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C3 glomerulopathy

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https://www.readbyqxmd.com/read/29729982/c3-glomerulopathy-associated-with-monoclonal-ig-is-a-distinct-subtype
#1
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
Monoclonal immunoglobulins (MIg) may play a causal role in C3 glomerulopathy (C3G) by impairing regulation of the alternative pathway of complement. Ninety-five patients with C3G were tested for MIg of which 36 were positive. Their mean age at diagnosis was 60 years and among patient 50 years and older, 65.1% had a MIg. At presentation, median serum creatinine and proteinuria were 1.9 mg/dL and 3.0 g/24 hours. Hematuria was present in 32 (88.9%) patients. Twelve (34.3%) patients had low C3 levels. C3 nephritic factor was detected in 45...
May 2, 2018: Kidney International
https://www.readbyqxmd.com/read/29724182/favorable-effect-of-bortezomib-in-dense-deposit-disease-associated-with-monoclonal-gammopathy-a-case-report
#2
Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki
BACKGROUND: Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29724174/eculizumab-as-salvage-therapy-for-recurrent-monoclonal-gammopathy-induced-c3-glomerulopathy-in-a-kidney-allograft
#3
Philipp Moog, Philipp J Jost, Maike Büttner-Herold
BACKGROUND: Monoclonal gammopathy causes several kinds of renal pathology. A rare and special form is monoclonal gammopathy-induced C3 glomerulopathy (MG-C3G). Like idiopathic C3G, MG-C3G frequently leads to end-stage renal disease. MG-C3G frequently recurs after renal transplantation, leading to graft failure in most of the patients. While there is some evidence for successful treatment of recurrent idiopathic C3 glomerulopathy with eculizumab after renal transplantation, nothing is known about its efficacy in the setting of recurrent MG-C3G...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29713256/primary-glomerulonephritis-with-unique-c4d-deposition-and-concurrent-non-infectious-intermediate-uveitis-a-case-report-and-literature-review
#4
Jong Man Park, Harin Lee, Sangheon Song, Eun Young Seong, Ihm Soo Kwak, Sung Who Park, Young-Keum Kim, Nari Shin, Mee Young Sol
C4 glomerulopathy is a recently introduced entity that presents with bright C4d staining and minimal or absent immunoglobulin and C3 staining. We report a case of a 62-year-old man with C4 glomerulonephritis (GN) and uveitis. He presented to the nephrology department with proteinuria and hematuria. The patient also had intermediate uveitis along with proteinuria and hematuria. A kidney biopsy that was performed in light of continuing proteinuria and hematuria showed a focal proliferative, focal sclerotic glomerulopathy pattern on light microscopy, absent staining for immunoglobulin or C3 by immunofluorescence microscopy, with bright staining for C4d on immunohistochemistry, and electron-dense deposits on electron microscopy...
April 30, 2018: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29706017/distribution-of-exogenous-complement-factor-h-in-mice-in-vivo
#5
Aino R Koskinen, Zhu-Zhu Cheng, Matthew C Pickering, Kalevi Kairemo, Taru Meri, H Terence Cook, Seppo Meri, T Sakari Jokiranta
Factor H is an important regulator of complement activation in plasma and on cell surfaces in both humans and mice. If FH function is compromised, inappropriate complement activation on self-surfaces can have disastrous effects as seen in the kidney diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy. Since FH constructs have been proposed to be used in treatment for these diseases, we studied the distribution of exogenous FH fragments in mice. Full-length mFH, mFH1-5 and mFH18-20 fragments were radiolabeled and their distribution was examined in WT, FH-/- and FH-/- C3-/- mice in vivo...
April 29, 2018: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#6
Sanjeev Sethi, S Vincent Rajkumar, Vivette D D'Agati
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage...
April 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29670616/overactivity-of-alternative-pathway-convertases-in-patients-with-complement-mediated-renal-diseases
#7
Marloes A H M Michels, Nicole C A J van de Kar, Marcin Okrój, Anna M Blom, Sanne A W van Kraaij, Elena B Volokhina, Lambertus P W J van den Heuvel
Overactivation of the alternative pathway of the complement system is associated with the renal diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). C3 nephritic factors (C3NeF) play an important role in C3G pathogenesis by stabilizing the key enzymatic complex of complement, the C3 convertase. However, the reliability of assays detecting these autoantibodies is limited. Therefore, in this study, we validated and optimized a prototype hemolytic method for robust detection and characterization of factors causing convertase overactivity in large patient cohorts...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29594148/c3-glomerulopathy-and-atypical-hemolytic-uremic-syndrome-two-important-manifestations-of-complement-system-dysfunction
#8
Ravneet Bajwa, John A DePalma, Taimoor Khan, Anmol Cheema, Sheila A Kalathil, Mohammad A Hossain, Attiya Haroon, Anne Madhurima, Min Zheng, Ali Nayer, Arif Asif
The advances in our understanding of the alternative pathway have emphasized that uncontrolled hyperactivity of this pathway causes 2 distinct disorders that adversely impact the kidney. In the so-called atypical hemolytic uremic syndrome (aHUS), renal dysfunction occurs along with thrombocytopenia, anemia, and target organ injury to multiple organs, most commonly the kidney. On the other hand, in the so-termed C3 glomerulopathy, kidney involvement is not associated with thrombocytopenia, anemia, or other system involvement...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29592796/c3-glomerulopathy-in-cystic-fibrosis-a-case-report
#9
Domenico Santoro, Rossella Siligato, Carmela Vadalà, Mariacristina Lucanto, Simona Cristadoro, Giovanni Conti, Michele Buemi, Stefano Costa, Ettore Sabadini, Giuseppe Magazzù
BACKGROUND: C3 glomerulonephritis is a rare glomerulopathy characterized at renal biopsy by C3 deposition, alone or with scanty immunoglobulins, as well as by an electron-dense material in mesangium, subendothelial and subepithelial space. An abnormal systemic activation of the alternative pathway of the complement cascade is responsible for the development of the disease if triggered by several possible environmental conditions. We report the first case in literature of a patient affected by cystic fibrosis and C3GN...
March 28, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29588430/an-engineered-complement-factor-h-construct-for-treatment-of-c3-glomerulopathy
#10
Yi Yang, Harriet Denton, Owen R Davies, Kate Smith-Jackson, Heather Kerr, Andrew P Herbert, Paul N Barlow, Matthew C Pickering, Kevin J Marchbank
Background C3 glomerulopathy (C3G) is associated with dysregulation of the alternative pathway of complement activation, and treatment options for C3G remain limited. Complement factor H (FH) is a potent regulator of the alternative pathway and might offer a solution, but the mass and complexity of FH makes generation of full-length FH far from trivial. We previously generated a mini-FH construct, with FH short consensus repeats 1-5 linked to repeats 18-20 (FH1-5^18-20 ), that was effective in experimental C3G...
March 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29566171/genetic-analysis-of-the-complement-pathway-in-c3-glomerulopathy
#11
Weiwei Zhao, Yin Ding, Jianping Lu, Tao Zhang, Dacheng Chen, Haitao Zhang, Caihong Zeng, Zhihong Liu, Huimei Chen
Background: C3 glomerulopathy often presents with a membranoproliferative glomerulonephritis (MPGN) pattern, and is principally caused by unrestricted activation of the complement alternative pathway. Genetic abnormalities of the complement system critically implicate in the pathogenesis of C3 glomerulopathy, but a systemic profile remains open, especially in Asia. Methods: In this study, we completed a comprehensive screen of 11 candidate alternative pathway genes by using targeted genomic enrichment and massively parallel sequencing on 43 patients with sporadic C3 glomerulopathy, which were classified as dense deposit disease (DDD; n = 10) and C3 glomerulonephritis (C3GN; n = 33) cases...
March 16, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29522131/the-complexity-of-renal-involvment-in-ibd-c3-glomerulopathy-in-ulcerative-colitis
#12
Pedro Marques da Costa, Lurdes Correia, Luís Araújo Correia
No abstract text is available yet for this article.
April 23, 2018: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/29517501/evolving-complexity-of-complement-related-diseases-c3-glomerulopathy-and-atypical-haemolytic-uremic-syndrome
#13
H Terence Cook
PURPOSE OF REVIEW: The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS). RECENT FINDINGS: C3 glomerulopathy and aHUS are associated with abnormalities of control of the alternative pathway of complement. Recent articles have provided new insights into the classification of C3 glomerulopathy and its relationship to idiopathic immune complex-mediated glomerulonephritis...
May 2018: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/29516279/eculizumab-reversed-severe-distal-ischemic-syndrome-and-glomerulonephritis-with-isolated-c3-deposits-associated-with-anti-factor-h-autoantibodies-a-case-report
#14
REVIEW
Samuel Deshayes, Nicolas Martin Silva, Valérie Chatelet, Sylvain Chantepie, Moglie Le Quintrec, François Comoz, Frank Bridoux, Marie-Agnès Dragon-Durey, Achille Aouba
B-cell clones can produce a monoclonal immunoglobulin, which may be responsible for visceral involvements. Kidney involvement is frequent, affecting 20 to 50% of patients with multiple myeloma. One mechanism underlying this involvement is a dysregulation of the complement alternative pathway, leading to C3 glomerulopathies. We report a patient who had a multiple myeloma, C3 glomerulopathy related to factor H autoantibody, and digital ischemia, who was treated successfully with eculizumab, an anti-complement therapy, without any relapse in 2 years of follow-up...
April 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29500241/statistical-validation-of-rare-complement-variants-provides-insights-into-the-molecular-basis-of-atypical-hemolytic-uremic-syndrome-and-c3-glomerulopathy
#15
Amy J Osborne, Matteo Breno, Nicolo Ghiringhelli Borsa, Fengxiao Bu, Véronique Frémeaux-Bacchi, Daniel P Gale, Lambertus P van den Heuvel, David Kavanagh, Marina Noris, Sheila Pinto, Pavithra M Rallapalli, Giuseppe Remuzzi, Santiago Rodríguez de Cordoba, Angela Ruiz, Richard J H Smith, Paula Vieira-Martins, Elena Volokhina, Valerie Wilson, Timothy H J Goodship, Stephen J Perkins
Atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) are associated with dysregulation and overactivation of the complement alternative pathway. Typically, gene analysis for aHUS and C3G is undertaken in small patient numbers, yet it is unclear which genes most frequently predispose to aHUS or C3G. Accordingly, we performed a six-center analysis of 610 rare genetic variants in 13 mostly complement genes ( CFH , CFI , CD46 , C3 , CFB , CFHR1 , CFHR3 , CFHR4 , CFHR5 , CFP , PLG , DGKE , and THBD ) from >3500 patients with aHUS and C3G...
April 1, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29483359/fhr5-binds-to-laminins-uses-separate-c3b-and-surface-binding-sites-and-activates-complement-on-malondialdehyde-acetaldehyde-surfaces
#16
Ramona B Rudnick, Qian Chen, Emma Diletta Stea, Andrea Hartmann, Nikolina Papac-Milicevic, Fermin Person, Michael Wiesener, Christoph J Binder, Thorsten Wiech, Christine Skerka, Peter F Zipfel
Factor H related-protein 5 (CFHR5) is a surface-acting complement activator and variations in the CFHR5 gene are linked to CFHR glomerulonephritis. In this study, we show that FHR5 binds to laminin-521, the major constituent of the glomerular basement membrane, and to mesangial laminin-211. Furthermore, we identify malondialdehyde-acetaldehyde (MAA) epitopes, which are exposed on the surface of human necrotic cells ( Homo sapiens ), as new FHR5 ligands. Using a set of novel deletion fragments, we show that FHR5 binds to laminin-521, MAA epitopes, heparin, and human necrotic cells (HUVECs) via the middle region [short consensus repeats (SCRs) 5-7]...
April 1, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29477994/recurrent-postinfectious-glomerulonephritis-an-unusual-evolution-compatible-with-c3-glomerulopathy
#17
Jose Luis Espinosa-Figueroa, Marta Cano-Megías, Patricia Martínez-Miguel, Mercedes Velo-Plaza
Acute endocapillary glomerulonephritis, as its name suggests, is a one-time process, which usually resolves within weeks. However, in a small percentage of patients, the disease becomes chronic. In these cases, a deregulation in the alternative complement pathway, which can be caused by mutations or autoantibodies, has been proposed as a pathophysiological mechanism. As a result, the alternative complement pathway remains active after resolution of infection. We report a patient with two episodes of acute renal failure, both times diagnosed by renal biopsy of acute endocapillary glomerulonephritis, with slow recovery after two episodes of low-serum complement C3, haematuria and proteinuria...
February 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29475991/glucocorticoids-in-the-treatment-of-glomerular-diseases-pitfalls-and-pearls
#18
REVIEW
Claudio Ponticelli, Francesco Locatelli
Glucocorticoids exert anti-inflammatory and immunosuppressive activities by genomic and nongenomic effects. The classic genomic effects are mediated by cytosolic glucocorticoid receptors that can upregulate the expression of anti-inflammatory proteins in the nucleus (transactivation) or repress the translocation of proinflammatory transcription factors from the cytosol into the nucleus (transrepression). The nongenomic effects are probably mediated by membrane glucocorticoid receptors. Glucocorticoid receptors are expressed also in podocytes and experimental data suggest that glucocorticoids may protect from podocyte injury...
May 7, 2018: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29457859/post-infectious-acute-glomerulonephritis-with-podocytopathy-induced-by-parvovirus-b19-infection
#19
Satoshi Hara, Masayoshi Hirata, Kiyoaki Ito, Ichiro Mizushima, Hiroshi Fujii, Kazunori Yamada, Michio Nagata, Mitsuhiro Kawano
Human parvovirus B19 infection causes a variety of glomerular diseases such as post-infectious acute glomerulonephritis and collapsing glomerulopathy. Although each of these appears independently, it has not been fully determined why parvovirus B19 provokes such a variety of different glomerular phenotypes. Here, we report a 68-year-old Japanese man who showed endocapillary proliferative glomerulonephritis admixed with podocytopathy in association with parvovirus B19 infection. The patient showed acute onset of heavy proteinuria, microscopic hematuria and kidney dysfunction with arthralgia and oliguria after close contact with a person suffering from erythema infectiosum...
March 2018: Pathology International
https://www.readbyqxmd.com/read/29450785/the-role-of-the-alternative-pathway-of-complement-activation-in-glomerular-diseases
#20
REVIEW
Emilia Łukawska, Magdalena Polcyn-Adamczak, Zofia I Niemir
The complement system (CS) has recently been recognized as a bridge between innate and adaptive immunity that constitutes a very complex mechanism controlling the clearance of pathogens, cellular debris, and immune complexes. Out of three known pathways of complement activation, the alternative pathway (AP) plays a critical role in host defense by amplifying the complement response, independently of initiation pathway and continuously maintaining low-level activity in a process called 'thick-over.' A key molecule of the CS is C3, in which the AP is constantly activated...
February 15, 2018: Clinical and Experimental Medicine
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