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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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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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Helical Organization of Blood Coagulation Factor VIII on Lipid Nanotubes
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Published on: June 3, 2014

[Factor XIII deficiency in a newborn].

R Diehl1, S Thouvenin, J Reynaud

  • 1Unité d'hématologie pédiatrique, CHU de Saint-Etienne, hôpital Nord, avenue Albert-Raimond, 42055 Saint-Etienne cedex 02, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|April 25, 2007
PubMed
Summary

Factor XIII deficiency, a rare inherited bleeding disorder, can present with unusual initial symptoms like caput succedaneum. Early diagnosis via FXIII assay is crucial despite normal screening tests for effective treatment.

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Area of Science:

  • Hematology
  • Pediatric Medicine
  • Genetics

Background:

  • Factor XIII deficiency is a rare, inherited bleeding disorder.
  • Classic symptoms include umbilical cord bleeding and intracranial hemorrhage.
  • Standard screening tests may appear normal in affected individuals.

Observation:

  • A case report details a child with Factor XIII deficiency.
  • The initial manifestation was a caput succedaneum.
  • Umbilical cord bleeding was a subsequent symptom.

Findings:

  • Factor XIII deficiency can present with atypical initial symptoms.
  • Quantitative FXIII assay is essential when clinical suspicion is high.
  • Normal results in standard screening tests do not rule out Factor XIII deficiency.

Implications:

  • Highlights the importance of considering Factor XIII deficiency in pediatric bleeding cases.
  • Emphasizes the need for specific FXIII assays for accurate diagnosis.
  • Timely diagnosis and treatment are critical due to significant therapeutic consequences.