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"Rapidly progressive glomerulonephritis"

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https://www.readbyqxmd.com/read/29184126/genetic-and-pharmacological-inhibition-of-microrna-92a-maintains-podocyte-cell-cycle-quiescence-and-limits-crescentic-glomerulonephritis
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
https://www.readbyqxmd.com/read/28891817/neutrophil-fc%C3%AE-riia-promotes-igg-mediated-glomerular-neutrophil-capture-via-abl-src-kinases
#11
Hiroshi Nishi, Kazuhiro Furuhashi, Xavier Cullere, Gurpanna Saggu, Mark J Miller, Yunfeng Chen, Florencia Rosetti, Samantha L Hamilton, Lihua Yang, Spencer P Pittman, Jiexi Liao, Jan M Herter, Jeffrey C Berry, Daniel J DeAngelo, Cheng Zhu, George C Tsokos, Tanya N Mayadas
The kidney glomerular capillaries are frequent sites of immune complex deposition and subsequent neutrophil accumulation in post-infectious and rapidly progressive glomerulonephritis. However, the mechanisms of neutrophil recruitment remain enigmatic, and there is no targeted therapeutic to avert this proximal event in glomerular inflammation. The uniquely human activating Fc receptor FcγRIIA promotes glomerular neutrophil accumulation and damage in anti-glomerular basement membrane-induced (anti-GBM-induced) glomerulonephritis when expressed on murine neutrophils...
October 2, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/28891413/post-infectious-glomerulonephritis
#12
Ramnath Balasubramanian, Stephen D Marks
Post-infectious glomerulonephritis (PIGN) is one of the most common causes of acute glomerulonephritis in children. Although post-streptococcal glomerulonephritis (PSGN) is still common, there is a wide spectrum of causative agents of PIGN. Non-streptococcal organisms are emerging as the main aetiological agents in high-income countries. Nephritis-associated plasmin receptor (NAPlr) and streptococcal pyrogenic exotoxin B (SPeB) are the two common antigens implicated in the pathogenesis of PSGN. Both NAPlr and SPeB activate the alternative complement pathway, resulting in low serum complement levels, and have an affinity to plasmin and glomerular proteins...
September 11, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28853702/familial-antiglomerular-basement-membrane-disease-in-zero-human-leukocyte-antigen-mismatch-siblings%C3%A2
#13
Andrea Angioi, Wisit Cheungpasitporn, Sanjeev Sethi, An S De Vriese, Nicola Lepori, Thomas R Schwab, Fernando C Fervenza
Reported cases of familial Antiglomerular basement membrane (anti-GBM) disease are extremely rare. The single gene mutations that may play a role in the development of familial anti-GBM disease are currently unidentified. While human leukocyte antigen (HLA)-DR15 is known to be associated with an increased risk of anti-GBM disease, HLA types in patients with familial anti-GBM disease have never been reported. We present a case of a 65-year-old woman with rapidly-progressive glomerulonephritis and pulmonary involvement, consistent with Goodpasture's syndrome...
November 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28796467/anti-glomerular-basement-membrane-antibody-diagnostics-in-a-large-cohort-tertiary-center-should-we-trust-serological-findings
#14
Abdulla Watad, Nicola Luigi Bragazzi, Kassem Sharif, Ora Shovman, Boris Gilburd, Howard Amital, Yehuda Shoenfeld
BACKGROUND: Anti-glomerular basement membrane (GBM) antibody disease, or Goodpasture's disease, is the clinical manifestation of the production of anti-GBM antibodies, which causes rapidly progressive glomerulonephritis with or without pulmonary hemorrhage. Anti-GBM antibody detection is mandatory for the diagnosis of Goodpasture's disease either from the serum or kidney biopsy. Renal biopsy is necessary for disease confirmation; however, in cases in which renal biopsy is not possible or is delayed, serum detection of anti-GBM antibody is the only way for diagnosis...
July 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28765179/status-epilepticus-as-the-initial-presentation-of-antibody-negative-goodpasture-s-syndrome
#15
Ingrid Pl Ting, Sanihah Abdul Halim, Azreen Adnan, Hasnan Jaafar
Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage...
August 1, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28729970/severe-cyclophosphamide-related-hyponatremia-in-a-patient-with-acute-glomerulonephritis
#16
Pasquale Esposito, Maria Valentina Domenech, Nicoletta Serpieri, Marta Calatroni, Ilaria Massa, Alessandro Avella, Edoardo La Porta, Luca Estienne, Elena Caramella, Teresa Rampino
Cyclophosphamide is frequently used to treat cancer, autoimmune and renal diseases, such as rapidly progressive glomerulonephritis. Its side effects are well-known, including bone marrow depression, infections, alopecia, sterility, bladder malignancy and hemorrhagic cystitis. Moreover, in some cases cyclophosphamide use has been related to the onset of hyponatremia, by development of a syndrome of inappropriate antidiuresis. Indeed, severe hyponatremia has been previously reported in patients treated with high-dose or moderate-dose of intravenous cyclophosphamide, while only few cases have been reported in patients treated with low dose...
July 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28679995/thrombotic-microangiopathy-due-to-malignant-hypertension-complicated-with-late-onset-bleeding-after-renal-biopsy
#17
Saki Ameda, Hiroyuki Kuroda, Michiko Yamada, Ken Sato, Shogo Miura, Hiroya Sakano, Takanori Shibata, Naoki Uemura, Tomoyuki Abe, Shigeyuki Fujii, Masahiro Maeda, Miri Fujita, Masayoshi Kobune, Junji Kato
A 47-year-old man presented at a local ophthalmological hospital with blurred vision. He had been diagnosed with hypertensive retinopathy and renal failure and was referred to our hospital for treatment. A renal biopsy was done to evaluate pathology of high proteinuria, hematuria, and rapidly progressive glomerulonephritis. Blood pressure remained high despite antihypertensive therapy; anemia and thrombocytopenia gradually progressed. Thrombotic microangiopathy (TMA) was suspected based on red blood cell fragmentation due to hemolytic anemia, thrombocytopenia, and renal failure...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28638567/nephrology-key-information-for-internists
#18
Sohail Abdul Salim, Juan A Medaura, Bharat Malhotra, Vishnu Garla, Shradha Ahuja, Nicki Lawson, Amaleswari Pamarthy, Hardik Sonani, Karthik Kovvuru, Venkataraman Palabindala
Hospitalists and primary care physicians encounter renal disease daily. Although most cases of acute kidney injury (AKI) are secondary to dehydration and resolve by giving fluids, many cases of AKI are due to not uncommon but unfamiliar causes needing nephrology evaluation. Common indications to consult a nephrologist on an emergency basis include hyperkalemia or volume overload in end stage renal disease patients (ESRD). Other causes of immediate consultation are cresenteric glomerulonephritis / rapidly progressive glomerulonephritis in which renal prognosis of the patient depends on timely intervention...
March 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/28559644/severe-microscopic-polyangiitis-with-unilateral-vocal-cord-paralysis-as-initial-manifestation
#19
Luis Felipe Flores-Suárez, Marco Antonio Alba, Gabriel Tona
CASE DESCRIPTION: A 16 year-old female who presented with initial ear, nose and throat manifestations who later progressed to severe renal disease, requiring hemodialysis after 11 months of unique laryngeal involvement. CLINICAL FINDINGS: Unilateral vocal cord paralysis without other symptoms or signs, but with positive perinuclear anti-neutrophil cytoplasmic antibodies (ANCA) and anti-myeloperoxidase autoantibodies, followed an unfavorable course months later with rapidly progressive glomerulonephritis...
March 30, 2017: Colombia Médica: CM
https://www.readbyqxmd.com/read/28509128/a-case-of-rapid-amelioration-of-hepatitis-c-virus-associated-cryoglobulinemic-membranoproliferative-glomerulonephritis-treated-by-interferon-free-directly-acting-antivirals-for-hcv-in-the-absence-of-immunosuppressant
#20
Fumiaki Obata, Taichi Murakami, Junko Miyagi, Sayo Ueda, Taizo Inagaki, Masanori Minato, Hiroyuki Ono, Kenji Nishimura, Eriko Shibata, Masanori Tamaki, Sakiya Yoshimoto, Fumi Kishi, Seiji Kishi, Motokazu Matsuura, Kojiro Nagai, Hideharu Abe, Toshio Doi
Mixed cryoglobulinemic syndrome, which is a systemic vasculitis characterized by the immune complex deposition in small- and medium-sized arteries and most often due to chronic hepatitis C virus (HCV) infection, sometimes clinically manifests as refractory glomerulonephritis or nephritic syndrome. Patients with mixed cryoglobulinemic nephropathy who have a rapidly progressive glomerulonephritis should receive immunosuppressive therapy. After disease stabilization, patients should receive concurrent therapy for the underlying HCV infection...
May 2017: CEN Case Reports
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