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https://www.readbyqxmd.com/read/29329521/treating-c3-glomerulopathy-with-eculizumab
#1
Thomas Welte, Frederic Arnold, Julia Kappes, Maximilian Seidl, Karsten Häffner, Carsten Bergmann, Gerd Walz, Elke Neumann-Haefelin
BACKGROUND: C3 glomerulopathy (C3G) is a rare, but severe glomerular disease with grim prognosis. The complex pathogenesis is just unfolding, and involves acquired as well as inherited dysregulation of the alternative pathway of the complement cascade. Currently, there is no established therapy. Treatment with the C5 complement inhibitor eculizumab may be a therapeutic option. However, due to rarity of the disease, parameters predicting treatment response remain largely unknown. METHODS: Seven patients with C3G (five with C3 glomerulonephritis and two with dense deposit disease) were treated with eculizumab...
January 12, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29312858/de-novo-glomerular-diseases-after-renal-transplantation-how-is-it-different-from-recurrent-glomerular-diseases
#2
REVIEW
Fedaey Abbas, Mohsen El Kossi, Jon Kim Jin, Ajay Sharma, Ahmed Halawa
The glomerular diseases after renal transplantation can occur de novo, i.e., with no relation to the native kidney disease, or more frequently occur as a recurrence of the original disease in the native kidney. There may not be any difference in clinical features and histological pattern between de novo glomerular disease and recurrence of original glomerular disease. However, structural alterations in transplanted kidney add to dilemma in diagnosis. These changes in architecture of histopathology can happen due to: (1) exposure to the immunosuppression specifically the calcineurin inhibitors (CNI); (2) in vascular and tubulointerstitial alterations as a result of antibody mediated or cell-mediated immunological onslaught; (3) post-transplant viral infections; (4) ischemia-reperfusion injury; and (5) hyperfiltration injury...
December 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/29310824/c3-glomerulonephritis-and-dense-deposit-disease-share-a-similar-disease-course-in-a-large-united-states-cohort-of-patients-with-c3-glomerulopathy
#3
Andrew S Bomback, Dominick Santoriello, Rupali S Avasare, Renu Regunathan-Shenk, Pietro A Canetta, Wooin Ahn, Jai Radhakrishnan, Maddalena Marasa, Paul E Rosenstiel, Leal C Herlitz, Glen S Markowitz, Vivette D D'Agati, Gerald B Appel
C3 glomerulonephritis (C3GN) and dense deposit disease comprise the two classes of C3 glomerulopathy. Studies from Europe and Asia have aided our understanding of this recently defined disorder, but whether these data apply to a diverse United States patient population remains unclear. We, therefore, reviewed clinical and histopathological data, including generation of a C3 Glomerulopathy Histologic Index to score biopsy activity and chronicity, to determine predictors of progression to end-stage renal disease (ESRD) and advanced chronic kidney disease (CKD) in 111 patients (approximately 35% non-white) with C3 glomerulopathy: 87 with C3GN and 24 with dense deposit disease...
January 6, 2018: Kidney International
https://www.readbyqxmd.com/read/29310419/proliferative-lupus-nephritis-in-the-absence-of-overt-systemic-lupus-erythematosus-a-historical-study-of-12-adult-patients
#4
Maxime Touzot, Cécile Saint-Pastou Terrier, Stanislas Faguer, Ingrid Masson, Hélène François, Lionel Couzi, Aurélie Hummel, Nathalie Quellard, Guy Touchard, Noémie Jourde-Chiche, Jean-Michel Goujon, Eric Daugas
Severe lupus nephritis in the absence of systemic lupus erythematosus (SLE) is a rare condition with an unclear clinical presentation and outcome.We conducted a historical observational study of 12 adult (age >18 years) patients with biopsy-proven severe lupus nephritis or lupus-like nephritis without SLE immunological markers at diagnosis or during follow-up. Excluded were patients with chronic infections with HIV or hepatitis B or C; patients with a bacterial infectious disease; and patients with pure membranous nephropathy...
December 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29245053/influence-of-diabetes-on-the-foreign-body-response-to-nitric-oxide-releasing-implants
#5
Robert J Soto, Elizabeth P Merricks, Dwight A Bellinger, Timothy C Nichols, Mark H Schoenfisch
The foreign body response (FBR) to nitric oxide (NO)-releasing subcutaneous implants was compared between healthy and streptozotocin-induced diabetic swine by evaluating inflammation, collagen capsule formation, and angiogenesis. Steel wire substrates were first modified with polyurethane membranes capable of diverse NO-release kinetics (NO fluxes and release durations of 0.8-630.0 pmol cm-2 s-1 and 2-13 d, respectively). The NO-releasing materials were implanted in the subcutis for 3, 10, or 25 d for histological and immunohistochemical evaluation of the FBR...
November 30, 2017: Biomaterials
https://www.readbyqxmd.com/read/29237409/iga-nephropathy-featuring-massive-wire-loop-like-deposits-in-two-patients-with-alcoholic-cirrhosis
#6
Daisuke Takada, Keiichi Sumida, Akinari Sekine, Ryo Hazue, Masayuki Yamanouchi, Tatsuya Suwabe, Noriko Hayami, Junichi Hoshino, Naoki Sawa, Kenmei Takaichi, Takeshi Fujii, Kenichi Ohashi, Yoshifumi Ubara
BACKGROUND: Various renal manifestations are known to develop in patients with liver disease, including chronic hepatitis and cirrhosis. CASE PRESENTATION: We evaluated renal disease in two 47-year-old Japanese men with liver cirrhosis and chronic alcoholism for 34 years and 27 years, respectively. Renal biopsy demonstrated massive wire loop-like deposits in the subendothelial space of the glomerular basement membrane and in the mesangium. However, immunofluorescence was only positive for IgA and C3, and electron microscopy did not reveal any organized structures in the electron-dense deposits...
December 13, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/29220360/substitutions-of-prp-n-terminal-histidine-residues-modulate-scrapie-disease-pathogenesis-and-incubation-time-in-transgenic-mice
#7
Sabina Eigenbrod, Petra Frick, Uwe Bertsch, Gerda Mitteregger-Kretzschmar, Janina Mielke, Marko Maringer, Niklas Piening, Alexander Hepp, Nathalie Daude, Otto Windl, Johannes Levin, Armin Giese, Vignesh Sakthivelu, Jörg Tatzelt, Hans Kretzschmar, David Westaway
Prion diseases have been linked to impaired copper homeostasis and copper induced-oxidative damage to the brain. Divalent metal ions, such as Cu2+ and Zn2+, bind to cellular prion protein (PrPC) at octapeptide repeat (OR) and non-OR sites within the N-terminal half of the protein but information on the impact of such binding on conversion to the misfolded isoform often derives from studies using either OR and non-OR peptides or bacterially-expressed recombinant PrP. Here we created new transgenic mouse lines expressing PrP with disrupted copper binding sites within all four histidine-containing OR's (sites 1-4, H60G, H68G, H76G, H84G, "TetraH>G" allele) or at site 5 (composed of residues His-95 and His-110; "H95G" allele) and monitored the formation of misfolded PrP in vivo...
2017: PloS One
https://www.readbyqxmd.com/read/29208248/kidney-diseases-associated-with-alternative-complement-pathway-dysregulation-and-potential-treatment-options
#8
REVIEW
Prateek Sanghera, Mythili Ghanta, Fatih Ozay, Venkatesh K Ariyamuthu, Bekir Tanriover
Atypical hemolytic uremic syndrome and C3 glomerulopathy (dense deposit disease and C3 glomerulonephritis) are characterized as inappropriate activation of the alternative complement pathway. Genetic mutations affecting the alternative complement pathway regulating proteins (complement factor H, I, membrane cofactor protein and complement factor H-related proteins) and triggers (such as infection, surgery, pregnancy and autoimmune disease flares) result in the clinical manifestation of these diseases. A decade ago, prognosis of these disease states was quite poor, with most patients developing end-stage renal disease...
December 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29208205/dense-deposit-disease-associated-with-multiple-myeloma%C3%A2
#9
Ahmed M Alkhunaizi, Adil A AlKhatti, Samih H Nasr, Mariam P Alexander
Dense deposit disease (DDD) is a very rare pathological finding associated with activation of the final complement pathway. DDD has been rarely found in association with multiple myeloma (MM). Here we report a patient with MM presenting with renal failure, anemia, and bone pain. The renal biopsy showed DDD. The patient received anti-myeloma treatment and responded well. The case is discussed and literature reviewed.
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December 6, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29191133/focal-segmental-glomerulosclerosis-in-related-miniature-schnauzer-dogs
#10
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
#11
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/29151252/dominant-c3-glomerulopathy-new-roles-for-an-old-actor-in-renal-pathology
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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