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[PMID]:27771173
[Au] Autor:Farkas P; Csuka D; Mikes B; Sinkovits G; Réti M; Németh E; Rácz K; Madách K; Gergely M; Demeter J; Prohászka Z
[Ad] Endereço:3rd Department of Internal Medicine, Research Laboratory and Füst György Complement Diagnostic Laboratory, Semmelweis University, Budapest, Hungary.
[Ti] Título:Complement activation, inflammation and relative ADAMTS13 deficiency in secondary thrombotic microangiopathies.
[So] Source:Immunobiology;222(2):119-127, 2017 02.
[Is] ISSN:1878-3279
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The secondary forms of hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (secondary TMA) emerge as complications of coexisting diseases. OBJECTIVES: We hypothesized that secondary TMA could be characterized by the presence of relative ADAMTS13 deficiency and complement activation, and this relationship may have a prognostic value for outcome. PATIENTS AND METHODS: Fifty-three patients with thrombotic microangiopathy (TMA) and coexisting disease (such as malignancies, sepsis, heart surgery with extracorporeal circulation, solid organ transplantation, systemic autoimmune disorders), 41 patient controls, and 34 healthy controls were enrolled in our case-control study with 30days follow-up. Complement profile (from serum) and activation products, von Willebrand factor (VWF, from EDTA plasma), and ADAMTS13 activity were determined. RESULTS: ADAMTS13 activity was reduced, while VWF level was elevated in secondary TMA patients. The activity of the classical, lectin and alternative pathways, as well as the levels of C3, C4, and Factor H were significantly lower in secondary TMA patients, and were accompanied by high activation product levels (C3a and sC5b-9). Factor H concentration correlated to relative ADAMTS13 deficiency (i.e. VWF/ADAMTS13 ratio (r=-0.368, p=0.019)). 28/53 patients (53%) died during the follow-up period. Increased sC5b-9, C3a, and C reactive protein levels were all associated with a poor patient outcome. CONCLUSIONS: Our results indicate that the secondary TMA syndrome and its poor outcome is characterized by relative ADAMTS13 deficiency, inflammation, and complement activation with consumption via the classical and alternative pathways. It is yet to be determined whether complement inhibition could be a possible therapeutic option for patients with secondary TMA.
[Mh] Termos MeSH primário: Proteína ADAMTS13/deficiência
Ativação do Complemento/imunologia
Proteínas do Sistema Complemento/imunologia
Inflamação/complicações
Microangiopatias Trombóticas/etiologia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Biomarcadores
Estudos de Casos e Controles
Criança
Pré-Escolar
Comorbidade
Ativação do Complemento/genética
Proteínas do Sistema Complemento/metabolismo
Feminino
Seres Humanos
Masculino
Meia-Idade
Prognóstico
Modelos de Riscos Proporcionais
Microangiopatias Trombóticas/diagnóstico
Microangiopatias Trombóticas/metabolismo
Microangiopatias Trombóticas/mortalidade
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Biomarkers); 9007-36-7 (Complement System Proteins); EC 3.4.24.87 (ADAMTS13 Protein)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:180125
[Lr] Data última revisão:
180125
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161025
[St] Status:MEDLINE


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[PMID]:29298144
[Au] Autor:Crowley MP; McDonald V; Scully M
[Ad] Endereço:Guy's and St. Thomas' Hospital, London, United Kingdom maeve.crowley@gstt.nhs.uk.
[Ti] Título:Ofatumumab for TTP in a Patient with Anaphylaxis Associated with Rituximab.
[So] Source:N Engl J Med;378(1):92-93, 2018 01 04.
[Is] ISSN:1533-4406
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Anafilaxia/induzido quimicamente
Anticorpos Monoclonais/uso terapêutico
Fatores Imunológicos/efeitos adversos
Púrpura Trombocitopênica Trombótica/tratamento farmacológico
Rituximab/efeitos adversos
[Mh] Termos MeSH secundário: Proteína ADAMTS13/deficiência
Proteína ADAMTS13/imunologia
Feminino
Seres Humanos
Fatores Imunológicos/uso terapêutico
Meia-Idade
Rituximab/uso terapêutico
[Pt] Tipo de publicação:CASE REPORTS; LETTER
[Nm] Nome de substância:
0 (Antibodies, Monoclonal); 0 (Immunologic Factors); 4F4X42SYQ6 (Rituximab); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human); M95KG522R0 (ofatumumab)
[Em] Mês de entrada:1801
[Cu] Atualização por classe:180118
[Lr] Data última revisão:
180118
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180104
[St] Status:MEDLINE


  3 / 1495 MEDLINE  
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[PMID]:29186156
[Au] Autor:Lynch CJ; Cawte AD; Millar CM; Rueda D; Lane DA
[Ad] Endereço:Department of Medicine, Centre for Haematology, Imperial College London, United Kingdom.
[Ti] Título:A common mechanism by which type 2A von Willebrand disease mutations enhance ADAMTS13 proteolysis revealed with a von Willebrand factor A2 domain FRET construct.
[So] Source:PLoS One;12(11):e0188405, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Rheological forces in the blood trigger the unfolding of von Willebrand factor (VWF) and its A2 domain, exposing the scissile bond for proteolysis by ADAMTS13. Under quiescent conditions, the scissile bond is hidden by the folded structure due to the stabilisation provided by the structural specialisations of the VWF A2 domain, a vicinal disulphide bond, a calcium binding site and a N1574-glycan.The reduced circulating high MW multimers of VWF in patients with type 2A von Willebrand disease (VWD) may be associated with mutations within the VWF A2 domain and this is attributed to enhanced ADAMTS13 proteolysis. We investigated 11 VWF A2 domain variants identified in patients with type 2A VWD. In recombinant full-length VWF, enhanced ADAMTS13 proteolysis was detected for all of the expressed variants in the presence of urea-induced denaturation. A subset of the FLVWF variants displayed enhanced proteolysis in the absence of urea. The mechanism of enhancement was investigated using a novel VWF A2 domain FRET construct. In the absence of induced unfolding, 7/8 of the expressed mutants exhibited a disrupted domain fold, causing spatial separation of the N- and C- termini. Three of the type 2A mutants were not secreted when studied within the VWF A2 domain FRET construct. Urea denaturation revealed for all 8 secreted mutants reduced unfolding cooperativity and stability of the VWF A2 domain. As folding stability was progressively disrupted, proteolysis by ADAMTS13 increased. Due to the range of folding stabilities and wide distribution of VWF A2 domain mutations studied, we conclude that these mutations disrupt regulated folding of the VWF A2 domain. They enhance unfolding by inducing separation of N- and C-termini, thereby promoting a more open conformation that reveals its binding sites for ADAMTS13 and the scissile bond.
[Mh] Termos MeSH primário: Proteína ADAMTS13/genética
Mutação
Doença de von Willebrand Tipo 2/genética
[Mh] Termos MeSH secundário: Proteína ADAMTS13/química
Transferência Ressonante de Energia de Fluorescência
Seres Humanos
Conformação Proteica
Dobramento de Proteína
Proteólise
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
EC 3.4.24.87 (ADAMTS13 Protein)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171226
[Lr] Data última revisão:
171226
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171130
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0188405


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[PMID]:28751574
[Au] Autor:Xiao J; Feng Y; Li X; Li W; Fan L; Liu J; Zeng X; Chen K; Chen X; Zhou X; Zheng XL; Chen S
[Ad] Endereço:From the Department of Obstetrics & Gynecology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China (J.X., Y.F., W.L., L.F., J.L., X. Zeng, K.C., X.C., S.C.); Department of Urology, Zhengzhou First People's Hospital, Henan, China (X.L.); De
[Ti] Título:Expression of ADAMTS13 in Normal and Abnormal Placentae and Its Potential Role in Angiogenesis and Placenta Development.
[So] Source:Arterioscler Thromb Vasc Biol;37(9):1748-1756, 2017 Sep.
[Is] ISSN:1524-4636
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 repeats, member 13) is primarily synthesized in liver. The biosynthesis of ADAMTS13 and its physiological role in placenta are not known. APPROACH AND RESULTS: We used real-time polymerase chain reaction, immunohistochemistry, and Western blotting analyses, as well as proteolytic cleavage of FRETS (fluorescent resonance energy transfers)-VWF73, to determine ADAMTS13 expression in placenta and trophoblasts obtained from individuals with normal pregnancy and patients with severe preeclampsia. We also determined the role of ADAMTS13 in extravillous trophoblasts using a 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide assay, wound scratch assay, transwell migration assay, tube formation assay, and tissue outgrowth assays. We showed that full-length and proteolytically active ADAMTS13 was expressed in normal human placenta, primarily in the trophoblasts and villous core fetal vessel endothelium during pregnancy. Placental expression of mRNA, protein, and proteolytic activity was at the highest levels during the first trimester and significantly reduced at the term of gestation. Additionally, significantly reduced levels of placental ADAMTS13 expression was detected under hypoxic conditions and in patients with preeclampsia. In addition, recombinant ADAMTS13 protease stimulated proliferation, migration, invasion, and network formation of trophoblastic cells in culture. Finally, knockdown of ADAMTS13 expression attenuated the ability of tube formation in trophoblast (HTR-8/SVNEO) cells and the extravillous trophoblast outgrowth in placental explants. CONCLUSIONS: Our results demonstrate for the first time the expression of mRNA and protein in normal and abnormal placental tissues and its role in promoting angiogenesis and trophoblastic cell development. The findings support the potential role of the ADAMTS13-von Willebrand factor pathway in normal pregnancy and pathogenesis of preeclampsia.
[Mh] Termos MeSH primário: Proteína ADAMTS13/metabolismo
Células Endoteliais/enzimologia
Neovascularização Fisiológica
Placenta/irrigação sanguínea
Placenta/enzimologia
Placentação
Pré-Eclâmpsia/enzimologia
[Mh] Termos MeSH secundário: Proteína ADAMTS13/genética
Adulto
Animais
Células CHO
Estudos de Casos e Controles
Movimento Celular
Proliferação Celular
Cricetulus
Feminino
Regulação da Expressão Gênica no Desenvolvimento
Seres Humanos
Pré-Eclâmpsia/genética
Pré-Eclâmpsia/fisiopatologia
Gravidez
Trimestres da Gravidez
Proteólise
Interferência de RNA
RNA Mensageiro/genética
RNA Mensageiro/metabolismo
Transdução de Sinais
Técnicas de Cultura de Tecidos
Transfecção
Trofoblastos/enzimologia
Fator de von Willebrand/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (RNA, Messenger); 0 (von Willebrand Factor); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170906
[Lr] Data última revisão:
170906
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170729
[St] Status:MEDLINE
[do] DOI:10.1161/ATVBAHA.117.309735


  5 / 1495 MEDLINE  
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[PMID]:28737602
[Au] Autor:Aledort LM; Singleton TC; Ulsh PJ
[Ad] Endereço:*Department of Hematology and Medical Oncology, Icahn School of Medicine, New York, NY †Department of Pediatric Hematology/Oncology, Tulane University, New Orleans, LA ‡Kedrion Biopharma, Fort Lee, NJ.
[Ti] Título:Treatment of Congenital Thrombotic Thrombocytopenia Purpura: A New Paradigm.
[So] Source:J Pediatr Hematol Oncol;39(7):524-527, 2017 Oct.
[Is] ISSN:1536-3678
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Congenital thrombotic thrombocytopenia purpura (cTTP) is a very rare disorder worldwide. Standard treatment of recognized cases has been to administer fresh frozen plasma as the source of ADAMTS13, to replenish the absent ADAMTS13 enzyme. An alternative source, a plasma-derived factor VIII concentrate used for hemophilia A, and found to contain this enzyme, was reported to be effective in 1 patient in the United States. We now report details on a US cohort of 8 cTTP patients who have been successfully treated for varying periods with a marketed antihemophilic factor concentrate Koate-DVI. This biological product has been used successfully on demand in varying doses to treat acute exacerbations, as well as prophylactically (3 to 6 U ADAMTS13 every 3 to 21 d). Self-infused at home, in lieu of fresh frozen plasma therapy in the hospital setting, this product has effectively prevented episodes of thrombocytopenia, microangiopathic hemolytic anemia, and the concomitant organ damage in these patients. This specific virus inactivated product can be used to prevent further manifestations of this congenital enzyme deficiency.
[Mh] Termos MeSH primário: Proteína ADAMTS13/administração & dosagem
Púrpura Trombocitopênica Trombótica/tratamento farmacológico
[Mh] Termos MeSH secundário: Proteína ADAMTS13/deficiência
Adolescente
Adulto
Anemia Hemolítica/prevenção & controle
Criança
Estudos de Coortes
Fator VIII/administração & dosagem
Fator VIII/química
Seres Humanos
Trombocitopenia/prevenção & controle
Estados Unidos
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (F8 protein, human); 9001-27-8 (Factor VIII); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171025
[Lr] Data última revisão:
171025
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170725
[St] Status:MEDLINE
[do] DOI:10.1097/MPH.0000000000000917


  6 / 1495 MEDLINE  
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[PMID]:28652401
[Au] Autor:Bettoni S; Galbusera M; Gastoldi S; Donadelli R; Tentori C; Spartà G; Bresin E; Mele C; Alberti M; Tortajada A; Yebenes H; Remuzzi G; Noris M
[Ad] Endereço:IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Centro di Ricerche Cliniche per le Malattie Rare "Aldo e Cele Daccò," 24020 Ranica Bergamo, Italy.
[Ti] Título:Interaction between Multimeric von Willebrand Factor and Complement: A Fresh Look to the Pathophysiology of Microvascular Thrombosis.
[So] Source:J Immunol;199(3):1021-1040, 2017 Aug 01.
[Is] ISSN:1550-6606
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:von Willebrand factor (VWF), a multimeric protein with a central role in hemostasis, has been shown to interact with complement components. However, results are contrasting and inconclusive. By studying 20 patients with congenital thrombotic thrombocytopenic purpura (cTTP) who cannot cleave VWF multimers because of genetic deficiency, we investigated the mechanism through which VWF modulates complement and its pathophysiological implications for human diseases. Using assays of ex vivo serum-induced C3 and C5b-9 deposits on endothelial cells, we documented that in cTTP, complement is activated via the alternative pathway (AP) on the cell surface. This abnormality was corrected by restoring ADAMTS13 activity in cTTP serum, which prevented VWF multimer accumulation on endothelial cells, or by an anti-VWF Ab. In mechanistic studies we found that VWF interacts with C3b through its three type A domains and initiates AP activation, although assembly of active C5 convertase and formation of the terminal complement products C5a and C5b-9 occur only on the VWF-A2 domain. Finally, we documented that in the condition of ADAMTS13 deficiency, VWF-mediated formation of terminal complement products, particularly C5a, alters the endothelial antithrombogenic properties and induces microvascular thrombosis in a perfusion system. Altogether, the results demonstrated that VWF provides a platform for the activation of the AP of complement, which profoundly alters the phenotype of microvascular endothelial cells. These findings link hemostasis-thrombosis with the AP of complement and open new therapeutic perspectives in cTTP and in general in thrombotic and inflammatory disorders associated with endothelium perturbation, VWF release, and complement activation.
[Mh] Termos MeSH primário: Complemento C3b/metabolismo
Via Alternativa do Complemento
Células Endoteliais/imunologia
Microvasos/patologia
Trombose/fisiopatologia
Fator de von Willebrand/metabolismo
[Mh] Termos MeSH secundário: Proteína ADAMTS13/sangue
Proteína ADAMTS13/deficiência
Proteína ADAMTS13/imunologia
Proteína ADAMTS13/metabolismo
Adolescente
Adulto
Criança
Pré-Escolar
Convertases de Complemento C3-C5/metabolismo
Complemento C3b/imunologia
Complemento C5a/imunologia
Complemento C5a/metabolismo
Complexo de Ataque à Membrana do Sistema Complemento/imunologia
Complexo de Ataque à Membrana do Sistema Complemento/metabolismo
Células Endoteliais/metabolismo
Células Endoteliais/patologia
Feminino
Seres Humanos
Recém-Nascido
Masculino
Microvasos/imunologia
Púrpura Trombocitopênica Trombótica/congênito
Púrpura Trombocitopênica Trombótica/imunologia
Púrpura Trombocitopênica Trombótica/fisiopatologia
Trombose/imunologia
Adulto Jovem
Fator de von Willebrand/imunologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Complement Membrane Attack Complex); 0 (von Willebrand Factor); 80295-43-8 (Complement C3b); 80295-54-1 (Complement C5a); EC 3.4.21.- (Complement C3-C5 Convertases); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171010
[Lr] Data última revisão:
171010
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170628
[St] Status:MEDLINE
[do] DOI:10.4049/jimmunol.1601121


  7 / 1495 MEDLINE  
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[PMID]:28635017
[Au] Autor:Li A; Bendapudi PK; Uhl L; Hamdan A; Kaufman RM; Makar RS
[Ad] Endereço:Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts.
[Ti] Título:Clinical features and outcomes in patients with thrombotic microangiopathy not associated with severe ADAMTS13 deficiency.
[So] Source:Transfusion;57(9):2151-2158, 2017 Sep.
[Is] ISSN:1537-2995
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The a disintegrin and metalloprotease with thrombospondin type 1 motifs, member 13 (ADAMTS13) activity assay has become important in distinguishing autoimmune thrombotic thrombocytopenic purpura from other forms of thrombotic microangiopathy (TMA). Although the significance of severe deficiency in ADAMTS13 (activity levels 10% or less) has been well defined, little data are available on the clinical importance of mild to moderate deficiency (activity levels 11%-70%) among patients with TMA. STUDY DESIGN AND METHODS: We conducted a retrospective study using the Harvard TMA Research Collaborative Registry. Among 254 patients who met the inclusion criteria for TMA, 186 patients with ADAMTS13 activity levels greater than 10% were divided into moderate-deficiency (11%-40%), mild-deficiency (41%-70%), and no-deficiency (greater than 70%). RESULTS: Compared with mild or no deficiency, moderate ADAMTS13 deficiency correlated with older age; higher bilirubin and international normalized ratio; and increased frequency of sepsis, shock, or multiorgan failure. Platelet counts, lactate dehydrogenase levels, and the presence of renal or neurologic dysfunction did not vary across the three patient cohorts. While moderate ADAMTS13 deficiency was associated with increased 90-day mortality in univariate analysis, this association was no longer significant in multivariate analysis. Variables that independetly predicted 90-day mortality in this cohort of patients included Charlson comorbidity index, alanine aminotransferase level, platelet count, creatinine, and the presence of sepsis, shock, or multiorgan failure. CONCLUSION: Moderately deficient ADAMTS13 activity identifies a cohort of patients with TMA who are at increased risk for 90-day mortality. The ADAMTS13 activity level in this group is not an independent predictor of poor outcomes but instead appears to be a marker of disease acuity.
[Mh] Termos MeSH primário: Proteína ADAMTS13/deficiência
Microangiopatias Trombóticas/mortalidade
[Mh] Termos MeSH secundário: Adulto
Idoso
Biomarcadores
Estudos de Coortes
Feminino
Seres Humanos
Masculino
Meia-Idade
Prognóstico
Estudos Retrospectivos
Índice de Gravidade de Doença
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171027
[Lr] Data última revisão:
171027
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170622
[St] Status:MEDLINE
[do] DOI:10.1111/trf.14181


  8 / 1495 MEDLINE  
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[PMID]:28591212
[Au] Autor:Denorme F; Kraft P; Pareyn I; Drechsler C; Deckmyn H; Vanhoorelbeke K; Kleinschnitz C; De Meyer SF
[Ad] Endereço:Laboratory for Thrombosis Research, KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.
[Ti] Título:Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease.
[So] Source:PLoS One;12(6):e0179258, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Von Willebrand Factor (VWF) plays a major role in thrombosis and hemostasis and its thrombogenicity is controlled by ADAMTS13. Whereas increasing evidence shows a clear association between VWF levels and acute ischemic stroke, little is known about a correlation with ADAMTS13. Therefore, the aim of this study was to compare plasma levels of ADAMTS13 between 85 healthy volunteers (HV), 104 patients with acute ischemic stroke and 112 patients with a chronic cerebrovascular disease (CCD). In this case-control study, plasma ADAMTS13 antigen levels were measured by ELISA and plasma VWF levels, measured previously, were next used to calculate VWF:ADAMTS13 ratios. ADAMTS13 levels and VWF:ADAMTS13 ratios were subsequently correlated with key demographic and clinical parameters. ADAMTS13 levels were significantly lower in acute ischemic stroke patients (82.6 ± 21.0%) compared with HV (110.6 ± 26.9%). Also, CCD patients (99.6 ± 24.5%) had significantly lower ADAMTS13 levels compared with HV however these were still higher than in acute stroke patients. Furthermore, when assessing the VWF:ADAMTS13 ratios, an even greater difference was revealed between stroke patients (2.7 ± 1.9), HV (1.1 ± 0.5) and CCD patients (1.7 ± 0.7). The VWF:ADAMTS13 ratio was significantly associated with stroke severity and modality. In conclusion, both in acute and chronic cerebrovascular disease patients, ADAMTS13 levels were significantly decreased, with the lowest ADAMTS13 levels found in acute stroke patients. This difference was even more distinct when the ratio of VWF:ADAMTS13 was considered. These results demonstrate the potentially important involvement of the VWF/ADAMTS13 axis in ischemic stroke.
[Mh] Termos MeSH primário: Proteína ADAMTS13/sangue
Transtornos Cerebrovasculares/sangue
Acidente Vascular Cerebral/sangue
Fator de von Willebrand/metabolismo
[Mh] Termos MeSH secundário: Doença Aguda/epidemiologia
Adulto
Idoso
Idoso de 80 Anos ou mais
Transtornos Cerebrovasculares/epidemiologia
Transtornos Cerebrovasculares/fisiopatologia
Doença Crônica/epidemiologia
Feminino
Seres Humanos
Masculino
Meia-Idade
Fatores de Risco
Acidente Vascular Cerebral/epidemiologia
Acidente Vascular Cerebral/fisiopatologia
Trombose/sangue
Trombose/fisiopatologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (von Willebrand Factor); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170913
[Lr] Data última revisão:
170913
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170608
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0179258


  9 / 1495 MEDLINE  
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[PMID]:28576877
[Au] Autor:Alwan F; Vendramin C; Vanhoorelbeke K; Langley K; McDonald V; Austin S; Clark A; Lester W; Gooding R; Biss T; Dutt T; Cooper N; Chapman O; Cranfield T; Douglas K; Watson HG; van Veen JJ; Sibson K; Thomas W; Manson L; Hill QA; Benjamin S; Ellis D; Westwood JP; Thomas M; Scully M
[Ad] Endereço:Department of Haematology and.
[Ti] Título:Presenting ADAMTS13 antibody and antigen levels predict prognosis in immune-mediated thrombotic thrombocytopenic purpura.
[So] Source:Blood;130(4):466-471, 2017 Jul 27.
[Is] ISSN:1528-0020
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Immune-mediated thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder caused by antibodies against ADAMTS13. From the United Kingdom TTP registry, we undertook a prospective study investigating the impact of the presenting anti-ADAMTS13 IgG antibody and ADAMTS13 antigen on mortality. A total of 312 episodes involving 292 patients over 87 months were included; 68% were female, median age 46 (range, 11-88 years), and median presenting ADAMTS13 of <5% (range, <5%-18%). The mortality rate was 10.3% (n = 32); 68% of patients had a raised troponin at presentation conferring a sixfold increase in mortality compared with those with normal troponin levels (12.1% vs 2.0%, = .04). Twenty-four percent had a reduced Glasgow Coma Score (GCS) at presentation with a ninefold increase in mortality (20% vs 2.2% for normal GCS at presentation, < .0001). Mortality increased with higher anti-ADAMTS13 antibody levels and lower ADAMTS13 antigen levels. Those with antibody levels in the upper quartile (antibody >77%) had a mortality of 16.9% compared with 5.0% for the lowest quartile (antibody <20%) ( = .004). Those with an antigen level in the lowest quartile (antigen <1.5%) had a mortality of 18% compared with 3.8% for the highest quartile (antigen >11%) ( = .005). The synergistic effect of anti-ADAMTS13 IgG antibody in the upper quartile and ADAMTS13 antigen in the lowest quartile had the highest mortality of 27.3%. We conclude that both anti-ADAMTS13 IgG antibody and ADAMTS13 antigen levels correlate with outcome in TTP with increased cardiac and neurological involvement and increased mortality.
[Mh] Termos MeSH primário: Proteína ADAMTS13
Autoanticorpos
Imunoglobulina G
Púrpura Trombocitopênica Trombótica
[Mh] Termos MeSH secundário: Proteína ADAMTS13/sangue
Proteína ADAMTS13/imunologia
Adolescente
Adulto
Idoso
Idoso de 80 Anos ou mais
Autoanticorpos/sangue
Autoanticorpos/imunologia
Criança
Intervalo Livre de Doença
Feminino
Seres Humanos
Imunoglobulina G/sangue
Imunoglobulina G/imunologia
Masculino
Meia-Idade
Púrpura Trombocitopênica Trombótica/sangue
Púrpura Trombocitopênica Trombótica/imunologia
Púrpura Trombocitopênica Trombótica/mortalidade
Taxa de Sobrevida
[Pt] Tipo de publicação:CLINICAL TRIAL; JOURNAL ARTICLE; MULTICENTER STUDY
[Nm] Nome de substância:
0 (Autoantibodies); 0 (Immunoglobulin G); EC 3.4.24.87 (ADAMTS13 Protein); EC 3.4.24.87 (ADAMTS13 protein, human)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170604
[St] Status:MEDLINE
[do] DOI:10.1182/blood-2016-12-758656


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[PMID]:28550351
[Au] Autor:Matsumoto M; Fujimura Y; Wada H; Kokame K; Miyakawa Y; Ueda Y; Higasa S; Moriki T; Yagi H; Miyata T; Murata M; For TTP group of Blood Coagulation Abnormalities Research Team, Research on Rare and Intractable Disease supported by Health, Labour, and Welfare Sciences Research Grants
[Ad] Endereço:Department of Blood Transfusion Medicine, Nara Medical University, 840 Shijyo-cho, Kashihara, Nara, 634-8522, Japan. mmatsumo@naramed-u.ac.jp.
[Ti] Título:Diagnostic and treatment guidelines for thrombotic thrombocytopenic purpura (TTP) 2017 in Japan.
[So] Source:Int J Hematol;106(1):3-15, 2017 Jul.
[Is] ISSN:1865-3774
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:Thrombotic thrombocytopenic purpura (TTP) can rapidly progress into a life-threatening condition, thus the importance of appropriate diagnosis and treatment cannot be overstated. Until recently, TTP has mainly been diagnosed by clinical findings such as thrombocytopenia and non-immune hemolytic anemia. In addition to these clinical findings, however, reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) below 10% has been accepted internationally as a diagnostic criterion for TTP. In the present guidelines, we have taken all of these criteria into consideration. TTP is classified as acquired if the patient is positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are detected. Fresh-frozen plasma (FFP) transfusion is performed in patients with congenital TTP to supplement ADAMTS13. Plasma exchange therapy using FFP is conducted in patients with acquired TTP to supplement ADAMTS13 and remove anti-ADAMTS13 autoantibodies. To suppress autoantibody production, corticosteroid therapy may be administered in conjunction with plasma exchange. Recent reports show that the monoclonal anti-CD-20 antibody rituximab is effective in patients with refractory or relapsed TTP.
[Mh] Termos MeSH primário: Púrpura Trombocitopênica Trombótica/diagnóstico
Púrpura Trombocitopênica Trombótica/terapia
[Mh] Termos MeSH secundário: Proteína ADAMTS13/sangue
Proteína ADAMTS13/metabolismo
Algoritmos
Terapia Combinada
Diagnóstico Diferencial
Ensaios Enzimáticos/métodos
Testes Hematológicos
Seres Humanos
Japão
Proteólise
Púrpura Trombocitopênica Trombótica/epidemiologia
Púrpura Trombocitopênica Trombótica/etiologia
Recidiva
Índice de Gravidade de Doença
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; PRACTICE GUIDELINE
[Nm] Nome de substância:
EC 3.4.24.87 (ADAMTS13 Protein)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:171109
[Lr] Data última revisão:
171109
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170528
[St] Status:MEDLINE
[do] DOI:10.1007/s12185-017-2264-7



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