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idiopathic nephrotic syndrome

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https://www.readbyqxmd.com/read/28213687/ofatumumab-for-the-treatment-of-childhood-nephrotic-syndrome
#1
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...
February 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210837/toll-like-receptor-3-tlr-3-tlr-4-and-cd80-expression-in-peripheral-blood-mononuclear-cells-and-urinary-cd80-levels-in-children-with-idiopathic-nephrotic-syndrome
#2
Om P Mishra, Ravindra Kumar, Gopeshwar Narayan, Pradeep Srivastava, Abhishek Abhinay, Rajniti Prasad, Ankur Singh, Vineeta V Batra
BACKGROUND: The aims of this study were (1) to detect toll-like receptor (TLR)-3, TLR-4 and CD80 expression in peripheral blood mononuclear cells (PBMCs) and estimate urinary CD80 levels in children with idiopathic nephrotic syndrome and (2) to investigate the utility of these markers to differentiate between biopsy-proven minimal change disease (MCD) and focal segmental glomeruloscelerosis (FSGS). METHODS: The study included 70 patients with idiopathic nephrotic syndrome (NS), of whom 40 had steroid-sensitive NS (SSNS; 25 with active NS, 15 in remission) and 30 had steroid-resistant NS (SRNS) patients, and 23 healthy controls...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28176955/urinary-excretion-of-il-1%C3%AE-il-6-and-il-8-cytokines-during-relapse-and-remission-of-idiopathic-nephrotic-syndrome
#3
Amal A Al-Eisa, Maysoun Al Rushood, Rajaa J Al-Attiyah
BACKGROUND/AIM: The role of pro-inflammatory cytokines in the immunopathogenesis of idiopathic nephrotic syndrome had been widely postulated. Reports on the release of cytokines, during idiopathic nephrotic syndrome (INS) activation, were conflicting in defining a specific interleukin pattern during relapse and remission of the disease. The aim of this study was to explore the role of IL-1β, IL-6 and IL-8 in the pathophysiology of INS during relapse and remission. PATIENTS AND METHODS: A total of 37 INS patients were included...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28175986/does-zika-virus-infection-induce-prolonged-remissions-in-children-with-idiopathic-nephrotic-syndrome
#4
Carolina Peralta-Aros, Víctor García-Nieto
BACKGROUND: Zika is an emerging mosquito-borne flavivirus. We report two pediatric patients diagnosed with idiopathic nephrotic syndrome who achieved complete remission of the disease after suffering Zika virus (ZIKV) infection. CASE DIAGNOSIS/TREATMENT: The first patient was a young girl aged 2.5 years with steroid-dependent nephrotic syndrome who was subsequently diagnosed with ZIKV infection. Following the infection, the steroid dose could be reduced until complete withdrawal...
February 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28143416/combination-therapy-with-rituximab-low-dose-cyclophosphamide-and-prednisone-for-idiopathic-membranous-nephropathy-a-case-series
#5
Frank B Cortazar, David E Leaf, Charles T Owens, Karen Laliberte, William F Pendergraft, John L Niles
BACKGROUND: Membranous nephropathy is a common cause of the nephrotic syndrome. Treatment with standard regimens fails to induce complete remission in most patients. We evaluated the efficacy of combination therapy with rituximab, low-dose, oral cyclophosphamide, and an accelerated prednisone taper (RCP) for the treatment of idiopathic membranous nephropathy. METHODS: We analyzed 15 consecutive patients with idiopathic membranous nephropathy treated with RCP at Massachusetts General Hospital...
February 1, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28137906/idiosyncratic-drug-reactions-and-membranous-glomerulopathy
#6
Amrit Kirpalani, Michael J Rieder, Kevin C Bax, Guido Filler
An infant boy with steroid-resistant nephrotic syndrome (idiopathic membranous glomerulonephropathy) achieved remission with ciclosporin but developed eosinophilia and high IgE levels (max 19 000  iU/mL). Conversion to tacrolimus resulted in chronic diarrhoea (eosinophilic gastroenteritis), muscle weakness, polyserositis and failure-to-thrive. In contrast, a trial without tacrolimus resulted in a ciclosporin-responsive relapse, therapy-resistant focal seizures with generalised spikes, worsening muscle weakness and diarrhoea...
January 30, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28098116/membranous-glomerulopathy-and-massive-cervical-lymphadenopathy-due-to-immunoglobulin-g4-disease
#7
Kamel El-Reshaid, Shaikha Al-Bader, John Madda
A 32-year-old male presented with acute and severe nephrotic syndrome as well as massive right cervical lymphadenopathy for <2 years. Computed tomography scan of the chest, abdomen, and pelvis did not reveal any lymphadenopathy. Histopathology and immunohistochemical testing of his lymph node biopsy showed infiltrate enriched with immunoglobulin G4 (IgG4)-positive plasma cells. His kidney biopsy showed granular membranous deposits of IgG4 in the basement membrane without interstitial infiltrate. Antiphospholipid 2 receptor antibodies were absent excluding its "idiopathic" nature...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28093933/acute-kidney-injury-in-idiopathic-nephrotic-syndrome-of-childhood-is-a-major-risk-factor-for-the-development-of-chronic-kidney-disease
#8
Afshan Yaseen, Vina Tresa, Ali Asghar Lanewala, Seema Hashmi, Irshad Ali, Sabeeta Khatri, Muhammed Mubarak
BACKGROUND: Acute kidney injury (AKI) is an important complication of idiopathic nephrotic syndrome (INS) and is associated with adverse outcomes, especially the development of chronic kidney disease (CKD). We aimed to determine the clinical profile of children with INS who developed AKI and its short-term outcome. MATERIAL AND METHODS: This prospective study was conducted from March 2014 to October 2015. A total of 119 children of INS (age: 2-18 years) fulfilling the pediatric RIFLE criteria for the diagnosis of AKI were enrolled and followed up for 3 months to determine the outcome...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/28090051/different-effects-of-tolvaptan-in-patients-with-idiopathic-membranous-nephropathy-with-nephrotic-syndrome
#9
Atsushi Tanaka, Tsukasa Nakamura, Eiichi Sato, Yoshihiko Ueda, Koichi Node
This case report discusses the clinical indication for immunosuppressants in patients with idiopathic membranous nephropathy (IMN). Because this disease occasionally shows spontaneous remission, it is necessary to determine the predictive values for a therapeutic effect in order to provide appropriate treatment. Two distinct cases described herein illustrate the different effects of tolvaptan in responders and non-responders, according to the pre-treatment levels of AQP-2 immunostaining in the samples from renal biopsy and urinary levels of AQP-2 and osmolality, suggesting that these values may be useful predictors of response to tolvaptan in patients with nephrotic IMN...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28065518/membranous-nephropathy-integrating-basic-science-into-improved-clinical-management
#10
REVIEW
Daniel C Cattran, Paul E Brenchley
Idiopathic membranous nephropathy (INM) remains a common cause of the nephrotic syndrome in adults. The autoimmune nature of IMN was clearly delineated in 2009 with the identification of the glomerular-deposited IgG to be a podocyte receptor, phospholipase A2 receptor (PLA2R) in 70% to 75% of cases. This anti-PLA2R autoantibody, predominantly the IgG4 subclass, has been quantitated in serum using an enzyme-linked immunosorbent assay and has been used to aid diagnosis and monitor response to immunosuppressive therapy...
January 5, 2017: Kidney International
https://www.readbyqxmd.com/read/28056860/evaluating-tacrolimus-treatment-in-idiopathic-membranous-nephropathy-in-a-cohort-of-408-patients
#11
Hua-Zhang Qin, Lei Liu, Shao-Shan Liang, Jing-Song Shi, Chun-Xia Zheng, Qing Hou, Ying-Hui Lu, Wei-Bo Le
BACKGROUND: The KDIGO Clinical Practice Guidelines for Glomerulonephritis recommended tacrolimus as an alternative regimen for the initial therapy for Idiopathic membranous nephropathy (IMN), however, large observational studies evaluating tacrolimus treatment in IMN remains rare. METHODS: A total of 408 consecutive IMN patients with nephrotic syndrome who were treated with tacrolimus in Jinling Hospital were included. The effectiveness and safety of tacrolimus treatment in IMN were analyzed in this study...
January 5, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28051228/nephrotic-syndrome-in-children-risk-factors-for-steroid-dependence
#12
Manel Jellouli, Meriem Brika, Kamel Abidi, Meriem Ferjani, Ouns Naija, Yousra Hammi, Tahar Gargah
Background - Most patients with idiopathic nephrotic syndrome are steroid-responsive, about 50% relapse and often become steroid-dependent and exposed to long-term steroid complications. The aim of this study was to determine predictive risk factors for steroid dependence using clinical and biological variables present at onset of the disease. It may be useful to adapt the therapeutic strategy. Methods - Retrospective hospital-based cohort study in the department of pediatric nephrology of Charles Nicolle, Tunis, between 2002 and 2012 included 52 children with idiopathic steroid-responsive nephrotic...
July 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/28001187/treatment-of-idiopathic-light-chain-deposition-disease-complete-remission-with-bortezomib-and-dexamethasone
#13
João Tadeu Damian Souto Filho, Jorge Murilo Grillo Monteiro, Inêz Barcellos de Andrade
Light chain deposition disease (LCDD) is a rare clinical entity characterized by the deposition of light chain immunoglobulins in different tissues and primarily affects the kidneys, followed by the liver and heart. This disease often manifests as nephrotic syndrome with marked proteinuria and rapid deterioration of renal function. More than 50% of cases are secondary to multiple myeloma or other lymphoproliferative diseases, with a well-established treatment aimed at controlling the underlying disease. In rare cases, there is no detection of an associated hematological disease, referred to as idiopathic LCDD...
December 2016: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/27940460/minimal-change-disease
#14
Marina Vivarelli, Laura Massella, Barbara Ruggiero, Francesco Emma
Minimal change disease (MCD) is a major cause of idiopathic nephrotic syndrome (NS), characterized by intense proteinuria leading to edema and intravascular volume depletion. In adults, it accounts for approximately 15% of patients with idiopathic NS, reaching a much higher percentage at younger ages, up to 70%-90% in children >1 year of age. In the pediatric setting, a renal biopsy is usually not performed if presentation is typical and the patient responds to therapy with oral prednisone at conventional doses...
February 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27904864/nephrotic-range-proteinuria-and-peripheral-edema-in-a-child-not-only-idiopathic-nephrotic-syndrome
#15
Valentina Dolcemascolo, Marina Vivarelli, Manuela Colucci, Francesca Diomedi-Camassei, Rossella Piras, Marta Alberti, Francesco Emma
Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...
September 2016: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/27890680/the-putative-role-of-maldi-msi-in-the-study-of-membranous-nephropathy
#16
Andrew Smith, Vincenzo L'Imperio, Elena Ajello, Franco Ferrario, Niccolò Mosele, Martina Stella, Manuel Galli, Clizia Chinello, Federico Pieruzzi, Goce Spasovski, Fabio Pagni, Fulvio Magni
Membranous Nephropathy (MN) is an immunocomplex mediated renal disease that represents one of the most frequent glomerulopathies worldwide. This glomerular disease can manifest as primary (idiopathic) or secondary and this distinction is crucial when choosing the most appropriate course of treatment. In secondary cases, the best strategy involves treating the underlying disease, whereas in primary forms, the identification of confirmatory markers of the idiopathic etiology underlining the process is requested by clinicians...
November 24, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27885584/wt1-and-nphs2-gene-mutation-analysis-and-clinical-management-of-steroid-resistant-nephrotic-syndrome
#17
Aravind Selvin Kumar Ramanathan, Murali Vijayan, Srilakshmi Rajagopal, Padmaraj Rajendiran, Prabha Senguttuvan
Nephrotic syndrome (NS) is a kidney disease predominantly present in children with idiopathic condition; final stage of the disease progresses into end-stage renal disease. Generally, NS is treated using standard steroid therapy, however; most of the children are steroid sensitive and about 15-20% are non-responders (SRNS). Non-responsiveness of these children would be a risk with the possibility of mutational changes in podocyte genes (NPHS1, NPHS2, WT1, PLCE1). The mutation in podocyte genes is associated with SRNS...
February 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/27833453/benign-acute-childhood-myositis-complicating-influenza-b-infection-in-a-boy-with-idiopathic-nephrotic-syndrome
#18
Piotr Skrzypczyk, Joanna Przychodzień, Małgorzata Pańczyk-Tomaszewska
INTRODUCTION: Benign acute childhood myositis (BACM) is an acute complication of an infection characterized by calf pain, limitation of lower limb mobility, an increase in serum creatine kinase, and a self-limiting course. No reports of BACM in children with idiopathic nephrotic syndrome (INS) can be found in the literature. CASE REPORT: A 5-year-old boy with steroid-sensitive INS presented with fever, leg pain, and problems with walking. Physical examination showed pharyngeal erythema, preserved movements in all joints, and weakness of leg muscles...
2016: Central-European Journal of Immunology
https://www.readbyqxmd.com/read/27821253/bone-mineral-density-in-children-with-idiopathic-nephrotic-syndrome
#19
Ghada Mohamed El-Mashad, Mahmoud Ahmed El-Hawy, Sally Mohamed El-Hefnawy, Sanaa Mansour Mohamed
OBJECTIVES: To assess bone mineral density (BMD) in children with idiopathic nephrotic syndrome (NS) and normal glomerular filtration rate (GFR). METHODS: Cross-sectional case-control study carried out on 50 children: 25 cases of NS (16 steroid-sensitive [SSNS] and nine steroid-resistant [SRNS] under follow up in the pediatric nephrology unit of Menoufia University Hospital, which is tertiary care center, were compared to 25 healthy controls with matched age and sex...
November 5, 2016: Jornal de Pediatria
https://www.readbyqxmd.com/read/27796697/characteristics-of-patients-diagnosed-with-renal-vein-thrombosis-and-glomerulopathy-a-case-series
#20
Oliver Ross, Andrew Pourmoussa, Michael Batech, John J Sim
BACKGROUND: Few contemporary studies have evaluated the clinical characteristics of patients with biopsy-proven glomerulopathy diagnosed with renal vein thrombosis (RVT). METHODS: Retrospective case series study within an integrated health system in a 12-year period (January 1, 2000 through December 31, 2011) investigating clinical characteristics of all adult patients who underwent native or transplant kidney biopsy and also had a diagnosis of RVT. Patient characteristics, diagnostic studies, and outcomes were evaluated...
October 28, 2016: International Urology and Nephrology
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