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https://www.readbyqxmd.com/read/28729035/a-novel-cfhr1-cfhr5-hybrid-leads-to-a-familial-dominant-c3-glomerulopathy
#1
Shambhuprasad K Togarsimalemath, Sidharth K Sethi, Rajan Duggal, Moglie Le Quintrec, Pranaw Jha, Régis Daniel, Florence Gonnet, Shyam Bansal, Lubka T Roumenina, Veronique Fremeaux-Bacchi, Vijay Kher, Marie-Agnes Dragon-Durey
The intrinsic similarity shared between the members of the complement factor H family, which comprises complement factor H and five complement factor H-related (CFHR) genes, leads to various recombination events. In turn these events lead to deletions of some genes or abnormal proteins, which are found in patients with atypical hemolytic uremic syndrome or C3 glomerulopathies. Here we describe a novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#2
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/28712854/c5-nephritic-factors-drive-the-biological-phenotype-of-c3-glomerulopathies
#3
Maria-Chiara Marinozzi, Sophie Chauvet, Moglie Le Quintrec, Morgane Mignotet, Florent Petitprez, Christophe Legendre, Mathilde Cailliez, Georges Deschenes, Michel Fischbach, Alexandre Karras, Francois Nobili, Christine Pietrement, Marie-Agnes Dragon-Durey, Fadi Fakhouri, Lubka T Roumenina, Veronique Fremeaux-Bacchi
C3 Glomerulopathies, which include Dense Deposit Disease and C3 Glomerulonephritis, are associated with genetic and acquired dysregulation of the C3 convertase alternative pathway of complement. The potential role of the activation of the C5 convertase has not been studied extensively. Here we analyzed IgG samples from patients with C3 Glomerulopathies to identify circulating autoantibodies that stabilize the C3 alternative pathway (C3 Nephritic Factors) as well as C5 convertases (C5 Nephritic Factors), thus preventing decay of these enzyme complexes...
July 13, 2017: Kidney International
https://www.readbyqxmd.com/read/28638323/sortilin-fragments-deposit-at-senile-plaques-in-human-cerebrum
#4
Xia Hu, Zhao-Lan Hu, Zheng Li, Chun-Sheng Ruan, Wen-Ying Qiu, Aihua Pan, Chang-Qi Li, Yan Cai, Lu Shen, Yaping Chu, Bei-Sha Tang, Huaibin Cai, Xin-Fu Zhou, Chao Ma, Xiao-Xin Yan
Genetic variations in the vacuolar protein sorting 10 protein (Vps10p) family have been linked to Alzheimer's disease (AD). Here we demonstrate deposition of fragments from the Vps10p member sortilin at senile plaques (SPs) in aged and AD human cerebrum. Sortilin changes were characterized in postmortem brains with antibodies against the extracellular and intracellular C-terminal domains. The two antibodies exhibited identical labeling in normal human cerebrum, occurring in the somata and dendrites of cortical and hippocampal neurons...
2017: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/28617098/effects-of-chronic-exposure-to-mercury-and-cadmium-alone-and-in-combination-on-the-coagulation-system-of-sprague-dawley-rats
#5
Sandra Arbi, Hester Magdalena Oberholzer, Mia Jeanne Van Rooy, Chantelle Venter, Megan Jean Bester
Water contamination with heavy metals may adversely affect our health. High metal levels lead to changes in blood coagulation processes, increasing the risk for cardiovascular disease. Exposure is not limited to a single metal but usually involves a mixture of metals. In this study 24 male Sprague-Dawley rats were exposed to cadmium (Cd) and mercury (Hg), alone and in combination, for 28 days at dosages equivalent to 1000 times the World Health Organization water limits. Scanning electron microscopy analysis revealed that both metals caused platelet activation...
June 15, 2017: Ultrastructural Pathology
https://www.readbyqxmd.com/read/28609139/evidence-and-role-of-phlebitis-and-lipid-infiltration-in-the-onset-and-pathogenesis-of-wooden-breast-disease-in-modern-broiler-chickens
#6
Michael Babak Papah, Erin Brannick, Carl J Schmidt, Behnam Abasht
Wooden Breast Disease (WBD), a myopathy that frequently affects modern broiler chickens, is a disorder that has been associated with significant economic losses in the poultry industry. To examine tissue changes associated with the onset and early pathogenesis of this disorder, a time-series experiment was conducted using chickens from a high-breast-muscle-yield, purebred commercial broiler line. Birds were raised for up to seven weeks, with a subset of birds sampled weekly. Breast muscle tissues were extracted at necropsy and processed for analysis by light microscopy and transmission electron microscopy...
June 13, 2017: Avian Pathology: Journal of the W.V.P.A
https://www.readbyqxmd.com/read/28593446/c3-glomerulonephritis-with-a-severe-crescentic-phenotype
#7
Aishwarya Ravindran, Fernando C Fervenza, Richard J H Smith, Sanjeev Sethi
BACKGROUND: C3 glomerulopathy (C3G) is rare type of glomerulonephritis resulting from the glomerular deposition of C3 due to dysregulation of the alternative pathway of complement. It is further subdivided into C3 glomerulonephritis (C3GN) and dense deposit disease (DDD), depending on the ultrastructural features. C3GN usually presents with a membranoproliferative pattern of injury. Crescents may or may not be present. However, we have noted a severe necrotizing and crescentic glomerulonephritis in a small subset of C3GN patients...
June 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28573137/rituximab-for-treatment-of-membranoproliferative-glomerulonephritis-and-c3-glomerulopathies
#8
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
https://www.readbyqxmd.com/read/28490967/comparative-study-of-fatty-acid-composition-of-table-eggs-from-the-jeddah-food-market-and-effect-of-value-addition-in-omega-3-bio-fortified-eggs
#9
Shahida Aziz Khan, Aziz Khan, Sarah A Khan, Mohd Amin Beg, Ashraf Ali, Ghazi Damanhouri
Health consciousness has increased the desire of people around the world to consume functional foods. Omega-3 essential fatty acids are one among these beneficial and important health supplements without which a general predisposition to degenerative and stress related disorders can occur. Saudi Arabia has shown an alarming increase in obesity (Al-Nozha et al., 2005), diabetes (Alqurashi et al., 2011), and cardiovascular disease (Al-Nozha et al., 2004) in the last few decades mainly due to nutritional transitions and lifestyle alterations (Amuna and Zotor, 2008)...
May 2017: Saudi Journal of Biological Sciences
https://www.readbyqxmd.com/read/28487790/delayed-manifestation-of-shunt-nephritis-a-case-report-and-review-of-the-literature
#10
Michael Babigumira, Benjamin Huang, Sherry Werner, Wajeh Qunibi
We present an unusual case of shunt nephritis in a 39-year-old male who presented 21 years after placement of a ventriculoperitoneal (VP) shunt. He complained of fevers, headaches, dizziness, and urticarial plaques on arms, trunks, and legs and was found to have anemia, low complement levels, elevated serum creatinine, proteinuria, and new onset microhematuria. Blood and urine cultures were negative. Renal biopsy showed features of acute tubulointerstitial nephritis attributed to vancomycin use. Glomeruli showed increased mesangial hypercellularity and segmental endocapillary proliferation...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28455699/an-in-vivo-11-c-r-pk11195-pet-and-in-vitro-pathology-study-of-microglia-activation-in-creutzfeldt-jakob-disease
#11
Leonardo Iaccarino, Rosa Maria Moresco, Luca Presotto, Orso Bugiani, Sandro Iannaccone, Giorgio Giaccone, Fabrizio Tagliavini, Daniela Perani
Microgliosis is part of the immunobiology of Creutzfeldt-Jakob disease (CJD). This is the first report using (11)C-(R)-PK11195 PET imaging in vivo to measure 18 kDa translocator protein (TSPO) expression, indexing microglia activation, in symptomatic CJD patients, followed by a postmortem neuropathology comparison. One genetic CJD (gCJD) patient, two sporadic CJD (sCJD) patients, one variant CJD (vCJD) patient (mean ± SD age, 47.50 ± 15.95 years), and nine healthy controls (mean ± SD age, 44.00 ± 11...
April 28, 2017: Molecular Neurobiology
https://www.readbyqxmd.com/read/28391334/complement-activation-in-pauci-immune-necrotizing-and-crescentic-glomerulonephritis-results-of-a-proteomic-analysis
#12
Sanjeev Sethi, Ladan Zand, An S De Vriese, Ulrich Specks, Julie A Vrana, Siddak Kanwar, Paul Kurtin, Jason D Theis, Andrea Angioi, Lynn Cornell, Fernando C Fervenza
Background: Complement activation plays an important role in the pathophysiology of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), although it remains unclear which pathway is activated. Whether pauci-immune necrotizing crescentic glomerulonephritis (pauci-immune GN) with negative ANCA serology is part of the spectrum of AAV or a different disease entity is essentially unknown. Methods: We used proteomic analysis to delineate the complement profile in a series of 13 kidney biopsies of patients with pauci-immune GN, with either proteinase 3 (PR3) (five patients) or myeloperoxidase (MPO) antibodies (four patients) or with consistently negative ANCA serology (four patients)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28385149/successful-treatment-with-bortezomib-and-dexamethasone-for-proliferative-glomerulonephritis-with-monoclonal-igg-deposits-in-multiple-myeloma-a-case-report
#13
Rio Noto, Nozomu Kamiura, Yuichiro Ono, Sumie Tabata, Shigeo Hara, Hideki Yokoi, Akihiro Yoshimoto, Motoko Yanagita
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a form of renal involvement by monoclonal IgG deposits that was found in mesangial, subendothelial or subepithelial regions. The distribution of glomerular deposits was completely different from that in monoclonal immunoglobulin deposition disease. PGNMID is reported to be rarely associated with a hematological malignancy. Previously, only five cases of PGNMID with multiple myeloma have been reported...
April 6, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28284385/igg4-related-tubulointerstitial-nephritis
#14
REVIEW
Pingchuan Zhang, Lynn D Cornell
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a fibroinflammatory disorder that can involve nearly any organ. The disorder has increasingly become known as a distinct clinical entity during the last decade. IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most common manifestation of IgG4-RD in the kidney. Many patients with IgG4-TIN are diagnosed after IgG4-RD has been recognized in other organ systems, but the kidney may also be the first or only site involved. The presenting clinical features of IgG4-TIN are most commonly kidney insufficiency, kidney mass lesion(s), or both...
March 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/28195109/metastatic-nasopharyngeal-carcinoma-presenting-as-an-isolated-breast-mass-a-diagnostic-pitfall-and-a-review-of-literature
#15
REVIEW
Trupti Pai, Nita Nair, Gauri Pantvaidya, Kedar Deodhar, Tanuja Shet
Metastases to breast are much rarer than primary breast tumors. We now present a case of 45-year-old female, who presented with an isolated breast mass. A positron emission tomography-computed tomography (PET-CT) done revealed hypermetabolic right breast nodules, soft tissue deposits, and multiple nodal involvement. The biopsy from the breast and axillary lymph node showed dense lymphoid infiltrate and was interpreted initially as granulomatous inflammation. However, the lumps were hard and suspicious for primary breast cancer, so an immunohistochemistry for cytokeratin was performed which highlighted the epithelial cell clusters masked within the inflammatory infiltrate and the diagnosis of undifferentiated carcinoma, lymphoepithelioma-like was made...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28183811/annexin-ii-binding-immunoglobulins-in-patients-with-lupus-nephritis-and-their-correlation-with-disease-manifestations
#16
Kwok Fan Cheung, Susan Yung, Mel K M Chau, Desmond Y H Yap, Kwok Wah Chan, Cheuk Kwong Lee, Colin S O Tang, Tak Mao Chan
Annexin II on mesangial cell surface mediates the binding of anti-dsDNA antibodies and consequent downstream inflammatory and fibrotic processes. We investigated the clinical relevance of circulating annexin II-binding immunoglobulins (Igs) in patients with severe proliferative lupus nephritis, and renal annexin II expression in relation to progression of nephritis in New Zealand Black and White F1 mice (NZBWF1/J) mice. Annexin II-binding Igs in serum were measured by ELISA. Ultrastructural localization of annexin II was determined by electron microscopy...
April 25, 2017: Clinical Science (1979-)
https://www.readbyqxmd.com/read/28157068/clinical-and-pathological-characteristics-of-fabry-disease-combined-with-iga-nephropathy-in-chinese-patients%C3%A2
#17
Nannan Yang, Xia Wang, Feng Xu, Caihong Zeng, Jinquan Wang, Zhihong Liu
AIMS: To improve diagnosis and treatment, we characterized Fabry disease combined with IgA nephropathy and its response to treatment clinically and pathologically in Chinese patients. MATERIALS AND METHODS: Clinical and pathological characteristics of 6 Chinese patients with renal biopsy-proven Fabry disease combined with IgA nephropathy were retrospectively analyzed. RESULTS: There were 4 males and 2 females in this study. All of the 6 patients presented with proteinuria...
April 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28129849/in-vivo-imaging-and-quantification-of-regional-adiposity-in-zebrafish
#18
J E N Minchin, J F Rawls
Adipose tissues (ATs) are lipid-rich structures that supply and sequester energy-dense lipid in response to the energy status of an organism. As such, ATs provide an organism energetic insurance during periods of adverse physiological burden. ATs are deposited in diverse anatomical locations, and excessive accumulation of particular regional ATs modulates disease risk. Therefore, a model system that facilitates the visualization and quantification of regional adiposity holds significant biomedical promise. The zebrafish (Danio rerio) has emerged as a new model system for AT research in which the entire complement of regional ATs can be imaged and quantified in live individuals...
2017: Methods in Cell Biology
https://www.readbyqxmd.com/read/28039031/worsening-of-memory-deficit-induced-by-energy-dense-diet-in-a-rat-model-of-early-alzheimer-s-disease-is-associated-to-neurotoxic-a%C3%AE-species-and-independent-of-neuroinflammation
#19
Pamela V Martino Adami, Pablo Galeano, Marina L Wallinger, Celia Quijano, Alejandro Rabossi, Eleonora S Pagano, Natividad Olivar, Carlos Reyes Toso, Daniel Cardinali, Luis I Brusco, Sonia Do Carmo, Rafael Radi, Goar Gevorkian, Eduardo M Castaño, A Claudio Cuello, Laura Morelli
Diet is a modifiable risk factor for Alzheimer's disease (AD), but the mechanisms linking alterations in peripheral metabolism and cognition remain unclear. Since it is especially difficult to study long-term effects of high-energy diet in individuals at risk for AD, we addressed this question by using the McGill-R-Thy1-APP transgenic rat model (Tg(+/-)) that mimics presymptomatic AD. Wild-type and Tg(+/-) rats were exposed during 6months to a standard diet or a Western diet (WD), high in saturated fat and sugar...
March 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28017869/endosteal-like-extracellular-matrix-expression-on-melt-electrospun-written-scaffolds
#20
Maria Lourdes Muerza-Cascante, Ali Shokoohmand, Kiarash Khosrotehrani, David Haylock, Paul D Dalton, Dietmar W Hutmacher, Daniela Loessner
Tissue engineering technology platforms constitute a unique opportunity to integrate cells and extracellular matrix (ECM) proteins into scaffolds and matrices that mimic the natural microenvironment in vitro. The development of tissue-engineered 3D models that mimic the endosteal microenvironment enables researchers to discover the causes and improve treatments for blood and immune-related diseases. The aim of this study was to establish a physiologically relevant in vitro model using 3D printed scaffolds to assess the contribution of human cells to the formation of a construct that mimics human endosteum...
April 1, 2017: Acta Biomaterialia
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