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Management of surgical procedures in children with severe FV deficiency: experience of 13 surgeries

M Mathias1, O Tunstall, K Khair

  • 1Great Ormond Street Hospital of Children NHS Foundation Trust, London, UK. mary.mathias@gosh.nhs.uk

Haemophilia : the Official Journal of the World Federation of Hemophilia
|November 24, 2012
PubMed

Insights

Severe factor V deficiency presents varied bleeding risks, even with very low factor levels. This study details surgical management in three children with severe factor V deficiency, highlighting experiences with fresh frozen plasma.

Area of Science:

  • Hematology
  • Pediatric Surgery
  • Coagulation Disorders

Background:

  • Severe homozygous factor V (FV) deficiency is rare, affecting approximately one in a million individuals.
  • Patients with FV levels below 0.01 IU mL(-1) exhibit a spectrum of bleeding phenotypes, from mild to severe neonatal intracerebral hemorrhage.
  • Current treatment options for FV deficiency are limited to fresh frozen plasma (FFP) or solvent detergent FFP.

Observation:

  • This study reports on the surgical hemostatic management of three pediatric patients with severe FV deficiency.
  • A total of 13 surgical procedures were performed across these three children.
  • The experience focuses on the use of FFP for hemostatic cover during surgery.

Findings:

  • The study provides practical insights into managing surgical bleeding in severe FV deficiency.
  • It underscores the challenges associated with inadequate hemostasis in this rare bleeding disorder.
  • The data contributes to understanding the clinical application of FFP in complex surgical scenarios for FV deficient patients.

Implications:

  • The findings may inform surgical strategies for patients with severe FV deficiency.
  • Further research into optimized hemostatic agents for severe FV deficiency is warranted.
  • Improved management protocols could reduce bleeding complications in pediatric patients undergoing surgery.

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