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Amyloidosis

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https://www.readbyqxmd.com/read/28550295/effects-of-protein-corona-on-iapp-amyloid-aggregation-fibril-remodelling-and-cytotoxicity
#1
Emily H Pilkington, Yanting Xing, Bo Wang, Aleksandr Kakinen, Miaoyi Wang, Thomas P Davis, Feng Ding, Pu Chun Ke
Aggregation of islet amyloid polypeptide (IAPP), a peptide hormone co-synthesized and co-stored with insulin in pancreatic cells and also co-secreted to the circulation, is associated with beta-cell death in type-2 diabetes (T2D). In T2D patients IAPP is found aggregating in the extracellular space of the islets of Langerhans. Although the physiological environments of these intra- and extra-cellular compartments and vascular systems significantly differ, the presence of proteins is ubiquitous but the effects of protein binding on IAPP aggregation are largely unknown...
May 26, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28550043/al-amyloidosis-patients-with-low-amyloidogenic-free-light-chain-levels-at-first-diagnosis-have-an-excellent-prognosis
#2
Tobias Dittrich, Tilmann Bochtler, Christoph Kimmich, Natalia Becker, Anna Jauch, Hartmut Goldschmidt, Anthony D Ho, Ute Hegenbart, Stefan O Schönland
The difference between involved minus uninvolved serum free light chains (dFLC) has been established as invaluable hematologic parameter in systemic light chain amyloidosis (AL). However, patients with initial dFLC below 50mg/L are currently deemed not evaluable for response to therapy. Therefore, we aimed to characterize this subgroup and to define novel hematologic response parameters. We retrospectively analyzed 783 AL patients newly diagnosed at our center between 2002 and 2016. Patients with dFLC<50 showed a smaller bone marrow plasmacytosis (7% vs...
May 26, 2017: Blood
https://www.readbyqxmd.com/read/28550039/a-phase-1-2-study-of-the-oral-proteasome-inhibitor-ixazomib-in-relapsed-or-refractory-light-chain-al-amyloidosis
#3
Vaishali Sanchorawala, Giovanni Palladini, Vishal Kukreti, Jeffrey A Zonder, Adam D Cohen, David C Seldin, Angela Dispenzieri, Arnaud Jaccard, Stefan O Schönland, Deborah Berg, Huyuan Yang, Neeraj Gupta, Ai-Min Hui, Raymond L Comenzo, Giampaolo Merlini
This phase 1/2 study assessed the safety, tolerability, and preliminary efficacy of the oral proteasome inhibitor (PI) ixazomib in patients with relapsed/refractory AL amyloidosis (RRAL). Ixazomib was administered to adult patients with RRAL after ≥1 prior line of therapy (including bortezomib) on days 1, 8, and 15 of 28-day cycles, for up to 12 cycles. Patients with less than partial response after 3 cycles received oral dexamethasone (40 mg, days 1-4) from cycle 4. A 3+3 dose-escalation phase was followed by 2 expansion cohorts (PI-naïve and PI-exposed patients) at the maximum tolerated dose (MTD)...
May 26, 2017: Blood
https://www.readbyqxmd.com/read/28546143/patients-with-light-chain-al-amyloidosis-and-low-free-light-chain-burden-have-distinct-clinical-features-and-outcome
#4
Paolo Milani, Marco Basset, Francesca Russo, Andrea Foli, Giampaolo Merlini, Giovanni Palladini
The validated criteria of hematologic response in AL amyloidosis are based on the measurement of circulating free light chains (FLC). Patients with a difference between involved and uninvolved FLC (dFLC) <50 mg/L cannot be assessed for response and are excluded from clinical trials. We compared the clinical characteristics and outcome of 203 newly diagnosed patients with dFLC<50 mg/L (low-dFLC) with 866 patients with measurable dFLC (high-dFLC), evaluated between 2004 and 2015. Heart involvement was significantly less common and less advanced in the low-dFLC group (43% vs...
May 25, 2017: Blood
https://www.readbyqxmd.com/read/28546074/outcomes-from-autologous-hematopoietic-cell-transplantation-versus-chemotherapy-alone-for-the-management-of-light-chain-al-amyloidosis
#5
Oluchi Oke, Tarsheen Sethi, Stacey Goodman, Sharon Phillips, Ilka Decker, Samuel Rubinstein, Beatrice Concepcion, Sarah Horst, Madan Jagasia, Adetola Kassim, Shelton L Harrell, Anthony Langone, Daniel Lenihan, Kyle T Rawling, David Slosky, R Frank Cornell
Light chain amyloidosis (AL) results in tissue deposition of misfolded proteins causing organ dysfunction. In an era of modern therapies such as bortezomib, reassessment of the benefit of autologous hematopoietic cell transplantation (AHCT) should be considered. This study compares the difference in outcomes between patients with AL receiving chemotherapy alone (CT) vs. AHCT. Seventy-four patients with AL were retrospectively analyzed. Two cohorts of patients were studied, those receiving CT (n=31) or AHCT (n=43)...
May 22, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28544119/common-fibril-structures-imply-systemically-conserved-protein-misfolding-pathways-in%C3%A2-vivo
#6
Karthikeyan Annamalai, Falk Liberta, Marie-Theres Vielberg, William Close, Hauke Lilie, Karl-Heinz Gührs, Angelika Schierhorn, Rolf Koehler, Andreas Schmidt, Christian Haupt, Ute Hegenbart, Stefan Schönland, Matthias Schmidt, Michael Groll, Marcus Fändrich
Systemic amyloidosis is caused by the misfolding of a circulating amyloid precursor protein and the deposition of amyloid fibrils in multiple organs. Chemical and biophysical analysis of amyloid fibrils from human AL and murine AA amyloidosis reveal the same fibril morphologies in different tissues or organs of one patient or diseased animal. The observed structural similarities concerned the fibril morphology, the fibril protein primary and secondary structures, the presence of post-translational modifications and, in case of the AL fibrils, the partially folded characteristics of the polypeptide chain within the fibril...
May 23, 2017: Angewandte Chemie
https://www.readbyqxmd.com/read/28541019/amyloidosis-and-device-therapy-an-open-debate
#7
Eluisa LA Franca, Egle Corrado, Giuseppe Inga, Gianfranco Ciaramitaro, Salvatore Novo, Giuseppe Coppola
No abstract text is available yet for this article.
May 24, 2017: International Angiology: a Journal of the International Union of Angiology
https://www.readbyqxmd.com/read/28540895/pattern-of-acute-glomerulonephritis-in-adult-population-in-dubai-a-single-center-experience
#8
Amna K Alhadari, Fakhriya J Alalawi, Ayman Aly Seddik, Kaneez Zahra, Dileep Kumar, Hussain Yousif, Hind Alnour, Michael Jansen, Mohammad J Railey
Epidemiological data of renal diseases have great geographic variability throughout the world. Due to the lack of a national renal data registry system, there is no information on the prevalence rate, clinical and pathological features of various glomerulonephritis (GN) in the United Arab Emirates (UAE). In a retrospective cross-sectional study, we analyzed 158 renal biopsies done in Dubai Hospital, UAE, between the years of 2005 and September 2014, with an aim to determine the prevalence rate and frequency of different pathological patterns of GN in adult patients who presented with proteinuria ± hematuria...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28540891/chronic-renal-failure-an-autopsy-study
#9
Anitha Padmanabhan, Sanjay Gohil, N M Gadgil, Prerna Sachdeva
Diabetes and hypertension are at present the major causes of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide. The stages 0-5 of CKD are defined according to the estimated glomerular filtration rate. The term chronic renal failure (CRF) typically corresponds to CKD stages 3-5. Cardiovascular disease is the main cause of morbidity and mortality in patients of CRF and ESRD. This study was undertaken to analyze the age and sex incidence, clinical features, etiology, pathology of various organs in detail, and causes of death of CRF patients...
May 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28539873/pharmacological-stimulation-of-phagocytosis-enhances-amyloid-plaque-clearance-evidence-from-a-transgenic-mouse-model-of-attr-neuropathy
#10
Eleni Fella, Kleitos Sokratous, Revekka Papacharalambous, Kyriacos Kyriacou, Joy Phillips, Sam Sanderson, Elena Panayiotou, Theodoros Kyriakides
Hereditary ATTR V30M amyloidosis is a lethal autosomal dominant sensorimotor and autonomic neuropathy caused by deposition of aberrant transthyretin (TTR). Immunohistochemical examination of sural nerve biopsies in patients with amyloidotic neuropathy show co-aggregation of TTR with several proteins; including apolipoprotein E, serum amyloid P and components of the complement cascade. Complement activation and macrophages are increasingly recognized to play a crucial role in amyloidogenesis at the tissue bed level...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28538749/complications-of-hidradenitis-suppurativa
#11
Joyce T Yuan, Haley B Naik
Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease characterized by recurrent painful nodules and abscesses involving intertriginous areas. Repeated episodes of profound inflammation in HS can lead to a number of complications, causing significant morbidity and decreasing quality of life. Complications of HS may affect the skin alone or may have systemic impact. Cutaneous complications of HS include sinus tracts, fistulae, scarring and contractures, squamous cell carcinoma, and lymphedema...
June 2017: Seminars in Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/28538281/gout-and-aa-amyloidosis-a-case-report-and-review-of-the-literature
#12
Evert-Jan Ter Borg, Michelle A Wegewijs, Peter de Bruin
No abstract text is available yet for this article.
June 2017: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/28537855/multifocal-primary-cutaneous-nodular-amyloidosis
#13
Martha Emily Matsumoto, Mary-Katharine Collins, Anastasios Raptis, Jaroslaw Jedrych, Timothy Patton
Nodular cutaneous amyloidosis (NCA), the least common form of primary cutaneous amyloidosis, is characterized clinically by waxy, purpuric plaques and nodules and histologically by amyloid deposits in the dermis and subcutaneous tissue. We present a patient who developed multiple, non-contiguous NCA lesions over a three year period without evidence of systemic disease. We reviewed the literature and found few other cases of this unusual presentation.
May 15, 2017: Dermatology Online Journal
https://www.readbyqxmd.com/read/28537770/automated-description-of-regional-left-ventricular-motion-in-patients-with-cardiac-amyloidosis-a-quantitative-study-using-heart-deformation-analysis
#14
Leng Meng, Kai Lin, Jeremy Collins, Michael Markl, James C Carr
OBJECTIVE: The purpose of this article is to test the hypothesis that heart deformation analysis can automatically quantify regional myocardial motion patterns in patients with cardiac amyloidosis. SUBJECTS AND METHODS: Eleven patients with cardiac amyloidosis and 11 healthy control subjects were recruited to undergo cardiac MRI. Cine images were analyzed using heart deformation analysis and feature tracking. Heart deformation analysis-derived myocardial motion indexes in radial and circumferential directions, including radial and circumferential displacement, radial and circumferential velocity, radial and circumferential strain, and radial and circumferential strain rate, were compared between the two groups...
May 24, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28537040/accuracy-of-99mtc-hydroxymethylene-diphosphonate-scintigraphy-for-diagnosis-of-transthyretin-cardiac-amyloidosis
#15
Francesco Cappelli, Chiara Gallini, Carlo Di Mario, Egidio Natalino Costanzo, Luca Vaggelli, Francesca Tutino, Alfonso Ciaccio, Simone Bartolini, Paola Angelotti, Sabrina Frusconi, Silvia Farsetti, Giuseppe Vergaro, Assuero Giorgetti, Paolo Marzullo, Dario Genovesi, Michele Emdin, Federico Perfetto
BACKGROUND AND AIM: Either 99mTechnetium diphosphonate (Tc-DPD) or pyrophosphate (Tc-PYP) scintigraphy plays a relevant role in diagnosing transthyretin cardiac amyloidosis (CA), and labeled radiotracers have been extensively studied in diagnosing CA. Few studies have analyzed and validated 99mTc-Hydroxymethylene diphosphonate (Tc-HMDP). Our aim was to validate the diagnostic accuracy of Tc-HMDP total-body scintigraphy in a cohort of patients with biopsy-proven transthyretin CA. METHODS AND RESULTS: We retrospectively evaluated all patients undergoing 99mTc-HMDP total-body scintigraphy, in adjunct to a comprehensive diagnostic work-up for suspected CA...
May 23, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28536823/efficacy-and-safety-of-anakinra-in-tumor-necrosis-factor-receptor-associated-periodic-syndrome-traps-complicated-by-severe-renal-failure-a-report-after-long-term-follow-up-and-review-of-the-literature
#16
REVIEW
Stefano Gentileschi, Donato Rigante, Antonio Vitale, Jurgen Sota, Bruno Frediani, Mauro Galeazzi, Luca Cantarini
Tumor necrosis factor receptor-associated periodic syndrome (TRAPS), caused by mutations in the TNFRSF1A gene, is the most frequent autosomal dominant autonflammatory disease displaying a relevant risk of reactive AA amyloidosis, if left untreated. Our report deals with one adult with TRAPS complicated by amyloidosis-related renal failure, treated with the recombinant human interleukin-1 receptor antagonist anakinra at a higher than conventional dosage. This treatment did not present any adverse event and led remarkably to the disappearance of all TRAPS-related manifestations and prompt decrease of laboratory abnormalities, including proteinuria...
May 23, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28536682/a-case-of-intramural-coronary-amyloidosis-associated-with-hemodialysis
#17
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/28536362/functionalisation-of-polyvinylpyrrolidone-on-gold-nanoparticles-enhances-its-anti-amyloidogenic-propensity-towards-hen-egg-white-lysozyme
#18
Tulika Das, Vidyalatha Kolli, Srijeeb Karmakar, Nandini Sarkar
Protein amyloids are characterized by aggregates that usually consist of fibres containing misfolded proteins and having a cross β-sheet conformation. These aggregates can eventually lead to several degenerative diseases like Alzheimer's disease, amyotrophic lateral sclerosis (ALS), Huntington's disease and Parkinson's disease. The present study describes the effect of chemically synthesized polyvinylpyrrolidone (PVP)-conjugated gold nanoparticles (PVP-AuNps) on hen egg white lysozyme (HEWL) amyloids. The synthesized nanoparticles have been characterized using various biophysical techniques like Ultraviolet-Visible (UV-Vis) Spectroscopy, Transmission electron microscopy (TEM), X-ray diffraction (XRD) analysis, dynamic light scattering (DLS), zeta-potential measurement and Fourier transform infrared spectroscopy (FTIR)...
May 3, 2017: Biomedicines
https://www.readbyqxmd.com/read/28536221/middle-lobe-syndrome-an-intriguing-presentation-of-tracheobronchial-amyloidosis
#19
Shekhar Kunal, Shashi Dhawan, Arvind Kumar, Ashok Shah
Pulmonary involvement in amyloidosis is a distinct rarity. This clinical entity usually presents as tracheobronchial amyloidosis (TBA). A 32-year-old, never-smoker man presented with episodic dyspnoea and wheezing along with cough and mucoid sputum. The chest radiograph was suggestive of a middle lobe syndrome (MLS). High-resolution CT (HRCT) of the chest confirmed the presence of MLS. In addition, HRCT showed circumferential thickening of the trachea and the main bronchi, with thickening of the posterior membranous wall of trachea...
May 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28535110/successful-treatment-of-lichen-amyloidosis-accompanied-by-atopic-dermatitis-by-fractional-co2-laser
#20
Howard Chu, Jung U Shin, Jungsoo Lee, Chang Ook Park, Kwang Hoon Lee
Lichen amyloidosis is a subtype of primary cutaneous amyloidosis that presents as persistent, multiple grouped hyperkeratotic papules usually located on the shins, back, forearms, or thighs. The treatment of lichen amyloidosis has been considered to be difficult, and of the various methods available, recent studies have reported the efficacy of fractional CO2 lasers. Lichen amyloidosis may be accompanied by atopic dermatitis, in which the treatment options may be more limited. Herein, we report three cases of lichen amyloidosis accompanied by atopic dermatitis treated by fractional CO2 laser...
May 23, 2017: Journal of Cosmetic and Laser Therapy: Official Publication of the European Society for Laser Dermatology
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