scholarly journals Congenital Fibrinogen Deficiency in India and Role of Human Fibrinogen Concentrate

2021 ◽  
pp. 1-8
Author(s):  
M. Joseph John ◽  
Poojitha Byreddy ◽  
Ketan Modak ◽  
Mridul Makkar
Keyword(s):  
Volume Overload ◽  
Fresh Frozen Plasma ◽  
Viral Transmission ◽  
Fibrinogen Concentration ◽  
Fibrinogen Concentrate ◽  
Human Fibrinogen ◽  
Fresh Frozen ◽  
High Doses ◽  
Frozen Plasma

Congenital fibrinogen deficiency is an inherited disorder due to genetic mutations with diverse presentations arising from reduced fibrinogen levels (hypofibrinogenemia), absence of fibrinogen in circulation (afibrinogenemia), abnormal functioning (dysfibrinogenemia) or both reduced levels and abnormal functioning (hypodysfibrinogenemia) of fibrinogen. The decreased fibrinogen concentration in congenital fibrinogen deficiency necessitates fibrinogen replacement therapy with fresh frozen plasma, cryoprecipitate, or human fibrinogen concentrate. However, the use of fresh frozen plasma and cryoprecipitate is limited owing to their longer transfusion time, requirement of high doses, volume overload, risk of viral transmission, and other safety concerns. The availability of human fibrinogen concentrate has made it the preferred replacement alternative due to its reduced risk of viral transmission, smaller infusion volume, and accurate dosing. The hemostatic efficacy and safety of human fibrinogen concentrate in congenital fibrinogen deficiency is well established in the literature. We review the prevalence of congenital fibrinogen deficiency in India and the current role of human fibrinogen concentrate in its management.

scholarly journals The Effect of Adjunctive Fresh Frozen Plasma Administration on Coagulation Parameters and Survival in a Canine Model of Antivenom-treated Brown Snake Envenoming

2005 ◽  
Vol 33 (1) ◽  
pp. 36-40 ◽  
Author(s):  
G. A. Jelinek ◽  
A. Smith ◽  
D. Lynch ◽  
A. Celenza ◽  
I. Irving ◽  
...  
Keyword(s):  
Platelet Count ◽  
Early Death ◽  
Canine Model ◽  
Fresh Frozen Plasma ◽  
Dose Administration ◽  
Fresh Frozen ◽  
Venom Dose ◽  
Clotting Disorder ◽  
Frozen Plasma

This study aimed to assess the effects of dugite envenoming on blood coagulation and platelet count in a canine model, and the efficacy of fresh frozen plasma (FFP) in reversing the clotting disorder after both adequate and inadequate venom neutralization. Following initial dosing and administration studies, an intravenous venom dose of 1μg/kg was administered to eleven dogs. This was followed 30 minutes later by antivenom in either adequate or inadequate doses. A further 30 minutes later, the animals were given either two units of their own FFP or saline. Fibrinogen, aPTT and platelet levels were monitored for eight hours. Of the six study dogs given antivenom plus FFP, two died at around 60 to 90 minutes post envenoming, at the end of the FFP infusions, and all but one of the survivors had persistent afibrinogenaemia. Of the five study dogs given antivenom and no FFP, all but one had return of detectable fibrinogen at eight hours after envenoming. The platelet count fell in all animals with recovery independent of antivenom dose, administration of FFP, or regeneration of fibrinogen. Post mortem examinations of dogs that died during dosage and administration studies showed massive intracardiac clots. We conclude that early death from Brown Snake envenoming may be due to massive intravascular clotting. FFP administration was associated with persistent afibrinogenaemia regardless of antivenom dose. In the absence of any evidence for its efficacy, this study suggests that the role of FFP after Brown Snake envenoming should be reconsidered.

Perioperative management of coagulation

2006 ◽  
Vol 26 (S 02) ◽  
pp. S3-S14 ◽  
Author(s):  
P. Innerhofer
Keyword(s):  
Coagulation Factor ◽  
Fresh Frozen Plasma ◽  
Laboratory Evaluation ◽  
Blood Component ◽  
Actual Case ◽  
Fresh Frozen ◽  
Coagulation Factor Concentrates ◽  
Hemostatic Therapy ◽  
Frozen Plasma

SummaryGuidelines of official societies for diagnosis and therapy of intraoperatively occurring hypocoagulability rely mainly on data of patients receiving whole blood transfusions. They recommend -provided that laboratory evaluation shows deficiency (values >1.5 fold normal)- administration of fresh frozen plasma, cryoprecipitate and platelet concentrates (platelet count <50 000 or <100 000/μl). This article describes the pathogenesis of coagulopathy in the light of the special intraoperative setting, emphasizes recent changes of blood component preparation, transfusion triggers, effects of volume therapy and challenges standard laboratory assays as reliable guide for intraoperative hemostatic therapy. The role of thrombelastographic monitoring is discussed as well as an alternative strategy to compensate deficiencies by the use of coagulation factor concentrates instead of or in addition to transfusion of FFP, a new concept which is illustrated by the presentation of an actual case report.

The effect of plasmapheresis on plasma cholinesterase levels in a patient with organophosphate poisoning

2004 ◽  
Vol 23 (7) ◽  
pp. 365-368 ◽  
Author(s):  
Muhammet Güven ◽  
Murat Sungur ◽  
Bülent Eser
Keyword(s):  
Normal Values ◽  
Fresh Frozen Plasma ◽  
Plasma Cholinesterase ◽  
Organophosphate Poisoning ◽  
Fresh Frozen ◽  
Medical Intensive Care ◽  
High Level ◽  
Plasma Measurements ◽  
Frozen Plasma

Objective: To describe the role of plasmapheresis in management of organophosphate poisonings. Design: Case report. Setting: A medical intensive care unit of a medical faculty. Patient: A patient with organophosphate poisoning whose cholinesterase levels continuously decline and then increase up to a normal level after plasmapheresis is performed for his sepsis. Interventions: Plasmapheresis with fresh frozen plasma. Measurements and main results: Baseline plasma cholinesterase (ChE) level was 4001 IU/L (normal values: 4000-10000 IU/L). Aspiration pneumonia was developed on day 3, and sepsis occurred on day 5. During this period, ChE levels gradually decreased. On day 5, plasmapheresis was performed for sepsis. Interestingly, plasma ChE levels increased from 2101 IU/L to 6144 IU/L after plasmapheresis. Atropine and pralidoxime were stopped, and a high level of ChE continued during hospitalization. The patient was successfully weaned from mechanical ventilation 3 days after plasmapheresis. Conclusion: Plasma exchange therapy may be considered for patients with organophosphate poisoning unresponsive to atropine and pralidoxime.

Fibrinogen concentrate and cryoprecipitate but not fresh frozen plasma correct low fibrinogen concentrations following in vitro haemodilution

2013 ◽  
Vol 131 (5) ◽  
pp. e210-e213 ◽  
Author(s):  
Christian Fenger-Eriksen ◽  
Kirsten Christiansen ◽  
John Laurie ◽  
Benny Sørensen ◽  
Catherine Rea
Keyword(s):  
Fresh Frozen Plasma ◽  
Fibrinogen Concentrate ◽  
Fresh Frozen ◽  
Frozen Plasma

The In Vitro Effects of Fibrinogen Concentrate, Factor XIII and Fresh Frozen Plasma on Impaired Clot Formation After 60% Dilution

2008 ◽  
Vol 106 (5) ◽  
pp. 1360-1365 ◽  
Author(s):  
Thorsten Haas ◽  
Dietmar Fries ◽  
Corinna Velik-Salchner ◽  
Christian Reif ◽  
Anton Klingler ◽  
...  
Keyword(s):  
Factor Xiii ◽  
Fresh Frozen Plasma ◽  
Clot Formation ◽  
Fibrinogen Concentrate ◽  
Fresh Frozen ◽  
In Vitro Effects ◽  
Frozen Plasma

Clinical experience of factor XI deficiency: the role of fresh frozen plasma and factor XI concentrate

Haemophilia ◽  
1995 ◽  
Vol 1 (4) ◽  
pp. 227-231 ◽  
Author(s):  
P.W. COLLINS ◽  
E. GOLDMAN ◽  
P. LILLEY ◽  
K. J. PASI ◽  
C. A. LEE
Keyword(s):  
Clinical Experience ◽  
Fresh Frozen Plasma ◽  
Factor Xi ◽  
Factor Xi Deficiency ◽  
Fresh Frozen ◽  
Frozen Plasma

Severe Pediatric Blunt Trauma—Successful ROTEM-Guided Hemostatic Therapy With Fibrinogen Concentrate and No Administration of Fresh Frozen Plasma or Platelets

2012 ◽  
Vol 19 (4) ◽  
pp. 453-459 ◽  
Author(s):  
Bernhard Ziegler ◽  
Christa Schimke ◽  
Peter Marchet ◽  
Birgit Stögermüller ◽  
Herbert Schöchl ◽  
...  
Keyword(s):  
Blunt Trauma ◽  
Fresh Frozen Plasma ◽  
Fibrinogen Concentrate ◽  
Fresh Frozen ◽  
Hemostatic Therapy ◽  
Frozen Plasma

Utility of Whole Blood Hemostatometry Using the Clot Signature Analyzer®for Assessment of Hemostasis in Cardiac Surgery

2002 ◽  
Vol 96 (5) ◽  
pp. 1115-1122 ◽  
Author(s):  
Nauder Faraday ◽  
Eliseo Guallar ◽  
Valerie A. Sera ◽  
Everlie D. Bolton ◽  
Robert B. Scharpf ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Whole Blood ◽  
Thrombus Formation ◽  
Fresh Frozen Plasma ◽  
Fibrinogen Concentration ◽  
Plasma Transfusion ◽  
Predictive Values ◽  
Signature Analyzer ◽  
Fresh Frozen ◽  
Frozen Plasma

Background A hemostatic monitor capable of rapid, accurate detection of clinical coagulopathy within the operating room could improve management of bleeding after cardiopulmonary bypass (CPB). The Clot Signature Analyzer is a hemostatometer that measures global hemostasis in whole blood. The authors hypothesized that point-of-care hemostatometry could detect a clinical coagulopathic state in cardiac surgical patients. Methods Fifty-seven adult patients scheduled for a variety of elective cardiac surgical procedures were studied. Anesthesia, CPB, heparin anticoagulation, protamine reversal, and transfusion for post-CPB bleeding were all managed by standardized protocol. Clinical coagulopathy was defined by the need for platelet or fresh frozen plasma transfusion. The Clot Signature Analyzer collagen-induced thrombus formation (CITF) assay measured platelet-mediated hemostasis in vitro. The activated clotting time, platelet count, prothrombin time, activated partial thromboplastin time, and fibrinogen concentration were also measured. Results The postprotamine CITF was greater in patients who required hemostatic transfusion than in those who did not (17.6 +/- 8.0 min vs. 10.5 +/- 5.7 min, respectively; P &lt; 0.01). Postprotamine CITF values were highly correlated with platelet and fresh frozen plasma transfusion (Spearman r = 0.50, P &lt; 0.001 and r = 0.40, P &lt; 0.005, respectively). Receiver operator characteristic curves showed a highly significant relation between the postprotamine CITF and intraoperative platelet and fresh frozen plasma transfusion (area under the curve, 0.78-0.81, P &lt; 0.005) with 60-80% sensitivity, specificity, positive and negative predictive values at cutoffs of 12-14 min. Logistic regression demonstrated that the CITF was independently predictive of post-CPB hemostatic transfusion, but standard hemostatic assays were not. Conclusions The Clot Signature Analyzer CITF detects a clinical coagulopathic state after CPB and is independently predictive of the need for hemostatic transfusion. Hemostatometry has potential utility for monitoring hemostasis in cardiac surgery.

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