| Literature DB >> 6662138 |
H Wehinger, O Klinge, E Alexandrakis, J Schürmann, J Witt, H H Seydewitz.
Abstract
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.Entities:
Mesh：
Substances：
Year: 1983 PMID： 6662138 DOI： 10.1007/bf00496800
Source DB: PubMed Journal: Eur J Pediatr ISSN： 0340-6199 Impact factor: 3.183