National questionnaire survey of TMA

Ito, Nobuyasu; Wada, Hideo; Matsumoto, Masanori; Fujimura, Yoshihiro; Murata, Mitsuru; Izuno, Takashi; Sugita, Minoru; Ikeda, Yasuo

doi:10.1007/s12185-009-0421-3

Cited by 5 publications

(7 citation statements)

References 24 publications

(13 reference statements)

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“…The high frequency of TMA/ADAM-TS13 in the national questionnaire survey might have thus been caused by physician's bias. With regard to the diseases underlying the development of TMA, severe infection and transplantation were the most frequent in this study, but O-157 infection and autoimmune disease were the most frequent in the national questionnaire survey [18,19]. The deficiency of ADAMTS13 is a known cause of TMA, but the over-release of UL-VWFM from vascular endothelial cell may also be important.…”

Section: Discussionmentioning

confidence: 65%

“…In our cases, almost all patients with TMA/ADAMTS13 had a low titer of inhibitor demonstrated no relapse. The frequency of TMA/other was markedly high compared with national questionnaire survey done by the Japanese Ministry of Health, Labor and Welfare [18,19] and other report [20]. As there are many reports of TMA due to abnormalities of ADAMTS13, most physicians look for a decrease in ADAMTS13 activity in patients with TMA.…”

Section: Discussionmentioning

confidence: 85%

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Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome

et al. 2010

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Thrombotic microangiopathy (TMA) is associated with vascular endothelial cell injury and is sometimes linked with poor outcome. Von Willebrand factor (VWF) propeptide (VWFpp) is considered to be a marker of vascular endothelial cell injury. The plasma levels of VWF, VWFpp, and thrombomodulin (TM) were evaluated for their use in the diagnosis of TMA in 75 patients with TMA. There were 30 TMA patients with marked decreases in ADAMTS13 (TMA/ADAMTS13) and 45 without the decrease (TMA/other). The plasma levels of TM, VWF, and VWFpp values were significantly high in patients with TMA, especially TMA/other group. The plasma levels of TM and VWFpp were significantly high in non-survivor with TMA. In the TMA/other group, the plasma levels of VWFpp were negatively correlated with ADAMTS13 activity. The plasma levels of TM correlated with the renal function, but the plasma levels of VWFpp did not. A ROC analysis indicated that VWFpp and TM were useful markers for the prediction of a poor outcome. These findings suggest that VWFpp is an useful marker for the diagnosis of TMA and for the prediction of poor outcome.

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Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 85%

Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome

et al. 2010

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“…The first questionnaire survey [19] was sent to specialists in hematology, and the second questionnaire survey was sent to general hospital departments, including hematology, rheumatology and hemodialysis departments. There were no significant differences in the data collected between the patients recruited for the two surveys.…”

Section: Discussionmentioning

confidence: 99%

“…This is likely because, PE and steroids are more effective against ADAMTS13 TMA than against other TMA. The mortality rate of TMA in Japan was 26.8% in 1988 [27], 26.0% in 1999 [29], 22.0% in 2005 [19] and 18.0% in 2006, suggesting that the mortality rate of TMA is improving.…”

Section: Discussionmentioning

confidence: 99%

“…Two questionnaire surveys for Japanese patients with TMA were carried out in 2004 [18,19] and 2005. In the present study, the frequency, underlying diseases, symptoms, laboratory abnormalities, treatments, efficacy of treatment and outcome were analyzed from all of the data collected in the two questionnaire surveys conducted in 2004 and 2005.…”

Section: Introductionmentioning

confidence: 99%

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A second national questionnaire survey of TMA

et al. 2010

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A second questionnaire survey of Japanese patients with thrombotic microangiopathy (TMA) was carried out to investigate the frequency, laboratory abnormalities and outcome in 2004 and 2005. The first and second surveys evaluated 397 patients including 19 with familial TMA and 378 with acquired TMA. The patients with acquired TMA included 165 with Escherichia coli O-157 infection-related TMA (O-157 TMA), 70 with ADAMTS13-related TMA (ADAMTS13 TMA) and 38 with other types of TMA (other TMA). The rate of ADAMTS13 TMA was significantly higher in patients with collagen diseases than in patients with all other underlying diseases (p < 0.001). The treatment of acquired TMA included plasma exchange (PE), steroids, antiplatelet agents, and anticoagulants, PE was carried out in 91.4% of patients with ADAMTS13 TMA, 68.4% of patients with other TMA and 12.7% of patients with O-157 TMA. The efficacy of PE and steroid therapy tended to be higher in patients with ADAMTS13 TMA than in those with other TMA. The complete remission rate was the highest and the mortality rate was the lowest in the patients with O-157 TMA. The mortality rate tended to be lower in patients with ADAMTS13 TMA than in those with other TMA. However, not all of the patients in our study were examined for ADAMTS13 at the time that this questionnaire survey was conducted.

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Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: concentration of C3 p.I1157T mutation

et al. 2014

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Atypical hemolytic uremic syndrome (aHUS) is caused by abnormalities of the complement system and has a significantly poor prognosis. The clinical phenotypes of 12 patients in nine families with aHUS with familial or recurrent onset and ADAMTS13 activity of ≥20 % treated at the Mie University Hospital were examined. In seven of the patients, the first episode of aHUS occurred during childhood and ten patients experienced a relapse. All patients had renal dysfunction and three had been treated with hemodialysis. Seven patients experienced probable triggering events including common cold, influenza, bacterial infection and/or vaccination for influenza. All patients had entered remission, and renal function was improved in 11 patients. DNA sequencing of six candidate genes, identified a C3 p.I1157T missense mutation in all eight patients in six families examined and this mutation was causative for aHUS. A causative mutation THBD p.D486Y was also identified in an aHUS patient. Four missense mutations, CFH p.V837I, p.Y1058H, p.V1060L and THBD p.R403K may predispose to aHUS manifestation; the remaining seven missense mutations were likely neutral. In conclusion, the clinical phenotypes of aHUS are various, and there are often trigger factors. The C3 p.I1157T mutation was identified as the causative mutation for aHUS in all patients examined, and may be geographically concentrated in or around the Mie prefecture in central Japan.

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National questionnaire survey of TMA

Cited by 5 publications

References 24 publications

Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome

Elevated Von Willebrand factor propeptide for the diagnosis of thrombotic microangiopathy and for predicting a poor outcome

A second national questionnaire survey of TMA

Analysis of patients with atypical hemolytic uremic syndrome treated at the Mie University Hospital: concentration of C3 p.I1157T mutation

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