Focal segmental glomerulosclerosis in pediatric kidney transplantation: 30years' experience

From 1982 to 2011, 53 kidney transplantations (KT) for pediatric focal segmental glomerulosclerosis (FSGS) were recorded in the National Israeli Kidney Transplant Registry (NIKTR): 22primary (1?) FSGS, 25proved/suspected genetic-secondary (2?) FSGS, six lost/incomplete files/other. Half (56%) of 23 patients with 2? FSGS were Israeli-Arabs vs 29% of 1? FSGS KT recipients. 1? FSGS recurrence occurred in 64% (14/22) of 22 KT in 17 patients aged (median) 14years vs 1/25 of 2? FSGS (P<.001). Early graft days/nonfunction occurred in 9/14 (64%), 2/8 (25%) and 2/25 (4%) of recurrent 1? FSGS (rFSGS), nonr1? FSGS and 2? FSGS, respectively. Twelve biopsies performed in nine of these grafts at (median) 8days (range 5-60days) post-KT showed: ATN-5, suspected rejection-4, rFSGS-2, normal kidney-1; rFSGS was diagnosed eventually in 8/9. Dialysis need during the first month post-KT was significantly associated with FSGS recurrence: 6/14 (43%) for rFSGS vs 2/8 (25%) for non-rFSGS. Plasmapheresis (PP) achieved complete and partial rFSGS remission in 5/9 and 2/9 grafts, respectively. Three grafts were excised during the first 60days post-KT for: nonfunction (1) and bleeding (2). Remaining grafts' GFR was: 78, 42, and 91mL/min (median) at 5.3, 4.75, and 8years follow-up for non-rFSGS, rFSGS, and 2? FSGS grafts, respectively. Conclusions: Early PP implementation should be considered after KT for 1? FSGS patients with early graft dysfunction despite delayed proteinuria and nonspecific biopsy.