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recurrent FSGS in kidney transplant

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https://www.readbyqxmd.com/read/29117638/renal-transplant-outcomes-in-primary-fsgs-compared-with-other-recipients-and-risk-factors-for-recurrence-a-national-review-of-the-irish-transplant-registry
#1
S Cormican, C Kennedy, P O'Kelly, B Doyle, A Dorman, A Awan, P J Conlon
INTRODUCTION: Primary focal segmental glomerular sclerosis (p-FSGS) is commonly complicated by recurrence (r-FSGS) post-transplantation. Our objective was to describe Irish outcomes for transplantation after ESRD due to p-FSGS, specifically rates of, and treatments for, r-FSGS. PATIENTS AND METHODS: Irish patients with biopsy proven FSGS were identified from the Irish National Kidney Transplant database (1982-2015). Medical record review was performed to identify predictors of r-FSGS and treatments for r-FSGS...
November 8, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/29038887/mutations-in-inf2-may-be-associated-with-renal-histology-other-than-focal-segmental-glomerulosclerosis
#2
Anja K Büscher, Nora Celebi, Peter F Hoyer, Hanns-Georg Klein, Stefanie Weber, Julia Hoefele
BACKGROUND: In 2010, INF2 mutations were associated with autosomal-dominant focal segmental glomerulosclerosis (FSGS), clinically presenting with moderate proteinuria in adolescence. However, in the meantime, cases with more severe clinical courses have been described, including progression to end-stage renal disease (ESRD) during childhood. INF2 mutations in patients with isolated FSGS are clustered in exons 2 to 4, encoding the diaphanous inhibitory domain, involved in the regulation of the podocyte actin cytoskeleton...
October 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28992235/treatment-by-immunoadsorption-for-recurrent-focal-segmental-glomerulosclerosis-after-paediatric-kidney-transplantation-a-multicentre-french-cohort
#3
Lise Allard, Theresa Kwon, Saoussen Krid, Justine Bacchetta, Arnaud Garnier, Robert Novo, Georges Deschenes, Rémi Salomon, Gwenaëlle Roussey, Emma Allain-Launay
Background: Primary focal segmental glomerulosclerosis (FSGS) frequently recurs after kidney transplantation (KTx) in children. This can lead to delayed graft loss. As the management of children with recurrent FSGS is not well established, apheresis strategies could be a cornerstone to control the disease. Immunoadsorption (IA) is a recent apheresis therapy. There have been few studies examining IA in this setting. We report the results of IA for management of recurrent FSGS after KTx in children in France...
July 28, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28832357/recurrent-glomerular-disease-after-kidney-transplantation
#4
Christopher D Blosser, Roy D Bloom
PURPOSE OF REVIEW: With improving short-term kidney transplant outcomes, recurrent glomerular disease is being increasingly recognized as an important cause of chronic allograft failure. Further understanding of the risks and pathogenesis of recurrent glomerular disease enable informed transplant decisions, along with the development of preventive and treatment strategies. RECENT FINDINGS: Multiple observational studies have highlighted differences in rates and outcomes for various recurrent glomerular diseases, although these rates have not markedly improved over the last decade...
November 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28658201/targeted-next-generation-sequencing-in-brazilian-children-with-nephrotic-syndrome-submitted-to-renal-transplant
#5
Luciana S Feltran, Patricia Varela, Elton D Silva, Camila L Veronez, Maria C Franco, Alvaro P Filho, Maria F Camargo, Paulo C Koch Nogueira, Joao B Pesquero
BACKGROUND: The aims of this study were to identify the genetic mutations profile in Brazilian children with nephrotic syndrome and to determine a genotype-phenotype correlation in this disease. METHODS: Next generation sequencing (NGS) and mutation analysis were performed on 24 genes related to nephrotic syndrome in a cross-sectional study involving 95 children who underwent kidney transplantation due to nephrotic syndrome, excluding congenital cases. RESULTS: A total of 149 variants were identified in 22 out of 24 sequenced genes...
June 28, 2017: Transplantation
https://www.readbyqxmd.com/read/28614191/anti-cd20-blocker-rituximab-in-kidney-transplantation
#6
Puneet Sood, Sundaram Hariharan
Rituximab is a chimeric anti-CD20 monoclonal protein used in various clinical scenarios in kidney transplant recipients. However, its evidence-based use there remains limited due to lack of controlled studies, limited sample size, short follow-up and poorly defined endpoints. Rituximab is indicated for CD20+ PTLD. It may be beneficial for treating recurrent MN and recurrent allograft ANCA vasculitis and possibly for recurrent FSGS. Rituximab, in combination with IVIG/PP, appears to decrease antibody level and increase the odds of transplantation in sensitized recipients...
June 13, 2017: Transplantation
https://www.readbyqxmd.com/read/28601198/recurrent-glomerulonephritis-after-kidney-transplantation-risk-factors-and-allograft-outcomes
#7
Penelope J Allen, Steve J Chadban, Jonathan C Craig, Wai H Lim, Richard D M Allen, Philip A Clayton, Armando Teixeira-Pinto, Germaine Wong
Recurrent glomerulonephritis after kidney transplantation is a feared complication because it is unpredictable and may have a negative impact on graft outcomes. To better understand this we collected data from the Australia and New Zealand Dialysis and Transplant (ANZDATA) Registry accumulated over 30 years. The incidence, risk factors, and outcomes of recurrent glomerulonephritis in transplant recipients were determined using adjusted Cox proportional hazard and competing risk modeling. A total of 6,597 recipients with biopsy-proven glomerulonephritis as the primary cause of end-stage kidney disease were followed for 51,871 person-years (median duration 7...
June 7, 2017: Kidney International
https://www.readbyqxmd.com/read/28560690/extrarenal-determinants-of-kidney-filter-function
#8
REVIEW
Eunsil Hahm, Vasil Peev, Jochen Reiser
The kidney is an organ involved in cross talk with many human organs. The link between the immune system and the kidney has been studied in some detail, although data precisely elucidating their interaction are sparse, in particular with regard to the function of the kidney filter apparatus. Current research suggests that an understanding of the impairment of this cross talk between the bone marrow, as a fundament of the immune system and the kidney will provide meaningful insights into the pathophysiological mechanisms of impaired kidney filter function...
July 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28546764/optimal-management-of-primary-focal-segmental-glomerulosclerosis-in-adults
#9
REVIEW
Séverine Beaudreuil, Hans Kristian Lorenzo, Michele Elias, Erika Nnang Obada, Bernard Charpentier, Antoine Durrbach
Focal segmental glomerulosclerosis (FSGS) is a frequent glomerular kidney disease that is revealed by proteinuria or even nephrotic syndrome. A diagnosis can be established from a kidney biopsy that shows focal and segmental glomerulosclerosis. This histopathological lesion may be caused by a primary podocyte injury (idiopathic FSGS) but is also associated with other pathologies (secondary FSGS). The first-line treatment for idiopathic FSGS with nephrotic syndrome is a prolonged course of corticosteroids. However, steroid resistance or steroid dependence is frequent, and despite intensified immunosuppressive treatment, FSGS can lead to end-stage renal failure...
2017: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/28540902/primary-focal-segmental-glomerulosclerosis-recurring-rapidly-as-collapsing-glomerulopathy-in-a-renal-allograft-recipient
#10
Vinita Agrawal, Narayan Prasad, Pritpal Singh
Recurrent focal segmental glomerulosclerosis (FSGS) develops in about 30%-40% of patients of FSGS undergoing renal transplantation. We report a patient who received a live- related renal transplant for end-stage renal disease due to a primary FSGS (not otherwise specified) in the native kidney and presented with graft dysfunction in the immediate posttransplant period. The first and the second biopsy showed no evidence of rejection or glomerular lesion. A repeat biopsy done on the 30th day revealed recurrent FSGS morphologically presenting as collapsing variant...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28370393/removal-of-focal-segmental-glomerulosclerosis-fsgs-factor-supar-using-cytosorb
#11
Heiko Schenk, Janina Müller-Deile, Roland Schmitt, Jan Hinrich Bräsen, Hermann Haller, Mario Schiffer
Treatment of primary focal segmental glomerulosclerosis (FSGS) and its recurrence after kidney transplantation associated with rapid deterioration of kidney function remains to be challenging despite advances in immunosuppressive therapy. The presence of circulating factors has been postulated to be a pivotal player in the pathogenesis of FSGS, although suPAR and CLCF-1 have been identified as the most promising causative factors. The potential therapeutic effect of suPAR elimination in an FSGS patient using CytoSorb, a hemoadsorption device that gained attention in the cytokine elimination in septic patients, was studied...
March 29, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28333603/focal-segmental-glomerulosclerosis-in-a-patient-with-ambiguous-genitalia-a-diagnostic-dilemma
#12
Oliver E Ross, Dean A Kujubu, John J Sim
The renal condition referred to as focal segmental glomerulosclerosis (FSGS) presents a diagnostic dilemma for the clinician. It encompasses and displays a nonspecific histologic appearance on a kidney biopsy specimen, rather than a unique disease entity. This characteristic of FSGS often makes treatment decisions and prognostication difficult. A 34-year-old man, who was born with ambiguous genitalia, had received a diagnosis of FSGS in young adulthood and now had advanced kidney disease. He underwent genetic testing to determine whether a genetic disorder was underlying his kidney disease and to ascertain his risk of FSGS recurrence if he were to receive a kidney transplant...
2017: Permanente Journal
https://www.readbyqxmd.com/read/28242845/focal-segmental-glomerulosclerosis
#13
Avi Z Rosenberg, Jeffrey B Kopp
Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease worldwide. The presumed etiology of primary FSGS is a plasma factor with responsiveness to immunosuppressive therapy and a risk of recurrence after kidney transplant-important disease characteristics. In contrast, adaptive FSGS is associated with excessive nephron workload due to increased body size, reduced nephron capacity, or single glomerular hyperfiltration associated with certain diseases. Additional etiologies are now recognized as drivers of FSGS: high-penetrance genetic FSGS due to mutations in one of nearly 40 genes, virus-associated FSGS, and medication-associated FSGS...
March 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28213687/ofatumumab-for-the-treatment-of-childhood-nephrotic-syndrome
#14
Chia-Shi Wang, Rochelle Schmidt Liverman, Rouba Garro, Roshan Punnoose George, Anastacia Glumova, Alana Karp, Stephanie Jernigan, Barry Warshaw
BACKGROUND: Ofatumumab is a humanized anti-CD20 monoclonal antibody that has recently garnered interest as a potential therapeutic agent for nephrotic syndrome. We report our center's experience in administering ofatumumab to five pediatric patients with idiopathic nephrotic syndrome. METHODS: Between March 2015 and November 2016, five patients were treated with ofatumumab. One patient had post-transplant recurrent focal segmental glomerulosclerosis (FSGS) which had been resistant to plasmapheresis and numerous immunosuppressive agents...
May 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28203563/unsuccessful-treatment-with-abatacept-in-recurrent-focal-segmental-glomerulosclerosis-after-kidney-transplantation
#15
Tilde Kristensen, Per Ivarsen, Johan Vestergaard Povlsen
Recurrence of focal segmental glomerulosclerosis (FSGS) after renal transplantation occurs in up to 20-50% of FSGS patients and is associated with inferior allograft survival. Treatment of both primary FSGS as well as recurrent FSGS after transplantation with plasma exchange and immunosuppression is often unsuccessful and remains a major challenge as the disease still leads to end-stage renal disease and decreased graft survival. Previous case reports have described patients with recurrent FSGS who were successfully treated with a B7-1 inhibitor (abatacept) inducing partial or complete remission...
January 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28117207/coq6-mutations-in-children-with-steroid-resistant-focal-segmental-glomerulosclerosis-and-sensorineural-hearing-loss
#16
Eujin Park, Yo Han Ahn, Hee Gyung Kang, Kee Hwan Yoo, Nam Hee Won, Kyoung Bun Lee, Kyung Chul Moon, Moon-Woo Seong, Tae Rin Gwon, Sung Sup Park, Hae Il Cheong
The phenotypic combination of steroid-resistant focal segmental glomerulosclerosis (SR-FSGS) and sensorineural hearing loss has been mainly reported in patients with mitochondrial cytopathies, including primary coenzyme Q10 (CoQ10) deficiency. In this report of 10 children with SR-FSGS and sensorineural hearing loss, we found 6 patients with biallelic COQ6 mutations. Median age at the onset of nephrotic syndrome was 29 (range, 15-47) months. All patients progressed to end-stage renal disease within a median of 13 (range, 1-27) months after the onset...
July 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27797890/long-term-outcome-of-kidney-transplantation-in-recipients-with-focal-segmental-glomerulosclerosis
#17
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
#18
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
#19
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
#20
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
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