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Dialysis amyloidosis

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https://www.readbyqxmd.com/read/29136724/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-renal-impairment-of-multiple-myeloma
#1
(no author information available yet)
Renal impairment (RI) is a common complication of multiple myeloma (MM), which is presented as chronic kidney disease (CKD) or acute kidney injury (AKI). The typical pathological feature is cast nephropathy. Presently international system staging (ISS) is used in evaluating MM. Although the classic Durie-Salmon staging system could be still used in clinical practice, it may miss out some patients with renal impairment. For evaluations of RI in MM patients with CKD, it's recommended to assess the estimated glomerular filtration rate (eGFR) by creatinine based formula CKD-epidemiology collaboration (EPI) or modification of diet in renal disease(MDRD) and to stage the renal injuries according to 2013 Kidney Disease Improving Global Outcomes (KDIGO) CKD guidelines...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29116115/characterization-of-apoj-reconstituted-high-density-lipoprotein-rhdl-nanodisc-for-the-potential-treatment-of-cerebral-%C3%AE-amyloidosis
#2
Sofía Fernández-de-Retana, Mary Cano-Sarabia, Paula Marazuela, Jose Luis Sánchez-Quesada, Annabel Garcia-Leon, Alex Montañola, Joan Montaner, Daniel Maspoch, Mar Hernández-Guillamon
Cerebral β-amyloidosis is a major feature of Alzheimer's disease (AD), characterized by the accumulation of β-amyloid protein (Aβ) in the brain. Several studies have implicated lipid/lipoprotein metabolism in the regulation of β-amyloidosis. In this regard, HDL (High Density Lipoprotein)-based therapies could ameliorate pathological features associated with AD. As apolipoprotein J (ApoJ) is a natural chaperone that interacts with Aβ, avoiding its aggregation and toxicity, in this study we propose to prepare reconstituted rHDL-rApoJ nanoparticles by assembling phospholipids with recombinant human ApoJ (rApoJ)...
November 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28960840/selection-of-dna-aptamer-that-blocks-the-fibrillogenesis-of-a-proteolytic-amyloidogenic-fragment-of-%C3%AE-2-m
#3
Kanon Fukasawa, Yuichiro Higashimoto, Yukio Ando, Yoshihiro Motomiya
Dialysis-related amyloidosis (DRA) is a severe complication of hemodialysis that results in progressive destruction of bones and joints. Elevated concentrations of the β2 -microglobulin (β2 m) level in the serum of subjects on hemodialysis promote the formation of amyloid fibrils in osteoarticular tissues. β2 m lacking the N-terminal six residues of the mature protein (ΔN6β2 m) constitutes 25-30% of β2 m in ex vivo DRA amyloid. Unlike full-length wild-type β2 m, ΔN6β2 m forms amyloid fibrils at neutral pH in vitro...
September 28, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28919633/clinical-presentation-and-outcomes-in-light-chain-amyloidosis-patients-with-non-evaluable-serum-free-light-chains
#4
S Sidana, N Tandon, A Dispenzieri, M A Gertz, F K Buadi, M Q Lacy, D Dingli, A L Fonder, S R Hayman, M A Hobbs, W I Gonsalves, Y L Hwa, P Kapoor, R A Kyle, N Leung, R S Go, J A Lust, S J Russell, S R Zeldenrust, S V Rajkumar, S K Kumar
Hematologic response criteria in light chain (AL) amyloidosis require the difference in involved and uninvolved free light chains (dFLC) to be at least 5 mg/dl. We describe the clinical presentation and outcomes of newly diagnosed amyloidosis patients with dFLC <5 mg/dl (non-evaluable dFLC; 14%, n=165) compared with patients with dFLC ⩾5 mg/dl (evaluable dFLC; 86%, n=975). Patients with non-evaluable dFLC had less cardiac involvement (40% vs 80%, P<0.001), less liver involvement (11% vs 17%, P=0...
September 18, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28894888/-posttherapeutic-changes-in-bone-marrow
#5
REVIEW
T Geith, A-C Stellwag, A Baur-Melnyk
The bone marrow basically consists of red blood-forming bone marrow and yellow fat. In the skeleton, there is an age-dependent distribution of these two parts. In the context of medical interventions or therapies, bone marrow changes can occur, whereby the normal bone marrow can basically be replaced by fat, edema, or fibrosis/sclerosis. Here, specific signal intensities and patterns are shown in imaging. After irradiation therapies, edematous changes, hemorrhages, and osteoradionecroses are observed. Likewise, insufficiency fractures, impairment of the growth gaps, or the development of tumors is possible...
November 2017: Der Radiologe
https://www.readbyqxmd.com/read/28882602/neurosurgical-aspects-of-dialysis-related-spinal-amyloidosis-report-of-three-cases-and-a-review-of-the-literature
#6
M Dalolio, F Lucarella, P Rampini, G P Bulfamante, S Aldea, P Graveleau, S Gaillard, P Scarone
BACKGROUND AND PURPOSE: Osteoarticular manifestations of beta-2 microglobulin amyloidosis are often diagnosed in long-term dialyzed patients. However, spinal involvement is rare (10-25% of patients), and generally not associated with neurological deterioration. Compression of the spinal cord or roots is extremely rare, and probably under-recognized. METHODS: The authors describe three cases of spinal stenosis presenting with neurological signs in long-term dialyzed patients, prospectively collected over 2 years in two different institutions and treated by surgical decompression...
September 4, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28874122/doxycycline-treatment-in-dialysis-related-amyloidosis-discrepancy-between-antalgic-effect-and-inflammation-studied-with-fdg-positron-emission-tomography-a-case-report
#7
Giorgina Barbara Piccoli, Mammar Hachemi, Ida Molfino, Jean Philippe Coindre, Charles Boursot
BACKGROUND: No effective treatment is currently available and dialysis related amyloidosis continues to be invalidating in long-term dialysis patients. A recent case series reported reduction of osteoarticular pain on doxycycline treatment, extending the indications of this drug, used in other uncommon forms of amyloidosis, to dialysis patients. Explanations of the antalgic effect were the anti-inflammatory properties and anti-coiling effects of tetracycline. CASE PRESENTATION: Our report regards a 54-year-old woman, who was never transplanted and has been on hemodialysis and hemodiafiltration for overall 37 years, due to renal hypoplasia...
September 6, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28865972/high-dose-melphalan-and-stem-cell-transplantation-in-patients-on-dialysis-due-to-immunoglobulin-light-chain-amyloidosis-and-monoclonal-immunoglobulin-deposition-disease
#8
Felipe Batalini, Laura Econimo, Karen Quillen, J Mark Sloan, Shayna Sarosiek, Dina Brauneis, Andrea Havasi, Lauren Stern, Laura Dember, Vaishali Sanchorawala
The kidney is the most common organ affected by immunoglobulin light-chain (AL) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD), often leading to end-stage renal disease (ESRD). High-dose melphalan and stem cell transplantation (HDM/SCT) is effective for selected patients with AL amyloidosis, with high rates of complete hematologic response and potential for improved organ dysfunction. Data on tolerability and response to HDM/SCT in patients with ESRD due to AL amyloidosis and MIDD are limited...
September 1, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28859624/living-kidney-transplantation-between-brothers-with-unrecognized-renal-amyloidosis-as-the-first-manifestation-of-familial-mediterranean-fever-a-case-report
#9
Ramón Peces, Sara Afonso, Carlos Peces, Julián Nevado, Rafael Selgas
BACKGROUND: Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent episodes of fever and polyserositis and by the onset of reactive amyloid-associated amyloidosis. Amyloidosis due to familial Mediterranean fever can lead to end-stage renal disease, culminating in kidney transplantation for some patients. In this study, we report the clinical outcome of two brothers with familial Mediterranean fever who were the inadvertent donor and recipient, respectively, of a kidney...
August 31, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28774247/severe-destructive-tendinopathy-in-the-wrist-due-to-dialysis-related-amyloidosis
#10
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
#11
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
#12
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
#13
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...
November 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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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