Base de dados : MEDLINE
Pesquisa : C15.378.100.802.687.680 [Categoria DeCS]
Referências encontradas : 4029 [refinar]
Mostrando: 1 .. 10   no formato [Detalhado]

página 1 de 403 ir para página                         

  1 / 4029 MEDLINE  
              next record last record
seleciona
para imprimir
Fotocópia
[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


  2 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
[PMID]:28471571
[Au] Autor:Marco-Hernández J; Prieto-González S; Blasco M; Castro P; Cid J; Espinosa G
[Ad] Endereço:Department of Internal Medicine, Hospital Clínic, Barcelona, Catalonia, Spain.
[Ti] Título:Thrombotic Microangiopathy: a Challenging Diagnosis Always.
[So] Source:Isr Med Assoc J;18(7):437-438, 2016 Jul.
[Is] ISSN:1565-1088
[Cp] País de publicação:Israel
[La] Idioma:eng
[Mh] Termos MeSH primário: Púrpura Trombocitopênica Trombótica/diagnóstico
Microangiopatias Trombóticas/diagnóstico
[Mh] Termos MeSH secundário: Feminino
Seres Humanos
Meia-Idade
Microangiopatias Trombóticas/fisiopatologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171128
[Lr] Data última revisão:
171128
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE


  3 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28768626
[Au] Autor:Sadler JE
[Ad] Endereço:Departments of Medicine and Biochemistry and Molecular Biophysics, Washington University School of Medicine, St. Louis, MO.
[Ti] Título:Pathophysiology of thrombotic thrombocytopenic purpura.
[So] Source:Blood;130(10):1181-1188, 2017 Sep 07.
[Is] ISSN:1528-0020
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The discovery of a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, member 13 (ADAMTS13) revolutionized our approach to thrombotic thrombocytopenic purpura (TTP). Inherited or acquired ADAMTS13 deficiency allows the unrestrained growth of microthrombi that are composed of von Willebrand factor and platelets, which account for the thrombocytopenia, hemolytic anemia, schistocytes, and tissue injury that characterize TTP. Most patients with acquired TTP respond to a combination of plasma exchange and rituximab, but some die or acquire irreversible neurological deficits before they can respond, and relapses can occur unpredictably. However, knowledge of the pathophysiology of TTP has inspired new ways to prevent early deaths by targeting autoantibody production, replenishing ADAMTS13, and blocking microvascular thrombosis despite persistent ADAMTS13 deficiency. In addition, monitoring ADAMTS13 has the potential to identify patients who are at risk of relapse in time for preventive therapy.
[Mh] Termos MeSH primário: Púrpura Trombocitopênica Trombótica/fisiopatologia
[Mh] Termos MeSH secundário: Proteínas ADAM/deficiência
Seres Humanos
Adesividade Plaquetária
Púrpura Trombocitopênica Trombótica/patologia
Púrpura Trombocitopênica Trombótica/terapia
Recidiva
Fatores de Risco
Fator de von Willebrand/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (von Willebrand Factor); EC 3.4.24.- (ADAM Proteins)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:171008
[Lr] Data última revisão:
171008
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170804
[St] Status:MEDLINE
[do] DOI:10.1182/blood-2017-04-636431


  4 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[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


  5 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28697902
[Au] Autor:Alijotas-Reig J
[Ad] Endereço:Systemic Autoimmune Diseases Unit, Department of Internal Medicine, Vall d'Hebron University Hospital, Passeig Vall d'Hebron 119-129, Barcelona 080, Spain. Electronic address: jalijotas@vhebron.net.
[Ti] Título:Acquired thrombotic thrombocytopenic purpura and pregnancy: More light than shade but controversies remain.
[So] Source:Thromb Res;156:195-197, 2017 08.
[Is] ISSN:1879-2472
[Cp] País de publicação:United States
[La] Idioma:eng
[Mh] Termos MeSH primário: Troca Plasmática
Púrpura Trombocitopênica Trombótica
[Mh] Termos MeSH secundário: Proteínas ADAM
Feminino
Seres Humanos
Luz
Gravidez
Complicações Hematológicas na Gravidez
[Pt] Tipo de publicação:EDITORIAL; COMMENT
[Nm] Nome de substância:
EC 3.4.24.- (ADAM Proteins)
[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:170713
[St] Status:MEDLINE


  6 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[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 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28592771
[Au] Autor:Miyakawa Y
[Ad] Endereço:Department of General Internal Medicine, Center for Thrombosis and Hemostasis, Saitama Medical University Hospital.
[Ti] Título:TTP and aHUS: new insights.
[So] Source:Rinsho Ketsueki;58(5):530-536, 2017.
[Is] ISSN:0485-1439
[Cp] País de publicação:Japan
[La] Idioma:jpn
[Ab] Resumo:Both TTP and aHUS are officially designated as intractable diseases by a new Japanese law in 2015. New clinical practice guidelines for TTP and aHUS have been published. Both conditions share some common pathophysiology such as thrombotic microangiopathy. Unfortunately, most professionals in the fields of hematology/oncology are unaware of these diseases. Therefore, I will provide an updated overview of TTP and aHUS in this article.
[Mh] Termos MeSH primário: Síndrome Hemolítico-Urêmica Atípica
Púrpura Trombocitopênica Trombótica
[Mh] Termos MeSH secundário: Anticorpos Monoclonais Humanizados/efeitos adversos
Anticorpos Monoclonais Humanizados/uso terapêutico
Síndrome Hemolítico-Urêmica Atípica/diagnóstico
Síndrome Hemolítico-Urêmica Atípica/terapia
Diagnóstico Diferencial
Seres Humanos
Fatores Imunológicos/uso terapêutico
Guias de Prática Clínica como Assunto
Púrpura Trombocitopênica Trombótica/diagnóstico
Púrpura Trombocitopênica Trombótica/terapia
Rituximab/uso terapêutico
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antibodies, Monoclonal, Humanized); 0 (Immunologic Factors); 4F4X42SYQ6 (Rituximab); A3ULP0F556 (eculizumab)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171108
[Lr] Data última revisão:
171108
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170609
[St] Status:MEDLINE
[do] DOI:10.11406/rinketsu.58.530


  8 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[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


  9 / 4029 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[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


  10 / 4029 MEDLINE  
              first record previous record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:28500622
[Au] Autor:Yoshii Y; Fujimura Y; Bennett CL; Isonishi A; Kurumatani N; Matsumoto M
[Ad] Endereço:Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Nara, Japan.
[Ti] Título:Implementation of a rapid assay of ADAMTS13 activity was associated with improved 30-day survival rate in patients with acquired primary thrombotic thrombocytopenic purpura who received platelet transfusions.
[So] Source:Transfusion;57(8):2045-2053, 2017 Aug.
[Is] ISSN:1537-2995
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Platelet (PLT) transfusions are probably harmful in patients with acquired idiopathic thrombotic thrombocytopenic purpura (aTTP). Introduction of a rapid assay for ADAMTS13 activity should reduce the time to definite diagnosis of aTTP, reduce the amount of inappropriately transfused PLT concentrates, and improve mortality and morbidity. STUDY DESIGN AND METHODS: We selected 265 aTTP patients with severe ADAMTS13 deficiency. Of these, 91 patients were diagnosed by March 2005 (Period 1), when ADAMTS13 activity was measured by von Willebrand factor multimer assay, which took 4 to 7 days until the result was reported. An additional 174 patients were diagnosed after April 2005 (Period 2), when the activity was measured by a chromogenic enzyme-linked immunosorbent assay, which took 1 to 2 days. RESULTS: We found no significant differences in 30-day survival rate between the two periods. Overall, 48 patients received PLT transfusions. Mortality was slightly greater between patients with (22.9%) versus without PLT transfusion (17.7%), but not significant. In Period 1, Cox proportional hazards regression analysis showed that older age (≥60 years) and PLT transfusion administration were independent factors associated with higher risks of 30-day mortality. In contrast, in Period 2, lower Rose-Eldor TTP severity score and use of plasma exchange and corticosteroid therapy were independent factors associated with higher survival rates while nonadministration of PLT transfusions was not. CONCLUSION: Our results indicate that PLT transfusions are harmful for aTTP patients when the definite diagnosis of severe ADAMTS13 deficiency is delayed. If it can be done as soon as possible, PLT transfusions for severe bleeding or surgical interventions might be allowed with subsequent plasmapheresis.
[Mh] Termos MeSH primário: Proteína ADAMTS13/análise
Transfusão de Plaquetas/mortalidade
Púrpura Trombocitopênica Trombótica/terapia
[Mh] Termos MeSH secundário: Proteína ADAMTS13/deficiência
Corticosteroides/uso terapêutico
Adulto
Fatores Etários
Idoso
Seres Humanos
Meia-Idade
Troca Plasmática
Púrpura Trombocitopênica Trombótica/diagnóstico
Púrpura Trombocitopênica Trombótica/mortalidade
Fatores de Risco
Taxa de Sobrevida
Fatores de Tempo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Adrenal Cortex Hormones); 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:IM
[Da] Data de entrada para processamento:170514
[St] Status:MEDLINE
[do] DOI:10.1111/trf.14152



página 1 de 403 ir para página                         
   


Refinar a pesquisa
  Base de dados : MEDLINE Formulário avançado   

    Pesquisar no campo  
1  
2
3
 
           



Search engine: iAH v2.6 powered by WWWISIS

BIREME/OPAS/OMS - Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde