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Hereditary Factor VII Deficiency
rare autosomal recessive inherited blood ...
semanticscholar +1 more source
Inhibitors to factor
We performed a prospective study of FVII inhibitor occurrence in a large number of patients with FVII deficiency who had received replacement therapy for spontaneous or traumatic bleeding episodes, major or minor surgical interventions or prophylaxis.Inhibitor development was detected in 2.6% (3/115) of patients, but the incidence of de novo inhibitors
Batorova A+11 more
openaire +3 more sources
As yet, there have been neither systematic reviews nor reports of randomized, controlled trials involving factor VII (FVII) deficiency. Hence, a picture of this disorder can only be drawn by reviewing and summarizing the data that is available. This article provides an overview of the understanding of this rare, inherited disorder of coagulation.
openaire +5 more sources
Clinical problems and surgical interventions in inherited factor VII deficiency
Aim: Factor VII deficiency is one of the hereditary coagulation disorders that has autosomal reccessive inheritance and is observed relatively frequently (1/500 000).
B. Şenol, B. Zülfikar
semanticscholar +1 more source
Immune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type I. [PDF]
BackgroundCardiovascular disease, a progressive manifestation of α-L-iduronidase deficiency or mucopolysaccharidosis type I, continues in patients both untreated and treated with hematopoietic stem cell transplantation or intravenous enzyme replacement ...
Dickson, Patricia I+7 more
core +6 more sources
Inhibitors in Patients with Congenital Bleeding Disorders Other Than Hemophilia [PDF]
The most worrying complication of replacement therapy for severe hemophilia A and B is currently the occurrence of inhibitory alloantibodies against infused factor VIII and factor IX, respectively.
Franchini, Massimo+6 more
core +1 more source
Congenital factor VII deficiency presenting first time as isolated recurrent hematuria at late age
Hematological conditions rarely present as isolated hematuria. Factor VII deficiency is a rare congenital coagulopathy inherited as autosomal recessive pattern. It usually presents a severe life-threatening bleeding at an early age.
Suman Sethi+5 more
doaj +1 more source
Factor VII (FVII) deficiency is the most common among the rare bleeding disorders, which is caused by mutations in coagulation factor VII. Clinical features caused by FVII deficiency vary from mild or asymptomatic to fatal cerebral hemorrhage.
Do-Hun Kim+3 more
doaj +1 more source
Dental extraction in congenital factor Vll deficiency with inhibitor – a case report
Hereditary factor VII (FVII) deficiency is a rare bleeding disorder with autosomal recessive inheritance, and FVII deficiency with an inhibitor is extremely rare.
Nayar Geeta+3 more
doaj +1 more source
Perinatal Gene Transfer to the Liver [PDF]
The liver acts as a host to many functions hence raising the possibility that any one may be compromised by a single gene defect. Inherited or de novo mutations in these genes may result in relatively mild diseases or be so devastating that death within
Buckley, SM+6 more
core +1 more source