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By P O Pediatrics, Nephrology
Tanja Kersnik Levart, Dušan Ferluga, Alenka Vizjak, Jerica Mraz, Nika Kojc
BACKGROUND: Understanding the role of alternative complement pathway dysregulation in membranoproliferative glomerulonephritis (MPGN) has led to a dramatic shift in its classification into two subgroups: immune complex-mediated MPGN and complement-mediated MPGN, consisting of dense deposit disease and C3 glomerulonephritis (C3GN). A limited number of C3GN cases have been published to date with not yet conclusive results since the novel therapeutic approach with eculizumab was introduced...
October 7, 2016: Diagnostic Pathology
Wei Peng, Yi Tang, Zheng Jiang, Zi Li, Xuhua Mi, Wei Qin
BACKGROUND: Immunoglobin A nephropathy (IgAN), the most prevalent form of primary glomerulonephritis, represents the leading cause of kidney failure among East Asian populations. Immunosuppressive treatment regimen, except for a 6-month trial of corticosteroids, has not been approved by the KDIGO guideline yet. Specific and effective treatment is still lacking. We decided to evaluate the efficacy and safety of the calcineurin inhibitors (CNIs) in the treatment of IgAN. METHODS: Database from the Cochrane library, PubMed, Embase, CBM, CNKI, and CENTRAL databases were searched and reviewed up to March 2016...
August 2016: Medicine (Baltimore)
Cristina Sarcina, Carmine Tinelli, Francesca Ferrario, Bianca Visciano, Antonello Pani, Annalisa De Silvestri, Ilaria De Simone, Lucia Del Vecchio, Veronica Terraneo, Silvia Furiani, Gaia Santagostino, Enzo Corghi, Claudio Pozzi
The clinical course of IgA nephropathy (IgAN) and its outcome are extremely variable. Proteinuria at baseline has been considered one of the most important risk factors. More recently, mean proteinuria of follow-up (time-average proteinuria: TAp) was described as a stronger marker of renal survival, suggesting to consider it as a marker of disease activity and response to treatment. We evaluated predictors of renal survival in IgAN patients with different degrees of renal dysfunction and histological lesions, focusing on the role of the therapy in influencing TAp...
2016: PloS One
Mazdak A Khalighi, Shihtien Wang, Kammi J Henriksen, Margret Bock, Mahima Keswani, Shane M Meehan, Anthony Chang
BACKGROUND: Post-infectious glomerulonephritis (PIGN) is an immune complex-mediated glomerular injury that typically resolves. Dominant C3 deposition is characteristic of PIGN, but with the emergence of C3 glomerulonephritis (C3GN) as a distinct entity, it is unclear how the pathologic similarities between PIGN and C3GN should be reconciled. Therefore, nephrologists and nephropathologists need additional guidance at the time of biopsy. METHODS: We studied 23 pediatric and young adult patients diagnosed with PIGN...
June 2016: Clinical Kidney Journal
Camilla Grøndahl, Søren Rittig, Johan Vestergaard Povlsen, Kostantinos Kamperis
Acute postinfectious glomerulonephritis (PIGN) affects children typically after upper respiratory tract or skin infections with streptococci but can complicate the course of other infections. In children, it is generally a self-limiting disease with excellent prognosis. This paper reports a previously healthy 4-year-old boy who experienced a protracted course of PIGN with persisting episodes of gross haematuria, proteinuria, decreased complement C3c levels but normal P-creatinine levels. Due to the protracted course and the nephrotic-range proteinuria, a renal biopsy was performed 6 months after the initial presentation and the overall pathology was consistent with acute endocapillary glomerulonephritis...
January 2016: Case Reports in Nephrology and Dialysis
Ken Iseri, Masayuki Iyoda, Yasutaka Yamamoto, Naoto Kobayashi, Takashi Oda, Yutaka Yamaguchi, Takanori Shibata
We herein report the case of an 18-year-old boy who developed nephrotic syndrome and hypertension after upper airway inflammation. Post-streptococcal acute glomerulonephritis was diagnosed on the basis of a high antistreptolysin O titer, hypocomplementemia, proteinuria, and microscopic hematuria. A renal biopsy was performed due to persistent proteinuria, and the pathological diagnosis was membranoproliferative glomerulonephritis (MPGN) type I. Glomeruli showed positive staining for nephritis-associated plasmin receptor (NAPlr), a nephritogenic group A streptococcal antigen, and plasmin activity was found in a similar distribution as NAPlr deposition...
2016: Internal Medicine
Andrea Angioi, Fernando C Fervenza, Sanjeev Sethi, Yuzhou Zhang, Richard J Smith, David Murray, Jens Van Praet, Antonello Pani, An S De Vriese
Kidney diseases resulting from abnormal control of the complement alternative pathway include atypical hemolytic uremic syndrome, C3 glomerulonephritis, and dense-deposit disease, as well as atypical postinfectious glomerulonephritis. Although clinically diverse, they all result from loss of surface or fluid-phase complement control, caused by acquired or genetic defects in the complement alternative pathway. As such, the diagnostic approach is similar and includes a comprehensive biochemical, genetic, and pathologic analysis of the complement pathway...
February 2016: Kidney International
Yoichi Iwafuchi, Tetsuo Morioka, Takashi Morita, Kanako Watanabe, Yuko Oyama, Ichiei Narita
Nephrotic syndrome without hematuria due to infection-related glomerulonephritis is uncommon. The present report describes a case of nephrotic syndrome due to infection-related glomerulonephritis without hematuria and hypertension in an older child. A 14-year-old boy was referred to our hospital because of a 5-day history of fever, nausea, weight gain and recent leg edema without hypertension. Laboratory data showed nephrotic-range proteinuria, hypoalbuminemia, mild hypocomplementemia and acute renal injury without hematuria...
January 2016: Case Reports in Nephrology and Dialysis
Claudia Yuste, Francisco Rivera, Juan Antonio Moreno, Juan Manuel López-Gómez
Recent studies suggest a pathogenic role for glomerular haematuria among renal function. However, there is no data on the prevalence of haematuria from a large renal biopsy registry. We analysed the prevalence of gross (GH) and microscopic (mH) haematuria in 19,895 patients that underwent native renal biopsies from the Spanish Registry of Glomerulonephritis. Haematuria's overall incidence was 63% (GH 8.6% and mH 55.1%), being more frequent in males (64.7% vs. 62.4%). GH was more prevalent in patients <18 years (21...
2016: Scientific Reports
Aadil Kakajiwala, Tricia Bhatti, Bernard S Kaplan, Rebecca L Ruebner, Lawrence Copelovitch
A 7-year-old male with poststreptococcal glomerulonephritis (PSGN) developed hemolytic uremic syndrome (HUS) and achieved remission. He was treated with eculizumab for 1 year. The eculizumab was discontinued and the patient remained in remission. This is the 10th reported case of PSGN associated with HUS. The histopathological feature observed at the 1-year follow-up was indistinguishable from the expected findings in an individual with healed PSGN without associated HUS. The relatively good prognosis in most prior cases and the absence of any reported recurrences strongly suggest that this form of atypical HUS does not warrant long-term eculizumab therapy...
February 2016: Clinical Kidney Journal
Wisit Cheungpasitporn, Claudia C Zacharek, Fernando C Fervenza, Lynn D Cornell, Sanjeev Sethi, Loren P Herrera Hernandez, Samih H Nasr, Mariam P Alexander
Anti-glomerular basement membrane (anti-GBM) disease is a major cause of rapidly progressive glomerulonephritis (RPGN). On the other hand, fibrillary glomerulonephritis (GN) typically presents as proteinuria, hematuria and renal insufficiency, but rarely as RPGN. Without electron microscopy, the diagnosis of fibrillary GN can be missed. We report a 68-year-old white woman who presented with RPGN with kidney biopsy demonstrating diffuse crescentic GN on light microscopy. By immunofluorescence, there was bright linear staining of the GBMs and smudgy mesangial staining for immunoglobulin G, C3, and kappa and lambda light chain...
February 2016: Clinical Kidney Journal
Joe Devasahayam, Gowrishankar Erode-Singaravelu, Zeenat Bhat, Tony Oliver, Arul Chandran, Xu Zeng, Paramesh Dakshinesh, Unni Pillai
C1q nephropathy is a rare glomerular disease with characteristic mesangial C1q deposition noted on immunofluorescence microscopy. It is histologically defined and poorly understood. Light microscopic features are heterogeneous and comprise minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), and proliferative glomerulonephritis. Clinical presentation is also diverse, and ranges from asymptomatic hematuria or proteinuria to frank nephritic or nephrotic syndrome in both children and adults...
2015: Analytical Cellular Pathology (Amsterdam)
Farah Tamirou, Bernard R Lauwerys, Maria Dall'Era, Meggan Mackay, Brad Rovin, Ricard Cervera, Frédéric A Houssiau
BACKGROUND: Although an early decrease in proteinuria has been correlated with good long-term renal outcome in lupus nephritis (LN), studies aimed at defining a cut-off proteinuria value are missing, except a recent analysis performed on patients randomised in the Euro-Lupus Nephritis Trial, demonstrating that a target value of 0.8 g/day at month 12 optimised sensitivity and specificity for the prediction of good renal outcome. The objective of the current work is to validate this target in another LN study, namely the MAINTAIN Nephritis Trial (MNT)...
2015: Lupus Science & Medicine
Kenji Ueki, Yuta Matsukuma, Kosuke Masutani, Akihiro Tsuchimoto, Kiichiro Fujisaki, Kumiko Torisu, Shigeru Tanaka, Tamotsu Kiyoshima, Satoshi Hisano, Takanari Kitazono, Kazuhiko Tsuruya
BACKGROUND: IgG4-related disease is a novel disease entity characterized by elevated serum IgG4 and tissue infiltration by IgG4-positive plasma cells. Typical renal pathology is tubulointerstitial nephritis with storiform fibrosis, although the co-existence of various glomerular lesions has been described. Here, we present the first report of a case of IgG4-related kidney disease and membranoproliferative glomerulonephritis showing the discrepancy in IgG subclasses between the kidney interstitium and glomeruli...
2015: BMC Nephrology
Neeraj Dhaun, Andrew Saunders, Christopher O Bellamy, Rocío Martinez Gallardo, Lynn Manson, David C Kluth
BACKGROUND: Current recommendations for ANCA-associated vasculitis (AAV) support its management within a dedicated clinical service. Therapies for AAV are imperfect with many patients failing to achieve disease control and others experiencing disease relapse. Plasma exchange (PEX) may be beneficial especially when the kidney is involved. METHODS: Within a new, dedicated service we retrospectively assessed, over a 6-year period, the benefits of PEX in two patient cohorts, discriminated by PEX treatment alone...
2015: BMC Musculoskeletal Disorders
Mohamed R Daha, Cees van Kooten
Immunoglobulin A nephropathy (IgAN) is characterized by the deposition of IgA in the mesangium of glomeruli. This mesangial IgA has been found to consist mainly of polymeric IgA1 which drives the activation of the mesangial cells and results in excessive production of several inflammatory mediators. The activation of mesangial cells is amplified by the ability of IgA to activate the complement system, originally thought to occur mainly via the alternative pathway of complement. However more recent studies indicate that lectin pathway involvement has a strong association with progression of renal disease...
February 2016: Journal of Nephrology
Sanjeev Sethi, Mark Haas, Glen S Markowitz, Vivette D D'Agati, Helmut G Rennke, J Charles Jennette, Ingeborg M Bajema, Charles E Alpers, Anthony Chang, Lynn D Cornell, Fernando G Cosio, Agnes B Fogo, Richard J Glassock, Sundaram Hariharan, Neeraja Kambham, Donna J Lager, Nelson Leung, Michael Mengel, Karl A Nath, Ian S Roberts, Brad H Rovin, Surya V Seshan, Richard J H Smith, Patrick D Walker, Christopher G Winearls, Gerald B Appel, Mariam P Alexander, Daniel C Cattran, Carmen Avila Casado, H Terence Cook, An S De Vriese, Jai Radhakrishnan, Lorraine C Racusen, Pierre Ronco, Fernando C Fervenza
Renal pathologists and nephrologists met on February 20, 2015 to establish an etiology/pathogenesis-based system for classification and diagnosis of GN, with a major aim of standardizing the kidney biopsy report of GN. On the basis of etiology/pathogenesis, GN is classified into the following five pathogenic types, each with specific disease entities: immune-complex GN, pauci-immune GN, antiglomerular basement membrane GN, monoclonal Ig GN, and C3 glomerulopathy. The pathogenesis-based classification forms the basis of the kidney biopsy report...
May 2016: Journal of the American Society of Nephrology: JASN
China Nagano, Yoshimitu Goto, Katuaki Kasahara, Yoshiyuki Kuroyanagi
BACKGROUND: Anti-glomerular basement membrane (GBM) antibody disease is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis caused by autoantibodies against the α3-chain of type IV collagen in the GBM. CASE PRESENTATION: An 8-year-old girl with hematuria and proteinuria due to anti-GBM nephritis was diagnosed with hematuria and proteinuria during a school urine screening program. Her blood pressure and serum creatinine levels were normal...
2015: BMC Nephrology
Hiroyuki Komatsu, Shouichi Fujimoto, Norishige Yoshikawa, Hiroshi Kitamura, Hitoshi Sugiyama, Hitoshi Yokoyama
BACKGROUND: The clinical presentation of Henoch-Schönlein purpura nephritis (HSPN) has not been thoroughly investigated among patients of different ages. We therefore compared the features of HSPN and IgA nephropathy (IgAN) based on data from the Japan Renal Biopsy Registry (J-RBR). METHODS: This cross-sectional study analyzed data from patients who were registered in the J-RBR between 2007 and 2012. Clinico-pathological findings at diagnosis were compared among children (aged ≤18 years), adult (aged 19-64 years) and elderly (aged ≥65 years) patients with HSPN (n = 513) and IgAN (n = 5679)...
August 2016: Clinical and Experimental Nephrology
Lucio Manenti, Elisa Gnappi, Augusto Vaglio, Landino Allegri, Marina Noris, Elena Bresin, Francesco Paolo Pilato, Elisabetta Valoti, Sonia Pasquali, Carlo Buzio
BACKGROUND: Primary or secondary glomerulonephritis has been anecdotally reported in association with atypical haemolytic uraemic syndrome (aHUS). We here report a series of six patients who developed aHUS and glomerulopathy, and review the literature on aHUS and glomerulonephritis. METHODS: Out of all patients diagnosed at our unit with biopsy-proven glomerular diseases between March 2007 and October 2011, selected cases developing aHUS during the follow-up are presented...
September 2013: Nephrology, Dialysis, Transplantation
2015-10-01 12:39:28
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