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Amyloidosis

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https://www.readbyqxmd.com/read/28343171/circulating-free-light-chain-measurement-in-the-diagnosis-prognostic-assessment-and-evaluation-of-response-of-al-amyloidosis-comparison-of-freelite-and-n-latex-flc-assays
#1
Giovanni Palladini, Arnaud Jaccard, Paolo Milani, David Lavergne, Andrea Foli, Sebastien Bender, Francesca Lavatelli, Tiziana Bosoni, Veronica Valentini, Laura Pirolini, Giovanni Ferraro, Marco Basset, Francesca Russo, Mario Nuvolone, Riccardo Albertini, Michel Cogne, Giampaolo Merlini
BACKGROUND: The measurement of circulating free light chain (FLC) is essential in the diagnosis, prognostic stratification and evaluation of response to therapy in light chain (AL) amyloidosis. For more than 10 years, this has been done with an immunonephelometric assay based on polyclonal antibodies (Freelite), and cutoffs for staging and response assessment have been validated with this method. Recently, a new assay based on monoclonal antibodies (N latex FLC) has been marketed in Europe...
March 27, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28342017/primary-localized-cutaneous-amyloidosis-a-systematic-treatment-review
#2
REVIEW
Till Weidner, Tanja Illing, Peter Elsner
BACKGROUND: Primary localized cutaneous amyloidosis (PLCA) is characterized by extracellular deposition of heterogenic amyloid proteins in the skin without systemic involvement. Lichen amyloidosis, macular amyloidosis, and (primary localized cutaneous) nodular amyloidosis are different subtypes of PLCA. OBJECTIVE: The aim of this study was to review the current reported treatment options for PLCA. METHODS: This systematic review was based on a search in the PubMed database for English and German articles from 1985 to 2016...
March 24, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28341732/novel-recurrent-chromosomal-aberrations-detected-in-clonal-plasma-cells-of-light-chain-amyloidosis-patients-show-potential-adverse-prognostic-effect-first-results-from-a-genome-wide-copy-number-array-analysis
#3
Martin Granzow, Ute Hegenbart, Katrin Hinderhofer, Dirk Hose, Anja Seckinger, Tilmann Bochtler, Kari Hemminki, Hartmut Goldschmidt, Stefan O Schönland, Anna Jauch
Immunoglobulin light chain amyloidosis is a rare plasma cell dyscrasia characterized by deposition of abnormal amyloid fibrils in multiple organs impairing their function. In the largest cohort studied up to now of 118 CD138-purified plasma cell samples from previously untreated immunoglobulin light chain amyloidosis patients, we assessed in parallel copy number alterations using high-density copy number arrays and interphase fluorescence in situ hybridization. We used fluorescence in situ hybridization probes for the IgH translocations t(11;14), t(4;14), and t(14;16) or any other IgH rearrangement as well as numerical aberrations of the chromosome loci 1q21, 8p21, 5p15/5q35, 11q22...
March 24, 2017: Haematologica
https://www.readbyqxmd.com/read/28341718/relationships-among-cortical-glutathione-levels-brain-amyloidosis-and-memory-in-healthy-older-adults-investigated-in-vivo-with-1-h-mrs-and-pittsburgh-compound-b-pet
#4
G C Chiang, X Mao, G Kang, E Chang, S Pandya, S Vallabhajosula, R Isaacson, L D Ravdin, D C Shungu
BACKGROUND AND PURPOSE: Oxidative stress has been implicated as an important pathologic mechanism in the development of Alzheimer disease. The purpose of this study was to assess whether glutathione levels, detected noninvasively with proton MR spectroscopy, are associated with brain amyloidosis and memory in a community-dwelling cohort of healthy older adults. MATERIALS AND METHODS: Fifteen cognitively healthy subjects were prospectively enrolled in this study...
March 24, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28340799/is-there-a-long-term-risk-for-donors-with-heterozygous-mefv-mutation-after-kidney-donation
#5
S Karakose, S Erdogmus, S Akturk, A Tuzuner, S Sengul, K Keven
BACKGROUND: Familial Mediterranean fever (FMF) is an autosomal-recessive autoinflammatory disorder manifested severely by systemic amyloidosis. It has been hypothesized that heterozygous carriers may also have susceptibility to certain symptoms or even diseases. Because the living kidney donors of patients with FMF are generally relatives of the kidney recipients, there is a high possibility that the donors will have a heterozygous mutation of the FMF gene. The goal of this study was to investigate the long-term kidney function of donors who are carriers of the Mediterranean fever (MEFV) gene...
April 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28340285/the-six-minute-walk-test-in-patients-with-al-amyloidosis-a-single-centre-case-series
#6
Vina Pulido, Gheorghe Doros, John L Berk, Vaishali Sanchorawala
The six-minute walk test (6MWT) has been widely used as an objective evaluation of functional exercise capacity and response to medical intervention in cardiopulmonary diseases. However, little is known about the 6MWT in evaluating patients with AL amyloidosis. We performed a retrospective study of 120 adults with systemic AL amyloidosis (60 with cardiac involvement and 60 without cardiac involvement) who had their initial evaluation at the Amyloidosis Center between 2013 and 2015 and had undergone 6MWT as a measure of functional exercise capacity...
March 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-adult-population-of-poland
#7
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
February 11, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28338670/encouraging-impact-of-doxycycline-on-early-mortality-in-cardiac-light-chain-al-amyloidosis
#8
A D Wechalekar, C Whelan
No abstract text is available yet for this article.
March 24, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/28336182/light-chain-amyloidosis-where-are-the-light-chains-from-and-how-they-play-their-pathogenic-role
#9
REVIEW
Chunlan Zhang, Xufei Huang, Jian Li
Amyloid light-chain (AL) amyloidosis is a plasma-cell dyscrasia, as well as the most common type of systematic amyloidosis. Pathogenic plasma cells that have distinct cytogenetic and molecular properties secrete an excess amount of amyloidogenic light chains. Assisted by post-translational modifications, matrix components, and other environmental factors, these light chains undergo a conformational change that triggers the formation of amyloid fibrils that overrides the extracellular protein quality control system...
March 8, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28335735/population-diversity-of-the-genetically-determined-ttr-expression-in-human-tissues-and-its-implications-in-ttr-amyloidosis
#10
Andrea Iorio, Flavio De Angelis, Marco Di Girolamo, Marco Luigetti, Luca G Pradotto, Anna Mazzeo, Sabrina Frusconi, Filomena My, Dario Manfellotto, Maria Fuciarelli, Renato Polimanti
BACKGROUND: Transthyretin (TTR) amyloidosis is a hereditary disease with a complex genotype-phenotype correlation. We conducted a literature survey to define the clinical landscape of TTR amyloidosis across populations worldwide. Then, we investigated whether the genetically determined TTR expression differs among human populations, contributing to the differences observed in patients. Polygenic scores for genetically determined TTR expression in 14 clinically relevant tissues were constructed using data from the GTEx (Genotype-Tissue Expression) project and tested in the samples from the 1,000 Genomes Project...
March 23, 2017: BMC Genomics
https://www.readbyqxmd.com/read/28334940/aav9-delivered-bispecific-nanobody-attenuates-amyloid-burden-in-the-gelsolin-amyloidosis-mouse-model
#11
Adriaan Verhelle, Nisha Nair, Inge Everaert, Wouter Van Overbeke, Lynn Supply, Olivier Zwaenepoel, Cindy Peleman, Jo Van Dorpe, Tony Lahoutte, Nick Devoogdt, Wim Derave, Marinee K Chuah, Thierry Vanden Driessche, Jan Gettemans
Gelsolin amyloidosis is a dominantly inherited, incurable type of amyloidosis. A single point mutation in the gelsolin gene (G654A is most common) results in the loss of a Ca2+  binding site in the second gelsolin domain. Consequently, this domain partly unfolds and exposes an otherwise buried furin cleavage site at the surface. During secretion of mutant plasma gelsolin consecutive cleavage by furin and MT1-MMP results in the production of 8 and 5 kDa amyloidogenic peptides. Nanobodies that are able to (partly) inhibit furin or MT1-MMP proteolysis have previously been reported...
February 21, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28334621/sequential-cyclophosphamide-bortezomib-dexamethasone-unmasks-the-harmful-cardiac-effect-of-dexamethasone-in-primary-light-chain-cardiac-amyloidosis
#12
Fabien Le Bras, Valerie Molinier-Frenkel, Aziz Guellich, Jehan Dupuis, Karim Belhadj, Soulef Guendouz, Karima Ayad, Magali Colombat, Nicole Benhaiem, Claire Marie Tissot, Anne Hulin, Arnaud Jaccard, Thibaud Damy
Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains...
March 20, 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28334015/correction-perinatal-choline-supplementation-reduces-amyloidosis-and-increases-choline-acetyltransferase-expression-in-the-hippocampus-of-the-appsweps1de9-alzheimer-s-disease-model-mice
#13
Tiffany J Mellott, Olivia M Huleatt, Bethany N Shade, Sarah M Pender, Yi B Liu, Barbara E Slack, Jan K Blusztajn
[This corrects the article DOI: 10.1371/journal.pone.0170450.].
2017: PloS One
https://www.readbyqxmd.com/read/28331795/pleural-amyloidosis-imitating-pleural-malignancy
#14
Frith Coolbear, Ana-Maria Bilawich, Jonathan Tongson, James Adamo, Andrew Churg
CT chest performed to investigate dyspnea and malaise in an 83 year old man demonstrated bilateral circumferential pleural thickening highly suspicious for pleural mesothelioma or metastatic pleural malignancy. Histopathology of a subsequent pleural biopsy returned a diagnosis of pleural amyloid. This case identifies pleural amyloid as a rare differential consideration for diffuse pleural thickening; the difficulties in distinguishing pleural amyloid from pleural malignancy are highlighted.
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28330445/cardiac-amyloidosis-mimicking-severe-aortic-valve-stenosis-a-case-report-demonstrating-diagnostic-pitfalls-and-role-of-dobutamine-stress-echocardiography
#15
Tim Salinger, Kai Hu, Dan Liu, Sebastian Herrmann, Kristina Lorenz, Georg Ertl, Peter Nordbeck
BACKGROUND: Aortic valve stenosis is a common finding diagnosed with high sensitivity in transthoracic echocardiography, but the examiner often finds himself confronted with uncertain results in patients with moderate pressure gradients and concomitant systolic heart failure. While patients with true-severe low-gradient aortic valve stenosis with either reduced or preserved left ventricular systolic function are primarily candidates for valve replacement, there is a relevant proportion of patients with pseudo-severe aortic valve stenosis anticipated not to benefit but actually rather deteriorate by interventional therapy or surgery...
March 22, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28330375/complex-p-t88n-w130r-mutation-in-the-lysozyme-gene-leading-to-hereditary-lysozyme-amyloidosis-with-biopsy-proven-cardiac-involvement
#16
Brett W Sperry, Angela Dispenzieri, Asad Ikram, Martha Grogan, Jason D Theis, Nelson Leung, W Edward Highsmith, Joseph J Maleszewski, Mazen Hanna
No abstract text is available yet for this article.
March 22, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28329248/clinical-characteristics-of-wild-type-transthyretin-cardiac-amyloidosis-disproving-myths
#17
Esther González-López, Christian Gagliardi, Fernando Dominguez, Cristina Candida Quarta, F Javier de Haro-Del Moral, Agnese Milandri, Clara Salas, Mario Cinelli, Marta Cobo-Marcos, Massimiliano Lorenzini, Enrique Lara-Pezzi, Serena Foffi, Luis Alonso-Pulpon, Claudio Rapezzi, Pablo Garcia-Pavia
Aims: Wild-type transthyretin amyloidosis (ATTRwt) is mostly considered a disease predominantly of elderly male, characterized by concentric LV hypertrophy, preserved LVEF, and low QRS voltages. We sought to describe the characteristics of a large cohort of ATTRwt patients to better define the disease. Methods and results: Clinical findings of consecutive ATTRwt patients diagnosed at 2 centres were reviewed. ATTRwt was diagnosed histologically or non-invasively (LV hypertrophy ≥12 mm, intense cardiac uptake at 99mTc-DPD scintigraphy and AL exclusion)...
March 1, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28326465/pet-ct-evaluation-of-amyloid-systemic-involvement-with-18-f-florbetaben-in-patient-with-proved-cardiac-amyloidosis-a-case-report
#18
Dario Genovesi, Giuseppe Vergaro, Michele Emdin, Assuero Giorgetti, Paolo Marzullo
BACKGROUND: One of the most frequent disorders which lead to cardiac amyloidosis is transthyretin-related amyloidosis (ATTR). Some PET radio-pharmaceuticals for the detection of beta-amyloid deposits within the brain have shown to be able to detect also cardiac amyloid deposits. We present a case of a man with ATTR studied with [(18)F]-florbetaben PET-CT. RESULTS: Total-body scan showed a moderate uptake in the bone marrow, especially in correspondence of the vertebral column, while no significant myocardial uptake was present...
March 21, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28326212/a-case-of-conjunctival-amyloidosis-with-repeated-subconjunctival-hemorrhage
#19
Takaaki Ando, Toshiyuki Oshitari, Mamiko Saito, Ayako Tawada, Takayuki Baba, Jiro Yotsukura, Shuichi Yamamoto
Conjunctival amyloidosis is a very rare disease, and its presence may be a sign of systemic amyloidosis. We present our ocular and systemic findings in a patient with conjunctival amyloidosis. A 43-year-old man had repeated subconjunctival hemorrhages (SCHs) for two years and was referred to the Chiba University Hospital. He had comprehensive ophthalmological and systemic examinations to determine the cause of the SCHs. His visual acuities were 1.2 OU, and the intraocular pressures were 13-14 mmHg OU. Magnetic resonance imaging was normal...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28321082/systemic-al-amyloidosis-with-colonic-submucosal-hematoma
#20
Sho Sasaki, Jun Nishikawa, Shinichi Hashimoto, Isao Sakaida
No abstract text is available yet for this article.
2017: Internal Medicine
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