A Serendipitous Finding of Coagulation Factor VII Deficiency in Two Asymptomatic Patients

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 pr...

Full description

Saved in:
Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2024-08, Vol.16 (8), p.e68133
Main Authors: Ouerradi, Nourelhouda, Ayad, Ghannam, Elouali, Aziza, Babakhouya, Abdeladim, Rkain, Maria
Format: Article
Language:English
Subjects:
Antigens
Asymptomatic
Circumcision
Congenital diseases
Disease prevention
Hematology
Hemoglobin
Hemophilia
Internal Medicine
Laboratories
Patients
Pediatrics
Plasma
Surgery
Citations: Items that this one cites
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.68133