Autor: |
Wehinger, H., Klinge, O., Alexandrakis, E., Schürmann, J., Witt, J., Seydewitz, H., Schürmann, J, Seydewitz, H H |
Zdroj: |
European Journal of Pediatrics; 1983, Vol. 141 Issue 2, p109-112, 4p |
Abstrakt: |
A family with hereditary autosomal dominant hypofibrinogenemia is described. The outstanding feature is massive deposition of fibrinogen/fibrin within hepatocytes, faintly visible in routine microscopic sections, but clearly demonstrable by immunohistologic techniques. Circulating fibrinogen shows normal electrophoretic mobility of A alpha-, B beta-, and gamma-chains. We assume that the hereditary defect in this family interferes with fibrinogen release from hepatocytes. Clinically there are fluctuating slight elevations of serum transaminase levels. Hemostasis and wound healing are undisturbed. [ABSTRACT FROM AUTHOR] |
Databáze: |
Complementary Index |
Externí odkaz: |
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