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C3 Glomerulonephritis

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https://www.readbyqxmd.com/read/29329521/treating-c3-glomerulopathy-with-eculizumab
#1
Thomas Welte, Frederic Arnold, Julia Kappes, Maximilian Seidl, Karsten Häffner, Carsten Bergmann, Gerd Walz, Elke Neumann-Haefelin
BACKGROUND: C3 glomerulopathy (C3G) is a rare, but severe glomerular disease with grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well as inherited dysregulation of the alternative pathway of the complement cascade. Currently, there is no established therapy. Treatment with the C5 complement inhibitor eculizumab may be a therapeutic option. However, due to rarity of the disease, parameters predicting treatment response remain largely unknown. METHODS: Seven patients with C3G (five with C3 glomerulonephritis and two with dense deposit disease) were treated with eculizumab...
January 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29319773/anca-positive-pauci-immune-crescentic-glomerulonephritis-in-a-patient-with-systemic-lupus-erythematosus
#2
Percy Herrera-Añazco, Percy Velásquez-Castillo, Josmel Pacheco-Mendoza, Germán Valenzuela-Rodriguez, Carmen Asato-Higa
The pauci-immune crescentic glomerulonephritis (PICGN) is generally associated with small-vessel vasculitis with a few reported cases associated with other autoimmune diseases such as Systemic Lupus Erythematosus (SLE). We present the case of a female 34-year-old patient with acute kidney injury symptoms with indication for renal replacement therapy in the context of clinical SLE diagnosis. A kidney biopsy was conducted and it was found that most glomeruli showed some segmental sclerosis with synechia to the Bowman's capsule...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29319772/clinical-and-histological-features-of-patients-with-membranoproliferative-glomerulonephritis-classified-by-immunofluorescence-findings
#3
Cristiane Bitencourt Dias, Leonardo Testagrossa, Lectícia Jorge, Denise Malheiros, Viktoria Woronik
BACKGROUND: New classification for membranoproliferative glomerulonephritis has been proposed in the literature. The aim of this study was to compare the clinical, biochemical, etiology and renal biopsy findings of these patients grouped by immunofluorescence as proposed by the new classification. METHODS: Patients with renal biopsy-proven membranoproliferative glomerulonephritis unrelated to systemic lupus erythematosus, diagnosed between 1999 and 2014. The patients were divided according to immunofluorescence: Immunoglobulin positive group, C3 positive only and negative immunofluorescence group...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29310824/c3-glomerulonephritis-and-dense-deposit-disease-share-a-similar-disease-course-in-a-large-united-states-cohort-of-patients-with-c3-glomerulopathy
#4
Andrew S Bomback, Dominick Santoriello, Rupali S Avasare, Renu Regunathan-Shenk, Pietro A Canetta, Wooin Ahn, Jai Radhakrishnan, Maddalena Marasa, Paul E Rosenstiel, Leal C Herlitz, Glen S Markowitz, Vivette D D'Agati, Gerald B Appel
C3 glomerulonephritis (C3GN) and dense deposit disease comprise the two classes of C3 glomerulopathy. Studies from Europe and Asia have aided our understanding of this recently defined disorder, but whether these data apply to a diverse United States patient population remains unclear. We, therefore, reviewed clinical and histopathological data, including generation of a C3 Glomerulopathy Histologic Index to score biopsy activity and chronicity, to determine predictors of progression to end-stage renal disease (ESRD) and advanced chronic kidney disease (CKD) in 111 patients (approximately 35% non-white) with C3 glomerulopathy: 87 with C3GN and 24 with dense deposit disease...
January 6, 2018: Kidney International
https://www.readbyqxmd.com/read/29259790/complement-levels-and-risk-of-organ-involvement-in-patients-with-systemic-lupus-erythematosus
#5
Ignacio Javier Gandino, Marina Scolnik, Emmanuel Bertiller, Valeria Scaglioni, Luis Jose Catoggio, Enrique Roberto Soriano
Objective: Complement plays a major role in SLE. Complement participation has been linked to disease activity and damage. Our objective was to estimate the association of complement behaviour with clinical manifestations, visceral injury and mortality in patients with SLE. Methods: Complement determinations (C3 and C4 levels) were analysed in patients with SLE (fulfilling American College of Rheumatology (ACR) or Systemic Lupus International Collaborating Clinics (SLICC)criteria) seen at a university hospital between 2000 and 2013...
2017: Lupus Science & Medicine
https://www.readbyqxmd.com/read/29249234/high-dose-melphalan-and-autologous-hematopoietic-stem-cell-transplant-in-patient%C3%A2-with-c3-glomerulonephritis-associated%C3%A2-with-monoclonal-gammopathy%C3%A2
#6
Nicola Lepori, Wisit Cheungpasitporn, Sanjeev Sethi, David Murray, Shaji Kumar, Nelson Leung, Karthik V Giridhar, Fernando C Fervenza
There is currently no standard treatment for monoclonal immunoglobulin (MIg)-associated C3 glomerulopathy, and treatment is often dictated by the extent of the monoclonal gammopathy. Although chemotherapy treatment for MIg-associated C3 glomerulopathy may stabilize renal function, the overall renal prognosis of MIg-associated C3 glomerulopathy is still poor with frequent progression to end-stage renal disease. We present a case of a 55-year-old man with IgG-κ gammopathy-associated C3 glomerulonephritis (C3GN) with bone marrow biopsy demonstrating 5 - 10 κ-restricted plasma cells...
December 18, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29230710/a-case-report-of-proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-m-kappa-deposits-without-associated-lymphoproliferative-disorder-or-detectable-paraproteinemia
#7
Yoshito Yamaguchi, Kunihiko Maeda, Katsuyuki Nagatoya, Atsushi Yamauchi
A 53-year-old man presented with proteinuria and hematuria. No significant abnormality was detected in his physical examination or laboratory tests, including evidence of paraprotein in serum and urine. Renal biopsy revealed mesangial proliferation, thickened glomerular basement membranes, and spike formation. Immunofluorescence revealed deposition of immunoglobulin (Ig) M heavy chain, kappa (κ) light chain, and complement component C3 along capillary walls in the glomeruli. Light chain staining indicated significant restriction, because only κ chain, not lambda chain, was present in glomeruli...
December 11, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/29217879/iga-dominant-infection-related-glomerulonephritis-in-india-a-single-center-experience
#8
J Dhanapriya, T Balasubramaniyan, S P Maharajan, T Dineshkumar, R Sakthirajan, N Gopalakrishnan, M Nagarajan
IgA-dominant infection-related glomerulonephritis (IRGN) is a distinct morphologic variant of IRGN, characterized by dominant or codominant glomerular deposits of IgA, mostly in elderly and patients with diabetes. More cases are being reported in recent times due to increased awareness of the disease entity and increased rate of Staphylococcus infection. It usually presents as rapidly progressive renal failure with proteinuria, and treatment guidelines for this disease entity are not well defined. We report here 12 cases of IgA-dominant IRGN seen over a period of 5 years from a single center...
November 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29208248/kidney-diseases-associated-with-alternative-complement-pathway-dysregulation-and-potential-treatment-options
#9
REVIEW
Prateek Sanghera, Mythili Ghanta, Fatih Ozay, Venkatesh K Ariyamuthu, Bekir Tanriover
Atypical hemolytic uremic syndrome and C3 glomerulopathy (dense deposit disease and C3 glomerulonephritis) are characterized as inappropriate activation of the alternative complement pathway. Genetic mutations affecting the alternative complement pathway regulating proteins (complement factor H, I, membrane cofactor protein and complement factor H-related proteins) and triggers (such as infection, surgery, pregnancy and autoimmune disease flares) result in the clinical manifestation of these diseases. A decade ago, prognosis of these disease states was quite poor, with most patients developing end-stage renal disease...
December 2017: American Journal of the Medical Sciences
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
#10
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/29166869/multimodal-imaging-of-retinal-pigment-epithelial-detachments-in-patients-with-c3-glomerulopathy-case-report-and-review-of-the-literature
#11
Valeria Kheir, Ali Dirani, Matthieu Halfon, Jean-Pierre Venetz, Georges Halabi, Yan Guex-Crosier
BACKGROUND: To describe the optical coherence tomography angiograhy (OCTA) of drusenoid pigment epithelial detachments (PEDs) in a woman affected by Complement 3 (C3) glomerulopathy, which represents a spectrum of glomerular diseases characterized on fluorescent microscopy by C3 accumulation with absent, or scanty, immunoglobulin deposits. It is due to acquired or genetically defective alternative pathway control and is generally associated with drusen-like deposits in Bruch's membrane, as well as choriocapillaris...
November 22, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/29151252/dominant-c3-glomerulopathy-new-roles-for-an-old-actor-in-renal-pathology
#12
REVIEW
Nicola Pirozzi, Antonella Stoppacciaro, Paolo Menè
Recently, a number of reports have described dominant C3 deposits in renal biopsies of patients with infection-related glomerulonephritis (GN). While acute post-infectious GN and membranoproliferative GN are commonly characterized by immune deposits containing C3 and/or C4, the absence of immunoglobulin (Ig) and/or immune complexes at light or electron microscopy is a rather unusual observation. Dominant C3 deposition is believed to result from the alternative pathway of complement activation via the C3bBb "tickover" convertase...
November 18, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/29104670/expression-of-foxp3-in-renal-tissue-of-patients-with-hbv-associated-glomerulonephritis-and-their-clinical-and-pathological-characteristics
#13
Wenge Zhao, Yanli Ma, Mingmin Wang, Changhe Shi, Jianping Sun, Kaiqiu Chu, Chuansheng Liu
Our study retrospectively investigated the expression of forkhead/winged helix transcription factor (Foxp3) in renal tissue and clinical features of patients with hepatitis B virus (HBV)-associated glomerulonephritis (HBV-GN). A total of 58 patients with HBV-GN were assigned to group A; 45 serum and renal tissue HBsAg-negative patients with nephritis were group B; 24 serum HBsAg-positive and renal tissue HBsAg-negative patients with slightly increased serum creatinine without nephritis were group C. Clinical manifestations, laboratory indices and renal biopsies were recorded...
November 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29097196/recurrent-allograft-c3-glomerulonephritis-and-unsuccessful-eculizumab-treatment
#14
Kati Kaartinen, Leena Martola, Anne Räisänen-Sokolowski, Seppo Meri
There is a great lack of efficient treatments for membranoproliferative glomerulonephritis (MPGN) and recently emerged complement therapies have been proposed to be useful. We report a patient with a complement-mediated MPGN having recurrencies in kidney allografts and an unsuccessful treatment with complement inhibitor, eculizumab (anti-C5 monoclonal antibody). Nephritic factor (C3Nef), an autoantibody against C3bBb, in the patient serum activated C3 but not C5 showing that major damage was mediated by C3 activation with clearly less involvement of C5 explaining unresponsiveness to eculizumab...
October 31, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29043148/membranoproliferative-glomerulonephritis-associated-with-rosai-dorfman-disease
#15
Keisuke Sugimoto, Satoshi Ueda, Mitsuru Okada, Tsukasa Takemura
Rosai-Dorfman disease is also known as sinus histiocytosis with massive lymphadenopathy. Extranodal Rosai-Dorfman disease has been reported in ~ 43% of cases; the most frequent extranodal sites - skin, soft tissue, bone, respiratory tract, and eye - are usually involved in association with lymphadenopathy. Lack of lymph node involvement is rare, especially when patients manifest renal disease. Here, we describe a patient who developed membranoproliferative glomerulonephritis when lymphadenopathy was absent...
2017: Clinical Nephrology. Case Studies
https://www.readbyqxmd.com/read/29030465/cluster-analysis-identifies-distinct-pathogenetic-patterns-in-c3-glomerulopathies-immune-complex-mediated-membranoproliferative-gn
#16
Paraskevas Iatropoulos, Erica Daina, Manuela Curreri, Rossella Piras, Elisabetta Valoti, Caterina Mele, Elena Bresin, Sara Gamba, Marta Alberti, Matteo Breno, Annalisa Perna, Serena Bettoni, Ettore Sabadini, Luisa Murer, Marina Vivarelli, Marina Noris, Giuseppe Remuzzi
Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement-mediated C3 glomerulopathy (C3G) and immune complex-mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN...
January 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28988671/c3-deposits-worsens-the-prognosis-in-type-iii-extracapillary-glomerulonephritis
#17
Marina Sánchez-Agesta Martínez, Cristina Rabasco Ruiz, Rafael Sánchez Sánchez, Rosa Ortega Salas, María López Andreu, Pedro Aljama García, Mario Espinosa Hernández
INTRODUCTION: Type iii extracapillary glomerulonephritis (PEGN) is a common cause of rapidly progressive glomerulonephritis and it is usually associated with circulating anti-neutrophil cytoplasmic antibodies (ANCAs). Recent evidence points to complement activation as an important factor in the pathogenesis of PEGN. The aim of the present study was to assess the value of C3 deposits in the prognosis of PEGN. METHODS: All patients diagnosed of PEGN from 1995 to 2015 (n=72) were included in this study...
October 5, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28975104/proliferative-glomerulonephritis-with-monoclonal-igg-deposits-an-unusual-cause-of-de-novo-disease-in-kidney-allograft
#18
Sabiha M Hussain, Kalathil K Sureshkumar
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a newly described and rare entity that can develop in native and very rarely in transplanted kidneys. We present a patient who developed de novo PGNMID in the kidney allograft along with a review of the literature. CASE PRESENTATION: A 38-year old female with type 1 diabetes who underwent successful simultaneous pancreas-kidney (SPK) transplantation 6 years earlier presented with rising serum creatinine, nephrotic range proteinuria and microhematuria...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28892995/concurrent-acute-glomerulonephritis-and-retropharyngeal-abscess-in-10-year-boy-a-case-report
#19
Sudha Yadav, Deepali Garg, Narain Das Vaswani, Jaya Shankar Kaushik, Seema Rohilla
Postinfectious Glomerulonephritis (PIGN) usually follows 1-2 weeks after respiratory tract infection and 4-6 weeks after skin infection. Acute Glomerulonephritis (AGN) is uncommon with simultaneous severe throat infections. We describe a 10-year-old boy who was presented with high grade fever, dysphagia and tender swelling over left side of neck. Examination also revealed enlarged multiple cervical lymph nodes on the same side of neck. Magnetic Resonance Imaging (MRI) of soft tissue of neck revealed evidence of retropharyngeal abscess...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28881045/diagnostic-accuracy-of-immunofluorescence-versus-immunoperoxidase-staining-to-distinguish-immune-complex-mediated-glomerulonephritis-and-c3-dominant-glomerulopathy
#20
Yassine Bouatou, Jesper Kers, Marie S N Chevalier-Florquin, Nike Claessen, Tri Q Nguyen, Jeffrey Damman, Henrique Proença, Joris J T H Roelofs, Sandrine Florquin
AIMS: Membranoproliferative glomerulonephritis (MPGN) has been reclassified from an electron microscopy to an immunofluorescence (IF) based semi quantitative classification with immunoperoxidase (IP) technique as a backup option when IF is not possible. However, no data are available on the interobserver variability, the correlation and the reclassification of MPGN based on these two techniques. METHODS & RESULTS: We retrospectively analyzed cases of type 1 MPGN...
September 7, 2017: Histopathology
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