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In patients with acute severe hepatic failure the synthesis of clotting factors and inhibitors is considerably diminished. The decrease of clotting factors may be enhanced by liberation of thromboplastic substances from liver cell debris, leading to thrombus formation in the sinusoids and to further cell damage. At the low levels of clotting factors and inhibitors signs of disseminated intravascular coagulation as well as hyperfibrinolysis have been demonstrated. Treatment with heparin to prevent coagulation is insufficient at low levels of antithrombin III (AT III). Therefore, Vogel und Fritsche 1979 suggested the substitution of AT III in these cases.We now report about 3 patients who were admitted to the clinic together with severe signs of liver damage after oral uptake of CCl4 On the day of admission several clotting factors plasminogen and alpha2-antiplasmin were significantly diminished; AT III levels between 25-45% of the norm were found. (Diss. Eckhardt-Klaßnitz). Therefore we started treatment with AT III concentrate from Behringwerke (1000-2000 I.U. daily for 3 to 14 days) and fresh frozen plasma (total volume of 2 1 within the first 3 days).AT III was simultaneously determined by clotting test, a chromogenic substrate test, and immunologically. Hemodialysis was necessary in 2 patients. Unter treatment with AT III and fresh frozen plasma no bleeding tendency occured Though two of the patients showed severe intoxication on admission all could be dismissed with only slight histological signs of liver alterations. Treatment with AT III concentrates, therefore, seems of value in patients with acute yellow liver dystrophy.

70 Stunden bestimmt. Nach der Substitution traten etwa sechs Wochen lang keine spontanen »mikrotraumatischen« Blutungen auf. Ob die Substitution mit dem Faktor-XIII-Konzentrat der Behringwerke auch über Jahre durchgeführt werden kann, muß weiteren Beobachtungen vorbehalten bleiben. Patienten mit kongenitalem Faktor-XIII-Mangel sind nach Schädeitraumen durch intrakranielle Blutungen gefährdet (1, 2, 8, 9, 11, 21, 22). Da die Blutungen oder schweren Nachblutungen meist erst 24-48 Stunden nach Substitution treatment of factor XIII deficiency with a new factor XIII concentrate A 31/s-year-old boy with congenital factor XIII deficiency was given a new factor XIII concentrite, wihch increased factor XIII activity from O to l8/o of normal, as measured by two different tests. Half-life of the preparation was about 70 hours. For about six weeks after administration of the concentrate no spontaneous ,>microtraumaticv bleedings occurred. The effect of long-term administration remains to be investigated.dem Schädeltrauma auftreten, sollte die Therapie innerhalb dieses Intervalls beginnen.Die sogenannte Intervaliblutung bei kongenitalem Faktor-XIII-Mangel ist ein pathognomonisches Symptom. Nr. 13, 31. März 1972, 97. Jg. Trobisch, Egbring Neues Faktor-XIl1-Konzentrat bei kongenitalem Faktor-Xlll-Mangel 499Heruntergeladen von: NYU. Urheberrechtlich geschützt.

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