[Acquired dysfibrinogenemia in a child in the course of a liver disease].
This report documents the results of coagulation studies in a 15-year old boy with hepatic disease of possibly autoimmune origin. Thrombin- and reptilase clotting times were prolonged. The results of fibrinogen determinations with different methods (heat precipitation, kinetic assay according to Clauss) lead to the assumption of a qualitative anomaly of fibrinogen (dysfibrinogenaemia). Investigations with purified fibrinogens from the patient and a healthy control confirmed this assumption. The thrombin clotting time of purified fibrinogen from the patient was clearly prolonged in comparison to the purified fibrinogen from a healthy control. The fibrin monomers of the patient exhibited impaired polymerization. No structural abnormalities were detected by electrophoretic techniques, particularly the thrombin-induced release of fibrinopeptides was found to be normal. The coagulation abnormalities resolved after glucocorticoid administration.
Maak
B
B
Universitäts-Kinderklinik Jena.
Meyer
M
M
Dietze
H
H
D000293:Adolescent; D000347:Afibrinogenemia / Q000097:blood*; D001327:Autoimmune Diseases; D001780:Blood Coagulation Tests; D005340:Fibrinogen / Q000737:chemistry; D006801:Humans; D008107:Liver Diseases / Q000097:blood* / Q000188:drug therapy / Q000276:immunology; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use; D013918:Thrombin Time
D005340:Fibrinogen; D011239:Prednisolone
Erworbene Dysfibrinogenämie bei einem Kind im Verlauf einer Lebererkrankung.