Factor VII Deficiency (Hypoproconvertinemia)

An autosomal recessive characteristic or a coagulation disorder acquired in association with VITAMIN K DEFICIENCY. FACTOR VII is a Vitamin K dependent glycoprotein essential to the extrinsic pathway of coagulation.

Also Known As:

Hypoproconvertinemia; Deficiency, Factor 7; Deficiency, Factor Seven; Deficiency, Factor VII; Factor 7 Deficiency; Deficiencies, Factor 7; Deficiencies, Factor Seven; Deficiencies, Factor VII; Factor 7 Deficiencies; Factor Seven Deficiencies; Factor Seven Deficiency; Factor VII Deficiencies; Hypoproconvertinemias

Networked: 325 relevant articles (7 outcomes, 17 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Thrombosis (Thrombus)
2.	Intracranial Hemorrhages (Intracranial Hemorrhage)
3.	Venous Thrombosis (Deep-Vein Thrombosis)
4.	Hemophilia A (Haemophilia)
5.	Hemorrhage

Experts

1.	Mariani, Guglielmo: 6 articles (03/2017 - 02/2002)
2.	Ingerslev, J: 5 articles (11/2015 - 04/2005)
3.	Karimi, M: 5 articles (11/2015 - 07/2008)
4.	Mariani, G: 5 articles (11/2015 - 06/2001)
5.	Bernardi, Francesco: 4 articles (03/2018 - 02/2002)
6.	Napolitano, Mariasanta: 4 articles (03/2017 - 02/2011)
7.	Giansily-Blaizot, Muriel: 4 articles (05/2016 - 02/2003)
8.	Batorova, A: 4 articles (11/2015 - 01/2009)
9.	Giansily-Blaizot, M: 4 articles (11/2015 - 02/2007)
10.	Dolce, Alberto: 4 articles (04/2013 - 03/2005)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Factor VII Deficiency:

1.	recombinant FVIIa (rFVIIa)FDA Link 01/01/2000 - "From the available data it appears that rFVIIa is a safe and effective treatment modality for patients with congenital factor VII deficiency." 05/01/2006 - "Thromboelastographic monitoring of the efficacy of recombinant factor VIIa administration in a parturient with factor VII deficiency." 07/01/2012 - "In this study, we demonstrate that a single and very low dose of recombinant factor VIIa enabled asymptomatic patients with factor VII deficiency to go through major surgery safely. " 09/01/2023 - "Factor VII deficiency, for instance, is often corrected with exogenous administration of recombinant factor VIIa. " 01/01/2022 - "Results: The thromboelastometry results showed significant shortening of the clotting time in the extrinsic and the intrinsic pathway in the hypoproconvertinemia patient after rFVIIa administration in relation to healthy pregnant women. "
2.	Factor VIIa (Activated Factor VII)IBA 08/01/2013 - "Prophylaxis with plasma-derived or recombinant activated factor VII is beneficial in severe factor VII deficiency. " 05/01/2014 - "[Anaesthetic management of a parturient with a severe factor VII deficiency: was activated factor VII really necessary?]." 11/01/2011 - "[Prophylactic use of a recombinant activated factor VII in delivery haemorrhage by caesarean in a woman with major factor VII deficiency: a case report]." 07/01/2011 - "Long-term follow-up of prophylaxis with recombinant activated factor VII in patients with congenital factor VII deficiency." 02/01/2011 - "Recombinant, activated factor VII for surgery in factor VII deficiency: a prospective evaluation - the surgical STER."
3.	Factor XI (Plasma Thromboplastin Antecedent)IBA 07/01/1989 - "A prolonged APTT with normal PT, in the clinical setting, implies a deficiency of factor XI, IX, or VIII. A prolonged PT with normal APTT indicates factor VII deficiency. " 09/01/2016 - "Congenital factor XI and factor VII deficiencies assure an apparent opposite protection against arterial or venous thrombosis: An intriguing observation." 11/01/2007 - "The accompanying coagulopathies consisted of severe or moderate factor VIII (FVIII) deficiency (haemophilia A) in six (37.5%); severe or moderate factor IX deficiency (haemophilia B) in three (18.75%); severe or moderate factor XI deficiency in two (12.5%); severe factor VII deficiency in two (12.5%); combined factor XI and XII deficiencies in one (6.25%); qualitative platelet abnormality in one (6.25%); and one had combined type 2B and type 2N VWD (6.25%). " 01/01/2005 - "This product which was designed for patients with inhibitors to factor VIII or factor IX, and factor VII deficiency, has now been given successfully to four patients with factor XI inhibitors." 08/15/1990 - "The mean concentration of FIXP was markedly reduced to 22.7 pmol/L in nine patients with hereditary factor VII deficiency (factor VII coagulant activity less than 7%) but was not significantly different from normal controls in nine subjects with factor XI deficiency (factor XI coagulant activity less than 8%). "
4.	Factor VII (Proconvertin)IBA 09/05/2018 - "A new coagulopathy workup revealed prolonged INR to 1.5, corrected prothrombin time mixing study, and a low factor VII level (29%), suggesting acquired factor VII deficiency. " 07/01/1980 - "It seemed appropriate to continue the study in order to evaluate the factor VII capacity of inhibiting an specific antibody, to confirm heterozygosity of partents and to prove that this case is a genetic variant of factor VII deficiency with good prognosis." 08/01/1964 - "A CLINICAL AND FAMILY STUDY OF HEREDITARY PROCONVERTIN (FACTOR VII) DEFICIENCY." 08/01/1958 - "[Congenital factor VII deficiency; family investigation and physiological studies on factor VII]." 11/20/2023 - "Further examinations of her coagulopathy revealed that she had low factor VII activity, suggesting a diagnosis of factor VII deficiency. "
5.	Factor V (Coagulation Factor V)IBA 04/01/1999 - "A patient with combined factor V and factor VII deficiency is described together with a family study. " 06/01/2006 - "First molecular characterization of a patient with combined factor V and factor VII deficiency." 04/01/1999 - "Other family members were found to have isolated factor V or factor VII deficiency. " 04/01/1999 - "The propositus appeared to be double heterozygous for factor V and factor VII deficiency. " 04/01/1999 - "Combined factor V and factor VII deficiency due to an independent segregation of the two defects."
6.	Hemostatics (Antihemorrhagics)IBA 12/01/2022 - "Reproductive health and hemostatic issues in women and girls with congenital factor VII deficiency: A systematic review." 05/01/1982 - "Managing hemostatic control during surgery in patients with severe factor VII deficiency is relatively easy." 01/01/2008 - "It is approved in many countries for use as an intravenous hemostatic agent in patients with congenital hemophilia with inhibitors, and also for acquired hemophilia, factor VII deficiency, and Glanzmann thrombasthenia in some countries. " 01/01/2008 - "Studies have shown it to be effective and generally well tolerated when used intravenously to treat bleeding episodes or provide hemostatic cover during surgery in patients with congenital hemophilia with inhibitors, acquired hemophilia, factor VII deficiency or Glanzmann thrombasthenia. " 01/01/2018 - "To review safety, efficacy, and dosing data regarding CI of rFVIIa for hemostatic management of patients with congenital hemophilia with inhibitors (CHwI) or congenital factor VII deficiency (C7D). "
7.	Prothrombin (Factor II)IBA 05/01/1985 - "Multiple extractions were carried out in a patient with factor VII deficiency treated with prothrombin complex concentrate. " 11/01/1975 - "All congenital coagulation disorders of the prothrombin complex yielded a prolonged K-test value but for Factor VII deficiency. " 10/21/1972 - "Congenital factor VII deficiency with cerebral haemorrhage treated with prothrombin concentrate." 01/01/2016 - "Continuous infusion of human prothrombin complex in a patient with congenital factor VII deficiency undergoing laparoscopic cholecystectomy: A case report from China." 01/01/1958 - "[Study of prothrombin conversion in a case of congenital proconvertin deficiency (factor VII deficiency of serum prothrombin conversion accelerator deficiency]."
8.	von Willebrand FactorIBA 05/01/2012 - "One patient had low levels of von Willebrand factor, and one patient had mild factor VII deficiency. " 02/01/2012 - "Low von Willebrand factor (LVWF), unspecific platelet function disorders (UPFD) and mild factor VII deficiency were considered as mild bleeding disorders. " 11/01/2019 - "All patients born before 1990 with haemophilia, von Willebrand factor Disease, factor V deficiency, factor VII deficiency or afibrinogenemia were included in this study. "
9.	Factor VII PaduaIBA 09/01/2011 - "Activated FVII levels in factor VII Padua (Arg304Gln) coagulation disorder and in true factor VII deficiency: a study in homozygotes and heterozygotes." 01/01/2010 - "Factor VII Padua is a variant form of factor VII deficiency characterized by a prolongation of the prothrombin time (PT), when the assay is performed using rabbit brain thromboplastin. "
10.	PhosphofructokinasesIBA 01/01/2017 - "Therefore, the objective of this study was to screen a large mixed-breed canine population for the presence of mutant alleles associated with five autosomal recessive disorders: hyperuricosuria and hyperuricemia (HUU), cystinuria (CYST), factor VII deficiency (FVIID), myotonia congenita (MYC) and phosphofructokinase deficiency (PKFD). "

Therapies and Procedures

1.	Induction Chemotherapy 01/01/2019 - "The factor VII deficiency cleared after induction chemotherapy and with the disappearance of the cytogenetic and molecular abnormalities. "
2.	Therapeutics 03/28/2017 - "Factor VII Deficiency: Clinical Phenotype, Genotype and Therapy." 12/01/2016 - "Anticoagulation Therapy Considerations in Factor VII Deficiency." 11/01/2015 - "Replacement therapy in inherited factor VII deficiency: occurrence of adverse events and relation with surgery." 01/01/2000 - "Prophylactic therapy in a pregnant woman with severe factor VII deficiency." 06/01/1990 - "Pre and postoperative replacement therapy is essential to the patient with congenital factor VII deficiency for the safety of operation."
3.	Liver Transplantation 09/01/2016 - "Inhibitor development after liver transplantation in congenital factor VII deficiency." 09/01/2015 - "First living-related liver transplant to cure factor VII deficiency." 10/01/2014 - "Liver transplant for congenital factor VII deficiency." 05/01/2010 - "We report a case of mixed-type AIHA that developed in a child after hepatocyte and living-related orthotopic liver transplantation for factor VII deficiency." 12/15/2001 - "We report two children, sisters, both with severe, recurrent hemorrhagic complications from factor VII deficiency, successfully treated with orthotopic liver transplantation. "
4.	Coronary Artery Bypass (Coronary Artery Bypass Surgery) 04/01/2023 - "We present a case of a man with inherited severe factor VII deficiency who underwent successful coronary artery bypass grafting." 04/01/2023 - "Coronary Artery Bypass Grafting in a Patient With Severe Factor VII Deficiency." 04/01/2021 - "Aortic valve and coronary artery bypass surgery in a patient with factor VII deficiency." 07/01/2013 - "Triple coronary artery bypass graft surgery in a patient with factor VII deficiency: a case report." 01/01/2019 - "Successful coronary artery bypass graft surgery in severe congenital factor VII deficiency: Perioperative treatment with pd-factor VII concentrate."
5.	Anesthesia 07/01/1994 - "[Anesthesia in congenital factor VII deficiency]." 01/01/1990 - "[Anesthesia and factor VII deficiency]." 01/01/1990 - "[Anesthesia and factor VII deficiency]." 09/01/1988 - "[Anesthesia in a patient with hereditary factor VII deficiency]." 06/01/2009 - "A retrospective review was carried out on the methods of obstetric analgesia/anesthesia used in 80 pregnancies amongst 63 women with inherited bleeding disorders (19 factor XI deficiency, 16 carriers of haemophilia, 15 von Willebrand disease, seven platelet function disorders, four factor VII deficiency, one factor VII and XI deficiency and one factor X deficiency). "