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Factor VII deficiency is the most common of the rare coagulation deficiencies. A hemorrhagic syndrome may occur in patients with FVII deficiency below 20%, although no correlation exist between the plasma FVII activity level (FVII:C) and the bleeding risk. Therefore, the management of surgery in patients with FVII deficiency remains challenging. Laboratory monitoring of FVII:C level may be helpful but should be interpreted with caution, because the dosage of FVII:C level may vary depending on the origin of the thromboplastin used. Herein, we report the case of the management of a woman who had been fortuitously diagnosed during pregnancy with FVII deficiency due to FVII variant Padua, which have induced discrepant results between two different laboratories.
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http://dx.doi.org/10.1684/abc.2020.1545 | DOI Listing |
Pediatr Int
August 2025
Department of Pediatric Hematology and Oncology, Ankara City Hospital, Yıldırım Beyazıt University, Cankaya, Turkey.
Background: A history and physical examination can predict most bleeding disorders. This study aimed to reveal possible hemostatic disorders in patients referred to the pediatric hematology department due to the incidentally detected prolonged prothrombin time (PT) or activated partial thromboplastin time (aPTT).
Methods: Pediatric patients without known hematologic disease and referred to investigate the incidental prolonged PT and/or aPTT were included.
Res Pract Thromb Haemost
July 2025
Hematology Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Background: Low doses of recombinant activated factor (F)VII (rFVIIa), used to manage bleeding in patients with severe FXI deficiency, have been proposed to bypass effects of the FXI/FXIa inhibitor abelacimab.
Objectives: To test whether low concentrations of rFVIIa could abolish changes in coagulation parameters induced by abelacimab as measured by rotational thromboelastometry.
Methods: Whole blood specimens obtained in citrated tubes from 6 healthy donors were incubated with 15 and 30 μg/mL of abelacimab or vehicle for 10 minutes at 37 °C.
Medicine (Baltimore)
August 2025
School of Medicine, China Medical University, Taichung, Taiwan.
Rationale: Hemophilia, an inherited bleeding disorder, is rarely caused by congenital factor VII (FVII) deficiency, occurring in 1 in 500,000 patients. Oftentimes, the presenting symptom of newborns with congenital FVII deficiency is intracranial hemorrhage, which can lead to adverse neurological outcomes.
Patient Concerns: A 2-day-old male infant, born late preterm, presented with symptoms of coffee-ground emesis, lethargy, and feeding difficulties.
Blood Coagul Fibrinolysis
August 2025
Ege University Faculty of Medicine, Department of Pediatric Genetics, İzmir.
Background: Factor VII (FVII) deficiency is a rare autosomal recessive bleeding disorder caused by pathogenic variants in the F7 gene. Clinical manifestations vary widely, ranging from asymptomatic cases to severe bleeding episodes, including gastrointestinal bleeding and intracranial hemorrhage.
Objective: This study aims to evaluate the clinical and molecular characteristics of Turkish patients diagnosed with FVII deficiency and explore genotype-phenotype correlations.
Mol Ther Methods Clin Dev
September 2025
Department of Molecular Hematology, Sanquin Research and Landsteiner Laboratory of the Amsterdam UMC, 1066 CX Amsterdam, the Netherlands.
Factor VII (FVII) deficiency is a rare bleeding disorder with a prevalence of approximately 1:300,000-500,000 individuals. We explored whether adeno-associated virus (AAV)-mediated gene therapy can achieve durable and functional expression of human FVII (hFVII) . Wild-type hFVII (hFVIIwt) and a naturally occurring splice variant designated hFVII(-22) (GenBank: NM_019616.
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