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Rituximab and nephrotic

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https://www.readbyqxmd.com/read/27900977/unconventional-strategies-in-the-battle-of-focal-and-segmental-glomerulosclerosis
#1
Satish Mendonca, R S V Kumar, Devika Gupta, Pooja Gupta, Satish Barki, M L Sharma
A 24-year-old male presented with classic features of the nephrotic syndrome. An initial renal biopsy revealed minimal change disease and thereafter, a second biopsy showed features of focal and segmental glomerulosclerosis. There was no response to conventional immunosuppression, and the patient had to be given rituximab; in spite of this, he went on to develop end-stage renal disease. He continued to have heavy proteinuria leading to severe hypoalbuminemia, thrombosis, infections, and malnutrition, placing the patient in a life-threatening situation...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27812262/membranoproliferative-glomerulonephritis-secondary-to-a-low-grade-lymphoproliferative-disorder-a-rare-cause-of-renal-dysfunction
#2
Tushar Sehgal, Nidhi Jain, Gaurav Prakash, Ritambhra Duseja, Neelam Varma
Chronic lymphocytic leukemia is a B-cell neoplasm with an indolent clinical course. Most patients are asymptomatic and are diagnosed incidentally on a routine blood count. The malignant cells of this low-grade neoplasm infiltrate various organs and tissues. However, the resultant end organ damage is a rare phenomenon. Here we describe a case of chronic lymphocytic leukemia that presented with an unusual cause of acute renal dysfunction. The patient had deranged renal parameters with a nephrotic range proteinuria...
December 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27726125/interventions-for-idiopathic-steroid-resistant-nephrotic-syndrome-in-children
#3
REVIEW
Elisabeth M Hodson, Sophia C Wong, Narelle S Willis, Jonathan C Craig
BACKGROUND: The majority of children who present with their first episode of nephrotic syndrome achieve remission with corticosteroid therapy. Children who fail to respond may be treated with immunosuppressive agents including calcineurin inhibitors (cyclosporin or tacrolimus) and with non-immunosuppressive agents such as angiotensin-converting enzyme inhibitors (ACEi). Optimal combinations of these agents with the least toxicity remain to be determined. This is an update of a review first published in 2004 and updated in 2006 and 2010...
October 11, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27692613/-membranous-nephropathy-diagnosis-new-insights-in-pathophysiology-and-therapeutic-approach
#4
K Dahan
Membranous nephropathy (MN) accounts for about 20% of cases of nephrotic syndrome in the adult. Thickening of glomerular capillary walls results from subepithelial formation of immune deposits containing IgG and the membrane attack complex of complement, which is the major mediator of proteinuria, and antigens. Idiopathic forms of MN (IMN) represent 70 to 80% of all cases. A major breakthrough was the identification of the podocyte antigen PLA2R as the target of circulating antibodies in about 70% of IMN, which confirmed that the disease was auto-immune in nature...
May 25, 2016: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/27687621/ofatumumab-in-two-pediatric-nephrotic-syndrome-patients-allergic-to-rituximab
#5
Marina Vivarelli, Manuela Colucci, Alice Bonanni, Martina Verzani, Jessica Serafinelli, Francesco Emma, Gianmarco Ghiggeri
BACKGROUND: Rituximab, a chimeric anti-CD20 monoclonal antibody, is an effective treatment in steroid-dependent nephrotic syndrome (SDNS). However, some patients develop adverse reactions. CASE-DIAGNOSIS/TREATMENT: Patient 1, a 14-year-old boy with SDNS since the age of 2, was treated with oral prednisone, cyclosporine A (CsA) and mycophenolate mofetil. A first infusion of rituximab at age 12 years was well tolerated, but this was followed by a prolonged relapse unresponsive to oral prednisone, mycophenolate mofetil and CsA...
September 29, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27682967/idiopathic-nephrotic-syndrome-the-ebv-hypothesis
#6
REVIEW
Claire Dossier, Agnès Jamin, Georges Deschênes
Steroid sensitive nephrotic syndrome is marked by a massive proteinuria and loss of podocytes foot processes. The mechanism of the disease remains debated but recent publications suggest a primary role of Epstein-Barr Virus (EBV). EBV replication in the peripheral blood is found in 50% of patients during the first flare of the disease. The genetic locus of steroid sensitive nephrotic syndrome was also identified as influencing antibodies directed against EBNA1. EBV is able to establish, latent benign infection in memory B cells that display phenotypes similar to antigen-selected memory B cells...
November 9, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27676651/rituximab-in-membranous-nephropathy-not-all-studies-are-created-equal
#7
Paolo Cravedi
Many prospective studies and a recent randomized controlled trial have shown that the B-cell-depleting monoclonal antibody, rituximab, safely promotes the remission of nephrotic syndrome in approximately 65% of patients with membranous nephropathy (MN). Mechanistic studies have indicated that rituximab-induced proteinuria reduction is associated with clearance of anti-podocyte antigens phospholipase 2 receptor autoantibodies and subepithelial immune complexes, the hallmarks of the disease. A recently published study reported results which, at first sight, looked less favorable and implied that, due to a publication bias against negative results, the efficacy of rituximab in MN might be overestimated...
September 28, 2016: Nephron
https://www.readbyqxmd.com/read/27671102/eltrombopag-thrombopoietin-receptor-agonist-and-plasmapheresis-as-rescue-therapy-of-acute-post-renal-transplant-immune-thrombocytopenia-in-a-child-with-schimke-immuno-osseous-dysplasia-case-report
#8
Ryszard Grenda, Wioletta Jarmużek, Joanna Latoszyńska, Sylwester Prokurat, Jacek Rubik
SIOD is rare disorder related to SMARCAL1 or SMARCAL2 gene mutation, including (among other comorbidities) T-cell immunodeficiency, nephrotic syndrome, and renal failure. Up to 22% of primary patients may develop various autoimmune disorders. We report the case of 11-year-old male with SIOD, who presented ITP at 2 years after renal transplantation with decrease in platelet count (from normal) to 56 000/μL and then (gradually) to 2000/μL. There was no effect of iv. methylprednisolone/dexamethasone. As the presence of antibodies against GPIIb/IIIa, GPIb, and GPIaIIa platelet glycoproteins was confirmed, patient was given 50 g of IVIG and then was put on plasmapheresis; however, both showed poor direct effect...
September 26, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27558467/predictors-of-relapse-and-long-term-outcome-in-children-with-steroid-dependent-nephrotic-syndrome-after-rituximab-treatment
#9
Shuichiro Fujinaga, Daishi Hirano, Akira Mizutani, Koji Sakuraya, Akifumi Yamada, Shunsuke Sakurai, Toshiaki Shimizu
BACKGROUND: In patients with complicated steroid-dependent nephrotic syndrome (SDNS), rituximab (RTX) followed by immunosuppressive agent (IS) can maintain remission without the use of prednisolone (PSL). However, available data on the predictive factors for relapse and the long-term outcome after this protocol are few. METHODS: We retrospectively analyzed 43 SDNS patients who were followed-up for a long time (>2 years, mean 5.4 years) after a single dose of RTX (375 mg/m(2)) from September 2007...
August 24, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27536673/management-of-membranous-nephropathy-in-western-countries
#10
REVIEW
Talal Alfaadhel, Daniel Cattran
BACKGROUND: Idiopathic membranous nephropathy (IMN) is a common cause of nephrotic syndrome (NS) in adults in Western countries. In 2012, the KDIGO (Kidney Disease: Improving Global Outcomes) working group published guidelines for the management of glomerulonephritis, thus providing a template for the treatment of this condition. While being aware of the impact of the clinicians' acumen and that patients may choose a different therapeutic option due to the risks of specific drugs and also of the evolving guidelines, this review details our approach to the management of patients with IMN in a Western center (Toronto)...
September 2015: Kidney Diseases
https://www.readbyqxmd.com/read/27536672/management-of-membranous-nephropathy-in-asia
#11
REVIEW
Jing Xu, Xiaofan Hu, Jingyuan Xie, Nan Chen
BACKGROUND: Membranous nephropathy (MN) is the most common cause of nephrotic syndrome (NS) in adults, accounting for about 20.0% of all NS cases. With an increasing prevalence, especially in the elderly, it has received great attention in Asia. SUMMARY: Recently, the prevalence of idiopathic MN (IMN) has significantly increased among the elderly people in Asia and other places in the world. Although the exact mechanism of IMN remains unveiled, the identification of new antigens such as PLA2R and THSD7A has greatly enhanced our understanding of its pathogenesis...
September 2015: Kidney Diseases
https://www.readbyqxmd.com/read/27446549/single-dose-of-rituximab-in-children-with-steroid-dependent-minimal-change-nephrotic-syndrome
#12
Xiao-Ling Niu, Sheng Hao, Ping Wang, Wei Zhang, Gui-Mei Guo, Ying Wu, Xin-Yu Kuang, Guang-Hua Zhu, Wen-Yan Huang
Rituximab (RTX) can be used in children with nephrotic syndrome, particularly in those with steroid-dependent nephrotic syndrome (SDNS). However, at present there is no unified standard of how to use RTX, with regard to the amount of doses and frequency, in children with nephrotic syndrome. The study aimed to investigate the therapeutic efficacy of a single dose of RTX in children with steroid-dependent minimal change nephrotic syndrome (SD-MCNS). The patients with biopsy-proven minimal change disease (MCD) and clinical features of SDNS received a single dose of RTX (375 mg/m(2))...
August 2016: Biomedical Reports
https://www.readbyqxmd.com/read/27424706/minimal-change-disease-a-case-report-of-an-unusual-relationship
#13
Fahad Edrees, Robert M Black, Laszlo Leb, Helmut Rennke
Kidney injury associated with lymphoproliferative disorders is rare, and the exact pathogenetic mechanisms behind it are still poorly understood. Glomerular involvement presenting as a nephrotic syndrome has been reported, usually secondary to membranoproliferative glomerulonephritis. We report a case of a 63-year-old male who presented with bilateral leg swelling due to nephrotic syndrome and acute kidney injury. A kidney biopsy showed minimal change disease with light chain deposition; however, no circulating light chains were present...
July 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27422620/rituximab-for-nephrotic-syndrome-in-children
#14
Kazumoto Iijima, Mayumi Sako, Kandai Nozu
Idiopathic nephrotic syndrome is the most common chronic glomerular disease in children. At least 20 % of children with this syndrome show frequent relapses and/or steroid dependence during or after immunosuppressive therapies, a condition defined as complicated frequently relapsing/steroid-dependent nephrotic syndrome (FRNS/SDNS). Approximately 1-3 % of children with idiopathic nephrotic syndrome are resistant to steroids and all immunosuppressive agents, a condition defined as refractory steroid-resistant nephrotic syndrome (SRNS); these SRNS children have a high risk of end-stage renal failure...
July 15, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27409338/rituximab-in-children-with-steroid-dependent-nephrotic-syndrome-experience-of-a-tertiary-center-and-review-of-the-literature
#15
Ilse Van Horebeek, Noël Knops, Maria Van Dyck, Elena Levtchenko, Djalila Mekahli
OBJECTIVES: Rituximab (RTX) is a new treatment option in children with difficult-to-treat steroid-dependent nephrotic syndrome (SDNS). We evaluated the experience of our tertiary center and reviewed the current literature. METHODS: This is a retrospective single-center study evaluating the efficacy and safety of RTX in children with difficult-to-treat SDNS. Age at diagnosis, type and duration of immunosuppression, age at administration, dose of RTX, possible adverse events, number of relapses, duration of remission, and B-cell count after administration of RTX were analyzed...
July 13, 2016: Acta Clinica Belgica
https://www.readbyqxmd.com/read/27405390/the-efficacy-and-safety-of-rituximab-in-treating-childhood-nephrotic-syndrome-an-italian-perspective
#16
EDITORIAL
Dario Maratea, Monica Bettio, Maria Grazia Corti, Giovanni Montini, Francesca Venturini
BACKGROUND: Nephrotic syndrome is a disorder characterized by proteinuria, hypoalbuminemia and dyslipidemia. Low-dose alternate-day steroid regimen is the standard of care. In case of relapse or significant adverse events, steroid-sparing agents may be used. This analysis was aimed at assessing the efficacy and safety of rituximab for the treatment of children with nephrotic syndrome. RESULTS: Four studies were included in the final meta-analysis. The end-point of our analysis was the percentage of patients in remission at 6 months...
2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27387472/low-dose-rituximab-is-poorly-effective-in-patients-with-primary-membranous-nephropathy
#17
Gabriella Moroni, Federica Depetri, Lucia Del Vecchio, Beniamina Gallelli, Francesca Raffiotta, Elisa Giglio, Francesca Brunini, Marco D'Amico, Selena Longhi, Antonella Radice, Piergiorgio Messa, Renato Alberto Sinico
BACKGROUND: The optimal dosing and the efficacy of rituximab for primary membranous nephropathy (PMN) has not been established. This multicentric prospective study evaluates the efficacy and safety of low-dose rituximab (RTX) therapy in patients with PMN in clinical practice. METHODS: Thirty-four consecutive patients with PMN and nephrotic syndrome were included and received RTX (375 mg/m(2)) once (18 patients) or twice (16 patients). RTX was the first-line therapy for 19 (56%) and the second line for 15 (44%) patients...
July 6, 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/27364906/rituximab-is-not-a-magic-drug-in-post-transplant-recurrence-of-nephrotic-syndrome
#18
Ryszard Grenda, Wioletta Jarmużek, Jacek Rubik, Barbara Piątosa, Sylwester Prokurat
UNLABELLED: Pediatric patients with end-stage renal failure due to severe drug-resistant nephrotic syndrome are at risk of rapid recurrence after renal transplantation. Treatment options include plasmapheresis, high-dose of cyclosporine A/methylprednisolone and more recently-rituximab (anti-B CD20 monoclonal depleting antibody). We report five patients with immediate (1-2 days) post-transplant recurrence of nephrotic syndrome, treated with this kind of combined therapy including 2-4 weekly doses of 375 mg/m(2) of rituximab...
September 2016: European Journal of Pediatrics
https://www.readbyqxmd.com/read/27352623/rituximab-for-severe-membranous-nephropathy-a-6-month-trial-with-extended-follow-up
#19
Karine Dahan, Hanna Debiec, Emmanuelle Plaisier, Marine Cachanado, Alexandra Rousseau, Laura Wakselman, Pierre-Antoine Michel, Fabrice Mihout, Bertrand Dussol, Marie Matignon, Christiane Mousson, Tabassome Simon, Pierre Ronco
Randomized trials of rituximab in primary membranous nephropathy (PMN) have not been conducted. We undertook a multicenter, randomized, controlled trial at 31 French hospitals (NCT01508468). Patients with biopsy-proven PMN and nephrotic syndrome after 6 months of nonimmunosuppressive antiproteinuric treatment (NIAT) were randomly assigned to 6-month therapy with NIAT and 375 mg/m(2) intravenous rituximab on days 1 and 8 (n=37) or NIAT alone (n=38). Median times to last follow-up were 17.0 (interquartile range, 12...
June 27, 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27279884/pathogenesis-of-minimal-change-nephrotic-syndrome-an-immunological-concept
#20
REVIEW
Seong Heon Kim, Se Jin Park, Kyoung Hee Han, Andreas Kronbichler, Moin A Saleem, Jun Oh, Beom Jin Lim, Jae Il Shin
Idiopathic nephrotic syndrome (INS) in children is characterized by massive proteinuria and hypoalbuminemia. Minimal change nephrotic syndrome (MCNS) is the most common form of INS in children. The pathogenesis of MCNS still remains unclear, however, several hypotheses have been recently proposed. For several decades, MCNS has been considered a T-cell disorder, which causes the impairment of the glomerular filtration barrier with the release of different circulating factors. Increased levels of several cytokines are also suggested...
May 2016: Korean Journal of Pediatrics
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