Related Experiment Video
Updated: May 31, 2026
12:24
Helical Organization of Blood Coagulation Factor VIII on Lipid Nanotubes
Published on: June 3, 2014
Fibrinogen replacement therapy for congenital fibrinogen deficiency
L Bornikova1, F Peyvandi, G Allen
1Hemophilia and Thrombosis Center, Department of Medicine, University of Colorado, Aurora, CO 80045, USA.
Journal of Thrombosis and Haemostasis : JTH
|June 30, 2011
Summary
Fibrinogen replacement therapy is effective for congenital fibrinogen deficiency (CFD) bleeding, but requires careful monitoring. Afibrinogenemia patients face high thrombosis risk, even without treatment.
Area of Science:
- Hematology
- Clinical Pharmacology
- Genetics
Background:
- Congenital fibrinogen deficiency (CFD) encompasses afibrinogenemia, hypofibrinogenemia, and dysfibrinogenemia.
- Effective management of bleeding in CFD requires understanding fibrinogen replacement therapy (FRT) parameters.
Observation:
- A systematic review of 104 case reports (1961-2010) identified 50 CFD cases receiving FRT.
- Data extraction focused on dosing, efficacy, and adverse events, including thrombosis.
- Afibrinogenemia, hypofibrinogenemia, and dysfibrinogenemia were represented, with 35, 6, and 9 cases respectively.
Findings:
- FRT effectively controlled bleeding when fibrinogen levels were maintained above 50-100 mg/dL (non-surgical/obstetric) or 100-200 mg/dL (surgical).
- Increased fibrinogen clearance occurred during massive hemorrhage, major surgery, and advanced pregnancy.
- Thrombotic events occurred in 30% of patients (15/50), notably 67% (8/12) of adult non-obstetric afibrinogenemia patients, irrespective of FRT.
Implications:
- FRT regimens can be guided by published data for 50 CFD patients.
- Close monitoring of fibrinogen levels is crucial during FRT.
- Afibrinogenemia carries an inherent risk of thromboembolic complications, necessitating vigilant management.
Related Concept Videos
01:29Venous Thrombosis III: Interprofessional Care
Venous thrombosis requires effective prevention and treatment strategies to improve patient outcomes and reduce potential complications.Prevention StrategiesHealthcare providers must prioritize preventing venous thromboembolism (VTE) for all adult patients upon admission. Interventions depend on bleeding and thrombosis risk, medical history, current medications, diagnoses, planned procedures, and patient preferences. Patients on bed rest should change positions every two hours and, if not...
01:16Clot Retraction and Fibrinolysis
After a fibrin clot is formed, the next step is clot retraction, a vital process facilitated by platelet contractile proteins, such as actin and myosin. These proteins pull the fibrin strands closer together and condense the clot. This action reduces the size of the clot, creating a smaller, denser structure that effectively seals off the damaged vessel. Clot retraction consolidates the clot and helps with wound healing by bringing the edges of the damaged blood vessel closer together.
01:30Anticoagulant Drugs: Low-Molecular-Weight Heparins
Hemostasis is a crucial process that prevents excessive blood loss from damaged blood vessels. It involves various mechanisms such as vasoconstriction, platelet adhesion and activation, and fibrin formation. The importance of each mechanism depends on the type of vessel injury. In contrast, thrombosis is the abnormal formation of a blood clot within the blood vessels, leading to potential complications if the clot obstructs blood flow. Thrombosis can be caused by increased coagulability of the...
01:30Continuous Renal Replacement Therapy
Continuous Renal Replacement Therapy, also known as CRRT, is a procedural treatment for acute kidney injury (AKI) that gradually removes uremic toxins and fluids while maintaining acid-base balance and stabilizing electrolytes. It is particularly useful for hemodynamically unstable patients. Unlike intermittent hemodialysis, which is faster, CRRT provides a gentler approach over 24 hours, closely mimicking the function of natural kidneys. However, CRRT is not ideal for patients with...
01:20Extrinsic and Intrinsic Pathways of Hemostasis
Blood clotting or coagulation involves extrinsic and intrinsic pathways, which ultimately merge into the common pathway, forming a fibrin clot.
The Extrinsic Pathway
The extrinsic pathway of coagulation is typically initiated by tissue damage that exposes blood to tissue factor (TF), a protein released by the damaged tissue cells outside the blood vessels—this interaction with TF triggers biochemical reactions involving specific clotting factors. The key player here is Factor VII, which forms a...
The Extrinsic Pathway
The extrinsic pathway of coagulation is typically initiated by tissue damage that exposes blood to tissue factor (TF), a protein released by the damaged tissue cells outside the blood vessels—this interaction with TF triggers biochemical reactions involving specific clotting factors. The key player here is Factor VII, which forms a...
01:09Coagulation
The coagulation phase is a critical part of the body's process to prevent blood loss following injury to blood vessels. It involves chemical reactions that form a clot to seal the injured area. The clotting process begins shortly after injury, within 15-20 seconds for severe damage and 1-2 minutes for minor injuries.
During the coagulation phase, clotting factors, or procoagulants, play a vital role in initiating and progressing the coagulation cascade. This cascade is a series of reactions...
During the coagulation phase, clotting factors, or procoagulants, play a vital role in initiating and progressing the coagulation cascade. This cascade is a series of reactions...