Search results for "FACTOR VII DEFICIENCY"

showing 10 items of 26 documents

Thrombosis in inherited factor VII deficiency

2003

Thrombosis in congenital factor (F) VII deficiency was investigated through extensive phenotypic and molecular-genetic studies. Patients with a history of thrombosis among 514 entries in the FVII Deficiency Study Group database were evaluated. Thrombotic events were arterial in one case, disseminated intravascular coagulation in another and venous in seven. Gene mutations were characterized in eight patients: three were homozygous, three compound heterozygous and two heterozygous. FXa and IIa generation assays were consistent with the genetic lesions. One patient was heterozygous for the FV Leiden and one for the FIIG20210A mutation. In seven patients, surgical interventions and/or replacem…

AdultMaleHeterozygotemedicine.medical_specialtyPathologyTime FactorsAdolescentFactor VII DeficiencyGene mutationCompound heterozygosityThrombophiliaGastroenterologyInternal medicinemedicineHumansThrombophiliaAgedVenous ThrombosisDisseminated intravascular coagulationbiologybusiness.industryHomozygoteFactor VFactor VThrombosisHematologyCongenital FVII deficiency; Replacement therapy; Surgery; Thrombophilia; Thrombosis;Disseminated Intravascular CoagulationMiddle Agedmedicine.diseaseThrombosisZygosityVenous thrombosisPhenotypeDatabases as TopicFactor XaMutationbiology.proteinFemaleProthrombinbusinessJournal of Thrombosis and Haemostasis
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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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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 <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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Coronary artery stenosis treatment in aging patients with inherited Factor VII deficiency: Where do we stand?

2017

Aging with rare bleeding disorders such as factor VII (FVII) deficiency poses several challenges to treatment because of the occurrence of cerebral and cardiovascular age-related comorbidities and high bleeding risks. We report a case of long-term treatment with antiplatelet agents and contemporary prophylaxis of bleeding in a woman affected by severe FVII deficiency diagnosed with symptomatic coronary artery stenosis. Information on the management of antithrombotic treatment in rare bleeding disorders is lacking and mainly limited to anecdotal reports or side effects secondary to replacement therapy. We also briefly reviewed available data on the treatment of arterial thrombosis in FVII de…

AdultMalemedicine.medical_specialtyFactor VII DeficiencyCardiovascular risk factorsCoronary stenosis030204 cardiovascular system & hematology03 medical and health scienceschemistry.chemical_compoundAntithrombotic treatment0302 clinical medicineInternal medicinemedicineHumanscardiovascular diseasesFactor VII deficiencyChildAgedFactor VIIbusiness.industryCoronary StenosisHematologyMiddle Agedmedicine.diseaseThrombosischemistryCardiologyFemalebusiness030215 immunologyTransfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
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Replacement Therapy for Minor Surgery and Invasive Procedures in Factor VII Deficiency: The STER Experience

2011

Adverse events Inherited Factor VII deficiency
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Comunicazione orale

2011

Bleeding PhenotypeFactor VII deficiency
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Coagulation and clinical features associated with the Arg304Gln mutation (Factor VII Padua) in the IRF7 Study Group

2010

FACTOR VII DEFICIENCY
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Prophylaxis In Congenital Factor VII Deficiency, Indications, Efficacy and Safety: Results of the STER

2010

Abstract Abstract 665 Introduction Prophylaxis is considered a difficult endeavour in FVII deficiency, especially because of the very short FVII zymogen and FVIIa half-lives, mainly in childhood. The Seven Treatment Evaluation Registry (STER, www.targetseven.org) is a prospective observational, multi-centre, web-based registry concerned with the evaluation of treatments for spontaneous bleeding episodes, surgery and prophylaxis in patients with FVII deficiency. As regards prophylaxis, STER provides the frame for a structured and detailed data capture aiming at: a) identifying patients in whom prophylaxis is advisable, b) defining clinical settings in which prophylaxis is necessary and c) de…

Gastrointestinal bleedingPediatricsmedicine.medical_specialtybusiness.industryImmunologyCell BiologyHematologyHemarthrosismedicine.diseaseBiochemistryRegimenConcomitantSeverity of illnessmedicineProphylaxis in Factor VII deficiency inherited Factor VII deficiencyObservational studyDosingFresh frozen plasmabusinessBlood
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Pharmacokinetic properties of recombinant FVIIa in inherited FVII deficiency account for a large volume of distribution at steady state and a prolong…

2014

Pharmacokinetic properties of recombinant FVIIa in inherited FVII deficiency account for a large volume of distribution at steady state and a prolonged pharmacodynamic effect -

HeredityPharmacokinetic inherited Factor VII deficiencyFactor VII DeficiencySocio-culturaleFactor VIIaPharmacologySeverity of Illness IndexPharmacokineticsPredictive Value of Testshemic and lymphatic diseasesHumansMedicineGenetic Predisposition to DiseaseFVII deficiencyRegistriescardiovascular diseasesBlood CoagulationVolume of distributionbiologyCoagulantsbusiness.industryVascular biologyrFVIIaHematologyFactor VIIRecombinant ProteinsPhenotypeTreatment OutcomerFVIIa; FVII deficiency; pharmacokineticsRecombinant factor VIIaPharmacodynamicsbiology.proteinBlood Coagulation TestsSteady state (chemistry)Drug Monitoringbusinesspharmacokinetics
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Factor VII deficiency: evidence that in minor surgery one-day replacement therapy is sufficient

2011

Inherited FACTOR VII DEFICIENCY MINOR SURGERY INVASIVE PROCEDURESSettore MED/15 - Malattie Del Sangue
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