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https://www.readbyqxmd.com/read/29341230/successful-treatment-of-rituximab-and-steroid-resistant-nephrotic-syndrome-with-leukocytapheresis
#1
Maiko Takakura, Masaki Shimizu, Mao Mizuta, Natsumi Inoue, Yuko Tasaki, Kazuhide Ohta, Kengo Furuichi, Takashi Wada, Akihiro Yachie
Although rituximab (RTX) is a promising therapeutic agent for treating steroid-resistant nephrotic syndrome (SRNS) resistant to various immunosuppressive agents, some patients have shown resistance to RTX. We report the case of a patient with RTX-resistant nephrotic syndrome and SRNS who was successfully treated with leukocytapheresis (LCAP). After LCAP, there was a significant reduction in proteinuria and in the total number of lymphocytes, T cells, and HLA-DR+- activated T cells. Moreover, the patient became sensitive to steroids and RTX...
January 16, 2018: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29340328/nephrin-loss-can-be-used-to-predict-remission-and-long-term-renal-outcome-in%C3%A2-patients-with-minimal-change-disease
#2
Nina A van de Lest, Malu Zandbergen, Daphne H T IJpelaar, Ron Wolterbeek, Jan A Bruijn, Ingeborg M Bajema, Marion Scharpfenecker
Introduction: Minimal change disease is a common cause of nephrotic syndrome. In general, patients with minimal change disease respond to corticosteroids and have excellent long-term renal survival. However, some patients have less favorable outcome. These patients are often thought to have progressed to focal segmental glomerulosclerosis. We previously reported that a segmental loss of podocyte markers is present before the development of focal segmental glomerulosclerosis in a rat model...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29340324/temporal-association-between-pla2r-antibodies-and-clinical-outcomes-in-primary-membranous-nephropathy
#3
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
https://www.readbyqxmd.com/read/29340321/randomized-clinical-trial-design-to-assess-abatacept-in-resistant-nephrotic-syndrome
#4
Howard Trachtman, Debbie S Gipson, Michael Somers, Cathie Spino, Sharon Adler, Lawrence Holzman, Jeffrey B Kopp, John Sedor, Sandra Overfield, Ayanbola Elegbe, Michael Maldonado, Anna Greka
Introduction: Treatment-resistant nephrotic syndrome is a rare form of glomerular disease that occurs in children and adults. No Food and Drug Administration-approved treatments consistently achieve remission of proteinuria and preservation of kidney function. CD80 (B7-1) can be expressed on injured podocytes, and administration of abatacept (modified CTLA4-Ig based on a natural ligand to CD80) has been associated with sustained normalization of urinary protein excretion and maintenance of glomerular filtration rate in experimental and clinical settings...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29340242/a-diagnostic-model-for-minimal-change-disease-based-on-biological-parameters
#5
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
https://www.readbyqxmd.com/read/29340080/inhibition-of-tumor-necrosis-factor-signaling-attenuates-renal-immune-cell-infiltration-in-experimental-membranous-nephropathy
#6
Yen-Sung Huang, Shin-Huei Fu, Kuo-Cheng Lu, Jin-Shuen Chen, Hsin-Yi Hsieh, Huey-Kang Sytwu, Chia-Chao Wu
Idiopathic membranous nephropathy (MN) is an autoimmune-mediated glomerulonephritis and the most common cause of idiopathic nephrotic syndrome in adult humans. A tumor necrosis factor α (TNF-α)-mediated inflammatory response via TNF receptor 1 (TNFR1) and TNFR2 has been proposed as a pathogenic factor. In this study, we assessed the therapeutic response to blocking TNF signaling in experimental MN. Murine MN was induced experimentally by cationic bovine serum albumin (cBSA); phosphate-buffered saline was used in control mice...
December 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/29333833/-infrequent-mutation-in-renal-coloboma-syndrome-case-report-and-review
#7
Ignacio Ruiz Del Olmo Izuzquiza, Yolanda Romero Salas, Ana Rodríguez Valle, Inmaculada González Viejo, María L Justa Roldán
Renal-coloboma syndrome is an autosomal dominant disease characterized by renal hypodysplasia and coloboma. A case of a 12-year-old girl with chronic kidney disease, bilateral optic nerve colobomas and an exceptional PAX-2 gene mutation is presented. Diagnosed in prenatal scans with bilateral renal hypoplasia, she presented clinical and laboratory findings of chronic kidney disease at 5 days of life. Following tests showed grade II bilateral vesicoureteral reflux spontaneously solved, maintained non nephrotic proteinuria controlled with enalapril and bilateral colobomas with left macular atrophy...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29327821/chronic-kidney-disease-in-patients-with-chronic-hepatitis-c-virus-infection
#8
Omer Shahab, Pegah Golabi, Zobair M Younossi
Hepatitis C virus (HCV) infection affects many organs in the body, including the liver, kidneys, skin, joints and others. Although the hepatic manifestation of HCV has been widely studied, the extrahepatic manifestaions of HCV have not been fully appreciated. Studies have shown that patients with HCV have a higher risk of chronic kidney disease and end-stage renal disease, as well as poorer outcomes after kidney transplantation. Given these findings, it is important to screen HCV patients for presence of renal impairement in a timely manner...
January 10, 2018: Minerva Gastroenterologica e Dietologica
https://www.readbyqxmd.com/read/29325951/shunt-nephritis-an-increasingly-unfamiliar-diagnosis
#9
Tessa A Harland, Ken R Winston, Anna J Jovanovich, Richard J Johnson
BACKGROUND: Shunt nephritis is a rare, reversible immune-complex mediated complication of CSF shunt infection that can progress to end-stage renal disease and even death if diagnosis is delayed. CASE DESCRIPTION: The present case report details the manifestation and clinical course of shunt nephritis in a 50-year old patient who presented with symptoms of nephrotic syndrome 30 years after ventriculo-jugular shunt placement. Diagnosis was delayed due to initial negative CSF and blood cultures but later a CSF culture was positive for Propionibacterium acnes...
January 8, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29324775/the-optimal-threshold-of-serum-ceruloplasmin-in-the-diagnosis-of-wilson-s-disease-a-large-hospital-based-study
#10
Rong Xu, Yong-Fang Jiang, Yong-Hong Zhang, Xu Yang
BACKGROUND AND AIMS: A ceruloplasmin (CP) concentration <200 mg/L is conventionally considered as one of the major diagnostic criteria for Wilson's disease (WD). However, the diagnostic accuracy of this threshold has never been investigated in a sufficiently large group of patients. This study aims to present the results of serum CP measurements in various patients and to identify the optimal cutoff value of CP for the diagnosis of WD. MATERIALS AND METHODS: We identified patients whose CP levels were evaluated from January 1, 2016 to December 31, 2016 using a laboratory information database...
2018: PloS One
https://www.readbyqxmd.com/read/29321440/an-overview-of-kidney-disease-following-hematopoietic-cell-transplantation
#11
Minoru Ando
Hematopoietic stem cell transplantation (SCT) recipients are exposed to a large amount of anti-cancer drugs, immunosuppressors, and irradiation during the peri-SCT period. Thus, they have to overcome serious adverse events related to unavoidable but toxic procedures, including organ disorders. In particular, acute kidney injury (AKI) is one of the most critical complications, because it influences the mortality of patients. A few patients who survive AKI may develop nephrotic syndrome, and precedent AKI is also closely associated with chronic and progressive loss of the renal function in post-SCT patients...
January 11, 2018: Internal Medicine
https://www.readbyqxmd.com/read/29321142/differentiating-primary-genetic-and-secondary-fsgs-in-adults-a-clinicopathologic-approach
#12
An S De Vriese, Sanjeev Sethi, Karl A Nath, Richard J Glassock, Fernando C Fervenza
FSGS describes a renal histologic lesion with diverse causes and pathogenicities that are linked by podocyte injury and depletion. Subclasses of FSGS include primary, genetic, and secondary forms, the latter comprising maladaptive, viral, and drug-induced FSGS. Despite sharing certain clinical and histologic features, these subclasses differ noticeably in management and prognosis. Without an accepted nongenetic biomarker that discriminates among these FSGS types, classification of patients is often challenging...
January 10, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29320993/distribution-of-glomerular-diseases-in-taiwan-preliminary-report-of-national-renal-biopsy-registry-publication-on-behalf-of-taiwan-society-of-nephrology
#13
Hsien-Fu Chiu, Hung-Chun Chen, Kuo-Cheng Lu, Kuo-Hsiung Shu
BACKGROUND: Despite the development of biomarkers and noninvasive imaging tools, biopsy remains the only method for correctly diagnosing patients with unexplained hematuria, proteinuria and renal failure. Renal biopsy has been performed for several decades in Taiwan; however, a national data registry is still lacking until 2013. METHODS: The Renal Biopsy Registry Committee was established within the Taiwan Society of Nephrology in January 2013. A biopsy registry format, including basic demographic data, baseline clinical features, laboratory data, and clinical and pathological diagnosis was developed...
January 10, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29319779/recovery-of-renal-function-after-bilateral-renal-vein-thrombo-sis-episode-as-complication-of-membranous-glomerulopa-thy-case-report
#14
Ana Larissa Pedrosa Ximenes, Elizabeth De Francesco Daher, Pedro Duarte Barreto Castillo, Francisco Eduardo Siqueira da Rocha, Camila Freire Salem de Miranda, Flavio Bezerra de Araujo
Renal vein thrombosis (RVT) is a complication often associated with nephrotic syndrome. It occurs due to a state of hypercoagulability common in the diseases that attend to this syndromic diagnosis. It should be suspected whenever there is nephrotic syndrome associated with sudden flank pain, hematuria and worsening of proteinuria. Bilateral RVT also presents with frequently oliguric renal dysfunction. This case reports a 33-year-old patient hospitalized for a nephrotic syndrome, with etiologic investigation suggestive of primary membranous glomerulopathy, which evolved with bilateral RVT associated with deterioration of renal function and need for renal replacement therapy...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29319762/complement-system-and-c4d-expression-in-cases-of-membranous-nephropathy
#15
Fabiano Bichuette Custódio, Crislaine Aparecida da Silva, Fernanda Rodrigues Helmo, Juliana Reis Machado, Marlene Antônia Dos Reis
INTRODUCTION: Membranous nephropathy (MN) is one of the major causes of nephrotic syndrome. The complement system plays a key role in the pathophysiology of MN. OBJECTIVES: To identify the complement pathway possibly activated in MN cases and correlate the presence of C4d with more severe clinical and histological markers. METHODS: Sixty nine cases from renal biopsy with membranous nephropathy were investigated. The presence of C1q was analyzed by direct immunofluorescence; and expression of C4d by immunohistochemistry...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29310419/proliferative-lupus-nephritis-in-the-absence-of-overt-systemic-lupus-erythematosus-a-historical-study-of-12-adult-patients
#16
Maxime Touzot, Cécile Saint-Pastou Terrier, Stanislas Faguer, Ingrid Masson, Hélène François, Lionel Couzi, Aurélie Hummel, Nathalie Quellard, Guy Touchard, Noémie Jourde-Chiche, Jean-Michel Goujon, Eric Daugas
Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29310412/kimura-disease-of-buccal-region-in-a-pediatric-patient-with-nephrotic-syndrome-a-case-report
#17
Dong Hoon Lee, Ga-Eon Kim, Eunmi Yang, Tae Mi Yoon, Joon Kyoo Lee, Sang Chul Lim
RATIONALE: Kimura disease is a rare benign, chronic inflammatory disorder that typically presents with slowly enlarging, nontender, subcutaneous swellings in the head and neck region. The occurrence of Kimura disease in the oral cavity is extremely rare. PATIENT CONCERNS: A 16-year-old boy presented with a complaint of a right painless buccal mass of 3 years' duration. DIAGNOSIS: The patient had been diagnosed with nephrotic syndrome and treated with corticosteroid at the age of 5 years...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29305810/serum-sickness-with-refractory-nephrotic-syndrome-following-treatment-with-rituximab
#18
Ryo Maeda, Yukihiko Kawasaki, Shinichiro Ohara, Kazuhide Suyama, Mitsuaki Hosoya
Rituximab (RTX) is effective for treating childhood refractory nephrotic syndrome (NS), such as steroid-dependent (SD), frequently relapsing (FR), and steroid-resistant (SR) NS. While RTX has been proven to be effective in treating SDNS, FRNS, and SRNS, it may cause serum sickness, a rare illness characterized by fever, rash, and arthralgia, 10-14 days after primary antigen exposure or within a few days after secondary antigen exposure, by producing human anti-chimeric antibodies (HACAs). A 17-year-old girl with refractory SDNS treated with RTX and oral cyclosporine A was admitted with fever and arthralgia 10 days after the fifth RTX dose was administered...
January 5, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29301131/safety-of-outpatient-kidney-biopsies
#19
Bojana Šimunov, Mihaela Gunjača, Branislav Čingel, Dinko Škegro, Mladen Knotek
BACKGROUND: Kidney biopsy is frequently performed in our centre as an outpatient procedure. The aim of this study was to evaluate the safety of biopsy in the outpatient setting. METHODS: We analysed kidney biopsies performed from March 2013 to February 2017. Seven hundred twenty-five biopsies performed in the outpatient setting were identified: There were 592 transplant and 133 native biopsies including 3 solitary kidney biopsies. All were performed under ultrasound guidance using a 16G or 18G needle, with freehand technique...
January 4, 2018: Nephron
https://www.readbyqxmd.com/read/29299849/lipid-management-in-chronic-kidney-disease-systematic-review-of-pcsk9-targeting
#20
REVIEW
BinBin Zheng-Lin, Alberto Ortiz
Cardiovascular disease is the leading cause of death in patients with chronic kidney disease (CKD) and CKD is considered a coronary artery disease risk equivalent. So far, statins have been the mainstay of primary and secondary prevention of cardiovascular disease in the general population. However, their benefit on outcomes is limited and controversial in CKD patients and new therapeutic approaches to reduce cardiovascular risk are needed. Monoclonal antibodies targeting proprotein convertase subtilisin/kexin 9 (PCSK9) reduce low-density lipoprotein cholesterol (LDL-C) and lipoprotein(a) in high-risk populations and cardiovascular events in secondary prevention...
January 3, 2018: Drugs
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