Search results for "Factor VII"

showing 10 items of 101 documents

Invasive procedures and minor surgery in factor VII deficiency

2012

AdultMaleMINOR SURGERY INVASIVE PROCEDURES FACTOR VII DEFICIENCYAdolescentFactor VII DeficiencyBlood Loss SurgicalInfantHemorrhageHematologyFactor VIIHemostasiMiddle AgedSettore MED/15 - Malattie Del SangueMinor Surgical ProcedureYoung AdultCoagulantChild PreschoolSurgical Procedures OperativeFemaleChildGenetics (clinical)AgedHuman
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Efficacy and safety during formulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in pati…

2012

Summary Von Willebrand disease (VWD) is an inherited bleeding disorder caused by the quantitative or qualitative deficiency of von Willebrand factor (VWF). Replacement therapy with plasma-derived VWF/factor VIII (FVIII) concentrates is required in patients unresponsive to desmopressin. To assess the efficacy, safety and ease of use of a new, volume-reduced (VR) formulation of VWF/FVIII concentrate Haemate® P in patients requiring treatment for bleeding or prophylaxis for recurrent bleeding or for invasive procedures. Pharmacoeconomic variables were also recorded. Data were analysed using descriptive statistics. This was a multicentre, prospective, observational study. Consecutively enrolled…

AdultMalePediatricsmedicine.medical_specialtyAdolescentBlood Loss SurgicalSevere diseaseHemorrhageSettore MED/15 - Malattie Del SangueYoung AdultVon Willebrand factorCost of Illnesshemic and lymphatic diseaseshemophiliavon Willebrand FactorVon Willebrand diseasemedicineHumansIn patientProspective StudiesDesmopressinAdverse effectChildGenetics (clinical)AgedFactor VIIIbiologybusiness.industryDrug SubstitutionAnticoagulantsHematologyGeneral MedicineMiddle Agedmedicine.diseaseResponse to treatmentHospitalizationvon Willebrand DiseasesItalyChild Preschoolbiology.proteinPasteurizationObservational studyFemalebusinessmedicine.drug
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Secondary prophylaxis vs. on-demand treatment to improve quality of life in severe adult haemophilia A patients: a prospective study in a single cent…

2013

Background Retrospective publications show a decrease in the bleeding frequency and an improvement in the quality of life (QoL) in severe adult haemophilia A (SAHA) after switching from the on-demand treatment (DT) to secondary prophylaxis (SP). But there are no prospective studies which demonstrate, using a haemophilia-specific questionnaire, an improvement in the QoL after such treatment change. The main objective of this study is to prospectively compare the QoL and the musculoskeletal assessment after switching from DT to SP in SAHA using the A36 Hemofilia-QoL®. As secondary objective, we compare the haemarthrosis frequency and factor VIII consumption in DT and SP during a similar perio…

AdultMalePediatricsmedicine.medical_specialtyHaemophilia Ahaemophilia AHemorrhageHemophilia AQuality of lifeSurveys and QuestionnairesHemarthrosismedicineHumansProspective StudiesProspective cohort studyRetrospective StudiesmusculoskeletalFactor VIIIbusiness.industrySecondary prophylaxisHematologyGeneral MedicineMiddle Agedmedicine.diseasehumanitiesbleeding incidenceSingle centreOn demand treatmentquality of lifesecondary prophylaxisQuality of LifeFemalebusinessVox sanguinis
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Predictive factors of immune tolerance treatment response in severe haemophilia A patients with inhibitors: A real‐world report from a single centre,…

2019

AdultMalePediatricsmedicine.medical_specialtyTreatment responseOnline LettersMEDLINEHemophilia AImmune toleranceHumansMedicineProspective StudiesTreatment FailureChildLetters to the EditorProspective cohort studyLetter to the EditorGenetics (clinical)Retrospective StudiesFactor VIIIbusiness.industryRetrospective cohort studyHematologyGeneral MedicineSingle centreLong term learningFemaleSevere haemophilia AbusinessHaemophilia
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Treatment of severe von Willebrand disease with a high‐purity von Willebrand factor concentrate (Wilfactin®): a prospective study of 50 patients

2007

Background and objectives: A plasma-derived von Willebrand factor (VWF) concentrate with low factor VIII (FVIII) content was specifically developed to treat von Willebrand disease (VWD). Efficacy and safety were investigated by merging the results of two comparable protocols conducted prospectively in 5 European and 12 French centers. Methods and results: Fifty patients with clinically severe VWD (72% had VWF ristocetin cofactor activity less than 10 IU dL(-1) and 46% had FVIII < 20 IU dL(-1)) were treated with the concentrate as the only therapy, except for clinical situations requiring a priming dose of FVIII to rapidly correct an intrinsic coagulation defect. A total of 139 spontaneous b…

AdultMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyAdolescentHemorrhagePostoperative HemorrhageGastroenterologyVon Willebrand factorhemic and lymphatic diseasesInternal medicinevon Willebrand FactormedicineVon Willebrand diseaseHumansProspective StudiesChildProspective cohort studyAgedAged 80 and overHemostasisBleeding episodesFactor VIIIbiologybusiness.industryHematologyMiddle Agedmedicine.diseaseSurgeryClinical trialvon Willebrand DiseasesCoagulationChild PreschoolHemostasisConcomitantbiology.proteinFemaleSafetybusinessJournal of Thrombosis and Haemostasis
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Distribution, genetic and cardiovascular determinants of FVIII:c - Data from the population-based Gutenberg Health Study

2015

Background: Elevated levels of FVIII:c are associated with risk for both venous and arterial thromboembolism. However, no population-based study on the sex-specific distribution and reference ranges of plasma FVIII: c and its cardiovascular determinants is available. Methods: FVIII:c was analyzed in a randomly selected sample of 2533 males and 2440 females from the Gutenberg Health Study in Germany. Multivariable regression analyses for FVIII:c were performed under adjustment for genetic determinants, cardiovascular risk factors and cardiovascular disease. Results and conclusions: Females (126.6% (95% CI: 125.2/128)) showed higher FVIII:c levels than males (121.2% (119.8/122.7)). FVIII:c le…

AdultMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyLinkage disequilibriumGenotypeanimal diseasesPopulationFVIII:c reference valuesSingle-nucleotide polymorphismDiseaseAge DistributionVon Willebrand factorGermanyThromboembolismhemic and lymphatic diseasesInternal medicineVenous thrombosisHumansMedicineGenetic Predisposition to DiseaseProspective StudiesSex DistributioneducationAgededucation.field_of_studyEpidemiological studiesFactor VIIIPolymorphism Geneticbiologybusiness.industryIncidenceC-reactive proteinArterial thrombosisDNAMiddle AgedNomogrammedicine.diseaseVenous thrombosisPopulation SurveillanceImmunologybiology.proteinFemaleCardiology and Cardiovascular MedicinebusinessFollow-Up Studies
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Prothrombotic State Induced by Middle-Distance Endurance Exercise in Middle-Aged Athletes

2018

AbstractSince the impact of possible prothrombotic factors on blood coagulation resulting from exercise remains elusive, this study investigated the acute effects of middle-distance endurance running on blood coagulation parameters in middle-aged athletes. The study population consisted of 33 male endurance runners who were engaged in a 21.1 km run under competitive conditions. Blood samples were collected before the run, immediately after the run, and 3 hours after run completion. Samples were assessed for activated partial thromboplastin time (APTT), prothrombin time (PT), fibrinogen, D-dimer, factor VIII (FVIII), von Willebrand factor antigen (VWF:Ag), endogenous thrombin potential (area…

AdultMalecongenital hereditary and neonatal diseases and abnormalitiesmedicine.medical_specialtyphysical activity030204 cardiovascular system & hematologyFibrinogenRunningblood coagulation03 medical and health sciences0302 clinical medicineVon Willebrand factorEndurance traininghemic and lymphatic diseasesInternal medicineABO blood group systemvon Willebrand FactormedicineHumansExerciseblood coagulation; hemostasis; physical activity; thrombin generation; Adult; Blood Coagulation; Exercise; Factor VIII; Fibrinogen; Humans; Male; Middle Aged; Partial Thromboplastin Time; Physical Endurance; Running; Thrombin; Thrombosis; von Willebrand Factor; AthletesProthrombin timeFactor VIIImedicine.diagnostic_testbiologyChemistryThrombinFibrinogenThrombosisHematologyMiddle Agedprothrombotic factors blood coagulation sportprothrombotic factorsEndocrinologyCoagulationAthletesthrombin generationHemostasishemostasisPhysical Endurancebiology.proteinPartial Thromboplastin TimeCardiology and Cardiovascular Medicinesportcirculatory and respiratory physiology030215 immunologyPartial thromboplastin timemedicine.drug
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Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence f…

2018

Perioperative bleeding is a major concern in patients with factor VII (FVII) deficiency. Evaluating data of 95 FVII-deficient patients undergoing 110 surgical procedures (61 major, 49 minor), we assessed the impact of type of surgery, bleeding phenotype and FVII coagulant activity (FVII:C) levels on perioperative replacement therapy (RT). Compared to those with higher FVII:C levels, patients with &lt;3% FVII:C received a higher number of RT doses (8 vs. 2, P = 0·003) for a longer RT duration (3 days vs. 1 day, P = 0·001), with no difference in RT dose. Similarly, patients with a history of major bleeds received a higher number of RT doses (8·5 vs. 2-3, P = 0·013) for a longer RT duration (2…

AdultMalemedicine.medical_specialtyAdolescentClinical Decision-MakingSocio-culturaleHemorrhage030204 cardiovascular system & hematologyIndependent predictorGastroenterologyAsymptomaticsurgery03 medical and health scienceschemistry.chemical_compoundYoung Adult0302 clinical medicineInternal medicinemedicineHumansIn patientRegistriesFactor VII deficiencybleeding disordersbleeding disorderSurgical ProceduresFactor VIIbusiness.industryDisease ManagementPerioperativeHematologySurgical proceduresFactor VIIMiddle AgedCombined Modality TherapyOperativefactor VII deficiencyTreatment Outcomechemistry030220 oncology & carcinogenesisSurgical Procedures Operativebleeding disorders; factor VII deficiency; surgery; Adolescent; Adult; Clinical Decision-Making; Combined Modality Therapy; Disease Management; Factor VII; Factor VII Deficiency; Female; Hemorrhage; Humans; Male; Middle Aged; Registries; Surgical Procedures Operative; Symptom Assessment; Treatment Outcome; Young Adult; HematologyFemalemedicine.symptomSymptom AssessmentbusinessMajor bleeding
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Bayesian pharmacokinetic-guided prophylaxis with recombinant factor VIII in severe or moderate haemophilia A

2018

Introduction: Personalised pharmacokinetics (PK) using Bayesian analysis with limited sampling is assumed to help to optimise prophylaxis in haemophilia A (HA) patients. Materials and methods: Our prospective, observational study analysed the influence of PK parameters on clinical variables (bleeding rates, joint status, adherence, and consumption) using myPKFiT (R) in a cohort of twenty-one severe and moderate HA patients on prophylaxis with recombinant FVIII (Advate (R)) in two periods of one year, the first before PK-based tailoring and the second after PK-guided prophylaxis. Intra-individual and inter-individual coefficients of variation (CV) of half-life (t(1/2)) were calculated. Resul…

AdultMalemedicine.medical_specialtyAdolescentHaemophilia AModerate haemophilia A030204 cardiovascular system & hematologyHemophilia ARecombinant factor viiiRecombinant factor VIIIYoung Adult03 medical and health sciences0302 clinical medicinePharmacokineticsInternal medicineHaemophilia AArthropathymedicineLimited samplingHumansPharmacokineticsProspective StudiesAgedFactor VIIIbusiness.industryBayes TheoremHematologyBayesian estimationMiddle Agedmedicine.diseasePK-guided prophylaxis030220 oncology & carcinogenesisCohortFemaleObservational studybusinessmyPKFiTThrombosis Research
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Tailoring haemophilia A prophylaxis with BAY 81-8973: A case series

2020

BAY 81-8973 is an unmodified, full-length third generation recombinant factor VIII (rFVIII) which offers a more favorable pharmacokinetic (PK) profile, compared to its predecessor sucrose-formulated rFVIII (rFVIII-FS). We here report on a retrospective case series of nine patients affected by hemophilia A (HA), with variable disease severity, bleeding phenotype and comorbidities, to underline our clinical practice on prophylaxis with a recently introduced standard hall-life recombinant Factor VIII. The current case series highlights how the current clinical management of hemophilia is able to personalize treatment in several specific conditions like concomitant illnesses with thrombotic ris…

AdultMalemedicine.medical_specialtyAdolescentHemophilia A HemarthrosisHemophilic arthropathyrFVIIIPharmacokineticProphylaxisHaemophilia A030204 cardiovascular system & hematologyHemophilia ARecombinant factor viii03 medical and health sciencesYoung Adult0302 clinical medicinehemic and lymphatic diseasesInternal medicineMedicineHumansChildVariable disease severityAgedRetrospective StudiesThrombotic riskFactor VIIIbusiness.industryHematologyHemarthrosisMiddle Agedmedicine.diseaseThird generationClinical PracticeTreatment OutcomeConcomitantbusiness030215 immunology
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