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crescentic nephropathy

Oleg Palygin, Daria V Ilatovskaya, Alexander Staruschenko
Protease-activated receptors (PARs) are a well-known family of transmembrane G-protein-coupled receptors (GPCRs). To date, four PARs have been identified and PAR1 and PAR2 are the most abundant receptors, which were shown to be expressed in the kidney vascular and tubular cells. PAR signaling is mediated by an N-terminus tethered ligands that can be unmasked by serine protease cleavage. PARs are activated by endogenous serine proteases, such as thrombin (acts on PARs 1, 3 and 4) and trypsin (PAR2). PARs can be involved in glomerular, microvascular and inflammatory regulation of renal function in both normal and pathological conditions...
October 12, 2016: American Journal of Physiology. Renal Physiology
Turki Al Hussain, Hadeel Al Mana, Maged H Hussein, Mohammed Akhtar
The glomerulus has 3 resident cells namely mesangial cells that produce the mesangial matrix, endothelial cells that line the glomerular capillaries, and podocytes that cover the outer surface of the glomerular basement membrane. Parietal epithelial cells (PrECs), which line the Bowman's capsule are not part of the glomerular tuft but may have an important role in the normal function of the glomerulus. A significant progress has been made in recent years regarding our understanding of the role and function of these cells in normal kidney and in kidneys with various types of glomerulopathy...
September 26, 2016: Advances in Anatomic Pathology
Mark Haas, Jacobien C Verhave, Zhi-Hong Liu, Charles E Alpers, Jonathan Barratt, Jan U Becker, Daniel Cattran, H Terence Cook, Rosanna Coppo, John Feehally, Antonello Pani, Agnieszka Perkowska-Ptasinska, Ian S D Roberts, Maria Fernanda Soares, Hernan Trimarchi, Suxia Wang, Yukio Yuzawa, Hong Zhang, Stéphan Troyanov, Ritsuko Katafuchi
The Oxford Classification of IgA nephropathy does not account for glomerular crescents. However, studies that reported no independent predictive role of crescents on renal outcomes excluded individuals with severe renal insufficiency. In a large IgA nephropathy cohort pooled from four retrospective studies, we addressed crescents as a predictor of renal outcomes and determined whether the fraction of crescent-containing glomeruli associates with survival from either a ≥50% decline in eGFR or ESRD (combined event) adjusting for covariates used in the original Oxford study...
September 9, 2016: Journal of the American Society of Nephrology: JASN
Tomohiro Terasaka, Haruhito A Uchida, Ryoko Umebayashi, Keiko Tsukamoto, Keiko Tanaka, Masashi Kitagawa, Hitoshi Sugiyama, Hiroaki Tanioka, Jun Wada
BACKGROUND: A link between IgA nephropathy and Crohn's disease has recently been reported. Other researchers hypothesize that intestine-derived IgA complexes deposit in glomerular mesangial cells, eliciting IgA nephropathy. Intestinal mucosal plasma cells mainly secrete IgA2. Nevertheless, IgA1 deposition is strongly implicated as being the primary cause of IgA nephropathy. CASE PRESENTATION: A 46-year-old Japanese man developed IgA nephropathy 29 years ago, following tonsillectomy...
September 5, 2016: BMC Nephrology
M Mizerska-Wasiak, A Turczyn, A Such, K Cichoń-Kawa, J Małdyk, M Miklaszewska, J Pietrzyk, A Rybi-Szumińska, A Wasilewska, A Firszt-Adamczyk, R Stankiewicz, M Szczepańska, B Bieniaś, M Zajączkowska, A Pukajło-Marczyk, D Zwolińska, K Siniewicz-Luzeńczyk, M Tkaczyk, K Gadomska-Prokop, R Grenda, U Demkow, M Pańczyk-Tomaszewska
IgA nephropathy (IgAN) is the most common form of glomerulonephritis in pediatric population. The clinical presentation of the disease in children ranges from microscopic hematuria to end-stage kidney disease. The aim of the study was to retrospectively assess clinical and kidney biopsy features in children with IgAN. We assessed a cohort of 140 children, 88 boys, 52 girls with the diagnosis of IgAN in the period of 2000-2015, entered into the national Polish pediatric IgAN registry. The assessment included the following: proteinuria, hematuria, glomerular filtration rate (GFR), arterial blood pressure, and the renal pathological changes according to the Oxford classification and crescents formation, as modifiable and unmodifiable risk factors...
August 30, 2016: Advances in Experimental Medicine and Biology
Rosanna Coppo, Danilo Lofaro, Roberta R Camilla, Shubha Bellur, Daniel Cattran, H Terence Cook, Ian S D Roberts, Licia Peruzzi, Alessandro Amore, Francesco Emma, Laura Fuiano, Ulla Berg, Rezan Topaloglu, Yelda Bilginer, Loreto Gesualdo, Rosaria Polci, Malgorzata Mizerska-Wasiak, Yasar Caliskan, Sigrid Lundberg, Giovanni Cancarini, Colin Geddes, Jack Wetzels, Andrzej Wiecek, Magdalena Durlik, Stefano Cusinato, Cristiana Rollino, Milena Maggio, Manuel Praga, Hilde K Smerud, Vladimir Tesar, Dita Maixnerova, Jonathan Barratt, Teresa Papalia, Renzo Bonofiglio, Gianna Mazzucco, Costantinos Giannakakis, Magnus Soderberg, Diclehan Orhan, Anna Maria Di Palma, Jadwiga Maldyk, Yasemin Ozluk, Birgitta Sudelin, Regina Tardanico, David Kipgen, Eric Steenbergen, Henryk Karkoszka, Agnieszka Perkowska-Ptasinska, Franco Ferrario, Eduardo Gutierrez, Eva Honsova
BACKGROUND: There is a need for early identification of children with immunoglobulin A nephropathy (IgAN) at risk of progression of kidney disease. METHODS: Data on 261 young patients [age <23 years; mean follow-up of 4.9 (range 2.5-8.1) years] enrolled in VALIGA, a study designed to validate the Oxford Classification of IgAN, were assessed. Renal biopsies were scored for the presence of mesangial hypercellularity (M1), endocapillary hypercellularity (E1), segmental glomerulosclerosis (S1), tubular atrophy/interstitial fibrosis (T1-2) (MEST score) and crescents (C1)...
August 25, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Clemens L Bockmeyer, Karen Säuberlich, Juliane Wittig, Marc Eßer, Sebastian S Roeder, Udo Vester, Peter F Hoyer, Putri A Agustian, Philip Zeuschner, Kerstin Amann, Christoph Daniel, Jan U Becker
Small nucleolar RNAs (snoRNAs) have been used for normalization in glomerular microRNA (miRNA) quantification without confirmation of validity. Our aim was to identify glomerular reference miRNAs in IgA nephropathy. We compared miRNAs in human paraffin-embedded renal biopsies from patients with cellular-crescentic IgA-GN (n = 5; crescentic IgA-GN) and non-crescentic IgA-GN (n = 5; IgA-GN) to mild interstitial nephritis without glomerular abnormalities (controls, n = 5). Laser-microdissected glomeruli were used for expression profiling of 762 miRNAs by low-density TaqMan arrays (cards A and B)...
August 24, 2016: Scientific Reports
Xinfang Xie, Jicheng Lv, Sufang Shi, Li Zhu, Lijun Liu, Min Chen, Yu Wang, Zhao Cui, Xin Wang, Li Liu, Xiaojuan Yu, Fude Zhou, Ming-Hui Zhao, Hong Zhang
BACKGROUND: Crescentic IgA nephropathy (CreIgAN) has a poor prognosis despite aggressive immunosuppressive therapy. The efficacy of plasma exchange (PE) in CreIgAN is not well defined. METHODS: Twelve patients with severe CreIgAN who received PE as addition to routine immunosuppressive therapy, followed for more than 6 months, were involved. Twelve matched historical controls who received immunosuppressive therapy alone were selected by propensity score matching...
2016: American Journal of Nephrology
S K Rampelli, N G Rajesh, B H Srinivas, K T Harichandra Kumar, R P Swaminathan, P S Priyamvada
There is limited data on the etiology, clinical and histopathological spectrum and outcomes of crescentic glomerulonephritis (CrGN) in adult Indian population. This prospective study was done to evaluate the etiology, clinicohistological patterns and predictors of outcome of CrGN in South Indian population. All the patients received standard protocol based immunosuppression in addition to supportive care. Immune-complex glomerulonephritis (ICGN) was the most common etiology (n = 31; 77.5%) followed by pauci-immune glomerulonephritis (PauciGN; n = 8; 20%) and anti-glomerular basement membrane disease (n = 1; 2...
July 2016: Indian Journal of Nephrology
M G Chen, X H Ye, H Y Liang, Q Yang
OBJECTIVE: To investigate the incidence, etiology, clinico- pathological characteristics and prognosis in primary IgA nephropathy (IgAN) children with acute kidney injury (AKI). METHOD: Retrospective analysis of the clinical and pathological manifestations and follow-up results of 19 Chlidren, who were associated with AKI in 196 cases of children with IgA nephropathy treated in our department from January, 1996 to Jun, 2012 was performed. RESULT: (1) The 19 cases associated with AKI accounted for 9...
August 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Takeshi Yamazaki, Satoshi Sasaki, Takayuki Okamoto, Yasuyuki Sato, Asako Hayashi, Tadashi Ariga
BACKGROUND/AIMS: De novo expression of CD44 is considered as a marker of parietal epithelial cell (PEC) activation. The aim of our study was to explore CD74 expression, which can form a complex with CD44, in PECs during the progression of focal segmental glomerulosclerosis (FSGS). To clarify the role of CD74 expression and of its interaction with CD44, we generated a new mouse model with enhanced PEC activation through lipopolysaccharide (LPS) application to adriamycin (ADR)-induced nephropathy mice (LPS-treated ADR mice)...
July 28, 2016: Nephron
Michio Fukuda, Osamu Hotta, Masashi Mizuno, Yoshiaki Ogiyama, Nobuyuki Ohte
Proportions of elderly aged ≥65 and ≥75 within Japan will increase to 30 and 20 %, respectively, in 2025, when "Baby-Boom Generations" will reach the age of 75 years. Okabayashi and colleagues report that even in elderly patients with IgA nephropathy (IgAN), immunosuppressive treatment can reduce proteinuria, with no adverse events. Their findings remind us of recent finding from STOP-IgAN study; additional immunosuppressive therapy to intensive supportive care [specifically renin-angiotensin system (RAS) inhibitors (RASi)] did not improve the outcome...
July 21, 2016: Clinical and Experimental Nephrology
Gabriel Ştefan, Gener Ismail, Simona Stancu, Adrian Zugravu, Andreea Andronesi, Eugen Mandache, Gabriel Mircescu
The Oxford classification (OC) of IgA Nephropathy (IgAN) identified mesangial hypercellularity (M), endocapillary hypercellularity (E), segmental glomerulosclerosis (S), and tubular atrophy/interstitial fibrosis (T) as predictors of outcome. We aimed to validate the OC and to investigate the clinical significance of extracapillary hypercellularity and IgG immunostaining. We examined the renal outcome at December 31, 2014, of 121 adult patients with biopsy proven primary IgAN between 2003 and 2013. The primary endpoint was doubling of serum creatinine or renal replacement therapy initiation...
August 2016: Pathology International
Jun-Hua Liang, Yu-Wei Fang, An-Hung Yang, Ming Hsien Tsai
Methicillin-resistant Staphylococcus aureus (MRSA) is an emerging pathogen that infects the skin and soft tissue. However, there are few reports of renal complications from MRSA involving immunoglobulin (Ig)A-dominated rapidly progressive glomerulonephritis (GN). Favorable renal outcomes from IgA GN are achieved by administering timely therapy. In the present study, we describe the case of a healthy young woman suffering from a cutaneous MRSA infection that initially presented with gross hematuria. Six months after eradicating the infection, severe impairment of renal function was noted because of intractable nausea and vomiting...
June 2016: Medicine (Baltimore)
Yukihiko Kawasaki, Ryo Maeda, Shuto Kanno, Yuichi Suzuki, Shinichiro Ohara, Kazuhide Suyama, Mitsuaki Hosoya
To clarify the long-term efficacy of tonsillectomy plus methylprednisolone pulse therapy (tonsillectomy pulse therapy) for pediatric IgA nephropathy (IgAN), we evaluated the clinical and laboratory findings as well as the prognosis for IgAN treated with tonsillectomy pulse therapy at long-term follow up METHODS: We collected data on 33 IgAN children who had been treated with tonsillectomy pulse therapy. The children were retrospectively divided into two groups. Group 1 consisted of 18 children who had been treated with tonsillectomy pulse therapy as an initial therapy, and Group 2 consisted of 15 children who had been treated with tonsillectomy pulse therapy as a rescue therapy for a relapse of IgAN...
June 24, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Franz Maximilian Rasche, Frieder Keller, Wilma Gertrud Rasche, Stephan Schiekofer, Andreas Boldt, Ulrich Sack, Jeanette Fahnert
IgA Nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. Lifelong mesangial deposition of IgA1 complexes subsist inflammation and nephron loss but the complex pathogenesis in detail remains still unclear. In regard to the heterogeneous course, classical immunosuppressive and specific therapeutic regimes adapted to the loss of renal function will here discussed in addition to the essential common renal supportive therapy. Renal supportive therapy alleviates secondary, surrogate effects or sequelae on renal function and proteinuria of high intraglomerular pressure and subsequent nephrosclerosis by inhibition of the renin angiotensin system (RAASB)...
June 10, 2016: Clinical and Experimental Immunology
R Nada, A Kumar, V G Kumar, K L Gupta, K Joshi
Renal biopsy interpretation requires histopathology, direct immunofluorescence (DIF) and electron microscopy. Formalin-fixed, paraffin-embedded tissue (FFPE) sent for light microscopy can be used for DIF after antigen retrieval. However, complement staining has not been satisfactory. We standardized DIF using proteinase-K for antigen retrieval in FFPE renal biopsies. A pilot study was conducted on known cases of membranous glomerulonephritis (MGN), membranoproliferative type-1 (MPGN-1), immunoglobulin A nephropathy (IgAN), and anti-glomerular basement disease (anti-GBM)...
May 2016: Indian Journal of Nephrology
Min Tan, Wenge Li, Guming Zou, Cong Zhang, Jing Fang
BACKGROUND: The significance of segmental glomerular necrosis (SGN) was not evident in immunoglobulin A nephropathy (IgAN) patients. Especially, there were a number of patients who presented with slight histopathological damage except SGN. We, therefore, conducted a study to highlight the occurrence of these cases and to define their clinical characteristics and outcomes at our centre. METHODS: The clinical, laboratory and pathological manifestations and outcomes of these IgAN patients were collected and compared with IgAN patients with simily histopathological background but without SGN...
2016: American Journal of Nephrology
Chihiro Iwasaki, Takahito Moriyama, Kayu Tanaka, Takashi Takei, Kosaku Nitta
BACKGROUND: The relationship between hematuria and histological lesions, the effect of hematuria on response to steroid therapy, and the outcome in patients with immunoglobulin A nephropathy (IgAN) remain undetermined. OBJECTIVES: The aim of this study was to clarify the effect of hematuria on histological findings, response to steroid treatment, and the outcome in IgA nephropathy. PATIENTS AND METHODS: Seventy-five patients with IgAN and proteinuria > 1 g/day and treated with prednisolone were divided into two groups: those with low (≤20/high-power field [HPF]) urinary red blood cell (U-RBC) counts (L-RBC group, n=55) and those with high (>20/HPF) U-RBC counts (H-RBC group, n=20)...
April 2016: Journal of Nephropathology
Irfan Moinuddin, Machaiah Madhrira, Erika Bracamonte, Bijin Thajudeen, Amy Sussman
ANCA-associated vasculitis (AAV) is the most common cause of crescentic rapidly progressive glomerulonephritis (GN). Levamisole used as an adulterant in cocaine is increasingly recognized as a cause of AAV. We report the case of a 50 year old woman with atypical anti-MPO AAV associated with cocaine use and exposure to levamisole. In addition to the clinical and pathologic findings of crescentic GN, the patient also had biopsy evidence of secondary membranous nephropathy (MN). Although AAV and MN have been reported previously in the same patient and both have been induced by drug exposures, this is the first report of MN in a patient with AAV likely induced by levamisole...
July 2016: Pathology, Research and Practice
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