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https://www.readbyqxmd.com/read/28717939/neonatal-stroke-and-haematuria-answers
#1
Sally Kellett, Mathieu Lemaire, Steven P Miller, Christoph Licht, Grace Yoon, Nomazulu Dlamini, Damien Noone
BACKGROUND: This is a report of an infant born near term with neonatal stroke and haematuria. The renal phenotype, pathogenic genotype and pathological findings on renal biopsy are discussed. CASE-DIAGNOSIS: Prenatal magnetic resonance imaging revealed anomalies which persisted postnatally. Haematuria was detected during follow-up. The posttnatal renal ultrasound scan was normal, and there was no associated proteinuria. A likely pathogenic genetic mutation was detected...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28717936/neonatal-stroke-and-haematuria-questions
#2
Sally Kellett, Mathieu Lemaire, Steven P Miller, Christoph Licht, Grace Yoon, Nomazulu Dlamini, Damien Noone
This is a report of an infant born near term with neonatal stroke and haematuria. Changes were noted on foetal magnetic resonance images, and these persisted postnatally. A routine renal ultrasound scan during follow-up detected haematuria with no associated proteinuria. A likely pathogenic genetic mutation was identified. This case highlights a relatively newly discovered cause for hereditary nephropathy affecting the basement membrane, initially affecting the glomerular but later the renal tubular basement membranes...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#3
Takayuki Katsuno, Shige Mizuno, Masatsuna Mabuchi, Naotake Tsuboi, Atsushi Komatsuda, Shoichi Maruyama
BACKGROUND: Monoclonal immunoglobulin deposition disease (MIDD) is characterized by the non-amyloid deposition of monoclonal immunoglobulin fragments in the basement membranes. Heavy chain deposition disease (HCDD) is a type of MIDD. HCDD is an extremely rare disease, and only three cases have been reported in Japan up to the present. The prognosis of HCDD is very poor, and optimal treatment has not been established. Only a few cases of HCDD with favorable long-term renal prognosis have been reported to date...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28704582/autosomal-dominant-form-of-type-iv-collagen-nephropathy-exists-among-patients-with-hereditary-nephritis-difficult-to-diagnose-clinicopathologically
#4
Aya Imafuku, Kandai Nozu, Naoki Sawa, Eiko Hasegawa, Rikako Hiramatsu, Masahiro Kawada, Junichi Hoshino, Kiho Tanaka, Yasuo Ishii, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Kazumoto Iijima, Yoshifumi Ubara
AIM: Type IV collagen nephropathies include Alport Syndrome and thin basement membrane nephropathy (TBMN), which are caused by mutations in COL4A3/A4/A5 genes. Recently, the report of patients with heterozygous mutations in COL4A3/A4 have been increasing. The clinical course of these patients has a wide variety, and they are diagnosed as TBMN, autosomal dominant Alport syndrome (ADAS), or familial focal segmental glomerular sclerosis. However, diagnosis, frequency and clinicopathological manifestation of them remains unclear...
July 13, 2017: Nephrology
https://www.readbyqxmd.com/read/28700653/plasma-bradykinin-and-early-diabetic-nephropathy-lesions-in-type-1-diabetes-mellitus
#5
Kevin M Wheelock, Jian Cai, Helen C Looker, Michael L Merchant, Robert G Nelson, Gudeta D Fufaa, E Jennifer Weil, Harold I Feldman, Ramachandran S Vasan, Paul L Kimmel, Brad H Rovin, Michael Mauer, Jon B Klein
OBJECTIVE: To examine the association of bradykinin and related peptides with the development of diabetic nephropathy lesions in 243 participants with type 1 diabetes (T1D) from the Renin-Angiotensin System Study who, at baseline, were normoalbuminuric, normotensive and had normal or increased glomerular filtration rate (GFR). DESIGN: Plasma concentrations of bradykinin and related peptides were measured at baseline by quantitative mass spectrometry. All participants were randomly assigned at baseline to receive placebo, enalapril or losartan during the 5 years between kidney biopsies...
2017: PloS One
https://www.readbyqxmd.com/read/28687864/stressed-podocytes-mechanical-forces-sensors-signaling-and-response
#6
REVIEW
Karlhans Endlich, Felix Kliewe, Nicole Endlich
Increased glomerular capillary pressure (glomerular hypertension) and increased glomerular filtration rate (glomerular hyperfiltration) have been proven to cause glomerulosclerosis in animal models and are likely to be operative in patients. Since podocytes cover the glomerular basement membrane, they are exposed to tensile stress due to circumferential wall tension and to fluid shear stress arising from filtrate flow through the narrow filtration slits and through Bowman's space. In vitro evidence documents that podocytes respond to tensile stress as well as to fluid shear stress...
July 7, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28685678/glomerular-endothelium-and-its-impact-on-glomerular-filtration-barrier-in-diabetes-are-the-gaps-still-illusive
#7
Joseph Fomusi Ndisang
BACKGROUND: Glomerular capillaries are lined with highly specialized fenestrated endothelium which are primarily responsible to regulate high flux filtration of fluid and small solutes. During filtration, plasma passes through the fenestrated endothelium and basement membrane before it reaches the slit diaphragm, a specialized type of intercellular junction that connects neighbouring podocytes. METHODS: A PubMed search was done for recent articles on components of the glomerular filtration barrier such as glomerular endothelial cells, podocytes and glomerular basement membrane, and the effect of diabetes on these structures...
July 5, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28681095/nail-patella-syndrome
#8
REVIEW
Ralph Witzgall
The pathognomonic symptoms of patients with nail-patella syndrome are their small or absent patellae and dysplastic or absent finger- and toenails. Many of the patients suffer from renal symptoms which also affect their prognosis. In 1998, mutations in the gene encoding the transcription factor LMX1B were identified as underlying this autosomal-dominant disease. The LMX1B gene is expressed in a variety of tissues, and the symptoms are reflected nicely by its expression pattern. LMX1B is essential for dorso-ventral pattern formation in the limbs, for differentiation of the anterior portions of the eyes, for development of certain neuron populations in the central nervous system, and for the differentiation and maintenance of podocytes...
July 5, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28659572/effect-of-vitamin-d-and-tacrolimus-combination-therapy-on-iga-nephropathy
#9
Dong Yuan, Zhan Fang, Fang Sun, Jing Chang, Jian Teng, Shuhua Lin, Xiaoming Liu
BACKGROUND To explore the effects and the mechanism of vitamin D (VD) and tacrolimus (TAC) combinatorial therapy in the treatment of IgA nephropathy (IgAN) in a rat model. MATERIAL AND METHODS IgAN rat models constructed by oral immunization with bovine serum albumin (BSA) and lipopolysaccharide (LPS) (n=30) and were treated with: saline (model group), TAC (TAC group), or TAC+VD therapy (TAC+VD group) through gavage daily for 14 days. Serum creatinine (Scr), albumin (ALB), blood urea nitrogen (BUN), and urinary protein (UAE) levels were determined...
June 29, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/28656209/hydrogen-sulfide-reduced-renal-tissue-fibrosis-by-regulating-autophagy-in-diabetic-rats
#10
Lin Li, Ting Xiao, Fang Li, Yan Li, Ou Zeng, Maojun Liu, Biao Liang, Zining Li, Chun Chu, Jun Yang
The present study aimed to explore the effect of hydrogen sulfide (H2S) on renal tissue fibrosis and its mechanism in diabetic rats. Rats were randomly divided into four groups (n=13/group): Control group; induced diabetes mellitus group (STZ); induced diabetes mellitus treated with H2S group (STZ + H2S); normal rats treated with H2S group (H2S). The diabetic model was induced by intraperitoneal (i.p.) injections of 40 mg/kg body weight streptozotocin (STZ); the control group was treated with saline every day (i...
August 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28655312/transplantation-of-human-fetal-pancreatic-progenitor-cells-ameliorates-renal-injury-in-streptozotocin-induced-diabetic-nephropathy
#11
Yongwei Jiang, Wenjian Zhang, Shiqing Xu, Hua Lin, Weiguo Sui, Honglin Liu, Liang Peng, Qing Fang, Li Chen, Jinning Lou
BACKGROUND: Diabetic nephropathy (DN) is a severe complication of diabetes mellitus (DM). Pancreas or islet transplantation has been reported to prevent the development of DN lesions and ameliorate or reverse existing glomerular lesions in animal models. Shortage of pancreas donor is a severe problem. Islets derived from stem cells may offer a potential solution to this problem. OBJECTIVE: To evaluate the effect of stem cell-derived islet transplantation on DN in a rat model of streptozotocin-induced DM...
June 27, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28647853/are-podocytes-motile
#12
REVIEW
Nicole Endlich, Florian Siegerist, Karlhans Endlich
Podocytes, the postmitotic and highly branched epithelial cells of the glomerulus, play a pivotal role for the function of the glomerular filtration barrier and the development of chronic kidney disease. It has long been discussed whether podocytes in vivo are motile and can laterally migrate in a coordinated way along the capillaries until they reach the position of naked glomerular basement membrane often found in podocytopathies. Such motility would also be the prerequisite for the replacement of lost podocytes by progenitor cells...
June 24, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28646861/c-terminal-fragment-of-agrin-caf-levels-predict-acute-kidney-injury-after-acute-myocardial-infarction
#13
Spyridon Arampatzis, Georgios Chalikias, Vasilios Devetzis, Stavros Konstantinides, Uyen Huynh-Do, Dimitrios Tziakas
BACKGROUND: Patients with acute myocardial infarction are at high risk for acute kidney injury. Novel biomarkers that can predict acute kidney injury in AMI may allow timely interventions. C-terminal fragment of agrin (CAF), a proteoglycan of the glomerular and tubular basement membrane, have been recently associated with rapid renal function deterioration and proximal tubular dysfunction. It is unknown whether elevated CAF levels may serve as a novel AKI biomarker in patients presenting with AMI...
June 24, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28613962/significant-association-between-renal-function-and-area-of-amyloid-deposition-in-kidney-biopsy-specimens-in-both-aa-amyloidosis-associated-with-rheumatoid-arthritis-and-al-amyloidosis
#14
Takeshi Kuroda, Naohito Tanabe, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Nakatsue, Yoko Wada, Yumi Ito, Naofumi Imai, Mitsuhiro Ueno, Masaaki Nakano, Ichiei Narita
The kidney is a major target organ for systemic amyloidosis, which results in proteinuria and an elevated serum creatinine level. The clinical manifestations and precursor proteins of amyloid A (AA) and light-chain (AL) amyloidosis are different, and the renal damage due to amyloid deposition also seems to differ. The purpose of this study was to clarify haw the difference in clinical features between AA and AL amyloidosis are explained by the difference in the amount and distribution of amyloid deposition in the renal tissues...
June 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28597189/a-podocyte-view-of-membranous-nephropathy-from-heymann-nephritis-to-the-childhood-human-disease
#15
REVIEW
Pierre Ronco, Hanna Debiec
Membranous nephropathy (MN) is characterized by an accumulation of immune deposits on the subepithelial side of the glomerular basement membrane, which results in complement activation and proteinuria. Since 2002, several major antigens of the podocyte have been identified in human MN, the first one being neutral endopeptidase (NEP), the alloantigen involved in neonatal cases of MN that occur in newborns from NEP-deficient mothers. This discovery opened the field to the major advances that have occurred since then in the pathophysiology and treatment of MN...
June 8, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28595914/ischemia-induced-glomerular-parietal-epithelial-cells-hyperplasia-commonly-misdiagnosed-cellular-crescent-in-renal-biopsy
#16
Yeting Zeng, Xinrui Wang, Feilai Xie, Zhiyong Zheng
Ischemic pseudo-cellular crescent (IPCC) that is induced by ischemia and composed of hyperplastic glomerular parietal epithelial cells resembles cellular crescent. In this study, we aimed to assess the clinical and pathological features of IPCC in renal biopsy to avoid over-diagnosis and to determine the diagnostic basis. 4 IPCC cases diagnosed over a 4-year period (2012-2015) were evaluated for the study. Meanwhile, 5 cases of ANCA-associated glomerulonephritis and 5 cases of lupus nephritis (LN) were selected as control...
April 20, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28584626/mmp2-and-mmp9-associate-with-crescentic-glomerulonephritis
#17
Tessa M Phillips, Mitali Fadia, Tom N Lea-Henry, Jonathan Smiles, Giles D Walters, Simon H Jiang
Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiple organ involvement. Lupus nephritis (LN) is a common manifestation with a wide variety of histological appearances. Matrix metalloproteinases (MMP) 2 and 9 are gelatinases capable of degrading glomerular basement membrane type IV collagen, which have been associated with LN. We examine the expression of MMP2 and MMP9 in different classes of LN. Methods: MMP2 and MMP9 expression was detected by immunohistochemistry in sections from renal biopsy specimens with class III, class IV and class V LN (total n = 31), crescentic immunoglobulin A nephropathy (n = 6), pauci-immune glomerulonephritis (n = 7), minimal change disease (n = 2), mesangiocapillary glomerulonephritis (n = 7), diabetic nephropathy (n = 12) and histologically normal controls (n = 8)...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28578628/relationship-between-immunoglobulin-deposition-and-early-lesions-of-progressive-glomerulonephropathy-in-young-common-marmosets
#18
Naoaki Yamada, Nanako Hashimoto, Junichi Kamiie, Takuya Doi, Junko Sato, Takeshi Inoue, Kinji Shirota, Minoru Tsuchitani
The authors previously investigated progressive glomerulonephropathy in 2- to 11-year-old common marmosets and characterized age-related changes of the renal glomeruli and development of tubulointerstitial lesions. In this study, immunoglobulin deposition and ultrastructural changes of the glomeruli were investigated in 5 young marmosets from 6 months to 3 years of age with pre-onset or early glomerulonephropathy. In all animals, the foot processes of podocytes were effaced, and IgM was deposited into the glomeruli...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28574576/vegf-a165-b-protects-against-proteinuria-in-a-mouse-model-with-progressive-depletion-of-all-endogenous-vegf-a-splice-isoforms-from-the-kidney
#19
Megan Stevens, Christopher R Neal, Andrew Hj Salmon, David O Bates, Steven J Harper, Sebastian Oltean
Chronic kidney disease (CKD) is strongly associated with a decrease in the expression of VEGF-A. However, little is known about the contribution of VEGF-A splice isoforms to kidney physiology and pathology. Previous studies suggest that the splice isoform VEGF-A165 b (resulting from alternative usage of a 3' splice site in the terminal exon) is protective for kidney function. We show here, in a quad-transgenic model, that over-expression of VEGF-A165 b alone is sufficient to rescue the increase in proteinuria as well as glomerular water permeability in the context of progressive depletion of all VEGF-A isoforms from the podocytes...
June 2, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28573137/rituximab-for-treatment-of-membranoproliferative-glomerulonephritis-and-c3-glomerulopathies
#20
REVIEW
Michael Rudnicki
Membranoproliferative glomerulonephritis (MPGN) is a histological pattern of injury resulting from predominantly subendothelial and mesangial deposition of immunoglobulins or complement factors with subsequent inflammation and proliferation particularly of the glomerular basement membrane. Recent classification of MPGN is based on pathogenesis dividing MPGN into immunoglobulin-associated MPGN and complement-mediated C3 glomerulonephritis (C3GN) and dense deposit disease (DDD). Current guidelines suggest treatment with steroids, cytotoxic agents with or without plasmapheresis only for subjects with progressive disease, that is, nephrotic range proteinuria and decline of renal function...
2017: BioMed Research International
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