A Serendipitous Finding of Coagulation Factor VII Deficiency in Two Asymptomatic Patients.
asymptomatic
blood coagulation disorder
blood coagulation factors
factor-vii deficiency
pediatric hematology
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
Aug 2024
Aug 2024
Historique:
accepted:
29
08
2024
medline:
30
9
2024
pubmed:
30
9
2024
entrez:
30
9
2024
Statut:
epublish
Résumé
Congenital Factor VII (FVII) deficiency is a rare autosomal recessive disorder with a prevalence of approximately 1:500,000. It plays a crucial role in initiating coagulation by activating Factors IX and X. Diagnosis typically involves prolonged prothrombin time (PT) and varies widely in clinical presentation. Management includes fresh frozen plasma (FFP), prothrombin complex concentrates (PCC), and recombinant activated FVII (rFVIIa), with rFVIIa often preferred due to its safety and efficacy. We present two pediatric cases: a five-year-old boy with a prolonged PT at 55% and FVII levels at 25.1%, and a two-year-old boy with a PT at 24% and FVII levels at 4.6%. Both cases highlight the importance of thorough hemostatic evaluation and tailored management strategies in FVII deficiency.
Identifiants
pubmed: 39347221
doi: 10.7759/cureus.68133
pmc: PMC11438495
doi:
Types de publication
Case Reports
Journal Article
Langues
eng
Pagination
e68133Informations de copyright
Copyright © 2024, Ouerradi et al.
Déclaration de conflit d'intérêts
Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.