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Membranoproliferative glomerulonephritis

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https://www.readbyqxmd.com/read/29907458/taci-deletion-protects-against-progressive-murine-lupus-nephritis-induced-by-baff-overexpression
#1
Tanvi Arkatkar, Holly M Jacobs, Samuel W Du, Quan-Zhen Li, Kelly L Hudkins, Charles E Alpers, David J Rawlings, Shaun W Jackson
B cells are known to promote the pathogenesis of systemic lupus erythematosus (SLE) via the production of pathogenic anti-nuclear antibodies. However, the signals required for autoreactive B cell activation and the immune mechanisms whereby B cells impact lupus nephritis pathology remain poorly understood. The B cell survival cytokine B cell activating factor of the TNF Family (BAFF) has been implicated in the pathogenesis of SLE and lupus nephritis in both animal models and human clinical studies. Although the BAFF receptor has been predicted to be the primary BAFF family receptor responsible for BAFF-driven humoral autoimmunity, in the current study we identify a critical role for signals downstream of Transmembrane Activator and CAML Interactor (TACI) in BAFF-dependent lupus nephritis...
June 12, 2018: Kidney International
https://www.readbyqxmd.com/read/29906312/successful-treatment-of-chronic-hepatitis-c-infection-with-crushed-elbasvir-grazoprevir-administered-via-a-percutaneous-endoscopic-gastrostomy-tube
#2
J E Yap, P Jaiswal, L Ton, R Szynkarek, B M Attar, S Gandhi
WHAT IS KNOWN AND OBJECTIVE: Elbasvir/grazoprevir is an all-oral regimen approved for patients with hepatitis C virus (HCV) genotypes 1 and 4, and in renal insufficiency. However, to date, no data exist on the efficacy of this regimen when it is crushed and administered through a percutaneous endoscopic gastrostomy (PEG) tube. Here, we illustrate the case of a 63-year-old man who is the only known patient with HCV infection in the English literature to have successfully achieved a sustained viral response (SVR) when elbasvir/grazoprevir oral combination was administered through a PEG tube...
June 15, 2018: Journal of Clinical Pharmacy and Therapeutics
https://www.readbyqxmd.com/read/29901607/membranoproliferative-glomerulonephritis-like-findings-for-tafro-syndrome-associated-with-an-anterior-mediastinal-tumor-a-case-report
#3
Yoshitaka Furuto, Hirotsugu Hashimoto, Hajime Horiuti, Yuko Shibuya
RATIONALE: TAFRO syndrome is a systemic inflammatory disease proposed recently from Japan. The cause of TAFRO syndrome is unclear. Moreover, the disease characteristics and kidney pathology are yet unknown well and there are few cases. Herein, we report a patient with TAFRO syndrome and present the features of the renal histopathology. PATIENT CONCERNS: A 55-year-old woman presented to our hospital with the main complaint of subacute dyspnoea. DIAGNOSIS: Physical findings included a low-grade fever and generalised oedema...
June 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29899355/long-term-outcomes-of-kidney-transplant-recipients-with-end-stage-kidney-disease-attributed-to-presumed-advanced-glomerulonephritis-or-unknown-cause
#4
Wai H Lim, Germaine Wong, Stephen P McDonald, Aron Chakera, Grant Luxton, Nicole M Isbel, Helen L Pilmore, Tom Barbour, Peter Hughes, Steven J Chadban
People with biopsy-proven glomerulonephritis (GN) as their cause of end-stage kidney disease (ESKD) who undergo kidney transplantation incur significant risk of recurrent GN-related graft failure, but the risk in recipients with ESKD where GN was suspected but not biopsy proven (presumed/advanced GN) and when the cause of ESKD is unknown remains uncertain. Using the Australia and New Zealand Dialysis and Transplant registry, we examined the associations between primary kidney transplant recipients whose ESKD was attributed to: 1) commonly-recurring GN (i...
June 13, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29884135/c3-glomerulonephritis-associated-with-monoclonal-gammopathy-of-renal-significance-case-report
#5
Juana Alonso-Titos, Lara Perea-Ortega, Eugenia Sola, Alvaro Torres-Rueda, Myriam León, Remedios Toledo, Ana D Duarte, Teresa Vazquez, Maria Dolores Martinez-Esteban, Alicia Bailen, Pedro Ruiz-Esteban, Domingo Hernandez
BACKGROUND: Morbidity associated with monoclonal gammopathy of renal significance is high due to the severe renal lesions and the associated systemic alterations. Accordingly, early diagnosis is fundamental, as is stopping the clonal production of immunoglobulins using specific chemotherapy. CASE PRESENTATION: A 75-year-old man with chronic renal disease of unknown origin since 2010 experienced rapid worsening of renal function over a period of 6 mos. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance...
June 8, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29877281/serial-manifestation-of-acute-kidney-injury-and-nephrotic-syndrome-in-a-patient-with-tafro-syndrome
#6
Seigo Ito, Takahiro Uchida, Hiroki Itai, Aoi Yamashiro, Akira Yamagata, Hidehito Matsubara, Toshihiko Imakiire, Hideyuki Shimazaki, Hiroo Kumagai, Naoki Oshima
A 76-year-old woman suddenly developed anasarca and a fever, and an examination revealed thrombocytopenia, reticulin fibrosis, and acute kidney injury, yielding the diagnosis of TAFRO syndrome. Renal replacement therapy and steroid treatment were soon started. Her proteinuria was minor at first; however, once the kidney function improved, nephrotic syndrome occurred. A kidney biopsy showed membranoproliferative glomerulonephritis-like glomerulopathy with massive macrophage infiltration. Although kidney dysfunction is often observed in TAFRO syndrome patients, its detailed mechanism is unclear...
June 6, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29850460/proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-g-lambda-deposits-report-of-the-first-pediatric-case
#7
Jose Torrealba, Jyothsna Gattineni, Allen R Hendricks
Proliferative glomerulonephritis with monoclonal immunoglobulin G deposits (PGNMID) is a recently described, uncommon renal disorder which is considered a monoclonal gammopathy of renal significance. Although some patients will have a detectable monoclonal spike, overt hematologic malignancy is found in only a minority. Most patients with PGNMID are over the age of 50 years, and to our knowledge no cases have been reported in children or adolescents. Renal biopsy shows variable histologic patterns by light microscopy, with membranoproliferative and membranous patterns being most common...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29845733/hepatitis-e-virus-associated-cryoglobulinemia-in-solid-organ-transplant-recipients
#8
Olivier Marion, Florence Abravanel, Arnaud Del Bello, Laure Esposito, Sebastien Lhomme, Bénédicte Puissant-Lubrano, Laurent Alric, Stanislas Faguer, Jacques Izopet, Nassim Kamar
BACKGROUND AND AIMS: An association between hepatitis E virus (HEV) infection and cryoglobulinemia has been suggested. The aims of this study were to assess the prevalence of cryoglobulinemia during HEV infection in solid-organ-transplant (SOT) recipients, to describe its outcomes under ribavirin therapy, and to evaluate its effects on kidney function and histology. METHODS: Between November 2005 and June 2016, 128 cases of HEV infection were diagnosed among SOT recipients followed in our institution...
May 30, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29804087/right-sided-hydrothorax-a-peritoneal-dialysis-dilemma
#9
Haytham Allaham, Dania Hudhud, William Salzer
We present a 23-year-old female patient with a chief complaint of progressively worsening dyspnoea of 2 days duration. Her medical history was significant for end stage renal disease secondary to membranoproliferative glomerulonephritis. A peritoneal dialysis (PD) catheter was placed 8 weeks prior to admission. She did not miss any of the PD sessions prior to this admission. Vital signs were significant for hypoxemia. Physical examination was remarkable for right-sided basilar crackles with no other signs of fluid overload...
May 26, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29784613/cryoglobulinemic-glomerulonephritis-associated-with-nodal-and-renal-infiltration-by-t-cell-lymphoma-of-t-follicular-helper-phenotype-a-case-report
#10
Jennifer Li, Marille Umakanathan, Chow Heok P'ng, Winny Varikatt, Fiona Kwok, Ming-Wei Lin, Mirna Vucak-Dzumhur
We present a unique case of cryoglobulinemic glomerulonephritis associated with nodal and renal infiltration by T-cell lymphoma of T-follicular helper phenotype. The patient presented with transient neurologic symptoms, severe nephritic syndrome with nephrotic-range proteinuria, and acute kidney injury. He had elevated double-stranded DNA levels, low complement levels, detectable cryoglobulin, and detectable immunoglobulin M (IgM) paraprotein. The kidney biopsy showed cryoglobulinemic glomerulonephritis with a membranoproliferative pattern and diffuse interstitial infiltrates on light microscopy; IgM, C3 but weak IgG, C1q, and negative C4d staining on immunofluorescence; and deposits with organized substructures on electron microscopy...
May 18, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29768670/patients-affected-by-a-new-variant-of-endemic-pemphigus-foliaceus-have-autoantibodies-colocalizing-with-myzap-p0071-desmoplakins-1-2-and-arvcf-causing-renal-damage
#11
A M Abreu-Velez, M S Howard, H Yi, A A Florez-Vargas
BACKGROUND: We have previously reported that about 30% of patients affected by a new variant of endemic pemphigus foliaceus (EPF) in El Bagre, Colombia (termed El Bagre-EPF or pemphigus Abreu-Manu) have systemic compromise. In the current study, we focused on studying autoreactivity to the kidney and its pathological correlations. AIM: To investigate patients with El Bagre-EPF for renal compromise. METHODS: We performed a case-control study, enrolling 57 patients with El Bagre-EPF and 57 controls from the endemic area, matched by age, sex, race, work activity, demographics and comorbidities...
May 16, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29725653/long-term-eculizumab-therapy-in-a-child-with-refractory-immune-complex-mediated-membranoproliferative-glomerulonephritis
#12
Rahul Chanchlani, Paul Thorner, Seetha Radhakrishnan, Diane Hebert, Valerie Langlois, Steven Arora, David Barth, Daniel Cattran, Michael Kirschfink, Christoph Licht
No abstract text is available yet for this article.
March 2018: KI Reports
https://www.readbyqxmd.com/read/29724182/favorable-effect-of-bortezomib-in-dense-deposit-disease-associated-with-monoclonal-gammopathy-a-case-report
#13
Shuma Hirashio, Ayaka Satoh, Takahiro Arima, Kouichi Mandai, Tadasuke Awaya, Kumi Oshima, Shigeo Hara, Takao Masaki
BACKGROUND: Complement component 3 (C3) glomerulopathy, which includes dense deposit disease (DDD) and C3 glomerulonephritis, is caused by dysregulation of the alternative complement pathway. In most cases, C3 glomerulopathy manifests pathologically with membranoproliferative glomerulonephritis-like features. An association between C3 glomerulopathy and monoclonal gammopathy was recently reported in several cases, raising the possibility that C3 glomerulopathy is the underlying pathological process in monoclonal gammopathy of renal significance...
May 3, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29671215/a-patient-presenting-with-isolated-hematuria-and-renal-dysfunction-as-rare-manifestation-of-cryoglobulinemic-glomerulonephritis-in-the-course-of-autoimmune-diseases-including-sj%C3%A3-gren-s-syndrome
#14
Masaki Yamanaka, Yoshihide Fujigaki, Hajime Kono, Michito Nagura, Shigeyuki Arai, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
Autoimmune diseases are sometimes associated with immune-mediated renal diseases and cryoglobulinemia is one of the causes. Cryoglobulinemia and cryoglobulinemic glomerulonephritis associated with primary Sjögren's syndrome are most frequent condition among non-hepatitis C virus-related condition. Its typical renal manifestation shows high amount of proteinuria with microscopic hematuria and renal insufficiency. We describe a case of 72-year-old woman with Hashimoto disease, autoimmune hepatitis, Sjögren's syndrome, and immune-related pancytopenia complicated by cryoglobulinemic glomerulonephritis...
April 18, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29616329/proliferative-glomerulonephritis-with-monoclonal-igg-deposits-in-children-and-young-adults
#15
Guolan Xing, Robert Gillespie, Badreldin Bedri, Albert Quan, Pingchuan Zhang, Xin J Zhou
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age. METHODS: The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved...
April 3, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29582966/-extra-hepatic-manifestations-associated-with-hepatitis-and-virus-infection
#16
Luca Piscitani, Maria Olimpia Longo, Xhensila Grabocka, Simona Silvestri, Marta Arazzi, Alessandro Pezzutto, Gabriella Micioni, Giorgia Di Fulvio, Lorenzo Ottavio Di Pietro, Alessandra Schiazza, Roberto Di Vito, Mario Bonomini
Hepatitis E virus (HEV) is a significant public health problem that affects almost 20 million individuals annually and cause acute liver injury in 3,5 million. Hepatitis E virus can cause acute, fulminant and chronic hepatitis and has been associated with a range of extrahepatic manifestation. The spectrum of these manifestation is still emerging. Acute pancreatitis and neurological, renal, hematologic, and muscoloskeletal manifestations have been described. Renal injury include membranoproliferative glomerulonephritis with or without cryoglobulinemia, membranous glomerulonephritis and tubular necrosis...
March 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29566171/genetic-analysis-of-the-complement-pathway-in-c3-glomerulopathy
#17
Weiwei Zhao, Yin Ding, Jianping Lu, Tao Zhang, Dacheng Chen, Haitao Zhang, Caihong Zeng, Zhihong Liu, Huimei Chen
Background: C3 glomerulopathy often presents with a membranoproliferative glomerulonephritis (MPGN) pattern, and is principally caused by unrestricted activation of the complement alternative pathway. Genetic abnormalities of the complement system critically implicate in the pathogenesis of C3 glomerulopathy, but a systemic profile remains open, especially in Asia. Methods: In this study, we completed a comprehensive screen of 11 candidate alternative pathway genes by using targeted genomic enrichment and massively parallel sequencing on 43 patients with sporadic C3 glomerulopathy, which were classified as dense deposit disease (DDD; n = 10) and C3 glomerulonephritis (C3GN; n = 33) cases...
March 16, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29545130/-renal-involvement-of-cryoglobulinemia
#18
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/29515303/dense-deposit-disease-involving-c3-and-c4d-deposits
#19
M Vankalakunti, R Augustine, R Jangamani, V Siddini, R Bonu, K Babu, S H Ballal
Dense deposit disease (DDD), earlier called Type II membranoproliferative glomerulonephritis is distinct disease having frequent relapses reaching end-stage kidney disease by 10-year in up to 50%-60% of cases and high recurrence rate in the allograft. The term DDD is derived from its distinctive ribbon-like osmiophilic deposits in the lamina densa of glomerular basement membrane by electron microscopy. Pathogenetically, alternate pathway dysfunction leads to this disease, which is diagnosed by ultrastructure...
January 2018: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29510686/fundus-changes-in-type-iii-membranoproliferative-glomerulonephritis-a-case-report
#20
Masato Takei, Akira Obana, Takenori Inomata, Takao Tanaka, Tina Shiang, Yuan Bae, Tamiko Takemura, Akira Murakami
BACKGROUND: Membranoproliferative glomerulonephritis (MPGN) is characterized by mesangial cell proliferation and is classified into types I, II and III based on structural changes in the glomerular capillary walls. The drusen-like deposits of MPGN type II have been studied, but the fundus changes in MPGN type III have yet to be clarified. We report a case of MPGN type III with multiple deposits in the retinal pigment epithelium (RPE). CASE PRESENTATION: A 40-year-old Japanese woman with MPGN type III developed numerous yellow-white patches in the central macula of both eyes...
March 6, 2018: BMC Ophthalmology
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