keyword
https://read.qxmd.com/read/38352852/monoclonal-gammopathy-of-renal-significance-an-atypical-presentation-of-waldenstr%C3%A3-m-s-disease
#1
Pablo Rodríguez-Doyágüez, Motornaya-Morozova, Patricia Martínez-Miguel, Carolina Castillo-Torres, Óscar Toldos-González, Juan José Gil-Fernández
Waldenström's disease is a rare lymphoproliferative syndrome in the bone marrow and sometimes in lymphoid organs which secretes high amounts of monoclonal immunoglobulin M into serum. It can remain indolent for years and rarely affects the kidney, with intraglomerular rather than intratubular damage being predominant, in contrast to multiple myeloma. Different studies identified AL amyloidosis as the most frequent renal lesion, followed by cryoglobulinemic glomerulonephritis. Signs and symptoms may be unspecific, as well as renal manifestations, so collaboration between nephrologists, hematologists, and pathologists is crucial to establish the role of paraprotein in the development of renal damage...
2024: Clinical Nephrology. Case Studies
https://read.qxmd.com/read/38213351/c3-glomerulonephritis-presenting-with-nephritic-and-nephrotic-syndromes-spontaneous-remission-after-six-months-on-dialysis
#2
Francisco Gonçalves, Nídia Marques, Roberto Silva, Luis Mendonça, Bernardo Faria
C3 glomerulopathy is a rare and complex renal disease driven by complement dysregulation, with variable presentation and pathophysiology. We report the case of a middle-aged male patient presenting with nephritic and nephrotic syndromes and low serum C3, whose biopsy established the diagnosis of C3 glomerulonephritis. He was found to be homozygous for the complement factor H-related protein (CFHR)3-CFHR1 deletion, which has been associated with the development of anti-factor H autoantibodies. However, the lack of consistent and accessible nephritic factor assays prevented full clarification of the mechanisms involved in the disease...
December 2023: Curēus
https://read.qxmd.com/read/38186493/syphilis-related-nephropathy-a-rare-manifestation-of-a-re-emerging-disease
#3
Aya Aal Hamad, Zeyana Al Hadhrami, Ali Al Lawati, Ibrahim Al Busaidi, Saja Mahmood
Syphilis is a curable sexually transmitted infection caused by the spirochete Treponema pallidum . Its clinical manifestations are variable as it has a remarkable aptitude to imitate a spectrum of clinical pictures. This phenomenon has bestowed upon it the epithet "the great imitator" within the medical literature. The escalating global prevalence of syphilis cases underscores the importance of shedding light on its rare manifestations. Syphilitic nephropathy is an uncommon manifestation of secondary syphilis...
December 2023: Curēus
https://read.qxmd.com/read/38117311/biomarkers-of-histologic-severity-in-children-with-severe-or-atypical-acute-post-streptococcal-glomerulonephritis
#4
JOURNAL ARTICLE
William Wong, Chanel Prestidge, Jonathan Zwi, Dug Yeo Han
BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is a common cause of acute kidney injury (AKI) in children; however, in a small subgroup, the presentation is one of rapidly progressive glomerulonephritis (RPGN) deteriorating kidney function associated with severe oligo-anuria or a mixed nephritic-nephrotic picture. This study reviewed potential clinical and laboratory factors which may assist the treating clinician to identify patients at high risk of severe disease. METHODS: All kidney biopsies for APSGN performed between 1996 and 2020 were obtained from a departmental biopsy database...
December 20, 2023: Pediatric Nephrology
https://read.qxmd.com/read/38072161/changes-in-renal-microcirculation-in-patients-with-nephrotic-and-nephritic-syndrome-the-role-of-resistive-index
#5
JOURNAL ARTICLE
Antonietta Gigante, Chiara Pellicano, Oriana De Marco, Eleonora Assanto, Georgia Sorato, Alberto Palladini, Edoardo Rosato, Silvia Lai, Maurizio Muscaritoli, Rosario Cianci
BACKGROUND: Renal Resistive Index (RRI) is an important and non-invasive parameter of renal damage and it is associated with abnormal microcirculation or to a parenchymal injury. The aim of our study was to compare the RRI in a cohort of patients with renal diseases categorized in three groups: nephrotic syndrome (NS), acute nephritic syndrome (ANS) and patients with urinary abnormalities (UA). METHODS: Four hundred eighty-two patients with median age of 48 years (IQR 34-62) with indications for kidney disease were included in the study...
March 2024: Microvascular Research
https://read.qxmd.com/read/38025239/a-qualitative-evaluation-of-advanced-training-programs-in-glomerular-diseases-results-from-a-program-directors-survey
#6
EDITORIAL
Kenar D Jhaveri, Insa M Schmidt, Jun Oh, Laurel J Damashek, Koyal Jain
No abstract text is available yet for this article.
November 2023: KI Reports
https://read.qxmd.com/read/37926528/discontinuing-hemodialysis-through-corticosteroid-treatment-in-a-patient-with-cryofibrinogen-associated-glomerulonephritis
#7
JOURNAL ARTICLE
Shigeyuki Arai, Rena Matsui, Wataru Ono, Shinichiro Asakawa, Osamu Yamazaki, Yoshifuru Tamura, Mika Terasaki, Kanade Hagiwara, Tohru Nakagawa, Ryuji Ohashi, Akira Shimizu, Shigeru Shibata, Yoshihide Fujigaki
Cryofibrinogen-associated glomerulonephritis (CryoFiGN) is rare, and its diagnosis is difficult while treatment is not established. We herein report an elderly woman with CryoFiGN who experienced recurrent purpura and nephritic features that subsequently progressed to nephrotic syndrome and required hemodialysis during the 18-month clinical course. The patient was treated with corticosteroids, which led to the discontinuation of hemodialysis. The diagnosis of CryoFiGN was based on the clinical presentation, characteristic glomerular deposits, and results of laser microdissection and liquid chromatography-tandem mass spectrometry of the glomeruli...
November 6, 2023: Internal Medicine
https://read.qxmd.com/read/37901697/an-unusual-case-of-seronegative-cryoglobulinemic-glomerulonephritis-with-dominant-organized-iga-deposits-associated-with-staphylococcal-infection-casual-or-causal-relationship
#8
José C De La Flor Merino, Jacqueline Apaza, Francisco Díaz, Edna Sandoval, Francisco Valga, Daniel Villa, Alexander Marschall, María Luisa Abascal, Andrea Rivas, Michael Cieza
INTRODUCTION: Cryoglobulinemia refers to the presence of cryoglobulins (CGs) in the serum, encompassing a group of diseases caused by the type of circulating GC. Cryoglobulinemic glomerulonephritis (CryoGN) is the principal manifestation of renal involvement. The diagnosis may be challenging because the hallmark of cryoglobulinemia is the detection of CG in the serum. However, cases of CryoGN without serological evidence of CGs are not uncommon in clinical practice, often diagnosed by anatomopathological findings in the renal biopsy...
2023: Glomerular diseases
https://read.qxmd.com/read/37827830/atypical-form-of-goodpasture-s-disease
#9
JOURNAL ARTICLE
Michaela Habánová, Petra Divácká, Jitka Řehořová, Iva Svobodová
Goodpasture's disease and anti-glomerular basement membrane nephritis (anti-GBM nephritis) are rare autoimmune small vessel vasculitis predominantly affecting young men. Goodpasture's disease plays an important part in differential diagnosis of pulmonary - renal syndrome. The evidence of circulating autoantibodies, a typical histological appearance of the kidney biopsy with finding of the crescent glomerulonephritis and clinical presentation of nephritic syndrome play an important role in diagnosis. Our case report describes a case of a young male with anti-GBM nephritis that presents as rapidly progressive glomerulonephritis (RPGN) with progression to dialysis-dependent renal failure...
2023: Vnitr̆ní Lékar̆ství
https://read.qxmd.com/read/37766840/immunoglobulin-a-vasculitis-nephritis-current-understanding-of-pathogenesis-and-treatment
#10
REVIEW
Michela Amatruda, Nicolina Stefania Carucci, Roberto Chimenz, Giovanni Conti
The clinical spectrum of immunoglobulin A vasculitis nephritis (IgAVN) ranges from the relatively common transitory microscopic hematuria and/or low-grade proteinuria to nephritic or nephrotic syndrome, rapidly progressive glomerulonephritis, or even renal failure. Clinical and experimental studies have shown a multifactor pathogenesis: Infection triggers, impaired glycosylation of IgA1, complement activation, Toll-like-receptor activation and B cell proliferation. This knowledge can identify IgAVN patients at a greater risk for adverse outcome and increase the evidence for treatment recommendations...
September 25, 2023: World Journal of Nephrology
https://read.qxmd.com/read/37667269/clinical-characteristics-of-infants-hospitalized-with-early-congenital-syphilitic-nephropathy-a-single-center-retrospective-cross-sectional-study-in-china
#11
JOURNAL ARTICLE
Caiying Wang, Wenhui Lun, Lin Pang
BACKGROUND: Early studies claimed that early congenital syphilitic (CS) nephropathy was rare, and systematic studies about this disease are absent, which may lead to poor awareness of early CS nephropathy in clinicians and result in misdiagnosis and poor patient prognosis. The present study systematically and comprehensively analyzes the clinical characteristics of infants with early CS nephropathy hospitalized in Beijing Ditan Hospital, an infectious disease hospital in China in order to improve the understanding and management of this disorder...
September 4, 2023: BMC Pediatrics
https://read.qxmd.com/read/37499240/multidisciplinary-approach-to-lupus-nephritis-clinical-pearls-pitfalls-and-positioning-of-newly-approved-agents
#12
REVIEW
Antonis Fanouriakis, George Bertsias, George Liapis, Smaragdi Marinaki, Aikaterini Papagianni, Maria Stangou, Alexandros Garyfallos, Sophia Lionaki, Maria G Tektonidou, John N Boletis, Dimitrios T Boumpas
Lupus nephritis (LN) is a major course of morbidity and mortality in patients with systemic lupus erythematosus (SLE), best managed by a multidisciplinary group. To this end, we gathered a group of rheumatologists, nephrologists and a nephropathologist to review current evidence regarding diagnosis and management of LN. In this consensus paper, we summarize the key points from this meeting and provide practice guidelines for the management of kidney involvement in SLE, in view of emerging new data concerning novel agents approved recently...
September 2023: Lupus
https://read.qxmd.com/read/37448894/iga-nephropathy-beyond-the-renal-cortex
#13
Balaji Kirushnan, Balasubramaniyum Raju, Anila A Kurien
Immunoglobulin A (IgA) nephropathy is the most common glomerular disease worldwide. It usually presents as a nephritic syndrome with macroscopic hematuria, oliguria, and proteinuria with or without azotemia. Rapidly progressive glomerulonephritis with crescents is being described in about 30% of cases and is mostly associated with nephrotic-range proteinuria, accelerated hypertension, and accelerated decline toward end-stage renal disease. Medullary angiitis is a rare finding in renal biopsy and is usually associated with pauci-immune glomerulonephritis...
2023: Indian Journal of Nephrology
https://read.qxmd.com/read/37303444/a-rare-presentation-of-iga-nephropathy-in-a-62-year-old-hispanic-female
#14
Sarah C Kurkowski, Michael J Thimmesch, Osama Elkhider, Yasir Abdelgadir
A case of immunoglobulin A (IgA) nephropathy is presented here that demonstrates an unusual clinical presentation in multiple ways and is vitally important for clinicians to consider. The patient is a Hispanic female in her 7th decade of life that presented with nephrotic-range proteinuria without hematuria ultimately leading to a diagnosis of IgA nephropathy. After diagnosis, her clinical course was complicated by continued poorly controlled type II diabetes mellitus and hypertension, and ultimately her kidney disease progressed to chronic kidney disease IV and then end-stage renal disease requiring hemodialysis...
May 2023: Curēus
https://read.qxmd.com/read/37288211/sj%C3%A3-gren-s-syndrome-overlap-with-lupus-nephritis-a-case-report-and-literature-review-of-a-rare-entity
#15
Sujith K Palleti, Maria M Picken, Anuradha Wadhwa
Overlap syndrome is a connective tissue disorder that satisfies the diagnostic criteria for at least two well-known autoimmune diseases. In this report, we describe a rare case of lupus overlap in an elderly woman with primary Sjögren's syndrome who presented with features of nephritic-nephrotic syndrome and renal biopsy results typical of lupus nephritis combined with multiple positive autoantibodies. The kidney biopsy results were assigned the highest weightage based on the revised 2019 systemic lupus erythematosus (SLE) classification criteria developed by the European League Against Rheumatism (EULAR) and the American College of Rheumatology (ACR)...
May 2023: Curēus
https://read.qxmd.com/read/37197040/pediatric-glomerular-diseases-in-north-india-epidemiology-and-clinicopathologic-correlation
#16
JOURNAL ARTICLE
Zafirah Zahir, Asif Sadiq Wani, Manoj Jain, Vinita Agrawal, Shweta Jain
BACKGROUND: Glomerular diseases vary with age, and it is important to investigate the spectrum of glomerular diseases in pediatric patients to help in a more precise clinical diagnosis and optimize the management of patients. We aimed to study the clinicopathologic pattern of pediatric glomerular diseases in North India. METHODS: This is a 5-year retrospective, single-center cohort study. The database was searched to identify all pediatric patients with glomerular diseases in their native kidney biopsies...
2023: Indian Journal of Nephrology
https://read.qxmd.com/read/36920554/incidence-and-risk-factors-of-acute-kidney-injury-among-childhood-nephrotic-syndrome-a-prospective-cohort-study
#17
JOURNAL ARTICLE
Sanchari Ghosh, Shakil Akhtar, Subal Kumar Pradhan, Subhankar Sarkar, Deblina Dasgupta, Ruhi Parween, Shina Menon, Rajiv Sinha
Acute kidney injury (AKI) is a known independent risk factor for morbidity/mortality but there is scarcity of robust data on it among childhood nephrotic syndrome (NS). We assessed the incidence of AKI among hospitalized children with NS as well as looked for any significant risk factors. Prospective observational study conducted across two tertiary pediatric hospitals in Eastern India from September 2020 to August 2021. Children aged 1-18 years admitted with NS and without any nephritic features or pre-existing chronic kidney disease (CKD) were included...
May 2023: European Journal of Pediatrics
https://read.qxmd.com/read/36913081/iga-nephropathy-to-proliferative-glomerulonephritis-with-monoclonal-iga%C3%AE%C2%BA-deposits-a-pattern-switch
#18
JOURNAL ARTICLE
Jin-Pu Li, Ya-Ting Du, Chuan Guo, Xiang-Rong Rao, Shen Li
We report the case of a 31-year-old male who presented with repeated episodes of nephritic-nephrotic syndrome in concomitance with infection. IgA was diagnosed and was initially responsive to treatment with immunosuppressors but further disease flare did not respond to treatment. Based on three consecutive renal biopsies over 8 years, a pattern switch from endocapillary proliferative IgA nephropathy to membranous proliferative glomerulonephritis with monoclonal IgAκ deposits was observed...
March 13, 2023: Journal of Nephrology
https://read.qxmd.com/read/36853224/interventions-for-preventing-and-treating-kidney-disease-in-iga-vasculitis
#19
REVIEW
Deirdre Hahn, Elisabeth M Hodson, Jonathan C Craig
BACKGROUND: IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is the most common vasculitis of childhood but may also occur in adults. This small vessel vasculitis is characterised by palpable purpura, abdominal pain, arthritis or arthralgia and kidney involvement. This is an update of a review first published in 2009 and updated in 2015. OBJECTIVES: To evaluate the benefits and harms of different agents (used singularly or in combination) compared with placebo, no treatment or any other agent for (1) the prevention of severe kidney disease in people with IgAV with or without kidney involvement at onset, (2) the treatment of established severe kidney disease (macroscopic haematuria, proteinuria, nephritic syndrome, nephrotic syndrome with or without acute kidney failure) in IgAV, and (3) the prevention of recurrent episodes of IgAV-associated kidney disease...
February 28, 2023: Cochrane Database of Systematic Reviews
https://read.qxmd.com/read/36726429/clinicopathological-characteristics-and-disease-chronicity-in-native-kidney-biopsies-in-flanders
#20
JOURNAL ARTICLE
Dries Deleersnijder, Wim Laurens, Johan De Meester, Evert Cleenders, Amélie Dendooven, Evelyne Lerut, An S De Vriese, Tom Dejagere, Mark Helbert, Rachel Hellemans, Priyanka Koshy, Bart Maes, Lissa Pipeleers, Amaryllis H Van Craenenbroeck, Steven Van Laecke, Johan Vande Walle, Marie M Couttenye, Gert Meeus, Ben Sprangers
BACKGROUND: The Flemish Collaborative Glomerulonephritis Group (FCGG) registry provides complete population data on kidney disease epidemiology in the region of Flanders (Belgium), as it captures all native kidney biopsies performed in its population of 6.5 million inhabitants. METHODS: From 2017 until 2019, 2054 adult kidney biopsies were included from 26 nephrology centers (one biopsy per patient). Data on nephrotic and nephritic syndrome were available in 1992 and 2026 biopsies, respectively...
January 2023: Clinical Kidney Journal
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