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Dialysis amyloidosis

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https://www.readbyqxmd.com/read/27846001/unexpected-diffuse-18f-naf-uptake-in-the-lung-parenchyma-in-a-patient-with-severe-hypercalcemia-due-to-myelomatosis
#1
Ali Asmar, Lene Simonsen, Birgitte Svolgaard, Jens Bülow
An 84-year-old man was admitted to the intensive care unit because of hypercalcemic crisis leading to acute renal failure needing dialysis. The patient had no other history of illness. However, because prostate-specific antigen levels were increased, the patient was referred to F-NaF PET/CT on suspicion of active skeletal metastases. The patient was finally diagnosed with myelomatosis. Although the skeletal uptake of F-NaF was without signs of focal metastasis, the F-NaF PET/CT scanning surprisingly revealed diffuse high accumulation of F-NaF in the lung parenchyma, possibly because of calcium deposition in the lung parenchyma associated to amyloidosis seen in patients with myelomatosis...
January 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27575781/continuous-ambulatory-peritoneal-dialysis-as-a-promising-therapy-for-light-chain-amyloidosis-with-congestive-heart-failure
#2
Julio Núñez, Anabel Teruel, Carmen Quiñones-Torrelo, Sergio García-Blas, Arturo Carratalá, Gema Miñana, Enrique Santas, Isidro Torregrosa, Carlos Solano, Miguel González
No abstract text is available yet for this article.
November 15, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27569937/comparative-analysis-of-outcomes-of-kidney-transplantation-in-patients-with-aa-amyloidosis-and-chronic-glomerulonephritis
#3
T Sahutoglu, K Atay, Y Caliskan, E Kara, H Yazici, A Turkmen
BACKGROUND: Amyloid A (AA) amyloidosis is a multisystemic, progressive, and severe disease. Renal involvement is a prominent feature of the disease, and the outcome of patients on dialysis is poor. We aimed to analyze the outcomes of kidney transplantation in patients with AA amyloidosis in comparison with chronic glomerulonephritis (CGN). METHODS: Charts of patients who underwent kidney transplantation between 1988 and 2012 were reviewed; 41 patients with AA amyloidosis were identified, and 41 age- and sex-matched control patients with chronic CGN were included...
July 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27501122/outcomes-of-patients-with-renal-monoclonal-immunoglobulin-deposition-disease
#4
Taxiarchis V Kourelis, Samih H Nasr, Angela Dispenzieri, Shaji K Kumar, Morie A Gertz, Fernando C Fervenza, Francis K Buadi, Martha Q Lacy, Stephen B Erickson, Fernando G Cosio, Prashant Kapoor, John A Lust, Suzanne R Hayman, Vincent Rajkumar, Steven R Zeldenrust, Stephen J Russell, David Dingli, Yi Lin, Wilson Gonsalves, Elizabeth C Lorenz, Ladan Zand, Robert A Kyle, Nelson Leung
Recent reports suggest that deep hematologic responses to chemotherapy are associated with improved renal outcomes in monoclonal immunoglobulin deposition disease (MIDD). Here we describe the long term outcomes and identify prognostic factors after first line treatment of the largest reported series of patients with MIDD. Between March 1992 and December 2014, 88 patients with MIDD were seen at Mayo Clinic, MN. Renal responses were defined using criteria used for light chain amyloidosis (AL) or those used by the IMWG...
November 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27491541/hemodynamic-deterioration-after-aortic-valve-replacement-in-a-patient-with-mixed-systemic-amyloidosis
#5
Tatsuya Seki, Atsuo Hattori, Toshihito Yoshida
We report a case of hemodynamic deterioration after aortic valve replacement in a patient with mixed systemic amyloidosis. A 77-year-old male with severe aortic valve stenosis and 19 years hemodialysis underwent aortic valve replacement. Postoperatively, the patient died of hemodynamic deterioration. Autopsy findings showed massive, whole-body edema and mixed systemic amyloidosis (dialysis-related and AA amyloidosis). Clinical and autopsy findings implied that hemodynamic deterioration was caused by increased vascular permeability...
August 4, 2016: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/27467899/characterization-of-salt-induced-oligomerization-of-human-%C3%AE-2-microglobulin-at-low-ph
#6
Dominic Narang, Anubhuti Singh, Hema M Swasthi, Samrat Mukhopadhyay
Misfolding and amyloid aggregation of human β2-microglobulin (β2m) have been linked to dialysis-related amyloidosis. Previous studies have shown that in the presence of different salt concentrations and at pH 2.5, β2m assembles into aggregates with distinct morphologies. However, the structural and mechanistic details of the aggregation of β2m, giving rise to different morphologies, are poorly understood. In this work, we have extensively characterized the salt-induced oligomers of the acid-unfolded state of β2m using an array of biophysical tools including steady-state and time-resolved fluorescence, circular dichroism, dynamic light scattering, and atomic force microscopy imaging...
August 18, 2016: Journal of Physical Chemistry. B
https://www.readbyqxmd.com/read/27453712/confronting-practical-problems-for-initiation-of-on-line-hemodiafiltration-therapy
#7
REVIEW
Yang Wook Kim, Sihyung Park
Conventional hemodialysis, which is based on the diffusive transport of solutes, is the most widely used renal replacement therapy. It effectively removes small solutes such as urea and corrects fluid, electrolyte and acid-base imbalance. However, solute diffusion coefficients decreased rapidly as molecular size increased. Because of this, middle and large molecules are not removed effectively and clinical problem such as dialysis amyloidosis might occur. Online hemodiafiltration which is combined by diffusive and convective therapies can overcome such problems by removing effectively middle and large solutes...
June 2016: Electrolyte & Blood Pressure: E & BP
https://www.readbyqxmd.com/read/27340120/renal-amyloid-a-amyloidosis-as-a-complication-of-hidradenitis-suppurativa
#8
Kristine D Schandorff, Iben M Miller, Dorrit Krustrup, Gregor B E Jemec, Peter Marckmann
Rheumatic disease is the dominant cause of amyloid A (AA) amyloidosis, but other chronic inflammatory diseases may have similar consequences. Hidradenitis suppurativa (HS) is a relatively common, but little known skin disease characterized by chronic inflammation. Here we present a case of chronic HS leading to biopsy-verified severe renal AA amyloidosis and dialysis dependency.
July 2016: Clinical Nephrology
https://www.readbyqxmd.com/read/27339443/a-clinical-staging-score-to-measure-the-severity-of-dialysis-related-amyloidosis
#9
Junichi Hoshino, Masahiro Kawada, Aya Imafuku, Koki Mise, Keiichi Sumida, Rikako Hiramatsu, Eiko Hasegawa, Noriko Hayami, Masayuki Yamanouchi, Tatsuya Suwabe, Naoki Sawa, Yoshifumi Ubara, Kenmei Takaichi
BACKGROUND: The ongoing effort to prevent dialysis-related amyloidosis (DRA) has been hampered by lack of any way to measure DRA's severity. Yet, such measurement is essential for assessing the effect of DRA treatment. Accordingly, we developed a scoring system focused on the physical manifestations of DRA. METHODS: Forty-four patients on maintenance hemodialysis with DRA, and 96 without it, were enrolled. The SF-36v2 Health Survey ascertained whether patients experienced general bodily pain and/or physical dysfunction with any attendant specific pain (dysfunction)...
June 23, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27225299/stepwise-unfolding-of-human-%C3%AE-2-microglobulin-into-a-disordered-amyloidogenic-precursor-at-low-ph
#10
Dominic Narang, Anubhuti Singh, Samrat Mukhopadhyay
Amyloid fibril formation by human β2-microglobulin (β2m) is associated with dialysis-related amyloidosis. In order to understand the mechanism of protein misfolding, it is important to characterize the nature and properties of various intermediates formed during protein unfolding. In this work, we studied the effect of pH change on the unfolding of β2m using a range of spectroscopic readouts. In order to investigate the local structural changes, we created single tryptophan (W60 and W95) mutants of β2m...
May 25, 2016: European Biophysics Journal: EBJ
https://www.readbyqxmd.com/read/27208169/the-role-of-fibrinogen-glycation-in-attr-evidence-for-chaperone-activity-loss-in-disease
#11
Daniel Fonseca, Samuel Gilberto, Cristina Ribeiro-Silva, Raquel Ribeiro, Inês Batista Guinote, Susana Saraiva, Ricardo A Gomes, Élia Mateus, Ana Viana, Eduardo Barroso, Ana Ponces Freire, Patrick Freire, Carlos Cordeiro, Gonçalo da Costa
Transthyretin amyloidosis (ATTR) belongs to a class of disorders caused by protein misfolding and aggregation. ATTR is a disabling disorder of autosomal dominant trait, where transthyretin (TTR) forms amyloid deposits in different organs, causing dysfunction of the peripheral nervous system. We previously discovered that amyloid fibrils from ATTR patients are glycated by methylglyoxal. Even though no consensus has been reached about the actual role of methylglyoxal-derived advanced glycation end-products in amyloid diseases, evidence collected so far points to a role for protein glycation in conformational abnormalities, being ubiquitously found in amyloid deposits in Alzheimer's disease, dialysis-related amyloidosis and Parkinson's diseases...
July 15, 2016: Biochemical Journal
https://www.readbyqxmd.com/read/27183092/effect-of-severe-hypoalbuminemia-on-toxicity-of-high-dose-melphalan-and-autologous-stem-cell-transplantation-in-patients-with-al-amyloidosis
#12
S Y Lee, R S Meehan, D C Seldin, J M Sloan, K Quillen, A Shelton, D Brauneis, V Sanchorawala
High-dose melphalan with stem cell transplantation (HDM/SCT) extends survival and induces hematologic and clinical responses in patients with light chain (AL) amyloidosis. Eighty percent of melphalan is bound to plasma proteins (60% albumin-bound). We hypothesized that patients with profound hypoalbuminemia have a greater free melphalan fraction and more toxicity. Patients with AL amyloidosis treated with HDM/SCT between 2011 and 2014 with severe hypoalbuminemia (SH), defined as serum albumin ⩽2 g/dL were studied retrospectively...
May 16, 2016: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/27150430/rational-design-of-mutations-that-change-the-aggregation-rate-of-a-protein-while-maintaining-its-native-structure-and-stability
#13
Carlo Camilloni, Benedetta Maria Sala, Pietro Sormanni, Riccardo Porcari, Alessandra Corazza, Matteo De Rosa, Stefano Zanini, Alberto Barbiroli, Gennaro Esposito, Martino Bolognesi, Vittorio Bellotti, Michele Vendruscolo, Stefano Ricagno
A wide range of human diseases is associated with mutations that, destabilizing proteins native state, promote their aggregation. However, the mechanisms leading from folded to aggregated states are still incompletely understood. To investigate these mechanisms, we used a combination of NMR spectroscopy and molecular dynamics simulations to compare the native state dynamics of Beta-2 microglobulin (β2m), whose aggregation is associated with dialysis-related amyloidosis, and its aggregation-resistant mutant W60G...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27116248/long-term-prognosis-of-al-and-aa-renal-amyloidosis-a-japanese-single-center-experience
#14
Masatoyo Ozawa, Atsushi Komatsuda, Hiroshi Ohtani, Mizuho Nara, Ryuta Sato, Masaru Togashi, Naoto Takahashi, Hideki Wakui
BACKGROUND: Few studies have been conducted on the long-term prognosis of patients with amyloid light chain (AL) and amyloid A (AA) renal amyloidosis in the same cohort. METHODS: We retrospectively examined 68 patients with biopsy-proven renal amyloidosis (38 AL and 30 AA). Clinicopathological findings at the diagnosis and follow-up data were evaluated in each patient. We analyzed the relationship between clinicopathological parameters and survival data. RESULTS: Significant differences were observed in several clinicopathological features, such as proteinuria levels, between the AL and AA groups...
April 26, 2016: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/27061684/we-use-impure-water-to-make-dialysate-for-hemodialysis
#15
Ashish Upadhyay, Bertrand L Jaber
Patients receiving hemodialysis are exposed to a large volume of water, used to prepare dialysate for each treatment session. Technological advancements now make it possible to generate ultrapure dialysate that has substantially lower bacterial and endotoxin counts than the standard dialysate used in the United States. Low-level water contamination is thought to propagate a state of chronic inflammation seen in hemodialysis patients, and a number of studies demonstrate that the use of ultrapure dialysate has a favorable effect on laboratory parameters of inflammation, nutrition, erythropoietin responsiveness, dialysis-associated amyloidosis, and atherosclerosis...
July 2016: Seminars in Dialysis
https://www.readbyqxmd.com/read/26757617/a-systematic-molecular-dynamics-approach-to-the-structural-characterization-of-amyloid-aggregation-propensity-of-%C3%AE-2-microglobulin-mutant-d76n
#16
P Chandrasekaran, R Rajasekaran
Beta-2 microglobulin (β2m) is an amyloidogenic protein belongs to the immunoglobulin superfamily, responsible for the dialysis-related amyloidosis (DRA). Misfolding of β2m is a prerequisite to the formation of systemic amyloidosis that has an effect on the structure and function of the affected organ. The aim of our present study is to intensively explore the structural characterization of amyloid aggregation propensity of recently identified natural mutation D76N by applying the classical molecular dynamics (MD) approach...
March 2016: Molecular BioSystems
https://www.readbyqxmd.com/read/26728585/trends-in-survival-and-renal-recovery-in-patients-with-multiple-myeloma-or-light-chain-amyloidosis-on-chronic-dialysis
#17
Alexandre Decourt, Bertrand Gondouin, Jean Christophe Delaroziere, Philippe Brunet, Marion Sallée, Stephane Burtey, Bertrand Dussol, Vadim Ivanov, Regis Costello, Cecile Couchoud, Noemie Jourde-Chiche
BACKGROUND AND OBJECTIVES: Monoclonal gammopathies (MGs) with renal involvement can lead to ESRD caused by myeloma cast nephropathy (MCN), immunoglobulin light chain amyloidosis (ALA), or light-chain deposition disease (LCDD). Few studies have focused on the prognosis of patients with MG on chronic dialysis. We evaluated the outcomes of patients with MG incident on chronic dialysis in France. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: All incident patients registered in the Renal Epidemiology and Information Network Registry between 2002 and 2011 with ESRD caused by ALA, LCDD, or MCN were included...
March 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/26627634/the-impact-of-dialysis-on-the-survival-of-patients-with-immunoglobulin-light-chain-al-amyloidosis-undergoing-autologous-stem-cell-transplantation
#18
Nelson Leung, Shaji K Kumar, Siobhan V Glavey, Angela Dispenzieri, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, David Dingli, Prashant Kapoor, Steven R Zeldenrust, Stephen J Russell, John A Lust, William J Hogan, S Vincent Rajkumar, Dennis A Gastineau, Taxiarchis V Kourelis, Yi Lin, Wilson I Gonsalves, Ronald S Go, Morie A Gertz
BACKGROUND: Acute renal failure requiring dialysis is associated with high mortality during autologous stem cell transplantation (ASCT). This study examined the association between acute renal failure and mortality in immunoglobulin light chain (AL) amyloidosis during ASCT. METHODS: Between 1996 and 2010, 408 ASCT patients were evaluated. Data were collected from electronic medical records. RESULTS: Dialysis was performed on 72 (17.6%) patients...
August 2016: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/26586063/analysis-of-histopathological-pattern-of-kidney-biopsy-specimens-in-kuwait-a-single-center-five-year-prospective-study
#19
Emad Abdallah, Bassam Al-Helal, Reem Asad, Shreeram Kannan, Wael Draz, Zeyad Abdelgawad
Glomerulonephritis (GN) varies in incidence in different geographical areas due to different socioeconomic conditions and ethnicity, genetic variability and environmental factors. Our study is aimed to determine the histopathological pattern of kidney biopsies in Kuwait over the preceding five years. In a prospective study, we analyzed the clinical and pathological data of 214 kidney biopsies that were performed during the period from November 2009 to November 2014 at the Al-Khezam Dialysis Center, Al-Adan Hospital, Kuwait...
November 2015: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/26557519/bacteriological-quality-of-treated-water-and-dialysate-in-haemodialysis-unit-of-a-tertiary-care-hospital
#20
Shiva Verma, V A Indumathi, K C Gurudev, Shalini Ashok Naik
INTRODUCTION: Haemodialysis is one of the treatment modalities for patients suffering from end stage renal disease (ESRD). Dialysis patients are exposed to large volumes of water for production of dialysis fluids. Treated water and dialysate come in direct contact with the patient's bloodstream. Such patients suffer from abnormalities of the immune system, making them more susceptible to infections. Microbial contamination of the treated water and dialysate can lead to biofilm formation and release of endotoxins in Haemodialysis system...
October 2015: Journal of Clinical and Diagnostic Research: JCDR
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