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Rossana Malatesta-Muncher, Karen W Eldin, Laurence H Beck, Mini Michael
BACKGROUND: Idiopathic membranous nephropathy is an uncommon cause of nephrotic syndrome in children and can present treatment challenges. The current treatment options of steroids and cyclophosphamide, cyclosporine, or mycophenolate require prolonged treatment durations and the associated side effects may result in nonadherence in children, especially in adolescents. CASE-DIAGNOSIS: We report two adolescent patients with idiopathic membranous nephropathy with nephrotic range proteinuria and elevated anti-phospholipase A2 receptor levels who did not achieve remission with steroids and were later treated with rituximab...
March 15, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Yu Wang, Yi-Xin He, Tian-Tian Diao, Shi-Yao Wei, Wen-Rui Qi, Cen-Cen Wang, Shu-Min Song, Min Bi, Chun-Mei Li, Cai-Xia Zhang, Yan-Pei Hou, Qiu-Ju Wei, Bing Li
Since urine samples more directly reflect kidney alterations and damage than blood samples, we investigated whether urine anti-PLA2 R antibody (uPLA2 R-Ab) could be utilized similarly to serum anti-PLA2 R antibody (sPLA2 R-Ab) as a noninvasive biomarker of idiopathic membranous nephropathy (IMN). In this study, we performed a qualitative analysis using an indirect immunofluorescence test (IIFT) and measured uPLA2 R-Ab and sPLA2 R-Ab concentrations using an enzyme-linked immunosorbent assay (ELISA) in 28 patients with biopsy-proven IMN and 12 patients with secondary membranous nephropathy (SMN)...
January 2, 2018: Oncotarget
Hanyu Zhu, Qiuxia Han, Dong Zhang, Yong Wang, Jing Gao, Xiaoli Yang, Wenjia Geng, Xiangmei Chen
Background: Membranous nephropathy (MN) represents a distinct glomerular disease which has been considered as a major cause of nephrotic syndrome (NS) in adults. Evidences show that the clinicopathological features of MN are various among MN cases. This study aimed to summarize and analyze the clinicopathological features of patients with MN. Methods: A total of 231 MN patients were recruited in this study. Their clinical and pathological features were collected and analyzed according to their age, gender, pathological stages, and anti-phospholipase A2 receptor (anti-PLA2R) antibodies tests...
2018: International Journal of Nephrology and Renovascular Disease
Andrew S Bomback
No abstract text is available yet for this article.
January 29, 2018: Clinical Journal of the American Society of Nephrology: CJASN
Eiichiro Uchino, Daisuke Takada, Haruta Mogami, Takeshi Matsubara, Tatsuo Tsukamoto, Motoko Yanagita
Pregnancy and membranous nephropathy (MN) can occur concurrently with nephrotic syndrome. However, the pathophysiology of MN associated with pregnancy remains unclear, including the involvement of anti-M-type phospholipase A2 receptor (PLA2R) antibody, the major antigen of idiopathic MN (iMN). A treatment for the condition is also not established. We present the case of a 43-year-old pregnant female with incidental proteinuria and hypoalbuminemia. We made a diagnosis of nephrotic syndrome at 11 week gestation...
January 18, 2018: CEN Case Reports
R Ramachandran, A K Yadav, V Kumar, N Inamdar, R Nada, K L Gupta, V Jha
Introduction: Autoantibodies to M-type phospholipase A2 receptor (aPLA2R) are seen in two-thirds of patients with primary membranous nephropathy (PMN) and are associated with disease activity. However, the precise temporal dynamics between the presence and amount of aPLA2R in circulation, as well as the clinical activity, are not known. We evaluated the temporal association between disease activity and serum aPLA2R during and after treatment in PMN. Methods: The study included all patients with PMN and elevated aPLA2R who were started on immunosuppressive therapy for persistent nephrotic syndrome at a single center between December 2014 and December 2015...
January 2018: KI Reports
Hanyu Zhu, Qiuxia Han, Dong Zhang, Yong Wang, Jing Gao, Wenjia Geng, Xiaoli Yang, Xiangmei Chen
Background: Minimal change disease (MCD) is a kind of nephrotic syndrome (NS). In this study, we aimed to establish a mathematical diagnostic model based on biological parameters to classify MCD. Methods: A total of 798 NS patients were divided into MCD group and control group. The comparison of biological indicators between two groups were performed with t -tests. Logistic regression was used to establish the diagnostic model, and the diagnostic value of the model was estimated using receiver operating characteristic (ROC) analysis...
2018: PeerJ
Xin Wang, Zhao Cui, Yi-Miao Zhang, Zhen Qu, Fang Wang, Li-Qiang Meng, Xu-Yang Cheng, Gang Liu, Fu-de Zhou, Ming-Hui Zhao
Background: Rituximab had been shown to be effective in inducing remission of nephrotic syndrome in patients with idiopathic membranous nephropathy (iMN). This study applied rituximab therapy for 36 non-responsive iMN patients to investigate its effects and safety. Methods: Thirty-six iMN patients who were non-responsive to prior immunosuppression were enrolled. Rituximab was used for B-cell depletion in patients, with a goal of <5 B cells/mm3 in the circulation...
November 14, 2017: Nephrology, Dialysis, Transplantation
Yipeng Liu, Xuan Li, Chaoqun Ma, Ping Wang, Ju Liu, Hong Su, Hao Zhuo, Xianglei Kong, Dayu Xu, Dongmei Xu
BACKGROUND: The M-type phospholipase A2 receptor (PLA2R) is a specific target autoantigen identified in idiopathic membranous nephropathy (IMN). The autoantibody against PLA2R (anti-PLA2R) may be used to diagnose IMN. However, the appropriate diagnosis cut-off value for Chinese patients with IMN has not been established. METHODS: In total, 119 patients who underwent renal biopsy (57 patients with IMN and 62 patients with non-IMN glomerulonephritis) and 22 healthy individuals were recruited for our observation study from Qianfoshan Hospital between September 2011 and March 2016...
January 2018: Clinica Chimica Acta; International Journal of Clinical Chemistry
A Radice, F Pieruzzi, B Trezzi, G Ghiggeri, P Napodano, M D'Amico, T Stellato, R Brugnano, F Ravera, D Rolla, G Pesce, M E Giovenzana, F Londrino, V Cantaluppi, F Pregnolato, A Volpi, G Rombolà, G Moroni, G Ortisi, Renato A Sinico
Autoantibody against phospholipase A2 receptor (anti-PLA2R) is a sensitive and specific biomarker of idiopathic membranous nephropathy (iMN), being found in approximately 70% of iMN patients and only occasionally in other glomerular diseases. However, whereas its diagnostic specificity vs. normal controls and other glomerulonephritides (GN) has been firmly established, its specificity vs. membranous nephropathy associated with various diseases (sMN) has given inconsistent results. The aim of our study was to evaluate the prevalence of anti-PLA2R antibodies in iMN in comparison with various control groups, including sMN...
April 2018: Journal of Nephrology
Fujun Lin, Dan Zhang, Juan Chang, Xuanli Tang, Wenbin Guan, Gengru Jiang, Chun Zhu, Fan Bian
Target antigens in idiopathic membranous nephropathy (MN) include the phospholipase A2 receptor (PLA2R), and in some cases, the thrombospondin type 1 domain-containing 7A (THSD7A). A notable phenomenon is the high rate of cancer (reported to be as high as 20%) in patients with THSD7A-associated MN. Neurofibromatosis type 1 (NF1) is an autosomal dominant disease caused by NF1 gene mutation, and clinically characterized by multiple cutaneous neurofibromas and café-au-lait spots. In this article, we report a patient with NF1 who developed THSD7A-associated MN when the NF1 skin lesions deteriorated...
October 24, 2017: European Journal of Medical Genetics
Yue Dong, Longxing Cao, Hua Tang, Xiangyi Shi, Yongning He
M-type phospholipase A2 receptor (M-PLA2R) is a member of the mannose receptor family and known as the receptor of secretory phospholipase A2s. It has also been identified as the major autoantigen of idiopathic membranous nephropathy, one of the most common causes for nephrotic syndrome in adults. Here we determine the structure of human M-PLA2R ectodomain by cryo-electron microscopy. The results show that the ectodomain has high internal flexibility and forms a compact dual-ring-shaped conformation at acidic pH and adopts extended conformations at basic pH...
December 8, 2017: Journal of Molecular Biology
Norifumi Hayashi, Keiichirou Okada, Yuki Matsui, Keiji Fujimoto, Hiroki Adachi, Hideki Yamaya, Misao Matsushita, Hitoshi Yokoyama
Background: The M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A (THSD7A) were identified as intrinsic antigens in primary membranous nephropathy (MN). Complement activation via the lectin pathway in intrinsic antigen-related MN is still unclear. Methods: We retrospectively enrolled 60 primary Japanese MN patients and detected activated complement pathways by staining complement proteins in glomerular deposition. According to the findings of PLA2R and THSD7A staining in glomeruli, they were classified into intrinsic antigen-related or -unrelated MN...
July 18, 2017: Nephrology, Dialysis, Transplantation
Qiuhua Zhang, Biao Huang, Xiaobin Liu, Bin Liu, Yi Zhang, Zhijian Zhang, Jia Hua, Yun Fan, Ling Hu, Meijuan Meng, Mian Wu, Liang Wang, Zhigang Hu, Zhuxing Sun
Anti-phospholipase A2 receptor antibody (PLA2R-Ab) is useful for affirming the diagnosis of idiopathic membranous nephropathy (IMN). Time-resolved fluoroimmunoassay (TRFIA) is highly sensitive and quantitative for measuring serum PLA2R-Ab immunoglobulin (IgG). We measured PLA2R-Ab levels with TRFIA in sera from 172 patients with IMN (n = 69), secondary MN (n = 9), and those with other glomerulonephritis (n = 94) at the time of renal biopsy compared to healthy controls (n = 286). Serum anti-PLA2R-IgG levels in healthy volunteers ranged from 0...
September 21, 2017: Scientific Reports
Andreas Kronbichler, Jun Oh, Björn Meijers, Gert Mayer, Jae Il Shin
Primary membranous nephropathy (MN) is the leading cause of nephrotic syndrome in adults. Discovery of several antibodies has contributed to an increased understanding of MN. Antibodies against the M-type phospholipase A2 receptor (PLA2R) are present in 50-100% with primary MN and are associated with a lower frequency of spontaneous remission. High levels are linked with a higher probability of treatment resistance, higher proteinuria, and impaired renal function, as well as a more rapid decline of kidney function during follow-up...
2017: BioMed Research International
A Gudipati, M S Uppin, R K Kalidindi, G Swarnalatha, U Das, G Taduri, S B Raju, L Rajasekhar, Aruna K Prayaga
Membranous nephropathy (MN) is one of the common cause of nephrotic syndrome. The discrimination between primary MN (iMN) and secondary MN is essential because of treatment implications. Immunohistochemical (IHC) evaluation with the help of anti-phospholipase A2 receptor (PLA2R) antibody helps in tissue evaluation of iMN, which is an easy, cost-effective, and pathologist-friendly technique. The study included 82 cases of MN over a period of 3 years. IHC using PLA2R antibody was performed on iMN and secondary cases with adequate tissue...
September 2017: Indian Journal of Nephrology
Shiva Kalantari, Mohsen Nafar
Membranous nephropathy (MN) is relatively major cause of nephrotic syndrome in adults which is recognized as an organ-specific autoimmune disease. The etiology of most cases is idiopathic, whereas the secondary MN is caused by systemic autoimmune diseases, infections, medications and malignancies. The idiopathic disease is developed by the formation of sub-epithelial immune complex deposits most likely due to binding the circulating auto-antibodies to intrinsic antigen on podocytes. The major auto antibody is the anti-phospholipase A2 receptor (anti-PLA2R), however, it is not enough sensitive...
September 11, 2017: Biomarkers in Medicine
Andrea Angioi, Nicola Lepori, Ana Coloma López, Sanjeev Sethi, Fernando C Fervenza, Antonello Pani
In the last 10 years, basic science and clinical research have made important contributions to the understanding and management of primary membranous nephropathy (MN). The identification of antibodies directed against the M-type phospholipase A2 receptor (PLA2R) and thrombospondin type-1 domain-containing 7A protein have added a new perspective on diagnosis, monitoring the immunological activity, predicting prognosis and guiding therapy in patients with primary MN. Mounting evidence suggests that quantification and follow-up of antiPLA2R Abs levels can help in assessing prognosis and evaluate the response to treatment...
September 5, 2017: Journal of Nephrology
Jia Wang, Zhao Cui, Jie Lu, Christian Probst, Yi-Miao Zhang, Xin Wang, Zhen Qu, Fang Wang, Li-Qiang Meng, Xu-Yang Cheng, Gang Liu, Hanna Debiec, Pierre Ronco, Ming-Hui Zhao
BACKGROUND AND OBJECTIVES: Thrombospondin type-I domain-containing 7A (THSD7A) was recently identified as the target antigen in about 10% of patients with M-type phospholipase A2 receptor (PLA2R)-negative membranous nephropathy in European and North American populations. The prevalence of THSD7A in other populations and their clinical associations deserve further clarification. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Immunofluorescence assay was performed to investigate anti-THSD7A antibodies in 578 consecutive patients with biopsy-proven idiopathic membranous nephropathy, 114 patients with secondary membranous nephropathy, 64 disease controls, and 20 healthy controls...
October 6, 2017: Clinical Journal of the American Society of Nephrology: CJASN
Kristine Lindhard, Martin Egfjord, Otto Clemmesen
Ascites is common in patients with liver cirrhosis. It may present as a clinical manifestation in nephrotic syndrome in adults, typically with heart- or liver disease together with other oedema. We describe a 64-year-old male patient - with no liver or heart disease - with relapsing ascites and no other oedema, who was surprisingly diagnosed with primary membranous nephropathy (MN), and the autoantibody anti-PLA2R was positive. Through immunotherapy the ascites disappeared. Anti-PLA2R and anti-TSHD7A can be used in the diagnosis (primary/secondary MN) and may play a role in the treatment and prognosis...
July 24, 2017: Ugeskrift for Laeger
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