scholarly journals Successful use of recombinant activated factor VII administered via automated bolus pump following emergency laparoscopic appendectomy in a patient with mild congenital FVII deficiency: Case report

2020 ◽  
Author(s):  
Robert Klaassen ◽  
Heather Perkins
Keyword(s):  
Laparoscopic Appendectomy ◽  
Perioperative Management ◽  
Healthcare Providers ◽  
Factor Vii ◽  
Severe Bleeding ◽  
Bleeding Disorders ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Hospital Resources ◽  
Fvii Deficiency

Surgery in patients with factor VII (FVII) deficiency may be complicated by severe bleeding, requiring regular bolus doses of replacement therapy. Eptacog alfa (activated) is a recombinant activated FVII (rFVIIa) used for the treatment of bleeds and perioperative management in patients with approved bleeding disorders, including FVII deficiency. We report that using the B-Braun Perfusor® Space syringe pump to automatically deliver regular bolus rFVIIa doses provided effective hemostasis and no safety concerns in a patient with mild FVII deficiency undergoing emergency laparoscopic appendectomy. Additional benefits included saving nursing/hospital resources, reducing treatment burden and reassurance for the patient/family, and healthcare providers.

scholarly journals Congenital Deficiency in Factor VII Revealed by Menorrhagia: Case Report

2020 ◽  
pp. 1-5
Author(s):  
Nadia Mebrouk ◽  
Abdelilah Radi ◽  
Mohamed Selouti ◽  
Amal Hassani ◽  
Abdelhakim Ourrai ◽  
...  
Keyword(s):  
Treatment Options ◽  
Specific Treatment ◽  
Fresh Frozen Plasma ◽  
Factor Vii ◽  
Activity Measurement ◽  
Recombinant Activated Factor Vii ◽  
Congenital Deficiency ◽  
Activated Factor Vii ◽  
Fresh Frozen ◽  
Fvii Deficiency

Factor VII (FVII) deficiency is the most common among rare inherited autosomal recessive bleeding disorders. It is a multifaceted disease because of the lack of a direct correlation between plasma levels of coagulation FVII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even in homozygous subjects—to severe, life-threatening bleedings (e.g., central nervous system and gastrointestinal bleeding). Menorrhagia is a frequent type of bleeding in FVII deficiency, with a prevalence rate of two in three women aged 10 to 50 years and with a peak prevalence in teenagers. When menorrhagia is observed and once the gynecological causes are excluded, it is important to carry out a hemostasis assessment because, if an anomaly is found, specific treatment can be administered and preventive measures taken. Basic diagnostic work-up includes routine assays, prothrombin level, activated partial thromboplastin time and platelet count, followed by FVII coagulant activity measurement for isolated decreased prothrombin level. To confirm the diagnosis, FVII assay should be repeated at least once. Several treatment options are currently available for FVII deficiency: Recombinant activated Factor VII (rFVIIa), plasma-derived Factor VII, fresh frozen plasma and prothrombin complex concentrates. rFVIIa is the most used replacement therapy. Other medical therapies of menorrhagia includes hemostatic agents and hormonal treatments (combined oral contraceptives, levonorgestrel intrauterine devices), in combination or not with rFVIIa. We report the case of a fourteen-and-a-half-year-old girl who presented menorrhagia of great abundance at the age of thirteen, the exploration of which revealed a congenital deficit in FVII.

Continuous infusion of recombinant factor VIIa for surgery in patients with deficiency of factor VII

2005 ◽  
Vol 94 (12) ◽  
pp. 1177-1180 ◽  
Author(s):  
Geir E. Tjønnfjord ◽  
Richard Wallensten ◽  
Uri Martinowitz ◽  
Gili Kenet ◽  
Sam Schulman
Keyword(s):  
Continuous Infusion ◽  
Recombinant Factor Viia ◽  
Factor Viia ◽  
Single Case ◽  
Thromboembolic Complication ◽  
Factor Vii ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Different Types ◽  
Fvii Deficiency

SummaryThe administration of recombinant activated factor VII (rFVIIa) by continuous infusion has provided a safe and convenient alternative to bolus injections in haemophiliacs with inhibitors, but it has only been reported in a single case with congenital factorVII (FVII) deficiency. The results of 12 consecutive surgical procedures in 7 patients with congenital FVII deficiency are reported here. rFVIIa was always given in continuous infusion, aiming at plasma FVII activity of 0.5 IU/mL. Treatment was given for 2 to 7 days with a mean total dose of 7.8 mg rFVIIa. Blood loss was as expected from the different types of procedures and the only thromboembolic complication was a superficial thrombophlebitis at the infusion site. This mode of substitution was therefore safe, effective and well tolerated.

Bleeding after cardiac surgery

2006 ◽  
Vol 26 (S 02) ◽  
pp. S76-S86
Author(s):  
C. Spies ◽  
H. Grubitzsch ◽  
H. Schönfeld ◽  
M. Sander ◽  
Th. Volk ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Blood Loss ◽  
Factor Vii ◽  
Coagulation System ◽  
Severe Bleeding ◽  
International Studies ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Increased Risk ◽  
Heart Lung Machine

SummaryCardiac surgery carries the risk of significant blood loss requiring the transfusion of blood products. In addition to such blood loss, international studies have shown that severe bleeding necessitating re-operation occurs in 3–5% of patients. Morbidity and mortality are significantly increased, so effective and safe haemostatic measures will decisively improve outcome of patients.Recombinant activated factor VII (rFVIIa) has been approved for the treatment of patients with inhibitor haemophilia, as well as with Glanzmann’s thrombasthenia and factor VII deficiency. Many publications have appeared in the last few years which report the successful and reliable use of rFVIIa for the treatment of refractory bleeding after cardiac surgery. This review presents the pathophysiological changes in the coagulation system which occur when a heart-lung machine is used and which have been blamed for an increased risk of bleeding in patients who have undergone cardiac surgery. Published experience with rFVIIa in paediatric and adult cardiac surgery is presented and discussed critically with regard to the efficacy and safety of its use.

scholarly journals Evidence Supporting the Use of Recombinant Activated Factor VII in Congenital Bleeding Disorders

2011 ◽  
Vol 07 (01) ◽  
pp. 71
Author(s):  
Pär I Johansson ◽  
Sisse R Ostrowski ◽  
◽  
Keyword(s):  
Randomised Controlled Trials ◽  
Thrombin Generation ◽  
English Language ◽  
Factor Vii ◽  
Controlled Trials ◽  
Bleeding Disorders ◽  
Recombinant Activated Factor Vii ◽  
Activated Factor Vii ◽  
Number Of Patients ◽  
Randomised Controlled

Recombinant activated factor VII ([rFVIIa] NovoSeven) was introduced in 1996 for the treatment of haemophilia patients with inhibitors (HPIs) to factor VIII or IX. This article reviews the evidence for the use of rFVIIa in congenital bleeding disorders. English-language databases were searched in September 2009 for reports of randomised controlled trials (RCTs) evaluating the effect of rFVIIa on haemostasis in congenital bleeding disorders. Eight RCTs comprising 256 HPIs were identified. The evidence for the use of rFVIIa in HPIs in terms of dose, clinical setting, modes of administration, efficacy and adverse events was weak, given the limited number of patients included and the heterogeneity of the RCTs. Overall, the haemostatic efficacy of rFVIIa varied from 25 to 100% in the studies reviewed; <1% of the patients receiving rFVIIa developed a thromboembolic adverse event. The authors suggest that the addition FVIIa therapy to HPIs should be based on the patient’s ability to generate thrombin and form a clot, rather than being based on weight alone. Assays reflecting thrombin generation, such as whole-blood thrombelastography, have the potential to significantly improve the treatment of these patients.

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