Trial reportHaematologica2013
Prophylaxis in congenital factor VII deficiency: indications, efficacy and safety. Results from the Seven Treatment Evaluation Registry (STER).
Trial report in Haematologica, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers, 1 of them a synthesis that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
24 citing papers in PubMed, 1 synthesis or guideline pooled it, 92 citations in OpenAlex.
- Principles of treatment and update of recommendations for the management of haemophilia and congenital bleeding disorders in Italy.Blood transfusion = Trasfusione del sangue · 2014Guideline
- Article
- Perioperative Prophylaxis in Pediatric Factor VII Deficiency: Experience From Rabat Children's Hospital.Cureus · 2026Article
- Current status and future prospects of activated recombinant coagulation factor VIIa, NovoSeven®, in the treatment of haemophilia and rare bleeding disorders.Annals of hematology · 2024Review
- Rare inherited coagulation disorders: no longer orphan and neglected.Research and practice in thrombosis and haemostasis · 2024Review
- Thromboelastometry as an Ancillary Tool for Evaluation of Coagulation Status after rFVIIa Therapy in a Pregnant Woman with Severe Hypoproconvertinemia-A Case Series and Review of the Literature.International journal of environmental research and public health · 2022Review
- Case report of recurrent bleeding in an infant with isolated prolonged prothrombin time due to congenital factor VII deficiency---aJournal of family medicine and primary care · 2022Article
- Gene Therapy for Inherited Bleeding Disorders.Seminars in thrombosis and hemostasis · 2021Review
- Biochemical, molecular and clinical aspects of coagulation factor VII and its role in hemostasis and thrombosis.Haematologica · 2021Review
- Inhibitor in Congenital Factor VII Deficiency; a Rare but Serious Therapeutic Challenge-A Systematic Literature Review.Journal of clinical medicine · 2021Review
- [Congenital factor Ⅶ deficiency: a retrospective analysis of 43 cases].Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi · 2020Article
- Clinical problems and surgical interventions in inherited factor VII deficiency.Turk pediatri arsivi · 2020Article
- Psychosocial Impact and Disease Management in Patients with Congenital Factor VII Deficiency.Journal of blood medicine · 2020Article
- Congenital Factor Deficiencies in Children: A Report of a Single-Center Experience.Clinical and applied thrombosis/hemostasis : official journal of the International Academy of Clinical and Applied Thrombosis/Hemostasis · 2018Article
- Recurrent Bleedings in Newborn: A Factor VII Deficiency Case Report.Transfusion medicine and hemotherapy : offizielles Organ der Deutschen Gesellschaft fur Transfusionsmedizin und Immunhamatologie · 2018Article
- Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic.Journal of blood medicine · 2018Review
- Diagnosis and Treatment of von Willebrand Disease and Rare Bleeding Disorders.Journal of clinical medicine · 2017Review
- Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy.Journal of clinical medicine · 2017Review
- Heterozygous congenital Factor VII deficiency with the 9729del4 mutation, associated with severe spontaneous intracranial bleeding in an adolescent male.Blood cells, molecules & diseases · 2016Article
- Sustained correction of FVII deficiency in dogs using AAV-mediated expression of zymogen FVII.Blood · 2016Article
Corrections and comments
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Authors and funding
14 authors at 12 institutions in 9 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Because of the very short half-life of factor VII, prophylaxis in factor VII deficiency is considered a difficult endeavor. The clinical efficacy and safety of prophylactic regimens, and indications for their use, were evaluated in factor VII-deficient patients in the Seven Treatment Evaluation Registry. Prophylaxis data (38 courses) were analyzed from 34 patients with severe factor VII deficiency (<1-45 years of age, 21 female). Severest phenotypes (central nervous system, gastrointestinal, joint bleeding episodes) were highly prevalent. Twenty-one patients received recombinant activated factor VII (24 courses), four received plasma-derived factor VII, and ten received fresh frozen plasma. Prophylactic schedules clustered into "frequent" courses (three times weekly, n=23) and "infrequent" courses (≤ 2 times weekly, n=15). Excluding courses for menorrhagia, "frequent" and "infrequent" courses produced 18/23 (78%) and 5/12 (41%) "excellent" outcomes, respectively; relative risk, 1.88; 95% confidence interval, 0.93-3.79; P=0.079. Long term prophylaxis lasted from 1 to >10 years. No thrombosis or new inhibitors occurred. In conclusion, a subset of patients with factor VII deficiency needed prophylaxis because of severe bleeding. Recombinant activated factor VII schedules based on "frequent" administrations (three times weekly) and a 90 μg/kg total weekly dose were effective. These data provide a rationale for long-term, safe prophylaxis in factor VII deficiency.
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