Early recurrent nephrotic syndrome after renal transplantation in children with focal segmental glomerulosclerosis

Cheong, Hae Ii; Han, Hye Won; Park, Hye Won; Ha, Ii Soo; Han, Kyu Sup; Lee, Hyun Soon; Kim, Sang Joon; Choi, Yong

doi:10.1093/ndt/15.1.78

Cited by 106 publications

(81 citation statements)

References 18 publications

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“…Plasmapheresis Plasma-based treatments, primarily plasmapheresis, have been used as a first-line treatment for FSGS and for recurrent FSGS after transplantation for nearly 20 years [5,[46][47][48][49][50][51][52][53][54][55][56][57][58][59]. The results of select studies performed since the year 2000 with larger numbers of patients are summarized in Table 2.…”

Section: Treatmentmentioning

confidence: 99%

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: Treatmentmentioning

confidence: 99%

Recurrent focal segmental glomerulosclerosis after kidney transplantation

2015

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“…Moreover, in patients with FSGS the risk of recurrence after kidney transplantation is estimated to be 11%-50%, an event that again leads to terminal renal failure. [2][3][4] The risk for recurrence of FSGS in patients with inherited forms of SRNS is lower than that in patients with nonhereditary FSGS. 5,6 SRNS is one of the most intractable diseases in nephrology.…”

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confidence: 99%

A Single-Gene Cause in 29.5% of Cases of Steroid-Resistant Nephrotic Syndrome

Sadowski

Lovric

Ashraf

et al. 2015

Journal of the American Society of Nephrology

546

642

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Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of ESRD in the first two decades of life. Effective treatment is lacking. First insights into disease mechanisms came from identification of single-gene causes of SRNS. However, the frequency of single-gene causation and its age distribution in large cohorts are unknown. We performed exon sequencing of NPHS2 and WT1 for 1783 unrelated, international families with SRNS. We then examined all patients by microfluidic multiplex PCR and next-generation sequencing for all 27 genes known to cause SRNS if mutated. We detected a single-gene cause in 29.5% (526 of 1783) of families with SRNS that manifested before 25 years of age. The fraction of families in whom a single-gene cause was identified inversely correlated with age of onset. Within clinically relevant age groups, the fraction of families with detection of the single-gene cause was as follows: onset in the first 3 months of life (69.4%), between 4 and 12 months old (49.7%), between 1 and 6 years old (25.3%), between 7 and 12 years old (17.8%), and between 13 and 18 years old (10.8%). For PLCE1, specific mutations correlated with age of onset. Notably, 1% of individuals carried mutations in genes that function within the coenzyme Q 10 biosynthesis pathway, suggesting that SRNS may be treatable in these individuals. Our study results should facilitate molecular genetic diagnostics of SRNS, etiologic classification for therapeutic studies, generation of genotype-phenotype correlations, and the identification of individuals in whom a targeted treatment for SRNS may be available.

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“…The treatment of FSGS recurrence is uncertain with no available international guidelines; few studies reported beneficial results of plasmapheresis (ranging from 10-23 sessions), with or without rituximab [12]. Moreover, some centres combined plasmapheresis with cyclophosphamide (while the antimetabolite immune suppression transiently discontinued) [13,14]. Both strategies showed promising results with complete or partial remission of FSGS.…”

Section: Discussionmentioning

confidence: 99%

Recurrence of Focal Segmental Glomerulosclerosis (FSGS) in the Renal Allograft; an Update

Ahmed¹

2017

UNOAJ

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Patients with primary focal segmental glomerulosclerosis (FSGS) have a high risk of disease recurrence post renal transplantation; this is usually characterised by significant proteinuria and associated with poor graft survival. FSGS recurrence is a challenging medical condition, not only because there is no recognised predictor of FSGS recurrence in the allograft, but also because there are no available international guidelines for treatment of this condition which add more mystery to the dilemma created by this vague syndrome. Our work aimed to draw the attention to the latest available evidence addressing recurrence of FSGS in the renal allograft, in an attempt to improve our understanding of this condition. This also helps to guide the future studies towards the promising approaches in diagnosis and management of this condition. Ultimately this will result in better graft survival and lower the demands for another renal transplant giving the universal shortage of transplantable organs.

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Early recurrent nephrotic syndrome after renal transplantation in children with focal segmental glomerulosclerosis

Cited by 106 publications

References 18 publications

Recurrent focal segmental glomerulosclerosis after kidney transplantation

Recurrent focal segmental glomerulosclerosis after kidney transplantation

A Single-Gene Cause in 29.5% of Cases of Steroid-Resistant Nephrotic Syndrome

Recurrence of Focal Segmental Glomerulosclerosis (FSGS) in the Renal Allograft; an Update

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