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Dialysis amyloidosis

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https://www.readbyqxmd.com/read/28774247/severe-destructive-tendinopathy-in-the-wrist-due-to-dialysis-related-amyloidosis
#1
Dongmin Kim, In Cheul Choi, Jong Woong Park
Dialysis-related amyloidosis (DRA) is a specific subtype of amyloidosis with several clinical presentations. Herein we report a case of severe destructive tendinopathy around the wrist associated with long-standing hemodialysis (HD). A 63-year-old female patient who had been on regular HD for 23 years suffered from symptoms of pain and a palpable mass around the wrist. Magnetic resonance imaging showed an ill-defined soft tissue mass around the extensor tendons that partially invaded the wrist joint. We performed surgical excision and tenolysis for the mass...
September 2017: Journal of Hand Surgery Asian-Pacific Volume
https://www.readbyqxmd.com/read/28758811/novel-pathologic-scoring-tools-predict-end-stage-kidney-disease-in-light-chain-al-amyloidosis
#2
Samuel Rubinstein, Robert F Cornell, Liping Du, Beatrice Concepcion, Stacey Goodman, Shelton Harrell, Sara Horst, Daniel Lenihan, David Slosky, Agnes Fogo, Anthony Langone
BACKGROUND AND OBJECTIVES: Light chain (AL) amyloidosis frequently involves the kidney, causing significant morbidity and mortality. A pathologic scoring system with prognostic utility has not been developed. We hypothesized that the extent of amyloid deposition and degree of scarring injury on kidney biopsy, could provide prognostic value, and aimed to develop pathologic scoring tools based on these features. METHODS: This is a case-control study of 39 patients treated for AL amyloidosis with biopsy-proven kidney involvement at a large academic medical center...
July 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28745926/safety-and-efficacy-of-empirical-interleukin-1-inhibition-using-anakinra-in-aa-amyloidosis-of-uncertain-aetiology
#3
Thirusha Lane, Ashutosh D Wechalekar, Julian D Gillmore, Philip N Hawkins, Helen J Lachmann
OBJECTIVE: AA amyloidosis is a serious complication of persistent inflammation, which, untreated will progress to renal failure and death. Effective suppression of the underlying inflammatory disease is the focus of treatment. However, in approximately 20% of cases the underlying condition remains uncertain, presenting a dilemma as to choice of treatment. METHODS: We conducted a retrospective study of a cohort of 11 patients diagnosed with AA amyloidosis of unknown aetiology, who had been empirically treated with anakinra...
July 26, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28745031/a-tale-of-two-tails-the-importance-of-unstructured-termini-in-the-aggregation-pathway-of-%C3%AE-2-microglobulin
#4
Rui J S Loureiro, Diogo Vila-Viçosa, Miguel Machuqueiro, Eugene I Shakhnovich, Patricia F N Faísca
The identification of intermediate states for folding and aggregation is important from a fundamental standpoint and for the design of novel therapeutic strategies targeted at conformational disorders. Protein human β2-microglobulin (HB2m) is classically associated with dialysis-related amyloidosis, but the single point mutant D76N was recently identified as the causative agent of a hereditary systemic amyloidosis affecting visceral organs. Here, we use D76N as a model system to explore the early stage of the aggregation mechanism of HB2m by means of an integrative approach framed on molecular simulations...
July 26, 2017: Proteins
https://www.readbyqxmd.com/read/28729034/prolonged-renal-survival-in-light-chain-amyloidosis-speed-and-magnitude-of-light-chain-reduction-is-the-crucial-factor
#5
Tamer Rezk, Helen J Lachmann, Marianna Fontana, Sajitha Sachchithanantham, Shameem Mahmood, Aviva Petrie, Carol J Whelan, Jennifer H Pinney, Darren Foard, Thirusha Lane, Taryn Youngstein, Ashutosh D Wechalekar, Paul Bass, Philip N Hawkins, Julian D Gillmore
Renal involvement causing progressive chronic kidney disease (CKD) is present in 70% of patients with systemic Ig light-chain (AL) amyloidosis at diagnosis. Chemotherapy that substantially suppresses free light chain production is associated with improved patient survival, but its benefit in delaying the onset of renal replacement therapy among patients who present with established advanced CKD has not been studied. To evaluate this, we studied 1000 patients enrolled in the prospective UK AL amyloidosis chemotherapy study (ALchemy)...
July 17, 2017: Kidney International
https://www.readbyqxmd.com/read/28657666/assessing-the-effect-of-d59p-mutation-in-the-de-loop-region-in-amyloid-aggregation-propensity-of-%C3%AE-2-microglobulin-a-molecular-dynamics-simulation-study
#6
Simranjeet Singh Narang, Suniba Shuaib, Deepti Goyal, Bhupesh Goyal
Dialysis-related amyloidosis (DRA) is a severe condition characterized by the accumulation of amyloidogenic β2-microglobulin (β2m) protein around skeletal joints and bones. The recent studies highlighted a critical role of the DE loop region for β2m stability and amyloid aggregation propensity. Despite significant efforts, the molecular mechanism of enhanced aggregation due to D59P mutation in the DE loop region remain elusive. In the present study, explicit-solvent molecular dynamics (MD) simulations were performed to examine the key changes in the structural and dynamic properties of wild type (wt) β2m upon D59P mutation...
June 28, 2017: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/28556529/a-multicenter-cross-sectional-study-for-bone-articular-lesions-associated-with-dialysis-related-amyloidosis-in-japan
#7
Shinichi Nishi, Junichi Hoshino, Suguru Yamamoto, Shunsuke Goto, Hideki Fuji, Yoshifumi Ubara, Yoshihiro Motomiya, Hiroyuki Morita, Kenmei Takaichi, Kunihiro Yamagata, Takashi Shigematsu, Mitsuharu Ueda, Yukio Ando
AIM: Dialysis-related amyloidosis (DRA) exhibits multiple bone-articular lesions, such as carpal tunnel syndrome (CTS), trigger finger (TF), spinal canal stenosis (SCS), destructive spondyloarthropathy (DSA), bone cysts, and joint pains. DRA leads to a decrease in activities of daily living (ADL). We investigated the initiation of CTS and TF, and evaluated the relationship between walking disturbances and bone-articular lesions or joint pains. METHODS: A multicenter cross-sectional study was performed...
May 26, 2017: Nephrology
https://www.readbyqxmd.com/read/28551910/-level-and-oxidation-of-beta-2-microglobulin-in-hemodialysis-patients-treated-with-intravenous-iron-during-dialysis-with-high-flux-compared-to-low-flux-dialyzers
#8
Regina Michelis, Shifra Sela, Alexander Lanzmann, Olga Tanchilevski, Revital Shurtz-Swirski, Batya Kristal
INTRODUCTION: Serum levels of β2-microglobulin (b2M) are significantly higher in patients with end stage renal failure undergoing hemodialysis (HD) and its accumulation accelerates Dialysis Related Amyloidosis (DRA). In HD patients low-flux dialysis, intravenous (IV) iron (administered for the treatment of anemia) affects ß2M removal during dialysis. IV iron also affects the oxidation of plasma proteins, including b2M. AIMS: To examine the effect of intravenous iron therapy on ß2M levels and oxidation in HD patients treated with high-flux compared with low-flux dialyzers...
May 2017: Harefuah
https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#9
Faisal M Huq Ronny, George Kleinman, Paul James Kurtin, John Thomas Fallon
Dialysis-related amyloidosis predominantly occurs in osteo-articular structures and dialysis-related amyloid (DRA) substances also deposit in extra-articular tissues. Clinical manifestations of DRA include odynophagia, gastrointestinal hemorrhage, intestinal obstruction, kidney stones, myocardial dysfunction, and subcutaneous tumors. The pathological characteristics of DRA in the heart of hemodialysis patients have rarely been reported. We report the case of a 73-year-old female with a history of cerebral palsy and end-stage renal disease status post two failed renal transplants who had been on hemodialysis for 30 years...
January 2017: Autopsy & case reports
https://www.readbyqxmd.com/read/28468825/small-molecule-mediated-inhibition-of-%C3%AE-2-microglobulin-based-amyloid-fibril-formation
#10
Tyler M Marcinko, Jia Dong, Raquel LeBlanc, Kate V Daborowski, Richard W Vachet
In dialysis patients, β-2 microglobulin (β2m) can aggregate and eventually form amyloid fibrils in a condition known as dialysis-related amyloidosis, which deleteriously affects joint and bone function. Recently, several small molecules have been identified as potential inhibitors of β2m amyloid formation in vitro Here we investigated whether these molecules are more broadly applicable inhibitors of β2m amyloid formation by studying their effect on Cu(II)-induced β2m amyloid formation. Using a variety of biophysical techniques, we also examined their inhibitory mechanisms...
June 23, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28462876/-aa-amyloidosis
#11
Katia Stankovic Stojanovic, Sophie Georgin-Lavialle, Gilles Grateau
AA amyloidosis remains one of the three main types of systemic amyloidosis with AL and ATTR. Its incidence has been however decreasing recently in Western countries. Chronic inflammatory diseases are currently the first cause of AA amyloidosis, including rheumatoid arthritis, spondyloarthritis and autoinflammatory diseases. Castleman's disease is a specific cause of AA amyloidosis that can be cured by surgery. A chronic inflammatory response is required to develop amyloidosis. Other genetic and environmental factors are also involved...
April 24, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28455257/molecular-insights-into-the-inhibitory-mechanism-of-rifamycin-sv-against-%C3%AE-2-microglobulin-aggregation-a-molecular-dynamics-simulation-study
#12
Simranjeet Singh Narang, Suniba Shuaib, Bhupesh Goyal
Dialysis-related amyloidosis (DRA) is a severe condition characterized by the accumulation of amyloidogenic β2-microglobulin (β2m) protein around skeletal joints and bones. The small molecules that modulate β2m aggregation have been identified in vitro, however, the underlying inhibitory mechanism remain elusive. In the present study, molecular docking and molecular dynamics (MD) simulations were performed to elucidate the inhibitory mechanism of an antibiotic, rifamycin SV (C1) reported for its in vitro anti-aggregation activity against β2m...
September 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28434302/fibrinogen-a-alpha-chain-amyloidosis-a-non-negligible-cause-of-chronic-kidney-disease-in-dialysis-patients
#13
Isabel Tavares, Luciana Moreira, Paulo Pinho Costa, Luísa Lobato
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434292/prognostication-of-survival-and-progression-to-dialysis-in-aa-amyloidosis
#14
Giovanni Palladini, Eloisa Riva, Marco Basset, Francesca Russo, Paolo Milani, Ettore Pasquinucci, Andrea Foli, Francesca Lavatelli, Mario Nuvolone, Simona Casarini, Laura Obici, Giampaolo Merlini
No abstract text is available yet for this article.
March 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28370245/renal-outcomes-in-patients-with-al-amyloidosis-prognostic-factors-renal-response-and-the-impact-of-therapy
#15
Efstathios Kastritis, Maria Gavriatopoulou, Maria Roussou, Magdalini Migkou, Despina Fotiou, Dimitrios C Ziogas, Nikos Kanellias, Evangelos Eleutherakis-Papaiakovou, Ioannis Panagiotidis, Stavroula Giannouli, Erasmia Psimenou, Smaragdi Marinaki, Theofanis Apostolou, Hariklia Gakiopoulou, Anna Tasidou, Ioannis Papassotiriou, Evangelos Terpos, Meletios A Dimopoulos
A staging system for patients with renal AL amyloidosis, based on eGFR (<50 ml/min/1.73 m(2) ) and proteinuria (≥5 g/day) at diagnosis, as well as criteria for renal progression (≥25% eGFR reduction) and response (≥30% reduction of proteinuria without renal progression) were recently proposed. We validated these criteria in a cohort of 125 patients with renal AL amyloidosis, mostly treated with bortezomib or lenalidomide. We confirmed the prognostic value of the renal staging system but also identified the limitations of renal progression criteria which are based only on eGFR reduction...
July 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28359658/unrecognized-fibrinogen-a-%C3%AE-chain-amyloidosis-results-from%C3%A2-targeted-genetic-testing
#16
Isabel Tavares, João Paulo Oliveira, Ana Pinho, Luciana Moreira, Liliana Rocha, Josefina Santos, Joaquim Pinheiro, Paulo Pinho Costa, Luísa Lobato
BACKGROUND: Fibrinogen A α-chain (AFib) amyloidosis results from autosomal-dominant mutations in the gene encoding AFib (FGA). Patients with this disorder typically present with proteinuria. Isolated cases of AFib amyloidosis, carrying the FGA p.Glu545Val variant, were identified in the district of Braga, in northwest Portugal. This observation led us to hypothesize that this disorder might be an unrecognized cause of kidney disease in that region and prompted us to carry out targeted genetic testing for the p...
August 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28272335/proline-residues-as-switches-in-conformational-changes-leading-to-amyloid-fibril-formation
#17
Ajda Taler-Verčič, Samra Hasanbašić, Selma Berbić, Veronika Stoka, Dušan Turk, Eva Žerovnik
Here we discuss studies of the structure, folding, oligomerization and amyloid fibril formation of several proline mutants of human stefin B, which is a protein inhibitor of lysosomal cysteine cathepsins and a member of the cystatin family. The structurally important prolines in stefin B are responsible for the slow folding phases and facilitate domain swapping (Pro 74) and loop swapping (Pro 79). Moreover, our findings are compared to β₂-microglobulin, a protein involved in dialysis-related amyloidosis...
March 7, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28264226/dialysis-related-amyloidosis-is-it-gone-or-should-it-be
#18
EDITORIAL
Laura Labriola, Michel Jadoul
The prevalence and severity of dialysis-related amyloidosis (DRA) appear to have decreased significantly over the last two decades, although recent, large-scale epidemiological studies show that DRA continues to occur. Recent experimental findings have documented a direct cellular toxicity of β2microglobulin (β2m) fibrils but the mechanisms of β2m fibrillogenesis remain incompletely understood. Although a high plasma concentration of β2m is still considered as a prerequisite for developing DRA, other factors have been clearly incriminated such as older age at dialysis onset and longer dialysis vintage, or suspected effects such as proinflammatory effects of bioincompatible dialysis techniques...
May 2017: Seminars in Dialysis
https://www.readbyqxmd.com/read/28261626/influence-of-heparin-molecular-size-on-the-induction-of-c-terminal-unfolding-in-%C3%AE-2-microglobulin
#19
Kanon Fukasawa, Yuichiro Higashimoto, Yoshihiro Motomiya, Yoshinori Uji, Yukio Ando
Dialysis-related amyloidosis (DRA) is characterized by accumulation of amyloid β2- microglobulin (β2m) in the interstitial matrix. Matrix substances such as heparin have reportedly been strongly implicated in the pathogenesis of dialysis-related amyloidosis. In clinical setting of hemodialysis, two types of heparin, i.e., high and low molecular heparin (H.M.H. and L.M.H.) have been routinely used. Still commonly used is H.M.H., followed by L.M.H. preparations with distinct advantages. Here, we studied that the interaction of native and two amyloidogenic β2m variants: ΔN6β2m and D76N β2m with H...
December 2016: Molecular Biology Research Communications
https://www.readbyqxmd.com/read/28249361/heparin-induced-amyloid-fibrillation-of-%C3%AE-2-microglobulin-explained-by-solubility-and-a-supersaturation-dependent-conformational-phase-diagram
#20
Masatomo So, Yasuko Hata, Hironobu Naiki, Yuji Goto
Amyloid fibrils are fibrillar deposits of denatured proteins associated with amyloidosis and are formed by a nucleation and growth mechanism. We revisited an alternative and classical view of amyloid fibrillation: amyloid fibrils are crystal-like precipitates of denatured proteins formed above solubility upon breaking supersaturation. Various additives accelerate and then inhibit amyloid fibrillation in a concentration-dependent manner, suggesting that the combined effects of stabilizing and destabilizing forces affect fibrillation...
May 2017: Protein Science: a Publication of the Protein Society
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