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C3 nephropathy

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https://www.readbyqxmd.com/read/27821389/staphylococcus-infection-associated-gn-spectrum-of-iga-staining-and-prevalence-of-anca-in-a-single-center-cohort
#1
Anjali A Satoskar, Sarah Suleiman, Isabelle Ayoub, Jessica Hemminger, Samir Parikh, Sergey V Brodsky, Cherri Bott, Edward Calomeni, Gyongyi M Nadasdy, Brad Rovin, Lee Hebert, Tibor Nadasdy
BACKGROUND AND OBJECTIVES: Staphylococcus infection-associated GN (SAGN) is a well recognized disease entity, particularly because of the frequent IgA-dominant glomerular immunoglobulin staining on kidney biopsy. Biopsy features can resemble two other disease entities - primary IgA nephropathy and Henoch-Schönlein purpura nephritis - posing a diagnostic pitfall. This is clinically relevant because of the crucial difference in the therapeutic approach. The diagnosis of SAGN is further complicated by the variability in the degree of glomerular IgA (and C3) staining, the extent of electron dense immune-type deposits, and positive ANCA serology in some patients...
November 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27718216/predictors-of-progression-in-iga-nephropathy-in-childhood
#2
M Mizerska-Wasiak, J Małdyk, A Turczyn, K Cichoń-Kawa, A Rybi-Szumińska, A Wasilewska, B Bieniaś, M Zajączkowska, M Miklaszewska, J Pietrzyk, U Demkow, M Roszkowska-Blaim, M Pańczyk-Tomaszewska
The aim of this retrospective study was to assess the usefulness of potential predictors of poor prognosis in IgA nephropathy in children. The study population consisted of 55 children aged 11 ± 4 years, diagnosed on the basis of the Oxford classification and MEST score of kidney biopsy findings. Proteinuria, glomerular filtration rate (GFR), and the IgA/C3 serum ratio were assessed in all patients twice: at onset and at follow-up. The patients were treated with steroids, immunosuppressive drugs, and/or angiotensin-converting enzyme inhibitors...
October 8, 2016: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/27691976/nephropathies-in-the-european-captive-cheetah-acinonyx-jubatus-population
#3
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/27631242/c3-glomerulonephritis-in-multiple-myeloma-a-case-report-and-literature-review
#4
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/27624649/longitudinal-assessment-of-monocyte-chemoattractant-protein-1-in-lupus-nephritis-as-a-biomarker-of-disease-activity
#5
Ranjan Gupta, Akhilesh Yadav, Amita Aggarwal
Urinary MCP-1 (uMCP-1) levels reflect lupus nephritis (LN) disease activity. However, long-term prospective studies evaluating it as a biomarker are lacking. SLE patients with active nephritis (AN), active disease without nephritis (ANR), and inactive disease (ID) were enrolled. AN patients were followed up every 3 months for 1 year. Urine and serum samples were collected at baseline from all and at follow-up visits in AN group. Urine samples from healthy subjects (HC), rheumatoid arthritis (RA), and diabetic nephropathy (DM) patients (20 each) served as controls...
November 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27611091/underweight-is-an-independent-risk-factor-for-renal-function-deterioration-in-patients-with-iga-nephropathy
#6
Yan Ouyang, Jingyuan Xie, Meng Yang, Xiaoyan Zhang, Hong Ren, Weiming Wang, Nan Chen
Studies on the relationship between body mass index (BMI) and renal progression in IgA Nephropathy (IgAN) were limited, especially for underweight patients with IgAN. To elucidate the clinical features and effect of underweight on renal function deterioration in this disease, we recruited IgAN patients with diagnostic age ≥18 years old and a baseline estimated glomerular filtration rate (eGFR) ≥15 ml/min/1.73m2 from our center between 1985 and 2014. Patients secondary to systemic diseases or follow-up less than 6 months were excluded...
2016: PloS One
https://www.readbyqxmd.com/read/27558026/clinical-and-pathological-analysis-of-iga-nephropathy-with-chronic-renal-failure
#7
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...
August 25, 2016: Renal Failure
https://www.readbyqxmd.com/read/27539757/frequency-of-iga-nephropathy-presenting-at-a-tertiary-care-hospital-in-pakistan
#8
Shahana Arshi, Humaira Nasir, Ghias-Ud-Din Butt, Aasiya Niazi, Nadira Mamoon, Sana Roshan Qamar, Rabia Mushtaq
OBJECTIVE: To determine the frequency of IgAnephropathy presenting at a tertiary care hospital. STUDY DESIGN: Across sectional, observational study. PLACE AND DURATION OF STUDY: Pakistan Institute of Medical Sciences, Islamabad and Shifa International Hospital, Islamabad, Pakistan, from November 2008 to November 2014. METHODOLOGY: This was a retrospective analysis of renal biopsies for the diagnosis of nephropathy during the study period...
August 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
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/27475991/complement-analysis-2016-clinical-indications-laboratory-diagnostics-and-quality-control
#10
REVIEW
Zoltán Prohászka, Bo Nilsson, Ashley Frazer-Abel, Michael Kirschfink
In recent years, complement analysis of body fluids and biopsies, going far beyond C3 and C4, has significantly enhanced our understanding of the disease process. Such expanded complement analysis allows for a more precise differential diagnosis and for critical monitoring of complement-targeted therapy. These changes are a result of the growing understanding of the involvement of complement in a diverse set of disorders. To appreciate the importance of proper complement analysis, it is important to understand the role it plays in disease...
November 2016: Immunobiology
https://www.readbyqxmd.com/read/27347034/clinical-effects-of-perazine-ferulate-tablets-combined-with-eucalyptol-limonene-pinene-enteric-soft-capsules-for-treatment-of-children-with-iga-nephropathy
#11
Zhizhen Liu, Jingmei Pan, Chunlei Sun, Juan Zhou, N A Li
The clinical effects of piperazine ferulate tablets combined with eucalyptol limonene pinene enteric soft capsules for treatment of children with IgA nephropathy were investigated. Sixty children with IgA nephropathy were included in the study and were randomly divided into the control (n=30) and observation (n=30) groups. The patients in the control group were treated with conservative or hormone therapy while patients in the observation group were treated with piperazine ferulate tablets combined with eucalyptol-limonene-pinene enteric soft capsules...
July 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27345360/a-previously-unrecognized-role-of-c3a-in-proteinuric-progressive-nephropathy
#12
Marina Morigi, Monica Locatelli, Cinzia Rota, Simona Buelli, Daniela Corna, Paola Rizzo, Mauro Abbate, Debora Conti, Luca Perico, Lorena Longaretti, Ariela Benigni, Carlamaria Zoja, Giuseppe Remuzzi
Podocyte loss is the initial event in the development of glomerulosclerosis, the structural hallmark of progressive proteinuric nephropathies. Understanding mechanisms underlying glomerular injury is the key challenge for identifying novel therapeutic targets. In mice with protein-overload induced by bovine serum albumin (BSA), we evaluated whether the alternative pathway (AP) of complement mediated podocyte depletion and podocyte-dependent parietal epithelial cell (PEC) activation causing glomerulosclerosis...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27274095/high-frequency-of-immunodeficiency-like-states-in-systemic-lupus-erythematosus-a-cross-sectional-study-in-300-consecutive-patients
#13
Sandro F Perazzio, Átila Granados, Reinaldo Salomão, Neusa P Silva, Magda Carneiro-Sampaio, Luis Eduardo C Andrade
OBJECTIVE: To determine the frequency of immunodeficiency-like states in SLE and related clinical features. METHODS: Three hundred and fifteen SLE patients and 301 controls were evaluated for C4A and C4B gene copy number, immunoglobulin isotypes, IgG subclasses, total haemolytic complement (CH50), C2, C3 and neutrophil oxidative burst. C2 and C3 genes were sequenced in cases of low C2 or C3 levels. Those presenting abnormal CH50 with normal C2 and C3 underwent C1q-C9 determination...
September 2016: Rheumatology
https://www.readbyqxmd.com/read/27194832/unmasking-of-complements-using-proteinase-k-in-formalin-fixed-paraffin-embedded-renal-biopsies
#14
R Nada, A Kumar, V G Kumar, K L Gupta, K Joshi
Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM)...
May 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27160380/effectiveness-of-sulodexide-might-be-associated-with-inhibition-of-complement-system-in-hepatitis-b-virus-associated-membranous-nephropathy-an-inspiration-from-a-pilot-trial
#15
Yang Yang, Lu Ma, Chao Wang, Deyang Kong, YaPing Wang, Changlin Mei
BACKGROUND: The activation of complement system is associated with the development of hepatitis B virus-associated membranous nephropathy (HBV-MN) and heparin could inhibit the activation of complement system. METHODS: This was a three-center trial. Seventy-nine patients with HBV-MN participated in the study. The follow-up of the study consisted of two periods: Stage 1 (S1) and Stage 2 (S2). All patients received 0.5mg entecavir plus 150-300mg/day of irbesartan but sulodexide was prescribed during S1...
July 2016: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/27153925/phospholipase-a2-receptor-related-membranous-nephropathy-and-mannan-binding-lectin-deficiency
#16
Stéphane Bally, Hanna Debiec, Denise Ponard, Frédérique Dijoud, John Rendu, Julien Fauré, Pierre Ronco, Chantal Dumestre-Perard
Most patients with idiopathic membranous nephropathy (IMN) have IgG4 autoantibodies against phospholipase A2 receptor (PLA2R). C3 and C5b-9 are found in immune deposits of IMN kidney biopsy specimens, but the pathway of complement activation in IMN remains elusive. We report the case of a patient who developed IMN with intense staining for PLA2R, IgG4, C3, C5b-9, factor B, and properdin and very weak staining for C1q, C4d, and IgG1. Measurement of mannan binding lectin (MBL) antigenic level and activity revealed MBL deficiency...
December 2016: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/27148252/the-origin-and-activities-of-iga1-containing-immune-complexes-in-iga-nephropathy
#17
REVIEW
Barbora Knoppova, Colin Reily, Nicolas Maillard, Dana V Rizk, Zina Moldoveanu, Jiri Mestecky, Milan Raska, Matthew B Renfrow, Bruce A Julian, Jan Novak
IgA nephropathy (IgAN) is the most common primary glomerulonephritis, frequently leading to end-stage renal disease, as there is no disease-specific therapy. IgAN is diagnosed from pathological assessment of a renal biopsy specimen based on predominant or codominant IgA-containing immunodeposits, usually with complement C3 co-deposits and with variable presence of IgG and/or IgM. The IgA in these renal deposits is galactose-deficient IgA1, with less than a full complement of galactose residues on the O-glycans in the hinge region of the heavy chains...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27055509/-clinico-pathological-characteristics-and-prognosis-of-iga-nephropathy-patients-with-microalbuminuria-and-deposition-of-complement-c3
#18
Z Y Guo, S G Zhou, Y Y Wang, X Li, Y Xu, X Y Du, W Zhang, Y M Wu
OBJECTIVE: To analyze the clinical and pathological data and prognosis of IgA nephropathy patients with microalbuminuria and deposition of C3, and to investigate the significance of C3 deposition in IgA nephropathy with microalbuminuria. METHODS: The clinical and pathological data of 127 IgA nephropathy patients with microalbuminuria confirmed by renal biopsy in the Jining No.1 People's Hospital from January 2009 to January 2015 and minimum 6-month follow-up was reviewed, and patients were divided into positive group (72 cases, 56...
March 8, 2016: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/26972096/-clinical-approach-to-primary-membranoproliferative-glomerulonephritis
#19
Fadi Fakhouri
Membranoproliferative glomerulonephritis represent a heterogeneous group of nephropathies. During the last five years, our understanding of membranoproliferative glomerulonephritis has greatly improved. Animal models and the study of complement genetics led to the dissection of the physiopathology of membranoproliferative glomerulonephritis, to the individualization of a new entity, C3 glomerulopathy, and to a new classification of these nephropathies. The study of large cohorts has also changed the clinical picture of membranoproliferative glomerulonephritis that has been long dominated by the severity of a single type of dense deposits disease...
April 2016: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/26971743/proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-g-deposits-complicated-by-immunoglobulin-a-nephropathy-in-the-renal-allograft
#20
Anri Sawada, Kunio Kawanishi, Shigeru Horita, Junki Koike, Kazuho Honda, Ayami Ochi, Mizuki Komoda, Yoichiro Tanaka, Kohei Unagami, Masayoshi Okumi, Tomokazu Shimizu, Hideki Ishida, Kazunari Tanabe, Yoji Nagashima, Kosaku Nitta
Immunoglobulin (Ig) A nephropathy (IgAN) is a known autoimmune disease due to abnormal glycosylation of IgA1, and occasionally, IgG co-deposition occurs. The prognosis of IgG co-deposition with IgAN is adverse, as shown in the previous studies. However, in the clinical setting, monoclonality of IgG co-deposition with IgAN has not been observed. We describe a case of proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) combined with IgAN in a renal allograft. A-21-year-old man developed end-stage renal failure with unknown aetiology and underwent living-donor kidney transplantation from his mother 2 years after being diagnosed...
July 2016: Nephrology
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