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C3 nephropathy

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https://www.readbyqxmd.com/read/29312858/de-novo-glomerular-diseases-after-renal-transplantation-how-is-it-different-from-recurrent-glomerular-diseases
#1
REVIEW
Fedaey Abbas, Mohsen El Kossi, Jon Kim Jin, Ajay Sharma, Ahmed Halawa
The glomerular diseases after renal transplantation can occur de novo, i.e., with no relation to the native kidney disease, or more frequently occur as a recurrence of the original disease in the native kidney. There may not be any difference in clinical features and histological pattern between de novo glomerular disease and recurrence of original glomerular disease. However, structural alterations in transplanted kidney add to dilemma in diagnosis. These changes in architecture of histopathology can happen due to: (1) exposure to the immunosuppression specifically the calcineurin inhibitors (CNI); (2) in vascular and tubulointerstitial alterations as a result of antibody mediated or cell-mediated immunological onslaught; (3) post-transplant viral infections; (4) ischemia-reperfusion injury; and (5) hyperfiltration injury...
December 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/29243784/value-of-immunofluorescence-mediated-detection-of-ig-c1q-c3-and-fra-for-the-identification-and-diagnosis-of-atypical-membranous-nephropathy
#2
W-X Wang, C-Y Hu
OBJECTIVE: The present study was to investigate the value of immunofluorescence-mediated detection of Ig, C1q, C3, and FRA for the identification and diagnosis of atypical membranous nephropathy (AMN). PATIENTS AND METHODS: Fifty-five patients with AMN and 135 patients with idiopathic membranous nephropathy (IMN) diagnosed by renal biopsy in our hospital were consecutively selected. The positive expressions of Ig, C1q, C3, and FRA by immunofluorescence were analyzed...
December 2017: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29241200/atypical-hemolytic-uremic-syndrome-associated-with-complement-factor-h-mutation-and-iga-nephropathy-a-case-report-successfully-treated-with-eculizumab
#3
Hironori Nakamura, Mariko Anayama, Mutsuki Makino, Yasushi Makino, Katsuhiko Tamura, Masaki Nagasawa
We present a rare case of IgA nephropathy in a patient who developed atypical hemolytic uremic syndrome (aHUS) associated with a complement factor H (CFH) gene mutation, and who was successfully treated with eculizmab. A 76-year-old man was admitted as the patients had thrombotic microangiopathies findings. The patient was treated with plasma exchange, hemodialysis and methylprednisolone. A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 level was not decreased. Light microscopy findings were consistent with hemolytic uremic syndrome and immunofluorescence analysis revealed IgA and C3 were detected...
December 14, 2017: Nephron
https://www.readbyqxmd.com/read/29237409/iga-nephropathy-featuring-massive-wire-loop-like-deposits-in-two-patients-with-alcoholic-cirrhosis
#4
Daisuke Takada, Keiichi Sumida, Akinari Sekine, Ryo Hazue, Masayuki Yamanouchi, Tatsuya Suwabe, Noriko Hayami, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
BACKGROUND: Various renal manifestations are known to develop in patients with liver disease, including chronic hepatitis and cirrhosis. CASE PRESENTATION: We evaluated renal disease in two 47-year-old Japanese men with liver cirrhosis and chronic alcoholism for 34 years and 27 years, respectively. Renal biopsy demonstrated massive wire loop-like deposits in the subendothelial space of the glomerular basement membrane and in the mesangium. However, immunofluorescence was only positive for IgA and C3, and electron microscopy did not reveal any organized structures in the electron-dense deposits...
December 13, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29191199/paraffin-immunofluorescence-for-detection-of-immune-complexes-in-renal-biopsies-an-efficient-salvage-technique-for-diagnosis-of-glomerulonephritis-in-dogs
#5
Akira Yabuki, Mariko Sawa, Moeko Kohyama, Takeshi Hamamoto, Osamu Yamato
BACKGROUND: Renal biopsy is an essential tool for the diagnosis of proteinuric kidney diseases in dogs, and evaluation of immune complexes (IC) by immunofluorescence (IF) of frozen sections (IF-F) is required for the diagnosis of IC-mediated glomerulonephritis (ICGN). However, the use of frozen sections from renal biopsies can have limitations. The aim of this study was to develop a reliable IF method using formalin-fixed and paraffin-embedded (FFPE) sections to detect ICs in dog ICGN...
December 1, 2017: BMC Veterinary Research
https://www.readbyqxmd.com/read/29178575/serum-iga-c3-and-glomerular-c3-staining-predict-severity-of-iga-nephropathy
#6
Yukihiko Kawasaki, Ryo Maeda, Shinichiro Ohara, Kazuhide Suyama, Mitsuaki Hosoya
BACKGROUND: We evaluated whether the serum IgA/C3 ratio and glomerular C3 staining predict prognosis in IgA nephropathy. METHODS: We collected data for 44 IgA nephropathy children treated with multi-drug combination therapy. The children were retrospectively divided into four groups based on the serum IgA/C3 ratio and glomerular C3 staining. Group A consisted of 9 children with a higher than median IgA/C3 ratio and glomerular C3 staining ≧2.0, Group B consisted of 7 children with a higher than median IgA/C3 ratio and glomerular C3 staining <2...
November 27, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29114042/deletion-variants-of-cfhr1-and-cfhr3-associate-with-mesangial-immune-deposits-but-not-with-progression-of-iga-nephropathy
#7
Perrine Jullien, Blandine Laurent, Guillaume Claisse, Ingrid Masson, Miriana Dinic, Damien Thibaudin, Francois Berthoux, Eric Alamartine, Christophe Mariat, Nicolas Maillard
Activation of complement through the alternative pathway has a key role in the pathogenesis of IgA nephropathy (IgAN). Large, international, genome-wide association studies have shown that deletion of complement factor H-related genes 1 and 3 (CFHR3,1Δ) is associated with a reduced risk of developing IgAN, although the prognostic value of these deletions in IgAN remains unknown. Here, we compared the renal outcomes of patients with IgAN according to their CFHR3,1Δ genotype. This retrospective, monocentric cohort study included 639 white patients with biopsy-proven IgAN since 1979 (mean age at diagnosis, 40...
November 7, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29043511/implication-of-decreased-serum-complement-3-in-patients-with-diabetic-nephropathy
#8
Junlin Zhang, Yiting Wang, Rui Zhang, Hanyu Li, Qianqian Han, Ruikun Guo, Tingli Wang, Li Li, Fang Liu
AIMS: The serum complement 3 (C3) level was reduced in many patients with type 2 diabetes mellitus (T2DM) and diabetic nephropathy (DN). However, the clinical implications of such change are still less understood. This study was aimed to explore the association between C3 level and the baseline clinicopathological characteristics and the prognosis of T2DM patients with DN. METHODS: A total of 171 T2DM patients with biopsy-proven DN who received follow-up for at least 1 year were recruited...
October 17, 2017: Acta Diabetologica
https://www.readbyqxmd.com/read/28975092/self-limited-membranous-nephropathy-after-intravitreal-bevacizumab-therapy-for-age-related-macular-degeneration
#9
Gebran Khneizer, Ahmad Al-Taee, Bahar Bastani
BACKGROUND: Monoclonal antibodies targeting vascular endothelial growth factor (VEGF), such as bevacizumab, are administered intravitreally for the treatment of wet or exudative age-related macular degeneration (ARMD). Systemic use of bevacizumab has been linked to a wide range of renal adverse effects including proteinuria and hypertension. CASE PRESENTATION: We present the case of a 77-year-old Caucasian male with a past medical history of hypertension, vitamin D deficiency and paroxysmal atrial fibrillation who presented to primary care clinic with a 2-week history of bilateral lower extremity edema, 2 months after completing four monthly intravitreal injections of bevacizumab for ARMD...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28843828/vinpocetine-mitigates-proteinuria-and-podocytes-injury-in-a-rat-model-of-diabetic-nephropathy
#10
Walaa Wadie, Dalia M El-Tanbouly
Podocyte injury and glomerular basement membrane thickening have been considered as essential pathophysiological events in diabetic nephropathy. The aim of this study was to investigate the possible beneficial effects of vinpocetine on diabetes-associated renal damage. Male Wistar rats were made diabetic by injection of streptozotocin (STZ). Diabetic rats were treated with vinpocetine in a dose of 20mg/kg/day for 6 weeks. Treatment with vinpocetine resulted in a marked decrease in the levels of blood glucose, glycosylated haemoglobin, creatinine, blood urea nitrogen, urinary albumin and albumin/creatinine ratio along with an elevation in creatinine clearance rate...
November 5, 2017: European Journal of Pharmacology
https://www.readbyqxmd.com/read/28838443/first-treatment-of-relapsing-rapidly-progressive-iga-nephropathy-with-eculizumab-after-living-kidney-donation-a-case-report
#11
A L Herzog, C Wanner, K Amann, K Lopau
BACKGROUND: IgA nephropathy (IGAN) rarely can present as a crescent and progressive form leading to end-stage renal disease (ESRD) in a short period of time. Recurrence of IGAN after kidney transplantation is frequent, and complement components such as C3, C4d, and C5 seem to be involved. We present a case of a young male patient with ESRD caused by rapidly progressive IGAN and who demonstrated rapid recurrence of crescentic IGAN after kidney donation. CASE REPORT: In September 2014, a 28-year-old male patient was hospitalized due to IGAN with 60% of crescents...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28832357/recurrent-glomerular-disease-after-kidney-transplantation
#12
Christopher D Blosser, Roy D Bloom
PURPOSE OF REVIEW: With improving short-term kidney transplant outcomes, recurrent glomerular disease is being increasingly recognized as an important cause of chronic allograft failure. Further understanding of the risks and pathogenesis of recurrent glomerular disease enable informed transplant decisions, along with the development of preventive and treatment strategies. RECENT FINDINGS: Multiple observational studies have highlighted differences in rates and outcomes for various recurrent glomerular diseases, although these rates have not markedly improved over the last decade...
November 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28830653/recombinant-human-igg1-based-fc-multimers-with-limited-fcr-binding-capacity-can-effectively-inhibit-complement-mediated-disease
#13
Haoping Sun, Henrik S Olsen, Emmanuel Y Mérigeon, Edward So, Erin Burch, Susan Kinsey, John C Papadimitriou, Cinthia B Drachenberg, Søren M Bentzen, David S Block, Scott E Strome, Xiaoyu Zhang
There is a lack of effective targeted therapies for the treatment of complement dependent diseases. We developed two recombinant Fc multimers, G207 and G211, with limited ability to interact with low/moderate affinity FcγRs, but with high avidity for C1q. These drugs effectively inhibited complement dependent cytotoxicity (CDC) in vitro, and prevented the deposition of C1q, C3b and MAC, on the surface of Ab-opsonized cells. Importantly, these inhibitory effects were both C1q dependent and independent. In order to determine the biologic relevance of our findings, we evaluated the clinical efficacy of these drugs in three different animal models, acute RBC hemolysis, anti-Thy-1 nephritis and passive Heymann's nephropathy (PHN), in which disease pathophysiology relies preferentially on complement activation...
August 19, 2017: Journal of Autoimmunity
https://www.readbyqxmd.com/read/28821363/living-donor-kidney-transplantation-in-atypical-hemolytic-uremic-syndrome-a-case-series
#14
Caroline Duineveld, Jacobien C Verhave, Stefan P Berger, Nicole C A J van de Kar, Jack F M Wetzels
BACKGROUND: The development of complement inhibitors has greatly improved the outcome of patients with atypical hemolytic uremic syndrome (aHUS), making kidney transplantation a more feasible option. Although prophylactic eculizumab therapy may prevent recurrent disease after transplantation, its necessity for all transplant recipients is debated. STUDY DESIGN: A case series. SETTING & PARTICIPANTS: Patients with aHUS who underwent living donor kidney transplantation after 2011 at 2 university centers, prospectively followed up with a protocol of eculizumab therapy limited to only recipients with documented posttransplantation recurrent thrombotic microangiopathy...
December 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28793954/interstitial-complement-c3-activation-and-macrophage-infiltration-in-patients-with-hypertensive-nephropathy%C3%A2
#15
Jiong Cui, Jianxin Wan, Danyu You, Zhenhuan Zou, Yi Chen, Zhenzhou Li, Qiujian Lian
BACKGROUND: The interplay between interstitial complement C3 activation and macrophage infiltration might play an important role in the pathogenesis of hypertensive nephropathy (HN), but human data are limited. We sought to investigate interstitial complement C3 expression and macrophage infiltration in HN as well as the relationships between C3 activation and macrophage infiltration, their association with clinicopathologic data, and changes in renal function. MATERIALS AND METHODS: Using immunohistochemistry, we analyzed 20 renal tissue specimens from HN patients and 40 control specimens for complement C3, angiotensin (AGT), angiotensin II, and macrophage marker CD68 levels...
August 10, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28729648/a-haplotype-in-cfh-family-genes-confers-high-risk-of-rare-glomerular-nephropathies
#16
Yin Ding, Weiwei Zhao, Tao Zhang, Hao Qiang, Jianping Lu, Xin Su, Shuzhen Wen, Feng Xu, Mingchao Zhang, Haitao Zhang, Caihong Zeng, Zhihong Liu, Huimei Chen
Despite distinct renal lesions, a series of rare glomerular nephropathies are reportedly mediated by complement overactivation. Genetic variations in complement genes contribute to disease risk, but the relationship of genotype to phenotype has not been straightforward. Here, we screened 11 complement genes from 91 patients with atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G) and membranoproliferative glomerulonephritis type I (MPGN I), and identified the concomitant presence of three missense variations located within the human complement Factor H (CFH) gene cluster...
July 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28697742/increased-c4-and-decreased-c3-levels-are-associated-with-a-poor-prognosis-in-patients-with-immunoglobulin-a-nephropathy-a-retrospective-study
#17
Min Pan, Ji Zhang, Zhanyuan Li, Lingwei Jin, Yu Zheng, Zhihong Zhou, Su Zhen, Guoyuan Lu
BACKGROUND: An association between serum complement levels and poor renal prognosis in patients with immunoglobulin A nephropathy (IgAN) remains controversial. METHODS: We conducted a retrospective study examining the relationship between serum complement levels and prognosis in patients with IgAN. Between 2009 and 2013, patients with biopsy-confirmed IgAN were identified from the Second Affiliated Hospital of Wenzhou Medical College, China, and various parameters were documented during follow-up until 2015...
July 11, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28658762/role-of-immunofluorescence-in-adult-onset-nephrotic-syndrome-a-study-in-a-tertiary-care-centre-of-western-india
#18
Sharada Rane, Prerana Mutyal, Nicholas Dcunha, Mayur Parkhi, Meenal Jadhav
INTRODUCTION: Study of renal Immunofluorescence (IF) is an ancillary but essential technique in evaluation of renal biopsies in glomerulopathies and also it enlightens on the pathogenesis of nephrotic syndrome. AIM: To determine the role of IF in evaluating definite subtyping and diagnosis of adult onset nephrotic syndrome and attempting clinicopathological correlation. MATERIALS AND METHODS: A total of 52 patients of adult onset nephrotic syndrome were evaluated clinically and with pertinent investigations; and subjected to USG guided percutaneous renal biopsy which was processed and stained for light microscopy and for immunofluorescence by direct method (DIF) using antibodies against IgG, IgM, IgA and C3...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28637589/elevated-factor-h-related-protein-1-and-factor-h-pathogenic-variants-decrease-complement-regulation-in%C3%A2-iga-nephropathy
#19
Agustín Tortajada, Eduardo Gutiérrez, Elena Goicoechea de Jorge, Jaouad Anter, Alfons Segarra, Mario Espinosa, Miquel Blasco, Elena Roman, Helena Marco, Luis F Quintana, Josué Gutiérrez, Sheila Pinto, Margarita Lopez-Trascasa, Manuel Praga, Santiago Rodriguez de Córdoba
IgA nephropathy (IgAN), a frequent cause of chronic kidney disease worldwide, is characterized by mesangial deposition of galactose-deficient IgA1-containing immune complexes. Complement involvement in IgAN pathogenesis is suggested by the glomerular deposition of complement components and the strong protection from IgAN development conferred by the deletion of the CFHR3 and CFHR1 genes (ΔCFHR3-CFHR1). Here we searched for correlations between clinical progression and levels of factor H (FH) and FH-related protein 1 (FHR-1) using well-characterized patient cohorts consisting of 112 patients with IgAN, 46 with non-complement-related autosomal dominant polycystic kidney disease (ADPKD), and 76 control individuals...
June 18, 2017: Kidney International
https://www.readbyqxmd.com/read/28632965/frequent-col4-mutations-in-familial-microhematuria-accompanied-by-later-onset-alport-nephropathy-due-to-focal-segmental-glomerulosclerosis
#20
L Papazachariou, G Papagregoriou, D Hadjipanagi, P Demosthenous, K Voskarides, C Koutsofti, K Stylianou, P Ioannou, D Xydakis, I Tzanakis, A Papadaki, N Kallivretakis, N Nikolakakis, G Perysinaki, D P Gale, A Diamantopoulos, P Goudas, D Goumenos, A Soloukides, I Boletis, C Melexopoulou, E Georgaki, E Frysira, F Komianou, D Grekas, C Paliouras, P Alivanis, G Vergoulas, A Pierides, E Daphnis, C Deltas
Familial microscopic hematuria (FMH) is associated with a genetically heterogeneous group of conditions including the collagen-IV nephropathies, the heritable C3/CFHR5 nephropathy and the glomerulopathy with fibronectin deposits. The clinical course varies widely, ranging from isolated benign familial hematuria to end-stage renal disease (ESRD) later in life. We investigated 24 families using next generation sequencing (NGS) for 5 genes: COL4A3, COL4A4, COL4A5, CFHR5 and FN1. In 17 families (71%), we found 15 pathogenic mutations in COL4A3/A4/A5, 9 of them novel...
June 20, 2017: Clinical Genetics
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