Pediatric Factor VII Deficiency. About Medscape Reference Medscape's clinical reference is the most authoritative and accessible point-of-care medical reference for physicians and healthcare professionals, available online and via all major mobile devices. All content is free. The clinical information represents the expertise and practical knowledge of top physicians and pharmacists from leading academic medical centers in the United States and worldwide. The topics provided are comprehensive and span more than 30 medical specialties, covering: Diseases and Conditions More than 6000 evidence-based and physician-reviewed disease and condition articles are organized to rapidly and comprehensively answer clinical questions and to provide in-depth information in support of diagnosis, treatment, and other clinical decision-making.
Procedures Anatomy More than 100 anatomy articles feature clinical images and diagrams of the human body's major systems and organs. Drug Monographs Drug Interaction Checker Formulary Information. Congenital factor VII deficiency Hypoproconvertinemia. Factor VII (FVII) deficiency is a rare hereditary hemorrhagic disease caused by the diminution or absence of this coagulation factor.
Prevalence appears to be close to 1/300,000. The clinical expression of this disorder is highly variable, and no consistent relationship has been found between the severity of the hemorrhagic syndrome and the residual levels of FVII activity. The clinical picture can be very severe, with the early occurrence of intracerebral hemorrhages or repeated hemarthroses, or, in contrast, moderate with cutaneous-mucosal hemorrhages (epistaxis, menorrhagia) or hemorrhages provoked by a surgical intervention.
Finally, numerous subjects are completely asymptomatic despite a very low FVII level. The disease is transmitted in an autosomal recessive manner and is caused by mutations in the F7 gene (13q34) coding for FVII. Only homozygotes or compound heterozygotes develop a hemorrhagic syndrome; heterozygotes are asymptomatic. Expert reviewer(s) F7 coagulation factor VII (serum prothrombin conversion accelerator) [Homo sapiens] - Gene - NCBI. Entrez Genome view.
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Tel: 01223 348 866 See also: > Non routine tests > DNA extraction and sequencing service > Sample requirements and test request card. Entry - #227500 - FACTOR VII DEFICIENCY. Factor VII deficiency.