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[PMID]:28604077
[Au] Autor:Udvardy M
[Ad] Endereço:Belgyógyászat Intézet, Hematológia Tanszék, Debreceni Egyetem, Általános Orvostudományi Kar Debrecen, Nagyerdei krt. 98., 4032.
[Ti] Título:[Activated Factor VII - 31 years experience on clinical grounds].
[Ti] Título:Az aktivált VII. faktor ­ 31 éves klinikai alkalmazás tanulságai..
[So] Source:Orv Hetil;158(24):923-928, 2017 Jun.
[Is] ISSN:0030-6002
[Cp] País de publicação:Hungary
[La] Idioma:hun
[Ab] Resumo:The author provides an overview of the use of recombinant activated FVII (rFVIIa, Novoseven), which is used over 30 years, based upon international publications and also on some modest own experience. Standard, approved indications (inhibitory cases, Glanzmann thrombasthenia, prophylaxis experience) are in the focus of this paper, emphasizing the specially rapid and efficacious way of Novoseven therapy, drawing attention to excellent safety issues regarding very low immunogenicity along with low number of thrombogenic complications. A careful, cautious and critical evaluation of Novoseven therapy is also provided in rather special forms of critical bleeding conditions considering international recommendations and institutional registry data. Orv Hetil. 2017; 158(24): 923-928.
[Mh] Termos MeSH primário: Transtornos da Coagulação Sanguínea/tratamento farmacológico
Coagulantes/uso terapêutico
Fator VIIa/uso terapêutico
[Mh] Termos MeSH secundário: Seres Humanos
Proteínas Recombinantes/uso terapêutico
Trombastenia/tratamento farmacológico
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Coagulants); 0 (Recombinant Proteins); AC71R787OV (recombinant FVIIa); EC 3.4.21.21 (Factor VIIa)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170808
[Lr] Data última revisão:
170808
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170613
[St] Status:MEDLINE
[do] DOI:10.1556/650.2017.30758


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[PMID]:28370369
[Au] Autor:Recht M; Rajpurkar M; Chitlur M; d'Oiron R; Zotz R; Di Minno G; Cooper DL; Poon MC
[Ad] Endereço:Oregon Health & Science University, Portland, Oregon, USA.
[Ti] Título:Independent adjudicator assessments of platelet refractoriness and rFVIIa efficacy in bleeding episodes and surgeries from the multinational Glanzmann's thrombasthenia registry.
[So] Source:Am J Hematol;92(7):646-652, 2017 Jul.
[Is] ISSN:1096-8652
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Glanzmann's thrombasthenia (GT) is a rare congenital bleeding disorder associated with decreased platelet aggregation due to qualitative/quantitative deficiencies of the fibrinogen receptor. Severe bleeding episodes and perioperative bleeding are typically managed with platelet transfusions, although patients can develop anti-platelet antibodies or experience clinical refractoriness. The GT Registry (GTR) was established to collect efficacy/safety data on hemostatic treatments for GT, including recombinant factor VIIa (rFVIIa). At the request of the United States Food and Drug Administration, three hematology experts evaluated platelet refractoriness, antibody status, and rFVIIa efficacy data on a case-by-case basis to support a potential indication for rFVIIa in GT. Adjudication included 195 patients with 810 events (619 severe bleeding episodes, 192 surgeries), and a consensus algorithm was developed to describe adjudicators' coding of refractoriness and antibody status based on treatment patterns over time. Most rFVIIa-treated events were in patients without refractoriness or antibodies. Adjudicators rated most rFVIIa-treated bleeding episodes as successful (251/266, 94.4%; rFVIIa only, 101/109, 92.7%; rFVIIa ± platelets ± other agents, 150/157, 95.5%); efficacy was consistent in patients with platelet refractoriness ± antibodies (75/79, 94.9%), antibodies only (10/10, 100.0%), and neither/unknown (166/177, 93.8%). Adjudicators also rated most rFVIIa-treated surgeries as successful (159/160, 99.4%; rFVIIa only, 65/66, 98.5%; rFVIIa ± platelets ± other agents, 94/94, 100.0%); efficacy was consistent in patients with platelet refractoriness ± antibodies (69/70, 98.6%), antibodies only (24/24, 100.0%), and neither/unknown (66/66, 100.0%). Unblinding the adjudicators to investigator efficacy ratings changed few assessments. Doses of rFVIIa were narrowly distributed, regardless of other hemostatic agents used.
[Mh] Termos MeSH primário: Plaquetas/imunologia
Fator VIIa/uso terapêutico
Hemorragia/tratamento farmacológico
Hemorragia/etiologia
Isoanticorpos/imunologia
Procedimentos Cirúrgicos Operatórios/efeitos adversos
Trombastenia/complicações
[Mh] Termos MeSH secundário: Adolescente
Criança
Pré-Escolar
Coagulantes/uso terapêutico
Quimioterapia Combinada
Feminino
Seres Humanos
Lactente
Recém-Nascido
Masculino
Proteínas Recombinantes/uso terapêutico
Trombastenia/diagnóstico
Trombastenia/cirurgia
Resultado do Tratamento
Adulto Jovem
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Coagulants); 0 (Isoantibodies); 0 (Recombinant Proteins); AC71R787OV (recombinant FVIIa); EC 3.4.21.21 (Factor VIIa)
[Em] Mês de entrada:1707
[Cu] Atualização por classe:170728
[Lr] Data última revisão:
170728
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170404
[St] Status:MEDLINE
[do] DOI:10.1002/ajh.24741


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[PMID]:28341450
[Au] Autor:Segna E; Artoni A; Sacco R; Giannì AB
[Ad] Endereço:Resident, Department of Maxillofacial Surgery and Odontostomatology, IRCCS Fondazione Ca' Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy. Electronic address: elesegna@hotmail.it.
[Ti] Título:Oral Surgery in Patients With Glanzmann Thrombasthenia: A Case Series.
[So] Source:J Oral Maxillofac Surg;75(2):256-259, 2017 Feb.
[Is] ISSN:1531-5053
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Glanzmann thrombasthenia is a severe defect of platelet function caused by an inherited deficiency or dysfunction of the glycoprotein IIb/IIIa complex, the platelet fibrinogen receptor. Patients with Glanzmann thrombasthenia experience lifelong spontaneous and post-traumatic mucocutaneous bleeding diathesis. Surgery is usually very challenging, requiring close cooperation among surgeons, hematologists, and anesthesiologists. For anatomic reasons, oral surgery is particularly difficult owing to the inherent risk of hemorrhage and the difficulty in achieving local hemostasis. In the present report, we describe 3 successful cases of oral surgery in patients with Glanzmann thrombasthenia and report the surgical and hematologic management of each case.
[Mh] Termos MeSH primário: Procedimentos Cirúrgicos Bucais/efeitos adversos
Trombastenia/complicações
[Mh] Termos MeSH secundário: Adolescente
Perda Sanguínea Cirúrgica/prevenção & controle
Criança
Feminino
Seres Humanos
Masculino
Procedimentos Cirúrgicos Bucais/métodos
Cisto Radicular/complicações
Cisto Radicular/cirurgia
Extração Dentária/efeitos adversos
Extração Dentária/métodos
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Em] Mês de entrada:1704
[Cu] Atualização por classe:170419
[Lr] Data última revisão:
170419
[Sb] Subgrupo de revista:AIM; D; IM
[Da] Data de entrada para processamento:170326
[St] Status:MEDLINE


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[PMID]:27696190
[Au] Autor:Ogawa Y; Kunishima S; Yanagisawa K; Osaki Y; Uchiyama Y; Matsumoto N; Tokiniwa H; Horiguchi J; Nojima Y; Handa H
[Ad] Endereço:Department of Medicine and Clinical Science, Gunma University Graduate School of Medicine, 3-39-22 Showa, Maebashi, Gunma, 371-8511, Japan. yo-ogawa@gunma-u.ac.jp.
[Ti] Título:Successful management of perioperative hemostasis in a patient with Glanzmann thrombasthenia who underwent a right total mastectomy.
[So] Source:Int J Hematol;105(2):221-225, 2017 Feb.
[Is] ISSN:1865-3774
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:Perioperative hemostatic management is a challenge in patients with Glanzmann thrombasthenia (GT). The standard means of preventing surgical bleeding in GT patients is platelet transfusion. However, GT patients often possess alloantibodies against GPIIb/IIIa and/or HLA, which cause resistance to platelet transfusion. HLA-matched platelet transfusion, plasmapheresis, or recombinant human-activated factor VII (rFVIIa) are alternative interventions in such cases. Monitoring of hemostasis is also critical in the management of GT patients who undergo surgery. Here, we report the case of a 56-year-old female GT patient with anti-HLA antibodies, who underwent a right total mastectomy without significant blood loss under HLA-matched platelet transfusion. Bleeding at the surgical site, which occurred on the 18th postoperative day, was successfully treated by immediate bolus administration of rFVIIa and subsequent HLA-matched platelet transfusion. The perioperative hemostatic state was monitored in combination with bleeding time, platelet aggregation assay, and flow cytometric analysis of GPIIb/IIIa expression. Although a flow cytometric analysis is not a functional assay, it enabled the estimation of transfused platelet counts, and helped to inform the decision regarding whether to perform the surgery. Thus, perioperative hemostasis was successfully managed in our GT patient by HLA-matched platelet transfusion, rFVIIa administration, and the close monitoring of hemostasis.
[Mh] Termos MeSH primário: Hemostasia
Mastectomia Radical/métodos
Assistência Perioperatória/métodos
Trombastenia/sangue
[Mh] Termos MeSH secundário: Gerenciamento Clínico
Fator VIIa/administração & dosagem
Fator VIIa/uso terapêutico
Feminino
Seres Humanos
Meia-Idade
Monitorização Fisiológica/métodos
Transfusão de Plaquetas
Proteínas Recombinantes/administração & dosagem
Trombastenia/terapia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Recombinant Proteins); AC71R787OV (recombinant FVIIa); EC 3.4.21.21 (Factor VIIa)
[Em] Mês de entrada:1704
[Cu] Atualização por classe:171109
[Lr] Data última revisão:
171109
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161004
[St] Status:MEDLINE
[do] DOI:10.1007/s12185-016-2096-x


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[PMID]:27273143
[Au] Autor:Ganapule A; Jain P; Abubacker FN; Korula A; Abraham A; Mammen J; George B; Mathews V; Srivastava A; Viswabandya A
[Ad] Endereço:aDepartment of Haematology bDepartment of Immunohaematology and Transfusion Medicine, Christian Medical College, Vellore, Tamil Nadu, India.
[Ti] Título:Surgical procedures in patients with Glanzmann's thrombasthenia: case series and literature review.
[So] Source:Blood Coagul Fibrinolysis;28(2):171-175, 2017 Mar.
[Is] ISSN:1473-5733
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Glanzmann's thrombasthenia is a rare platelet function disorder with an autosomal recessive pattern of inheritance. Achieving haemostasis in such patients who undergo surgical procedures always poses a significant challenge. Herein we report six cases of Glanzmann's thrombasthenia, who underwent nine surgeries under the cover of platelet-rich concentrates with or without recombinant activated factor VII . Of these, five were major surgeries such as thyroidectomy, laparotomy, Hartmann's procedure, reversal of Hartmann's procedure and a complete dental extraction. All five procedures were successfully done without any major bleeding. The major cost incurred in these procedures is due to the large number of blood products used and recombinant activated factor VII if used.
[Mh] Termos MeSH primário: Fator VIIa/uso terapêutico
Trombastenia/cirurgia
[Mh] Termos MeSH secundário: Adolescente
Adulto
Criança
Fator VIIa/administração & dosagem
Feminino
Seres Humanos
Masculino
Proteínas Recombinantes/administração & dosagem
Proteínas Recombinantes/uso terapêutico
Trombastenia/tratamento farmacológico
Adulto Jovem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Recombinant Proteins); AC71R787OV (recombinant FVIIa); EC 3.4.21.21 (Factor VIIa)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170302
[Lr] Data última revisão:
170302
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160609
[St] Status:MEDLINE
[do] DOI:10.1097/MBC.0000000000000524


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[PMID]:26825624
[Au] Autor:Grassetto A; Fullin G; Lazzari F; Panizzo F; Polese F; Gessoni G; Farnia A
[Ad] Endereço:aUOC Anestesia e Rianimazione, Ospedale dell'Angelo di Mestre bUOC Ginecologia e Ostetricia Ospedale dell'Angelo di Mestre cUOC Immunoematologia e Trasfusionale, Ospedale dell'Angelo di Mestre, Zelarino, Venezia, Italy.
[Ti] Título:Perioperative ROTEM and ROTEMplatelet monitoring in a case of Glanzmann's thrombasthenia.
[So] Source:Blood Coagul Fibrinolysis;28(1):96-99, 2017 Jan.
[Is] ISSN:1473-5733
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Glanzmann's thrombasthenia is a rare, inherited disease, which consists in abnormality of the glycoprotein IIa/IIIb genes that causes impaired platelet function. Glanzmann's thrombasthenia patients show prolongation of bleeding time and may experience life-threatening hemorrhage after surgery. We present a case of a young woman having Glanzmann's thrombasthenia, who underwent gynecological operation for ovarian cyst. Perioperative monitoring included thromboelastometry with ROTEM and impedance aggregometry with ROTEMplatelet. In this case, despite acceptable ROTEM results after platelet transfusion, ROTEMplatelet was more indicative of hemostatic impairment and reflected the bleeding occurred in the postoperative period. When treating patients with congenital or acquired platelet dysfunction, some intrinsic limits of thromboelastometry to assess platelet function may be overcome with the combined use of a point-of-care device that measures platelet aggregation.
[Mh] Termos MeSH primário: Espectroscopia Dielétrica/métodos
Trombastenia/genética
Tromboelastografia/métodos
[Mh] Termos MeSH secundário: Adulto
Feminino
Seres Humanos
Período Perioperatório
Sistemas Automatizados de Assistência Junto ao Leito
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170817
[Lr] Data última revisão:
170817
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160131
[St] Status:MEDLINE
[do] DOI:10.1097/MBC.0000000000000532


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[PMID]:28051053
[Au] Autor:Jan IA; AlKaabi SK; AlMughadam A; Murthy DS; AlHamoudi B
[Ad] Endereço:Department of Pediatric Surgery, Zayed Military Hospital, Abu Dhabi, United Arab Emirates.
[Ti] Título:Simultaneous occurrence of intussusception and duodenal haematoma in a patient with Glanzmann's thrombasthenia.
[So] Source:Afr J Paediatr Surg;13(4):202-205, 2016 Oct-Dec.
[Is] ISSN:0974-5998
[Cp] País de publicação:India
[La] Idioma:eng
[Ab] Resumo:Glanzmann's thrombasthenia (GT) is a rare autosomal recessive disorder in which the platelets have anomalies of fibrinogen receptors causing bleeding tendencies. The disease usually presents with mucosal bleeding, petechial haemorrhages or gastrointestinal (GI) bleeding. Only sporadic cases of intussusception or duodenal haematoma have been reported with GT. We present a case of 5-year-old girl admitted with non-bilious vomiting, watery diarrhoea and abdominal pain. She is a known patient of Glanzmann's thrombasthenia.
[Mh] Termos MeSH primário: Duodenopatias/etiologia
Hematoma/etiologia
Doenças do Íleo/etiologia
Valva Ileocecal
Intussuscepção/etiologia
Trombastenia/complicações
[Mh] Termos MeSH secundário: Angiografia
Pré-Escolar
Duodenopatias/diagnóstico
Duodenopatias/cirurgia
Feminino
Hematoma/diagnóstico
Hematoma/cirurgia
Seres Humanos
Doenças do Íleo/diagnóstico
Doenças do Íleo/cirurgia
Intussuscepção/diagnóstico
Intussuscepção/cirurgia
Laparoscopia/métodos
Imagem por Ressonância Magnética
[Pt] Tipo de publicação:CASE REPORTS
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170901
[Lr] Data última revisão:
170901
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170105
[St] Status:MEDLINE
[do] DOI:10.4103/0189-6725.194676


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[PMID]:27655515
[Au] Autor:Imperiale L; Manganaro L; Ticino A; Piacenti I; Anastasi E; Resta S; Benedetti Panici P; Porpora MG
[Ad] Endereço:Department of Gynecology, Obstetrics and Urology, Sapienza, University of Rome, Policlinico Umberto I, Rome, Italy.
[Ti] Título:Endometriosis and Glanzmann’s thrombasthenia.
[So] Source:J Biol Regul Homeost Agents;30(3):877-882, 2016 Jul-Sep.
[Is] ISSN:0393-974X
[Cp] País de publicação:Italy
[La] Idioma:eng
[Ab] Resumo:Glanzmann’s thrombasthenia (GT) is a rare bleeding syndrome characterized by deficiency or defect of platelet aggregation complex. The pathogenesis of endometriosis is controversial but the strongest evidence leans towards retrograde menstruation. GT probably predisposes to endometriosis. The management of women affected by this disease can be difficult due to the risk of bleeding complications, especially during surgical treatment. We describe the cases of three sisters affected by endometriosis and GT, referred to our Department, who received different therapeutic management.
[Mh] Termos MeSH primário: Endometriose/etiologia
Trombastenia/complicações
[Mh] Termos MeSH secundário: Adulto
Anticoncepcionais Orais Hormonais/efeitos adversos
Anticoncepcionais Orais Hormonais/uso terapêutico
Suscetibilidade a Doenças
Doenças em Gêmeos
Endometriose/diagnóstico por imagem
Endometriose/tratamento farmacológico
Endometriose/cirurgia
Fator VIIa/uso terapêutico
Feminino
Hematometra/etiologia
Transtornos Hemorrágicos/tratamento farmacológico
Transtornos Hemorrágicos/etiologia
Seres Humanos
Dispositivos Intrauterinos Medicados
Levanogestrel/uso terapêutico
Acetato de Medroxiprogesterona/uso terapêutico
Menorragia/etiologia
Doenças Ovarianas/diagnóstico por imagem
Doenças Ovarianas/tratamento farmacológico
Doenças Ovarianas/etiologia
Doenças Ovarianas/cirurgia
Assistência Perioperatória
Proteínas Recombinantes/uso terapêutico
Doenças Retais/diagnóstico por imagem
Doenças Retais/tratamento farmacológico
Doenças Retais/etiologia
Trombastenia/genética
Ácido Tranexâmico/uso terapêutico
Pamoato de Triptorrelina/uso terapêutico
Doenças Vaginais/diagnóstico por imagem
Doenças Vaginais/tratamento farmacológico
Doenças Vaginais/etiologia
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Contraceptives, Oral, Hormonal); 0 (Recombinant Proteins); 57773-63-4 (Triptorelin Pamoate); 5W7SIA7YZW (Levonorgestrel); 6T84R30KC1 (Tranexamic Acid); AC71R787OV (recombinant FVIIa); C2QI4IOI2G (Medroxyprogesterone Acetate); EC 3.4.21.21 (Factor VIIa)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170202
[Lr] Data última revisão:
170202
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160923
[St] Status:MEDLINE


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[PMID]:27539755
[Au] Autor:Iqbal I; Farhan S; Ahmed N
[Ad] Endereço:Department of Hematology, Postgraduate Medical Institute/ Ameer Uddin Medical College, Lahore.
[Ti] Título:Glanzmann Thrombasthenia: A Clinicopathological Profile.
[So] Source:J Coll Physicians Surg Pak;26(8):647-50, 2016 Aug.
[Is] ISSN:1681-7168
[Cp] País de publicação:Pakistan
[La] Idioma:eng
[Ab] Resumo:OBJECTIVE: To describe the clinical presentation of patients with Glanzmann's thrombasthenia (GT) and evaluate their diagnostic, clinical, and laboratory parameters including platelet aggregometry. STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Department of Hematology and Blood Transfusion, The Children Hospital and Institute of Child Health, Lahore, from January 2006 to December 2013. METHODOLOGY: Patients presenting with mucocutaneous bleeding during study period and evaluated for diagnosis of inherited platelet function disorder, were included. Clinical data and family history were recorded. Laboratory investigations including complete blood count (CBC), peripheral blood smear (PBS), bleeding time (BT), activated partial thromboplastin time (APTT), prothrombin time (PT), and platelet aggregation studies were evaluated. RESULTS: Among 796 patients, 163 (20.4%) patients were diagnosed with Glanzmann's thrombasthenia. The male to female ratio was 1.2:1. Their mean age was 7 ±2.5 years ranging from 3 months to 35 years. Consanguinity was observed in 65% patients. Common presenting symptoms included easy bruisibility (76.6%), gum bleeding (56.4%), epistaxis (62.5%), and prolonged bleeding after injury (47.2%). Bleeding time was prolonged in 92%. Platelet aggregation studies showed decreased aggregation with ADP, Collagen and Epinephrine in 100% of these patients and 9.2% showed decreased aggregation with Ristocetin also. CONCLUSION: Glanzmann thrombasthenia was seen in a substantial number of patients (20.4%), possibly due to consanguineous marriages. GTpatients presented from early age to adulthood and raised awareness hoping to help in early diagnosis and more appropriate management. Extensive collaborated studies are needed to predict the true incidence of GTin Pakistan.
[Mh] Termos MeSH primário: Plaquetas
Agregação Plaquetária
Trombastenia/diagnóstico
[Mh] Termos MeSH secundário: Adulto
Contagem de Células Sanguíneas
Criança
Pré-Escolar
Consanguinidade
Feminino
Citometria de Fluxo
Seres Humanos
Masculino
Contagem de Plaquetas
Testes de Função Plaquetária
Trombastenia/sangue
Trombastenia/fisiopatologia
[Pt] Tipo de publicação:EVALUATION STUDIES; JOURNAL ARTICLE
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170630
[Lr] Data última revisão:
170630
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160820
[St] Status:MEDLINE
[do] DOI:2396


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[PMID]:27478149
[Au] Autor:Eghbali A; Melikof L; Taherahmadi H; Bagheri B
[Ad] Endereço:Department of Pediatric Hematology and Oncology, Arak University of Medical Sciences, Arak, Iran.
[Ti] Título:Efficacy of tranexamic acid for the prevention of bleeding in patients with von Willebrand disease and Glanzmann thrombasthenia: a controlled, before and after trial.
[So] Source:Haemophilia;22(5):e423-6, 2016 Sep.
[Is] ISSN:1365-2516
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Reducing bleeding episodes is very important in haematology disorders like von Willebrand disease (VWD) and Glanzmann thrombasthenia (GT). Replacement factors are very expensive although prophylactic drugs are affordable. OBJECTIVE: To study the prophylactic effects of tranexamic acid (TXA) for reduction of bleeding episodes in patients with VWD and GT in non-invasive conditions. METHODS: A controlled, double-blind before and after single-centre trial was performed in Amir-Kabir Hospital (Arak, Iran). The study was done on 17 patients with VWD and three patients with GT with minimum age of 2 years. Patients were received placebo for 6 months to evaluate the frequency and severity of bleeding and also to record the frequency of use of factor concentrates and platelet transfusion. After that, patients were given oral single dose of TXA 25 mg kg(-1) day(-1) for 6 months. The mentioned outcomes were studied and compared between two phases of study. Safety assessment was done in all patients. RESULTS: Tranexamic acid caused a significant reduction in number of Grade 1 and Grade 2 bleeding episodes in VWD patients (P < 0.001 and P < 0.01 respectively). In addition, TXA therapy was associated with significant decrease in the use of factor concentrates (P < 0.05). Number of bleeding episodes decreased in GT patients who used TXA; however, difference between two phases of studies was not significant (P = 0.1). TXA had no effect in the frequency of platelet transfusions in GT patients. TXA therapy was associated with headache, back pain and musculoskeletal pain. No case of allergy or thromboembolic events was seen following treatment. CONCLUSIONS: The results suggest that TXA is safe and effective to reduce bleeding and use of factor concentrates in VWD patients. In addition, TXA therapy can decrease bleeding in GT patients.
[Mh] Termos MeSH primário: Antifibrinolíticos/uso terapêutico
Hemorragia/prevenção & controle
Trombastenia/tratamento farmacológico
Ácido Tranexâmico/uso terapêutico
Doenças de von Willebrand/tratamento farmacológico
[Mh] Termos MeSH secundário: Pré-Escolar
Estudos Controlados Antes e Depois
Método Duplo-Cego
Feminino
Hemorragia/etiologia
Seres Humanos
Lactente
Irã (Geográfico)
Masculino
Transfusão de Plaquetas
Trombastenia/complicações
Resultado do Tratamento
Doenças de von Willebrand/complicações
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antifibrinolytic Agents); 6T84R30KC1 (Tranexamic Acid)
[Em] Mês de entrada:1703
[Cu] Atualização por classe:170315
[Lr] Data última revisão:
170315
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160802
[St] Status:MEDLINE
[do] DOI:10.1111/hae.13051



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