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Glomerular basement membrane

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https://www.readbyqxmd.com/read/29208058/low-protein-diet-supplemented-with-ketoacids-delays-the-progression-of-diabetic-nephropathy-by-inhibiting-oxidative-stress-in-the-kkay-mice-model
#1
Dongmei Liu, Ming Wu, Lin Li, Xiang Gao, Bo Yang, Shuqin Mei, Lili Fu, Changlin Mei
Diabetic nephropathy (DN) is a major cause of chronic kidney disease. We aimed to investigate the effect of the low-protein diets (LPD) supplemented with ketoacids (LPD+KA) in KKAy mice, an early type 2 DN model. KKAy mice were treated with normal protein diet (NPD), LPD or LPD+KA from 12 to 24 weeks of age. A period of 12-week treatment with LPD significantly reduced albuminuria as compared with that observed after NPD treatment. Treatment with LPD+KA further reduced albuminuria as compared with that observed with LPD treatment alone...
December 6, 2017: British Journal of Nutrition
https://www.readbyqxmd.com/read/29207131/lysyl-oxidases-expression-and-histopathological-changes-of-the-diabetic-rat-nephron
#2
Jun Chen, Jie Ren, Wings T Y Loo, Liang Hao, Min Wang
Diabetic nephropathy (DN) is a major complication of diabetes, the accumulation of extracellular matrix (ECM) is considered an indication of nephropathological changes. Lysyl oxidases (LOXs) are also associated with ECM. However, the majority of studies on LOXs have focused on their potential role in renal fibrogenesis and there has no examination of LOXs expression or the correlation with histopathological changes of DN, including glomerular basement membrane (GBM) thickening and glomerulosclerosis. In this study, the association between histological changes and LOXs was explored using a type 2 diabetes model of male Zucker diabetic fatty rats...
November 29, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29198685/thin-glomerular-basement-membrane-in-a-kidney-transplant-of-an-alport-s-syndrome-patient-a-case-report
#3
S Santos, S Marques, T Golper, A Langone, A B Fogo
Alport syndrome (AS) and thin basement membrane lesions are caused by various mutations in type IV collagen genes. Although AS is considered a rare disease, thin basement membrane is a frequent pattern, especially in families with a history of persistent hematuria. We report a patient with a diagnosis of AS who developed end-stage kidney disease (ESKD) and received a kidney transplant from a living unrelated donor. The graft biopsy specimen surprisingly showed a pattern of thin basement membranes.
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29196624/rna-seq-of-serial-kidney-biopsies-obtained-during-progression-of-chronic-kidney-disease-from-dogs-with-x-linked-hereditary-nephropathy
#4
Candice P Chu, Jessica A Hokamp, Rachel E Cianciolo, Alan R Dabney, Candice Brinkmeyer-Langford, George E Lees, Mary B Nabity
Dogs with X-linked hereditary nephropathy (XLHN) have a glomerular basement membrane defect that leads to progressive juvenile-onset renal failure. Their disease is analogous to Alport syndrome in humans, and they also serve as a good model of progressive chronic kidney disease (CKD). However, the gene expression profile that affects progression in this disease has only been partially characterized. To help fill this gap, we used RNA sequencing to identify differentially expressed genes (DEGs), over-represented pathways, and upstream regulators that contribute to kidney disease progression...
December 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29191133/focal-segmental-glomerulosclerosis-in-related-miniature-schnauzer-dogs
#5
Wilson Yau, Lisa Mausbach, Meryl P Littman, Rachel E Cianciolo, Cathy A Brown
Focal segmental glomerulosclerosis (FSGS) recently has been recognized as a common cause of proteinuria in dogs in general, and in Miniature Schnauzer dogs in particular. This study describes the morphologic features present in the kidneys of 8 related proteinuric Miniature Schnauzer dogs. The FSGS, characterized by solidification of portions of the capillary tuft, affected 32% to 49% of examined glomeruli in these dogs. Synechiae, often accompanied by hyalinosis, were present in 13% to 54% of glomeruli and were more prevalent in older dogs...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/29190833/lupus-nephritis-progression-in-fc%C3%AE-riib-yaa-mice-is-associated-with-early-development-of-glomerular-electron-dense-deposits-and-loss-of-renal-dnase-i-in-severe-disease
#6
Kjersti Daae Horvei, Hege Lynum Pedersen, Silje Fismen, Dhivya Thiyagarajan, Andrea Schneider, Ole Petter Rekvig, Thomas H Winkler, Natalya Seredkina
FcγRIIB-/-yaa mice develop severe lupus glomerulonephritis due to lack of an inhibitory immune cell receptor combined with a Y-chromosome linked autoimmune accelerator mutation. In the present study, we have investigated nephritis development and progression in FcγRIIB-/-yaa mice to find shared features with NZB/NZW F1 lupus prone mice and human disease. We sacrificed 25 male FcγRIIB-/-yaa mice at various disease stages, and grouped them according to activity and chronicity indices for lupus nephritis. Glomerular morphology and localization of electron dense deposits containing IgG were further determined by immune electron microscopy...
2017: PloS One
https://www.readbyqxmd.com/read/29169037/a-nephron-model-for-study-of-drug-induced-acute-kidney-injury-and-assessment-of-drug-induced-nephrotoxicity
#7
Yueyang Qu, Fan An, Yong Luo, Yao Lu, Tingjiao Liu, Weijie Zhao, Bingcheng Lin
In this study, we developed a multilayer microfluidic device to simulate nephron, which was formed by "glomerulus", "Bowman's capsule", "proximal tubular lumen" and "peritubular capillary". In this microdevice, artificial renal blood flow was circulating and glomerular filtrate flow was single passing through, mimicking the behavior of a nephron. In this dynamic artificial nephron, we observed typical renal physiology, including the glomerular size-selective barrier, glomerular basement membrane charge-selective barrier, glucose reabsorption and para-aminohippuric acid secretion...
November 14, 2017: Biomaterials
https://www.readbyqxmd.com/read/29167507/characterization-of-the-early-molecular-changes-in-the-glomeruli-of-cd151-mice-highlights-induction-of-mindin-and-mmp-10
#8
Crystal Naudin, Brian Smith, Danielle R Bond, Matthew D Dun, Rodney J Scott, Leonie K Ashman, Judith Weidenhofer, Séverine Roselli
In humans and FVB/N mice, loss of functional tetraspanin CD151 is associated with glomerular disease characterised by early onset proteinuria and ultrastructural thickening and splitting of the glomerular basement membrane (GBM). To gain insight into the molecular mechanisms associated with disease development, we characterised the glomerular gene expression profile at an early stage of disease progression in FVB/N Cd151 -/- mice compared to Cd151 +/+ controls. This study identified 72 up-regulated and 183 down-regulated genes in FVB/N Cd151 -/- compared to Cd151 +/+ glomeruli (p < 0...
November 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29163933/a-novel-glomerular-basement-membrane-segmentation-using-neutrsophic-set-and-shearlet-transform-on-microscopic-images
#9
Yanhui Guo, Amira S Ashour, Baiqing Sun
Purpose: Glomerular basement membrane segmentation is an ultimate step in several image processing applications for kidney diseases and abnormalities in microscopic images. However, extracting the glomerular basement membrane (GBM) regions accurately is considered challenging because of the large variants in the microscopic images. The contribution of this work is to propose a computer-aided detection system to provide accurate GBM segmentation. Methods: A novel GBM segmentation algorithm is developed based on neutrsophic set and shearlet transform...
December 2017: Health Information Science and Systems
https://www.readbyqxmd.com/read/29162595/predicting-outcome-in-patients-with-anti-gbm-glomerulonephritis
#10
Emma E van Daalen, J Charles Jennette, Stephen P McAdoo, Charles D Pusey, Marco A Alba, Caroline J Poulton, Ron Wolterbeek, Tri Q Nguyen, Roel Goldschmeding, Bassam Alchi, Meryl Griffiths, Janak R de Zoysa, Beula Vincent, Jan A Bruijn, Ingeborg M Bajema
BACKGROUND AND OBJECTIVES: Large studies on long-term kidney outcome in patients with anti-glomerular basement membrane (anti-GBM) GN are lacking. This study aimed to identify clinical and histopathologic parameters that predict kidney outcome in these patients. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective analysis included a total of 123 patients with anti-GBM GN between 1986 and 2015 from six centers worldwide. Their kidney biopsy samples were classified according to the histopathologic classification for ANCA-associated GN...
November 21, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29142973/renal-amyloidosis-associated-with-5-novel%C3%A2-variants-in-the-fibrinogen-a-alpha-chain-protein
#11
Dorota Rowczenio, Maria Stensland, Gustavo A de Souza, Erik H Strøm, Janet A Gilbertson, Graham Taylor, Nigel Rendell, Shane Minogue, Yvonne A Efebera, Helen J Lachmann, Ashutosh D Wechalekar, Philip N Hawkins, Ketil R Heimdal, Kristian Selvig, Inger K Lægreid, Nathalie Demoulin, Selda Aydin, Julian D Gillmore, Tale N Wien
Introduction: Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK. Patients typically present with kidney impairment and progress to end-stage renal disease over a median time of 4.6 years. Methods: Six patients presented with proteinuria, hypertension, and/or lower limb edema and underwent detailed clinical and laboratory investigations...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29142939/negative-staining-for-col4a5-correlates-with-worse-prognosis-and-more-severe-ultrastructural-alterations-in-males-with-alport-syndrome
#12
Samar M Said, Mary E Fidler, Anthony M Valeri, Brooke McCann, Wade Fiedler, Lynn D Cornell, Mariam Priya Alexander, Ahmed M Alkhunaizi, Anne Sullivan, Carl H Cramer, Marie C Hogan, Samih H Nasr
Introduction: Alport syndrome (AS) is a genetic disorder characterized by progressive hematuric nephropathy with or without sensorineural hearing loss and ocular lesions. Previous studies on AS included mostly children. Methods: To determine the prognostic value of loss of staining for collagen type IV alpha 5 (COL4A5) and its relationship with the ultrastructural glomerular basement membrane alterations, we performed direct immunofluorescence using a mixture of fluorescein isothiocyanate-conjugated and Texas-red conjugated antibodies against COL4A5 and COL4A2, respectively, on renal biopsies of 25 males with AS (including 16 who were diagnosed in adulthood)...
January 2017: KI Reports
https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#13
Yongzhen Li, Ying Wang, Qingnan He, Xiqiang Dang, Yan Cao, Xiaochuan Wu, Shuanghong Mo, Xiaoxie He, Zhuwen Yi
Focal segmental glomerulosclerosis (FSGS) is a pathological lesion rather than a disease, with a diverse etiology. FSGS may result from genetic and non‑genetic factors. FSGS is considered a podocyte disease due to the fact that in the majority of patients with proven‑FSGS, the lesion results from defects in the podocyte structure or function. However, FSGS does not result exclusively from podocyte‑associated genes, however also from other genes including collagen IV‑associated genes. Patients who carry the collagen type IVA3 chain (COL4A3) or COL4A4 mutations usually exhibit Alport Syndrome (AS), thin basement membrane neuropathy or familial hematuria (FH)...
November 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29104431/a-case-of-rare-diffuse-alveolar-hemorrhage-and-review-of-literature
#14
Viviana Scollo, Luca Zanoli, Elisa Russo, Giulio Distefano, Francesco Rapisarda
It is well known that some disorders can cause concomitant kidney dysfunction with lung involvement. These syndromes, characterized by the simultaneous presence of intra-alveolar hemorrhage and acute glomerulonephritis, are caused by numerous and variable disorders. The most frequent are the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and Goodpasture syndrome. A quick discerning of the underlying causes and initiation of adequate treatment is crucial to prevent acute respiratory failure and irreversible loss of renal function...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29098738/urine-derived-podocytes-lineage-cells-a-promising-tool-for-precision-medicine-in-alport-syndrome
#15
S Daga, M Baldassarri, C Lo Rizzo, C Fallerini, V Imperatore, I Longo, E Frullanti, E Landucci, L Massella, C Pecoraro, G Garosi, F Ariani, M A Mencarelli, F Mari, A Renieri, A M Pinto
Alport Syndrome (ATS) is a rare genetic disorder caused by collagen IV genes mutations, leading to Glomerular Basement Membrane (GBM) damage up to end-stage renal disease. Podocytes, the main component of the glomerular structure, are the only cells able to produce all the three collagens IV alpha chains associated to ATS and thus, they are key-players in ATS pathogenesis. However, podocytes-targeted therapeutic strategies, have been hampered by the difficulty of non-invasively isolating them and transcripts-based diagnostic approaches are complicated by the inaccessibility of other COL4 chains-expressing cells...
November 2, 2017: Human Mutation
https://www.readbyqxmd.com/read/29089023/phenotype-variability-in-a-large-spanish-family-with-alport-syndrome-associated-with-novel-mutations-in-col4a3-gene
#16
C Cervera-Acedo, A Coloma, E Huarte-Loza, M Sierra-Carpio, E Domínguez-Garrido
BACKGROUND: Alport syndrome is an inherited renal disorder characterized by glomerular basement membrane lesions with hematuria, proteinuria and frequent hearing defects and ocular abnormalities. The disease is associated with mutations in genes encoding α3, α4, or α5 chains of type IV collagen, namely COL4A3 and COL4A4 in chromosome 2 and COL4A5 in chromosome X. In contrast to the well-known X-linked and autosomal recessive phenotypes, there is very little information about the autosomal dominant...
October 31, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29079545/thsd7a-associated-membranous-nephropathy-in-a-patient-with-neurofibromatosis-type-1
#17
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
https://www.readbyqxmd.com/read/29074737/ldl-receptor-related-protein-2-megalin-as-a-target-antigen-in-human-kidney-anti-brush-border-antibody-disease
#18
Christopher P Larsen, Claire Trivin-Avillach, Paige Coles, A Bernard Collins, Michael Merchant, Hong Ma, Daniel W Wilkey, Josephine M Ambruzs, Nidia C Messias, L Nicholas Cossey, Ivy A Rosales, Thomas Wooldridge, Patrick D Walker, Robert B Colvin, Jon Klein, David J Salant, Laurence H Beck
Primary renal tubulointerstitial disease resulting from proximal tubule antigen-specific antibodies and immune complex formation has not been well characterized in humans. We report a cohort of patients with a distinct, underappreciated kidney disease characterized by kidney antibrush border antibodies and renal failure (ABBA disease). We identified ten patients with ABBA disease who had a combination of proximal tubule damage, IgG-positive immune deposits in the tubular basement membrane, and circulating antibodies reactive with normal human kidney proximal tubular brush border...
October 26, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29057733/membranoproliferative-glomerulonephritis-with-unusual-deposits-of-parallel-arrangement-striated-structure-a-new-pathological-entity-%C3%A2
#19
Kensei Yahata, Yuko Kikuchi, Mitsuteru Koizumi, Koichi Seta, Hideki Wakui, Atsushi Komatsuda, Akira Shimizu
A 71-year-old male with a past history of lower limb arteriosclerosis obliterans developed nephrotic syndrome and renal dysfunction. Renal biopsy showed diffuse global endocapillary proliferative lesions with infiltration of mononuclear cells and occasional foam cells. An irregular double contour of the glomerular basement membrane and global mild-to-moderate mesangial proliferative lesions were observed, indicating membranoproliferative glomerulonephritis. Congo red staining was negative. Routine immunofluorescence studies showed no obvious immunoglobulin or complement depositions...
October 23, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29054981/immunoadsorption-in-anti-gbm-glomerulonephritis-case-report-in-a-child-and-literature-review
#20
REVIEW
Guillaume Dorval, Mathilde Lion, Sophie Guérin, Saoussen Krid, Louise Galmiche-Rolland, Rémi Salomon, Olivia Boyer
Antiglomerular basement membrane glomerulonephritis (anti-GBM GN) is a rare autoimmune disease that is characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage. Anti-GBM GN is caused by autoantibodies (classically type G immunoglobulin) directed against the α3 subunit of type IV collagen. Without any appropriate treatment, the disease is generally fulminant, and patient and kidney survival is poor. The current guidelines recommend the use of plasma exchanges and immunosuppressive drugs...
November 2017: Pediatrics
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