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https://www.readbyqxmd.com/read/29054981/immunoadsorption-in-anti-gbm-glomerulonephritis-case-report-in-a-child-and-literature-review
#1
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...
October 20, 2017: Pediatrics
https://www.readbyqxmd.com/read/29045953/-detection-of-large-deletions-in-x-linked-alport-syndrome-using-competitive-multiplex-fluorescence-polymerase-chain-reaction
#2
F Wang, Y Q Zhang, J Ding, L X Yu
OBJECTIVE: To evaluate the ability of multiplex competitive fluorescence polymerase chain reaction in detection of large deletion and duplication genotypes of X-linked Alport syndrome. METHODS: Clinical diagnosis of X-linked Alport syndrome was based on either abnormal staining of type IV collagen α5 chain in the epidermal basement membrane alone or with abnormal staining of type IV collagen α5 chain in the glomerular basement membrane and Bowman's capsule/ultrastructural changes in the glomerular basement membrane typical of Alport syndrome...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29042902/unusual-case-of-posterior-reversible-encephalopathy-syndrome-in-a-patient-with-anti-glomerular-basement-membrane-antibody-glomerulonephritis-a-case-report-and-review-of-the-literature
#3
Boram Cha, Dae Young Kim, Hyunil Jang, Seun Deuk Hwang, Huck Jei Choi, Moon-Jae Kim
Posterior reversible encephalopathy syndrome (PRES) is characterized by a clinical and radiological entity with the sudden onset of seizures, headache, altered consciousness, and visual disturbances in patients with the findings of reversible vasogenic subcortical edema without infarction. Hypertension, renal disease, and autoimmune disease are co-morbid conditions of PRES. Nevertheless, there have only been a few case reports of PRES in a patient with anti-glomerular basement membrane antibody glomerulonephritis (anti-GBM GN)...
September 2017: Electrolyte & Blood Pressure: E & BP
https://www.readbyqxmd.com/read/29038743/mature-induced-pluripotent-stem-cell-derived-human-podocytes-reconstitute-kidney-glomerular-capillary-wall-function-on-a-chip
#4
Samira Musah, Akiko Mammoto, Thomas C Ferrante, Sauveur S F Jeanty, Mariko Hirano-Kobayashi, Tadanori Mammoto, Kristen Roberts, Seyoon Chung, Richard Novak, Miles Ingram, Tohid Fatanat-Didar, Sandeep Koshy, James C Weaver, George M Church, Donald E Ingber
An in vitro model of the human kidney glomerulus - the major site of blood filtration - could facilitate drug discovery and illuminate kidney-disease mechanisms. Microfluidic organ-on-a-chip technology has been used to model the human proximal tubule, yet a kidney-glomerulus-on-a-chip has not been possible because of the lack of functional human podocytes - the cells that regulate selective permeability in the glomerulus. Here, we demonstrate an efficient (> 90%) and chemically defined method for directing the differentiation of human induced pluripotent stem (hiPS) cells into podocytes that express markers of the mature phenotype (nephrin+, WT1+, podocin+, Pax2-) and that exhibit primary and secondary foot processes...
2017: Nature biomedical engineering
https://www.readbyqxmd.com/read/29038391/embryohistiogenesis-of-vascular-tufts-of-glomeruli-a-possible-hypothesis
#5
Shahriar Dabiri, Mohammad Mehdi Moeini-Aghtaei, Bahram Dabiri
INTRODUCTION: Embryogenesis of the kidney glomeruli, especially its vascular component, has not been well documented. Glomeruli capillary tuft is surrounded and enveloped by visceral epithelial cells, which is a unique portal system that connects afferent with efferent arteriole without interaction with venular circulation. We hypothesized that the portal system embryologically has developed by extension of the intima of afferent arteriole into the stroma of glomerulus. We also hypothesized that juxtaglomeruli apparatus was developed from remnants of smooth muscle cells of the media of afferent arteriole at the anastomosing site with the Bowman capsule entrance...
October 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29027535/transplant-glomerulopathy
#6
REVIEW
Edward J Filippone, Peter A McCue, John L Farber
In the renal allograft, transplant glomerulopathy represents a morphologic lesion and not a specific diagnosis. The hallmark pathologic feature is glomerular basement membrane reduplication by light microscopy or electron microscopy in the absence of immune complex deposits. Transplant glomerulopathy results from chronic, recurring endothelial cell injury that can be mediated by HLA alloantibodies (donor-specific antibodies), various autoantibodies, cell-mediated immune injury, thrombotic microangiopathy, or chronic hepatitis C...
October 13, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29024123/morphological-characterization-of-ckd-in-cats-insights-of-fibrogenesis-to-be-recognized
#7
G B Morais, D A Viana, J M Verdugo, M G Roselló, J O Porcel, D D Rocha, F A F Xavier Júnior, K D S M Barbosa, F M O Silva, G A C Brito, C M S Sampaio, J S A M Evangelista
Renal fibrosis is characterized by glomerulosclerosis and tubulointerstitial fibrosis and its pathogenesis is associated with the activity of mesenchymal cells (fibroblasts), being essentially characterized by a process of excessive accumulation resulting from the deposition of extracellular matrix components. The aim of this study was to characterize the morphological presentation of chronic and fibrotic lesions in the glomerular, tubular, interstitial, and vascular compartments in feline CKD, as well as the possible participation of myofibroblasts in renal fibrotic processes in this species...
October 12, 2017: Microscopy Research and Technique
https://www.readbyqxmd.com/read/28992267/white-blood-cell-fractions-correlate-with-lesions-of-diabetic-kidney-disease-and-predict-loss-of-kidney-function-in-type-2-diabetes
#8
Kevin M Wheelock, Pierre-Jean Saulnier, Stephanie K Tanamas, Pavithra Vijayakumar, E Jennifer Weil, Helen C Looker, Robert L Hanson, Kevin V Lemley, Berne Yee, William C Knowler, Samy Hadjadj, Bezhad Najafian, Michael Mauer, Robert G Nelson
Background: Inflammation linked to diabetic kidney disease (DKD) may affect white blood cell (WBC) counts and differentials. We examined the cross-sectional associations of total WBC count and WBC fractions with structural lesions of DKD in 108 Pima Indians with Type 2 diabetes who underwent research kidney biopsies. We also examined the longitudinal association of these WBC variables with renal function loss (RFL) in 941 Europeans with Type 2 diabetes from the SURDIAGENE study. Methods: Associations of WBC variables with morphometric parameters were assessed by linear regression...
July 18, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28992095/the-reduction-of-heparan-sulphate-in-the-glomerular-basement-membrane-does-not-augment-urinary-albumin-excretion
#9
Satoshi Aoki, Akiko Saito-Hakoda, Takeo Yoshikawa, Kyoko Shimizu, Kiyomi Kisu, Susumu Suzuki, Kiyoshi Takagi, Shuji Mizumoto, Shuhei Yamada, Toin H van Kuppevelt, Atsushi Yokoyama, Taiji Matsusaka, Hiroshi Sato, Sadayoshi Ito, Akira Sugawara
Background: Heparan sulphate proteoglycan (HSPG) is present in the glomerular basement membrane (GBM) and is thought to play a major role in the glomerular charge barrier. Reductions and structural alterations of HSPG are observed in different types of kidney diseases accompanied by proteinuria. However, their causal relations remain unknown. Methods: We generated podocyte-specific exostosin-like 3 gene ( Extl3 ) knockout mice ( Extl3KO ) using a Cre-loxP recombination approach...
July 8, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28990100/low-molecular-weight-heparin-may-benefit-nephrotic-remission-in-steroid%C3%A2-sensitive-nephrotic-syndrome-via-inhibiting-elastase
#10
Songhui Zhai, Lijuan Hu, Lin Zhong, Yuhong Tao, Zheng Wang
Low molecular weight heparin (LMWH) has a structure similar to heparan sulfate, which exerts anti‑inflammatory effects via inhibiting elastase (Ela) activity. Release of Ela along the glomerular capillary wall may induce glomerular injury and proteinuria. The present study aimed to investigate the influence of LMWH on steroid‑sensitive nephrotic syndrome (SSNS) and the potential underlying mechanism. A total of 40 SSNS patients and 20 healthy controls were recruited. SSNS patients were treated with LMWH and prednisone simultaneously (LMWH+pred group) or with prednisone alone (pred group)...
October 3, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28990057/id3-may-protect-mice-from-anti%C3%A2-gbm-glomerulonephritis-by-regulating-the-differentiation-of-th17-and-treg-cells
#11
Huan Zhou, Le Wang, Qing Xu, Qingquan Liu, Hui Liu, Wenhui Qiu, Tingyang Hu, Yongman Lv, Qian Zhang
Anti‑glomerular basement membrane glomerulonephritis (anti‑GBM GN) is an autoimmune disease that leads to severe and rapidly progressive renal injury. Inhibition of DNA‑binding factor 3 (ID3) serves a key role in autoimmune diseases, such as asthma and Sjögren's syndrome, and in experimental allergic encephalitis models. However, the role of ID3 in the progression of anti‑GBM GN remains unknown. In the present study, ID3 mRNA expression increased between 3‑ and 20‑fold in the renal tissues of anti‑GBM GN mice compared with the Control group, with a peak at day 14 post‑induction...
October 4, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28984775/clinicopathological-features-of-progressive-renal-involvement-in-tafro-syndrome-a-case-report-and-literature-review
#12
REVIEW
Mari Tanaka, Hiraku Tsujimoto, Kojiro Yamamoto, Saeko Shimoda, Kazumasa Oka, Hiroya Takeoka
RATIONALE: TAFRO syndrome is a systemic inflammatory disease characterized by a constellation of symptoms: Thrombocytopenia, Anasarca, MyeloFibrosis, Renal dysfunction, and Organomegaly. Progressive renal insufficiency is a predominant symptom; however, the mechanism of acute kidney injury (AKI) remains unclear, probably because severe thrombocytopenia prevents kidney biopsy. We report a rare case of TAFRO syndrome with histologically confirmed renal involvement. PATIENTS CONCERNS: A 70-year-old man developed fever, anasarca, AKI, thrombocytopenia, and hepatosplenomegaly...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28979697/the-zdsd-rat-a-novel-model-of-diabetic-nephropathy
#13
Richard G Peterson, Charles Van Jackson, Karen M Zimmerman
The ZDSD rat is a new obese-diabetic rat model that expresses type 2 diabetes in the presence of an intact leptin pathway. During a long pre-diabetic state, the animals exhibit most of the features of metabolic syndrome including obesity, hyperlipidemia, hypertension, insulin resistance and decreased glucose disposal. The animals used in these studies were either allowed to become spontaneously diabetic at 16-30 weeks of age, or diabetes was induced with a diabetogenic diet. In the presence of either spontaneous or diet-induced diabetes, they develop progressive albuminuria as well as increases in other urinary markers of impaired renal function (kidney injury molecule-1 (KIM-1), β2-microglobulin, clusterin and cystatin C)...
2017: American Journal of Translational Research
https://www.readbyqxmd.com/read/28951332/involvement-of-glucose-regulated-protein-78-and-spliced-x-box-binding-protein-1-in-the-protective-effect-of-gliclazide-in-diabetic-nephropathy
#14
Ying-Wen Zhang, Xiuping Wang, Xiaodan Ren, Manling Zhang
AIMS: To testing whether endoplasmic reticulum (ER) stress contributes to the development of diabetic nephropathy. Investigated the effect of gliclazide, an oral antihyperglycemic agent, in a rat model of diabetic nephropathy and the underlying mechanism related to the ER stress response. METHODS: Sixty SD rats were divided into six groups. Diabetic nephropathy was induced in 30 rats with a streptozotocin (STZ) injection and high fat diet, which were then treated with saline, gliclazide or 4-PBA...
May 4, 2017: Diabetes Research and Clinical Practice
https://www.readbyqxmd.com/read/28938947/foreseeing-the-future-of-glomerular-disease-through-slits-mir-npnt-axis
#15
Reiko Inagi, Yu Ishimoto, Tzu-Ming Jao
Podocyte biology is an important key factor for the maintenance of glomerular function and structure. Nephronectin is a podocyte-derived extracellular matrix protein in the glomerular basement membrane. Suppression of nephronectin expression by microRNA-378a-3p significantly induced proteinuria due to loss of integrity of the glomerular filtration barrier in zebrafish and mice, demonstrating the relevance of the microRNA-nephronectin axis in regulation of the podocyte-glomerular basement membrane interaction and integrity of the glomerular filtration barrier...
October 2017: Kidney International
https://www.readbyqxmd.com/read/28937063/a-mini-epidemic-of-anti-glomerular-basement-membrane-disease-clinical-and-epidemiological-study
#16
Umesh Lingaraj, Shivaprasad Sasivehalli Mallappa, Rajiv Elkal Neminah, Satishkumar Madakala Mohan, Leelavathi Venkatesh, Sreedhara Chikkanayakanahalli Gurusiddaiah, Niranjan Manibally Rachaiah
Acute glomerulonephritis due to anti-glomerular basement membrane (anti-GBM) antibody disease is rare, estimated to occur in fewer than one case per million population and accounts for less than 20% of rapidly progressive glomerulonephritis. The prevalence among patients evaluated for potential glomerular disease is lower. It accounts for fewer than 3% of all kidney biopsies done with crescentic glomerulonephritis. Cases of anti-GBM disease occurring in a cluster have rarely been reported. All biopsy proven anti-GBM disease cases were collected from January 2015 to March 2015 at our Institute...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28933341/c4d-expressing-glomerulopathy-and-proteinuria-post-transplantation-of-a-%C3%A2-too-big-for-size-mismatched-kidney-allograft-an-unusual-case-with-good-outcome%C3%A2
#17
Francois Gougeon, Alexei V Mikhailov, Keisha Gibson, Tomasz Kozlowski, Harsharan K Singh, Volker Nickeleit
A 5-year-old severely growth-retarded child with tubulointerstitial, oliguric end-stage renal disease received an adult-size kidney transplant. Three years post grafting under standard triple immunosuppression (mycophenolate mofetil, tacrolimus, and prednisone) de novo nephrotic range proteinuria without the nephrotic syndrome developed. Graft function was normal (serum creatinine: 0.2 - 0.3 mg/dL), there were no donor-specific HLA antibodies (DSA), and the urine sediment was inactive. Two biopsies collected 3 and 4 years post-transplantation showed severe glomerular capillary wall remodeling and associated pseudolinear C4d staining as morphologic correlates for the proteinuria...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28927772/-antiglomerular-basement-disease-in-children-literature-review-and-therapeutic-options
#18
G Dorval, S Guérin, L Berteloot, S Krid, R Salomon, L Galmiche-Rolland, O Boyer
Antiglomerular basement membrane glomerulonephritis is a rare autoimmune disease characterized by rapidly progressive glomerulonephritis that may be associated with pulmonary hemorrhage (Goodpasture syndrome). The disease is caused by autoantibodies (classically IgGs) directed against the α3 subunit of type IV collagen. This is a rare disease in the adult population and extremely rare in children, with a reported cumulative annual incidence at 1/10(6) people/year. Among scarce reported pediatric cases (n=31), most are girls (M/F sex ratio, 1:4), and the mean age at diagnoses is 9...
September 15, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28923616/histopathologic-findings-of-potential-kidney-donors-with-asymptomatic-microscopic-hematuria-impact-on-donation
#19
E A Hassan, T Z Ali, A Abdulbaki, I A Ibrahim, H M Almanae, H A Aleid
INTRODUCTION: Isolated microscopic hematuria (IMH) is not uncommon in potential kidney donors. AIM: The aim was to study the kidney biopsy findings of potential kidney donors with IMH and the impact of the histopathologic diagnoses on the decision to accept or decline such donors from kidney donation. METHODS: In this retrospective study, all the potential kidney donors with IMH were identified from the medical records of patients who underwent kidney biopsies between January 2010 and December 2016...
October 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28916834/ultrastructural-characterization-of-the-glomerulopathy-in-alport-mice-by-helium-ion-scanning-microscopy-him
#20
Kenji Tsuji, Hani Suleiman, Jeffrey H Miner, James M Daley, Diane E Capen, Teodor G Păunescu, Hua A Jenny Lu
The glomerulus exercises its filtration barrier function by establishing a complex filtration apparatus consisting of podocyte foot processes, glomerular basement membrane and endothelial cells. Disruption of any component of the glomerular filtration barrier leads to glomerular dysfunction, frequently manifested as proteinuria. Ultrastructural studies of the glomerulus by transmission electron microscopy (TEM) and conventional scanning electron microscopy (SEM) have been routinely used to identify and classify various glomerular diseases...
September 15, 2017: Scientific Reports
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