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Thromb Haemost 1974; 32(02/03): 564-577
DOI: 10.1055/s-0038-1647725
DOI: 10.1055/s-0038-1647725
Original Article
Fibrinogen Valencia A New Case of Congenital Dysfibrinogenemia
Weitere Informationen
Publikationsverlauf
Received
30. Juli 1974
Accepted
12. September 1974
Publikationsdatum:
30. Juni 2018 (online)
Summary
A new case of dysfibrinogenemia is described, the proband had low fibrinogen level and a moderate hyperfibrinolysis. Attention is drawn to the florid hemorrhagic picture and its early presentation.
The family history showed a moderate hyperfibrinolysis.
It appeared that the cause of the dysfibrinogenemia was due to a deficient polymerization of the α chains of the fibrinogen.
It is not yet known if this is caused by a defective liberation of fibrinopeptides. Due to the peculiar characteristic of this case and in accordance with the international norms, we propose to call this dysfibrinogenemia “Fibrinogen Valencia.”
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References
- 1 Aguercif M, Anner R, Ritschared J, Nydegger U, Soria J, Bouvier C. A. 1972; Syndromes de dysfibrinogénémies congenitales et familiales. Pédiatrie 27: 317.
- 2 Aznar J, Rego J. AFernandez, Pla M. AFernandez, Aznar J. A. 1970; Congenital hypofibrinogenemia in three members of a family. Coagulation 03: 279.
- 3 Aznar J, Tascon A, Aznar J. A. Rodriguez-Ineba 1973; Técnica räpida para la valoraciön del fibrinogen o por el método de precipitaciön por calor. Revista de Diagnöstico Biolögico 22: 373.
- 4 Beck E. A. 1970; Diagnosis of structural abnormalities of human fibrinogen. Thrombosis et Diathesis Haemorrhagica, Supplement 39: 323.
- 5 Brummel M. C, Montgomery R. 1970; Acrylamide gel electrophoresis of the s-sulfo derivatives of fibrinogen. Analytical Biochemistry 33: 28.
- 6 Caen J, Larrieu M. J, Samama M. 1968 L’hémostase. Méthode d’exploration et diagnostic practique. Expansion Scientifique. Editorial Paris..
- 7 Cohn E. 1946; Preparation and properties of serum and plasma proteins. Journal of the American Chemical Society 68: 459.
- 8 Egeberg O. 1967; Inherited abnormality causing thrombophilia. Thrombosis et Diathesis Haemorrhagica 17: 176.
- 9 Fante P. 1969; Hypofibrinogenemia with a possible defective fibrinogen associated with fibrinolysis. Australasian Annals of Medicine 18: 43.
- 10 Goa J. 1953; A micro biuret method for protein determination of total protein. The Scandinavian Journal of Clinical Laboratory Investigation 05: 218.
- 11 Imperato DI C, eDettori A. G. 1958; Ipofibrinogenemia congenita con fibrinoastenia. Helvetica Paediatrica Acta 14: 380.
- 12 Jacobsen C. D, Hoak J. D. 1973; Fibrinogen Iowa City: an abnormal fibrinogen with no clinical symptoms. Thrombosis Research 02: 261.
- 13 Jackson D. P, Beck E. A. 1970. Inherited Abnormal fibrinogens. In: Brinkhous K. M. (ed.) Hemophilia and New Hemorrhagic States. University of North Carolina Press; Chapel Hill: 225.
- 14 Janssen C. L, Vreeken J. 1971; Fibrinogen Amsterdam, another hereditary abnormality of fibrinogen. British Journal of Haematology 20: 3.
- 15 Krause W. H, Heene D. L, Lasch H. G. 1973 Congenital dysfibrinogenemia (fibrinogen Giessen) Thrombosis et Diathesis Haemorrhagica. 29. 547.
- 16 Laki K. 1951; The polymerization of proteins: the action of thrombin in fibrinogen. Archives of Biochemistry and Biophysics 32: 317.
- 17 Mammen E. F, Prasad A. S, Barnhart M. I, Au C. C. 1969. Congenital dysfibrinogenemia: ‘‘Fibrinogen Detroit.”. Journal of Clinical Investigation. 48 235.
- 18 Martinez J, Holburn R. R, Shapiro S. S, Ersley A. J. 1972; Hereditary hypodysfibrinogenemia characterized by fibrinogen hypercatabolism. The Journal of Clinical Investigation. Abstract 51: 62a.
- 19 McKee P. A, Mattock P, Hill P. L. 1970; Subunit structure of human fibrinogen soluble fibrin and cross-linked insoluble fibrin. Proceedings of the National Academy of Sciences U.S.A 66: 738.
- 20 Menache D. 1973; Abnormal fibrinogens. A review. Thrombosis et Diathesis Haemorrhagica 29: 525.
- 21 Merskey C, Lalezari P, Johnson A. J. 1969; A rapid simple method for measuring fibrinogen split products in human serum. Proceedings of the Society for Experimental Biology and Medicine 131: 871.
- 22 Mihalyi E. 1968; Physicochemical studies of bovine fibrinogen. Biochemistry 07: 208.
- 23 Ouchterlony O. 1958; Diffusion-in-gel method for immunological analysis. Progress in Allergy 05: 1.
- 24 Rodbard D, Reynolds J. A, Tanford C. 1970; The gross conformation of protein-sodium dodecyl sulfate complexes. The Journal of Biological Chemistry 245: 5161.
- 25 Soria J, Soria C, Boulard C. H. 1972; Anomalie de structure du fibrinogéne Metz localisée sur la chaîne (a) de la molécule. Biochimica et Biophysica Acta 54: 415.
- 26 Soria J, Soria C, Samama M, Poirot E, Kling C. 1972; “Fibrinogen Troyes,” “Fibrinogen Metz.” Two new cases of congenital dysfibrinogenemia. Thrombosis et Diathesis Haemorrhagica 27: 619.
- 27 Thaler E, Lechner K, Deutsch E. 1973. Fibrinogen Wien. Verhandlungen der Deutschen Arbeitsgemeinschaft für Blutgerinnungsforschung. 14. Tagung in Münster 15-17. Februar. F. K. Schattauer Verlag; Stuttgart: (in Press).
- 28 Verstraete M. 1970; In a discussion. Thrombosis et Diathesis Haemorrhagica, Supplement 34: 334.
- 29 Weber K, Osborn N. 1969; The reliability of molecular weight determinations by dodecyl sulfate-polyacrylamide gel electrophoresis. The Journal of Biological Chemistry 244: 4406.