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https://www.readbyqxmd.com/read/29729982/c3-glomerulopathy-associated-with-monoclonal-ig-is-a-distinct-subtype
#1
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
Monoclonal immunoglobulins (MIg) may play a causal role in C3 glomerulopathy (C3G) by impairing regulation of the alternative pathway of complement. Ninety-five patients with C3G were tested for MIg of which 36 were positive. Their mean age at diagnosis was 60 years and among patient 50 years and older, 65.1% had a MIg. At presentation, median serum creatinine and proteinuria were 1.9 mg/dL and 3.0 g/24 hours. Hematuria was present in 32 (88.9%) patients. Twelve (34.3%) patients had low C3 levels. C3 nephritic factor was detected in 45...
May 2, 2018: Kidney International
https://www.readbyqxmd.com/read/29717936/different-effects-of-global-osteopontin-and-macrophage-osteopontin-in-glomerular-injury
#2
Jessica Trostel, Luan D Truong, Carlos A Roncal-Jimenez, Makoto Miyazaki, Shinobu Miyazaki-Anzai, Masanari Kuwabara, Rachel H McMahan, Ana Andres-Hernando, Yuka Sato, Thomas Jensen, Miguel A Lanaspa, Richard J Johnson, Gabriela E Garcia
Osteopontin (OPN) is a pro-and ant-inflammatory molecule that simultaneously attenuates oxidative stress. Both inflammation and oxidative stress play a role in the pathogenesis of glomerulonephritis and in the progression of kidney injury. Importantly, OPN is highly induced in nephritic kidney. To further characterize the role of OPN in kidney injury we used OPN-/- mice in anti-glomerular basement membrane reactive serum-induced immune nephritis (NTS), an inflammatory and progressive model of kidney disease...
May 2, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29671873/t-cell-derived-ifn-%C3%AE-downregulates-protective-group-2-innate-lymphoid-cells-in-murine-lupus-erythematosus
#3
Mathis Düster, Martina Becker, Ann-Christin Gnirck, Malte Wunderlich, Ulf Panzer, Jan-Eric Turner
Innate lymphoid cells (ILCs) are important regulators of the immune response and play a crucial role in the restoration of tissue homeostasis after injury. GATA-3+ IL-13- and IL-5-producing group 2 innate lymphoid cells (ILC2s) have been shown to promote tissue repair in barrier organs, but despite extensive research on ILCs in the recent years, their potential role in autoimmune diseases is still incompletely understood. In the present study, we investigate the role of ILC2s in the MRL/MpJ-Faslpr (MRL-lpr) mouse model for severe organ manifestation of systemic lupus erythematosus (SLE)...
April 19, 2018: European Journal of Immunology
https://www.readbyqxmd.com/read/29670616/overactivity-of-alternative-pathway-convertases-in-patients-with-complement-mediated-renal-diseases
#4
Marloes A H M Michels, Nicole C A J van de Kar, Marcin Okrój, Anna M Blom, Sanne A W van Kraaij, Elena B Volokhina, Lambertus P W J van den Heuvel
Overactivation of the alternative pathway of the complement system is associated with the renal diseases atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). C3 nephritic factors (C3NeF) play an important role in C3G pathogenesis by stabilizing the key enzymatic complex of complement, the C3 convertase. However, the reliability of assays detecting these autoantibodies is limited. Therefore, in this study, we validated and optimized a prototype hemolytic method for robust detection and characterization of factors causing convertase overactivity in large patient cohorts...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29661452/staphylococcus-infection-associated-glomerulonephritis-in-a-kidney-transplant-patient-case-report
#5
D Cascais de Sá, L Rodrigues, L Santos, C Romãozinho, F Macário, C Marinho, J Pratas, R Alves, A Figueiredo
BACKGROUND: Staphylococcus infection-associated glomerulonephritis is a rare cause of graft dysfunction in kidney transplant. Suspicion should be high in the setting of elevation of serum creatinine, active urinary sediment, with or without hypocomplementemia, and simultaneous Staphylococcus aureus infection. A kidney biopsy is usually diagnostic. CASE REPORT: A 56-year-old man, who received a kidney transplant in 1998, with basal serum creatinine of 1.2 mg/dL and normal urinary sediment, was admitted to our kidney transplantation unit with graft dysfunction and a urinary tract infection caused by S aureus with septicemia, treated with antibiotics, in the context of recently intensified immunosuppression for a primary immune thrombocytopenia diagnosed 3 weeks earlier...
April 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29624903/lupus-nephritis-low-urinary-dnase-i-levels-reflect-loss-of-renal-dnase-i-and-may-be-utilized-as-a-biomarker-of-disease-progression
#6
Hege L Pedersen, Kjersti D Horvei, Dhivya Thiyagarajan, Gudrun E Norby, Natalya Seredkina, Gabriella Moroni, Gro Ø Eilertsen, Hallvard Holdaas, Erik H Strøm, Gunnstein Bakland, Pier-Luigi Meroni, Ole P Rekvig
Renal DNase I is lost in advanced stages of lupus nephritis. Here, we determined if loss of renal DNase I reflects a concurrent loss of urinary DNase I, and whether absence of urinary DNase I predicts disease progression. Mouse and human DNase I protein and DNase I endonuclease activity levels were determined by western blot, gel, and radial activity assays at different stages of the murine and human forms of the disease. Cellular localization of DNase I was analyzed by immunohistochemistry, immunofluorescence, confocal microscopy, and immunoelectron microscopy...
February 21, 2018: Journal of Pathology. Clinical Research
https://www.readbyqxmd.com/read/29545130/-renal-involvement-of-cryoglobulinemia
#7
Alexandre Karras
Cryoglobulins are immunoglobulins that undergo reversible precipitation at low temperatures. They can induce systemic vasculitis, characterized by purpuric cutaneous lesions, arthritis, peripheral neuropathy, hypocomplementemia and glomerular disease. Renal pathology reveals membranoproliferative glomerulonephritis, with particularly intense mesangial cell proliferation and infiltration by macrophages, associated with intracapillary thrombi. This renal disease presents as a nephritic syndrome, with heavy proteinuria, haematuria severe hypertension and rapidly progressive kidney failure that can lead to end-stage renal disease...
April 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29515299/mycophenolate-versus-cyclophosphamide-for-lupus-nephritis
#8
M Sahay, Y Saivani, K Ismal, P S Vali
Systemic lupus erythematosus is common in our country, and renal involvement is an important cause of chronic kidney disease. This study was aimed at comparing the three regimens, i.e., cyclophosphamide-based regimes (low dose and high dose) and mycophenolate mofetil (MMF)-based regime and determining if cyclophosphamide (CPM)-based regime can be an effective, safe, and cheap alternative to MMF-based regime in a resource-limited setting. Out of 144 patients, females constituted 89%. Nephrotic nephritic presentation was the most common...
January 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#9
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29415117/attenuated-macrophage-infiltration-in-glomeruli-of-aged-mice-resulting-in-ameliorated-kidney-injury-in-nephrotoxic-serum-nephritis
#10
Yoshikatsu Kaneko, Takamasa Cho, Yuya Sato, Kei Goto, Suguru Yamamoto, Shin Goto, Michael P Madaio, Ichiei Narita
Senescent cells have deleterious effects on the tissue microenvironment through proinflammatory senescence-associated secretory phenotypes; meanwhile, the onset of glomerulonephritis is predominant in younger adults. To clarify the influence of aging on the onset and development of glomerulonephritis, we used a murine model of antibody-mediated nephritis. Sheep nephrotoxic serum was administered in C57BL/6J mice at 12 weeks (adult) or 18 months old (aged) after pre-immunization with sheep IgG. Depositions of sheep IgG and autologous mouse IgG along the glomerular basement membrane and the serum titer of anti-sheep IgG-specific mouse IgG were similar between adult and aged mice...
February 3, 2018: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/29395335/the-atypical-chemokine-receptor-2-limits-renal-inflammation-and-fibrosis-in-murine-progressive-immune-complex-glomerulonephritis
#11
Andrei Bideak, Alexander Blaut, John M Hoppe, Martin B Müller, Giuseppina Federico, Nuru Eltrich, Hermann-Josef Gröne, Massimo Locati, Volker Vielhauer
The atypical chemokine receptor 2 (ACKR2), also named D6, regulates local levels of inflammatory chemokines by internalization and degradation. To explore potential anti-inflammatory functions of ACKR2 in glomerulonephritis, we induced autologous nephrotoxic nephritis in C57/BL6 wild-type and Ackr2-deficient mice. Renal ACKR2 expression increased and localized to interstitial lymphatic endothelium during nephritis. At two weeks Ackr2-/- mice developed increased albuminuria and urea levels compared to wild-type mice...
April 2018: Kidney International
https://www.readbyqxmd.com/read/29385997/proteinuric-kidney-disease-in-children-at-queen-elizabeth-central-hospital-malawi
#12
Zondiwe Victor Mwanza, Mignon McCulloch, Mark Drayson, Timothy Plant, David V Milford, Gavin Dreyer
BACKGROUND: There is a paucity of data on paediatric kidney disease in developing countries such as Malawi. Descriptive research on kidney disease is essential to improving patient outcomes. METHODS: We conducted a cross-sectional study at a tertiary hospital in Malawi from 2012 to 2013. Children under 14 years with proteinuric kidney disease were enrolled from paediatric wards and outpatient clinics at Queen Elizabeth Central Hospital (QECH). Demographic, clinical and laboratory data were collected from patients at enrolment and at 3 months review at which point clinical status and disease outcome were ascertained...
January 31, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29359288/-renal-manifestation-in-anca-associated-vasculitis
#13
Marion Haubitz
In patients with ANCA-associated vasculitis renal involvement is frequently seen and the severity of renal manifestation is very important for therapeutic strategies and prognosis. Clinically rapid loss of renal function, nephritic sediment and proteinuria in a non-nephrotic range are characterizing a focal segmental necrotizing pauci-immune glomerulonephritis with extrarenal proliferations. Induction treatment depends on the severity of manifestations. With a normal renal function methotrexate can be used in combination with steroids...
January 2018: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29357434/neuraminidase-activity-mediates-il-6-production-by-activated-lupus-prone-mesangial-cells
#14
Kamala Sundararaj, Jessalyn I Rodgers, Subathra Marimuthu, Leah J Siskind, Evelyn Bruner, Tamara K Nowling
The development of nephritis is a leading cause of morbidity and mortality in lupus patients. Although the general pathophysiological progression of lupus nephritis is known, the molecular mediators and mechanisms are incompletely understood. Previously, we demonstrated that the glycosphingolipid (GSL) catabolic pathway is elevated in the kidneys of MRL/lpr lupus mice and human lupus patients with nephritis. Specifically, the activity of neuraminidase (NEU) and expression of Neu1, an enzyme in the GSL catabolic pathway is significantly increased...
April 1, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29325951/shunt-nephritis-an-increasingly-unfamiliar-diagnosis
#15
Tessa A Harland, Ken R Winston, Anna J Jovanovich, Richard J Johnson
BACKGROUND: Shunt nephritis is a rare, reversible immune-complex mediated complication of cerebrospinal fluid (CSF) shunt infection that can progress to end-stage renal disease and even death if diagnosis is delayed. CASE DESCRIPTION: The present case report details the manifestation and clinical course of shunt nephritis in a 50-year-old patient who presented with symptoms of nephrotic syndrome 30 years after ventriculojugular shunt placement. Diagnosis was delayed due to initial negative CSF and blood cultures, but a later CSF culture was positive for Propionibacterium acnes...
March 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29325287/-the-diagnostic-value-and-limits-of-diagnostic-parameters-for-wilson-s-disease
#16
X Yang
Wilson disease (WD) is a rare and treatable genetic disorder. This paper describes the new advances and author's long-term experiences in the diagnosis of WD. The characteristics in clinical and routine tests are: the age of presentation can be quite broad, the WD could not be excluded based on age only; the patients usually have mild digestive symptoms but obvious chronic liver disease signs; liver function tests may reveal normal or a mild elevation in bilirubin, ALT and AST, but quite abnormal in serum albumin and prothrombin time in most patients; Coombs-negative hemolytic anemia, normal or markedly subnormal serum alkaline phosphatase (typically < 40 IU/L) are useful for the diagnosis of fulminant WD...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29320993/distribution-of-glomerular-diseases-in-taiwan-preliminary-report-of-national-renal-biopsy-registry-publication-on-behalf-of-taiwan-society-of-nephrology
#17
Hsien-Fu Chiu, Hung-Chun Chen, Kuo-Cheng Lu, Kuo-Hsiung Shu
BACKGROUND: Despite the development of biomarkers and noninvasive imaging tools, biopsy remains the only method for correctly diagnosing patients with unexplained hematuria, proteinuria and renal failure. Renal biopsy has been performed for several decades in Taiwan; however, a national data registry is still lacking until 2013. METHODS: The Renal Biopsy Registry Committee was established within the Taiwan Society of Nephrology in January 2013. A biopsy registry format, including basic demographic data, baseline clinical features, laboratory data, and clinical and pathological diagnosis was developed...
January 10, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29301131/safety-of-outpatient-kidney-biopsies
#18
Bojana Šimunov, Mihaela Gunjača, Branislav Čingel, Dinko Škegro, Mladen Knotek
BACKGROUND: Kidney biopsy is frequently performed in our centre as an outpatient procedure. The aim of this study was to evaluate the safety of biopsy in the outpatient setting. METHODS: We analysed kidney biopsies performed from March 2013 to February 2017. Seven hundred twenty-five biopsies performed in the outpatient setting were identified: There were 592 transplant and 133 native biopsies including 3 solitary kidney biopsies. All were performed under ultrasound guidance using a 16G or 18G needle, with freehand technique...
2018: Nephron
https://www.readbyqxmd.com/read/29274588/%C3%AE-asarone-reduce-proteinuria-by-restoring-antioxidant-enzymes-activities-and-regulating-necrosis-factor-%C3%AE%C2%BAb-signaling-pathway-in-doxorubicin-induced-nephrotic-syndrome
#19
Brijesh Sutariya, Madhusudan Saraf
α-asarone is natural bioactive compound that has been reported to have many benefits and medicinal properties. The present study aimed to assess the protective effect of α-asarone against doxorubicin (DOX) induced nephrotic syndrome in rats. An experimental nephrotic syndrome was induced by single intravenous injection of DOX (7 mg/kg) in rats. Animals were orally administered α-asarone (10 and 20 mg kg-1  d-1 ) for 4 weeks. Blood, urine and kidney tissues were collected for analyses at the end of the study...
February 2018: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29199592/nephrotoxicity-in-rats-exposed-to-paracetamol-the-protective-role-of-moralbosteroid-a-steroidal-glycoside
#20
Gaurav Gupta, Dinesh Kumar Chellappan, Irene Satiko Kikuchi, Terezinha de Jesus Andreoli Pinto, Kavita Pabreja, Mohit Agrawal, Yogendra Singh, Juhi Tiwari, Kamal Dua
Paracetamol (PCM) has an acceptable safety profile when used at prescribed doses. However, it is now understood that paracetamol can damage the kidneys when administered as an overdose. In addition, oxidative stress can play a major role in causing nephrotoxicity. This investigation studies the efficacy of moralbosteroid isolated from M. alba stem bark. Nephrotoxicity was induced with administration of paracetamol. Nephroprotection was studied using two doses of the extract. The experimental animals were divided into four groups (n = 6)...
2017: Journal of Environmental Pathology, Toxicology and Oncology
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