Recurrence of focal segmental glomerulosclerosis after renal transplantation in patients with mutations of podocin

Bertelli, Roberta; Ginevri, Fabrizio; Caridi, Gianluca; Dagnino, Monica; Sandrini, Silvio; Duca, Marco Di; Emma, Francesco; Sanna‐Cherchi, Simone; Scolari, Francesco; Neri, Tauro Maria; Murer, Luisa; Massella, Laura; Basile, Giancarlo; Rizzoni, Gianfranco; Perfumo, Francesco; Ghiggeri, Gian Marco

doi:10.1016/s0272-6386(03)00364-0

Cited by 144 publications

(137 citation statements)

References 20 publications

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“…This is consistent with reports published by Weber et al [22] and Ruff et al [23]. Bertelli et al [24] found no difference between those with or without mutations but failed to separate patients who were homozygous for podocin mutations from those who were heterozygous in their analysis. Only two patients out of 14 who were homozygous had recurrent disease.…”

Section: Pathogenesissupporting

confidence: 90%

Recurrent focal segmental glomerulosclerosis after kidney transplantation

2015

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Focal segmental glomerulosclerosis (FSGS) is an important cause of glomerular disease in children and adolescents and nearly 50 % of affected patients will progress to endstage kidney disease over a 5 to 10-year period. Unfortunately, there is no established treatment for disease in the native kidney. Moreover, up to 55 % of patients develop recurrent disease after receiving a kidney transplant, with a substantially higher risk in patients who have already experienced recurrent disease in a prior transplant. A number of clinical and laboratory factors have been identified as risk factors for this complication. In addition, new investigations into podocyte biology and circulating permeability factors have shed light on the cause of recurrent the disease. While a number of novel therapeutic agents have been applied in the management of this problem, there still is no proven treatment. In this review, we summarize recent advances in the epidemiology, pathophysiology, and treatment of recurrent FSGS in pediatric patients who have received a kidney transplant.

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Section: Pathogenesissupporting

confidence: 90%

Recurrent focal segmental glomerulosclerosis after kidney transplantation

2015

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“…However, the discovery of at least 1 patient in our study and other patients from previous publications with sporadic FSGS and mutations of podocin (NPHS2, which is actually an inherited disease) who received a renal graft requires a reevaluation of how to define sporadic incidence. 16 Interestingly enough, we noted that all recurrent cases in our study had received a kidney from a related donor. Recurrence occurred in 57% of related donor grafts versus 0% of unrelated donor grafts.…”

Section: Discussionmentioning

confidence: 60%

Untitled

2016

Exp Clin Transplant

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Objectives: Recurrent disease occurs in around 30% of children transplanted for steroid-resistant nephrotic syndrome. Its precipitating risk factors have rarely been studied in the Middle East. The aim of our study was to determine what characterizes posttransplant recurrence of nephrotic syndrome in Syrian children. Materials and Methods:We performed a retrospective analysis of 12 nephrotic children who received 1 renal allograft at the Kidney Hospital in Damascus from 2002 to 2013. Results: Native kidney biopsy results showed focal segmental glomerulosclerosis in 9 of 10 patients. Four patients had 1 or more sibling affected with nephrotic syndrome, and the remaining patients were labeled as having sporadic disease. Genetic screening for NPHS2, NPHS1, and Wilms tumor gene (WT1) mutations were done for 6 patients, and 1 novel homozygous NPHS2 mutation was identified in 1 patient. All patients received transplants from living donors. Four patients had recurrence of initial disease after transplant (overall recurrence rate of 33%). However, 1 patient showed complete and spontaneous remission 20 months after transplant; As expected, the patient with NPSH2 mutation had no recurrence. Patients with sporadic disease showed risk of recurrence 5 times higher than patients with familial disease (P = .24). Interestingly, all recurrent cases had received a kidney from a related donor and were initially classified as having sporadic disease. Although not statistically significant, the risk of recurrence from related donor grafts was 6.75 times higher than from unrelated donors (P = .16). To the best of our knowledge, this observation, the first of its kind, has never been investigated or pointed out in the literature. Conclusions:Further research is needed to confidently determine whether living related donor grafts are associated with increased incidence of recurrence of nephrotic syndrome.

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“…Patients with high pretransplant P alb activity have a higher risk of FSGS recurrence (30). The impact of recipient age on FSGS recurrence was confirmed by Bertelli et al in patients without mutation of podocin with early recurrence of proteinuria in 44% of younger patients and in 23% of the older ones (30). It is taken as a proof of the existence of circulating PF(s) that are also putative effectors of original proteinuria in these patients (30).…”

Section: Fsgs Recurrence After Kidney Transplantationmentioning

confidence: 89%

“…According to this possibility, removal of the putative PF by plasmapheresis (PP) or selective procedures with protein A column associated with a course of immunosuppression would restore a good long-term outcome of the graft. Patients with high pretransplant P alb activity have a higher risk of FSGS recurrence (30). The impact of recipient age on FSGS recurrence was confirmed by Bertelli et al in patients without mutation of podocin with early recurrence of proteinuria in 44% of younger patients and in 23% of the older ones (30).…”

Section: Fsgs Recurrence After Kidney Transplantationmentioning

confidence: 91%