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Fsgs transplant

Antonia Bouts, Floor Veltkamp, Burkhard Tönshoff, Marina Vivarelli
INTRODUCTION: Primary FSGS is an important cause of ESRD in children. FSGS recurrence after kidney transplantation is associated with early graft loss. No guidelines for treatment of FSGS recurrence exist. We conducted a survey to gain insight into variation of treatment between centers. METHODS: A survey was sent to all members of the ESPN on behalf of the "Renal Transplantation" and "Idiopathic Nephrotic Syndrome" working groups. RESULTS: Fifty-nine nephrologists from 31 countries responded, reporting 807 FSGS patients, with 241 (30%) FSGS recurrences after transplantation...
March 1, 2019: Pediatric Transplantation
Asako Hayashi, Takayuki Okamoto, Takeshi Yamazaki, Yasuyuki Sato, Toshiyuki Takahashi, Tadashi Ariga
BACKGROUND/AIMS: Calcineurin inhibitors (CNIs) such as cyclosporine A (CsA) and tacrolimus are immunosuppressants that are frequently used as "key drugs" in the fields of transplantation, steroid resistance, refractory nephrotic syndrome, and autoimmune disease. However, long-term CNI use causes nephrotoxicity, the features of which are arteriolar hyalinosis, tubular atrophy, striped interstitial fibrosis, and focal segmental glomerulosclerosis (FSGS). We investigated whether FSGS in CNI-induced nephrotoxicity is associated with CD44-positive glomerular parietal epithelial cells (PECs), which play a significant role in experimental and human FSGS pathogenesis...
February 20, 2019: Nephron
Pankaj Srivastava, Ashish K Solanki, Ehtesham Arif, Bethany J Wolf, Michael G Janech, Milos N Budisavljevic, Sang-Ho Kwon, Deepak Nihalani
Definitive diagnosis of glomerular disease requires a kidney biopsy, an invasive procedure that may not be safe or feasible to perform in all patients. We developed a noninvasive, accurate, and economical diagnostic assay with easy commercial adaptability to detect recurrent focal segmental glomerulosclerosis (rFSGS) after kidney transplant. Since FSGS involves podocyte damage and death, our approach involved mRNA profiling of cultured podocytes treated with plasma from patients with rFSGS to identify upregulated genes involved in podocyte damage...
January 26, 2019: Kidney International
Efrat Harel, Jun Shoji, Vivek Abraham, Loan Miller, Zoltan Laszik, Tine Thurison, Andrew King, Adam Olshen, Joey Leung, Gyula Szabo, Byron Hann, Gunilla Høyer-Hansen, Charles S Craik, Flavio Vincenti
BACKGROUND: We investigated circulating levels of individual soluble urokinase plasminogen activation receptor (suPAR) forms to determine if specific circulating fragments of suPAR (II-III) and (I) can better serve as clinical biomarkers for FSGS and the risk of recurrence after transplantation. MATERIALS AND METHODS: Serum levels of intact suPAR and its cleaved forms were measured with 2 assays, ELISA and TR-FIA 2. RESULTS: suPAR levels in healthy controls were significantly lower than those who had glomerular diseases but were not significantly different between FSGS patients and glomerular controls...
January 28, 2019: Clinical Transplantation
Konstantinos Koutroutsos, Rawya Charif, Linda Moran, Jill Moss, Terence Cook, Candice Roufosse, Charles Pusey, David Taube, Marina Loucaidou
BACKGROUND: Post-transplant focal segmental glomerulosclerosis (FSGS) is associated with renal allograft loss. Currently, optimal treatment remains controversial. METHODS: The aim of our study was to examine the efficacy and safety of therapeutic plasma exchange (TPE), and rituximab (RTX), in the management of post-transplant FSGS. The treatment protocol consisted of RTX and monthly cycles of 5 plasma exchanges for 6 months. We treated 10 transplant recipients with biopsy-proven post-transplant FSGS...
January 14, 2019: Clinical and Experimental Nephrology
D Argiolas, E Carta, G Mascia, M B Michittu, G B Piredda
BACKGROUND: Recurrence of focal segmental glomerulosclerosis (FSGS) in renal allograft recipients after first transplant occurs in the second graft in virtually all patients. There is little evidence regarding optimal treatment. CASE PRESENTATION: A 55-year-old man with primary FSGS and disease recurrence in both the first and the second kidney grafts is presented. In 1999, the patient developed FSGS 3 years after transplant, which was treated with plasmapheresis and cyclophosphamide...
January 2019: Transplantation Proceedings
R M Hanna, U Selamet, H Hasnain, M El-Masry, S Saab, W D Wallace, B Yanny, J Wilson
Transplant patients are at risk for hemodynamic injury and glomerular diseases such as focal segmental glomerulosclerosis (FSGS) and thrombotic microangiopathy (TMA). Calcineurin inhibitors (CNI) can cause various patterns of acute kidney injury (AKI) in transplant patients and their effects must be differentiated from kidney injury due to other agents. Transplant populations are also at risk for atypical infections and malignancies. These conditions and the agents that are used to treat them can then induce their own set of glomerular diseases...
December 2018: Transplantation Proceedings
T Seeman, K Vondrak
Mutations in nucleoporin 93 (NUP93) gene have been shown recently to be one of the very rare causes of genetic steroid-resistant nephrotic syndrome (SRNS). Until now, none of the 7 published cases with NUP93-SRNS, experienced recurrence of nephrotic syndrome (NS) after transplantation. Here, we present the first case of recurrent NS in a patient with NUP93-SRNS ever reported. A 3-year-old boy with infantile SRNS was started on chronic peritoneal dialysis because of end-stage renal failure owing to biopsy-proven focal segmental glomerulosclerosis (FSGS)...
December 2018: Transplantation Proceedings
Nada Alachkar, Jing Li, Dany Matar, Vikas Vujjini, Sami Alasfar, Melissa Tracy, Jochen Reiser, Changli Wei
BACKGROUND: Therapeutic plasma exchange (TPE) is an important therapy for recurrent focal segmental glomerulosclerosis (rFSGS) post kidney transplant. suPAR has been causally implicated in rFSGS, and shown to be a unique biomarker for the occurrence and progression of chronic kidney disease. This study was targeted to evaluate the application of monitoring suPAR in TPE treated rFSGS. METHODS: A retrospective (n = 19) and a prospective (n = 15) cohort of post transplant FSGS patients treated with TPE and rituximab were enrolled...
December 17, 2018: BMC Nephrology
S H Jiang, A L Kennard, G D Walters
BACKGROUND: Recurrence of primary glomerulonephritis in the post-transplant period has been described in the literature but the risk remains poorly quantified and its impact on allograft outcomes and implications for subsequent transplants remain under-examined. Here we describe the rates and timing of post-transplant glomerulonephritis recurrence for IgA nephropathy, focal segmental glomerulosclerosis, mesangiocapillary GN and membranous GN based on 28 years of ANZDATA registry transplant data...
December 3, 2018: BMC Nephrology
Ru-Chun Yang, Xiao-Ling Zhu, Jun Wang, Feng Wan, Hua-Qin Zhang, Yi Lin, Xuan-Li Tang, Bin Zhu
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is the most common glomerular etiology of end-stage kidney disease (ESKD). Increasing evidence has indicated the reparative potential of mesenchymal stem cells (MSCs) in damaged diseased kidneys. However, the effect of bone marrow mesenchymal stem cells (BMSCs) on the FSGS progression remains unclear. This study aimed to investigate the protective effects of BMSCs on FSGS progression. METHODS: A rat model of FSGS was generated via unilateral nephrectomy plus adriamycin injection...
November 22, 2018: BMC Nephrology
Natàlia Puig-Gay, Conxita Jacobs-Cacha, Joana Sellarès, Lluís Guirado, Francisco González Roncero, Carlos Jiménez, Sofía Zárraga, Javier Paul, Ricardo Lauzurica, Ángel Alonso, Ana Fernández, Isabel Beneyto, Auxiliadora Mazuecos, Domingo Hernández, Alberto Rodriguez-Benot, Antonio Franco, Luisa Jimeno, Marta Crespo, Anna Meseguer, Francesc Moreso, Daniel Seron, Joan Lopez-Hellin, Carmen Cantarell
Recurrence of idiopathic focal segmental glomerulosclerosis (FSGS) is a serious complication after kidney transplantation. FSGS relapse is suspected by a sudden increase in proteinuria but there is not an accurate noninvasive diagnostic tool to confirm this entity or to detect patients at risk. We aimed to validate the diagnostic performance of ApoA-Ib to detect FSGS relapses by measuring urinary ApoA-Ib in a retrospective cohort of 61 kidney transplanted patients (37 FSGS and 24 non-FSGS). In addition, to assess the ApoA-Ib predictive ability, ApoA-Ib was measured periodically in a prospective cohort of 13 idiopathic FSGS patients who were followed during 1 year after transplantation...
March 2019: Transplant International: Official Journal of the European Society for Organ Transplantation
Olivier Gribouval, Olivia Boyer, Aurélie Hummel, Jacques Dantal, Frank Martinez, Rebecca Sberro-Soussan, Isabelle Etienne, Dominique Chauveau, Michel Delahousse, Arnaud Lionet, Julien Allard, Claire Pouteil Noble, Marie-Josèphe Tête, Laurence Heidet, Corinne Antignac, Aude Servais
Monogenic forms of Steroid-Resistant Nephrotic Syndrome (SRNS) have been widely characterized, but genetic screening paradigms preferentially address congenital, infantile onset, and familial cases. Our aim was to characterize the distribution of disease-causing gene mutations in adults with sporadic SRNS or focal segmental glomerulosclerosis (FSGS). We selected adult patients with non-syndromic, biopsy-proven FSGS or SRNS in the absence of known family history. Strict clinical criteria included lack of response to glucocorticoids and cyclosporine, and no recurrence after kidney transplantation...
November 2018: Kidney International
S Jung, K-M Park, J Hah, K C Hong, S-D Hwang, J Song
Focal segmental glomerulosclerosis (FSGS) is the most common form of post-transplant glomerulonephritis. We describe a case where a biopsy proved that early recurrence of FSGS on postoperative day 1 was the cause of delayed graft function. A 39-year-old man, on hemodialysis for 15 years due to polycystic kidney disease, received a cadaveric renal transplantation. On postoperative day 1, his hourly urine output decreased from 700-800 mL to 50 mL. The graft biopsy showed a mild acute kidney injury confusing nephrotic syndrome...
October 2018: Transplantation Proceedings
R Deng, Y Dai, H Zhang, L Liu, J Li, Y Xiong, S Deng, Q Fu, C Wang
Glomerulonephritis recurrence has emerged as one of the leading causes of allograft loss. We aimed to investigate the effect of living-related and deceased donation on the incidence of renal allograft glomerulonephritis and its effect on renal allograft survival. METHODS: Adult renal allograft recipients with primary glomerulonephritis were enrolled. Transplantation date was from Feb 2004 to Dec 2015. Exclusion criteria included combined organ transplantation, structural abnormality, diabetic nephropathy, hypertension nephropathy, obstructive nephropathy, and primary uric acid nephropathy...
October 2018: Transplantation Proceedings
Jae-Hyung Chang, S Ali Husain, Dominick Santoriello, Michael B Stokes, Clifford D Miles, Kirk W Foster, Yifu Li, Leigh-Anne Dale, Russell J Crew, David J Cohen, Krzysztof Kiryluk, Ali G Gharavi, Sumit Mohan
The presence of 2 APOL1 risk variants (G1/G1, G1/G2, or G2/G2) is an important predictor of focal segmental glomerulosclerosis (FSGS) and chronic kidney disease in individuals of African descent. Although recipient APOL1 genotype is not associated with allograft survival, kidneys from deceased African American donors with 2 APOL1 risk variants demonstrate shorter graft survival. We present a series of cases of presumed de novo collapsing FSGS in 5 transplanted kidneys from 3 deceased donors later identified as carrying 2 APOL1 risk alleles, including 2 recipients from the same donor whose kidneys were transplanted in 2 different institutions...
January 2019: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
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
BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome (SRNS) due to focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is a leading cause of end-stage kidney disease in children. Recurrence of primary disease following transplantation is a major cause of allograft loss. The clinical determinants of disease recurrence are not completely known. Our objectives were to determine risk factors for recurrence of FSGS/MCD following kidney transplantation and factors that predict response to immunosuppression following recurrence...
October 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Parnaz Daneshpajouhnejad, Erfan Behzadi, Sanaz Amoushahi, Ahmadreza Aghabozorgi, Aida Farmani, Sayed-Mohsen Hosseini, Diana Taheri
Native kidney biopsy reports in previous studies that are mostly originated in Western countries show various results in different parts of the world. In this study, we aimed to determine the prevalence of renal biopsy disorders in Iran and compare it with that of other studies in the world. This cross-sectional study evaluated consecutive native kidney biopsies performed in four centers in Isfahan, Iran, from 2009 to 2014. We also reviewed other relevant studies in Iran and the world. Overall, 1547 renal biopsies were reviewed; 493 cases were excluded (transplant or re-biopsy cases) and 1054 cases (43...
May 2018: Saudi Journal of Kidney Diseases and Transplantation
Izumi Yamamoto, Takafumi Yamakawa, Ai Katsuma, Mayuko Kawabe, Haruki Katsumata, Aki Mafune Hamada, Yasuyuki Nakada, Akimitsu Kobayashi, Hiroyasu Yamamoto, Takashi Yokoo
The extent of recurrence of original kidney disease after kidney transplantation has been underestimated for several reasons. First, the duration of observation varies among studies. Second, the criteria used to schedule protocol and episode biopsies differ among institutions. And third, diagnostic modalities used for early detection of recurrent original kidney disease also vary. Thus, rates of graft loss attributable to a recurrence of original kidney disease vary among institutions and are often underestimated...
July 2018: Nephrology
Katrin Kienzl-Wagner, Alejandra Rosales, Stefan Scheidl, Thomas Giner, Claudia Bösmüller, Michael Rudnicki, Rupert Oberhuber, Christian Margreiter, Afschin Soleiman, Dietmar Öfner, Siegfried Waldegger, Stefan Schneeberger
Primary focal segmental glomerulosclerosis (FSGS) recurs in up to 55% of patients after kidney transplantation. Herein we report the successful management of recurrent FSGS. A 5-year-old boy with primary FSGS received a deceased donor renal transplant. Immediate and fulminant recurrence of FSGS caused anuric graft failure that was resistant to plasmapheresis and rituximab. After exclusion of structural or immunologic damage to the kidney by repeated biopsies, the allograft was retrieved from the first recipient on day 27 and transplanted into a 52-year-old second recipient who had vascular nephropathy...
November 2018: American Journal of Transplantation
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