Features of renal replacement therapy in a patient with hemophilia A

Background. Hemophilia A is a congenital coagulation disorder caused by a deficiency of blood coagulation factor VIII. The method of choice for renal replacement therapy ensuring vascular access and safe anticoagulant therapy during hemodialysis in patients with hemophilia A and developing renal failure have not been studied. The aim of this clinical observation is to show the peculiarities of renal replacement therapy in a patient with severe hemophilia A. Observation. A clinical case of a patient with hemophilia A is presented. The patient needed programmed hemodialysis due to chronic kidney disease. An arteriovenous fistula was originally formed as a vascular access. Hemodialysis sessions were carried out without the use of anticoagulants, using only natural hypocoagulation due to hemophilia. However, before the end of the procedure, the factor VIII concentrate was administered to prevent bleeding from punctures of arteriovenous fistula. Two years after the start of programmed hemodialysis, the patient underwent allogenic cadaveric kidney transplantation. Due to the self-cancellation of an immunosuppressive therapy, graft rejection has developed, and therefore the graft was removed a year later. Programmed dialysis was resumed. As a vascular access, a permanent tunneled catheter was used that allowed one to performe hemodialysis sessions without anticoagulant therapy using only natural hypocoagulation, and without a subsequent introduction of the factor FVIII concentrate. Conclusion. A second case of kidney transplantation in a patient with hemophilia in Russia is described. The tactics of anticoagulant therapy in hemophilia patients during hemodialysis procedures depends on the activity of the deficient factor in the blood plasma and the selected vascular access.

хроническая болезнь почек, гемодиализ, трансплантация почки, биопсия почки, гемофилия А, фактор VIII, концентрат фактора VIII, chronic kidney disease, hemodialysis, kidney transplantation, kidney biopsy, hemophilia A, coagulation factor VIII, concentrate factor VIII

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