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"C3 glomerulonephritis"

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https://www.readbyqxmd.com/read/27878657/c3-glomerulopathy-and-current-dilemmas
#1
Naoko Ito, Ryuji Ohashi, Michio Nagata
C3 glomerulopathy (C3G) is a recently identified disease entity caused by dysregulation of the alternative complement pathway, and dense deposit disease (DDD) and C3 glomerulonephritis (C3GN) are its components. Because laboratory detection of complement dysregulation is still uncommon in practice, "dominant C3 deposition by two orders greater than that of immunoglobulins in the glomeruli by immunofluorescence", as stated in the consensus report, defines C3G. However, this morphological definition possibly includes the cases with glomerular diseases of different mechanisms such as post-infectious glomerulonephritis...
November 23, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27717365/severe-active-c3-glomerulonephritis-triggered-by-immune-complexes-and-inactivated-after-eculizumab-therapy
#2
Tanja Kersnik Levart, Dušan Ferluga, Alenka Vizjak, Jerica Mraz, Nika Kojc
BACKGROUND: Understanding the role of alternative complement pathway dysregulation in membranoproliferative glomerulonephritis (MPGN) has led to a dramatic shift in its classification into two subgroups: immune complex-mediated MPGN and complement-mediated MPGN, consisting of dense deposit disease and C3 glomerulonephritis (C3GN). A limited number of C3GN cases have been published to date with not yet conclusive results since the novel therapeutic approach with eculizumab was introduced...
October 7, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27631242/c3-glomerulonephritis-in-multiple-myeloma-a-case-report-and-literature-review
#3
Guang Yin, Zhen Cheng, Cai-Hong Zeng, Zhi-Hong Liu
BACKGROUND: C3 glomerulonephritis (C3 GN) is a recently defined entity characterized by predominant C3 deposition in glomeruli due to abnormal activation of the alternative pathway of complement system. C3 GN has been reported to be associated with several systemic diseases. However, the association between C3 GN and multiple myeloma (MM) has not been well established. METHODS: We herein describe a case presenting with C3 GN on top of MM. RESULTS: A 64-year-old Chinese female presented with gross hematuria, renal dysfunction, anemia, and weight loss...
September 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27507892/characterization-of-c3-in-c3-glomerulopathy
#4
Sanjeev Sethi, Julie A Vrana, Fernando C Fervenza, Jason D Theis, Amit Sethi, Paul J Kurtin, Yuzhou Zhang, Richard J H Smith
BACKGROUND: C3 glomerulopathy (C3G) is caused by overactivity of the alternative pathway of complement that results in bright glomerular C3 staining with minimal or no deposition of immunoglobulins on immunofluorescence microscopy. Laser microdissection and mass spectrometry of the two subtypes, C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), have identified C3 as the predominant glomerular complement protein, although lesser amounts of C9, C5, C6, C7 and C8 are detectable...
August 8, 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/27490940/familial-c3-glomerulonephritis-caused-by-a-novel-cfhr5-cfhr2-fusion-gene
#5
Xue Xiao, Cybele Ghossein, Agustín Tortajada, Yuzhou Zhang, Nicole Meyer, Michael Jones, Nicolo Ghiringhelli Borsa, Carla M Nester, Christie P Thomas, Santiago Rodríquez de Córdoba, Richard J H Smith
C3 glomerulopathy (C3G) is an ultra-rare complement-mediated renal disease characterized histologically by the predominance of C3 deposition within in the glomerulus. Familial cases of C3G are extremely uncommon and offer unique insight into the genetic drivers of complement dysregulation. In this report, we describe a patient who presented with C3G. Because a relative carried the same diagnosis, we sought an underlying genetic commonality to explain the phenotype. As part of a comprehension genetic screen, we completed multiplex ligation-dependent probe amplification across the complement factor H related region and identified amplification alterations consistent with a genomic rearrangement...
September 2016: Molecular Immunology
https://www.readbyqxmd.com/read/27460033/c3-mesangial-proliferative-glomerulonephritis-initially-presenting-with-atypical-hemolytic-uremic-syndrome-a-case-report
#6
Can Huzmeli, Ferhan Candan, Ayse Seker, Esin Yildiz, Hatice Terzi, Mansur Kayatas
BACKGROUND: Hemolytic uremic syndrome is characterized by acute renal failure, thrombocytopenia, and Coombs-negative hemolytic anemia. In C3 mesangial proliferative glomerulonephritis, an increase in mesangial cell proliferation without thickening in the glomerular capillary wall can be seen under light microscopy, but the definitive diagnosis is made with the immunohistologic demonstration of isolated C3 deposits in the mesangium. C3 glomerulonephritis may be detected in childhood; however, in this case report we describe the first case of isolated C3 glomerulonephritis together with atypical hemolytic uremic syndrome in an adult patient...
2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27356907/understanding-the-complement-mediated-glomerular-diseases-focus-on-membranoproliferative-glomerulonephritis-and-c3-glomerulopathies
#7
REVIEW
Sophia Lionaki, Hara Gakiopoulou, John N Boletis
An enhanced understanding of the role of complement in the pathogenesis of membranoproliferative glomerulonephritis has led to reclassification of the latter into immunoglobulin-mediated and non-immunoglobulin-mediated disease. The new classification schema resulted in improved diagnostic clinical algorithms, while it brought into light again the diseases, which are characterized by the presence of glomerular deposits, composed predominantly by C3, in the absence of significant amounts of immunoglobulins in renal biopsy, namely, C3 glomerulopathies (dense deposit disease and C3 glomerulonephritis)...
September 2016: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/27274823/revisiting-post-infectious-glomerulonephritis-in-the-emerging-era-of-c3-glomerulopathy
#8
Mazdak A Khalighi, Shihtien Wang, Kammi J Henriksen, Margret Bock, Mahima Keswani, Shane M Meehan, Anthony Chang
BACKGROUND: Post-infectious glomerulonephritis (PIGN) is an immune complex-mediated glomerular injury that typically resolves. Dominant C3 deposition is characteristic of PIGN, but with the emergence of C3 glomerulonephritis (C3GN) as a distinct entity, it is unclear how the pathologic similarities between PIGN and C3GN should be reconciled. Therefore, nephrologists and nephropathologists need additional guidance at the time of biopsy. METHODS: We studied 23 pediatric and young adult patients diagnosed with PIGN...
June 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27156109/complement-and-the-kidney-in-the-setting-of-shiga-toxin-hemolytic-uremic-syndrome-organ-transplantation-and-c3-glomerulonephritis
#9
REVIEW
Lindsay S Keir, Craig B Langman
OBJECTIVES: To review the role of complement in glomerular pathologies focusing on thrombotic microangiopathies (TMA) caused by Shiga toxin (Stx) and organ transplantation associated hemolytic uremic syndrome (HUS) as well as C3 glomerulopathy (C3G). METHODS: Examination of literature discussing TMA associated with Stx HUS, transplantation related HUS and C3G. RESULTS: There is an emerging role for complement biology in the renal glomerulus where its inappropriate over-activation is integral to several diseases...
April 2016: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/27146825/testing-the-activity-of-complement-convertases-in-serum-plasma-for-diagnosis-of-c4nef-mediated-c3-glomerulonephritis
#10
Anna M Blom, Fernando Corvillo, Michal Magda, Grzegorz Stasiłojć, Pilar Nozal, Miguel Ángel Pérez-Valdivia, Virginia Cabello-Chaves, Santiago Rodríguez de Córdoba, Margarita López-Trascasa, Marcin Okrój
Autoantibodies termed C3-nephritic factor (C3NeF), which stabilize convertases of the alternative complement pathway, often stimulate autoinflammatory diseases. However, knowledge about analogous autoantibodies acting on the classical pathway (C4NeF) is limited to a few reports, which indicate association with kidney dysfunction, systemic lupus erythematous, and infections. C4NeF may appear independently from C3NeF, but the lack of a routine diagnostic method predisposes C4NeF for being an underestimated player in autoinflammatory episodes...
July 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27056062/c3-glomerulopathy
#11
Magdalena Riedl, Paul Thorner, Christoph Licht
Recent advances in our understanding of the disease pathology of membranoproliferative glomerulonephritis has resulted in its re-classification as complement C3 glomerulopathy (C3G) and immune complex-mediated glomerulonephritis (IC-GN). The new consensus is based on its underlying pathomechanism, with a key pathogenetic role for the complement alternative pathway (AP), rather than on histomorphological characteristics. In C3G, loss of AP regulation leads to predominant glomerular C3 deposition, which distinguishes C3G from IC-GN with predominant immunoglobulin G staining...
January 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/27004158/concomitant-granulomatosis-with-polyangiitis-and-c3-glomerulonephritis-causing-renal-failure
#12
Aadel A Chaudhuri, Jason T Davis
A 75-year-old male with no prior history of vasculitis or renal deficiency presented with a history of 36 hours of anterior epistaxis, one month of worsening shortness of breath, severe anemia requiring transfusion, thrombocytopenia, coagulopathy with INR 4.9, and renal failure requiring hemodialysis. A peripheral blood smear revealed no evidence of microangiopathic hemolytic anemia. We performed an autoimmune workup, which revealed high levels of serum serine protease 3 antibody (C-ANCA), elevated rheumatoid factor, low serum C3, and normal levels of serum C4...
2016: Curēus
https://www.readbyqxmd.com/read/26986539/de-novo-c3-glomerulonephritis-in-a-renal-allograft
#13
Ji Hae Nahm, Seung Hwan Song, Yu Seun Kim, Hae-Il Cheong, Beom Jin Lim, Beom Seok Kim, Hyeon Joo Jeong
C3 glomerulonephritis (C3GN) is a recently described, rare glomerular disease characterized by predominant or sole glomerular C3 deposits. Morphologic features of C3GN are similar to those of dense deposit disease (DDD); however, ribbon-like intramembranous electron-dense deposits are absent in the former. We report a case of de novo C3GN in a renal allograft with morphologic transformation to DDD. A 6-year-old boy presented with congenital left renal agenesis and right ureteropelvic junction obstruction. The patient underwent pyeloplasty but experienced recurrent urinary tract infections...
2016: Ultrastructural Pathology
https://www.readbyqxmd.com/read/26921911/primary-glomerulonephritides
#14
REVIEW
Jürgen Floege, Kerstin Amann
Most glomerulonephritides, even the more common types, are rare diseases. They are nevertheless important since they frequently affect young people, often cannot be cured, and can lead to chronic kidney disease, including end-stage renal failure, with associated morbidity and cost. For example, in young adults, IgA nephropathy is the most common cause of end-stage renal disease. In this Seminar, we summarise existing knowledge of clinical signs, pathogenesis, prognosis, and treatment of glomerulonephritides, with a particular focus on data published between 2008 and 2015, and the most common European glomerulonephritis types, namely IgA nephropathy, membranous glomerulonephritis, minimal change disease, focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, and the rare complement-associated glomerulonephritides such as dense deposit disease and C3 glomerulonephritis...
May 14, 2016: Lancet
https://www.readbyqxmd.com/read/26885475/reanalysis-of-membranoproliferative-glomerulonephritis-patients-according-to-the-new-classification-a-multicenter-study
#15
Sung Ae Woo, Hye Young Ju, Soon Hyo Kwon, Ji-Hye Lee, Soo Jeong Choi, Dong Cheol Han, Seung Duk Hwang, Sae-Yong Hong, So-Young Jin, Hyo-Wook Gil
BACKGROUND: All types of membranoproliferative glomerulonephritis (MPGN) are progressive diseases with poor prognoses. Recently, a newly proposed classification of these diseases separated them into immune complex- and complement-mediated diseases. We investigated the frequency of C3 glomerulonephritis among previously diagnosed MPGN patients. METHODS: We conducted a retrospective study of patients diagnosed with MPGN at three tertiary care institutions between 2001 and 2010...
December 2014: Kidney Research and Clinical Practice
https://www.readbyqxmd.com/read/26846787/differences-in-clinical-findings-pathology-and-outcomes-between-c3-glomerulonephritis-and-membranoproliferative-glomerulonephritis
#16
Yukihiko Kawasaki, Syuto Kanno, Atsushi Ono, Yuichi Suzuki, Shinichiro Ohara, Masatoki Sato, Kazuhide Suyama, Koichi Hashimoto, Mitsuaki Hosoya
BACKGROUND: To clarify the clinical manifestations of pediatric complement component C3 glomerulonephritis (C3GN), we retrospectively evaluated differences in the clinicopathological findings and prognosis between C3GN and immune-complex-mediated membranoproliferative glomerulonephritis (IC-MPGN). METHODS: Thirty-seven patients diagnosed with "idiopathic MPGN" were enrolled in this retrospective study. The patients were divided into two groups, with Group 1 consisting of 19 patients diagnosed with IC-MPGN and Group 2 consisting of 18 patients diagnosed with C3GN...
July 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/26806831/diagnosis-of-complement-alternative-pathway-disorders
#17
Andrea Angioi, Fernando C Fervenza, Sanjeev Sethi, Yuzhou Zhang, Richard J Smith, David Murray, Jens Van Praet, Antonello Pani, An S De Vriese
Kidney diseases resulting from abnormal control of the complement alternative pathway include atypical hemolytic uremic syndrome, C3 glomerulonephritis, and dense-deposit disease, as well as atypical postinfectious glomerulonephritis. Although clinically diverse, they all result from loss of surface or fluid-phase complement control, caused by acquired or genetic defects in the complement alternative pathway. As such, the diagnostic approach is similar and includes a comprehensive biochemical, genetic, and pathologic analysis of the complement pathway...
February 2016: Kidney International
https://www.readbyqxmd.com/read/26704376/proliferative-c4-dense-deposit-disease-acute-thrombotic-microangiopathy-a-monoclonal-gammopathy-and-acute-kidney-failure
#18
Arshad Ali, Lynn Schlanger, Samih H Nasr, Sanjeev Sethi, Steven M Gorbatkin
Dense deposit disease (DDD) is a rare form of glomerulonephritis that has recently been reclassified under the broad group of C3 glomerulopathy, which also includes C3 glomerulonephritis. C3 glomerulopathy is characterized by predominant C3 staining on immunofluorescence microscopy and dysregulation of the alternative complement pathway. We present a case of DDD concurrent with acute thrombotic microangiopathy (TMA) in a 54-year-old white man. The patient presented with acute kidney injury, and a kidney biopsy showed segmental highly electron-dense intramembranous deposits and large rounded mesangial electron-dense deposits consistent with DDD and coexisting glomerular and vascular thrombosis consistent with concurrent acute TMA...
March 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/26680069/likely-recurrence-of-c3-glomerulonephritis-in-kidney-transplantation-an-entity-to-bear-in-mind-case-report
#19
L Perea-Ortega, M León-Fradejas, J Alonso-Titos, M Cabello-Díaz, R Toledo-Rojas, E Sola-Moyano, G Martín-Reyes, V López-Jiménez, D Burgos-Rodríguez, D Hernández-Marrero
BACKGROUND: C3 glomerulonephritis (C3GN) is an unusual entity that is caused by dysregulation and hyperactivity of the alternative complement pathway. Renal biopsy immunofluorescence study shows C3 deposits with absence of immunoglobulins and markers of the classical complement pathway. More than 50% of cases develop end-stage renal disease. Less well-known is the course of C3GN after kidney transplantation. CASE REPORT: We present the case of a 60-year-old woman with chronic kidney disease secondary to chronic glomerulonephritis of unknown origin who received a kidney transplant...
November 2015: Transplantation Proceedings
https://www.readbyqxmd.com/read/26649668/overlap-of-ultrastructural-findings-in-c3-glomerulonephritis-and-dense-deposit-disease
#20
Sanjeev Sethi, Fernando C Fervenza, Richard J H Smith, Mark Haas
No abstract text is available yet for this article.
December 2015: Kidney International
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