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C3 Glomerulonephritis

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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
https://www.readbyqxmd.com/read/28058212/complement-related-kidney-diseases-recurrence-after-transplantation
#7
REVIEW
Maurizio Salvadori, Elisabetta Bertoni
The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss after kidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome (HUS), the membranoproliferative glomerulonephritis (MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital (genetic) or acquired (auto-antibodies) modifications of the alternative pathway of complement...
December 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#8
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27941331/interleukin-17ra-promotes-humoral-responses-and-glomerular-injury-in-experimental-rapidly-progressive-glomerulonephritis
#9
Joanna R Ghali, Kim M O'Sullivan, Peter J Eggenhuizen, Stephen R Holdsworth, A Richard Kitching
BACKGROUND/AIMS: Interleukin (IL)-17A and IL-17F are proinflammatory cytokines, which signal through a receptor complex consisting of IL-17RA and IL-17RC subunits. We sought to define the role of IL-17RA expression by leukocytes and stromal cells in nephritogenic immunity and injury in experimental glomerulonephritis. METHODS: Glomerulonephritis was induced in wild-type and IL-17RA-deficient (IL-17RA-/-) mice by sheep anti-mouse glomerular basement membrane globulin...
December 10, 2016: Nephron
https://www.readbyqxmd.com/read/27878657/c3-glomerulopathy-and-current-dilemmas
#10
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
#11
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/27692553/-accidental-mercury-poisoning-in-a-12-year-old-girl
#12
F Alby-Laurent, N Honoré-Goldman, A Cavau, N Bellon, S Allali, V Abadie
INTRODUCTION: Exposure to metallic mercury can cause severe accidental intoxications in children, whose clinical symptoms can vary depending on the route of administration, the dose, as well as the time and duration of the exposure. It has become unusual in France, yet it must be considered when taking a patient's medical history in cases of multisystemic involvement without a clear explanation. CLINICAL CASE: We report the case of a 12-year-old patient hospitalized because of a cough, poor general condition, chills, night sweats, psychomotor retardation, and skin lesions that had been developing for several weeks...
November 2016: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/27691976/nephropathies-in-the-european-captive-cheetah-acinonyx-jubatus-population
#13
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/27683044/blockade-of-c5-in-severe-acute-postinfectious-glomerulonephritis-associated-with-anti-factor-h-autoantibody
#14
Hassib Chehade, Samuel Rotman, Véronique Frémeaux-Bacchi, Vincent Aubert, Salima Sadallah, Loukia Sifaki, Rémi Salomon, Manuel Pascual
Activation of the complement cascade plays an important role in the pathogenesis of postinfectious glomerulonephritis. We report successful terminal complement pathway blockade using an anti-C5 monoclonal antibody (eculizumab) in an 8-year-old child with severe acute postinfectious glomerulonephritis requiring hemodialysis. The child presented with clinical, serologic, and histopathologic criteria for diffuse crescentic postinfectious glomerulonephritis. Complement measurements showed low C3 and C4 levels, with increased SC5b-9 titers...
September 24, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27631242/c3-glomerulonephritis-in-multiple-myeloma-a-case-report-and-literature-review
#15
REVIEW
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/27595516/use-of-c4d-as-a-diagnostic-tool-to-classify-membranoproliferative-glomerulonephritis
#16
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/27558026/clinical-and-pathological-analysis-of-iga-nephropathy-with-chronic-renal-failure
#17
Yuyuan Liu, Qinfeng Hu, Ping Shen, Li Tang, Gang Yuan, Yongmei Zhou, Huaqi Chai
OBJECTIVE: To investigative clinical and pathological characteristics of IgA nephropathy with chronic renal failure. METHOD: Clinical and pathological findings from 65 cases of IgA nephropathy with chronic renal failure were reviewed. Pathological characteristics of all the cases were analyzed according to WHO definition and Oxford Classification. Evaluating the severity of pathological lesions by the Katafuchi R semiquantitative scoring system, and analyzing their relationship with clinical indexes of renal function...
October 2016: Renal Failure
https://www.readbyqxmd.com/read/27543874/an-unusual-case-of-iga-dominant-postinfectious-glomerulonephritis-a-case-report-and-review-of-the-literature
#18
M Wagrowska-Danilewicz, M Danilewicz, I Fisiak, J Piskorska
We report a case of IgA-dominant postinfectious glomerulonephritis in a 49-year-old man presenting with acute kidney injury, nephrotic range proteinuria and hematuria. He suffered from ischemic heart disease, cardiac insufficiency, mitral regurgitation, tricuspid insufficiency, septal aneurysm and hypertension. Renal biopsy revealed segmental and focal endocapillary and mesangial hypercellularity, and thickening of the glomerular capillary wall. Immunofluorescence showed co-dominant strong coarse granular immunostaining of IgA, IgG and C3 mainly along the glomerular capillary wall...
June 2016: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
https://www.readbyqxmd.com/read/27510665/comparative-evaluation-of-immunoperoxidase-versus-immunofluorescent-techniques-in-interpretation-of-kidney-biopsies
#19
Swasti Shubham, Minakshi Bhardwaj, Himansu Sekhar Mahapatra
BACKGROUND: Immunofluorescence (IF) on frozen sections has been considered to be the gold standard for evaluation of kidney biopsy specimens. Immunohistochemistry (IHC) method can also be used for this purpose with advantages of being applicable on paraffin embedded tissue, providing permanent sections, and not requiring a specialized microscope for interpretation. Our aim was to evaluate IHC as an alternative to IF in the diagnostic assessment of kidney biopsy specimens. METHODS: One hundred kidney biopsy specimens were subjected to both IF and IHC staining for immunoglobulins (Ig), IgG, IgA, IgM and complement components c3 and c1q...
July 2016: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/27507892/characterization-of-c3-in-c3-glomerulopathy
#20
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
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