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recurrent FSGS in kidney transplant

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https://www.readbyqxmd.com/read/27797890/long-term-outcome-of-kidney-transplantation-in-recipients-with-focal-segmental-glomerulosclerosis
#1
Anna Francis, Peter Trnka, Steven J McTaggart
BACKGROUND AND OBJECTIVES: FSGS can recur after kidney transplantation and is associated with poor graft outcomes. We aimed to assess the incidence of FSGS recurrence post-transplant and determine the effect of graft source on recurrence and graft survival in patients with biopsy-proven FSGS. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Using the Australian and New Zealand Dialysis and Transplant Registry, we assessed incidence of FSGS, the influence of donor type on the risk of FSGS recurrence, and graft loss in recipients with ESRD caused by primary FSGS using Kaplan-Meier and logistic regression analyses...
November 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27733133/diagnosing-fsgs-without-kidney-biopsy-a-novel-inf2-mutation-in-a-family-with-esrd-of-unknown-origin
#2
Johannes Münch, Maik Grohmann, Tom H Lindner, Carsten Bergmann, Jan Halbritter
BACKGROUND: Patients on renal replacement therapy are often unaware of their underlying condition and hence suffer from so-called end-stage renal disease (ESRD) of unknown origin. However, an exact diagnosis is not only important for better estimating the prognosis, but also when preparing for kidney transplantation. Whilst patients with FSGS without a confirmed genetic cause have a high recurrence rate in the transplanted organ, patients with a mutation generally exhibit no recurrence and have a good prognosis...
October 12, 2016: BMC Medical Genetics
https://www.readbyqxmd.com/read/27473582/the-role-of-plasma-exchange-in-treating-post-transplant-focal-segmental-glomerulosclerosis-a-systematic-review-and-meta-analysis-of-77-case-reports-and-case-series
#3
Abdullah Kashgary, Jessica M Sontrop, Lihua Li, Ahmed A Al-Jaishi, Zainab N Habibullah, Roaa Alsolaimani, William F Clark
BACKGROUND: Evidence on the role of plasma exchange for treating recurrent post-transplant focal segmental glomerulosclerosis (FSGS) comes largely from individual cases and uncontrolled series. We conducted a systematic review and meta-analysis to estimate the remission rate after treatment with plasma exchange, and to determine if remission varied with patient or treatment characteristics. METHODS: We searched MEDLINE, EMBASE, Science Citation Index Expanded, and the Conference Proceedings Citation Index (Science and BIOSIS) for studies of patients with post-transplant recurrent FSGS who were treated with plasma exchange after recurrence (1950-2012)...
July 29, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27460535/role-of-race-in-kidney-transplant-outcomes-in-children-with-focal-segmental-glomerulosclerosis
#4
Ivan Guan, Pamela Singer, Rachel Frank, Nataliya Chorny, Lulette Infante, Christine B Sethna
It is well established that racial differences exist in kidney transplant outcomes; however, there are no studies which focus on the role of race in transplant outcomes specifically in children diagnosed with FSGS. Associations between race and transplant outcomes in FSGS children were evaluated using the Organ Procurement and Transplantation Network database from 2000 to 2012. Recipients aged 2-21 years who received a kidney-only transplant were included. Multivariate regression models were used to evaluate transplant outcomes by race...
September 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27312921/post-transplant-recurrence-of-focal-segmental-glomerulosclerosis-in-a-child-with-heterozygous-mutations-in-nphs1-and-nphs2
#5
Nina Battelino, Miha Arnol, Aljoša Kandus, Rafael Ponikvar, Gregor Novljan
Renal transplantation is the optimal renal replacement therapy (RRT) in children, but some primary diseases can recur after transplantation, and recurrence accounts for a significant proportion of graft losses, being second only to acute rejection. The risk of disease recurrence is highest among patients with idiopathic focal segmental glomerulosclerosis (FSGS), presumably due to a circulating permeability factor. Less is clear about the genetic forms of FSGS, where the data regarding the frequency of recurrence are rather conflicting...
June 2016: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/27274825/recurrent-focal-segmental-glomerulosclerosis-apparently-resistant-to-plasmapheresis-improves-after-surgical-repair-of-arteriovenous-fistula-aneurysms
#6
Yanli Ding, Jean Francis, Jeffrey Kalish, Anita Deshpande, Karen Quillen
Focal segmental glomerulosclerosis (FSGS) is a leading cause of end-stage renal disease and has a high recurrence rate after kidney transplantation, attributed to a circulating permeability factor. Plasmapheresis is the treatment of choice after recurrence to remove the circulating factor. We present a case of recurrent FSGS 6 years after transplantation. It is instructive because proteinuria did not respond to intensive plasmapheresis-combined with rituximab-until the possibility of ineffective apheresis secondary to multiple aneurysms in the arteriovenous fistula (AVF) was considered...
June 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27213154/recurrence-and-treatment-after-renal-transplantation-in-children-with-fsgs
#7
REVIEW
Hee Gyung Kang, Il-Soo Ha, Hae Il Cheong
Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease and a common pathologic diagnosis of idiopathic nephrotic syndrome (NS), especially in steroid-resistant cases. FSGS is known to recur after kidney transplantation, frequently followed by graft loss. However, not all patients with FSGS suffer from recurrence after kidney transplantation, and genetic and secondary FSGS have a negligible risk of recurrence. Furthermore, many cases of recurrence achieve remission with the current management of recurrence (intensive plasmapheresis/immunosuppression, including rituximab), and other promising agents are being evaluated...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27200372/circulating-permeability-factors-in-primary-focal-segmental-glomerulosclerosis-a-review-of-proposed-candidates
#8
REVIEW
Eva Königshausen, Lorenz Sellin
Primary focal segmental glomerulosclerosis (FSGS) is a major cause of the nephrotic syndrome and often leads to end-stage renal disease. This review focuses on circulating permeability factors in primary FSGS that have been implicated in the pathogenesis for a long time, partly due to the potential recurrence in renal allografts within hours after transplantation. Recently, three molecules have been proposed as a potential permeability factor by different groups: the soluble urokinase plasminogen activator receptor (suPAR), cardiotrophin-like cytokine factor-1 (CLCF-1), and CD40 antibodies...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27190346/nup107-mutations-in-children-with-steroid-resistant-nephrotic-syndrome
#9
Eujin Park, Yo Han Ahn, Hee Gyung Kang, Noriko Miyake, Hiroyasu Tsukaguchi, Hae Il Cheong
BACKGROUND: NUP107 is a novel gene associated with autosomal recessive steroid-resistant nephrotic syndrome (SRNS) with focal segmental glomerulosclerosis (FSGS) in children. The frequency of NUP107 mutations in children with SR-FSGS remains unknown. METHODS: Nine families with two siblings affected by childhood-onset SRNS or proteinuria were recruited. FSGS was confirmed by a kidney biopsy in at least one affected sibling in all families. Additionally, 69 sporadic pediatric cases with biopsy-proven SR-FSGS who had not responded to any treatment were included...
May 17, 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/27099858/treatment-of-focal-segmental-glomerulosclerosis-recurrence-in-the-renal-allograft-a-report-of-two-cases
#10
Minh-Ha Tran, Cynthia Chan, Whitney Pasch, Philip Carpenter, Hirohito Ichii, Clarence Foster
Focal segmental glomerulosclerosis (FSGS) causes glomerular lesions that can progress to end-stage renal disease. It is suspected to be caused by a circulating factor that is amenable to plasmapheresis removal and exhibits a risk for recurrence in the renal allograft. We present two patients with FSGS recurrence in their allograft kidneys diagnosed by biopsy after significant proteinuria developed in the posttransplant setting. Treatment with therapeutic plasma exchange induced long-term remission in both patients...
January 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27088031/successful-treatment-of-focal-segmental-glomerulosclerosis-after-kidney-transplantation-with-plasma-exchange-and-abatacept-in-a-patient-with-juvenile-rheumatoid-arthritis
#11
Hannelore Sprenger-Mähr, Emanuel Zitt, Afschin Soleiman, Karl Lhotta
Recurrent focal segmental glomerulosclerosis (FSGS) after renal transplantation is difficult to treat. Recently a series of four patients unresponsive to plasma exchange (PE) and rituximab, who were successfully treated with abatacept, has been reported. We present a 26-year-old Caucasian patient who suffered from juvenile rheumatoid arthritis and developed severe proteinuria eleven days after transplantation. An allograft biopsy was suggestive of recurrent focal segmental glomerulosclerosis. He did not respond to PE therapy...
2016: Case Reports in Transplantation
https://www.readbyqxmd.com/read/27043407/rituximab-for-recurrence-of-primary-focal-segmental-glomerulosclerosis-after-kidney-transplantation-clinical-outcomes
#12
Cyril Garrouste, Guillaume Canaud, Mathias Büchler, Joseph Rivalan, Charlotte Colosio, Frank Martinez, Julien Aniort, Caroline Dudreuilh, Bruno Pereira, Sophie Caillard, Carole Philipponnet, Dany Anglicheau, Anne Elisabeth Heng
BACKGROUND: Rituximab has shown encouraging results for the treatment of kidney transplantation recipients with focal segmental glomerulosclerosis (FSGS) recurrence. However, the correct, opportune, and safe use of rituximab for this indication remains to be determined. METHODS: This multicenter retrospective study reports on 19 new cases aged 35 (15-66) years who developed FSGS recurrence at 12 (1.5-27) days posttransplantation. Initial treatment consisted of plasma exchanges (PE), high doses of calcineurin inhibitors, and steroids...
April 13, 2016: Transplantation
https://www.readbyqxmd.com/read/27011905/update-on-the-treatment-of-focal-segmental-glomerulosclerosis-in-renal-transplantation
#13
REVIEW
Maria Messina, Ester Gallo, Alberto Mella, Fabiola Pagani, Luigi Biancone
Focal segmental glomerulosclerosis (FSGS) represents one of the most severe glomerular diseases, with frequent progression to end-stage renal disease and a high rate of recurrence in renal allografts (30%-50%). Recurrent FSGS portends a negative outcome, with the hazard ratio of graft failure being two-fold higher then that of other glomerulonephritis. Two patterns of clinical presentations are observed: Early recurrence, which is characterized by massive proteinuria within hours to days after implantation of the renal graft, and late recurrence, which occurs several months or years after the transplantation...
March 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/26701979/b7-1-blockade-does-not-improve-post-transplant-nephrotic-syndrome-caused-by-recurrent-fsgs
#14
Marianne Delville, Emilie Baye, Antoine Durrbach, Vincent Audard, Tomek Kofman, Laura Braun, Jérôme Olagne, Clément Nguyen, Georges Deschênes, Bruno Moulin, Michel Delahousse, Gwenaëlle Kesler-Roussey, Séverine Beaudreuil, Frank Martinez, Marion Rabant, Philippe Grimbert, Morgan Gallazzini, Fabiola Terzi, Christophe Legendre, Guillaume Canaud
FSGS is a common glomerular disorder that has a high propensity for recurrence after kidney transplant. The pathophysiology of FSGS is unknown, but podocytes seem to be the target of one or several circulating factors that lead to cytoskeleton reorganization and proteinuria. Research on podocytes has identified B7-1 as an important factor in podocyte biology and a new therapeutic target in renal disease. Indeed, in four patients with recurrent FSGS after transplant, treatment with the B7-1 blocker abatacept was associated with proteinuria remission...
August 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/26371597/recurrent-primary-focal-segmental-glomerulosclerosis-managed-with-intensified-plasma-exchange-and-concomitant-monitoring-of-soluble-urokinase-type-plasminogen-activator-receptor-mediated-podocyte-%C3%AE-3-integrin-activation
#15
Oliver Staeck, Torsten Slowinski, Ina Lieker, Kaiyin Wu, Birgit Rudolph, Danilo Schmidt, Susanne Brakemeier, Hans-Hellmut Neumayer, Changli Wei, Jochen Reiser, Klemens Budde, Fabian Halleck, Dmytro Khadzhynov
BACKGROUND: Primary focal segmental glomerulosclerosis (FSGS) often causes nephrotic proteinuria and frequently results in end-stage renal disease and recurrence after kidney transplantation. Recent studies describe soluble urokinase-type plasminogen activator receptor (suPAR) as a circulating factor implicated in FSGS. METHODS: This single-center study included 12 adult patients with histologically proven primary FSGS (n = 2) or recurrent FSGS after transplantation (n = 10)...
December 2015: Transplantation
https://www.readbyqxmd.com/read/26319246/b7-1-is-not-induced-in-podocytes-of-human-and-experimental-diabetic-nephropathy
#16
Elena Gagliardini, Rubina Novelli, Daniela Corna, Carlamaria Zoja, Barbara Ruggiero, Ariela Benigni, Giuseppe Remuzzi
The incidence of progressive kidney disease associated with diabetes continues to rise worldwide. Current standard therapy with angiotensin-converting enzyme inhibitors and/or angiotensin receptor blockers achieves only partial renoprotection, increasing the need for novel therapeutic approaches. Previous studies described B7-1 induction in podocytes of patients with proteinuria, including those with FSGS and type 2 diabetic nephropathy (DN). These findings sparked great excitement in the renal community, implying that abatacept, a costimulatory inhibitor that targets B7-1, could be a novel therapy for diabetic renal disease...
April 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/26247557/recurrence-of-focal-and-segmental-glomerulosclerosis-after-transplantation
#17
REVIEW
Guillaume Canaud, Marianne Delville, Christophe Legendre
Focal segmental glomerulosclerosis, which is a common glomerular disorder, manifests clinically with a nephrotic syndrome and has a high propensity for recurrence after kidney transplantation. The pathophysiology is currently unknown, and podocytes appear to be the target of one or several circulating factor(s) that lead to the recurrence of proteinuria after kidney transplantation. Identifying these circulating factor(s) and cells involved in its synthesis remains elusive; however, recently, our research on podocyte cytoskeleton biology has opened a new era of treatment...
February 2016: Transplantation
https://www.readbyqxmd.com/read/26031597/focal-segmental-glomerular-sclerosis-recurrence-with-massive-proteinuria-and-anuria-immediately-after-kidney-transplantation
#18
Michihiro Maruyama, Hiroshi Kitamura, Naotake Akutsu, Kazunori Otsuki, Masayuki Hasegawa, Hiromichi Aoyama, Ikuko Matsumoto, Kenichi Saigo, Takehide Asano
Here, we report a case of focal segmental glomerular sclerosis (FSGS) recurrence immediately (47 minutes) after transplantation. A 1-hour biopsy specimen showed large periodic acid-Schiff-positive granules within the cells of the swollen proximal tubule, while electron microscopy revealed podocyte swelling and partial foot process effacement. These findings were worse on day 2 biopsy. Massive proteinuria and anuria were then observed. Two courses (2 × 2 times) of plasmapheresis and rituximab were administered, and the graft function gradually recovered...
July 2015: Nephrology
https://www.readbyqxmd.com/read/25973696/pre-transplant-angiotensin-receptor-ii-type-1-antibodies-and-risk-of-post-transplant-focal-segmental-glomerulosclerosis-recurrence
#19
COMPARATIVE STUDY
Muhammad A Mujtaba, Asif A Sharfuddin, Benita L Book, William C Goggins, Ali A Khalil, Dennis P Mishler, Johnathan A Fridell, Muhammad S Yaqub, Tim E Taber
Post-kidney transplant recurrence of focal segmental glomerulosclerosis (FSGS) is a major problem. AT1R is expressed on podocyte; its expression is elevated in the proteinuric state. Using an ELISA, we tested pre-transplant sera of 28 patients with history of idiopathic FSGS for anti-AT1R levels and serum soluble urokinase-type plasminogen activator receptor (suPAR) as a biomarker for risk of recurrence of FSGS. Sera from 11 patients with polycystic kidney disease (PKD) were used as controls. Twelve patients had biopsy proven post-transplant FSGS recurrence at 1...
July 2015: Clinical Transplantation
https://www.readbyqxmd.com/read/25690943/recurrent-focal-segmental-glomerulosclerosis-after-kidney-transplantation
#20
Rebecca Trachtman, Simranjeet S Sran, Howard Trachtman
Focal segmental glomerulosclerosis (FSGS) is an important cause of glomerular disease in children and adolescents and nearly 50 % of affected patients will progress to end-stage 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...
October 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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