Search results for " FACTOR VII DEFICIENCY"

showing 10 items of 15 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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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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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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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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Oral Communication

2012

Inherited factor VII (FVII) deficiency is the most common of the rare autosomal recessive bleeding disorders (RBD), with an estimated prevalence of 1 per 300,000 in European countries.1,2 It is likely that the prevalence is higher in those countries where consanguineous marriages are frequent.We here report STER study results on 112 evaluable enrolled cases

Inherited Factor VII deficiency prophylaxisspontaneous bleeding episodesSettore MED/15 - Malattie Del Sangue
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Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER

2011

MAJOR SURGERY FACTOR VII DEFICIENCY
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Women with congenital factor VII deficiency: clinical phenotype and treatment options from two international studies

2016

Introduction A paucity of data exists on the incidence, diagnosis and treatment of bleeding in women with inherited factor VII (FVII) deficiency. Aim Here we report results of a comprehensive analysis from two international registries of patients with inherited FVII deficiency, depicting the clinical picture of this disorder in women and describing any gender-related differences. Methods A comprehensive analysis of two fully compatible, international registries of patients with inherited FVII deficiency (International Registry of Factor VII deficiency, IRF7; Seven Treatment Evaluation Registry, STER) was performed. Results In our cohort (N = 449; 215 male, 234 female), the higher prevalence…

MalePediatricsFactor VII Deficiency030204 cardiovascular system & hematologyCohort Studieschemistry.chemical_compound0302 clinical medicineAntifibrinolytic agentgynaecological bleedingRegistriesChildGenetics (clinical)Aged 80 and overFactor VIIIncidence (epidemiology)Hazard ratio[SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/HematologyHematologyGeneral MedicineFactor VIIMiddle AgedAntifibrinolytic AgentsRecombinant Proteins3. Good healthPhenotypeTreatment OutcomeChild PreschoolCohortFemalewomengynaecological bleeding; inherited factor VII deficiency; recombinant activated factor VII; womenCohort studyAdultmedicine.medical_specialtyAdolescentMucocutaneous zoneHemorrhageFactor VIIaYoung Adult03 medical and health sciencesmedicineHumansMenorrhagiaAgedProportional Hazards ModelsCoagulantsbusiness.industryProportional hazards modelInfantrecombinant activated factor VIISurgeryROC Curvechemistryinherited factor VII deficiencybusiness030215 immunology
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Replacement therapy for bleeding episodes in factor VII deficiency: A prospective evaluation

2013

Patients with inherited factor VII (FVII) deficiency display different clinical phenotypes requiring ad hoc management. This study evaluated treatments for spontaneous and traumatic bleeding using data from the Seven Treatment Evaluation Registry (STER). One-hundred one bleeds were analysed in 75 patients (41 females; FVII coagulant activity <1-20%). Bleeds were grouped as haemarthroses (n=30), muscle/subcutaneous haematomas (n=16), epistaxis (n=12), gum bleeding (n=13), menorrhagia (n=16), central nervous system (CNS; n=9), gastrointestinal (GI; n=2) and other (n=3). Of 93 evaluable episodes, 76 were treated with recombinant, activated FVII (rFVIIa), eight with fresh frozen plasma (FFP), s…

MaleRegistrieTime FactorsFactor VII Deficiency030204 cardiovascular system & hematologyReplacement therapyProspective evaluationchemistry.chemical_compound0302 clinical medicineMedicineProspective StudiesRegistriesYoung adultProspective cohort studyFactor VII deficiencyChildHematologyFactor VIIHematologyMiddle AgedRecombinant ProteinBlood Coagulation FactorsRecombinant ProteinsTreatment OutcomeCoagulantChild PreschoolFemaleBlood Coagulation FactorHumanAdultmedicine.medical_specialtyAdolescentTime FactorHemorrhageBlood Component TransfusionFactor VIIaBleeds; Factor VII deficiency; Replacement therapy; Adolescent; Adult; Aged; Blood Coagulation Factors; Child; Child Preschool; Coagulants; Drug Administration Schedule; Factor VII Deficiency; Factor VIIa; Female; Hemorrhage; Humans; Infant; Infant Newborn; Male; Middle Aged; Prospective Studies; Recombinant Proteins; Registries; Time Factors; Treatment Outcome; Young Adult; Blood Component Transfusion; HematologyDrug Administration Schedule03 medical and health sciencesYoung AdultInternal medicineHumansAgedBleeding episodesbusiness.industryCoagulantsInfant NewbornInfantSurgeryProspective StudieTreatment evaluationchemistryBleedbusiness030215 immunology
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