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systemic amyloidosis

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https://www.readbyqxmd.com/read/29161700/association-between-environmental-particulate-matter-and-carpal-tunnel-syndrome-in-patients-undergoing-hemodialysis
#1
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
https://www.readbyqxmd.com/read/29159946/extracellular-volume-with-bolus-only-technique-in-amyloidosis-patients-diagnostic-accuracy-correlation-with-other-clinical-cardiac-measures-and-ability-to-track-changes-in-amyloid-load-over-time
#2
Giulia Zumbo, Sharon V Barton, Douglas Thompson, Min Sun, Amna Abdel-Gadir, Thomas A Treibel, Daniel Knight, Ana Martinez-Naharro, Lane Thirusha, Julian D Gillmore, James C Moon, Philip N Hawkins, Marianna Fontana
BACKGROUND: Extracellular volume (ECV) by T1 mapping requires the contrast agent distribution to be at equilibrium. This can be achieved either definitively with a primed contrast infusion (infusion ECV), or sufficiently with a delay postbolus (bolus-only ECV). For large ECV, the bolus-only approach measures higher than the infusion ECV, causing some uncertainty in diseases such as amyloidosis. PURPOSE: To characterize the relationship between the bolus-only and current gold-standard infusion ECV in patients with amyloidosis...
November 21, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29155738/imaging-amyloid-tissues-stained-with-luminescent-conjugated-oligothiophenes-by-hyperspectral-confocal-microscopy-and-fluorescence-lifetime-imaging
#3
Sofie Nyström, Marcus Bäck, K Peter R Nilsson, Per Hammarström
Proteins that deposit as amyloid in tissues throughout the body can be the cause or consequence of a large number of diseases. Among these we find neurodegenerative diseases such as Alzheimer's and Parkinson's disease afflicting primarily the central nervous system, and systemic amyloidosis where serum amyloid A, transthyretin and IgG light chains deposit as amyloid in liver, carpal tunnel, spleen, kidney, heart, and other peripheral tissues. Amyloid has been known and studied for more than a century, often using amyloid specific dyes such as Congo red and Thioflavin T (ThT) or Thioflavin (ThS)...
October 20, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29153741/left-atrial-function-in-patients-with-light-chain-amyloidosis-a-transthoracic-3d-speckle-tracking-imaging-study
#4
Dania Mohty, Vincent Petitalot, Julien Magne, Bahaa M Fadel, Cyrille Boulogne, Dounia Rouabhia, Chahrazed ElHamel, David Lavergne, Thibaud Damy, Victor Aboyans, Arnaud Jaccard
BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography...
November 16, 2017: Journal of Cardiology
https://www.readbyqxmd.com/read/29136724/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-renal-impairment-of-multiple-myeloma
#5
(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/29135289/-what-should-we-know-about-cardiac-amyloidosis-from-clinical-signs-to-treatment
#6
Dóra Földeák, Attila Nemes, Anita Kalapos, Péter Domsik, Árpád Kormányos, László Krenács, Enikő Bagdi, Zita Borbényi
Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment...
November 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/29133141/abdominal-fat-pad-excisional-biopsy-for-the-diagnosis-and-typing-of-systemic-amyloidosis
#7
Yessica Garcia, A Bernard Collins, James R Stone
In the past, the diagnosis and typing of amyloidosis often required an invasive biopsy of an internal organ, such as the heart or kidneys. Abdominal fat pad excisional biopsy (FPEB) offers a less invasive approach, but the sensitivity of this technique has been unclear. To determine the sensitivity of FPEB for immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, we performed a retrospective clinicopathologic analysis of 97 patients who had undergone FPEB, of which 16 were positive for amyloid...
November 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/29131444/native-t1-reference-values-for-nonischemic-cardiomyopathies-and-populations-with-increased-cardiovascular-risk-a-systematic-review-and-meta-analysis
#8
REVIEW
Maaike van den Boomen, Riemer H J A Slart, Enzo V Hulleman, Rudi A J O Dierckx, Birgitta K Velthuis, Pim van der Harst, David E Sosnovik, Ronald J H Borra, Niek H J Prakken
BACKGROUND: Although cardiac MR and T1 mapping are increasingly used to diagnose diffuse fibrosis based cardiac diseases, studies reporting T1 values in healthy and diseased myocardium, particular in nonischemic cardiomyopathies (NICM) and populations with increased cardiovascular risk, seem contradictory. PURPOSE: To determine the range of native myocardial T1 value ranges in patients with NICM and populations with increased cardiovascular risk. STUDY TYPE: Systemic review and meta-analysis...
November 13, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29121956/regional-differences-in-prognostic-value-of-cardiac-valve-plane-displacement-in-systemic-light-chain-amyloidosis
#9
Marco M Ochs, Thomas Fritz, Nisha Arenja, Johannes Riffel, Florian Andre, Derliz Mereles, Fabian Aus dem Siepen, Ute Hegenbart, Stefan Schönland, Hugo A Katus, Matthias G W Friedrich, Sebastian J Buss
BACKGROUND: To compare the prognostic value of cardiac valve plane displacement (CVPD) on various locations in cardiac light chain (AL) amyloidosis. METHODS: Consecutive patients with biopsy-proven cardiac involvement in AL amyloidosis who had undergone cardiovascular magnetic resonance (CMR) between 2005 and 2014 in our institution, were retrospectively identified and data analyzed. The primary combined endpoint was all-cause mortality or heart transplantation...
November 9, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29117820/carpal-tunnel-syndrome-and-trigger-wrist-caused-by-localized-amyloidosis-a-case-report
#10
Takuma Wakasugi, Ritsuro Shirasaka, Toshiyuki Kawauchi, Koji Fujita, Atsushi Okawa
We report a case of carpal tunnel syndrome and trigger wrist caused by localized amyloidosis. A 37-year-old man, who worked as a manufacturer, presented with a 5-month history of tingling sensation in the three radial fingers of the left hand and a painful click at the volar aspect of the wrist during digital motion. We divided the transverse carpal ligament and detected diffuse tenosynovitis that was especially severe around the flexor digitorum profundus tendons. Histological findings led to a diagnosis of amyloidosis...
December 2017: Journal of Hand Surgery Asian-Pacific Volume
https://www.readbyqxmd.com/read/29114004/dysproteinemias-and-glomerular-disease
#11
Nelson Leung, Maria E Drosou, Samih H Nasr
Dysproteinemia is characterized by the overproduction of an Ig by clonal expansion of cells from the B cell lineage. The resultant monoclonal protein can be composed of the entire Ig or its components. Monoclonal proteins are increasingly recognized as a contributor to kidney disease. They can cause injury in all areas of the kidney, including the glomerular, tubular, and vascular compartments. In the glomerulus, the major mechanism of injury is deposition. Examples of this include Ig amyloidosis, monoclonal Ig deposition disease, immunotactoid glomerulopathy, and cryoglobulinemic GN specifically from types 1 and 2 cryoglobulins...
November 7, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29111123/prognostic-and-added-value-of-two-dimensional-global-longitudinal-strain-for-prediction-of-survival-in-patients-with-light-chain-amyloidosis-undergoing-autologous-hematopoietic-cell%C3%A2-transplantation
#12
Shawn C Pun, Heather J Landau, Elyn R Riedel, Jonathan Jordan, Anthony F Yu, Hani Hassoun, Carol L Chen, Richard M Steingart, Jennifer E Liu
BACKGROUND: Autologous hematopoietic cell transplantation (HCT) is a first-line therapy for prolonging survival in patients with light-chain (AL) amyloidosis. Cardiac involvement is the most important determinant of survival. However, patients with advanced cardiac involvement have often been excluded from HCT because of high risk for transplantation-related mortality and poor overall survival. Whether baseline left ventricular global longitudinal strain (GLS) can provide additional risk stratification and predict survival after HCT in this high-risk population remains unclear...
October 27, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29107186/a-bell-shaped-dependence-between-amyloidosis-and-gaba-accumulation-in-astrocytes-in-a-mouse-model-of-alzheimer-s-disease
#13
Bianca Brawek, Robert Chesters, Daniel Klement, Julia Müller, Chommanad Lerdkrai, Marina Hermes, Olga Garaschuk
Functioning at the interface between the nervous and immune systems, in the amyloid-depositing brain, astrocytes become hypertrophic and accumulate around senile plaques. Moreover, hippocampal astrocytes upregulate their γ-aminobutyric acid (GABA) content and enhance tonic inhibition, likely causing local circuit imbalance. It remains, however, unclear whether this effect is hippocampus specific and how it is regulated during disease progression. Here, we studied changes in astrocytic morphology and GABA content in the frontal cortex and dentate gyrus of control and amyloid-depositing mice...
October 6, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/29106288/localized-gastrointestinal-amyloidosis-presenting-with-protein-losing-enteropathy-and-massive-hemorrhage
#14
Bárbara Corrêa, Cristiane Kibune Nagasako, Ciro Garcia Montes, Marlone Cunha-Silva, Maria Aparecida Mesquita
Amyloidosis of the gastrointestinal tract is usually a systemic disease. Localized gastrointestinal amyloidosis without evidence of extraintestinal involvement or an associated plasma cell dyscrasia is uncommon and does not usually cause death. We report a case of a patient with localized gastrointestinal amyloidosis who presented with protein-losing enteropathy and a fatal upper gastrointestinal bleed.
November 6, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/29105927/resveratrol-interferes-with-an-early-step-in-the-fibrillation-pathway-of-human-lysozyme-and-modulates-it-towards-less-toxic-off-pathway-aggregates
#15
Fatima Kamal Zaidi, Rajiv Bhat
Effect of the red wine polyphenol, resveratrol, was investigated on the amyloid fibril formation of human lysozyme (HuL), towards elucidating the mechanism of resveratrol action and probing its role as a possible modulator of lysozyme aggregation and toxicity. Using a number of biophysical tools, resveratrol was observed to alter the fibrillation kinetics of HuL and inhibit its fibrillation by binding with weak to moderate affinity to the conformations populated at the early stages of the pathway with concomitant stabilization of these initial conformations...
November 4, 2017: Chembiochem: a European Journal of Chemical Biology
https://www.readbyqxmd.com/read/29101328/application-of-lysine-specific-labeling-to-detect-transient-interactions-present-during-human-lysozyme-amyloid-fibril-formation
#16
Minkoo Ahn, Christopher A Waudby, Ana Bernardo-Gancedo, Erwin De Genst, Anne Dhulesia, Xavier Salvatella, John Christodoulou, Christopher M Dobson, Janet R Kumita
Populating transient and partially unfolded species is a crucial step in the formation and accumulation of amyloid fibrils formed from pathogenic variants of human lysozyme linked with a rare but fatal hereditary systemic amyloidosis. The partially unfolded species possess an unstructured β-domain and C-helix with the rest of the α-domain remaining native-like. Here we use paramagnetic relaxation enhancement (PRE) measured by NMR spectroscopy to study the transient intermolecular interactions between such intermediate species...
November 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29101236/presentation-and-outcome-with-second-line-treatment-in-al-amyloidosis-previously-sensitive-to-non-transplant-therapies
#17
Giovanni Palladini, Paolo Milani, Andrea Foli, Marco Basset, Francesca Russo, Stefano Perlini, Giampaolo Merlini
The management of light chain (AL) amyloidosis has improved in recent years thanks to accurate biomarker-based staging systems and response criteria and availability of novel effective therapies. However, previous studies have focused on newly diagnosed patients, and little is known on relapsed patients, despite trials of new agents are often performed in this setting. In the present study we report the outcome of 259 patients who responded to upfront therapy. Ninety-two patients (35%) needed second-line therapy after a median of 49 months...
November 3, 2017: Blood
https://www.readbyqxmd.com/read/29093431/increased-prognostic-value-of-query-amyloid-late-enhancement-score-in-light-chain-cardiac-amyloidosis
#18
Ke Wan, Jiayu Sun, Yuchi Han, Hong Liu, Dan Yang, Weihao Li, Jie Wang, Wei Cheng, Qing Zhang, Zhi Zeng, Yucheng Chen
BACKGROUND: Late gadolinium enhancement (LGE) pattern is a powerful imaging biomarker for prognosis of cardiac amyloidosis. It is unknown if the query amyloid late enhancement (QALE) score in light-chain (AL) amyloidosis could provide increased prognostic value compared with LGE pattern.Methods and Results:Seventy-eight consecutive patients with AL amyloidosis underwent contrast-enhanced cardiovascular magnetic resonance imaging. Patients with cardiac involvement were grouped by LGE pattern and analyzed using QALE score...
November 2, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29093404/nationwide-survey-of-741-patients-with-systemic-amyloid-light-chain-amyloidosis-in-japan
#19
Chihiro Shimazaki, Hiroyuki Hata, Sinsuke Iida, Mitsuharu Ueda, Nagaaki Katoh, Yoshiki Sekishima, Shuichi Ikeda, Masahide Yazaki, Wakaba Fukushima, Yukio Ando
Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. Methods We conducted a survey of Japanese AL patients, who were treated between January 1, 2012, and December 31, 2014. Results A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71)...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29081441/amyloidosis-inducing-activity-of-blood-cells-in-mouse-aapoaii-amyloidosis
#20
Xin Ding, Yingye Liu, Mu Yang, Lin Li, Hiroki Miyahara, Jian Dai, Zhe Xu, Kiyoshi Matsumoto, Masayuki Mori, Keiichi Higuchi, Jinko Sawashita
Mouse senile amyloidosis is a disorder in which apolipoprotein A-II (APOA2) deposits as amyloid fibrils (AApoAII) in many organs. We previously reported that AApoAII amyloidosis can be transmitted by feces, milk, saliva and muscle originating from mice with amyloid deposition. In this study, the ability of blood components to transmit amyloidosis was evaluated in our model system. Blood samples were collected from SAMR1.SAMP1-Apoa2(c) amyloid-laden or amyloidosis-negative mice. The samples were fractionated into plasma, white blood cell (WBC) and red blood cell (RBC) fractions...
October 30, 2017: Experimental Animals
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