Abstract
We report two patients with C1-inhibitor deficiency on hemodialysis therapy who experienced frequent occlusion of their blood access. One patient, a 44-year-old woman with hereditary angioedema, has been on hemodialysis for lupus nephritis. The other patient, a 54-year-old man, was started on hemodialysis therapy for diabetic nephropathy. There were no signs of angbedema or complement abnormalities in other members of his family, so his angioedema seemed to be attributable to the acquired form of C1-inhibitor deficiency. We examined the influence of angioedema attacks on the complement, coagulation, fibrinolysis and kinin systems. The predialysis levels of thrombin-antithrombin III (TAT), plasmin-α2 plasmin inhibitor complex (PIC), and fibrinogen of 29 patients on hemodialysis therapy without vascular complications were almost all within the normal range. TAT, PIC and bradykinin levels of both patients were increased during the attacks that led to occlution of blood access. In 1 patient, though the levels increased during attacks, blood access complications did not follow. These findings suggest that the coagulation and kinin systems were affected during the attacks of angioedema, leading to acute occlusion of blood access.
