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Dialysis amyloidosis

Paolo Milani, Giampaolo Merlini, Giovanni Palladini
Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of dialysis is predicted by the severity of renal involvement, defined by the baseline proteinuria and glomerular filtration rate, and by the response to therapy...
2018: Mediterranean Journal of Hematology and Infectious Diseases
Kotaro Matsumoto, Jun Kikuchi, Yuko Kaneko, Hidekata Yasuoka, Kazuko Suzuki, Hirobumi Tokuyama, Kaori Kameyama, Kunihiro Yamaoka, Tsutomu Takeuchi
RATIONALE: Dialysis-related amyloidosis (DRA) can present rheumatic manifestations in patients on long-term hemodialysis. Typical articular symptoms with DRA involve carpal-tunnel syndrome, effusion in large joints, spondyloarthropathy, or cystic bone lesions, which are usually with non-inflammatory processes. PATIENT CONCERNS: A 64-year-old man on hemodialysis for >30 years was admitted because of intermittent fever, polyarthritis, and elevated serum C-reactive protein (CRP) level, which was continuous for 2 years...
January 2018: Medicine (Baltimore)
Hongli Liang, Bing Wang, Hailong Wang, Miao Yu, Lingna Zheng, Meng Wang, Aiping Zhang, Weiyue Feng
Amyloid fibrillation has been implicated in many neurodegenerations, dialysis-related amyloidosis, type II diabetes and more than 30 other amyloid-related diseases. Nanomaterials as potential inhibitors of amyloid fibrillation have attracted increasing interests. In the present study, the effects of gold nanorods (AuNRs) and nanoparticles (AuNPs) on amyloid fibrillation were investigated using hen egg white lysozyme (HEWL) as a model system. Our results indicated that AuNRs and AuNPs, especially AuNRs, present significant inhibitory effects on HEWL amyloid fibril formation during all the kinetic processes, from nucleation to elongation and equilibration stages...
May 1, 2018: Journal of Nanoscience and Nanotechnology
Amit P Java, Kevin L Greason, Angela Dispenzieri, Martha Grogan, Katherine S King, Joseph J Maleszewski, Richard C Daly, Mackram F Eleid, Alberto Pochettino, Hartzell V Schaff
BACKGROUND: Outcome data on aortic valve replacement in patients with amyloidosis are limited. To address this issue, we reviewed our experience of patients with amyloidosis who underwent aortic valve replacement. METHODS: We retrospectively reviewed the records of 16 patients with amyloidosis who underwent aortic valve replacement between May 2000 and February 2017. RESULTS: The cohort comprised 11 males (69%) and 5 females (31%). The median patient age was 76 years (interquartile range [IQR], 71-82 years), and Society of Thoracic Surgeons predicted rate of mortality was 5...
December 19, 2017: Journal of Thoracic and Cardiovascular Surgery
Efstathios Kastritis, Ioannis Papassotiriou, Giampaolo Merlini, Paolo Milani, Evangelos Terpos, Marco Basset, Athanasios Akalestos, Francesca Russo, Erasmia Psimenou, Filia Apostolakou, Maria Roussou, Maria Gavriatopoulou, Evangelos Eleutherakis Papaiakovou, Despina Fotiou, Dimitrios C Ziogas, Elektra Papadopoulou, Constantinos Pamboucas, Meletios A Dimopoulos, Giovanni Palladini
Growth differentiation factor-15 (GDF-15) improves prognostication in patients with cardiovascular disorders in addition to conventional cardiac markers (NT-proBNP, troponins) and has shown prognostic value in patients with renal diseases. In patients with AL amyloidosis cardiac involvement is the major determinant of prognosis and cardiac markers define prognosis, while biomarkers of renal involvement stratify renal risk. We explored the prognostic importance of serum levels of GDF-15 in patients with AL amyloidosis, in two independent cohorts...
January 31, 2018: Blood
P Vincent, J Gopinathan, R Narayanan
Delayed bowel erosion by peritoneal dialysis catheter is rare with fewer than thirty cases having been reported in the literature. This complication is usually encountered when the catheter is kept dormant. Two cases have also been reported with catheters in active use. The risk factors for bowel erosion include immunosuppression, diverticulosis, and amyloidosis. An 80-year-old male with chronic kidney disease Stage 5 due to hypertensive nephrosclerosis underwent chronic ambulatory peritoneal dialysis catheter insertion...
November 2017: Indian Journal of Nephrology
Yavuz Ayar, Alparslan Ersoy, Mustafa Ferhat Oksuz, Gokhan Ocakoglu, Berna Aytac Vuruskan, Abdülmecit Yildiz, Emel Isiktas, Aysegül Oruc, Sedat Celikci, Ismail Arslan, Ahmet Bilgehan Sahin, Mustafa Güllülü
AIM: Amyloid A amyloidosis is a rare complication of chronic inflammatory conditions. Most patients with amyloid A amyloidosis present with nephropathy and it leads to renal failure and death. We studied clinical characteristics and survival in patients with amyloid A amyloidosis. METHODS: A total of 81 patients (51 males, 30 females) with renal biopsy proven amyloid A amyloidosis were analyzed retrospectively. The patients were divided into good and poor outcomes groups according to survival results...
November 2017: Revista Brasileira de Reumatologia
Cheng-Hao Weng, Ching-Chih Hu, Tzung-Hai Yen, Wen-Hung Huang
BACKGROUND/AIMS: The deposition of β2-microglobulin induced by reactive inflammation causing carpal tunnel syndrome (CTS) is one of the complications of dialysis-related amyloidosis in maintenance hemodialysis (MHD) patients. Air pollution levels, especially particulate matter with an aerodynamic diameter of <2.5 mm (PM2.5), have significantly been associated with the elevation of systemic inflammatory markers. There is no previous research on possible associations between CTS and PM2...
November 21, 2017: Kidney & Blood Pressure Research
(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]
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
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
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
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
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
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
Felipe Batalini, Laura Econimo, Karen Quillen, J Mark Sloan, Shayna Sarosiek, Dina Brauneis, Andrea Havasi, Lauren Stern, Laura M 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...
January 2018: Biology of Blood and Marrow Transplantation
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
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
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
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
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