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"Rapidly progressive glomerulonephritis"

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https://www.readbyqxmd.com/read/29657218/rapidly-progressive-glomerulonephritis-a-wild-card-manifestation-of-lupus-nephritis
#1
Opeyemi O Komolafe
A significant proportion of individuals with systemic lupus erythematosus (SLE) will have clinical or laboratory evidence of renal involvement at some point in the course of their disease. Lupus nephritis (LN) is one of the more important systemic manifestations of SLE and although progression to end-stage renal disease (ESRD) is not usually the norm, a significant minority of patients will do so, some in a rather dramatic fashion. It is in such instances that LN constitutes a true nephrologic emergency. Gratifyingly, it is rarely a silent emergency and heightened awareness amongst clinicians about its unpredictable course should lead to earlier recognition and intervention...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29619486/assessment-of-the-etiologies-and-renal-outcomes-of-rapidly-progressive-glomerulonephritis-in-pediatric-patients-at-king-abdulaziz-university-hospital-jeddah-saudi-arabia
#2
Faisal G Mosaad, Omar Mohammed Saggaf, Khaled T Aletwady, Khaled Y Mohammed Jan, Khalid Al-Qarni, Rakan S Al-Harbi, Osama Y Safdar
OBJECTIVES: To investigate the etiologies and outcomes of rapidly progressive glomerulonephritis (RPGN) in pediatric patients at King Abdulaziz University Hospital (KAUH) in Jeddah, Saudi Arabia. METHODS: A retrospective study was conducted in 19 pediatric patients who were diagnosed with RPGN between 2006 and 2016 at the Department of Pediatric Medicine at KAUH. Associations between variables were evaluated using independent t-test, one-way analysis of variance (ANOVA) and Chi-squared tests...
April 2018: Saudi Medical Journal
https://www.readbyqxmd.com/read/29606269/-iga-nephropathy-unusual-forms
#3
Khalil El Karoui
IgA nephropathy can have various initial presentation and evolutive characteristics. In this article, specific forms of IgA nephropathy are described, such as hypertensive emergency, nephrotic syndrome, rapidly progressive glomerulonephritis, monotypic IgA deposits, or IgA nephropathy associated with inflammatory diseases. Identification of these specific forms is needed to better characterize and treat these rare pathologies.
April 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29503131/the-treatment-of-anca-associated-rapidly-progressive-glomerulonephritis-and-goodpasture-syndrome-with-therapeutic-apheresis
#4
REVIEW
Suheyla Apaydin
Therapeutic plasma aphresis (plasmapheresis) is one form of treatment that is frequently used in practice of Nephrology. Plasmapheresis is the most important part of the therapies for Goodpasture's syndrome and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis which are causes of rapidly progressive glomerulonephritis. The reason why the effectiveness of plasmapheresis therapy cannot be clearly demonstrated in renal involvement in these diseases is that it does not appear to be possible to recruit an adequate number of patients and plasmapheresis is not effective in advanced disease if early treatment is not initiated...
February 20, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29478072/treatment-of-rapidly-progressive-glomerulonephritis-in-the-elderly
#5
Gerald B Appel, Wai L Lau
As the population worldwide ages, the epidemic of kidney disease will also increase. Anti-neutrophil cytoplasmic antibodies (ANCA) positive rapidly progressive positive glomerulonephritis (RPGN) is the most common etiology for biopsied patients among the very elderly. Its pathological features and clinical course are well described, though there is still debate about the mechanism of injury involved in individual patients. From very ancient times, the cornerstone of treatment historically has been high-dose cyclophosphamide and a lengthy course of high-dose corticosteroids...
January 26, 2018: Blood Purification
https://www.readbyqxmd.com/read/29478046/management-of-elderly-patients-with-rapidly-progressive-glomerulonephritis
#6
Zdenka Hruskova, Vladimir Tesar
BACKGROUND: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid deterioration of renal function and by extracapillary proliferation in >50% of glomeruli. The most common type of RPGN is "pauci-immune" glomerulonephritis caused by anti-neutrophil cytoplasmic antibodies-associated vasculitis (AAV). SUMMARY: The incidence of AAV increases with age and pauci-immune glomerulonephritis is the most common diagnosis found in renal biopsies in the elderly population...
2018: Blood Purification
https://www.readbyqxmd.com/read/29352845/rapidly-progressive-glomerulonephritis-caused-by-overlap-syndrome-of-igg4-related-tubulointerstitial-nephritis-and-myeloperoxidase-antineutrophil-cytoplasmic-antibody-associated-necrotising-glomerulonephritis
#7
Toshiyuki Watanabe, Masatoshi Kanda, Shinji Fukaya, Yayoi Ogawa, Kazumasa Akikawa
No abstract text is available yet for this article.
January 10, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29315316/the-c3ar-promotes-macrophage-infiltration-and-regulates-anca-production-but-does-not-affect-glomerular-injury-in-experimental-anti-myeloperoxidase-glomerulonephritis
#8
Jonathan Dick, Poh-Yi Gan, A Richard Kitching, Stephen R Holdsworth
The anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are autoimmune diseases associated with significant morbidity and mortality. They often affect the kidney causing rapidly progressive glomerulonephritis. While signalling by complement anaphylatoxin C5a though the C5a receptor is important in this disease, the role of the anaphylatoxin C3a signalling via the C3a receptor (C3aR) is not known. Using two different murine models of anti-myeloperoxidase (MPO) glomerulonephritis, one mediated by passive transfer of anti-MPO antibodies, the other by cell-mediated immunity, we found that the C3aR did not alter histological disease severity...
2018: PloS One
https://www.readbyqxmd.com/read/29302827/colonic-perforation-due-to-severe-cytomegalovirus-disease-in-granulomatosis-with-polyangiitis-after-immunosuppression
#9
REVIEW
Alessandra Soriano, Nazareno Smerieri, Stefano Bonilauri, Loredana De Marco, Alberto Cavazza, Carlo Salvarani
Granulomatosis with polyangiitis (GPA) is a small-vessel necrotizing granulomatous vasculitis typically involving upper airways, lungs, and kidneys, which may lead to end-organ damage and life-threatening complications. Major infections during GPA course represent a considerable concern in the management of the disease. Cytomegalovirus (CMV) infection and disease are rare but significant complications in the course of GPA being associated with high morbidity and mortality rates. Colonic perforation due to CMV colitis is exceedingly rare and has so far almost exclusively been documented in HIV, renal transplant, and systemic lupus erythematosus patients...
January 4, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29275837/plasticity-and-heterogeneity-of-th17-in-immune-mediated-kidney-diseases
#10
REVIEW
Christian F Krebs, Ulf Panzer
Anti-neutrophil cytoplasmatic antibody (ANCA)-associated glomerulonephritis, anti-glomerular basement membrane (GBM) glomerulonephritis and lupus nephritis are the most common causes of rapid progressive glomerulonephritis (RPGN) in the Western world. These aggressive forms of autoimmune kidney diseases significantly contribute to end-stage renal disease and are associated with high morbidity and mortality. Moreover, patients show significant heterogeneity with respect to clinical outcome and response to therapy...
February 2018: Journal of Autoimmunity
https://www.readbyqxmd.com/read/29184126/genetic-and-pharmacological-inhibition-of-microrna-92a-maintains-podocyte-cell-cycle-quiescence-and-limits-crescentic-glomerulonephritis
#11
Carole Henique, Guillaume Bollée, Xavier Loyer, Florian Grahammer, Neeraj Dhaun, Marine Camus, Julien Vernerey, Léa Guyonnet, François Gaillard, Hélène Lazareth, Charlotte Meyer, Imane Bensaada, Luc Legrès, Takashi Satoh, Shizuo Akira, Patrick Bruneval, Stefanie Dimmeler, Alain Tedgui, Alexandre Karras, Eric Thervet, Dominique Nochy, Tobias B Huber, Laurent Mesnard, Olivia Lenoir, Pierre-Louis Tharaux
Crescentic rapidly progressive glomerulonephritis (RPGN) represents the most aggressive form of acquired glomerular disease. While most therapeutic approaches involve potentially toxic immunosuppressive strategies, the pathophysiology remains incompletely understood. Podocytes are glomerular epithelial cells that are normally growth-arrested because of the expression of cyclin-dependent kinase (CDK) inhibitors. An exception is in RPGN where podocytes undergo a deregulation of their differentiated phenotype and proliferate...
November 28, 2017: Nature Communications
https://www.readbyqxmd.com/read/29170179/anca-positive-iga-nephropathy-without-necrotising-or-crescentic-glomerulonephritis-a-clinical-conundrum
#12
Abhilash Koratala, Xu Zeng, Amir Kazory
IgA nephropathy, the most prevalent form of primary glomerular disease, usually portends a favourable outcome. Antineutrophil cytoplasmic autoantibodies (ANCAs) have been reported in association with IgA nephropathy in a small subset of patients, mostly presenting with rapidly progressive glomerulonephritis and necrotising crescentic lesions. Herein, we describe a case of IgA nephropathy, positive serum cytoplasmic and perinuclear ANCAs with anti-myeloperoxidase antibody, and preserved renal function without any histological evidence of necrotising or crescentic glomerulonephritis...
November 23, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29142188/a-case-of-microscopic-polyangiitis-with-subarachnoid-hemorrhage-and-cardiovascular-complications
#13
Sae Aratani, Yukinao Sakai, Shuichi Tsuruoka
Microscopic polyangiitis (MPA) is a primary systemic vasculitis that predominantly affects small and medium vessels. MPA is rarely complicated with central nervous system or cardiovascular disease. We report a very rare case of MPA complicated with cerebral infarction, cardiovascular disease, and fatal subarachnoid hemorrhage in a 54-year-old man. During the first six days of hospitalization the patient was diagnosed with rapid progressive glomerulonephritis (RPGN), cerebral infarction, and unstable angina...
2017: Journal of Nippon Medical School, Nippon Ika Daigaku Zasshi
https://www.readbyqxmd.com/read/29101552/long-term-renal-outcome-in-pediatric-glomerulonephritis-associated-with-crescent-formation
#14
Pornpimol Rianthavorn, Manunya Chacranon
BACKGROUND: Information on long-term renal outcome of pediatric glomerulonephritis associated with crescent formation is limited. A single center retrospective study was conducted to assess long-term renal survival and to determine whether the 2010 classification for antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis can predict renal outcome in pediatric glomerulonephritis associated with crescent formation. METHODS: Biopsy and clinical data of children, aged ≤ 18 years with ≥ 10 glomeruli and ≥ 10% crescentic glomeruli during January 1998 to December 2015, were reviewed...
November 3, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29054981/immunoadsorption-in-anti-gbm-glomerulonephritis-case-report-in-a-child-and-literature-review
#15
REVIEW
Guillaume Dorval, Mathilde Lion, Sophie Guérin, Saoussen Krid, Louise Galmiche-Rolland, Rémi Salomon, Olivia Boyer
Antiglomerular basement membrane glomerulonephritis (anti-GBM GN) is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Anti-GBM GN is caused by autoantibodies (classically type G immunoglobulin) directed against the α3 subunit of type IV collagen. Without any appropriate treatment, the disease is generally fulminant, and patient and kidney survival is poor. The current guidelines recommend the use of plasma exchanges and immunosuppressive drugs...
November 2017: Pediatrics
https://www.readbyqxmd.com/read/29043141/antineutrophil-cytoplasmic-antibody-positive-pauci-immune-glomerulonephritis-associated-with-mantle-cell-lymphoma
#16
Kana N Miyata, Nazia A Siddiqi, Lawrence P Kiss, Nikolas B Harbord, James F Winchester
Renal involvement in non-Hodgkin lymphoma, especially mantle cell lymphoma (MCL) is rare. A 77-year-old man presented with acute kidney injury (AKI), which rapidly progressed to dialysis dependence. Kidney biopsy revealed patchy B-cell lymphocytic aggregates in the interstitium, which were positive for cyclin D1, consistent with atypical CD5-negative MCL as confirmed by the detection of translocation t(11;14) by FISH. Crescents were noted in 3 of 26 glomeruli; while PR-3 antineutrophil cytoplasmic antibody (ANCA) positivity and negative immunofluorescence suggested an additional pauci-immune (rapidly progressive) glomerulonephritis pattern of injury...
2017: Clinical Nephrology. Case Studies
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/28937063/a-mini-epidemic-of-anti-glomerular-basement-membrane-disease-clinical-and-epidemiological-study
#18
Umesh Lingaraj, Shivaprasad Sasivehalli Mallappa, Rajiv Elkal Neminah, Satishkumar Madakala Mohan, Leelavathi Venkatesh, Sreedhara Chikkanayakanahalli Gurusiddaiah, Niranjan Manibally Rachaiah
Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM) antibody disease is rare, estimated to occur in fewer than one case per million population and accounts for less than 20% of rapidly progressive glomerulonephritis. The prevalence among patients evaluated for potential glomerular disease is lower. It accounts for fewer than 3% of all kidney biopsies done with crescentic glomerulonephritis. Cases of anti-GBM disease occurring in a cluster have rarely been reported. All biopsy proven anti-GBM disease cases were collected from January 2015 to March 2015 at our Institute...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28927772/-antiglomerular-basement-disease-in-children-literature-review-and-therapeutic-options
#19
G Dorval, S Guérin, L Berteloot, S Krid, R Salomon, L Galmiche-Rolland, O Boyer
Antiglomerular basement membrane glomerulonephritis is a rare autoimmune disease characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage (Goodpasture syndrome). The disease is caused by autoantibodies (classically IgGs) directed against the α3 subunit of type IV collagen. This is a rare disease in the adult population and extremely rare in children, with a reported cumulative annual incidence at 1/10(6) people/year. Among scarce reported pediatric cases (n=31), most are girls (M/F sex ratio, 1:4), and the mean age at diagnoses is 9...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28918828/crescentic-glomerulonephritis-with-immunoglobulin-g4-related-disease
#20
Inbar Raber, Asiri Ediriwickrema, John Higgins, Neeraja Kambham, Alan C Pao
Immunoglobulin G4 (IgG4)-related disease is an uncommon autoimmune disease that affects multiple organ systems. Renal involvement typically presents as tubulointerstitial nephritis and less commonly as membranous glomerulonephritis. In this case report, we discuss a 68-year-old patient who presented with rapidly progressive glomerulonephritis. His renal biopsy revealed a membranoproliferative pattern of injury with fibrocellular crescents and extensive infiltration of the tubulointerstitium with IgG4-positive plasma cells...
September 2017: American Journal of the Medical Sciences
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