Die Anwendung eines C1-Inhibitorkonzentrats zur präoperativen Kurzzeitprophylaxe bei zwei Patienten mit hereditärem Angioödem

A commercially available C1 inhibitor (C1-INH) concentrate was used for short-term prophylaxis before surgery in two patients with hereditary angioneurotic oedema. The patients suffered from recurrent subcutaneous and submucosal oedema of the face, extremities, and gastrointestinal tract as the result of a hereditary C1-INH deficiency. Both patients were receiving tranexamic acid or danazol therapy as oral long-term prophylaxis. Over the years the patients underwent several operations in regional and general anaesthesia, with mask ventilation or intubation. The C1-INH plasma concentrations measured preoperatively were always very low (0.02–0.06?g/l, normal range 0.15–0.35?g/l), despite the oral long-term prophylaxis. Substitution treatment with 500–1000?U C1-INH was performed 1?h before surgery. No side effects were seen following the concentrate infusions. With this substitution treatment no specific symptoms of hereditary angioneurotic oedema were recognized in either case. The measurement of C1-INH plasma concentration 2?h or 4?h after C1-INH substitution showed a marked rise in both patients, though normal values were not reached in either. We suggest that infusion of C1 concentrate is an appropriate form of preoperative substitution treatment in patients with hereditary angioneurotic oedema, in view of the lower risk of infection than with infusion of fresh-frozen plasma and the observed effectiveness.