keyword
https://read.qxmd.com/read/38538072/c3-concentrations-can-be-normal-in-patients-with-c3-glomerulopathy-secondary-to-c3-nephritic-factor
#1
JOURNAL ARTICLE
Hamish Anderson, Mark Van Voorthuizen, John O'Donnell, Sarah Beck
C3 glomerulopathy (C3G) is a rare kidney disease caused by the glomerular deposition of C3 fragments secondary to alternative pathway complement dysregulation. C3 nephritic factors (C3Nef) are the most common acquired cause, and their detection has treatment and prognostic implications. Although C3 concentration can be normal in the presence of C3Nef, many laboratories will only perform C3Nef testing when C3 is low. We performed a retrospective study of all positive C3Nef results from the authors' laboratory since 2015 and found that two of the four patients with positive C3Nef and biopsy-confirmed C3G had normal C3 concentrations...
March 27, 2024: Journal of Clinical Pathology
https://read.qxmd.com/read/38481517/c3-glomerulopathy-novel-treatment-paradigms
#2
REVIEW
Blanca Tarragon Estebanez, Andrew S Bomback
C3 glomerulopathy (C3G) is diagnosed by kidney biopsy, with immunofluorescence showing isolated or dominant C3 staining, indicating hyperactivity of the alternative complement pathway as the key driver of glomerular injury. Therefore, the lesion is defined by its complement-mediated pathogenesis as much as its histological pattern. As a bevy of complement-targeting agents are moving through development and clinical trials, we review the evolution in treatment paradigms for C3G. Here we survey the limited efficacy of noncomplement targeting therapy before focusing on the work being done on targeting various components of the complement cascade in aiming to provide disease-specific therapy...
March 2024: KI Reports
https://read.qxmd.com/read/38481513/digital-ischemia-as-a-rare-manifestation-of-c3-glomerulopathy-associated-with-monoclonal-gammopathy
#3
JOURNAL ARTICLE
Alistair Baber, Paul Legendre, Sophie Chauvet, Alexandre Karras, Samuel Deshayes, Antoine Huart, Marguerite Vignon, Kathleen Dessaix, Baptiste Hervier, Bruno Legallicier, Nicolas Martin Silva, Véronique Frémeaux-Bacchi, Benjamin Terrier
No abstract text is available yet for this article.
March 2024: KI Reports
https://read.qxmd.com/read/38447879/apolipoprotein-e-is-enriched-in-dense-deposits-and-is-a-marker-for-dense-deposit-disease-in-c3-glomerulopathy
#4
JOURNAL ARTICLE
Benjamin Madden, Raman Deep Singh, Mark Haas, Lilian Mp Palma, Alok Sharma, Maria J Vargas, LouAnn Gross, Vivian Negron, Torell Nate, M Cristine Charlesworth, Jason D Theis, Samih H Nasr, Karl A Nath, Fernando C Fervenza, Sanjeev Sethi
C3 glomerulopathy (C3G) is a rare disease resulting from dysregulation of the alternative pathway of complement. C3G includes C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), both of which are characterized by bright glomerular C3 staining on immunofluorescence studies. However, on electron microscopy (EM), DDD is characterized by dense osmiophilic mesangial and intramembranous deposits along the glomerular basement membranes (GBM), while the deposits of C3GN are not dense. Why the deposits appear dense in DDD and not in C3GN is not known...
March 4, 2024: Kidney International
https://read.qxmd.com/read/38423159/hot-spot-of-complement-factor-i-rare-variant-p-ile357met-in-patients-with-hemolytic-uremic-syndrome
#5
Nora Schwotzer, Fadi Fakhouri, Paula Vieira Martins, Yahsou Delmas, Sophie Caillard, Julien Zuber, Olivier Moranne, Laurent Mesnard, Véronique Frémeaux-Bacchi, Carine El-Sissy
Atypical haemolytic uremic syndrome (aHUS) is a rare kidney disease due to a dysregulation of the complement alternative pathway (AP). Complement factor I (CFI) negatively regulates the AP and CFI gene rare variants have been associated to aHUS with a low disease penetrance. We report 10 unrelated cases of HUS associated to a rare CFI variant p.Ile357Met (c.1071T>G). All patients with isolated p.Ile357Met CFI missense variant were retrospectively identified among patients included between January 2007 and January 2022 in the French HUS Registry...
February 27, 2024: American Journal of Kidney Diseases
https://read.qxmd.com/read/38412598/recurrent-c3-glomerulopathy-after-kidney-transplantation
#6
REVIEW
Shota Obata, Pedro A S Vaz de Castro, Leonardo V Riella, Paolo Cravedi
The complement system is part of innate immunity and is pivotal in protecting the body against pathogens and maintaining host homeostasis. Activation of the complement system is triggered through multiple pathways, including antibody deposition, a mannan-binding lectin, or activated complement deposition. C3 glomerulopathy (C3G) is a rare glomerular disease driven by complement dysregulation with high post-transplantation recurrence rates. Its treatment is mainly based on immunosuppressive therapies, specifically mycophenolate mofetil and glucocorticoids...
February 21, 2024: Transplantation Reviews
https://read.qxmd.com/read/38401844/triple-fusion-protein-trifu-a-potent-targeted-enzyme-like-inhibitor-of-all-three-complement-activation-pathways
#7
JOURNAL ARTICLE
Sophia J Sonnentag, Arthur Dopler, Katharina Kleiner, Brijesh K Garg, Marco Mannes, Nadja Späth, Amira Akilah, Britta Höchsmann, Hubert Schrezenmeier, Markus Anliker, Ruby Boyanapalli, Markus Huber-Lang, Christoph Q Schmidt
The introduction of a therapeutic anti-C5 antibody into clinical practice in 2007 inspired a surge into the development of complement-targeted therapies. This has led to the recent approval of a C3 inhibitory peptide, an antibody directed against C1s and a full pipeline of several complement inhibitors in preclinical and clinical development. However, no inhibitor is available that efficiently inhibits all three complement initiation pathways and targets host cell surface markers as well as complement opsonins...
February 22, 2024: Journal of Biological Chemistry
https://read.qxmd.com/read/38379641/case-report-thrombotic-thrombocytopenic-purpura-in-a-pregnant-woman-with-lupus-membranous-nephropathy-a-diagnostic-challenge
#8
Marina Leiva, Gustavo Navarro, J Daniel Carpio, Leopoldo Ardiles
A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2...
2024: Front Nephrol
https://read.qxmd.com/read/38373846/eculizumab-dose-tapering-should-take-into-account-the-nonlinearity-of-its-pharmacokinetics
#9
JOURNAL ARTICLE
Olivier Le Tilly, Philippe Gatault, Saida Semlali, Rebecca Sberro-Soussan, Christophe Passot, Dominique Bertrand, Céline Desvignes, Sophie Caillard, Gilles Paintaud, Jean-Michel Halimi, David Ternant
AIMS: Eculizumab is a monoclonal antibody targeting complement protein C5 used in renal diseases. As recommended dosing regimen leads to unnecessarily high concentrations in some patients, tailored dosing therapeutic drug monitoring was proposed to reduce treatment cost. The objectives of the present work were (i) to investigate the target-mediated elimination of eculizumab and (ii) whether a pharmacokinetic model integrating a nonlinear elimination allows a better prediction of eculizumab concentrations than a linear model...
February 19, 2024: British Journal of Clinical Pharmacology
https://read.qxmd.com/read/38368725/efficacy-of-galnac-c3-sirnas-in-factor-h-deficient-mice-with-c3-glomerulopathy
#10
JOURNAL ARTICLE
Cristina Zanchi, Monica Locatelli, Domenico Cerullo, Verena Aumiller, Daniela Corna, Daniela Rottoli, Steffen Schubert, Marina Noris, Susanna Tomasoni, Giuseppe Remuzzi, Carlamaria Zoja, Ariela Benigni
Complement alternative pathway (AP) dysregulation drives C3 glomerulopathy (C3G), a rare renal disorder characterized by glomerular C3 deposition and glomerular damage, for which no effective treatments are available. Blockade of complement C3 is emerging as a viable therapeutic option. In an earlier study we showed that SLN500, a small interfering RNA targeting liver C3 synthesis, was able to limit AP dysregulation and glomerular C3d deposits in mice with partial factor H (FH) deficiency (Cfh+/- mice). Here, we assessed the pharmacological effects of SLN501 - an optimized SLN500 version - in mice with complete FH deficiency (Cfh-/- mice) that exhibit a more severe C3G phenotype...
February 17, 2024: Molecular Immunology
https://read.qxmd.com/read/38344720/defining-nephritic-factors-as-diverse-drivers-of-systemic-complement-dysregulation-in-c3-glomerulopathy
#11
JOURNAL ARTICLE
Jill J Hauer, Yuzhou Zhang, Renee Goodfellow, Amanda Taylor, Nicole C Meyer, Sarah Roberts, Dingwu Shao, Lauren Fergus, Nicolo Ghiringhelli Borsa, Monica Hall, Carla M Nester, Richard J H Smith
INTRODUCTION: C3 glomerulopathy (C3G) is an ultrarare renal disease characterized by deposition of complement component C3 in the glomerular basement membrane (GBM). Rare and novel genetic variation in complement genes and autoantibodies to complement proteins are commonly identified in the C3G population and thought to drive the underlying complement dysregulation that results in renal damage. However, disease heterogeneity and rarity make accurately defining characteristics of the C3G population difficult...
February 2024: KI Reports
https://read.qxmd.com/read/38267800/rationale-and-design-of-the-japanese-biomarkers-in-nephrotic-syndrome-j-marine-study
#12
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/38252289/anti-factor-b-antibodies-in-atypical-hemolytic-uremic-syndrome
#13
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/38213351/c3-glomerulonephritis-presenting-with-nephritic-and-nephrotic-syndromes-spontaneous-remission-after-six-months-on-dialysis
#14
Francisco Gonçalves, Nídia Marques, Roberto Silva, Luis Mendonça, Bernardo Faria
C3 glomerulopathy is a rare and complex renal disease driven by complement dysregulation, with variable presentation and pathophysiology. We report the case of a middle-aged male patient presenting with nephritic and nephrotic syndromes and low serum C3, whose biopsy established the diagnosis of C3 glomerulonephritis. He was found to be homozygous for the complement factor H-related protein (CFHR)3-CFHR1 deletion, which has been associated with the development of anti-factor H autoantibodies. However, the lack of consistent and accessible nephritic factor assays prevented full clarification of the mechanisms involved in the disease...
December 2023: Curēus
https://read.qxmd.com/read/38158966/-comparative-characteristics-of-the-complement-system-in-patients-with-c3-glomerulopathy-and-atypical-hemolytic-uremic-syndrome-of-chronic-course-who-suffered-an-acute-episode-of-thrombotic-microangiopathy
#15
JOURNAL ARTICLE
V A Yurova, N L Kozlovskaya, L A Bobrova, L V Kozlov, S S Andina, K A Demyanova
AIM: To compare changes in the complement system in C3-glomerulopathy (C3-GP) and atypical hemolytic uremic syndrome (aHUS) after the relief of an acute episode of thrombotic microangiopathy. MATERIALS AND METHODS: The study included 8 patients diagnosed with C3-GP and 8 with aHUS in remission. The blood levels of the complement system components were determined: C3, C4, C3a, C5a, factor H (CFH), factor B (CFB), membrane-attacking complex (MAC), antibodies to C3b (anti-C3b-AT), the level of hemolytic activity (CH50), the content of factor D (CFD) in the urine...
August 17, 2023: Terapevticheskiĭ Arkhiv
https://read.qxmd.com/read/38106570/efficacy-and-safety-of-iptacopan-in-patients-with-c3-glomerulopathy
#16
JOURNAL ARTICLE
Edwin Wong, Carla Nester, Teresa Cavero, Alexandre Karras, Moglie Le Quintrec, Liz Lightstone, Ute Eisenberger, Maria Jose Soler, David Kavanagh, Erica Daina, Manuel Praga, Nicholas R Medjeral-Thomas, Anja Gäckler, Clara Garcia-Carro, Andrea Biondani, Frederique Chaperon, Kenneth Kulmatycki, Julie Milojevic, Nicholas J A Webb, Prasanna Kumar Nidamarthy, Guido Junge, Giuseppe Remuzzi
INTRODUCTION: Complement 3 glomerulopathy (C3G) is a rare inflammatory kidney disease mediated by dysregulation of the alternative complement pathway. No targeted therapy exists for this aggressive glomerulonephritis. Efficacy, safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) (measured by complement biomarkers) of iptacopan were assessed in patients with C3G. METHODS: In this phase 2, multicenter, open-label, single-arm, nonrandomized study, adults with biopsy-proven, native kidney C3G (native cohort) and kidney transplant recipients with C3G recurrence (recurrent kidney transplant [KT] cohort) received iptacopan twice daily (bid) for 84 days (days 1-21: 10-100 mg; days 22-84: 200 mg)...
December 2023: KI Reports
https://read.qxmd.com/read/38076230/c3-glomerulopathies-dense-deposit-disease-and-c3-glomerulonephritis
#17
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/38057242/-translated-article-pharmacokinetics-of-eculizumab-in-adult-and-pediatric-patients-with-atypical-hemolytic-uremic-syndrome-and-c3-glomerulopathy
#18
JOURNAL ARTICLE
Alba Pau Parra, Natalia Ramos, Janire Perurena-Prieto, Silvia Manrique-Rodríguez, Monica Climente, Laura García Quintanilla, Ángel Escolano, Marta Miarons
OBJECTIVE: The objective of the study was to analyze and describe the concentrations of eculizumab and the complement blockade in patients with atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy, and to define a therapeutic margin where there is a high probability of achieving therapeutic efficacy. METHODS: Observational, ambispective, and multicenter study that included adult and pediatric patients diagnosed with aHUS and C3 glomerulopathy from September 2020 to October 2022 in 5 hospitals in Spain...
December 5, 2023: Farmacia Hospitalaria
https://read.qxmd.com/read/38041748/complement-gene-mutations-in-children-with-c3-glomerulopathy-do-they-affect-the-response-to-mycophenolate-mofetil
#19
JOURNAL ARTICLE
Neslihan Günay, İsmail Dursun, İbrahim Gökçe, Mehtap Akbalık Kara, Demet Tekcan, Neslihan Çiçek, Meral Torun Bayram, Mustafa Koyun, Nida Dinçel, Hasan Dursun, Seha Saygılı, Zeynep Nagehan Yürük Yıldırım, Selçuk Yüksel, Osman Dönmez, Sibel Yel, Beltinge Demircioğlu Kılıç, Özlem Aydoğ, Bahriye Atmış, Aysun Çaltık Yılmaz, Sevcan A Bakkaloğlu, Mehmet Baha Aytaç, Mehmet Taşdemir, Belde Kasap Demir, Alper Soylu, Elif Çomak, Aslı Kantar Özşahin, Alper Kaçar, Nur Canpolat, Alev Yılmaz, İlknur Girişgen, Kadirye Betül Akkoyunlu, Harika Alpay, Hakan M Poyrazoğlu
BACKGROUND: C3 glomerulopathy (C3G) is a complement-mediated disease. Although genetic studies are not required for diagnosis, they are valuable for treatment planning and prognosis prediction. The aim of this study is to investigate the clinical phenotypes, kidney survival, and response to mycophenolate mofetil (MMF) treatment in pediatric C3G patients with and without mutations in complement-related genes. METHODS: Sixty pediatric C3G patients were included, divided into two groups based on complement-related gene mutations...
December 2, 2023: Pediatric Nephrology
https://read.qxmd.com/read/38025230/clinical-safety-and-efficacy-of-pegcetacoplan-in-a-phase-2-study-of-patients-with-c3-glomerulopathy-and-other-complement-mediated-glomerular-diseases
#20
JOURNAL ARTICLE
Bradley P Dixon, Larry A Greenbaum, Liwei Huang, Sandeep Rajan, Chunlei Ke, Yiwei Zhang, Li Li
INTRODUCTION: Dysregulated complement activation is likely the primary driver of disease in C3 glomerulopathy (C3G) and contributes to other complement-mediated diseases, including immunoglobulin A nephropathy (IgAN), lupus nephritis (LN), and primary membranous nephropathy (PMN). No complement inhibitors are proven to halt disease progression in these diseases. Pegcetacoplan, a targeted C3 and C3b inhibitor, may mitigate complement-mediated kidney damage in C3G and other glomerular diseases in which complement may have a pathogenic role...
November 2023: KI Reports
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