keyword
https://read.qxmd.com/read/38160799/ultrastructural-examination-of-glomerular-fibrillary-deposits-in-diabetic-nephropathy
#1
JOURNAL ARTICLE
Sophie I Nagelkerken, Peter H Neeskens, Joris I Rotmans, Volker Nickeleit, Jan A Bruijn, Ingeborg M Bajema
Glomerular fibrillary deposits have occasionally been reported in diabetic nephropathy, but no large-scale, ultrastructural evaluation of these deposits have been reported so far. Here, we report our study of glomerular non-Congophilic, DNAJB9 negative fibrillary deposits in diabetic nephropathy as characterized by transmission electron microscopy. Clinical data from 55 patients with biopsy-confirmed diabetic nephropathy and 18 healthy living donors were reviewed, and their biopsies evaluated by light microscopy, immunofluorescence, and electron microscopy...
December 29, 2023: Laboratory Investigation; a Journal of Technical Methods and Pathology
https://read.qxmd.com/read/37899889/dnajb9-fibrillary-glomerulonephritis-with-membranous-like-pattern-a-case-based-literature-review
#2
Nikolaos Sabanis, Paraskevi Liaveri, Virginia Geladari, George Liapis, George Moustakas
Fibrillary glomerulonephritis (FGN) is a rare immune-mediated glomerular disease traditionally characterized by the presence of amyloid-like, randomly aligned, fibrillary deposits in the capillary wall, measuring approximately 20 nm in diameter and composed of polyclonal IgG. FGN is usually a primary disease with no pathognomonic clinical or laboratory findings. More than that, on light microscopic evaluation, it can receive various histological patterns, rendering its diagnosis indistinguishable. However, the identification by immunohistochemistry of a novel biomarker, DNA-J heat-shock protein family member B9 (DNAJB9), has created a new era in FGN diagnosis even in the absence of electron microscopy...
October 2023: Curēus
https://read.qxmd.com/read/37749770/from-patterns-to-proteins-mass-spectrometry-comes-of-age-in-glomerular-disease
#3
JOURNAL ARTICLE
Sanjeev Sethi, Jason D Theis, Lilian Mp Palma, Benjamin Madden
Laser capture microdissection and mass spectrometry (LCM/MS) is technique that involves dissection of glomeruli from paraffin embedded biopsy tissue, followed by digestion of the dissected glomerular proteins by trypsin, and subsequently mass spectrometry to identify and semi quantitate the glomerular proteins. LCM/MS has played a crucial role in the identification of novel types of amyloidosis, biomarker discovery in fibrillary glomerulonephritis, and more recently discovery of novel target antigens in membranous nephropathy...
September 26, 2023: Journal of the American Society of Nephrology: JASN
https://read.qxmd.com/read/37448907/a-case-of-posttransplant-fibrillary-glomerulonephritis
#4
Shiva Kumar Ammayappan, Arul Rajagopalan, Manorajan Rajendran, Jegan Arunachalam, Arun Prasath, Rakesh Durai, Anila A Kurien
Fibrillary glomerulonephritis (FGN) is a rare form of glomerulonephritis, usually occurring in concurrence with other conditions such as hepatitis C, dysproteinemia, autoimmune conditions, diabetes mellitus, and malignancy. The diagnosis is made by the presence of randomly oriented fibrillar deposits with a mean diameter of 20 nm, which stain positive for IgG and C3 and are negative for congo red and thioflavin T stains. Staining for DNAJB9 (DnaJ homolog subfamily B member 9) is a recently discovered mode of diagnosis of FGN without electron microscopy...
2023: Indian Journal of Nephrology
https://read.qxmd.com/read/37113492/crescentic-fibrillary-glomerulonephritis-in-the-setting-of-immune-checkpoint-inhibitor-therapy-a-report-of-two-cases
#5
Lanny T DiFranza, Eleas Chafouleas, Swapna Katipally, M Barry Stokes, Satoru Kudose, Miroslav Sekulic
INTRODUCTION: Immune checkpoint inhibitor (ICPI) therapy is used to treat various malignancies; however, it can be associated with off-target effects including kidney injury. Acute tubulointerstitial nephritis is the most commonly described renal pathology associated with ICPIs, although less frequently, glomerulopathies may be identified when a kidney biopsy is performed in the work-up of acute kidney injury (AKI). CASE PRESENTATION: Two patients with small cell carcinoma of the lung were treated with etoposide, carboplatin, and the ICPI atezolizumab...
2023: Glomerular diseases
https://read.qxmd.com/read/37069992/proteomic-analysis-of-complement-proteins-in-glomerular-diseases
#6
JOURNAL ARTICLE
Sanjeev Sethi, Lilian Monteiro P Palma, Jason D Theis, Fernando C Fervenza
INTRODUCTION: Complement plays an important role in the pathogenesis of glomerulonephritis (GN). Even though the underlying etiology of GN might be different, complement activation with subsequent glomerular deposition of complement proteins result in glomerular injury and progression of the lesions. Routine immunofluorescence microscopy (IF) includes staining for only complement factors C3c and C1q. Therefore, with regard to evaluation of the complement pathways, routine kidney biopsy provides only limited information...
April 2023: KI Reports
https://read.qxmd.com/read/36942185/fibrillary-glomerulonephritis-in-a-patient-with-vulvar-squamous-cell-carcinoma
#7
Ashwin Jagadish, Venkata Vedantam, Neethu Vedantam, Hezborn M Magacha
Fibrillary glomerulonephritis is a rare condition characterized by glomerular accumulation of non-branching fibrils, leading to hematuria, proteinuria, and hypertension. It is often associated with malignancy but has no known cause. A 66-year-old Caucasian female with a history of vulvar squamous cell carcinoma presented with one month of painless hematuria in the setting of new-onset progressive renal dysfunction and nephrotic range proteinuria. Comprehensive evaluation, including renal biopsy with staining for DnaJ heat shock protein family member B9 (DNAJB9), provided a definitive diagnosis of fibrillary glomerulonephritis...
February 2023: Curēus
https://read.qxmd.com/read/36803980/-latest-updates-on-immunotactoid-glomerulopathy-and-fibrillary-glomerulonephritis
#8
REVIEW
Marie-Camille Lafargue, Camille Cohen
Various hematologic malignancies can lead to renal complications. The most common of these hemopathies to affect the kidney is multiple myeloma, however an increasing number of kidney diseases are associated with other monoclonal gammopathies. It is recognized that clones in small abundance can be responsible for severe organ damage, thus the concept of monoclonal gammopathy of renal significance (MGRS) has emerged. Although the hemopathy in these patients is more consistent with monoclonal gammopathy of undetermined significance (MGUS) than with multiple myeloma, the diagnosis of a renal complication changes the therapeutic management...
February 15, 2023: Bulletin du Cancer
https://read.qxmd.com/read/36751534/immunoglobulin-negative-fibrillary-glomerulonephritis-masked-in-diabetic-nephropathy-a-case-report-and-discussion-of-a-diagnostic-pitfall
#9
Gabriel B W Lerner, Gary G Singer, Christopher P Larsen, Tiffany N Caza
INTRODUCTION: Fibrillary glomerulonephritis (FGN) is a rare glomerular disease with poor prognosis, characterized by deposition of randomly arranged fibrillar material measuring 10-30 nm in diameter. This diagnosis is confirmed with DNAJB9 immunohistochemistry as well as ultrastructural examination. Ultrastructurally, the fibrillary material seen in this entity may be confused with diabetic fibrillosis occurring in diabetic nephropathy. CASE PRESENTATION: We present a case of a 63-year-old African American male with remote hepatitis C virus (HCV) infection and type II diabetes mellitus who presented with chronic kidney disease and nephrotic range proteinuria...
April 2022: Glomerular diseases
https://read.qxmd.com/read/36706240/clinicopathologic-spectrum-of-renal-lesions-following-anti-tnf-%C3%AE-inhibitor-therapy-a-single-center-experience
#10
JOURNAL ARTICLE
Joichi Usui, Steven P Salvatore, Kunihiro Yamagata, Surya V Seshan
KEY POINTS: This renal biopsy study documents clinical renal dysfunction and pathologic lesions encountered in patients after anti-TNF α therapy and compares them with similar patients without treatment. This study highlights the spectrum of autoimmune, serologic, and other kidney adverse effects of anti-TNF α therapy. Unrelated active or chronic renal lesions including amyloidosis secondary to the underlying systemic inflammatory states may be observed. BACKGROUND: Anti-TNF α inhibitors, as biological agents, are used in autoimmune inflammatory states, rheumatoid arthritis (RA), psoriatic arthritis (PA), and Crohn disease...
March 1, 2023: Kidney360
https://read.qxmd.com/read/36644363/subclass-changes-in-fibrillary-glomerulonephritis
#11
Laura E Biederman, Dalia Ibrahim, Anjali A Satoskar, Tibor Nadasdy, Sergey V Brodsky
No abstract text is available yet for this article.
January 2023: KI Reports
https://read.qxmd.com/read/36576710/iga-dominant-glomerulonephritis-with-dnajb9-negative-fibrillar-polytypic-immunoglobulin-deposits-in-the-subepithelium
#12
JOURNAL ARTICLE
Reiko Muto, Kayaho Maeda, Sosuke Fukui, Shoji Saito, Noritoshi Kato, Tomoki Kosugi, Akira Shimizu, Shoichi Maruyama
Fibrillary glomerulonephritis (FGN), a rare disease is pathologically characterized by glomerular fibril accumulation ranging from 12 to 24 nm in diameter with negative Congo red staining. Recently, the identification of DnaJ homolog subfamily B member 9 (DNAJB9) as a highly sensitive and specific marker for FGN has revolutionized diagnosis of this disease. However, few recent studies have reported DNAJB9-negative glomerulonephritis with fibrillar deposits. As such, it remains unclear whether DNAJB9-negative cases can be considered equivalent to FGN...
December 28, 2022: CEN Case Reports
https://read.qxmd.com/read/36305476/fibrillary-and-immunotactoid-glomerulopathies-in-the-hunter-region-a-retrospective-cohort-study
#13
JOURNAL ARTICLE
Adrienne W S Cohen, Eswari Vilayur
BACKGROUND: Fibrillary (FGN) and immunotactoid (IT) glomerulonephritis are uncommon. AIMS: To evaluate the prevalence, clinicopathological correlations and outcomes of FGN and IT in our regional centre in Australia. METHODS: We interrogated a renal biopsy database for cases of FGN and IT from 2000 to 2020. Data included demographics, serum creatinine, haematuria status, proteinuria, comorbidities and histopathological findings. RESULTS: We had 14 cases of FGN and t of IT...
October 28, 2022: Internal Medicine Journal
https://read.qxmd.com/read/36158379/a-case-of-fibrillary-glomerulonephritis
#14
Maamannan Venkataraj, Phani P Morisetti
Fibrillary glomerulonephritis (FGN) is a very rare manifestation of glomerulonephritis characterized by the presence of deposits of randomly oriented microfibrils (10-30 nm size) in the glomeruli and visible on electron microscopy. Our patient is a 63-year-old African American male who presented with a past medical history of cirrhosis; he was initially suspected to have hepatorenal syndrome, but on kidney biopsy, and was diagnosed with FGN. Possible multiple myeloma was suspected due to its strong association with FGN and an elevated serum kappa-lambda ratio in the patient...
August 2022: Curēus
https://read.qxmd.com/read/36140202/dnajb9-is-a-reliable-immunohistochemical-marker-of-fibrillary-glomerulonephritis-evaluation-of-diagnostic-efficacy-in-a-large-series-of-kidney-biopsies
#15
JOURNAL ARTICLE
Alessandro Gambella, Chiara Pitino, Antonella Barreca, Alberto Nocifora, Manuela Maria Giarin, Luca Bertero, Luigi Biancone, Dario Roccatello, Mauro Papotti, Paola Cassoni
Fibrillary glomerulonephritis (FGN) is a rare glomerular disease characterized by a challenging diagnostic workup requiring ultrastructural identification of 20 nm-thick randomly oriented fibrillar deposits. However, the recent introduction of DNAJB9 as a putative diagnostic marker of FGN could thoroughly improve this diagnostic scenario. This study aims to assess the DNAJB9 immunohistochemical expression in a large series of FGN cases and to eventually confirm its role as a diagnostic marker of FGN. We evaluated the immunohistochemical expression of DNAJB9 (Rabbit Polyclonal, ThermoFisher) in a series of 77 FGN and 128 non-FGN cases diagnosed between January 1992 and June 2022 at the Pathology Unit of the AOU Città della Salute e della Scienza Hospital...
August 27, 2022: Biomedicines
https://read.qxmd.com/read/35946300/fibrillary-glomerulonephritis-with-atypical-immunoglobulin-m-deposits-and-hypocomplementemia-revealed-human-immunodeficiency-virus-infection
#16
Aglaia Chalkia, Zoe Alexakou, Dimitrios Kourniotis, Margarita Mpora, Harikleia Gakiopoulou, Dimitrios Petras
Fibrillary glomerulonephritis (FGN) is a rare form of glomerulonephritis, and the incidence in native renal biopsies is less than 1%. The diagnosis of FGN is defined by the ultrastructural finding of organized, randomly oriented, nonbranching fibrils with a diameter of 10-30 nm. FGN is immune-mediated glomerulonephritis with predominant immunoglobulin (Ig) G deposits. Hypocomplementemia is very rare. We report the case of a 68-year-old Caucasian man with renal impairment, hematuria, subnephrotic proteinuria, hypocomplementemia (low C4, normal C3), and hypergammaglobulinemia...
2021: Saudi Journal of Kidney Diseases and Transplantation
https://read.qxmd.com/read/35900077/acute-kidney-injury-after-pfizer-covid-19-vaccine-due-to-crescentic-fibrillary-glomerulonephritis
#17
JOURNAL ARTICLE
Khaled Al-Sawalmeh, Michael Pandes, Jose Antonio Niño, Carmen Avila-Casado
Fibrillary glomerulonephritis (FGN) is a rare glomerular disease manifesting with proteinuria, renal impairment, hematuria, hypertension, and in a very small proportion can be associated with rapidly progressive glomerulonephritis and, rarely, crescent formation. The main modality for diagnosis is kidney biopsy, which ultrastructurally demonstrates randomly arranged non-branching mesangial and glomerular basement membrane (GBM) fibrils and positive staining for the biomarker DNAJB9. The pathogenesis is largely unknown...
October 2022: Clinical Nephrology
https://read.qxmd.com/read/35881843/fibrillary-glomerulonephritis-a-rare-entity-with-unique-ultrastructural-characteristics
#18
JOURNAL ARTICLE
Vlatko Karanfilovski, Galina Severova, Pavlina Dzekova-Vidimliski
No abstract text is available yet for this article.
July 25, 2022: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://read.qxmd.com/read/35865414/fibrillary-glomerulonephritis-a-great-mimicker-of-rapidly-progressive-glomerulonephritis
#19
Manisha Raikar, Asad Shafiq
Fibrillary glomerulonephritis (FGN) is a rare but severe kidney disease found to have non-amyloid fibrillary deposits in the mesangium and/or glomerular capillary wall. It was initially thought to be idiopathic, but recent studies show an association with autoimmune disease, malignancy, and hepatitis C infection. We report a case of a non-diabetic patient presenting with long-standing microscopic hematuria, progressive proteinuria, hypertension, and worsening kidney function. The kidney biopsy demonstrated subepithelial fibrillar deposits of size 17 mm randomly oriented with one partial cellular crescent on electron microscopy...
June 2022: Curēus
https://read.qxmd.com/read/35814329/deciphering-the-autoimmune-pathogenesis-in-fibrillary-glomerulonephritis-the-story-is-not-yet-complete
#20
JOURNAL ARTICLE
Muhammed Mubarak
No abstract text is available yet for this article.
2022: Indian Journal of Nephrology
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