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Thromb Haemost 1959; 03(03): 375-385
DOI: 10.1055/s-0038-1654398
Originalarbeiten — Original Articles — Travaux Originaux
Schattauer GmbH

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

Dieter Voss   *)
1   Institute for Thrombosis Research, University Hospital, (Rikshospitalet), Oslo, Norway. (Head: Professor Paul A. Owren)
,
Bjarne A Waaler
1   Institute for Thrombosis Research, University Hospital, (Rikshospitalet), Oslo, Norway. (Head: Professor Paul A. Owren)
› Author Affiliations
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Publication History

Publication Date:
08 June 2018 (online)

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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.


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


  • References

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    Search in Google Scholar
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    CrossrefPubMedSearch in Google Scholar
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    PubMedSearch in Google Scholar
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    CrossrefPubMedSearch in Google Scholar
  • 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
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    CrossrefPubMedSearch in Google Scholar
  • 23 Telfer T. P, Denson K. W, Wright D. R. A "new" coagulation defect. Brit. J. Haematol 1956; 2: 308
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar

  • 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
    Search in Google Scholar
  • 2 Ackroyd J. F. The function of factor VII. Brit. J. Haematol 1956; 2: 597
    PubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 6 Benthaus J. Über den Einfluß der Gerinnungsfaktoren auf die Retraktion. Verh. d. Dtsch. Ges. f. Inn. Med. 63. Kongreß. 1957
    PubMedSearch in Google Scholar
  • 7 Bergsagel D. E. Stages in the formation of blood thromboplastin. Thesis. Oxford, Univ. Coll: Oxford; 1955: 203 typed p.p.
    Search in Google Scholar
  • 8 Biggs R, Douglas A. S. The thromboplastin generation test. J. clin. Path 1953; 6: 23
    CrossrefPubMedSearch in Google Scholar
  • 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).
    PubMed
  • 10 Burmeister A. Zur Differentialdiagnose des angeborenen Faktor-VII-Mangels. Z. Kinderheilk 1958; 81: 88
    CrossrefPubMedSearch in Google Scholar
  • 11 Dann H. A, Fisher H.W, Burnett L, Briggs D. Congenital serum prothrombin conversion accelerator (SPCA) deficiency. Ann. intern. Med 1958; 49: 459
    CrossrefPubMedSearch in Google Scholar
  • 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
    Search in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 13 Hicks N. D. A coagulation disorder due to a factor VII-like defect. Med. J. Aust 1955; 42: 331
    PubMedSearch in Google Scholar
  • 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.
    PubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar
  • 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.
    PubMedSearch in Google Scholar
  • 17 Hougie C. Effect of Russell’s viper venom (Stypven) on Stuart clotting defect. Proc. Soc. exp. Biol. (N.Y) 1956; 93: 570
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 19 Lee R. I, White P. D. A clinical study of the coagulation time of blood. Amer. J. med. Sci 1913; 145: 495
    CrossrefPubMedSearch in Google Scholar
  • 20 Lewis J. H, Fresh J. W, Ferguson J. H. Congenital hypoproconvertin-emia. Proc. Soc. exp. Biol. (N.Y) 1953; 84: 651
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 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
    CrossrefPubMedSearch in Google Scholar
  • 23 Telfer T. P, Denson K. W, Wright D. R. A "new" coagulation defect. Brit. J. Haematol 1956; 2: 308
    CrossrefPubMedSearch in Google Scholar
  • 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
    PubMedSearch in Google Scholar

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