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• W2005164612 endingPage "91" @default.
• W2005164612 startingPage "71" @default.
• W2005164612 abstract "The evidence for the existence and function of blood coagulation factor VII (stable factor, SPCA, proconvertin) is critically reviewed. As is true of factors V and X, deficiency of factor vii lengthens the prothrombin time. This is usually the first clue to the diagnosis in a patient with a bleeding tendency. The concept that factors V and X act intrinsically (coagulation in a blood vessel or a test tube in the absence of tissue juice) and that factor VII acts extrinsically (after extravasation and, therefore, in contact with tissue juice) requires re-evaluation. In any case, the type of bleeding associated with a deficiency of factor VII is indistinguishable from that related to lack of factors V and X or of the hemophilic factors VIII and IX. All available publications on factor VII deficiency, as well as earlier ones describing+ “hypoprothrombinemia,” have been analyzed and categorized according to the likelihood of their representing true deficiencies of factor VII. Only thirty-six cases met our criteria. We have added three cases in this report. These three patients had noted lifelong bleeding tendencies. An eleven year old boy had experienced recurrent hemarthroses of the right knee; a thirteen year old girl had hemorrhaged severely with each menstrual period; a fortyeight year old man had bled after surgical procedures such as dental extractions and hemorrhoidectomy. Study of more than 100 relatives of the boy, both of whose parents were of Norwegian descent, indicated that the heterozygous (carrier) state is associated with a plasma concentration of factor VII of about 50 per cent of normal, and that the heterozygote is equally likely to be male or female. The heterozygote apparently represents two codominant alleles, one normal for the synthesis of factor VII, the other not leading to the production of factor VII. Neither of the terms “dominant” or “recessive” appears to apply to the abnormal allele, except in a clinical sense, since the heterozygotes are not bleeders. One relative, pregnant for five months, had a plasma concentration of factor VII three times normal; she was probably homozygously normal. The homozygous deficiency of factor VII is characterized by a severe bleeding diathesis that resists vitamin K therapy and has so far not been treated effectively by any agent except blood, plasma or plasma fractions. Even this treatment is beneficial for only a limited period. Whole blood was transfused into the adult patient, and 500 ml. of plasma was given to the girl. Within 24 hours, the plasma concentration of factor VII was negligible in each case." @default.
• W2005164612 created "2016-06-24" @default.
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• W2005164612 date "1964-07-01" @default.
• W2005164612 modified "2023-09-27" @default.
• W2005164612 title "Congenital deficiency of factor VII (hypoconvertinemia)" @default.
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