keyword
https://read.qxmd.com/read/38161837/retrospective-cross-sectional-study-reviewing-the-effectiveness-of-mycophenolate-mofetil-on-saudi-children-with-nephrotic-syndrome
#21
JOURNAL ARTICLE
Deemah A Aldakheel, Abdulrahman Alamir, Khawla K Almarshad, Zahra A Alsafwani, Roa A Alshaer, Abdullah T Alzulfah, Soud A Al Rasheed
Background Idiopathic nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children. It is often associated with minimal change disease (MCD). Corticosteroid therapy is the initial treatment, but many patients experience relapses, leading to steroid-dependent nephrotic syndrome (SDNS) or frequently relapsing steroid-sensitive nephrotic syndrome (FR-SSNS). To avoid prolonged steroid use, mycophenolate mofetil (MMF) is used as an immunosuppressive alternative. MMF is safe and effective for treating SDNS and FR-SSNS in children, with studies showing reduced relapse rates...
November 2023: Curēus
https://read.qxmd.com/read/38158962/-state-of-the-art-paradigm-of-corticosteroid-therapy-for-immune-mediated-inflammatory-kidney-diseases
#22
JOURNAL ARTICLE
N M Bulanov, I N Bobkova, S V Moiseev
Since 1950's corticosteroids (CS) have remained the cornerstone of immunosuppressive therapy for immune-mediated kidney diseases. However multiple adverse events, associated with the prolonged CS therapy, became the basis for the development of novel treatment approaches. Current evidence supports the implementation of the steroid-sparing regimens for the treatment of different types of glomerulonephritis. Randomised controlled trial PEXIVAS demonstrated the efficacy and safety of early steroid tapering, starting from the second week of therapy, in patients with ANCA-associated vasculitis with kidney involvement...
August 17, 2023: Terapevticheskiĭ Arkhiv
https://read.qxmd.com/read/38142361/clinical-predictors-of-steroid-resistance-in-childhood-nephrotic-syndrome
#23
JOURNAL ARTICLE
Neslihan Cicek, Nurdan Yıldız, Sercin Guven, Mehtap Kaya, Ibrahim Gokce, Harika Alpay
We aimed to evaluate the clinical parameters, histopathological findings of nephrotic syndrome (NS) patients, and independent factors predicting steroid resistance in a single tertiary center. One hundred and sixty-two children (57 girls and 105 boys) with NS who were followed between 1998 and 2018 were analyzed in this retrospective cohort. The median (interquartile range; range) age and follow-up time were 4.9 (5.7; 0.1-16.8) and 5.5 (5.4; 0.1-20.3) years. A total of 82.7% of the patients were steroid-sensitive nephrotic syndrome (SSNS) and 17...
December 24, 2023: Clinical Pediatrics
https://read.qxmd.com/read/38137694/idiopathic-nephrotic-syndrome-in-children-in-chad-epidemiology-and-clinical-outcomes
#24
JOURNAL ARTICLE
Guillaume Mahamat Abderraman, Youssouf Djidita Hagré, Hissein Ali Mahamat, Senoussi Charfadine, Ali Sakine Amne, Adoum Attimer Khadidja, Lionel Rostaing
INTRODUCTION: Nephrotic syndrome (NS) remains the most common presentation of glomerular diseases in children. Moreover, NS is primarily idiopathic, accounting for 90% of cases, with an average onset age between 2 and 10 years. The objective of our study was to describe the characteristics and outcomes of NS in children from three major hospitals in one of the world's poorest countries, Chad. PATIENTS AND METHODS: This observational, cross-sectional, descriptive, and multicenter study took place over a period of 36 months (1 January 2019-31 December 2021) and was carried out in three hospitals in N'Djamena, Chad...
December 11, 2023: Journal of Clinical Medicine
https://read.qxmd.com/read/38130516/effectiveness-of-rituximab-and-its-biosimilar-in-treating-adult-steroid-dependent-minimal-change-disease-and-relapse
#25
Hui Yi Shan
Minimal change disease (MCD) is an important cause of nephrotic syndrome in adults. Its course is often complicated by frequent relapses and steroid dependence. Most of the treatment experience of MCD comes from management of pediatric patients rather than adult patients. In this report, the author describes successful experience of using rituximab (RTX) and its biosimilar, RTX-pvvr (ruxience), to treat steroid-dependent MCD and relapses in adult patients. This is the first report of using a RTX biosimilar to treat MCD...
November 2023: Curēus
https://read.qxmd.com/read/38127456/scaffold-protein-sh3bp2-signalosome-is-pivotal-for-immune-activation-in-nephrotic-syndrome
#26
JOURNAL ARTICLE
Tarak Srivastava, Robert E Garola, Jianping Zhou, Varun Chandra Boinpelly, Mohammad H Rezaiekhaligh, Trupti Joshi, Yuexu Jiang, Diba Ebadi, Siddarth Sharma, Christine Sethna, Vincent S Staggs, Ram Sharma, Debbie S Gipson, Wei Hao, Yujie Wang, Laura H Mariani, Jeffrey B Hodgin, Robert Rottapel, Teruhito Yoshitaka, Yasuyoshi Ueki, Mukut Sharma
Despite clinical use of immunosuppressive agents, the immunopathogenesis of Minimal Change Disease (MCD) and Focal Segmental Glomerulosclerosis (FSGS) remains unclear. SH3BP2, a scaffold protein, forms an immune signaling complex (signalosome) with seventeen other proteins including PLCγ2 and VAV2. Bioinformatic analysis of human glomerular transcriptome (NEPTUNE cohort) revealed upregulated SH3BP2 in MCD (p=0.001) and FSGS (p<0.001). The SH3BP2-signalosome score and downstream MYD88, TRIF, and NFATc1 were significantly upregulated in MCD and FSGS (p=0...
December 21, 2023: JCI Insight
https://read.qxmd.com/read/38123711/kidney-biopsy-findings-in-children-with-diabetes-mellitus
#27
JOURNAL ARTICLE
Lasanthi Weerasooriya, Alexander J Howie, Matthew P Wakeman, Susan Cavanagh, David V Milford
BACKGROUND: Diabetic nephropathy may begin in childhood, but clinical kidney disease ascribable to this is uncommon in children with type 1 (insulin dependent) diabetes mellitus. METHODS: We reviewed our experience of kidney biopsies in children with type 1 diabetes mellitus. RESULTS: Between 1995 and 2022, there were biopsies in 17 children, with various clinical indications for kidney biopsy, making this the largest series of biopsies in diabetic children with clinical kidney abnormalities...
December 21, 2023: Pediatric Nephrology
https://read.qxmd.com/read/38106587/podocyte-infolding-glomerulopathy-a-special-morphology-of-podocyte-injury-caused-by-heterogeneous-diseases
#28
JOURNAL ARTICLE
Ling Hong, Lin Wang, Honglei Wang, Qihua Wang, Shicong Yang, Tian Tian, Tianjiao Cui, Shuling Yue, Xiaotao Hou, Zhihua Zheng, Wenfang Chen
INTRODUCTION: Podocyte infolding glomerulopathy (PIG) is a newly recognized rare glomerular injury. The clinical significance and mechanism of this injury pattern remains unclear. METHODS: We conducted a retrospective study of renal biopsies from January 2018 to December 2020 in Kingmed Diagnostics. The renal biopsy features and clinical data were reviewed. Laser scanning microdissection and mass spectrometry (LMD/MS) was conducted to analyze the potential mechanism...
December 2023: KI Reports
https://read.qxmd.com/read/38092712/urinary-apolipoprotein-a1-and-neutrophil-gelatinase-associated-lipocalin-in-children-with-idiopathic-nephrotic-syndrome
#29
JOURNAL ARTICLE
Suresh Murugesan, Abhijeet Saha, Bobbity Deepthi, Manpreet Kaur, Trayambak Basak, Shantanu Sengupta, Vineeta Batra, Ashish Dutt Upadhyay
Urinary biomarkers are a promising diagnostic modality whose role was explored in nephrotic syndrome (NS). We estimated urinary apolipoprotein A1 (Apo A1) and neutrophil gelatinase-associated lipocalin (NGAL) in children with first-episode NS (FENS) and controls with a longitudinal follow-up to see the serial changes during remission. The study groups comprised 35 children with FENS and an equal number of age- and sex-matched controls. Patients were followed up at regular intervals, and 32 patients were classified as having steroid-sensitive NS (SSNS) and 3 as having steroid-resistant NS (SRNS)...
January 1, 2023: Saudi Journal of Kidney Diseases and Transplantation
https://read.qxmd.com/read/38054033/longitudinal-analysis-of-prophylactic-anticoagulation-in-primary-nephrotic-syndrome-low-incidence-of-thromboembolic-complications
#30
JOURNAL ARTICLE
Thomas McDonnell, John Hartemink, Omar Ragy, Katherine Parker, Meshaal Shukkur, Jecko Thachil, Durga Kanigicherla
INTRODUCTION: Thromboembolic events (TEEs) are a serious and potentially fatal complication of nephrotic syndrome (NS). Despite this, there is a lack of evidence examining the benefits of prophylactic anticoagulation (PAC) in NS. It was our objective to review the risk factors, rates of TEEs, and patterns of PAC in patients with primary NS, with the aim to provide a pragmatic approach to PAC in primary NS. METHODS: This is a retrospective longitudinal cohort study of adult patients with primary NS...
2023: Glomerular diseases
https://read.qxmd.com/read/38034245/suplatast-tosilate-and-eicosapentaenoic-acid-as-a-possible-strategy-for-maintaining-remission-in-minimal-change-nephrotic-syndrome-a-case-report
#31
Ayako Y Shichijo, Hirotsugu Iwatani
We report a case of a sudden onset of minimal change nephrotic syndrome (MCNS) in a 33-year-old woman with type 1 diabetes mellitus (T1DM), stable microalbuminuria, and chronic thyroiditis. She was successfully treated with intravenous corticosteroids to finally attain a complete remission. Four years later, she also experienced a relapse of MCNS in the same season as the first onset. The chronological levels of serum immunoglobulin E (IgE) showed that extremely high serum IgE levels preceded the onset or the relapse of MCNS, which may suggest an allergic mechanism of MCNS...
October 2023: Curēus
https://read.qxmd.com/read/38025209/external-validation-of-a-urinary-biomarker-risk-score-for-the-prediction-of-steroid-responsiveness-in-adults-with-nephrotic-syndrome
#32
JOURNAL ARTICLE
Hillarey K Stone, Bin Huang, Chen Chen, Qing Ma, Michael R Bennett, Prasad Devarajan
INTRODUCTION: In idiopathic nephrotic syndrome, response to corticosteroids remains the best indicator of prognosis. Noninvasive markers to predict a patient's response to steroids would allow improved prognostication and a more personalized approach to management. We have previously derived a urinary biomarker risk score which can differentiate steroid sensitive nephrotic syndrome (SSNS) from steroid resistant nephrotic syndrome (SRNS) in children. The goal of this study was to validate this previously derived biomarker risk score in a cohort of steroid-naïve adult patients, to determine whether the panel could be used to predict steroid responsiveness at the time of initial diagnosis...
November 2023: KI Reports
https://read.qxmd.com/read/38022256/clinical-profile-and-outcomes-of-nephrotic-syndrome-with-acute-kidney-injury-in-adults-a-prospective-cohort-study
#33
JOURNAL ARTICLE
Rajaram Yadav, Om Kumar, Amresh Krishna, Prit P Singh, Prem S Patel
BACKGROUND: Nephrotic syndrome (NS) is characterized by various clinicopathological conditions like proteinuria, hypoalbuminemia, and anasarca. Patients with NS are prone to experience associated problems like acute kidney injury (AKI). The present study aimed to investigate the clinical profile and outcomes of NS with AKI in adults. MATERIALS AND METHODS: This prospective, observational study was conducted over a period of one year. Adult patients with NS diagnosed with AKI were enrolled in the study...
October 2023: Curēus
https://read.qxmd.com/read/37973491/drug-induced-glomerular-diseases
#34
JOURNAL ARTICLE
Anne-Sophie Garnier, Hélène Laubacher, Marie Briet
Drug-induced kidney diseases represent a wide range of diseases that are responsible for a significant proportion of all acute kidney injuries and chronic kidney diseases. In the present review, we focused on drug-induced glomerular diseases, more precisely podocytopathies - minimal change diseases (MCD), focal segmental glomerulosclerosis (FSGS) - and membranous nephropathies (MN), from a physiological and a pharmacological point of view. The glomerular filtration barrier is composed of podocytes that form foot processes tightly connected and directly in contact with the basal membrane and surrounding capillaries...
October 31, 2023: Thérapie
https://read.qxmd.com/read/37963441/efficacy-of-rituximab-for-minimal-change-disease-and-focal-segmental-glomerulosclerosis-with-frequently-relapsing-or-steroid-dependent-nephrotic-syndrome-in-adults-a-chinese-multicenter-retrospective-study
#35
MULTICENTER STUDY
Lan Lan, Yuxin Lin, Binfeng Yu, Yin Wang, Hong Pan, Huijing Wang, Xiaowei Lou, Xiabing Lang, Qiankun Zhang, Lie Jin, Yi Yang, Liang Xiao, Jianghua Chen, Fei Han
INTRODUCTION: Rituximab has been proven effective and safe in pediatric patients with frequently relapsing or steroid-dependent nephrotic syndrome (FR/SDNS). We aimed to analyze the efficacy and safety of rituximab in adult FR/SDNS patients with minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS). METHODS: A retrospective cohort study at three nephrology centers in China included adult FR/SDNS patients with biopsy-proven MCD or FSGS. Primary outcomes were relapse frequency and first relapse-free survival time...
2024: American Journal of Nephrology
https://read.qxmd.com/read/37957688/effects-of-a-novel-anln-e841k-mutation-associated-with-srns-on-podocytes-and-its-mechanism
#36
JOURNAL ARTICLE
Li Lin, Yuhong Ye, Haidong Fu, Weizhong Gu, Manli Zhao, Jingmiao Sun, Zhongkai Cao, Guoping Huang, Yi Xie, Fei Liu, Lu Li, Qiuyu Li, Jianhua Mao, Lidan Hu
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is characterized by unrelieved proteinuria after an initial 4-8 weeks of glucocorticoid therapy. Genes in podocytes play an important role in causing SRNS. OBJECTIVE: This study aimed to report a pathogenic mutation in SRNS patients and investigate its effects on podocytes, as well as the pathogenic mechanism. METHODS: We screened out a novel mutation by using whole-exon sequencing in the SRNS cohort and verified it via Sanger sequencing...
November 13, 2023: Cell Communication and Signaling: CCS
https://read.qxmd.com/read/37945450/glomerular-diseases-across-lifespan-key-differences-in-diagnostic-and-therapeutic-approaches
#37
REVIEW
Martin Windpessl, Balazs Odler, Ingeborg M Bajema, Duvuru Geetha, Marcus Säemann, Jiwon M Lee, Augusto Vaglio, Andreas Kronbichler
Glomerular diseases are common causes of chronic kidney disease in childhood, adolescence, and adulthood. The epidemiology of glomerular diseases differs between different age groups, with minimal change disease being the leading cause of nephrotic syndrome in childhood, while membranous nephropathy and focal segmental glomerulosclerosis are more common in adulthood. IgA vasculitis is also more common in childhood. Moreover, there is a difference in disease severity with more children presenting with a relapsing form of nephrotic syndrome and a more acute presentation of antineutrophil cytoplasmic antibody-associated vasculitis and concomitant glomerulonephritis, as highlighted by the higher percentage of cellular crescents on kidney biopsy specimens in comparison with older patients...
July 2023: Seminars in Nephrology
https://read.qxmd.com/read/37942512/patients-with-primary-focal-segmental-glomerulosclerosis-with-detectable-urinary-cd80-are-more-similar-to-patients-with-minimal-change-disease-in-clinicopathological-features
#38
JOURNAL ARTICLE
Xiaojie Gong, Jing Huang, Yimiao Zhang, Fang Wang, Xin Wang, Liqiang Meng, Xuyang Cheng, Gang Liu, Zhao Cui, Minghui Zhao
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is an important cause of refractory nephrotic syndrome (NS) in children and adults. Urinary CD80 is elevated in some patients with primary FSGS, however, its clinical value is not fully clarified. This study aims to evaluate the clinical and pathological significance of urinary CD80 in patients with primary FSGS. METHODS: Sixty-one adult patients with biopsy-proven primary FSGS, with standard treatment and long-term follow up, were enrolled retrospectively...
2023: Renal Failure
https://read.qxmd.com/read/37941318/exacerbation-of-minimal-change-disease-following-mrna-covid-19-vaccination
#39
JOURNAL ARTICLE
Afshin Pirzadeh, Sina Emami, Jonathan E Zuckerman, Niloofar Nobakht
BACKGROUND Minimal change disease is a common cause of nephrotic syndrome in adults. There are few reported cases of vaccine-related podocytopathy with nephrotic-range proteinuria in the setting of a minimal change disease history. There have been rare reports of acute renal damage following vaccination to prevent COVID-19 and some cases of exacerbation of ongoing nephropathy. This report is a 33-year-old man with a 22-year history of nephrotic syndrome due to minimal change disease which exacerbated following a third dose of an mRNA SARS-CoV-2 vaccine for COVID-19...
November 9, 2023: American Journal of Case Reports
https://read.qxmd.com/read/37926543/a-case-of-minimal-change-nephrotic-syndrome-secondary-to-methotrexate-associated-hodgkin-lymphoma
#40
JOURNAL ARTICLE
Misa Iimori, Kazuhiro Sonomura, Yuichi Ueyama, Yuki Oobayashi, Hiroya Adachi, Mayuka Nakayama
The patient was a 64-year-old man who had been receiving methotrexate (MTX) for rheumatoid arthritis for 8 years. Computed tomography (CT) obtained one month prior to admission revealed numerous enlarged lymph nodes. Lower leg edema appeared two weeks prior to admission. Severe proteinuria was confirmed, and the patient was admitted. A renal biopsy revealed minimal changes in glomeruli. CT on day 14 revealed shrinking lymph nodes, and the patient was diagnosed with Hodgkin lymphoma by a neck lymph node biopsy...
November 6, 2023: Internal Medicine
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