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https://www.readbyqxmd.com/read/29151252/dominant-c3-glomerulopathy-new-roles-for-an-old-actor-in-renal-pathology
#1
REVIEW
Nicola Pirozzi, Antonella Stoppacciaro, Paolo Menè
Recently, a number of reports have described dominant C3 deposits in renal biopsies of patients with infection-related glomerulonephritis (GN). While acute post-infectious GN and membranoproliferative GN are commonly characterized by immune deposits containing C3 and/or C4, the absence of immunoglobulin (Ig) and/or immune complexes at light or electron microscopy is a rather unusual observation. Dominant C3 deposition is believed to result from the alternative pathway of complement activation via the C3bBb "tickover" convertase...
November 18, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/29096623/substantial-fibrin-amyloidogenesis-in-type-2-diabetes-assessed-using-amyloid-selective-fluorescent-stains
#2
Etheresia Pretorius, Martin J Page, Lize Engelbrecht, Graham C Ellis, Douglas B Kell
BACKGROUND: We have previously shown that many chronic, inflammatory diseases are accompanied, and possibly partly caused or exacerbated, by various coagulopathies, manifested as anomalous clots in the form of 'dense matted deposits'. More recently, we have shown that these clots can be amyloid in nature, and that the plasma of healthy controls can be induced to form such clots by the addition of tiny amounts of bacterial lipopolysaccharide or lipoteichoic acid. Type 2 diabetes (T2D) is also accompanied by raised levels of LPS...
November 2, 2017: Cardiovascular Diabetology
https://www.readbyqxmd.com/read/29092674/autoantibodies-against-complement-components-in-systemic-lupus-erythematosus-role-in-the-pathogenesis-and-clinical-manifestations
#3
M H Hristova, V S Stoyanova
Many complement structures and a number of additional factors, i.e. autoantibodies, receptors, hormones and cytokines, are implicated in the complex pathogenesis of systemic lupus erythematosus. Genetic defects in the complement as well as functional deficiency due to antibodies against its components lead to different pathological conditions, usually clinically presented. Among them hypocomplementemic urticarial vasculitis, different types of glomerulonephritis as dense deposit disease, IgA nephropathy, atypical haemolytic uremic syndrome and lupus nephritis are very common...
December 2017: Lupus
https://www.readbyqxmd.com/read/29068987/membranous-nephropathy-in-a-patient-with-ankylosing-spondylitis-a-case-report
#4
Ruiying Chen, Fang Li, Qionghong Xie, Jun Xue, Lingyun Lai, Shaojun Liu, Liyin Zhang, Chuanming Hao
RATIONALE: Renal complications in ankylosing spondylitis (AS) were rarely observed, and proteinuria associated with AS can be seen often due to amyloidosis in this kind of complications, while membranous nephropathy (MN) is seldom considered. This article reports a case of coexistence of AS and MN, to provide the exact relationship of these 2 entities and recognized some causes of renal involvement in AS. PATIENT CONCERNS: A 44-year-old female presented with pain of the left leg for 4 years and pedal edema for 2 weeks...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29057733/membranoproliferative-glomerulonephritis-with-unusual-deposits-of-parallel-arrangement-striated-structure-a-new-pathological-entity-%C3%A2
#5
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/29053080/aerosolizing-lipid-dispersions-enables-antibiotic-transport-across-mimics-of-the-lung-airway-surface-even-in-the-presence-of-pre-existing-lipid-monolayers
#6
Steven V Iasella, Amy Z Stetten, Timothy E Corcoran, Stephen Garoff, Todd M Przybycien, Robert D Tilton
BACKGROUND: Secondary lung infections are the primary cause of morbidity associated with cystic fibrosis lung disease. Aerosolized antibiotic inhalation is potentially advantageous but has limited effectiveness due to altered airway aerodynamics and deposition patterns that limit drug access to infected regions. One potential strategy to better reach infected areas is to formulate aerosols with surfactants that induce surface tension gradients and drive postdeposition drug dispersal via Marangoni transport along the airway surface liquid (ASL)...
October 20, 2017: Journal of Aerosol Medicine and Pulmonary Drug Delivery
https://www.readbyqxmd.com/read/29036611/progranulin-mediated-deficiency-of-cathepsin-d-results-in-ftd-and-ncl-like-phenotypes-in-neurons-derived-from-ftd-patients
#7
Clarissa Valdez, Yvette C Wong, Michael Schwake, Guojun Bu, Zbigniew K Wszolek, Dimitri Krainc
Frontotemporal dementia (FTD) encompasses a group of neurodegenerative disorders characterized by cognitive and behavioral impairments. Heterozygous mutations in progranulin (PGRN) cause familial FTD and result in decreased PGRN expression, while homozygous mutations result in complete loss of PGRN expression and lead to the neurodegenerative lysosomal storage disorder neuronal ceroid lipofuscinosis (NCL). However, how dose-dependent PGRN mutations contribute to these two different diseases is not well understood...
September 25, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/29030465/cluster-analysis-identifies-distinct-pathogenetic-patterns-in-c3-glomerulopathies-immune-complex-mediated-membranoproliferative-gn
#8
Paraskevas Iatropoulos, Erica Daina, Manuela Curreri, Rossella Piras, Elisabetta Valoti, Caterina Mele, Elena Bresin, Sara Gamba, Marta Alberti, Matteo Breno, Annalisa Perna, Serena Bettoni, Ettore Sabadini, Luisa Murer, Marina Vivarelli, Marina Noris, Giuseppe Remuzzi
Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement-mediated C3 glomerulopathy (C3G) and immune complex-mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN...
October 13, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28986537/counterbalancing-anti-adhesive-effects-of-tenascin-c-through-fibronectin-expression-in-endothelial-cells
#9
Agata Radwanska, Dominique Grall, Sébastien Schaub, Stéphanie Beghelli-de la Forest Divonne, Delphine Ciais, Samah Rekima, Tristan Rupp, Anne Sudaka, Gertraud Orend, Ellen Van Obberghen-Schilling
Cellular fibronectin (FN) and tenascin-C (TNC) are prominent development- and disease-associated matrix components with pro- and anti-adhesive activity, respectively. Whereas both are present in the tumour vasculature, their functional interplay on vascular endothelial cells remains unclear. We have previously shown that basally-oriented deposition of a FN matrix restricts motility and promotes junctional stability in cultured endothelial cells and that this effect is tightly coupled to expression of FN. Here we report that TNC induces FN expression in endothelial cells...
October 6, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28975104/proliferative-glomerulonephritis-with-monoclonal-igg-deposits-an-unusual-cause-of-de-novo-disease-in-kidney-allograft
#10
Sabiha M Hussain, Kalathil K Sureshkumar
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a newly described and rare entity that can develop in native and very rarely in transplanted kidneys. We present a patient who developed de novo PGNMID in the kidney allograft along with a review of the literature. CASE PRESENTATION: A 38-year old female with type 1 diabetes who underwent successful simultaneous pancreas-kidney (SPK) transplantation 6 years earlier presented with rising serum creatinine, nephrotic range proteinuria and microhematuria...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28869938/mineral-and-bone-disorder-in-chronic-kidney-disease-a-case-report-from-vietnam
#11
Pham Van Bui, Nguyyen Minh Tuan, Huynh Thi Nguyen Nghia, Pham Nguyen Thanh Phuong
We report a case of calcium pyrophosphate dihydrate deposition disease (CPDD) involving a patient on maintenance hemodialysis (MHD). The 32-year-old man presented in August 2016 with a complaint of left shoulder swelling of 8 months' duration with no trauma or fever. He was diagnosed with nephrotic syndrome in 1998, which progressed to ESRD. He commenced MHD in 2012. Examination at our hospital revealed a soft nontender swelling of the left shoulder. Blood biochemistry showed elevated serum urate, phosphate, β2 microglobulin, and parathyroid hormone...
2017: Blood Purification
https://www.readbyqxmd.com/read/28838767/c4-nephritic-factors-in-c3-glomerulopathy-a-case-series
#12
Yuzhou Zhang, Nicole C Meyer, Fernando C Fervenza, Winnie Lau, Adam Keenan, Gabriel Cara-Fuentes, Dingwu Shao, Aalia Akber, Veronique Fremeaux-Bacchi, Sanjeev Sethi, Carla M Nester, Richard J H Smith
BACKGROUND: C3 glomerulopathy (C3G) defines a group of rare complement-mediated kidney diseases with a shared underlying pathophysiology: dysregulation of complement in the fluid phase and glomerular microenvironment. Dysregulation can be driven by autoantibodies to C3 and C5 convertases. STUDY DESIGN: Case series. SETTING & PARTICIPANTS: 168 patients with C3G (dense deposit disease, 68; C3 glumerulonephritis, 100) selected from our C3G biobank...
August 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28774457/conventional-radiology-in-crystal-arthritis-gout-calcium-pyrophosphate-deposition-and-basic-calcium-phosphate-crystals
#13
REVIEW
Thibaut Jacques, Paul Michelin, Sammy Badr, Michelangelo Nasuto, Guillaume Lefebvre, Neal Larkman, Anne Cotten
This article reviews the main radiographic features of crystal deposition diseases. Gout is linked to monosodium urate crystals. Classic radiographic features include subcutaneous tophi, large and well-circumscribed paraarticular bone erosions, and exuberant bone hyperostosis. Calcium pyrophosphate deposition (CPPD) can involve numerous structures, such as hyaline cartilages, fibrocartilages, or tendons. CPPD arthropathy involves joints usually spared by osteoarthritis. Basic calcium phosphate deposits are periarticular or intraarticular...
September 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28748900/dense-deposit-disease-in-a-child-with-febrile-sore-throat
#14
Giovanni Conti, Dominique De Vivo, Agata Vitale, Carmelo Fede, Domenico Santoro
Dense deposit disease or membranoproliferative glomerulonephritis type II is a rare glomerulopathy characterized on renal biopsy by deposition of abnormal electron-dense material in the glomerular basement membrane. The pathophysiologic basis is uncontrolled systemic activation of the alternate pathway of the complement cascade. C3 nephritic factor, an autoantibody directed against the C3 convertase of the alternate pathway, plays a key role. In some patients, complement gene mutations have been identified...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28748894/collagenofibrotic-collagen-type-iii-glomerulopathy-in-association-with-diabetic-nephropathy
#15
Khaled O Alsaad, Burhan Edrees, Khawla A Rahim, Abdulkareem Alanazi, Muawia Ahmad, Noura Aloudah
Collagenofìbrotic (collagen type III) glomerulopathy (CG) is a rare nonimmune-mediated glomerular disease. It is characterized by massive deposition of organized collagen type III fibers, which is localized in the mesangial and subendothelial glomerular areas and associated with increased serum levels of procollagen type III peptide. Association with systemic diseases and malignancies is extremely rare. Herein, we present a case of a nine-year-old girl, known case of type I diabetes mellitus, who presented with fever, nephrotic range proteinuria, generalized edema, and hypertension...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28745692/-changes-in-the-complement-system-in-membranoproliferative-glomerulonephritis
#16
V A Yurova, L A Bobrova, N L Kozlovskaya, Yu V Korotchaeva, A G Serova, L V Kozlov, S S Andina, K A Demyanova, A M Kuchieva, S V Roshchupkina
AIM: To compare the clinical manifestations membranoproliferative glomerulonephritis (MPGN) in its idiopathic variant, lupus nephritis (LN), and C3 glomerulopathy (C3-GP), by comparing them with changes in the complement system. SUBJECTS AND METHODS: The clinic of nephrology followed up 42 patients with different types of MPGN in 2013 to 2015. The study included 35 patients divided into 3 groups: 1) 8 patients with C3-GP, 2) 13 with idiopathic MPGN; 3) 14 with Class IV LN...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28729035/a-novel-cfhr1-cfhr5-hybrid-leads-to-a-familial-dominant-c3-glomerulopathy
#17
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...
October 2017: Kidney International
https://www.readbyqxmd.com/read/28716013/long-term-renal-survival-of-%C3%AE-3-heavy-chain-deposition-disease-a-case-report
#18
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
#19
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...
November 2017: Kidney International
https://www.readbyqxmd.com/read/28638323/sortilin-fragments-deposit-at-senile-plaques-in-human-cerebrum
#20
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
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