Renal transplantation in children with sickle cell disease: a report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS).

: Sickle cell disease is a rare cause of end-stage renal disease (ES-RD) in pediatrics accounting for only 0.5% and 0.2% of the patients registered in the dialysis and transplant arms of the NAPRTCS database, respectively. Accordingly, the single-center experience with this disorder is extremely rare, and little information on patient and graft outcome is available. Between 1989-1995, 9 patients with sickle cell nephropathy (5 male and 4 female) received 10 transplants [7 cadaver (CAD) and 3 living-related donor (LRD)]. The mean age at the time of the first transplant was 16.0 +/- 1.6 years. Prior to transplantation, all patients received maintenance dialysis (4 HD, 4 PD, 1 HD/PD) for 21.4 +/- 16.1 months (range 3-47 months), and all had received > 5 lifetime random blood transfusions. There was a mean 15.9 consecutive days of hospitalization from transplantation to discharge. Initial immunosuppression consisted of methyl predinsolone/prednisone (9 patients) cyclosporine (7 patients) and azathioprine (8 patients). ATG/ALG or OKT3 were used in 3 CAD transplants. There have been 21 acute rejection episodes in 7 of the 9 patients. Two patients had no rejections and 2 patients had 6 rejections each. Six of the 21 rejection episodes occurred within the first 100 days post-transplant. Nine (43%) of the rejections were completely reversed, the reversal rate varying from 71% for first rejections to 29% for second and subsequent rejections. Four (40%) of the 10 grafts have failed. Graft survival at 12 and 24 months post-transplant is 0.89 +/- 0.11 and 0.71 +/- 0.18, respectively. Patient survival is 89%. The present experience suggests that renal transplantation is a viable option for the adolescent patient with sickle cell nephropathy and ESRD.