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Results for “Coagulation Protein Disorders”

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At least 91 records · Page 5Linked to original sources
PubMed2000

Activated recombinant human coagulation factor VII (rFVIIa) therapy for abdominal bleeding in patients with inhibitory antibodies to factor VIII.

Eight patients with inhibitors to factor VIII (4 hemophilia A and 4 nonhemophilic) were treated with recombinant activated factor VII (rFVIIa) to control severe abdominal bleeding. The recombinant factor was supplied under an open-label, emergency-use program to patients previously unresponsive to one or more alternative therapies. Therapy with rFVIIa was administered for nine separate bleeding events; one patient was treated for two separate bleeding episodes. Patients were treated for an average of 9 days and received a mean total dose of 5.2 mg of rFVIIa for control of bleeding. Treatment was considered successful and hemostasis adequate in 7 of the 9 episodes (78%). Treatment with rFVIIa was partially successful in one other episode. Four patients in this series experienced serious adverse events; all the adverse events were considered unrelated to rFVIIa therapy. The results of this limited series indicate that rFVIIa is an effective means of managing life-threatening abdominal bleeding in individuals with hemophilia or acquired antibodies to factor VIII.

Abdomen↗
PubMed2006

Study of coagulation factor activities in apheresed thawed fresh frozen plasma at 1-6 degrees C for five days.

The concern for the loss of activities of coagulation factors in thawed fresh frozen plasma kept at 1-6 degrees C for long periods has prevented transfusion services from using thawed plasma beyond 24 hours of storage. There is no mention of the method of collection of the plasma and/or the study of the bacterial growth in the studies reported in the literature. The present project was undertaken to investigate coagulation factor activities and bacterial growth in apheresed fresh plasma. Twenty apheresed plasma units from different blood groups were used. After the 24-hour expiration time of the thawed plasma kept at 1-6 degrees C, aliquots were taken at day 1, day 3, and day 5 of expiration time and were immediately frozen at -70 degrees C. Samples were assayed for activities of coagulation factors II, V, VII, VIII, X, XI, and fibrinogen (Fib). Our study reveals no statistically significant change in activities of coagulation factors II, VII, X, XI, and fibrinogen from day 1 to day 5 storage of plasma at 1-6 degrees C; however, there is a mean decrease of 8.8 and 14.3% in activities of factors V and VIII, respectively. All culture samples taken on day 5 storage were negative at 7 days. In conclusion, our results do not show a significant change in the activity of most coagulation factors in the thawed apheresis plasma stored at 1-6 degrees C over a 5-day period. Hence, it is feasible to transfuse the plasma beyond the 24-hour period without compromising the clinical outcome of patients with coagulopathy.

Adult↗
PubMed1999

Influence of prothrombin complex concentrates on plasma coagulation in critically ill patients.

OBJECTIVE: To evaluate thrombogenicity of prothrombin complex concentrates (PCCs) in critically ill patients. DESIGN: Prospective clinical study. SETTING: Medical intensive care unit at a university hospital. PATIENTS: 16 consecutive patients suffering from acquired deficiencies of coagulation factors and with either overt bleeding from any site or a planned invasive procedure. INTERVENTIONS: 2000 factor IX units of PCCs intravenously. MEASUREMENTS AND RESULTS: Prothrombin time (PT), activated partial prothrombin time, fibrinogen, platelet count, plasma levels of coagulation factors II, V, VII, VIII, IX, X, antithrombin, protein C, thrombin-antithrombin complex (TAT), prothrombin fragment F(1+2), and the fibrin degradation product D-dimer were measured prior to and 1, 3, and 24 h after administration of PCCs. PT as well as coagulation factors II, VII, IX, and X, TAT, and F(1+2) showed a significant increase after administration of PCCs. All other parameters remained unchanged. CONCLUSIONS: Administration of PCCs induces thrombin generation. No evidence for induction of disseminated intravascular coagulation in biochemical terms could be found. When rapid correction of acquired coagulation factor disturbances is warranted, the use of PCCs seems reasonable, but the elevated risk of intravascular thrombus formation should be kept in mind.

Adult↗
PubMed1999

Emergency correction of coagulation for mitral valve replacement in an orally anticoagulated 17-year-old patient with pronounced hepatic dysfunction.

A 17-year-old patient with Shone's disease had to be readmitted to the hospital 3 months after implantation of an artificial aortic valve because of extreme mitral insufficiency with consecutive pulmonary edema and hepatic dysfunction. He had been orally anticoagulated and presented with a high international normalized ratio of 6.7. Emergency replacement of the mitral valve was possible only after administration of prothrombin-complex concentrate, as vitamin K(1) and fresh frozen plasma did not correct the hemostatic defect sufficiently.

Adolescent↗
PubMed2002

Restrictive interatrial communication with protein-losing enteropathy and coagulopathy in hypoplastic left heart syndrome after Norwood palliation.

The Norwood procedure is one option for neonates born with hypoplastic left heart syndrome. We describe a case of an infant with hypoplastic left heart syndrome, palliatively repaired with the Norwood procedure. The infant developed restriction of the interatrial communication, despite atrioseptectomy at the first stage of palliation. Consequently, a protein-losing enteropathy with severe coagulopathy developed which resolved after a repeat atrioseptectomy.

Coagulation Protein Disorders↗
PubMedDate not supplied

Ethnic differences in coagulation factor abnormalities after the Fontan procedure.

We tested the hypothesis that Chinese patients have a coagulation profile that is less prothrombotic than that of Caucasian counterparts after the Fontan procedure by determining the type and prevalence of anticoagulant and procoagulant deficiencies in Chinese patients and comparing the findings to those previously reported in Caucasian series. The liver function and coagulation factors were assessed in 21 ethnic Chinese patients, aged 17.0 +/- 5.6 years, at 10.7 +/- 4.0 years after the Fontan procedure. The results were compared to those of 21 age-matched Chinese controls with minor congenital heart disease. The prevalence of coagulation factor deficiencies in our patients was further compared to that reported in Caucasian patients. When compared with controls, patients had significantly lower protein C (p = 0.014), factors II (p = 0.024), V (p < 0.001), VII (p < 0.001), IX (p = 0.036), and X (p < 0.001), and higher bilirubin (p = 0.001) levels. The prevalence of protein C deficiency was 9.5%, whereas those of factor II, V, VII, IX, and X deficiencies were 0, 66.7, 9.5, 0, and 57.1%, respectively. When compared with Caucasian data, our data showed a significantly lower prevalence of protein C, total protein S, antithorombin III, factor II, and factor VII deficiencies. Furthermore, the previously reported increase in factor VIII levels was not found. In contrast, the prevalence of factor X deficiency was higher in our patients. This study provides the first evidence of ethnic differences in coagulation factor abnormalities after the Fontan procedure. The imbalance between procoagulant and anticoagulant pathways in Chinese patients favors a bleeding, rather than a thrombotic, tendency.

Adolescent↗
PubMed2000

Homozygous factor V Leiden mutation in a woman with multiple adverse pregnancy outcomes.

We report a case with one intrauterine fetal death (IUFD) at 32 weeks of gestation, one premature delivery at the same week, and one abortion of unknown etiology at 12 weeks of gestation. We discuss that the presence of homozygosity for Factor V Leiden may be associated with placental insufficiency in this woman. Application of anticoagulant therapy may have been beneficial in her current pregnancy.

Abortion, Habitual↗
PubMed2004

Antidotes to haemorrhage: recombinant factor VIIa.

Recombinant Factor VIIa (rFVIIa) concentrates were originally developed to treat the refractory bleeding complications associated with allo-antibody inhibitors in hemophilias A and B. As experience was gained in the hemophilias, the physiology of rFVIIa and its successes in controlling bleeds stimulated rFVIIa use in other challenging medical conditions complicated by bleeding. Thus, rFVIIa has assumed the role of a 'universal pancoagulant' without sufficient evidence-based data from well-designed, adequately powered clinical trials. This chapter discusses the anecdotal experience with rFVIIa based upon the few controlled trials that do exist, and emphasizes that these empirical dosing strategies have not yielded the best approach to achieve effective control of bleeding. Evidence-based data are necessary to establish the cost-benefit and risk-benefit profiles of rFVIIa, and to establish it as a standard treatment for bleeding.

Anticoagulants↗
PubMed2004

INR comparison between the CoaguChek Pro PT(N) and a standard laboratory method.

BACKGROUND: The prothrombin time (PT), also called thromboplastin time ("Quick"), is usually measured using citrated plasma from venous blood. Recently, portable coagulation monitors have been developed, which measure PT using non-anticoagulant capillary whole blood from a finger stick. In the present study, we compared the International Normalized Ratio (INR) of the standard laboratory method (INRven) with the newly developed reagent PT(N) for the CoaguChek Pro portable monitor (INRcap) in various patient groups: healthy individuals, patients with oral anticoagulation and patients with a deficiency of coagulation factor V, factor VII or factor X, respectively. METHODS: One hundred and fifty-five patients were included in this prospective open comparison study. Capillary PT was measured with the portable coagulation monitor CoaguChek Pro using the new disposable cartridge PT(N) (containing rabbit brain thromboplastin). In comparison, PT was measured using citrated venous plasma and the reagent Innovin on the coagulation analyzer STA-R. RESULTS: We found a correlation coefficient of 0.85 between capillary and venous INR values among the 100 patients with oral anticoagulation. The slope of the regression line was 1.4 and the y-intercept is -0.65. Agreement between both methods was found to be 80% (95% CI: 72-88%) and the standard-agreement was 85% (95% CI: 78-92%). Among the 30 healthy subjects, the individual differences between INRven and INRcap were in 4 cases 0, in 21 cases 0.1 and in 5 cases 0.2. CONCLUSION: The new test cartridge PT(N) was found to be a valuable tool for measuring PT among healthy subjects. However, among patients with oral anticoagulation, agreement between INRcap measured with the new cartridge PT(N) and INRven was only moderate. Our results show that improvements are necessary for a more valuable measurement of capillary PT with portable coagulation monitors.

Administration, Oral↗
PubMed2004

Sensitivity of commercial prothrombin time reagents to detect coagulation factor deficiencies in equine plasma.

The sensitivity of commercial prothrombin time (PT) tests was assessed based on a dilution series of equine pooled plasma (EPP) (experiment 1) and on 40 equine plasma samples with reduced activity of coagulation factors II, V, VII and X (experiment 2). Two different PT reagents (reagent 1, human placental thromboplastin; reagent 2, recombinant human tissue factor) were used according to the manufacturers' instructions (standard test, PT([ST])) and compared to a modified test procedure (modified test, PT([MT])) using sample dilution and fibrinogen addition. In all samples, sensitivity was lower (P<0.01) when using PT([ST]) with reagent 2 (0.20) than when using either PT([ST]) with reagent 1 (0.65) or PT([MT]) with both reagents (reagent 1, 0.60-0.75, reagent 2, 0.58-0.70, depending on sample dilution). The highest sensitivity was found for PT([MT]) when using a 1:20 sample dilution. In those samples in which at least one coagulation factor activity was decreased (by 20%; n=18), the sensitivity of PT([ST]) with reagent 2 (0.33) was found to be inadequate, in contrast to all other test procedures (0.83-0.94). This low sensitivity corresponded to shorter time intervals between different coagulation activity levels prepared by EPP dilution. The results indicate that adequate sensitivity of PT measurements in equine plasma can be achieved using a standard test procedure as long as a suitable reagent is used.

Animals↗
PubMed2001

Third-generation progestogen type influences hemostatic changes caused by oral contraceptives in Brazilian women.

We compared the effects of two third-generation progestogens, desogestrel (DSG) and gestodene (GSD), on coagulation and fibrinolysis in Brazilian users of oral contraceptives (OCs). Forty-six women were evaluated before treatment and after six cycles of treatment. The coagulation, anticoagulant, and fibrinolytic systems were investigated. During the use of the DSG-containing OC, the activity of factors VII, VIII, IX, X, and XII increased significantly whereas the GSD-containing OC caused no changes in coagulation parameters. Concerning the anticoagulant pathways, the DSG-containing OC increased protein C levels and decreased total protein S levels, and the GSD-containing OC only decreased total protein S. Both OCs increased plasminogen activity, although the DSG-containing OC increased fibrin degradation products levels and decreased the tissue plasminogen activator antigen. In conclusion, we have found that in Brazilian women the effects of DSG and GSD on hemostatic parameters are different and, therefore, third-generation progestogens may not contribute equally to the thrombotic risk.

Adult↗