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glomerulopathies

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https://www.readbyqxmd.com/read/29340314/dnajb9-is-a-specific-immunohistochemical-marker-for-fibrillary-glomerulonephritis
#1
Samih H Nasr, Julie A Vrana, Surendra Dasari, Frank Bridoux, Mary E Fidler, Sihem Kaaki, Nathalie Quellard, Alexia Rinsant, Jean Michel Goujon, Sanjeev Sethi, Fernando C Fervenza, Lynn D Cornell, Samar M Said, Ellen D McPhail, Loren P Herrera Hernandez, Joseph P Grande, Marie C Hogan, John C Lieske, Nelson Leung, Paul J Kurtin, Mariam P Alexander
Introduction: Fibrillary glomerulonephritis (FGN) is a rare disease with unknown pathogenesis and a poor prognosis. Until now, the diagnosis of this disease has required demonstration of glomerular deposition of randomly oriented fibrils by electron microscopy that are Congo red negative and stain with antisera to Igs. We recently discovered a novel proteomic tissue biomarker for FGN, namely, DNAJB9. Methods: In this work, we developed DNAJB9 immunohistochemistry and tested its sensitivity and specificity for the diagnosis of FGN...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29335241/the-mfhr1-fusion-protein-is-a-novel-synthetic-multitarget-complement-inhibitor-with-therapeutic-potential
#2
Stefan Michelfelder, Friedericke Fischer, Astrid Wäldin, Kim V Hörle, Martin Pohl, Juliana Parsons, Ralf Reski, Eva L Decker, Peter F Zipfel, Christine Skerka, Karsten Häffner
The complement system is essential for host defense, but uncontrolled complement system activation leads to severe, mostly renal pathologies, such as atypical hemolytic uremic syndrome or C3 glomerulopathy. Here, we investigated a novel combinational approach to modulate complement activation by targeting C3 and the terminal pathway simultaneously. The synthetic fusion protein MFHR1 links the regulatory domains of complement factor H (FH) with the C5 convertase/C5b-9 inhibitory fragment of the FH-related protein 1...
January 15, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29329521/treating-c3-glomerulopathy-with-eculizumab
#3
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/29328453/aldosterone-is-involved-in-the-pathogenesis-of-obesity-related-glomerulopathy-through-activation-of-wnt-%C3%AE-catenin-signaling-in-podocytes
#4
Jia-Jia Zhu, Yi-Pu Chen, Min Yang, Bao-Li Liu, Jing Dong, Hong-Rui Dong, Hong-Liang Rui, Hong Cheng
Obesity-related glomerulopathy (ORG) is morphologically characterized by glomerulomegaly with or without observable focal segmental glomerulosclerosis under light microscope, with decreased podocyte density and number, and with increased foot‑process width observed under electron microscope. The severity of podocyte injury is correlated with the degree of proteinuria and renal dysfunction. However, the pathogenesis of ORG is not well understood. The aim of the present study was to explore the possible pathogenic role of aldosterone (ALDO) in ORG...
January 5, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29327821/chronic-kidney-disease-in-patients-with-chronic-hepatitis-c-virus-infection
#5
Omer Shahab, Pegah Golabi, Zobair M Younossi
Hepatitis C virus (HCV) infection affects many organs in the body, including the liver, kidneys, skin, joints and others. Although the hepatic manifestation of HCV has been widely studied, the extrahepatic manifestaions of HCV have not been fully appreciated. Studies have shown that patients with HCV have a higher risk of chronic kidney disease and end-stage renal disease, as well as poorer outcomes after kidney transplantation. Given these findings, it is important to screen HCV patients for presence of renal impairement in a timely manner...
January 10, 2018: Minerva Gastroenterologica e Dietologica
https://www.readbyqxmd.com/read/29327071/diseases-of-complement-dysregulation-an-overview
#6
REVIEW
Edwin K S Wong, David Kavanagh
Atypical hemolytic uremic syndrome (aHUS), C3 glomerulopathy (C3G), and paroxysmal nocturnal hemoglobinuria (PNH) are prototypical disorders of complement dysregulation. Although complement overactivation is common to all, cell surface alternative pathway dysregulation (aHUS), fluid phase alternative pathway dysregulation (C3G), or terminal pathway dysregulation (PNH) predominates resulting in the very different phenotypes seen in these diseases. The mechanism underlying the dysregulation also varies with predominant acquired autoimmune (C3G), somatic mutations (PNH), or inherited germline mutations (aHUS) predisposing to disease...
January 11, 2018: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29326307/mycophenolate-mofetil-in-combination-with-steroids-for-treatment-of-c3-glomerulopathy-a-case-series
#7
Rupali S Avasare, Pietro A Canetta, Andrew S Bomback, Maddalena Marasa, Yasar Caliskan, Yasemin Ozluk, Yifu Li, Ali G Gharavi, Gerald B Appel
BACKGROUND AND OBJECTIVES: C3 glomerulopathy is a form of complement-mediated GN. Immunosuppressive therapy may be beneficial in the treatment of C3 glomerulopathy. Mycophenolate mofetil is an attractive treatment option given its role in the treatment of other complement-mediated diseases and the results of the Spanish Group for the Study of Glomerular Diseases C3 Study. Here, we study the outcomes of patients with C3 glomerulopathy treated with steroids and mycophenolate mofetil. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We conducted a retrospective chart review of patients in the C3 glomerulopathy registry at Columbia University and identified patients treated with mycophenolate mofetil for at least 3 months and follow-up for at least 1 year...
January 11, 2018: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29319779/recovery-of-renal-function-after-bilateral-renal-vein-thrombo-sis-episode-as-complication-of-membranous-glomerulopa-thy-case-report
#8
Ana Larissa Pedrosa Ximenes, Elizabeth De Francesco Daher, Pedro Duarte Barreto Castillo, Francisco Eduardo Siqueira da Rocha, Camila Freire Salem de Miranda, Flavio Bezerra de Araujo
Renal vein thrombosis (RVT) is a complication often associated with nephrotic syndrome. It occurs due to a state of hypercoagulability common in the diseases that attend to this syndromic diagnosis. It should be suspected whenever there is nephrotic syndrome associated with sudden flank pain, hematuria and worsening of proteinuria. Bilateral RVT also presents with frequently oliguric renal dysfunction. This case reports a 33-year-old patient hospitalized for a nephrotic syndrome, with etiologic investigation suggestive of primary membranous glomerulopathy, which evolved with bilateral RVT associated with deterioration of renal function and need for renal replacement therapy...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29319258/familial-lecithin-cholesterol-acyl-transferase-deficiency-with-chronic-kidney-disease
#9
N Gopalakrishnan, R Arul, J Dhanapriya, T Dinesh Kumar, R Sakthirajan, T Balasubramaniyan
Familial lecithin-cholesterol acyltransferase (LCAT) deficiency is a rare autosomal recessive (AR) disease caused by mutation in the LCAT gene. LCAT enzyme esterifies cholesterol molecules in high-density lipoprotein(HDL) and low density-lipoprotein (LDL) particles. This enzyme deficiency is characterised by progressive corneal opacification, glomerulopathy, mild - moderate haemolytic anaemia and very low plasma levels of HDL. We here report a 34 year-old lady who presented with hypertension, nephrotic proteinuria, renal failure, corneal ring opacities, anemia and dyslipidemia...
October 2017: Journal of the Association of Physicians of India
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
#10
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/29300739/lymphotoxin-expression-in-human-and-murine-renal-allografts
#11
Harald Seeger, Maja T Lindenmeyer, Clemens D Cohen, Carsten Jaeckel, Peter J Nelson, Jin Chen, Ilka Edenhofer, Nicolas Kozakowski, Heinz Regele, Georg Boehmig, Simone Brandt, Rudolf P Wuethrich, Mathias Heikenwalder, Thomas Fehr, Stephan Segerer
The kidney is the most frequently transplanted solid organ. Recruitment of inflammatory cells, ranging from diffuse to nodular accumulations with defined microarchitecture, is a hallmark of acute and chronic renal allograft injury. Lymphotoxins (LTs) mediate the communication of lymphocytes and stromal cells and play a pivotal role in chronic inflammation and formation of lymphoid tissue. The aim of this study was to assess the expression of members of the LT system in acute rejection (AR) and chronic renal allograft injury such as transplant glomerulopathy (TG) and interstitial fibrosis/tubular atrophy (IFTA)...
2018: PloS One
https://www.readbyqxmd.com/read/29300203/evolving-criteria-for-the-diagnosis-of-antibody-mediated-rejection-in-renal-allografts
#12
Mark Haas
PURPOSE OF REVIEW: To review changes in the Banff schema for antibody-mediated renal allograft rejection over the past decade, including key revisions agreed upon during and immediately subsequent to the 2017 Banff Conference on Allograft Pathology. RECENT FINDINGS: The original Banff schema for diagnosis of acute and chronic active antibody-mediated rejection (ABMR) in renal allografts was formulated at the 2001 and 2007 Banff Conferences, and required histologic (primarily microvascular inflammation and transplant glomerulopathy), immunohistologic (C4d in peritubular capillaries), and serologic [circulating donor-specific antibodies (DSA)] evidence for a definitive diagnosis of ABMR...
January 2, 2018: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/29296046/the-association-of-serum-leptin-levels-with-metabolic-diseases
#13
REVIEW
Jen-Pi Tsai
Leptin is a 167-amino-acid protein released by white adipose tissue and encoded by the obese gene. It has a role as a negative regulator of appetite control through sending a satiety signal to act on receptors within the hypothalamus. At normal levels, leptin can exert its effects on weight regulation according to white fat mass, induce sodium excretion, maintain vascular tone, and repair the myocardium. Beyond these effects, elevated serum leptin levels have been implicated in the pathogenesis of metabolic syndrome, diabetes mellitus, hypertension, and multiple cardiovascular diseases...
October 2017: Ci Ji Yi Xue za Zhi, Tzu-chi Medical Journal
https://www.readbyqxmd.com/read/29279276/acquired-partial-lipodystrophy-and-c3-glomerulopathy-dysregulation-of-the-complement-system-as-a-common-pathogenic-mechanism
#14
Fernando Corvillo, Margarita López-Trascasa
The activation of the alternative pathway of the complement is involved in the development of several renal diseases, such as atypical haemolytic uremic syndrome and C3 glomerulopathy. In C3 glomerulopathy, a high percentage of patients have circulating levels of the autoantibody called C3NeF, which causes systemic dysregulation of the complement system. In some cases, the presence of this antibody has been related with abnormalities of adipose tissue, causing acquired partial lipodystrophy (Barraquer-Simons syndrome)...
December 23, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29273332/global-glomerulosclerosis-with-nephrotic-syndrome-the-clinical-importance-of-age%C3%A2-adjustment
#15
Musab S Hommos, Caihong Zeng, Zhihong Liu, Jonathan P Troost, Avi Z Rosenberg, Matthew Palmer, Walter K Kremers, Lynn D Cornell, Fernando C Fervenza, Laura Barisoni, Andrew D Rule
Globally sclerotic glomeruli (GSG) occur with both normal aging and kidney disease. However, it is unknown whether any GSG or only GSG exceeding that expected for age is clinically important. To evaluate this, we identified patients with a glomerulopathy that often presents with nephrotic syndrome (focal segmental glomerulosclerosis, membranous nephropathy, or minimal change disease) in the setting of the Nephrotic Syndrome Study Network (NEPTUNE), China-Digital Kidney Pathology (DiKiP), and the Southeast Minnesota cohorts...
December 19, 2017: Kidney International
https://www.readbyqxmd.com/read/29250696/nephrotic-syndrome-after-autologous-hematopoietic-stem-cell-transplantation-a-case-report
#16
Bogdan Obrişcă, Adriana Roxana JurubiŢă, Andreea Gabriella Andronesi, Mihaela Gherghiceanu, Gener Ismail, George Mitroi, Mihai Cristian Hârza
Nephrotic syndrome (NS) is a rare complication of hematopoietic cell transplantation (HCT) and is thought to represent a renal manifestation of chronic graft-versus-host disease (cGVHD). Glomerulopathies occur less often in recipients of autologous as compared to allogeneic HCT and, in this setting, renal pathology is less well characterized. This case report describes a 54-year-old man admitted for the evaluation of a nephrotic-range proteinuria. His past medical history included a ? light-chain secreting multiple myeloma (MM) for which he underwent autologous HCT...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29250691/nephrotic-syndrome-secondary-to-amyloidosis-in-a-patient-with-monoclonal-gammopathy-with-renal-significance-mgrs
#17
Andrei Niculae, Ileana Peride, Vlad Vinereanu, Daniela Rădulescu, Ovidiu Gabriel Bratu, Bogdan Florin Geavlete, Ionel Alexandru ChecheriŢă
Monoclonal gammopathy with renal significance (MGRS) is a relative new-described entity, diagnosed especially in older patients and deriving from the group with monoclonal gammopathy of undetermined significance (MGUS). Various renal lesions may arise in MGRS, according to the ultrastructural characteristics of the monoclonal immunoglobulin deposition in the kidney, from proliferative glomerulopathies and amyloidosis to light chain proximal tubulopathy and crystal-storing histiocytosis. Although both are considered premalign or non-malignant hematological conditions, kidney involvement in MGRS aggravates the prognosis of the patients and need to be treated aggressively...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
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
#18
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/29246788/alteration-of-connexin43-expression-in-a-rat-model-of-obesity-related-glomerulopathy
#19
Yongli Zhao, Guohua Li, Yuchuan Wang, Zhengjuan Liu
It is accepted that alteration of connexin43 (Cx43) expression in glomeruli is a common pathological response in several forms of kidney diseases. To date, however the change of the Cx43 expression in obesity-related glomerulopathy (ORG) has not been reported. In this study, the alteration of Cx43 expression in the glomeruli of rat with ORG was defined. Five-week-old rats were fed with high-fat diet for 18weeks to establish the ORG model, then the histological change of glomeruli, the foot process effacement of podocyte, the markers for podocyte injury (nephrin,podocin and WT1) and Cx43 expression in glomeruli were examined respectively...
December 12, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29229167/glomerular-diseases-in-children
#20
REVIEW
Scott E Wenderfer, Joseph P Gaut
Unique challenges exist in the diagnosis and treatment of glomerular diseases with their onset during childhood. Mounting evidence supports the notion that earlier onset cases occur due to larger numbers of genetic risk alleles. Nearly all causes of adult-onset glomerulonephritis, nephrotic syndrome, and thrombotic microangiopathy have also been described in children, although the prevalence of specific causes differs. Postinfectious glomerulonephritis, Henoch-Schönlein purpura nephritis, and minimal change disease remain the most common causes of glomerular disease in younger children in the United States and can be diagnosed clinically without need for biopsy...
November 2017: Advances in Chronic Kidney Disease
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