Although considerable progress has been made during the past two decades in the use of androgens to prevent attacks of hereditary angioedema, replacement of the deficient C1-inhibitor protein would provide a useful menas of treatment once an attack has begun. We studied the clinical use of C1 inhibitor that was partly purified on a large scale from pooled plasma. The in vivo efficacy and safety of this protein concentrate were evaluated during 11 intravenous infusions in eight patients with hereditary angioedema. Three patients received the C1-inhibitor preparation during an asymptomatic period. Increases in serum C4 activity provided evidence of the biologic activity of the infused inhibitor. Intravenous administration of the concentrate during acute abdominal or laryngeal attacks of hereditary angioedema in five patients resulted in abatement of symptoms in addition to increased serum C4 activity. No untoward effects of the intravenous administration of the C1 inhibitor were observed in these eight patients. Thus, this C1-inhibitor preparation seems to offer the potential for safe, effective replacement therapy and may provide a means of controlling an attack of hereditary angioedema that is in progress.
Sixteen patients who received Konyne for bleeding episodes or for hemostasis during surgical procedures were evaluated for signs of posttransfusion hepatitis. Four patients died from their primary disease. Of the remaining 12 patients at risk, six patients developed posttransfusion hepatitis. Sera from four of the six patients with hepatitis were positive for the hepatitis B antigen (HBAg). Aliquots of Konyne from the specific lot (K4797) implicated in three cases of HBAg‐positive hepatitis were negative for HBAg by immunodiffusion, immunoelectroosmophoresis, and radioimmunoassay. The high incidence of hepatitis associated with Konyne considerably limits the use for this specialized coagulation factor concentrate. HBAg screening by available technics does not necessarily detect Konyne preparations capable of transmitting HBAg‐positive hepatitis.
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