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Related Concept Videos

Extrinsic and Intrinsic Pathways of Hemostasis01:20

Extrinsic 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.
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Disorders of Hemostasis01:24

Disorders of Hemostasis

Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
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Anticoagulant Drugs: Low-Molecular-Weight Heparins01:30

Anticoagulant 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...
Venous Thrombosis III: Interprofessional Care01:29

Venous 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...
Anticoagulant Drugs: Vitamin K Antagonists and Direct Oral Anticoagulants01:18

Anticoagulant Drugs: Vitamin K Antagonists and Direct Oral Anticoagulants

Oral anticoagulants are vital tools in preventing and treating blood clotting disorders. This diverse class of medications can be categorized as vitamin K antagonists, exemplified by warfarin, and direct thrombin inhibitors (DTIs), such as dabigatran, as well as factor Xa inhibitors, including rivaroxaban.
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Coagulation01:09

Coagulation

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Related Experiment Video

Updated: Jun 26, 2026

Measurement of Factor V Activity in Human Plasma Using a Microplate Coagulation Assay
13:08

Measurement of Factor V Activity in Human Plasma Using a Microplate Coagulation Assay

Published on: September 9, 2012

Factor XIII deficiency.

L Hsieh1, D Nugent

  • 1Division of Hematology, Children's Hospital of Orange County, Orange, CA 92868, USA. djn0@choc.org

Haemophilia : the Official Journal of the World Federation of Hemophilia
|January 15, 2009
PubMed
Summary
This summary is machine-generated.

Inherited Factor XIII (FXIII) deficiency is a rare bleeding disorder with diverse symptoms, including bleeding and miscarriage. Early diagnosis and treatment with FXIII concentrates offer an excellent prognosis for patients.

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Area of Science:

  • Hematology
  • Genetics

Background:

  • Inherited Factor XIII (FXIII) deficiency is a rare bleeding disorder.
  • FXIII is crucial for hemostasis, stabilizing blood clots by cross-linking fibrin and other proteins.
  • Clinical manifestations vary due to genetic heterogeneity, with over 60 mutations identified.

Purpose of the Study:

  • To summarize the clinical presentation, molecular basis, and management of inherited FXIII deficiency.
  • To highlight the heterogeneity in FXIII deficiency and its impact on clinical symptoms.

Main Methods:

  • Literature review of identified FXIII mutations and associated clinical presentations.
  • Analysis of FXIII's role in hemostasis and clot stabilization.

Main Results:

  • FXIII deficiency can cause umbilical bleeding, bruising, mucosal bleeding, intracranial hemorrhage, poor wound healing, and recurrent miscarriages.
  • Genetic heterogeneity, including mutations and SNPs, contributes to diverse clinical manifestations and FXIII activity levels.
  • Prophylactic treatment with cryoprecipitate or FXIII concentrate is effective.

Conclusions:

  • Inherited FXIII deficiency is a heterogeneous disorder requiring careful diagnosis and management.
  • Despite lifelong bleeding risk, current prophylactic treatments lead to an excellent prognosis.