Congenital Hypoproconvertinemia A Report on 12 Cases with Total Deficiency and 19 Cases with Partial Deficiency

Summary

Investigations on 12 patients with a total and 19 patients with a partial deficiency of proconvertin (factor VII) are reported. All patients proved to have the same deficiency and it was distinguishable from Stuart-Prower factor deficiency. The differentiation between proconvertin deficiency and Stuart-Prower factor deficiency is discussed.

^*) Present adress: II. med. Universitätsklinik, Hamburg.

• References

• 1 Aas K. Prokonvertin og konvertin. Undersokelser over blodets koagulasjon med spesielt henblikk på Prokonvertin og konvertin. Thesis. Oslo, Akad; Trykningssentral: 1952: 90 pp
• 2 Ackroyd J. F. The function of factor VII. Brit. J. Haematol 1956; 2: 597
• 3 Alexander B, Goldstein R, Landwehr G, Cook C. D. Congenital SPCA deficiency: a hitherto unrecognized coagulation defect with hemorrhage rectified by serum and serum fractions. J. clin. Invest 1951; 30: 596
• 4 Bachmann F, Duckert F, Flückiger P, Hitzig W, Koller F. Über einen neuartigen kongenitalen Gerinnungsdefekt (Mangel an Stuart-Faktor). Thromb. Diath. haem 1957; a 1: 87
• 5 Bachmann F, Duckert F, Geiger M, Baer P, Koller F. Differentiation of the factor VII complex. Studies on the Stuart-Prower factor. Thromb. Diath. haem 1957; 1: 167
• 6 Benthaus J. Über den Einfluß der Gerinnungsfaktoren auf die Retraktion. Verh. d. Dtsch. Ges. f. Inn. Med. 63. Kongreß. 1957
• 7 Bergsagel D. E. Stages in the formation of blood thromboplastin. Thesis. Oxford, Univ. Coll: Oxford; 1955: 203 typed p.p.
• 8 Biggs R, Douglas A. S. The thromboplastin generation test. J. clin. Path 1953; 6: 23
• 9 Borchgrevink C. F, Waaler B. A. The secondary bleeding time. A new method for the differentiation of hemorrhagic diseases. Acta med. scand. (In press).
• 10 Burmeister A. Zur Differentialdiagnose des angeborenen Faktor-VII-Mangels. Z. Kinderheilk 1958; 81: 88
• 11 Dann H. A, Fisher H.W, Burnett L, Briggs D. Congenital serum prothrombin conversion accelerator (SPCA) deficiency. Ann. intern. Med 1958; 49: 459
• 12 Graham J. B, Hougie C. The Stuart factor (hypoproconvertinemia?). The clotting defect and mode of inheritance. 6th Congr. Int. Soc. Hemat: Boston; 1956: p. 327
• 12a Hellem A. Demonstration of a factor in blood influencing the adhesivity of platelets to foreign surfaces. 8th. Congr. Int. Soc. Hemat. Rome, Official program N 1958; 313: p. 254
• 13 Hicks N. D. A coagulation disorder due to a factor VII-like defect. Med. J. Aust 1955; 42: 331
• 14 Hjort P. F. Intermediate reactions in the coagulation of blood with tissue thromboplastin. Convertin, accelerin, prothrombinase. Thesis. Scand. J. clin. Lab. Invest 1957; 9 suppl. (Suppl. 27) 183 pp.
• 15 Hjort P. F, Rapaport S. I, Owren P. A. A simple, specific one-stage prothrombin assay using Russel’s viper venom in cephalin suspension. J. Lab. clin. Med 1955; 46: 89
• 16 Hjort P. F, Stormorken H. A study of the in vitro and in vivo effects of a synthetic heparin-like anticoagulant: dextran sulphate. Scand. J. clin. Lab. Inves 1957; 9 suppl. (Suppl. 29) 86 pp.
• 17 Hougie C. Effect of Russell’s viper venom (Stypven) on Stuart clotting defect. Proc. Soc. exp. Biol. (N.Y) 1956; 93: 570
• 18 Hougie C, Barrow E. M, Graham J. B. Stuart clotting defect. I. Segregation of an hereditary hemorrhagic state from the heterogeneous group heretofore called "stable factor" (SPCA, proconvertin, factor VII) deficiency. J. clin. Invest 1957; 36: 485
• 19 Lee R. I, White P. D. A clinical study of the coagulation time of blood. Amer. J. med. Sci 1913; 145: 495
• 20 Lewis J. H, Fresh J. W, Ferguson J. H. Congenital hypoproconvertin-emia. Proc. Soc. exp. Biol. (N.Y) 1953; 84: 651
• 21 Owren P. A, Aas K. The control of Dicumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. clin. Lab. Invest 1951; 3: 201
• 22 Stapp W. F. A one-stage method for the assay of antihemophilic factor B (AHF-B). , with a comment on the antihemophilic factor B (AHF-B) concentration in phenylin-danedione (PID) therapy. Scand. J. clin. Lab. Invest 1958; 10: 169
• 23 Telfer T. P, Denson K. W, Wright D. R. A "new" coagulation defect. Brit. J. Haematol 1956; 2: 308
• 24 Waaler B. A. Simultaneous contribution to the formation of thrombin by the intrinsic and extrinsic blood clotting systems. Scand. J. clin. Lab. Invest 1957; 8: 322