6533b827fe1ef96bd12870e0
RESEARCH PRODUCT
Role of clinical and laboratory parameters for treatment choice in patients with inherited FVII deficiency undergoing surgical procedures: evidence from the STER registry
Matteo N. D. Di MinnoMariasanta NapolitanoAlberto DolceGuglielmo MarianiGuenter AuerswaldAngelica BatorovaFrancesco BernardiGiovanni Di MinnoMuriel Giansilly-balizotJorgen IngerslevUri MartinowitzMassimo MorfiniMirko PinottiAmy ShapiroSergio SiragusaJean Francois SchvedBenny Sørensensubject
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 bleedingdescription
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 days vs. 1 day, P = 0·005) as compared to those with a history of minor bleeds or to asymptomatic patients. No difference in RT was found among major and minor surgical procedures. Overall, multivariate analysis showed that history of major bleeding was the only independent predictor of number of RT doses (β = 0·352, P = 0·001) and RT duration (β = 0·405, P = 0·018). Overall, a ≈20 μg/kg perioperative RT was efficacious in 95·5% of cases. The infusion should be repeated ≈8 times in high-risk subsets (i.e. patients with a history of major bleeding).
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2018-01-01 |