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C3 glomerulopathy

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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/27784126/monitoring-of-complement-activation-biomarkers-and-eculizumab-in-complement-mediated-renal-disorders
#2
Cyrill Wehling, Oliver Amon, Martin Bommer, Bernd Hoppe, Karim Kentouche, Gesa Schalk, Rolf Weimer, Michael Wiesener, Bernd Hohenstein, Burkhard Tönshoff, Rainer Büscher, Henry Fehrenbach, Ömer-Necmi Gök, Michael Kirschfink
Various complement-mediated renal disorders are currently treated with the complement inhibitor eculizumab. By blocking the cleavage of C5 this monoclonal antibody prevents cell damage caused by complement-mediated inflammation. We included 23 patients with atypical hemolytic uremic syndrome (aHUS, n=12), C3 glomerulopathies (C3G, n=9) and acute antibody-mediated renal graft rejection (AMR, n=2), treated with eculizumab in 12 hospitals in Germany. We explored the course of complement activation biomarkers and the benefit of therapeutic drug monitoring of eculizumab...
October 26, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27760612/-pathology-and-differential-diagnosis-of-immunotactoid-glomerulopathy
#3
X L Sun, F C Zhang, Y Xiao, L Liu, Y Guan
Objective: To study the morphologic changes of immunotactoid glomerulopathy and to investigate the clinical pathological features and differential diagnosis. Methods: Renal biopsy was observed under the light microscope, immunofluorescence and electron microscopy in a case of newly diagnosed immunotactoid glomerulopathy. Results: This patient clinically presented with nephrotic syndrome and hypertension, without family history of renal diseases. Light microscopy showed that diffusely massive and specific protein deposition in the glomerulus in Masson staining...
October 8, 2016: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/27717414/overview-of-laboratory-testing-and-clinical-presentations-of-complement-deficiencies-and-dysregulation
#4
A Frazer-Abel, L Sepiashvili, M M Mbughuni, M A V Willrich
Historically, complement disorders have been attributed to immunodeficiency associated with severe or frequent infection. More recently, however, complement has been recognized for its role in inflammation, autoimmune disorders, and vision loss. This paradigm shift requires a fundamental change in how complement testing is performed and interpreted. Here, we provide an overview of the complement pathways and summarize recent literature related to hereditary and acquired angioedema, infectious diseases, autoimmunity, and age-related macular degeneration...
2016: Advances in Clinical Chemistry
https://www.readbyqxmd.com/read/27691976/nephropathies-in-the-european-captive-cheetah-acinonyx-jubatus-population
#5
Angelika Url, Verena Krutak, Anna Kübber-Heiss, Sonja Chvala-Mannsberger, Nadia Robert, Nora Dinhopl, Peter Schmidt, Chris Walzer
According to previous studies in captive cheetah ( Acinonyx jubatus ) populations, one of the most threatening diseases besides amyloidosis, myelopathy, veno occlusive disease, and gastritis, is renal failure. Contrary to captive cheetahs in North America and South Africa, morphological data concerning renal lesions in the cheetah European Endangered Species Program (EEP) are lacking. This study details the histological characterization as well as immunohistochemical and morphometrical analysis of nephropathies in 35 captive cheetahs from the EEP, which were necropsied between 1985 and 2003...
September 2016: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
https://www.readbyqxmd.com/read/27661791/podocytic-infolding-glomerulopathy-a-case-report
#6
Kye Won Kwon, Hyeon Joo Jeong, Jang Han Lee
Podocytic infolding glomerulopathy (PIG) is a rare glomerular abnormality involving glomerular basement membrane (GBM) bubbling viewable by light microscopy, extensive invagination of the podocytic cytoplasm, and the presence of microstructures viewable by electron microscopy. PIG was proposed as a new disease entity in 2008. However, cases have been reported exclusively in Japan and no case reports outside Japan have been published. Here, we report a case of PIG in a 44-year-old Korean female. The patient showed mild proteinuria without renal functional impairment or other systemic diseases...
November 2016: Ultrastructural Pathology
https://www.readbyqxmd.com/read/27617140/the-genetics-of-ultra-rare-renal-disease
#7
REVIEW
Melissa Muff-Luett, Carla M Nester
The complement-mediated renal diseases are a group of ultra-rare renal diseases that disproportionately affect children and young adults and frequently lead to irreversible renal failure. Genetic mutations in alternate pathway of complement genes are pathomechanistically involved in a significant number of these unique diseases. Here, we review our current understanding of the role of genetics in the primary complement-mediated renal diseases affecting children, with a focus on atypical hemolytic uremic syndrome and C3 glomerulopathy...
March 2016: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/27595516/use-of-c4d-as-a-diagnostic-tool-to-classify-membranoproliferative-glomerulonephritis
#8
Nirupama Gupta, Dara N Wakefield, William L Clapp, Eduardo H Garin
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN type I, II and III) was reclassified in 2013 as MPGN and C3 glomerulopathy (C3G) based on the complement system activation mechanism. OBJECTIVES: To evaluate whether C4d, a component of the classical pathway, could be a diagnostic tool in differentiating between MPGN and C3G. METHODS: We conducted a retrospective study of 15 MPGN type I, II and III and 13 minimal change disease (MCD) patients diagnosed between 2000 and 2012...
August 29, 2016: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/27526707/paraprotein-related-kidney-disease-kidney-injury-from-paraproteins-what-determines-the-site-of-injury
#9
Mona Doshi, Amit Lahoti, Farhad R Danesh, Vecihi Batuman, Paul W Sanders
Disorders of plasma and B cells leading to paraproteinemias are associated with a variety of renal diseases. Understanding the mechanisms of injury and associated nephropathies provides a framework that aids clinicians in prompt diagnosis and appropriate adjunctive treatment of these disorders. Glomerular diseases that may be associated with paraproteinemias include amyloid deposition, monoclonal Ig deposition disease, proliferative GN with monoclonal Ig deposits, C3 glomerulopathy caused by alterations in the complement pathway, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemia...
August 15, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27526706/paraprotein-related-kidney-disease-glomerular-diseases-associated-with-paraproteinemias
#10
Shveta S Motwani, Leal Herlitz, Divya Monga, Kenar D Jhaveri, Albert Q Lam
Paraproteins are monoclonal Igs that accumulate in blood as a result of abnormal excess production. These circulating proteins cause a diversity of kidney disorders that are increasingly being comanaged by nephrologists. In this review, we discuss paraprotein-related diseases that affect the glomerulus. We provide a broad overview of diseases characterized by nonorganized deposits, such as monoclonal Ig deposition disease (MIDD), proliferative GN with monoclonal Ig deposits (PGNMID), and C3 glomerulopathy, as well as those characterized by organized deposits, such as amyloidosis, immunotactoid glomerulopathy, fibrillary GN, and cryoglobulinemic GN, and rarer disorders, such as monoclonal crystalline glomerulopathies, paraprotein-related thrombotic microangiopathies, and membranous-like glomerulopathy with masked IgGκ deposits...
August 15, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27507892/characterization-of-c3-in-c3-glomerulopathy
#11
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
#12
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
#13
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/27452363/anti-complement-factor-h-associated-glomerulopathies
#14
REVIEW
Marie-Agnes Dragon Durey, Aditi Sinha, Shambhuprasad Kotresh Togarsimalemath, Arvind Bagga
Atypical haemolytic uraemic syndrome (aHUS), an important cause of acute kidney injury, is characterized by dysregulation of the complement pathway, frequent need for dialysis, and progression to end-stage renal disease. Autoantibodies against complement factor H (FH), the main plasma regulatory protein of the alternative pathway of the complement system, account for a considerable proportion of children with aHUS. The autoantibodies are usually associated with the occurrence of a homozygous deletion in the genes encoding the FH-related proteins FHR1 and FHR3...
September 2016: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/27402056/complement-inhibition-in-c3-glomerulopathy
#15
REVIEW
Carla M Nester, Richard J H Smith
C3 glomerulopathy (C3G) describes a spectrum of glomerular diseases defined by shared renal biopsy pathology: a predominance of C3 deposition on immunofluorescence with electron microscopy permitting disease sub-classification. Complement dysregulation underlies the observed pathology, a causal relationship that is supported by well described studies of genetic and acquired drivers of disease. In this article, we provide an overview of the features of C3G, including a discussion of disease definition and a review of the causal role of complement...
June 2016: Seminars in Immunology
https://www.readbyqxmd.com/read/27371974/modeling-complement-driven-diseases-in-transgenic-mice-values-and-limitations
#16
REVIEW
Yoshiyasu Ueda, Damodar Gullipalli, Wen-Chao Song
Remarkable advances have been made over past decades in understanding the pathogenesis of complement-mediated diseases. This has led to development of new therapies for, and in some cases re-classification of, complement-driven diseases. This success is due to not only insight from human patients but also studies using transgenic animal models. Animal models that mimic human diseases are useful tools to understand the mechanism of disease and develop new therapies but there are also limitations due to species differences in their complement systems...
October 2016: Immunobiology
https://www.readbyqxmd.com/read/27356907/understanding-the-complement-mediated-glomerular-diseases-focus-on-membranoproliferative-glomerulonephritis-and-c3-glomerulopathies
#17
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/27340286/all-things-complement
#18
Joshua M Thurman, Carla M Nester
The complement (C) cascade is an ancient system of proteins whose primary role is to initiate and modulate immune responses. During C activation, circulating proteins are cleaved and nascent cleavage fragments participate in a broad range of downstream innate and adaptive immune functions. Although the majority of these functions are either homeostatic or protective, a large body of experimental and clinical evidence also highlights a central role for the C system in the pathogenesis of many types of glomerular disease...
June 23, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27274824/kidney-transplant-outcomes-in-familial-c3-glomerulopathy
#19
Limy Wong, Sarah Moran, Peter J Lavin, Anthony M Dorman, Peter J Conlon
C3 glomerulopathy, a newly designated entity, is characterized by glomerular disease associated with dysregulation of the alternative complement pathway and is a rare cause of end-stage kidney disease. Overall disease characteristics that include clinical presentation, laboratory assessment, histopathology and genetic background have only been unravelled in recent years and have led to the development of anti-complement therapies targeting different levels of the alternative pathway. We describe the long-term outcomes following kidney transplantation in an Irish family with familial C3 glomerulopathy due to a hybrid CFHR3-1 gene...
June 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27274823/revisiting-post-infectious-glomerulonephritis-in-the-emerging-era-of-c3-glomerulopathy
#20
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
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