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Results for “Factor VII Deficiency”

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PubMed2005

A multicenter assessment of recombinant factor VIIa off-label usage: clinical experiences and associated outcomes.

BACKGROUND: Off-label use of recombinant factor VIIa (rFVIIa) occurs despite minimal data. The purpose of this study was to describe prescribing practices and clinical outcomes for off-label rFVIIa use. STUDY DESIGN AND METHODS: A retrospective, multicenter chart audit of 315 nonhemophiliac patients was performed. RESULTS: Off-label indications included prevention of bleeding primarily related to procedural manipulation in 37.8 percent of patients and treatment of bleeding in 62.2 percent of patients. Baseline coagulopathy was present in 79.7 percent of patients. Prescriber specialty varied. The median doses for prevention and treatment were 75.6 and 89.4 microg per kg, respectively (p = 0.0006). The international normalized ratio (INR) decreased (p < 0.0001) from baseline in both groups. Transfusions of blood products were provided to 85.1 percent of patients before rFVIIa administration with considerable practice variation. Few patients received other procoagulant agents. In the prevention group, 14.3 percent of patients bled within 6 hours of their procedure. No variable was independently associated with bleeding prevention. In the treatment group, 52.6 percent of patients stopped bleeding within 6 hours of rFVIIa administration. The only independent variable associated with bleeding cessation was an arterial pH value of less than 7.20, which was a negative predictor (odds ratio, 0.207; 95% confidence interval, 0.084-0.507). Possible rFVIIa-associated adverse events occurred in 9.8 percent of patients and primarily involved undesirable clotting. No variable was independently associated with adverse events. CONCLUSIONS: Off-label use of rFVIIa includes prevention and treatment of bleeding. rFVIIa is associated with INR reduction. Bleeding is rare with prophylactic rFVIIa but the cessation of bleeding is less than reported in the literature and may be related to pH. Possible adverse events are related to undesirable clotting.

Adult↗
PubMed2006

Segmental liver transplantation from non-heart beating donors--an early experience with implications for the future.

We report our experience of pediatric liver transplantation with partial grafts from non-heart beating donors (NHBD). Controlled donors less than 40 years of age with a warm ischemia time (WI) of less than 30 min were considered for pediatric recipients. Death was declared 5 min after asystole. A super-rapid recovery technique with aortic and portal perfusion was utilized. Mean donor age was 29 years and WI 14.6 min (range 11-18). Seven children, mean age 4.9 years (0.7-11), median weight 20 kg (8.4-53) received NHBD segmental liver grafts. Diagnoses included seronegative hepatitis, neonatal sclerosing cholangitis, familial intrahepatic cholestasis, hepatoblastoma, primary hyperoxaluria and factor VII deficiency (n=2). The grafts included four reduced and one split left lateral segments, one left lobe and one right auxiliary graft. Mean cold ischemia was 7.3 h (6.2-8.8). Complications included one pleural effusion and one biliary collection drained percutaneously. At 20 months (10-36) follow-up all children are alive and well with functioning grafts. Donation after cardiac death is a significant source of liver grafts for adults and children with careful donor selection and short cold ischemic times.

Adolescent↗
PubMed1960

Polycythaemia vera and Christmas disease with slightly prolonged prothrombin time.

A family is reported in which Christmas disease in one member and polycythaemia in another were associated with prolongations of the one-stage prothrombin time of the factor-VII deficiency type. Changes in the prothrombin time were present in the siblings of the case of Christmas disease and in a number of unrelated cases of polycythaemia vera. The use of "mixing procedures" did not reveal any significant mutual correction of the changes in prothrombin time.

Hemophilia A↗
PubMed1983

Regulation of the initiation of coagulation by factor VII.

Factor VII, a single-chain zymogen, has sufficient proteolytic activity to initiate blood coagulation. The reason that coagulation does not occur continuously is that the zymogen like its 2-chain derivative enzyme, factor VIIa, absolutely requires tissue factor. The latter, a lipid-dependent glycoprotein, is not normally present in the blood. Upon tissue injury, however, coagulation is initiated by the activation of factors IX and X. The relationship of these events to the possibility that hemophilia A and B are tissue factor-dependent diseases is discussed.

Blood Coagulation↗
PubMed1983

How to measure factor VII and factor VII activation.

This communication describes the different techniques that can be used to evaluate the activity state of factor VII in plasma samples. At the present time direct methods for quantitation of activated factor VII (factor alpha-VIIa) are not available. Combined methods are therefore used to measure the degree of factor VII activation. These methods can be summarized in the following way: a regular factor VII clotting assay measuring factor VII coagulant activity (factor VIIc) is carried out simultaneously with: (1) a factor VII antigen assay (factor VIIag): (2) a coupled amidolytic factor VII assay (factor VIIam), or (3) a factor VII clotting assay utilizing bovine tissue thromboplastin (VIIbt). The activity state of factor VII can then be calculated from either of the following ratios: f.VIIc/f.VIIag; f.VIIc/f.VIIam, or f.VIIbt/f.VIIc. A study of the potency of a 30-fold activated factor VII to activate factor X in the presence of phospholipids is also included. This experiment demonstrates that even a low factor VII concentration (0.01 U/ml in the coupled amidolytic assay and 0.30 U/ml in the factor VII clotting assay) causes a significant activation of factor X when incubated together in the presence of lipids and calcium ions. Activated factor VII may therefore possess potential thrombotic properties even in the absence of exposed tissue thromboplastin in the blood circulation.

Animals↗
PubMed2004

The use of rFVIIa in non-haemophilia bleeding conditions in paediatrics. A systematic review.

From its original envisioned use in patients with hemophilia and inhibitors, recombinant factor VIIa has been increasingly used in a variety of non-hemophilia bleeding/hemorrhagic situations with great efficacy. Most of the reported work has been in adult patients. This paper sets out to review its use in the pediatric non-hemophilia patients and the varied conditions it has been tried and used. Most of the published literature has shown that this agent is efficacious, safe and can be used as an adjunctive measure in the achievement of hemostasis. However, most of the published work is mainly anecdotal, case reports or small series. Randomized trials in children are eagerly awaited.

Blood Coagulation Disorders↗
PubMed1997

The Arg353Gln polymorphism reduces the level of coagulation factor VII. In vivo and in vitro studies.

Factor VII levels are regulated by environmental and genetic factors. Two polymorphisms, a G-to-A transversion at nucleotide 10,976 resulting in Arg353Gln and a decanucleotide insert at position -323 in the 5'-flanking region of the factor VII gene, have been associated with a 20% to 25% reduction in plasma factor VII levels. However Arg353Gln almost always segregates on alleles containing the insert in UK and Italian populations, thereby making it impossible to independently evaluate the impact of Arg353Gln on factor VII levels in these ethnic groups. We have evaluated the influence of genotype on factor VII levels in 99 healthy Polish blood donors and observed that Arg353Gln frequently occurs in the absence of the insert. In univariate analysis, the mean levels of factor VII coagulant activity (VII:C) and factor VII antigen (VII:Ag) were significantly lower in 16 people who were heterozygous for Arg353Gln and the insert compared with 72 normal subjects who had neither Arg353Gln nor the insert (88.8% of normal and 83.1% versus 102% and 100%, P = .019 and P = .0003, respectively). In nine subjects heterozygous for Arg353Gln alone, VII:C and VII:Ag were significantly decreased compared with the normal subjects (81.9% and 83%, respectively, P = .007 and P = .004). In multivariate analysis, Arg353Gln but not the insert significantly reduced VII:C and VII:Ag after adjustment for age and plasma triglycerides (P < .05 and P = .02, respectively). To evaluate the mechanism responsible for reduced factor VII levels in individuals with Arg353Gln, we performed transient transfection assays with factor VII cDNA containing the base substitution resulting in Gln353 and wild-type factor VII cDNA in COS-1 cells. The levels of VII:Ag in the cell lysates were similar, but the amino acid substitution significantly reduced factor VII secretion into the media to 74.9% of wild-type (P = .0001). Based on these in vivo and in vitro studies, we conclude that the Arg353Gln polymorphism alone can decrease plasma factor VII levels.

Animals↗
PubMed1981

Hematology of experimental acute Sarcocystis bovicanis infection in calves. II. Serum biochemistry and hemostasis studies.

Of four Holstein-Friesian calves infected with 200,000 sporocysts of Sarcocystis bovicanis, three become ill and died on days 35, 55, and 59 of a 63-day experiment. No control calves became ill or died. Serum biochemicals and hematologic indicators of hemostasis from both groups were measured throughout the experiment. Creatine phosphokinase values for both groups increased markedly during acute infection. Lactic dehydrogenase and aspartate aminotransferase values were high in infected calves on days 25 to 35 and days 24 to 63, respectively, indicating injury of muscle, liver, or other tissues. Sorbitol dehydrogenase values were significantly higher for infected than for control calves on days 25 and 35, indicating liver injury. Serum bilirubin and blood urea nitrogen values were significantly increased in three anemic infected calves from day 25 or 26 to day 35, probably reflecting destruction of erythrocytes. The fourth infected calf was not anemic and had no hyperbilirubinemia and only minimal azotemia. Serum protein and albumin values decreased in infected calves on days 21 to 30 or 35, when, although hypoalbuminemia persisted, total protein concentration increased. Glucose, calcium, sodium, and chloride values decreased in infected calves slightly before onset of illness and remained low throughout the experiment. Potassium, magnesium, and phosphorus values did not differ between infected and control calves. Activated partial thromboplastin time and Russell's viper venom time were normal; prothrombin time was significantly higher from day 27 to day 49 in infected calves. This pattern was interpreted as evidence for acquired factor VII deficiency. Abnormal retraction of blood clots and enlarged platelets in blood smears, which indicate platelet dysfunction and increased platelet turnover, respectively, were seen on days 27 through 35 in anemic infected calves. Values for thrombin time (three calves) and fibrin degradation product concentration (one calf) increased just before death of the infected calves.

Animals↗