H I Cheong1, H W Han, H W Park, I S Ha, K S Han, H S Lee, S J Kim, Y Choi. 1. Departments of Pediatrics, General Surgery, Clinical Pathology and Pathology, Seoul National University Children's Hospital, Seoul and Department of Pediatrics, Seoul City Boramae Hospital, Seoul, Korea.
Abstract
BACKGROUND: We analysed risk factors to predict the recurrence of nephrotic syndrome and the therapeutic efficacy of plasmapheresis combined with oral cyclophosphamide (PE+CPM) in early recurrent nephrotic syndrome after transplantation in children with focal segmental glomerulosclerosis (FSGS). METHODS: Medical records after 1990 of 16 children with biopsy-proven idiopathic FSGS and renal transplantation before the age of 18 years were reviewed. RESULTS: Early recurrence of nephrotic syndrome developed in six cases (37. 5%). While early kidney graft biopsies, performed within the first week after the onset of recurrence, revealed diffuse effacement of foot process only, late biopsies contained segmentally sclerosed glomeruli as well. Among several possible risk factors, the mean duration from onset of original nephrotic syndrome to development of end-stage renal disease was shorter in the recurrent group (P=0.045) and the percentage of globally sclerosed glomeruli was higher in the non-recurrent group (P=0.001). PE+CPM therapy resulted in complete remission of nephrotic syndrome if it was started early and if there was no evidence of accompanying acute rejection. CONCLUSION: These results support more liberal use of living-related donors for renal transplantation of children with FSGS and ESRD, considering the shortage of cadaveric donors in our society and relatively good efficacy of the early and intensive PE+CPM therapy for early recurrent nephrotic syndrome.
BACKGROUND: We analysed risk factors to predict the recurrence of nephrotic syndrome and the therapeutic efficacy of plasmapheresis combined with oral cyclophosphamide (PE+CPM) in early recurrent nephrotic syndrome after transplantation in children with focal segmental glomerulosclerosis (FSGS). METHODS: Medical records after 1990 of 16 children with biopsy-proven idiopathic FSGS and renal transplantation before the age of 18 years were reviewed. RESULTS: Early recurrence of nephrotic syndrome developed in six cases (37. 5%). While early kidney graft biopsies, performed within the first week after the onset of recurrence, revealed diffuse effacement of foot process only, late biopsies contained segmentally sclerosed glomeruli as well. Among several possible risk factors, the mean duration from onset of original nephrotic syndrome to development of end-stage renal disease was shorter in the recurrent group (P=0.045) and the percentage of globally sclerosed glomeruli was higher in the non-recurrent group (P=0.001). PE+CPM therapy resulted in complete remission of nephrotic syndrome if it was started early and if there was no evidence of accompanying acute rejection. CONCLUSION: These results support more liberal use of living-related donors for renal transplantation of children with FSGS and ESRD, considering the shortage of cadaveric donors in our society and relatively good efficacy of the early and intensive PE+CPM therapy for early recurrent nephrotic syndrome.
Authors: Jillian K Warejko; Weizhen Tan; Ankana Daga; David Schapiro; Jennifer A Lawson; Shirlee Shril; Svjetlana Lovric; Shazia Ashraf; Jia Rao; Tobias Hermle; Tilman Jobst-Schwan; Eugen Widmeier; Amar J Majmundar; Ronen Schneider; Heon Yung Gee; J Magdalena Schmidt; Asaf Vivante; Amelie T van der Ven; Hadas Ityel; Jing Chen; Carolin E Sadowski; Stefan Kohl; Werner L Pabst; Makiko Nakayama; Michael J G Somers; Nancy M Rodig; Ghaleb Daouk; Michelle Baum; Deborah R Stein; Michael A Ferguson; Avram Z Traum; Neveen A Soliman; Jameela A Kari; Sherif El Desoky; Hanan Fathy; Martin Zenker; Sevcan A Bakkaloglu; Dominik Müller; Aytul Noyan; Fatih Ozaltin; Melissa A Cadnapaphornchai; Seema Hashmi; Jeffrey Hopcian; Jeffrey B Kopp; Nadine Benador; Detlef Bockenhauer; Radovan Bogdanovic; Nataša Stajić; Gil Chernin; Robert Ettenger; Henry Fehrenbach; Markus Kemper; Reyner Loza Munarriz; Ludmila Podracka; Rainer Büscher; Erkin Serdaroglu; Velibor Tasic; Shrikant Mane; Richard P Lifton; Daniela A Braun; Friedhelm Hildebrandt Journal: Clin J Am Soc Nephrol Date: 2017-11-10 Impact factor: 8.237
Authors: Jonathan H Pelletier; Karan R Kumar; Rachel Engen; Adam Bensimhon; Jennifer D Varner; Michelle N Rheault; Tarak Srivastava; Caroline Straatmann; Cynthia Silva; T Keefe Davis; Scott E Wenderfer; Keisha Gibson; David Selewski; John Barcia; Patricia Weng; Christoph Licht; Natasha Jawa; Mahmoud Kallash; John W Foreman; Delbert R Wigfall; Annabelle N Chua; Eileen Chambers; Christoph P Hornik; Eileen D Brewer; Shashi K Nagaraj; Larry A Greenbaum; Rasheed A Gbadegesin Journal: Pediatr Nephrol Date: 2018-07-07 Impact factor: 3.714