Successful haemodialysis and renal transplantation in a patient with haemophilia A.
A 19-year old male with severe haemophilia A (factor VIII activity less than 1%) developed terminal renal insufficiency and was subsequently dialysed via an external arteriovenous shunt for one year. To prevent bleeding he received cryoprecipitate (2000-2500 units of factor VIII) three times a week during dialysis. After one year of uneventful dialysis he received a kidney graft from a cadaver donor that was matched for the B locus antigens. During the first two weeks after transplantation his factor VIII level was kept at approximately 70% by daily cryoprecipitate infusions. Thereafter he was free from bleeding at a level of 20% with prophylactic cryoprecipitate treatment (1000 units 3 times a week). He was discharged from the hospital five weeks after transplantation with excellent renal function (ECC 75 ml/min). No rejection crisis occurred. His factor VIII requirements remained unchanged after transplantation, indicating that the human kidney does not substantially contribute to the production of clotpromoting factor VIII.
D000328:Adult; D005169:Factor VIII / Q000096:biosynthesis / Q000627:therapeutic use; D006467:Hemophilia A / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D007668:Kidney / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy; D016030:Kidney Transplantation; D008297:Male; D006435:Renal Dialysis
D005169:Factor VIII