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C3 Glomerulonephritis

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https://www.readbyqxmd.com/read/28637300/commercial-rodent-diets-differentially-regulate-autoimmune-glomerulonephritis-epigenetics-and-microbiota-in-mrl-lpr-mice
#1
Michael R Edwards, Rujuan Dai, Bettina Heid, Thomas E Cecere, Deena Khan, Qinghui Mu, Catharine Cowan, Xin M Luo, S Ansar Ahmed
The course and severity of lupus in spontaneous murine lupus models varies among laboratories, which may be due to variations in diet, housing and/or local environmental conditions. In this study, we investigated the influence of common rodent diets while keeping other factors constant. Female lupus-prone MRL/lpr mice were subjected to the same housing conditions and given one of the three diets: Teklad 7013 containing isoflavone rich soy and alfalfa, Harlan 2018 isoflavone rich soy-based diet, or ResearchDiets Inc...
June 15, 2017: International Immunology
https://www.readbyqxmd.com/read/28628381/monoclonal-immunoglobulin-associated-proliferative-glomerulonephritis-characterized-by-organized-deposits-of-striated-ultra-substructures-a-case-report
#2
Shigeo Hara, Hiroyasu Tsukaguchi, Tastufumi Oka, Makiko Kusabe, Masayuki Mizui, Kensuke Joh
We herein report the case of a 64-year-old male who presented with progressive glomerulonephritis notable for organized and striated ultra-substructures. The patient was diagnosed with hypertension and proteinuria 3 years prior to admission and subsequently developed nephrotic syndrome and impairment of renal function. Laboratory tests did not reveal any evidence of infections or autoimmune diseases. Monoclonal gammopathy was not detected in serum or urine, although a small population of abnormal plasma cell clones was detected by flow cytometry...
June 19, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28614243/different-types-of-glomerulonephritis-associated-with-the-dysregulation-of-the-complement-alternative-pathway-in-2-brothers-a-case-report
#3
Pei Chen, Li Zhu, Feng Yu, Sha-Sha Han, Si-Jun Meng, Wei-Yi Guo, Hong Zhang, Yan Song
RATIONALE: C3 glomerulonephritis (C3GN) and complement-mediated hemolytic uremic syndrome (HUS) both result from the abnormal regulation of the complement system. A significant number of patients with C3GN or complement-mediated HUS have mutations of more than 1 complement protein. This discovery has had a major impact on identifying the underlying cause of familial C3GN or complement-mediated HUS. PATIENT CONCERNS: We report the cases of 2 brothers (herein referred to as patient II-1 and patient II-9), both with complement disorders that differed in their clinical and genetic features...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28593446/c3-glomerulonephritis-with-a-severe-crescentic-phenotype
#4
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
BACKGROUND: C3 glomerulopathy (C3G) is rare type of glomerulonephritis resulting from the glomerular deposition of C3 due to dysregulation of the alternative pathway of complement. It is further subdivided into C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), depending on the ultrastructural features. C3GN usually presents with a membranoproliferative pattern of injury. Crescents may or may not be present. However, we have noted a severe necrotizing and crescentic glomerulonephritis in a small subset of C3GN patients...
June 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28573137/rituximab-for-treatment-of-membranoproliferative-glomerulonephritis-and-c3-glomerulopathies
#5
REVIEW
Michael Rudnicki
Membranoproliferative glomerulonephritis (MPGN) is a histological pattern of injury resulting from predominantly subendothelial and mesangial deposition of immunoglobulins or complement factors with subsequent inflammation and proliferation particularly of the glomerular basement membrane. Recent classification of MPGN is based on pathogenesis dividing MPGN into immunoglobulin-associated MPGN and complement-mediated C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). Current guidelines suggest treatment with steroids, cytotoxic agents with or without plasmapheresis only for subjects with progressive disease, that is, nephrotic range proteinuria and decline of renal function...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28535523/mycophenolate-mofetil-a-possible-alternative-treatment-for-iga-nephropathy
#6
Olga Baraldi, Giorgia Comai, Vania Cuna, Maria Cappuccilli, Carla Serra, Claudio Ronco, Gaetano La Manna
IgA nephropathy (IgAN) is the most common primary glomerulonephritis in developed countries and a leading cause of chronic kidney disease. IgAN is a mesangial proliferative glomerulonephritis characterized by diffuse mesangial deposition of IgA, often accompanied by the deposition of IgG and the C3 component of complement in a similar distribution. This condition is in most cases oligosymptomatic, often discovered coincidentally. Currently, there is no specific treatment available for IgAN and the use of immunosuppression therapy is debated...
2017: Contributions to Nephrology
https://www.readbyqxmd.com/read/28391334/complement-activation-in-pauci-immune-necrotizing-and-crescentic-glomerulonephritis-results-of-a-proteomic-analysis
#7
Sanjeev Sethi, Ladan Zand, An S De Vriese, Ulrich Specks, Julie A Vrana, Siddak Kanwar, Paul Kurtin, Jason D Theis, Andrea Angioi, Lynn Cornell, Fernando C Fervenza
Background: Complement activation plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), although it remains unclear which pathway is activated. Whether pauci-immune necrotizing crescentic glomerulonephritis (pauci-immune GN) with negative ANCA serology is part of the spectrum of AAV or a different disease entity is essentially unknown. Methods: We used proteomic analysis to delineate the complement profile in a series of 13 kidney biopsies of patients with pauci-immune GN, with either proteinase 3 (PR3) (five patients) or myeloperoxidase (MPO) antibodies (four patients) or with consistently negative ANCA serology (four patients)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28385149/successful-treatment-with-bortezomib-and-dexamethasone-for-proliferative-glomerulonephritis-with-monoclonal-igg-deposits-in-multiple-myeloma-a-case-report
#8
Rio Noto, Nozomu Kamiura, Yuichiro Ono, Sumie Tabata, Shigeo Hara, Hideki Yokoi, Akihiro Yoshimoto, Motoko Yanagita
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a form of renal involvement by monoclonal IgG deposits that was found in mesangial, subendothelial or subepithelial regions. The distribution of glomerular deposits was completely different from that in monoclonal immunoglobulin deposition disease. PGNMID is reported to be rarely associated with a hematological malignancy. Previously, only five cases of PGNMID with multiple myeloma have been reported...
April 6, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28366510/controlling-the-anaphylatoxin-c5a-in-diseases-requires-a-specifically-targeted-inhibition
#9
Niels C Riedemann, Maria Habel, Jana Ziereisen, Marlen Hermann, Conny Schneider, Cyrill Wehling, Michael Kirschfink, Karim Kentouche, Renfeng Guo
The terminal complement split product C5a has been described as an important mediator in inflammatory diseases. C5a is generated upon cleavage of C5 and earlier research suggests that, besides the known C5 convertases formed upon activation of the complement pathways, various enzymes could activate C5 directly. We demonstrate that eculizumab effectively blocks C5 activation when mediated by C5-convertase formation, but fails to block C5a generation resulting from direct enzymatic cleavage by trypsin and thrombin...
July 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/28356669/c3-glomerulopathy-associated-to-multiple-myeloma-successfully-treated-by-autologous-stem-cell-transplant
#10
M A Hamzi, A Zniber, G E Badaoui, E Mahtat, Z Alhamany, R Bayahia, N Ouzeddoun
A 32-year-old male presented with advanced renal failure and nephrotic proteinuria due to lambda light chain multiple myeloma. Renal biopsy showed a proliferative glomerulonephritis with isolated C3 deposits. Renal recovery was obtained after chemotherapy and autologous stem cell transplant. We review previously described cases of C3 glomerulopathy associated with monoclonal gammopathy.
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28355401/schistosoma-mansoni-associated-glomerulopathy-with-iga-mesangial-deposits-case-report
#11
Fabiana Oliveira Gonçalves, Tânia Maria de Souza Fontes, Ana Paula Pereira Santana Lemes Canuto
INTRODUCTION: Renal involvement is a severe form of schistosomiasis and occurs in 10% to 15% of patients with the hepatosplenic form of the disease. Nephrotic syndrome is the most common clinical presentation. It is a complication caused by immune complexes (IC), it is rare to appear in the Brazilian context with a immunoglobulin A (IgA) deposits. When installed the renal injury by Schistosoma mansoni, classically presents as membranoproliferative glomerulonephritis (mesangiocapillary) with lobular accentuation...
March 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/28341889/complement-regulation-and-kidney-diseases-recent-knowledge-of-the-double-edged-roles-of-complement-activation-in-nephrology
#12
REVIEW
Masashi Mizuno, Yasuhiro Suzuki, Yasuhiko Ito
The complement activation system plays important roles to maintain homeostasis in the host and to fight foreign invaders to protect the host. Therefore, the complement system is considered a core part of innate immunity which also cross-talks to acquired immunity. In the history of nephrology, the complement system is familiar to us, because complement protein or fragment deposition, including C3, C4, C1q, and/or C4d, is routinely estimated by immunohistochemistry to diagnose renal pathologies. The relationships between pathological mechanisms and complement activation have been investigated for renal diseases such as post-infectious glomerulonephritis, lupus nephritis, and primary membranoproliferative glomerulonephritis, which are usually accompanied by hypocomplementemia...
March 24, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28320458/b-cell-epitope-of-human-cytomegalovirus-phosphoprotein-65-hcmv-pp65-induced-anti-dsdna-antibody-in-balb-c-mice
#13
Ao HoHsieh, Chin Man Wang, Yeong-Jian Jan Wu, Albert Chen, Ming-I Chang, Ji-Yih Chen
BACKGROUND: HCMV phosphoprotein 65 (HCMVpp65) is a putative immunogen that acts as an accelerator, inducing autoantibody and exacerbating autoimmune response in susceptible animals. The immunity to pp65336-439 instigates autoimmunity, suggesting that pp65336-439 contains crucial B cell epitope(s) for the development of nephritis. This study narrowed down the target epitope to pp65422-439 for immunization of BALB/c mice and mapping of B cell epitope. METHODS: The target epitope pp65422-439 reactivity and B cell epitope mapping was examined in serum from pp65422-439-immunized mice and patients with systemic lupus erythematosus (SLE)...
March 21, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28291506/immunotactoid-glomerulopathy-leading-to-the-discovery-of-poems-syndrome%C3%A2
#14
Carole Philipponnet, Jean-Louis Kemeny, Cyril Garrouste, Martin Soubrier, Anne-Elisabeth Heng
Monoclonal gammopathy of renal significance (MGRS) can manifest in many different ways depending on the nature of the immunoglobulin and its physicochemical properties. MGRS can lead to the discovery of a hematological malignancy. We report the case of a 32-year-old female patient who underwent renal biopsy on account of an impure nephrotic syndrome associated with immunoglobulin (Ig)G κ monoclonal gammopathy. Histological analysis revealed membranoproliferative glomerulonephritis with IgG, IgM, κ, λ, and C3 deposits...
March 14, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28210841/rare-genetic-variant-in-the-cfb-gene-presenting-as-atypical-hemolytic-uremic-syndrome-and-immune-complex-diffuse-membranoproliferative-glomerulonephritis-with-crescents-successfully-treated-with-eculizumab
#15
Khalid Alfakeeh, Mohammed Azar, Majid Alfadhel, Alsuayri Mansour Abdullah, Nourah Aloudah, Khaled O Alsaad
BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28210641/atypical-plasmacytic-proliferation-in-a-case-of-c3-glomerulopathy-pathophysiology-demystified
#16
Osama Elfituri, Nathan Aardsma, Suman Setty, Frederick Behm, Kimberly Czech
An 11-year-old Hispanic female underwent evaluation of asymptomatic proteinuria and hematuria. The patient denied fever, edema, and gross hematuria. Urinalysis showed mild proteinuria, and a urine microscopic examination revealed red blood cells. Screening tests for glomerulonephritis revealed a low C3 and negative ANA, ASO, DNAse-B, and ANCA. Histological examination of a renal biopsy specimen showed glomeruli with endocapillary proliferation, a predominant C3 deposition in the capillary loops by immunofluorescence, and electron dense deposits in the mesangium, paramesangium, and capillary walls by electron microscopy consistent with a diagnosis of C3 glomerulopathy...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28205354/mini-review-a-unique-case-of-crescentic-c3-glomerulonephritis
#17
Dharmenaan Palamuthusingam, Murty Mantha, Kimberley Oliver, Ketan Bavishi, Shyam Dheda
Kidney involvement is an under-recognized complication of non-Hodgkin lymphomas. They occur in a variety of mechanisms and differ widely in their clinical presentation. We take this opportunity to report a case of a 65 year-old man who developed a rapidly progressive glomerulonephritis within days after completing his first cycle of R-CHOP (Rituximab, Cyclophosphamide, Doxorubicin, Vincristine, Prednisolone) chemotherapy for newly diagnosed mantle cell lymphoma. He was odematous, hypertensive, oliguric with nephrotic range proteinuria and an active urine sediment...
March 2017: Nephrology
https://www.readbyqxmd.com/read/28176473/durable-remission-of-c3-glomerulonephritis-with-mycophenolate-mofetil
#18
REVIEW
Nicole Lioufas, Moira Finlay, Thomas Barbour
In C3 glomerulopathy, uncontrolled complement C3 activation via the alternative pathway results in glomerular C3 deposition and, in many cases, progressive renal failure. Despite advances in understanding of C3G pathogenesis over the last few years, there are no proven treatments. We describe a patient in whom C3 glomerulopathy was associated with renal impairment and elevated serum free kappa light chains. An initial response to corticosteroids was followed by relapse once steroids were weaned, prompting use of mycophenolate mofetil to maintain remission...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28162051/henoch-schonlein-purpura-post-streptococcal-glomerulonephritis-and-acute-rheumatic-carditis-after-group-a-%C3%AE-haemolytic-streptococcal-infection
#19
Seçil Arslansoyu Çamlar, Alper Soylu, İpek Akil, Mehtat Ünlü, Şenol Coşkun, Pelin Ertan, Salih Kavukçu
Besides association with acute rheumatic fever (ARF) and acute glomerulonephritis (APSGN), in up to 40% of cases, Group A β-haemolytic streptococcal (GABHS) infections are also implicated as a trigger for Henoch-Schonlein purpura (HSP). A 7-year-old girl with GABHS throat infection who developed HSP, APSGN and rheumatic carditis is reported. She presented with palpable purpura and arthritis in both ankles and later developed carditis characterised by mitral/aortic regurgitation and glomerulonephritis characterised by mixed nephritic/nephrotic syndrome...
February 6, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28098102/evaluation-of-renal-lesions-and-clinicopathologic-correlation-in-rheumatoid-arthritis
#20
Periyasamy Muthukumar, Jeyachandran Dhanapriya, Natarajan Gopalakrishnan, Thanigachalam Dineshkumar, Ramanathan Sakthirajan, T Balasubramaniyan
The most common causes of renal disease in rheumatoid arthritis (RA) are glomerulonephritis (GN), amyloidosis, tubulo-interstitial nephritis, and drug toxicity. Our aim was to evaluate the clinicopathologic correlation of renal lesions and to assess the course and prognosis of renal disease in patients with RA. We conducted a prospective observational study in all adult patients with RA between July 2010 and June 2015. The total number of patients studied was 90, with a female:male ratio of 2.3:1. Mean follow-up duration was 30 ± 6...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
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