Maak B, Meyer M, Dietze H
Universitäts-Kinderklinik Jena.
Monatsschr Kinderheilkd. 1990 Aug;138(8):446-50.
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.
本报告记录了一名15岁患可能为自身免疫性肝病男孩的凝血研究结果。凝血酶和蛇毒凝血时间延长。采用不同方法（热沉淀法、根据克劳斯法的动力学测定法）测定纤维蛋白原的结果提示存在纤维蛋白原定性异常（异常纤维蛋白原血症）。对患者和健康对照的纯化纤维蛋白原进行的研究证实了这一推测。与健康对照的纯化纤维蛋白原相比，患者的纯化纤维蛋白原的凝血酶凝血时间明显延长。患者的纤维蛋白单体聚合受损。电泳技术未检测到结构异常，尤其是发现凝血酶诱导的纤维蛋白肽释放正常。给予糖皮质激素后凝血异常得以缓解。
