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Keywords Membranoproliferative glomerul...

Membranoproliferative glomerulonephritis

https://read.qxmd.com/read/38398059/complement-activation-in-nephrotic-glomerular-diseases
#1
REVIEW
Dominik Nell, Robert Wolf, Przemyslaw Marek Podgorny, Tobias Kuschnereit, Rieke Kuschnereit, Thomas Dabers, Sylvia Stracke, Tilman Schmidt
The nephrotic syndrome holds significant clinical importance and is characterized by a substantial protein loss in the urine. Damage to the glomerular basement membrane or podocytes frequently underlies renal protein loss. There is an increasing belief in the involvement of the complement system, a part of the innate immune system, in these conditions. Understanding the interactions between the complement system and glomerular structures continually evolves, challenging the traditional view of the blood-urine barrier as a passive filter...
February 18, 2024: Biomedicines
https://read.qxmd.com/read/38358193/to-interpret-and-analyze-the-changing-patterns-of-histology-and-direct-immunofluorescence-findings-in-membranoproliferative-glomerulonephritis
#2
JOURNAL ARTICLE
Pragnya P Nayak, Pranati Pradhan, Dilleswari Pradhan, Nachiketa Mohapatra, Sarojini Raman, Pranabandhu Sahoo
BACKGROUND: Membranoproliferative glomerulonephritis has in the recent past been regrouped into immune complex-mediated (ICM MPGN) disease (driven by the classical complement pathway) and complement-mediated (C3GN) disease (driven by the alternative complement pathway) based on pathogenetic role of alternative complement pathway and immunofluorescence deposits. The proposed regrouping lent therapeutic and prognostic support in managing the disease of MPGN. AIMS AND OBJECTIVES: The present study is undertaken to study the patterns of MPGN based on histopathological and DIF examination and sub-categorize the cases into mainly complement dominant and immune complex-mediated diseases for better prognostic and therapeutic utility...
2024: Indian Journal of Pathology & Microbiology
https://read.qxmd.com/read/38343496/atypical-hemolytic-uremic-syndrome-a-nationwide-colombian-pediatric-series
#3
JOURNAL ARTICLE
Zilac Espitaleta, Alex Domínguez-Vargas, Johanna Villamizar-Martínez, Martha Carrascal-Guzmán, Gustavo Guerrero-Tinoco, Diana Silva-Díaz, Richard Baquero, Claudia Pinto-Bernal, Luz González-Chaparro, Luisa Rojas-Rosas, Pilar Amado-Niño, Mariángel Castillo-Arteaga, Yeferson Alvarez-Gómez, Laura Arguello-Muñoz, William Morales-Camacho, Oscar León-Guerra, Eduardo Egea, Ricardo Galeano-Rodríguez, Ana Quintero-Gómez, Gustavo Aroca-Martínez, Carlos G Musso
Objectives. Atypical hemolytic uremic syndrome (aHUS) is a rare complement-mediated kidney disease with genetic predisposition and represents up to 10% of pediatric hemolytic uremic syndrome (HUS) cases. Few studies have evaluated aHUS in Latin American population. We studied a Colombian pediatric cohort to delineate disease presentation and outcomes. Methods. A multicenter cohort of 27 Colombian children with aHUS were included. Patients were grouped by age at onset. Clinical features were compared using analysis of variance (ANOVA) and Fisher exact tests...
2024: Global Pediatric Health
https://read.qxmd.com/read/38337366/implementation-of-kidney-biopsy-in-one-of-the-poorest-countries-in-the-world-experience-from-zinder-hospital-niger
#4
JOURNAL ARTICLE
Hassane Moussa Diongolé, Zeinabou Maiga Moussa Tondi, Abdoulazize Garba, Kabirou Ganiou, Laouali Chaibou, Djibrilla Bonkano, Illiassou Aboubacar, Abdoul Aziz Seribah, Abdoul Madjid Abdoulaye Idrissa, Akinfenwa Atanda, Lionel Rostaing
Kidney biopsy (KB) has become essential in the nephrologist's approach to kidney diseases, both for diagnosis, treatment, and prognosis. Our objective is to describe the preliminary results of KBs in Niger, one of the poorest countries in the world. This is a descriptive cross-sectional study that took place over 36 months in the nephrology/dialysis department of the Zinder National Hospital. Biopsy results were obtained in less than 5 working days. Patients were responsible for covering the cost of the kidney biopsy...
January 24, 2024: Journal of Clinical Medicine
https://read.qxmd.com/read/38312795/iptacopan-in-idiopathic-immune-complex-mediated-membranoproliferative-glomerulonephritis-protocol-of-the-apparent-multicenter-randomized-phase-3-study
#5
JOURNAL ARTICLE
Marina Vivarelli, Andrew S Bomback, Matthias Meier, Yaqin Wang, Nicholas J A Webb, Uday Kiran Veldandi, Richard J H Smith, David Kavanagh
INTRODUCTION: Immune complex-mediated membranoproliferative glomerulonephritis (IC-MPGN) is an ultra-rare, fast-progressing kidney disease that may be idiopathic (primary) or secondary to chronic infection, autoimmune disorders, or monoclonal gammopathies. Dysregulation of the alternative complement pathway is implicated in the pathophysiology of IC-MPGN; and currently, there are no approved targeted treatments. Iptacopan is an oral, highly potent proximal complement inhibitor that specifically binds to factor B and inhibits the alternative pathway (AP)...
January 2024: KI Reports
https://read.qxmd.com/read/38267800/rationale-and-design-of-the-japanese-biomarkers-in-nephrotic-syndrome-j-marine-study
#6
JOURNAL ARTICLE
Shimon Kurasawa, Sawako Kato, Takaya Ozeki, Shin'ichi Akiyama, Takuji Ishimoto, Masashi Mizuno, Naotake Tsuboi, Noritoshi Kato, Tomoki Kosugi, Shoichi Maruyama
INTRODUCTION: Disease subtyping and monitoring are essential for the management of nephrotic syndrome (NS). Although various biomarkers for NS have been reported, their clinical efficacy has not been comprehensively validated in adult Japanese patients. METHODS: The Japanese Biomarkers in Nephrotic Syndrome (J-MARINE) study is a nationwide, multicenter, and prospective cohort study in Japan, enrolling adult (≥18 years) patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), C3 glomerulopathy (C3G), and lupus nephritis (LN)...
January 25, 2024: Clinical and Experimental Nephrology
https://read.qxmd.com/read/38254040/a-case-of-unexpected-diagnosis-of-fibronectin-glomerulopathy-with-histological-features-of-membranoproliferative-glomerulonephritis
#7
JOURNAL ARTICLE
Misa Hata, Takayasu Mori, Yurika Hirose, Yuriko Nishida, Shintaro Mandai, Fumiaki Ando, Koichiro Susa, Soichiro Iimori, Shotaro Naito, Eisei Sohara, Tatemitsu Rai, Towako Taguchi, Shohei Tomii, Kenichi Ohashi, Shinichi Uchida
Fibronectin (FN) glomerulopathy (FNG), a rare autosomal hereditary renal disease, is characterized by proteinuria resulting from the massive accumulation of FN in the glomeruli. It typically affects individuals aged 10-50 years. In this report, we describe the case of a 57-year-old man who was diagnosed with FNG through genetic analysis and histological examination that revealed membranoproliferative glomerulonephritis. Despite treatment with prednisolone, the therapeutic response was unsatisfactory. Prednisolone was subsequently tapered and discontinued because the patient had pulmonary thromboembolism...
January 22, 2024: BMC Nephrology
https://read.qxmd.com/read/38252289/anti-factor-b-antibodies-in-atypical-hemolytic-uremic-syndrome
#8
JOURNAL ARTICLE
Priyanka Khandelwal, Shreesha Nambiar, Rahul Saini, Savita Saini, Poonam Coshic, Aditi Sinha, Pankaj Hari, Jayanth Kumar Palanichamy, Arvind Bagga
BACKGROUND: The etiology of atypical hemolytic uremic syndrome (aHUS) is unknown in 30-40% of patients. Anti-factor B (FB) antibodies are reported in C3 glomerulopathy (C3G) and immune-complex membranoproliferative glomerulonephritis (IC-MPGN), though not in aHUS. METHODS: We screened patients < 18-year-old from cohorts of aHUS and C3G/idiopathic IC-MPGN. Anti-FB IgG antibodies were measured by ELISA and confirmed by Western blot. Normative levels were based on antibody levels in 103 healthy blood donors...
January 22, 2024: Pediatric Nephrology
https://read.qxmd.com/read/38222325/bartonella-henselae-infection-mediated-shunt-nephritis
#9
Jennifer Nhan, Shriprasad Deshpande, Craig Futterman, Dong Hyang Kwon, Aadil Kakajiwala
Serum anti-neutrophil cytoplasmic antibody (ANCA) positivity with membranoproliferative pattern on renal biopsy can be due to ANCA-associated vasculitis as well as chronic indolent infections. We present the case of an adolescent boy with congenital heart disease and history of cardiac surgery who presented with severe acute kidney injury requiring hemodialysis. Renal biopsy showed membranoproliferative glomerulonephritis with full-house immunofluorescence pattern. Low serum complements, PR3 ANCA positivity and elevated Bartonella immunoglobulin titers suggested a diagnosis of infective endocarditis-associated glomerulonephritis...
2024: Clinical Nephrology. Case Studies
https://read.qxmd.com/read/38171412/atypical-anti-glomerular-basement-membrane-nephritis-a-case-series-from-the-french-nephropathology-group
#10
JOURNAL ARTICLE
Bertrand Chauveau, Jean-Baptiste Gibier, Jérôme Olagne, Antoine Morel, Selda Aydin, Stephen P McAdoo, Nicolas Viallet, Hélène Perrochia, Emilie Pambrun, Virginie Royal, Nathalie Demoulin, Jean-Louis Kemeny, Carole Philipponnet, Alexandre Hertig, Jean-Jacques Boffa, Emmanuelle Plaisier, Camille Domenger, Isabelle Brochériou, Clément Deltombe, Jean-Paul Duong Van Huyen, David Buob, Candice Roufosse, Thomas Hellmark, Vincent Audard, Fabrice Mihout, Samih H Nasr, Karine Renaudin, Anissa Moktefi, Marion Rabant
RATIONALE & OBJECTIVE: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized by a bright linear immunoglobulin staining along the GBM by immunofluorescence without a diffuse crescentic glomerulonephritis nor serum anti-GBM antibodies by conventional enzyme-linked immunosorbent assay (ELISA). We characterized a series of patients with atypical anti-GBM disease. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: Patients identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022...
January 1, 2024: American Journal of Kidney Diseases
https://read.qxmd.com/read/38142361/clinical-predictors-of-steroid-resistance-in-childhood-nephrotic-syndrome
#11
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
#12
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/38076230/c3-glomerulopathies-dense-deposit-disease-and-c3-glomerulonephritis
#13
REVIEW
Claudio Ponticelli, Marta Calatroni, Gabriella Moroni
Dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are types of membranoproliferative glomerulonephritis classified as C3 glomerulopathies. These conditions are characterized by an increased number of intraglomerular cells and diffuse thickening of the glomerular capillary walls, along with the deposition of C3 and minimal or absent immunoglobulin deposits. The underlying cause of both DDD and C3Gn is an abnormal activation of the alternative complement pathway, which can result from acquired or genetic alteration...
2023: Frontiers in Medicine
https://read.qxmd.com/read/38053039/fibronectin-glomerulopathy-in-a-kidney-allograft-biopsy
#14
JOURNAL ARTICLE
Nathaniel Klair, Salman B Mahmood, Rasha El-Rifai, Cynthia C Nast, Lihong Bu, Adam Bregman
BACKGROUND: Fibronectin glomerulopathy is a rare genetic nephropathy with only a few cases of post-transplant recurrence being reported previously. We highlight a case that was initially misdiagnosed and emphasize the importance of full immunofluorescence and electron microscopy evaluation in allograft biopsies. CASE PRESENTATION: A 36-year-old male with a history of end-stage kidney disease secondary to biopsy-proven type 1 membranoproliferative glomerulonephritis (MPGN) status-post living unrelated donor kidney transplant 12 years prior, presented with increasing creatinine and proteinuria...
December 5, 2023: BMC Nephrology
https://read.qxmd.com/read/38021795/the-relationship-between-soluble-urokinase-type-plasminogen-activator-receptor-supar-levels-and-treatment-response-in-patients-with-glomerulonephritis-a-single-center-experience
#15
JOURNAL ARTICLE
Kubilay İşsever, Hamad Dheir
Aim Soluble urokinase-type plasminogen activator receptor (suPAR) is an important protein that is reported to increase in a broad range of inflammatory processes. We aimed to determine whether suPAR is a significant biomarker in glomerulonephritis for distinguishing patients with treatment response from patients without treatment response in our study. Materials and methods This was a prospective study in which 117 patients with biopsy-proven glomerulonephritis and 54 healthy individuals without a known chronic disease (control group) were enrolled...
October 2023: Curēus
https://read.qxmd.com/read/38008458/a-case-of-successful-pregnancy-and-delivery-in-a-chronic-renal-failure-patient-with-membranoproliferative-glomerulonephritis-and-preeclampsia-related-nephrotic-syndrome
#16
JOURNAL ARTICLE
Kumi Nakai, Koji Sato, Nao Nohara, Miyuki Takagi, Masao Kihara, Seiji Ueda, Tomohito Gohda, Yusuke Suzuki
A 37-year-old woman with chronic kidney disease stage (CKD) G4 with membranoproliferative glomerulonephritis was hospitalized for nephrotic syndrome and hypertension due to superimposed preeclampsia at 27 weeks into her third pregnancy. Proteinuria did not worsen significantly after pulse steroid therapy. Delivery was induced at 30 weeks' gestation due to the maternal renal function and fetal growth. No obvious fetal complications other than preterm delivery were observed. In this case, we successfully managed a high-risk patient with membranoproliferative glomerulonephritis complicated by advanced CKD, nephrotic syndrome, and hypertension, which are independent risk factors for pregnancy complications...
November 27, 2023: Internal Medicine
https://read.qxmd.com/read/37999817/low-c3-in-a-4-month-old-baby-is-it-a-problem
#17
JOURNAL ARTICLE
Gülşah Kaya Aksoy, Mustafa Gökhan Ertosun, Mustafa Koyun, Elif Çomak, Sema Akman
A 4-month-old male baby was admitted because his father and uncles had chronic kidney disease. His father was diagnosed with membranoproliferative glomerulonephritis at the age of 5, underwent a kidney transplant at the age of 22, and lost the graft due to recurrence of the disease. In contrast, the young uncle was diagnosed with C3 glomerulopathy and mycophenolate mofetil and eculizumab were initiated early. It was remarkable that our patient had normal kidney function and urine analyses but low serum C3 level (0...
November 24, 2023: Pediatric Nephrology
https://read.qxmd.com/read/37994841/-management-of-membranoproliferative-glomerulonephritis-secondary-to-infection-by-hepatitis-c-virus
#18
Ignacio Lasierra Lavilla, Amalia Perona Caro, Celia Del Agua Arias-Camisón, Julien Paola Caballero Castro
Membranoproliferative glomerulonephritis is an uncommon condition that affects the glomeruli of the kidneys; its prevalence has decreased in our environment. Membranoproliferative glomerulonephritis has a characteristic histology that can be associated to different diseases. The clinical presentation varies, and to achieve a definitive diagnosis a renal biopsy must be done. Treatment is based on the underlying disease; when a drop in glomerular filtration rate is detected, immunosuppressants are prescribed...
November 15, 2023: Anales del Sistema Sanitario de Navarra
https://read.qxmd.com/read/37926528/discontinuing-hemodialysis-through-corticosteroid-treatment-in-a-patient-with-cryofibrinogen-associated-glomerulonephritis
#19
JOURNAL ARTICLE
Shigeyuki Arai, Rena Matsui, Wataru Ono, Shinichiro Asakawa, Osamu Yamazaki, Yoshifuru Tamura, Mika Terasaki, Kanade Hagiwara, Tohru Nakagawa, Ryuji Ohashi, Akira Shimizu, Shigeru Shibata, Yoshihide Fujigaki
Cryofibrinogen-associated glomerulonephritis (CryoFiGN) is rare, and its diagnosis is difficult while treatment is not established. We herein report an elderly woman with CryoFiGN who experienced recurrent purpura and nephritic features that subsequently progressed to nephrotic syndrome and required hemodialysis during the 18-month clinical course. The patient was treated with corticosteroids, which led to the discontinuation of hemodialysis. The diagnosis of CryoFiGN was based on the clinical presentation, characteristic glomerular deposits, and results of laser microdissection and liquid chromatography-tandem mass spectrometry of the glomeruli...
November 6, 2023: Internal Medicine
https://read.qxmd.com/read/37926085/functional-magnetic-resonance-imaging-to-monitor-disease-progression-a-prospective-study-in-patients-with-primary-membranoproliferative-glomerulonephritis
#20
JOURNAL ARTICLE
Giulia Villa, Erica Daina, Paolo Brambilla, Sara Gamba, Valentina Fanny Leone, Camillo Carrara, Paola Rizzo, Marina Noris, Giuseppe Remuzzi, Andrea Remuzzi, Anna Caroli
INTRODUCTION: Primary membranoproliferative glomerulonephritis (MPGN) is a rare kidney disease with poor prognosis and no specific therapies. The disease heterogeneity and the difficulty of performing repeated kidney biopsies poses big challenges. This study investigates the correlation between non-contrast enhanced magnetic resonance imaging (MRI) and histologic and clinical findings in patients with primary MPGN. METHODS: Patients with primary MPGN underwent baseline and 1-year kidney MRI in addition to biopsy and laboratory testing as part of a prospective MRI subproject of a clinical trial (ClinicalTrials...
November 3, 2023: Nephron
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