Recombinant Activated Factor VII (Eptacog Alfa Activated, NovoSeven®) in Patients with Rare Congenital Bleeding Disorders. A Systematic Review on its Use in Surgical Procedures

Minno, Matteo Nicola Dario Di; Ambrosino, Pasquale; Myasoedova, Veronika A.; Amato, Manuela; Ventre, Itala; Tremoli, Elena; Minno, Alessandro Di

doi:10.2174/1381612822666161230143612

Cited by 8 publications

(6 citation statements)

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“…For invasive procedures and minor surgery, 1-day RT resulted sufficient, with an “average” total dose of rFVIIa of 20 μg/kg/body weight, possibly in more than one administration [ 39 ]. RT with rFVIIa has a very good safety-to-efficacy ratio [ 38 , 39 , 44 , 45 ]. Continuous infusion of rFVIIa during surgery has also been reported as effective, safe, and well-tolerated in patients with inherited Factor VII deficiency [ 46 ].…”

Section: Replacement Therapymentioning

confidence: 99%

Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy

Napolitano

Siragusa

Mariani

2017

JCM

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Factor VII deficiency is the most common among rare inherited autosomal recessive bleeding disorders, and is a chameleon disease due to the lack of a direct correlation between plasma levels of coagulation Factor VII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even in homozygous subjects—to severe life-threatening bleedings (central nervous system, gastrointestinal bleeding). Prediction of bleeding risk is thus based on multiple parameters that challenge disease management. Spontaneous or surgical bleedings require accurate treatment schedules, and patients at high risk of severe hemorrhages may need prophylaxis from childhood onwards. The aim of the current review is to depict an updated summary of clinical phenotype, laboratory diagnosis, and treatment of inherited Factor VII deficiency.

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Section: Replacement Therapymentioning

confidence: 99%

Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy

Napolitano

Siragusa

Mariani

2017

JCM

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“… 35 Recent reviews have also focused on treatment options for women with FVII deficiency 5 and for patients with FVII deficiency and other RBDs. 36 …”

Section: Discussionmentioning

confidence: 99%

Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic

Jain¹,

Donkin²,

Frey³

et al. 2018

JBM

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BackgroundOne of the most common rare inherited bleeding disorders, congenital factor VII (FVII) deficiency typically has a milder bleeding phenotype than other rare bleeding disorders. Categorizing severity in terms of factor activity associated with hemophilia (severe <1%, moderate 1%–5%, mild 6%–40%) has led to the observation that bleeding phenotype does not follow closely with FVII activity. Over the past decade, large-scale global registries have investigated bleeding phenotype more thoroughly. The International Society on Thrombosis and Haemostasis has reclassified FVII deficiency as follows: severe, FVII <10%, risk of spontaneous major bleeding; moderate, FVII 10%–20%, risk of mild spontaneous or triggered bleeding; mild, FVII 20%–50%, mostly asymptomatic disease.Case reportsEleven illustrative cases of congenital FVII deficiency adapted from clinical practice are described to demonstrate the variability in presentation and in relation to FVII activity levels. Severe FVII deficiency usually presents at a young age and carries the risk of intracranial hemorrhage, hemarthrosis, and other major bleeds. Moderate FVII deficiency tends to present later, often in adolescence and particularly in girls as they reach menarche. Milder disease may not be apparent until found incidentally on preoperative testing, during pregnancy/childbirth, or following unexplained bleeding when faced with hemostatic challenges.ConclusionIt is important for health care professionals to be aware of the new definitions of severity and typical presentations of congenital FVII deficiency. Failure to appreciate the risks of major bleeding, including intracerebral hemorrhage in those with FVII activity <10%, may put particularly young children at risk.

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“…In sum, FVII deficiency is a rare bleeding disorder with variable clinical symptoms [28][29][30][31][32][33][34][35][36][37] . However, in many cases, there is no direct correlation between the factor plasma levels and the severity of the disease symptoms [28][29][30]32,38] . In fact, some people with very low levels of FVII may demonstrate mild symptoms.…”

Section: Factor VII Deficiencymentioning

confidence: 99%

Factor VII Gene Defects: Review of Functional Studies and Their Clinical Implications

Shahbazi¹,

Mahdian²

2019

ibj

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Coagulation factors belong to a family of plasma glycosylated proteins that should be activated for appropriate blood coagulation. Congenital deficiencies of these factors cause inheritable hemorrhagic diseases. Factor VII (FVII) deficiency is a rare bleeding disorder with variable clinical symptoms. Various mutations have been identified throughout the F7 gene and can affect all the protein domains. The results of previous experiments have partly revealed the correlation between genotype and phenotype in patients with FVII deficiency. Nevertheless, each particular variant may affect the coagulative function of FVII, mainly via altering its expression level, extra-cellular secretion, tissue factor binding affinity, or proteolytic activity. The pathogenicity of the variants and molecular mechanisms responsible for clinical symptoms in patients with FVII deficiency should be characterized via in silico and in vitro, as well as in vivo functional studies. This review has highlighted the most important functional studies reported on F7 gene variants, including relevant reports regarding Iranian FVII deficiency patients.

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Recombinant Activated Factor VII (Eptacog Alfa Activated, NovoSeven®) in Patients with Rare Congenital Bleeding Disorders. A Systematic Review on its Use in Surgical Procedures

Cited by 8 publications

References 0 publications

Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy

Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy

Phenotypical variability in congenital FVII deficiency follows the ISTH-SSC severity classification guidelines: a review with illustrative examples from the clinic

Factor VII Gene Defects: Review of Functional Studies and Their Clinical Implications

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