Congenital factor X deficiency in Japan.

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  • MORI KAZUO
    The Third Department of Internal Medicine, Tohoku University School of Medicine
  • SAKAI HIDEAKI
    The Third Department of Internal Medicine, Tohoku University School of Medicine
  • NAKANO NOBORU
    The Third Department of Internal Medicine, Tohoku University School of Medicine
  • SUZUKI SOZO
    The Third Department of Internal Medicine, Tohoku University School of Medicine
  • SUGAI KOJI
    The Third Department of Internal Medicine, Tohoku University School of Medicine
  • HISA SHIZUE
    The Third Department of Internal Medicine, Tohoku University School of Medicine
  • GOTO YOSHIO
    The Third Department of Internal Medicine, Tohoku University School of Medicine

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Abstract

MORI, K., SAKAI, H., NAKANO, N., SUZUKI, S., SUGAI, K., HISA, S. and GOTO, Y. Congenital Factor X Defficiency in Japan. Tohoku J. exp. Med., 1981, 133 (1), 1-19 Congenital factor X deficiency is a very rare inheritedcoagulation abnormality. There have been reported 43 cases of this disorder in the world and only 2 cases in Japan. Recently, we have hemostatically and immunologically examined as many as 3 cases of this rare deasese, 18-year-old male, 11-year-old male and 6-year-old female. Hemostatic examinations revealed prolonged prothrombin time, partial thromboplastin time and decreased serum thromboplastic activity in these 3 cases. Stypven-cephalin clotting time was also abnormal. Factor X activities of our cases were low when assayed by either tissue thromboplastin and partial thromboplastin or by Stypven-cephalin mixture, which were 2.6, 1.5 and 4.5%, respectively. The half lives of infused factor X were 24, 38.6 and 56hr, respectiely, which are consistent with the data of other authors. Immunological assay of factor X (radioimmunoassay) showed 0.47μg/ml in the second case and 0.15μg/ml in the third case, from which our cases were considered to be classical factor X deficiency.

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