Congenital factor VII deficiency with normal Stuart activity: clinical, genetic and experimental observations.
نویسندگان
چکیده
s 1054
منابع مشابه
Preparation of factor VII concentrate using CNBr-activated
Background: Factor VII concentrates are used in patients with congenital or acquired factor VII deficiency or treatment of hemophilia patients with inhibitors. In this research, immunoaffinity chromatography was used to purify factor VII from prothrombin complex (Prothrombin-Proconvertin-Stuart Factor-Antihemophilic Factor B or PPSB) which contains coagulation factors II, VII, IX and X. The a...
متن کاملFactor VII Gene Defects: Review of Functional Studies and Their Clinical Implications
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 do...
متن کاملFunctional and Molecular Characterization of C91S Mutation in the Second Epidermal Growth Factor-like Domain of Factor VII
Background: Coagulation Factor VII is a vitamin K-dependent serine protease which has a pivotal role in the initiation of the coagulation cascade. The congenital Factor VII deficiency is a recessive hemorrhagic disorder that occurs due to mutations of F7 gene. In the present study C91S (p.C91S) substitution was detected in a patient with FVII deficiency. This mutation has not b...
متن کاملPREPARATION OF HIGHLY PURIFIED SOLVENTDETERGENT COA GULATION FACTOR VII AND FACTOR IX CONCENTRATES FROM PROTHROMBIN COMPLEX (PPSB)
In this study, anion-exchange chromatography was used to purify factor VII and factor IX from prothrombin complex (PPSB), which contains coagulation factors II, VII, IX and X. For this purpose, DEAE-Sepharose CL-6B gel , Pharmacia column XK-26 , high flow rate and two stepwise gradients with phosphate citrate buffer were used. The yield of the two lyophylized products, factor VII and factor...
متن کاملCongenital deficiency of factor VII in subarachnoid hemorrhage.
BACKGROUND Factor VII is essential for coagulation activation by the extrinsic pathway. Hemorrhages of the central nervous system in patients with congenital factor VII deficiency seem to have a higher incidence compared with other congenital coagulopathies. The purpose of this paper is to report two rare cases of subarachnoid hemorrhage and factor VII deficiency. CASE DESCRIPTION Two cases o...
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ورودعنوان ژورنال:
- Blood
دوره 15 شماره
صفحات -
تاریخ انتشار 1960