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:

Networked: 236 relevant articles (4 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Intracranial Hemorrhages (Intracranial Hemorrhage)
2.	Hemorrhage
3.	Hemophilia A (Haemophilia)
4.	Thrombasthenia (Glanzmann Thrombasthenia)
5.	Factor XI Deficiency (Hemophilia C)

Experts

1.	Karimi, M: 4 articles (11/2015 - 07/2008)
2.	Mariani, G: 4 articles (03/2014 - 06/2001)
3.	Ingerslev, J: 4 articles (03/2014 - 04/2005)
4.	Mariani, Guglielmo: 4 articles (04/2013 - 02/2002)
5.	Dolce, Alberto: 3 articles (04/2013 - 03/2005)
6.	Giansily-Blaizot, M: 3 articles (05/2012 - 02/2007)
7.	Dhawan, Anil: 3 articles (05/2010 - 12/2004)
8.	Bernardi, Francesco: 3 articles (03/2008 - 02/2002)
9.	Dolce, A: 2 articles (03/2014 - 05/2012)
10.	Sørensen, B: 2 articles (03/2014 - 04/2005)

Drugs and Biologics

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

1.	recombinant FVIIa (rFVIIa)IBA 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. " 01/01/2014 - "Successful use of rFVIIa for major breast surgery prophylaxis in congenital factor VII deficiency: a case report." 01/01/2014 - "Successful use of rFVIIa for major breast surgery prophylaxis in congenital factor VII deficiency."
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 VII (Proconvertin)IBA 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]." 03/01/2014 - "Inhibitors to factor VII in congenital factor VII deficiency." 09/01/2013 - "A novel homozygous missense mutation in the factor VII gene of severe factor VII deficiency in a pedigree: a description of two cases."
4.	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."
5.	Prothrombin (Factor II)IBA 05/01/1985 - "Multiple extractions were carried out in a patient with factor VII deficiency treated with prothrombin complex concentrate. " 10/21/1972 - "Congenital factor VII deficiency with cerebral haemorrhage treated with prothrombin concentrate." 01/01/1958 - "[Study of prothrombin conversion in a case of congenital proconvertin deficiency (factor VII deficiency of serum prothrombin conversion accelerator deficiency]." 09/01/2015 - "Prothrombin complex concentrate for factor VII replacement in a patient undergoing left ventricular assist device implantation with factor VII deficiency." 01/01/2006 - "When compared with Caucasian data, our data showed a significantly lower prevalence of protein C, total protein S, antithorombin III, factor II, and factor VII deficiencies. "
6.	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. "
7.	Retinaldehyde (Retinal)IBA 10/01/2000 - "Factor VII deficiency in a patient with retinal arteriolar tortuosity syndrome." 10/01/2000 - "Coagulation studies in the son revealed normal activated partial thromboplastin time (APTT), prolonged prothrombin time (PT) and 30% activity of factor VII. Factor VII deficiency may aggravate the haemorrhages in retinal arteriolar tortuosity syndrome. "
8.	Thromboplastin (Tissue Factor)IBA 01/01/2007 - "The importance of tissue factor source in the management of Factor VII deficiency." 06/15/1994 - "Severe factor VII deficiency caused by mutations abolishing the cleavage site for activation and altering binding to tissue factor." 04/01/1993 - "The importance of this protein to normal haemostasis may be inferred from the clinical effects of factor VII deficiency and by the fact that congenital tissue factor deficiency has not been described. " 06/01/1978 - "Prolonged thromboplastin time but normal partial thromboplastin time in coagulation analysis lead to the diagnosis of factor VII-deficiency. " 01/01/1977 - "Several patients showed a slight protongation of ox brain thromboplastin time due to an associated slight factor VII deficiency. "
9.	AntigensIBA 05/01/1977 - "A CRM-Positive variant of factor-VII deficiency and the detection of heterozygotes with the assay of factor-like antigen." 02/01/2000 - "A severe factor VII deficiency was identified in a 45-year old male whose plasma factor VII antigen was less than 60 ng/ml and expressed 5.2% of normal factor VII activity. " 04/01/1991 - "The level of factor VII antigen in 4 patients with congenital factor VII deficiency and their family members was investigated through this ELISA and the results correlated well with those obtained by electroimmunoassay previously described. " 01/01/1987 - "An 11-year-old girl with hereditary factor-VII deficiency and her family have been studied for factor VII coagulation activity (VII:C) and factor VII antigen level (VII:Ag). " 06/15/1986 - "Of 4 patients with congenital factor VII deficiency, 3 patients had no detectable factor VII antigen, one patient had reduced factor VII antigen."
10.	Factor IX (PTC)FDA LinkGeneric 07/01/1956 - "Combined mild PTC (plasma thromboplastin component) and factor VII deficiencies." 03/01/1988 - "The initial increase in FPA content occurred normally in patients with deficiencies of either factor IX or VIII, was markedly diminished in patients with factor X or V deficiency, and was delayed in patients with factor VII deficiency. " 01/01/2000 - "Recently, recombinant factor VIIa concentrate has become available to treat hemophilia patients with inhibitor and factor VII deficiency patients in Europe and also recombinant factor IX for the treatment of hemophilia B has been licensed in the United States and Europe. " 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." 01/01/1996 - "The administration of 10 mu g/kg body weight of recombinant factor VIIa to patients with factor VII deficiency increased the circulating levels 35-fold, but this only resulted in normalization of the activation of factor IX and factor X. Our data indicate that factor IXa is primarily responsible for the basal levels of free factor VIIa in vivo, and that changes in free factor VIIa in the blood do not necessarily lead to alterations in factor X activation."

Therapies and Procedures

1.	Transplants (Transplant) 09/01/2015 - "First living-related liver transplant to cure factor VII deficiency." 10/01/2014 - "Liver transplant for congenital factor VII deficiency." 03/01/1989 - "The immunosuppressive therapy and subsequent lymphohaematopoietic engraftment following allogeneic marrow transplant was accompanied by complete resolution of the factor VII deficiency." 07/01/2013 - "Triple coronary artery bypass graft surgery in a patient with factor VII deficiency: a case report." 10/01/2007 - "Successful coronary artery bypass graft surgery in severe congenital factor VII deficiency: perioperative treatment with factor VII concentrate."
2.	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). "
3.	Liver Transplantation 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. " 12/15/2001 - "Liver transplantation for factor VII deficiency." 01/01/1999 - "Transmission of factor VII deficiency through liver transplantation." 09/01/2011 - "3 ANESTHETIC MANAGEMENT OF LIVER TRANSPLANTATION FOR FACTOR VII DEFICIENCY IN A 6-MONTH-OLD CHILD-THROMBOELASTOGRAPHY-GUIDED INTRA-OPERATIVE REPLACEMENT OF RECOMBINANT FACTOR VIIa."
4.	Extracorporeal Circulation 01/01/2002 - "[The influence of factor VII deficiency on cardiac operations with extracorporeal circulation]." 09/01/1999 - "Substitution therapy of congenital factor VII deficiency at an extended operation with extracorporeal circulation." 01/01/1993 - "We describe here successful open heart surgery with extracorporeal circulation and haemodilution in a child with severe congenital factor VII deficiency. " 01/01/1993 - "Cardiac surgery with extracorporeal circulation in severe factor VII deficiency." 01/01/1982 - "[Severe hypoproconvertinemia and extracorporeal circulation for mitral valve replacement]."
5.	Transplantation (Transplant Recipients) 12/27/2004 - "Hepatocyte transplantation for inherited factor VII deficiency." 03/01/2002 - "Acquired factor VII deficiency in hematopoietic stem cell transplant recipients." 03/01/1989 - "The factor VII deficiency persisted from clinical presentation until approximately 50 d after allogeneic marrow transplantation when restoration of factor VII activity and antigen was noted. "