagawa
    Department of Pediatrics, Nara Medical University, Japan
    Nihon Jinzo Gakkai Shi 44:380-8. 2002
    ..The enhanced SIPA may play an important thrombogenic role in the acute phase of childhood MCNS...
  36. ncbi request reprint Anticoagulant activity of M-LAO, L-amino acid oxidase purified from Agkistrodon halys blomhoffii, through selective inhibition of factor IX
    Yoshihiko Sakurai
    Department of Pediatrics, Nara Medical University, 840 Shijo Cho, Nara Kashihara 634 8522, Japan
    Biochim Biophys Acta 1649:51-7. 2003
    ..These results indicate that M-LAO has an anticoagulant activity that impairs the intrinsic clotting by inhibiting factor IX...