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Thromb Haemost 1959; 03(01): 059-076
DOI: 10.1055/s-0038-1654370
DOI: 10.1055/s-0038-1654370
Originalarbeiten — Original Articles — Travaux Originaux
A “New” Family with Stuart-Prower Deficiency
Further Information
Publication History
Publication Date:
08 June 2018 (online)
Summary
A description is given of the coagulation disturbances in six patients with a haemorrhagic diathesis due to Stuart-Prower deficiency. Relatives of these patients, if heterozygous for this deficiency, showed no significant haemorrhagic diathesis; in the laboratory, their one-stage “prothrombin” times showed only a slight prolongation. Thromboplastin formation in these relatives was sufficient, but determination of the Stuart-Prower factor revealed lower values. The relatives in question were incapable of giving the same correction of the thromboplastin generation test in their bleeder relatives as normal serum does.
The relation between Stuart-Prower deficiency and Christmas factor is discussed.
One patient is described who combined heterozygotism for Stuart-Prower deficiency with alcaptonuria.
The mode of inheritance of the Stuart-Prower deficiency is discussed.
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References
- 1 Alexander B, Goldstein R, Landwehr G, Cook C. D. Congenital SPCA-deficiency. J. clin. Invest 1951; 30: 596
- 2 Bachmann F, Duckert F, Geiger M, Baer P, Koller F. Differentiation of the Factor VII Complex. Thromb. Diath. haem 1957; 1: 169
- 3 Bell W. N, Alton H. G. A brain extract as a substitute for platelet suspensions in the thromboplastin generation test. Nature (Lond.) 1954; 174: 880
- 4 Belle C. J. Van: Parahaemophilie. Thesis Utrecht: 1952
- 5 Biggs R, Douglas A. S. The thromboplastin generation test. J. clin. Path 1953; 6: 23
- 6 Bounameaux Y. Dosage du facteur VIII en un temps. Acta haemat. (Basel) 1957; 17: 355
- 7 Bunim J. J. Alcaptonuria. Ann. intern. Med 1957; 47: 1210
- 8 Clauss A. Gerinnungsphysiologische Schnellmethode zur Bestimmung des Fibrinogens. Acta haemat. (Basel) 1957; 17: 237
- 9 Geiger M, Duckert F, Koller F. Quantitative Bestimmungen von Faktor VIII und IX bei Blutersippen. 5th Congr Eur. Soc. Haemat Freiburg: 1955: 413
- 10 Gonyea L. M, Krivit W. Congenital coagulation deficiency of Stuart factor activity. J. Lab. clin. Med 1958; 51: 398
- 11 Greep C. to be published.
- 12 Hjort P, Rapaport S. I, Owren P. A. A simple specific onestage prothrombin assay using Russell’s viper venom in cephalin suspension. J. Lab. clin. Med 1955; 46: 89
- 13 Hörder M. H. Kongenitaler familiärer Faktor-VII-Mangel mit zusätzlichem Defekt in der Thromboplastinbildung. Acta haemat. (Basel) 1958; 19: 30
- 14 Hougie C, Barrow E, Graham J. B. Stuart clotting defect. J. clin. Invest 1957; 36: 485
- 15 Koller F. Is hemophilia a nosologic entity?. Blood 1954; 9: 286
- 16 Lee R. I, White P. D. A clinical study of the coagulation time of blood. Amer. J. med. Sci 1913; 145: 495
- 17 Merskey C. The consumption of prothrombin during coagulation. J. clin. Path 1950; 3: 130
- 18 Owren P. A, Aas K. The control of dicoumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. clin. Lab. Invest 1951; 3: 201
- 19 Quick A. J. The physiology and pathology of hemostasis. London: 1951
- 20 Roos J, Huizinga J. To be published
- 21 Telfer T. P, Denson K. W, Wright D. R. A “new” coagulation defect. Brit. J. Haematol 1956; 2: 308
- 22 Warner E. D, Brinkhous K. M, Smith H. P. A quantitative study on blood clotting. Prothrombin fluctuations under experimental conditions. Amer. J. Physiol 1936; 114: 667
-
References
- 1 Alexander B, Goldstein R, Landwehr G, Cook C. D. Congenital SPCA-deficiency. J. clin. Invest 1951; 30: 596
- 2 Bachmann F, Duckert F, Geiger M, Baer P, Koller F. Differentiation of the Factor VII Complex. Thromb. Diath. haem 1957; 1: 169
- 3 Bell W. N, Alton H. G. A brain extract as a substitute for platelet suspensions in the thromboplastin generation test. Nature (Lond.) 1954; 174: 880
- 4 Belle C. J. Van: Parahaemophilie. Thesis Utrecht: 1952
- 5 Biggs R, Douglas A. S. The thromboplastin generation test. J. clin. Path 1953; 6: 23
- 6 Bounameaux Y. Dosage du facteur VIII en un temps. Acta haemat. (Basel) 1957; 17: 355
- 7 Bunim J. J. Alcaptonuria. Ann. intern. Med 1957; 47: 1210
- 8 Clauss A. Gerinnungsphysiologische Schnellmethode zur Bestimmung des Fibrinogens. Acta haemat. (Basel) 1957; 17: 237
- 9 Geiger M, Duckert F, Koller F. Quantitative Bestimmungen von Faktor VIII und IX bei Blutersippen. 5th Congr Eur. Soc. Haemat Freiburg: 1955: 413
- 10 Gonyea L. M, Krivit W. Congenital coagulation deficiency of Stuart factor activity. J. Lab. clin. Med 1958; 51: 398
- 11 Greep C. to be published.
- 12 Hjort P, Rapaport S. I, Owren P. A. A simple specific onestage prothrombin assay using Russell’s viper venom in cephalin suspension. J. Lab. clin. Med 1955; 46: 89
- 13 Hörder M. H. Kongenitaler familiärer Faktor-VII-Mangel mit zusätzlichem Defekt in der Thromboplastinbildung. Acta haemat. (Basel) 1958; 19: 30
- 14 Hougie C, Barrow E, Graham J. B. Stuart clotting defect. J. clin. Invest 1957; 36: 485
- 15 Koller F. Is hemophilia a nosologic entity?. Blood 1954; 9: 286
- 16 Lee R. I, White P. D. A clinical study of the coagulation time of blood. Amer. J. med. Sci 1913; 145: 495
- 17 Merskey C. The consumption of prothrombin during coagulation. J. clin. Path 1950; 3: 130
- 18 Owren P. A, Aas K. The control of dicoumarol therapy and the quantitative determination of prothrombin and proconvertin. Scand. J. clin. Lab. Invest 1951; 3: 201
- 19 Quick A. J. The physiology and pathology of hemostasis. London: 1951
- 20 Roos J, Huizinga J. To be published
- 21 Telfer T. P, Denson K. W, Wright D. R. A “new” coagulation defect. Brit. J. Haematol 1956; 2: 308
- 22 Warner E. D, Brinkhous K. M, Smith H. P. A quantitative study on blood clotting. Prothrombin fluctuations under experimental conditions. Amer. J. Physiol 1936; 114: 667