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2.
J. venom. anim. toxins incl. trop. dis ; 29: e20220088, 2023. tab, graf, ilus
Article in English | LILACS, VETINDEX | ID: biblio-1440485

ABSTRACT

Abstract Background: Twenty-minute whole blood clotting test (20WBCT) and Modified Lee and White (MLW) method are the most routinely employed bedside tests for detecting coagulopathic snake envenomation. Our study compared the diagnostic utility of MLW and 20WBCT for snakebite victims at a tertiary care hospital in Central Kerala, South India. Methods: This single-center study recruited 267 patients admitted with snake bites. 20WBCT and MLW were performed simultaneously at admission along with the measurement of Prothrombin Time (PT). The diagnostic utility of 20WBCT and MLW was determined by comparing the sensitivity (Sn), specificity (Sp), positive and negative predictive values, likelihood ratios, and accuracy at admission with an INR value > 1.4. Results: Out of 267 patients, 20 (7.5%) patients had VICC. Amongst those who had venom-induced consumption coagulopathy (VICC), MLW was prolonged for 17 patients, (Sn 85% 95% confidence interval [CI]: 61.1-96.0) whereas 20WBCT was abnormal for 11 patients (Sn 55%, 95% CI: 32.04-76.17). MLW and 20WBCT were falsely positive for the same patient (Sp 99.6%, 95% CI: 97.4-99.9%). Conclusion: MLW is more sensitive than 20WBCT to detect coagulopathy at the bedside amongst snakebite victims. However, further studies are necessary for standardizing bedside coagulation tests in snakebite cases.


Subject(s)
Prothrombin Time/methods , Snake Bites/diagnosis , Blood Coagulation Disorders/diagnosis , Blood Coagulation Factors/analysis
3.
Journal of Experimental Hematology ; (6): 489-494, 2023.
Article in Chinese | WPRIM | ID: wpr-982085

ABSTRACT

OBJECTIVE@#To screen better promoters and provide more powerful tools for basic research and gene therapy of hemophilia.@*METHODS@#Bioinformatics methods were used to analyze the promoters expressing housekeeping genes with high abundance, so as to select potential candidate promoters. The GFP reporter gene vector was constructed, and the packaging efficiency of the novel promoter was investigated with EF1 α promoter as control, and the transcription and activities of the reporter gene were investigated too. The activity of the candidate promoter was investigated by loading F9 gene.@*RESULTS@#The most potential RPS6 promoter was obtained by screening. There was no difference in lentiviral packaging between EF1 α-LV and RPS6-LV, and their virus titer were consistent. In 293T cells, the transduction efficiency and mean fluorescence intensity of RPS6pro-LV and EF1 αpro-LV were proportional to the lentiviral dose. The transfection efficiency of both promoters in different types of cells was in the following order: 293T>HEL>MSC; Compared with EF1 αpro-LV, RPS6pro-LV could obtain a higher fluorescence intensity in MSC cells, and RPS6pro-LV was more stable in long-term cultured HEL cells infected with two lentiviruses respectively. The results of RT-qPCR, Western blot and FIX activity (FIX∶C) detection of K562 cell culture supernatant showed that FIX expression in the EF1 α-F9 and RPS6-F9 groups was higher than that in the unloaded control group, and there was no significant difference in FIX expression between the EF1 α-F9 and RPS6-F9 groups.@*CONCLUSION@#After screening and optimization, a promoter was obtained, which can be widely used for exogenous gene expression. The high stability and viability of the promoter were confirmed by long-term culture and active gene expression, which providing a powerful tool for basic research and clinical gene therapy of hemophilia.


Subject(s)
Humans , Transduction, Genetic , Genetic Vectors , Hemophilia A/genetics , Transfection , Blood Coagulation Factors/genetics , Lentivirus/genetics
4.
J. Health Biol. Sci. (Online) ; 10(1): 1-6, 01/jan./2022. tab, graf
Article in Portuguese | LILACS | ID: biblio-1411592

ABSTRACT

Objetivo: Avaliar o perfil clínico-terapêutico e a resposta à profilaxia em pacientes hemofílicos A e B em um centro de referência no Ceará. Métodos: Estudo de coorte retrospectivo, com dados de 133 hemofílicos A e B, em profilaxia entre 2016 e 2021, por meio de prontuários médicos e sistema Web Coagulopatias. Resultados: Os pacientes todos do sexo masculino em sua maioria foram hemofílicos A (93,2%), na forma grave, residentes em Fortaleza, com maior prevalência do município de Guaiúba. A maioria fazia uso de Fator VIII recombinante e em profilaxia secundária, em relação ao comprometimento articular a maioria não apresentou relato de hemartroses (66,9%), articulação-alvo (87,9%) ou artropatia (54,9%), porém os hemofílicos em profilaxia terciária apresentaram um maior comprometimento articular em relação a profilaxia primária e secundária. Verificou-se uma correlação negativa entre o tempo de profilaxia e a dose de fator utilizada, demonstrando que quanto maior o tempo de profilaxia menor a dose do fator utilizada. Um total de 13 hemofílicos A grave desenvolveram inibidor de fator VIII realizando imunotolerância (ITI) com sucesso total em 84,6%. Por meio da curva ROC, foi verificado uma associação entre a necessidade de ITI e a dose de fator de coagulação, com acurácia de 67,7% de que o uso de doses maiores de fator predispõe ao desenvolvimento de inibidores. Conclusão: Os dados do estudo permitem inferir que quanto mais precoce o tratamento de profilaxia menor é comprometimento articular, dose do fator utilizada e menor predisposição de desenvolver inibidores dos fatores da coagulação.


Objective: to evaluate the clinical-therapeutic profile and response to prophylaxis in hemophiliac A and B patients at a referral center in Ceará. Methods: Retrospective cohort study, with data from 133 hemophiliacs A and B, undergoing prophylaxis between 2016 and 2021, using medical records and the Web Coagulopathies system. Results: Most of the patients were male patients with severe hemophilia A (93.2%), residing in Fortaleza, with a higher prevalence in the city of Guaiúba. Most made use of recombinant Factor VIII and in secondary prophylaxis, in relation to joint involvement, the majority did not report hemarthroses (66.9%), target joint (87.9%) or arthropathy (54.9%). however, hemophiliacs on tertiary prophylaxis showed greater joint impairment in relation to primary and secondary prophylaxis. There was a negative correlation between the prophylaxis time and the factor dose used, demonstrating that the longer the prophylaxis time, the lower the factor dose used. A total of 13 severe A hemophiliacs developed factor VIII inhibitor performing immunotolerance (ITI) with total success in 84.6%. Using the ROC curve, an association was verified between the need for ITI and the dose of coagulation factor, with an accuracy of 67.7% that the use of higher doses of factor predisposes to the development of inhibitors. Conclusion: The study data allow us to infer that the earlier the prophylaxis treatment, the less joint impairment, the dose of the factor used and the less predisposition to develop coagulation factor inhibitors.


Subject(s)
Humans , Animals , Male , Young Adult , Hemophilia B/prevention & control , Hemophilia A/prevention & control , Blood Coagulation , Brazil/epidemiology , Blood Coagulation Factors/administration & dosage , Prevalence , Retrospective Studies , Hemophilia B/epidemiology , Disease Prevention , Evaluation of the Efficacy-Effectiveness of Interventions , Hemarthrosis/prevention & control , Hemophilia A/epidemiology , Joint Diseases/prevention & control
5.
Cambios rev. méd ; 21(1): 798, 30 Junio 2022. tabs, grafs.
Article in Spanish | LILACS | ID: biblio-1400361

ABSTRACT

INTRODUCCIÓN. La hemofilia es una condición rara hereditaria, crónica, potencialmente discapacitante e incapacitante, caracterizada por frecuentes sangrados debidos al déficit del factor VIII coagulante, Hemofilia A o del factor IX Hemofilia B. Las evaluaciones de calidad de vida en personas con hemofilia, basadas principalmente en el aspecto biológico, llevaron a considerar un importante enfoque bioético que evalúe la afectación de la autonomía y dignidad debida a la enfermedad. OBJETIVO. Registrar la percepción de la autonomía y dignidad de personas que viven con hemofilia. MATERIALES Y MÉTODOS. Estudio descriptivo transversal. Población de 92 y muestra de 28 varones mayores de 18 años con diagnóstico de hemofilia, atendidos en la Clínica de Coagulopatías Congénitas del Hospital de Especialidades Carlos Andrade Marín en el periodo marzo 2021 a agosto del 2021. Se excluyó a varones menores de 18 años atendidos en otras instituciones del Sistema Nacional de Salud. Estudio basado en el desarrollo de las capacidades centrales descritas por Martha Nussbaum. Se aplicó el test The Hemophilia Well Being Index que evaluó calidad de vida con relación al bienestar personal asociado a salud, y la herramienta Body Mapping que analizó en base al interpretativismo fenomenológico. RESULTADOS. El 100% de personas presentaron afectación en algún área de la vida investigada por el Hemophilia Well Being Index, que se confirma con las expresiones escritas y gráficas recopiladas por el Body Mapping. CONCLUSIÓN. La autonomía y dignidad se encuentran afectadas en las personas que viven con hemofilia, al igual que las capacidades centrales; es importante valorar cómo estos parámetros afectan la consecución de logros, lo que se debe considerar en estudios futuros.


INTRODUCTION. Hemophilia is a rare hereditary, chronic, potentially disabling and incapacitating condition, characterized by frequent bleeds due to deficiency of clotting factor VIII, Hemophilia A or factor IX Hemophilia B. Quality of life assessments in people with hemophilia, mainly based on the biological aspect, led to consider an important bioethical approach that evaluates the impairment of autonomy and dignity due to the disease. OBJECTIVE. To record the perception of autonomy and dignity of people living with hemophilia. MATERIALS AND METHODS. Cross-sectional descriptive study. Population of 92 and sample of 28 males over 18 years of age with a diagnosis of hemophilia, attended at the Congenital Coagulopathy Clinic of the Carlos Andrade Marin Specialty Hospital in the period March 2021 to August 2021. Males under 18 years of age attended in other institutions of the National Health System were excluded. The study was based on the development of the central capabilities described by Martha Nussbaum. The test The Hemophilia Well Being Index was applied, which evaluated quality of life in relation to personal wellbeing associated with health, and the tool Body Mapping which analyzed based on phenomenological interpretivism. RESULTS. 100% of people presented affectation in some area of life investigated by the Hemophilia Well Being Index, which is confirmed by the written and graphic expressions collected by the Body Mapping. CONCLUSION. Autonomy and dignity are affected in people living with hemophilia, as are core capacities; it is important to assess how these parameters affect achievement, which should be considered in future studies.


Subject(s)
Humans , Male , Adolescent , Adult , Middle Aged , Aged , Young Adult , Perception , Quality of Life , Hemophilia B , Personal Autonomy , Patient Care , Hemophilia A , Blood Coagulation , Blood Coagulation Factors , Factor IX , Factor XIII , Chronic Disease , Civil Rights , Chronic Disease Indicators
6.
Chinese Journal of Hematology ; (12): 26-30, 2022.
Article in Chinese | WPRIM | ID: wpr-929525

ABSTRACT

Objective: To analyze the clinical characteristics, laboratory examination, diagnosis, treatment, and outcome of hereditary factor Ⅹ (FⅩ) deficiency. Methods: Clinical data of 11 patients with congenital FⅩ deficiency were retrospectively analyzed from July 2009 to February 2021. Results: There were 3 males and 8 females. Median age was 39 (5-55) years. The media duration of follow-up was 81.67 (1.87-142.73) months. Of the 11 patients, 10 had bleeding symptoms, 7 had ecchymosis or hemorrhage after skin bump, 7 had nosebleed, 6 had gingival hemorrhage, and 1 had muscle hematoma. Among the female patients, 6 had menorrhagia and 1 experienced bleeding after vaginal delivery. Family history of FⅩ deficiency was found in one case. Eight patients had a history of surgery, and four had postoperative bleeding. Laboratory findings were characterized by significantly prolonged activated partial thromboplastin time, prothrombin time, and decreased FⅩ activity (FⅩ∶C) . Four cases underwent gene mutation analysis and five new mutations were found. Four cases were treated with prothrombin complex concentrates (PCC) and seven cases with fresh frozen plasma (FFP) . One female patient had significantly reduced menstrual volume after PCC prophylactic therapy. One patient received FFP for prophylactic infusion with no bleeding during and after the operation. Conclusion: Most patients with congenital FⅩ deficiency had bleeding symptoms and there was no significant correlation between severity of bleeding symptoms and FⅩ∶C. Prophylaxis should be applied in patients with severe bleeding tendencies. Gene mutation test is significant for screening, diagnosis, and prognosis prediction of congenital FX deficiency.


Subject(s)
Adolescent , Adult , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Young Adult , Blood Coagulation Factors/therapeutic use , Blood Coagulation Tests , Factor X Deficiency/genetics , Hemorrhage/drug therapy , Plasma , Retrospective Studies
7.
Acta Paul. Enferm. (Online) ; 35: eAPE02902, 2022. tab, graf
Article in Portuguese | LILACS, BDENF | ID: biblio-1364225

ABSTRACT

Resumo Objetivo Elaborar e validar tecnologias educativas para o cuidado domiciliar de pessoas com hemofilia em infusão endovenosa do fator de coagulação. Método Estudo metodológico, desenvolvido em três etapas: elaboração de tecnologias educativas, avaliação de conteúdo e aparência por juízes e apreciação por pessoas com hemofilia. As tecnologias educativas − cartilha e infográfico − foram elaboradas mediante revisão de literatura. Na etapa de avaliação com juízes, foi utilizada a técnica Delphi em duas rodadas, por meio do Índice de Validade de Conteúdo, concordância superior a 0,80 em relação a clareza de linguagem, pertinência prática e relevância teórica. Na avaliação com o público-alvo, foi considerado o nível de concordância de respostas positivas maior ou igual a 80% nos itens de organização, o estilo de escrita, a aparência e a motivação para a leitura. Resultados A cartilha apresentou índice de validade de conteúdo global de 0,88 na primeira rodada e 0,98 na segunda; e o infográfico, 0,88 na primeira rodada e 0,97 na segunda. Na apreciação das tecnologias educativas pelo público-alvo, o nível de concordância das respostas positivas foi superior a 80%. Conclusão Este estudo elaborou tecnologias educativas, cartilha e infográfico, que poderão contribuir com a adesão ao tratamento e promoção do cuidado, por meio de padronização das orientações às pessoas com hemofilia em infusão endovenosa domiciliar.


Resumen Objetivo Elaborar y validar tecnologías educativas para el cuidado domiciliario de personas con hemofilia sobre la infusión intravenosa domiciliaria del factor de coagulación. Métodos Estudio metodológico desarrollado en tres etapas: elaboración de tecnologías educativas, evaluación de contenido y apariencia por jueces y valoración por parte de personas con hemofilia. Las tecnologías educativas (cartilla e infográfico) fueron elaboradas mediate revisión de literatura. En la etapa de evaluación con jueces, se utilizó el método Delphi en dos rondas, por medio del Índice de Validez de Contenido, concordancia superior a 0,80 con relación a la claridad del lenguaje, pertinencia práctica y relevancia teórica. En la evaluación con el público destinatario, se consideró el nivel de concordancia de respuestas positivas mayor o igual a 80 % en los ítems organización, estilo de escritura, apariencia y motivación para lectura. Resultados La cartilla presentó un Índice de Validez de Contenido global de 0,88 en la primera ronda y 0,98 en la segunda. El infográfico presentó 0,88 en la primaria ronda y 0,97 en la segunda. En la valoración de las tecnologías educativas por el público destinatario, el nivel de concordancia de las respuestas positivas fue superior al 80 %. Conclusión En este estudio se elaboraron tecnologías educativas, cartilla e infográfico, que podrán contribuir con la adherencia al tratamiento y promoción del cuidado, mediante la estandarización de las instrucciones a personas con hemofilia sobre infusión intravenosa domiciliaria.


Abstract Objective To develop and validate educational technologies for home care of people with hemophilia on intravenous infusion of clotting factor. Method This is a methodological study, developed in three stages: development of educational technologies, content and appearance assessment by judges, and appraisal by people with hemophilia. The educational technologies - a booklet and an infographic - were developed through a literature review. In the assessment stage with judges, the Delphi technique was used in two rounds, through Content Validity Index, agreement greater than 0.80 in regarding clarity of language, practical relevance, and theoretical relevance. In the assessment with the target audience, the level of agreement of positive responses greater than or equal to 80% in the items of organization, writing style, appearance and motivation for reading was considered. Results The booklet presented a global content validity index of 0.88 in the first round and 0.98 in the second; and the infographic, 0.88 in the first round and 0.97 in the second. In the assessment of educational technologies by the target audience, the level of agreement of positive responses was higher than 80%. Conclusion This study developed educational technologies, a booklet and an infographic, which could contribute to adherence to treatment and promotion of care, by standardizing the guidelines for people with hemophilia in intravenous infusion at home.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Infusions, Intravenous , Blood Coagulation Factors , Home Infusion Therapy , Educational Technology , Educational and Promotional Materials , Hemophilia A/therapy , Home Nursing
9.
Journal of Experimental Hematology ; (6): 615-619, 2021.
Article in Chinese | WPRIM | ID: wpr-880122

ABSTRACT

OBJECTIVE@#To compare the plasma components of frozen plasma (FP) and fresh frozen plasma (FFP).@*METHODS@#Twenty samples of FP and 20 samples of FFP from Beijing Red Cross Blood Center were randomly selected. Immediately after plasma melting, 12 plasma components including coagulation factor, fibrinolytic system and anticoagulation protein were detected, including activated partial thromboplastin time (APTT), prothrombin time (PT), coagulation factor Ⅷ (FⅧ) activity, coagulation factor Ⅴ (FⅤ) activity, fibrinogen(FIB) level, ADAMTS-13 activity, von Willebrand factor(vWF) activity, D-dimer (D-dimer, DD), fibrin degradation products (FDP), antithrombin (AT), protein C (PC), and protein S (PS). All these coagulation components between the two types of plasma were compared and analyzed.@*RESULTS@#Compared with FFP, APTT in FP was significantly prolonged(t=3.428, P0.05).@*CONCLUSION@#FP can substitute FFP in the treatment of some diseases, although it is lack of some coagulation factors and anticoagulation protein.


Subject(s)
Humans , Beijing , Blood Coagulation , Blood Coagulation Factors , Blood Coagulation Tests , Plasma
11.
Interface (Botucatu, Online) ; 24: e180722, 2020.
Article in Portuguese | LILACS | ID: biblio-1101229

ABSTRACT

A hemofilia é uma doença rara, hereditária e caracterizada pela falta de fatores de coagulação, o que provoca sangramentos espontâneos e artropatias incapacitantes. O componente mais dispendioso em seu tratamento é a reposição do fator de coagulação. O presente ensaio examina, a partir da perspectiva da Bioética Crítica, modelo teórico baseado na articulação da Teoria Crítica com os Estudos da Colonialidade, o panorama do acesso global ao tratamento e o programa brasileiro. Demonstrou-se um quadro marcado por extremas disparidades de acesso em nível tanto global quanto nacional, cujas causas estão diretamente relacionadas com a formação histórica de um sistema-mundo baseado na dominação dos meios materiais, ideias e instituições pelos países centrais. Para seu enfrentamento, concluiu-se pela necessidade de contínuas pressões sociais, estímulo a uma produção científica e regulação tecnológica verdadeiramente comprometida com o cumprimento do direito fundamental à saúde.(AU)


Hemophilia is a rare, hereditary disease characterized by a lack of clotting factors, which causes spontaneous bleeding and disabling arthropathy. The most expensive component in its treatment is clotting factor replacement therapy. This essay examines, based on the perspective of Critical Bioethics - a theoretical model based on the articulation between Critical Theory and Coloniality Studies -, the panorama related to the global access to that treatment and the Brazilian program. A scenario marked by extreme disparities of access was found, both in the global and in the national levels, whose causes are directly related to the historical formation of a world system based on the domination of material means, ideas and institutions by central countries. To face this situation, a continuous social pressure is needed, as well as incentive to scientific production and technological regulation truly committed to the enforcement of the fundamental right to health.(AU)


La hemofilia es una enfermedad rara, hereditaria, caracterizada por la falta de factores de coagulación, lo que provoca sangrados espontáneos y artropatías discapacitantes. El componente más caro de su tratamiento es la reposición del factor de coagulación. El presente ensayo examina, a partir de la perspectiva de la Bioética Crítica, un modelo teórico basado en la articulación de la Teoría Crítica con los Estudios de la Colonialidad, el panorama del acceso global al tratamiento y el programa brasileño. Demostró ser un cuadro marcado por disparidades extremas de acceso, tanto en nivel global como nacional, cuyas causas están directamente relacionadas con la formación histórica de un sistema de mundo basado en la dominación de los medios materiales, ideas e instituciones por los países centrales. La conclusión para enfrentarlo fue la necesidad de continuas presiones sociales y estímulo a una producción científica y regulación tecnológica verdaderamente comprometidas con el cumplimiento del derecho fundamental a la salud.(AU)


Subject(s)
Humans , Bioethics/trends , Global Health/ethics , Health Services Accessibility/ethics , Hemophilia A/drug therapy , Brazil , Blood Coagulation Factors/therapeutic use , Public Health/ethics
12.
Rev. bras. cir. cardiovasc ; 34(3): 327-334, Jun. 2019. tab, graf
Article in English | LILACS | ID: biblio-1013461

ABSTRACT

Abstract Objective: The main goal of our study was to assess the impact of vascular procedures on the activity of hemostatic and fibrinolytic pathways. Methods: We enrolled 38 patients with ≥ 45 years old undergoing surgery for abdominal aortic aneurysm or peripheral artery disease under general or regional anesthesia and who were hospitalized at least one night after the procedure. Patients undergoing carotid artery surgery and those who had acute bypass graft thrombosis, cancer, renal failure defined as estimated glomerular filtration rate < 30 ml/min/1.73m2, venous thromboembolism three months prior to surgery, or acute infection were excluded from the study. We measured levels of markers of hemostasis (factor VIII, von Willebrand factor:ristocetin cofactor [vWF:CoR], antithrombin), fibrinolysis (D-dimer, tissue plasminogen activator [tPA], plasmin-antiplasmin complexes), and soluble cluster of differentiation 40 ligand (sCD40L) before and 6-12h after vascular procedure. Results: Significant differences between preoperative and postoperative levels of factor VIII (158.0 vs. 103.3, P<0.001), antithrombin (92.1 vs. 74.8, P<0.001), D-dimer (938.0 vs. 2406.0, P=0.005), tPA (10.1 vs. 12.8, P=0.002), and sCD40L (9092.9 vs. 1249.6, P<0.001) were observed. There were no significant differences between pre- and postoperative levels of vWF:CoR (140.6 vs. 162.8, P=0.17) and plasmin-antiplasmin complexes (749.6 vs. 863.7, P=0.21). Conclusion: Vascular surgery leads to significant alterations in hemostatic and fibrinolytic systems. However, the direction of these changes in both pathways remains unclear and seems to be different depending on the type of surgery. A study utilizing dynamic methods of coagulation and fibrinolysis assessment performed on a larger population is warranted.


Subject(s)
Humans , Male , Female , Middle Aged , Aged , Vascular Surgical Procedures/adverse effects , Blood Coagulation/physiology , Aortic Aneurysm, Abdominal/surgery , Peripheral Arterial Disease/surgery , Fibrinolysis/physiology , Postoperative Period , Reference Values , Blood Coagulation Factors/analysis , Enzyme-Linked Immunosorbent Assay , Biomarkers/blood , Pilot Projects , Risk Factors , Treatment Outcome , Statistics, Nonparametric , Preoperative Period
14.
Acta Academiae Medicinae Sinicae ; (6): 685-689, 2019.
Article in Chinese | WPRIM | ID: wpr-775975

ABSTRACT

Bullous pemphigoid(BP)is an autoimmune blister disease caused by antibodies to the basement membrane zone.It usually exhibits tense bullae in skin and mucous membrane.BP patients can suffer from thrombosis due to hypercoagulation and/or hemorrhage due to the presence of anti-coagulation factors,which may be explained by the eosinophils,antibodies to the coagulation factors,and fibrinolytic system hyperactivity or inhibition.


Subject(s)
Humans , Autoantibodies , Blister , Blood Coagulation Disorders , Blood Coagulation Factors , Eosinophils , Hemorrhage , Mucous Membrane , Pathology , Pemphigoid, Bullous , Skin , Pathology , Thrombosis
15.
Journal of Acute Care Surgery ; (2): 72-75, 2019.
Article in English | WPRIM | ID: wpr-785887

ABSTRACT

Acquired hemophilia A (AHA) is a rare disease where typically coagulation factor VIII is inhibited by autoantibodies. It occurs in patients with no personal or familial history of bleeding. In this case study a 61-year-old male presented with a huge psoas hematoma. He had no history of bleeding disorders. He was initially diagnosed with delayed traumatic hematoma. Despite conservative and surgical treatments, coagulopathy was not resolved and postoperative bleeding continued. Consequently, coagulation factor tests were performed and showed reduced activity of factor VIII (2.7%). In addition, factor VIII inhibitor was detected. The patient was diagnosed with AHA and administered recombinant factor VIII for 3 days which resulted in the cessation of bleeding. AHA can lead to a life-threatening hemorrhage, and needs to be considered in differential diagnoses in any patients presenting with unexplained and repeated bleeding, where there is no personal or familial history of bleeding disorders.


Subject(s)
Humans , Male , Middle Aged , Autoantibodies , Blood Coagulation Disorders , Blood Coagulation Factors , Diagnosis, Differential , Factor VIII , Hematoma , Hemophilia A , Hemorrhage , Rare Diseases
16.
Clinical Pediatric Hematology-Oncology ; : 55-59, 2019.
Article in English | WPRIM | ID: wpr-763504

ABSTRACT

Hemophilia, an inherited bleeding disorder, is caused by a deficiency of coagulation factor VIII or IX. Most of patients with hemophilia need vascular procedure, which can lead to complications. Even though these complications can also occur in normal people, hemophilia and coagulopathy are particular risk factors. We reviewed medical records of patients with hemophilia who underwent vascular procedures and investigated its complications. Vessel-related complications occurred in five patients. Three patients had pseudoaneurysms after radial arterial puncture. All patients underwent coagulation factor replacement or ultrasound-guided compression and showed improvement. Neuropathy developed in one patient due to a hematoma that occurred after blood sampling. The hematoma improved, but motor and sensory deficits remained and neuropathy was confirmed. One patient died of uncontrolled bleeding after angiography. Vascular procedures require more attention in patients with hemophilia. Caution and prevention of complications is essential, even before the patient is diagnosed with hemophilia.


Subject(s)
Humans , Aneurysm, False , Angiography , Blood Coagulation Factors , Factor VIII , Hematoma , Hemophilia A , Hemorrhage , Medical Records , Punctures , Risk Factors
17.
Anesthesia and Pain Medicine ; : 371-379, 2019.
Article in English | WPRIM | ID: wpr-785373

ABSTRACT

During pregnancy, the procoagulant activity increases (manifested by elevation in factor VII, factor VIII, factor X, and fibrinogen levels), while the anticoagulant activity decreases (characterized by reduction in fibrinolysis and protein S activity), resulting in hypercoagulation. Standard coagulation tests, such as prothrombin time or activated partial thromboplastin time, are still used despite the lack of evidence supporting its accuracy in evaluating the coagulation status of pregnant women. Thromboelastography and rotational thromboelastometry, which are used to assess the function of platelets, soluble coagulation factors, fibrinogen, and fibrinolysis, can replace standard coagulation tests. Platelet count and function and the effect of anticoagulant treatment should be assessed to determine the risk of hematoma associated with regional anesthesia. Moreover, anesthesiologists should monitor patients for postpartum hemorrhage (PPH), and attention should be paid when performing rapid coagulation tests, transfusions, and prohemostatic pharmacotherapy. Transfusion of a high ratio of plasma and platelets to red blood cells (RBCs) showed high hemostasis success and low bleeding-related mortality rates in patients with severe trauma. However, the effects of high ratios of plasma and platelets and the ratio of plasma to RBCs and platelets to RBCs in the treatment of massive PPH were not established. Intravenous tranexamic acid should be administered immediately after the onset of postpartum bleeding. Pre-emptive treatment with fibrinogen for PPH is not effective in reducing bleeding. If fibrinogen levels of less than 2 g/L are identified, 2–4 g of fibrinogen or 5–10 ml/kg cryoprecipitate should be administered.


Subject(s)
Female , Humans , Pregnancy , Anesthesia, Conduction , Blood Coagulation Factors , Blood Transfusion , Drug Therapy , Erythrocytes , Factor VII , Factor VIII , Factor X , Fibrinogen , Fibrinolysis , Hematoma , Hemorrhage , Hemostasis , Mortality , Partial Thromboplastin Time , Plasma , Platelet Count , Postpartum Hemorrhage , Postpartum Period , Pregnant Women , Protein S , Prothrombin Time , Thrombelastography , Tranexamic Acid
18.
Rev. méd. Chile ; 147(1): 73-82, 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-991375

ABSTRACT

Direct oral anticoagulants (DOACs), including the direct thrombin inhibitor dabigatran and the direct factor Xa inhibitors rivaroxaban, apixaban and edoxaban have at least comparable efficacy as vitamin K antagonists along with a better safety profile, reflected by a lower incidence of intracranial hemorrhage. Specific reversal agents have been developed in recent years. Namely, idarucizumab, a specific antidote for dabigatran, is currently approved in most countries. Andexanet, which reverses factor Xa inhibitors, has been recently approved by the FDA, and ciraparantag, a universal antidote targeted to reverse all DOACs, is still under investigation. In this review we provide an update on the pharmacology of DOACs, the risk of hemorrhagic complications associated with their use, the measurement of their anticoagulant effect and the reversal strategies in case of DOAC-associated bleeding.


Subject(s)
Humans , Blood Coagulation Factors/therapeutic use , Antithrombins/administration & dosage , Antithrombins/adverse effects , Antibodies, Monoclonal, Humanized/therapeutic use , Hemorrhage/chemically induced , Hemorrhage/therapy , Pyrazoles/administration & dosage , Pyrazoles/adverse effects , Pyridines/administration & dosage , Pyridines/adverse effects , Pyridones/administration & dosage , Pyridones/adverse effects , Thiazoles/administration & dosage , Thiazoles/adverse effects , Administration, Oral , Risk Factors , Rivaroxaban/administration & dosage , Rivaroxaban/adverse effects , Dabigatran/administration & dosage , Dabigatran/adverse effects , Antidotes/therapeutic use
19.
Rev. Soc. Cardiol. Estado de Säo Paulo ; 28(4): 421-427, out.-dez. 2018. ilus, tab
Article in English, Portuguese | LILACS | ID: biblio-970567

ABSTRACT

Os fibrinolíticos pertencem a uma classe de medicamentos especializada em promover a lise da fibrina e a consequente dissolução do trombo. Esse efeito baseia-se na transformação do plasminogênio em plasmina, potente enzima proteolítica. A sua aplicação nas diferentes síndromes cardiovasculares agudas alterou o curso natural do infarto agudo do miocárdio, da embolia pulmonar e do acidente vascular cerebral isquêmico agudo. Na prática, temos três gerações de fibrinolíticos disponíveis: estreptoquinase, alteplase e tenecteplase, essa última com alta afinidade à fibrina. As complicações hemorrágicas, embora raras, devem ser identificadas e tratadas de forma precoce. Isso se deve aos distúrbios da coagulação, especialmente, queda do fibrinogênio e outros fatores de coagulação, mais predominantes com a estreptoquinase. A correção baseia-se na administração de crioprecipitado e plasma fresco, ricos, respectivamente, em fibrinogênio e fatores de coagulação. A inexperiência ao administrar tais medicamentos e o receio hemorrágico tem sido fator agravante na sobrevida dessas doenças. Campanhas educacionais poderão amenizar tal cenário, especialmente, em centros mais carentes de recursos tecnológicos


Fibrinolytics belong to a class of drugs specialized in promoting lysis of fibrin and consequent dissolution of the thrombus. This effect is based on the conversion of plasminogen into plasmin, a potent proteolytic enzyme. Its application in different acute cardiovascular syndromes has changed the natural course of acute myocardial infarction, pulmonary embolism, and acute ischemic stroke. In practice, three generations of fibrinolytics are available: streptokinase, alteplase, and tenecteplase (the latter having high affinity to fibrin). Hemorrhagic complications, although rare, must be identified and treated early. This is due to coagulation disorders, especially the decrease in fibrinogen and other coagulation factors, more prevalent with streptokinase. Correction is based on the administration of cryoprecipitate and fresh plasma, which are rich in fibrinogen and coagulation factors, respectively. Inexperience in administering these drugs and hemorrhagic fear are aggravating factors of disease progression. Educational programs can mitigate such scenarios, especially in centers that lack technological resources.Inexperience administering such medicines and hemorrhagic fear has been an aggravating factor in the lives of these diseases. Educational campaigns will be able to mitigate such a scenario especially in the most lacking centers of technological resources


Subject(s)
Humans , Male , Female , Adult , Thrombolytic Therapy/methods , Fibrinolytic Agents/therapeutic use , Hemorrhage/complications , Myocardial Infarction/therapy , Pulmonary Embolism , Streptokinase , Blood Coagulation Factors , Cardiovascular Diseases , Reperfusion , Stroke
20.
Osong Public Health and Research Perspectives ; (6): 133-139, 2018.
Article in English | WPRIM | ID: wpr-715250

ABSTRACT

The Second Meeting of the National Control Laboratories for Vaccines and Biologicals in the Western Pacific, was jointly organized by the National Institute of Food and Drug Safety Evaluation of the Ministry of Food and Drug Safety in the Republic of Korea, and by the World Health Organization Regional Office for the Western Pacific. In the National Lot Release Systems session countries including Canada, China, Japan, Malaysia, Vietnam, and the Republic of Korea, all shared information on their current Lot Release Systems, including current practices and developments in risk-based official lot release of vaccines. In the session on Quality Control of Blood Products, experts from the National Institute for Biological Standards and Control shared quality control and research results for; blood coagulation factor VIII products, and the measurement of procoagulant activity in immunoglobulin products. Representatives from Japan proposed a regional collaborative study to test aggregated immunoglobulin free from complement activity. A cell-based Japanese encephalitis vaccine potency assay was proposed by representatives from Korea and they also called for voluntary participation of other National Control Laboratories in a collaborative study, on the first Korean Gloydius anti-venom standard. Participants agreed in general to continue communicating, and coordinate presentation of the study results.


Subject(s)
Blood Coagulation Factors , Canada , China , Complement System Proteins , Encephalitis, Japanese , Factor VIII , Immunoglobulins , Japan , Korea , Malaysia , Quality Control , Republic of Korea , Vaccine Potency , Vaccines , Vietnam , World Health Organization
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