0000000000201701

AUTHOR

J.-f. Schved

showing 2 related works from this author

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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