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Extrinsic and Intrinsic Pathways of Hemostasis01:20

Extrinsic and Intrinsic Pathways of Hemostasis

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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...
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Updated: Jun 21, 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.

Mehran Karimi1, Zsuzsanna Bereczky, Nader Cohan

  • 1Hematology Research Center, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Karimim@sums.ac.ir

Seminars in Thrombosis and Hemostasis
|July 15, 2009
PubMed
Summary
This summary is machine-generated.

Factor XIII (FXIII) deficiency causes severe bleeding disorders due to impaired fibrin stabilization. Diagnosis involves FXIII activity assays, with FXIII concentrate available for treatment and prophylaxis.

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Area of Science:

  • Hematology
  • Biochemistry
  • Genetics

Background:

  • Factor XIII (FXIII) is a critical transglutaminase in the coagulation cascade.
  • FXIIIa stabilizes fibrin clots and inhibits fibrinolysis.
  • Severe FXIII deficiency, particularly of the A subunit, leads to significant bleeding diathesis.

Purpose of the Study:

  • To review the clinical manifestations and diagnostic approaches for Factor XIII deficiency.
  • To discuss the analytical methods for assessing FXIII activity and antigen levels.
  • To provide an overview of current treatment options for FXIII deficiency.

Main Methods:

  • Review of clinical data and laboratory findings in FXIII deficiency.
  • Discussion of quantitative FXIII activity assays and antigen measurement techniques.
  • Analysis of genetic mutations associated with FXIII deficiency.

Main Results:

  • Severe FXIII-A deficiency presents with characteristic bleeding symptoms, including delayed umbilical stump bleeding and intracranial hemorrhage.
  • Impaired wound healing and recurrent spontaneous abortions are observed in women with FXIII deficiency.
  • FXIII concentrate is effective for treatment and prophylaxis; quantitative assays are recommended for diagnosis.

Conclusions:

  • Factor XIII deficiency is a rare but serious bleeding disorder requiring accurate diagnosis and management.
  • Quantitative FXIII activity assays are essential for screening and diagnosis.
  • Genetic analysis reveals point mutations in F13A1 and F13B genes as the primary cause.