title: Focal segmental glomerulosclerosis in pediatric kidney transplantation: 30 years' experience.
abstract: From 1982 to 2011, 53 kidney transplantations (KT) for pediatric focal segmental glomerulosclerosis (FSGS) were recorded in the National Israeli Kidney Transplant Registry (NIKTR): 22-primary (1◦) FSGS, 25-proved/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) 14 years 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) 8 days (range 5-60 days) 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 60 days post-KT for: nonfunction (1) and bleeding (2). Remaining grafts' GFR was: 78, 42, and 91 mL/min (median) at 5.3, 4.75, and 8 years follow-up for non-rFSGS, rFSGS, and 2◦ FSGS grafts, respectively.
  
  
  
  Early PP implementation should be considered after KT for 1◦ FSGS patients with early graft dysfunction despite delayed proteinuria and nonspecific biopsy.
authors.affiliation: Pediatric Nephrology Unit, Tel Aviv Sourasky Medical Center, Dana Dwek Children's Hospital, Tel Aviv, Israel. acleper@zahav.net.il.|Institute of Nephrology, Schneider Children's Medical Center of Israel, Petah Tikva, Israel. acleper@zahav.net.il.|Sackler Medical School, Tel Aviv University, Tel Aviv, Israel. acleper@zahav.net.il.
authors.forename: Roxana
authors.initials: R
authors.lastname: Cleper
authors.affiliation: Institute of Nephrology, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.|Sackler Medical School, Tel Aviv University, Tel Aviv, Israel.|Pediatric Department C, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.
authors.forename: Irit
authors.initials: I
authors.lastname: Krause
authors.affiliation: Department of Transplantation, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel.
authors.forename: Nathan
authors.initials: N
authors.lastname: Bar Nathan
authors.affiliation: Department of Transplantation, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel.
authors.forename: Maya
authors.initials: M
authors.lastname: Mor
authors.affiliation: Institute of Nephrology, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.|Sackler Medical School, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Amit
authors.initials: A
authors.lastname: Dagan
authors.affiliation: Department of Pediatric Nephrology and Dialysis, Western Galilee Hospital, Nahariya, Israel.|The Faculty of Medicine in the Galilee, Bar Ilan University, Safed, Israel.
authors.forename: Irith
authors.initials: I
authors.lastname: Weissman
authors.affiliation: Division of Pediatric Nephrology, Shaare Zedek Medical Center, Jerusalem, Israel.|Hadassah-Hebrew University School of Medicine, Jerusalem, Israel.
authors.forename: Yaakov
authors.initials: Y
authors.lastname: Frishberg
authors.affiliation: Sackler Medical School, Tel Aviv University, Tel Aviv, Israel.|Department of Transplantation, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel.
authors.forename: Ruty
authors.initials: R
authors.lastname: Rachamimov
authors.affiliation: Sackler Medical School, Tel Aviv University, Tel Aviv, Israel.|Department of Transplantation, Rabin Medical Center, Beilinson Hospital, Petah Tikva, Israel.
authors.forename: Eitan
authors.initials: E
authors.lastname: Mor
authors.affiliation: Institute of Nephrology, Schneider Children's Medical Center of Israel, Petah Tikva, Israel.|Sackler Medical School, Tel Aviv University, Tel Aviv, Israel.
authors.forename: Miriam
authors.initials: M
authors.lastname: Davidovits
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000175:diagnosis / Q000601:surgery*; D006084:Graft Rejection / Q000175:diagnosis / Q000453:epidemiology; D006085:Graft Survival; D006801:Humans; D007223:Infant; D016030:Kidney Transplantation; D008297:Male; D012008:Recurrence; D012042:Registries; D012189:Retrospective Studies; D016896:Treatment Outcome
keywords: acute tubular necrosis; focal segmental glomerulosclerosis; graft dysfunction; kidney transplantation; plasmapheresis; posttransplantation recurrence; proteinuria