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systemic amyloidosis

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https://www.readbyqxmd.com/read/28439924/prevalence-and-predictors-of-thyroid-functional-abnormalities-in-newly-diagnosed-al-amyloidosis
#1
E Muchtar, D S Dean, A Dispenzieri, D Dingli, F K Buadi, M Q Lacy, S R Hayman, P Kapoor, N Leung, S Russell, J A Lust, Yi Lin, R Warsame, W Gonsalves, T V Kourelis, R S Go, R Chakraborty, S Zeldenrust, R A Kyle, S Vincent Rajkumar, S K Kumar, M A Gertz
BACKGROUND: Data on the effect of systemic immunoglobulin light chain amyloidosis (AL amyloidosis) on thyroid function are limited. OBJECTIVE: To assess the prevalence of hypothyroidism in AL amyloidosis patients and determine its predictors. METHODS: 1142 newly diagnosed AL amyloidosis patients were grouped based on the thyroid-stimulating hormone (TSH) measurement at diagnosis: hypothyroid group (TSH above upper normal reference; >5 mIU L(-1) ; n = 217, 19% of study participants) and euthyroid group (n = 925, 81%)...
April 24, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28436370/clinical-neuropathology-image-3-2017-cns-involvement-in-systemic-amyloidosis-restricted-to-the-choroid-plexus
#2
Ellen Gelpi, Martin Susani, Robert Wiebringhaus, Andreas Aschauer, Andreas Kammerlander, Mirjam I Lutz, Johannes A Hainfellner
No abstract text is available yet for this article.
April 24, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#3
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28429761/a-specific-nanobody-prevents-amyloidogenesis-of-d76n-%C3%AE-2-microglobulin-in-vitro-and-modifies-its-tissue-distribution-in-vivo
#4
Sara Raimondi, Riccardo Porcari, P Patrizia Mangione, Guglielmo Verona, Julien Marcoux, Sofia Giorgetti, Graham W Taylor, Stephan Ellmerich, Maurizio Ballico, Stefano Zanini, Els Pardon, Raya Al-Shawi, J Paul Simons, Alessandra Corazza, Federico Fogolari, Manuela Leri, Massimo Stefani, Monica Bucciantini, Julian D Gillmore, Philip N Hawkins, Maurizia Valli, Monica Stoppini, Carol V Robinson, Jan Steyaert, Gennaro Esposito, Vittorio Bellotti
Systemic amyloidosis is caused by misfolding and aggregation of globular proteins in vivo for which effective treatments are urgently needed. Inhibition of protein self-aggregation represents an attractive therapeutic strategy. Studies on the amyloidogenic variant of β2-microglobulin, D76N, causing hereditary systemic amyloidosis, have become particularly relevant since fibrils are formed in vitro in physiologically relevant conditions. Here we compare the potency of two previously described inhibitors of wild type β2-microglobulin fibrillogenesis, doxycycline and single domain antibodies (nanobodies)...
April 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28426958/elucidating-the-role-of-trem2-in-alzheimer-s-disease
#5
REVIEW
Jason D Ulrich, Tyler K Ulland, Marco Colonna, David M Holtzman
Alzheimer's disease (AD) is the sixth leading cause of death in the United States and the most common cause of dementia in the elderly. Genetic factors, such as rare variants in the microglial-expressed gene TREM2, strongly impact the lifetime risk of developing AD. Several recent studies have described dramatic TREM2-dependent phenotypes in mouse models of amyloidosis that point to an important role for TREM2 in regulating the response of the innate immune system to Aβ pathology. Furthermore, elevations in the CSF levels of soluble TREM2 fragments implicate changes in inflammatory pathways as occurring coincident with markers of neuronal damage and the onset of clinical dementia in AD...
April 19, 2017: Neuron
https://www.readbyqxmd.com/read/28421832/experimental-induction-of-chicken-aa-amyloidosis-in-white-layer-chickens-by-inoculation-with-inactivated-vaccines
#6
Wazir Ahmad Habibi, Takuya Hirai, Mohammad Hakim Niazmand, Naoko Okumura, Ryoji Yamaguchi
We investigated the amyloidogenic potential of inactivated vaccines and the localized production of serum amyloid A (SAA) at the injection site in white layer chickens. Hens in the treated group were injected intramuscularly three times with high doses of inactivated oil-emulsion Salmonella Enteritidis vaccine and multivalent viral and bacterial inactivated oil-emulsion vaccines at two week intervals. Chickens in the control group did not receive any inoculum. In the treated group, emaciation, and granulomas were present, while several chickens died between 4-6 weeks after the first injection...
April 19, 2017: Avian Pathology: Journal of the W.V.P.A
https://www.readbyqxmd.com/read/28418090/natural-mortality-and-cause-of-death-analysis-of-the-captive-chimpanzee-pan-troglodytes-a-35-year-review
#7
REVIEW
Hannah Laurence, Shyamesh Kumar, Michael A Owston, Robert E Lanford, Gene B Hubbard, Edward J Dick
We present the spontaneous causes of mortality for 137 chimpanzees (Pan troglodytes) over a 35-year period. A record review of the pathology database was performed and a primary cause of mortality was determined for each chimpanzee. The most common causes of mortality were as follows: cardiomyopathy (40% of all mortalities), stillbirth/abortion, acute myocardial necrosis, chimpanzee-induced trauma, amyloidosis, and pneumonia. Five morphologic diagnoses accounted for 61% of mortalities: cardiomyopathy, hemorrhage, acute myocardial necrosis, amyloidosis, and pneumonia...
April 18, 2017: Journal of Medical Primatology
https://www.readbyqxmd.com/read/28412170/lipid-nanoparticle-systems-for-enabling-gene-therapies
#8
REVIEW
Pieter R Cullis, Michael J Hope
Genetic drugs such as small interfering RNA (siRNA), mRNA, or plasmid DNA provide potential gene therapies to treat most diseases by silencing pathological genes, expressing therapeutic proteins, or through gene-editing applications. In order for genetic drugs to be used clinically, however, sophisticated delivery systems are required. Lipid nanoparticle (LNP) systems are currently the lead non-viral delivery systems for enabling the clinical potential of genetic drugs. Application will be made to the Food and Drug Administration (FDA) in 2017 for approval of an LNP siRNA drug to treat transthyretin-induced amyloidosis, presently an untreatable disease...
April 13, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28412068/vitreous-amyloidosis-ocular-systemic-and-genetic-insights
#9
Pradeep Venkatesh, Harathy Selvan, Sundararajan Baskar Singh, Divya Gupta, Seema Kashyap, Shreyas Temkar, Varun Gogia, Koushik Tripathy, Rohan Chawla, Rajpal Vohra
PURPOSE: To report the unique clinical and surgical characteristics encountered in eyes with vitreous amyloidosis. Systemic evaluation and visual outcome after vitrectomy are discussed. A novel mutation in the transthyretin gene (TTR) in Indian patients with familial amyloid polyneuropathy (FAP) is described. DESIGN: Retrospective, observational study. PARTICIPANTS: Ten eyes of 5 patients from 2 pedigrees with a diagnosis of vitreous amyloidosis...
April 12, 2017: Ophthalmology
https://www.readbyqxmd.com/read/28394371/stem-cell-transplantation-in-patients-with-systemic-al-amyloidosis-referred-for-transplant-after-suboptimal-responses-to-bortezomib-based-initial-therapy
#10
S W Wong, D Larivee, M Warner, K A Sprague, T Fogaren, R L Comenzo
No abstract text is available yet for this article.
April 10, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28393574/transbronchial-biopsies-safely-diagnose-amyloid-lung-disease
#11
Praveen Govender, Colleen M Keyes, Elizabeth A Hankinson, Carl J O'Hara, Vaishali Sanchorawala, John L Berk
BACKGROUND: Autopsy identifies lung involvement in 58-92% of patients with the most prevalent forms of systemic amyloidoses. In the absence of lung biopsies, amyloid lung disease often goes unrecognized. Report of a death following transbronchial biopsies in a patient with systemic amyloidosis cautioned against the procedure in this patient cohort. We reviewed our experience with transbronchial biopsies in patients with amyloidosis to determine the safety and utility of bronchoscopic lung biopsies...
April 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28390893/targeting-glutamatergic-and-cellular-prion-protein-mechanisms-of-amyloid-%C3%AE-mediated-persistent-synaptic-plasticity-disruption-longitudinal-studies
#12
Dainan Zhang, Yingjie Qi, Igor Klyubin, Tomas Ondrejcak, Claire J Sarell, A Claudio Cuello, John Collinge, Michael J Rowan
Alzheimer's disease amyloid-β (Aβ) oligomers are synaptotoxic, inappropriately increasing extracellular glutamate concentration and glutamate receptor activation to thereby rapidly disrupt synaptic plasticity. Thus, acutely promoting brain glutamate homeostasis with a blood-based scavenging system, glutamate-oxaloacetate transaminase (GOT), and blocking metabotropic glutamate 5 (mGlu5) receptor or its co-receptor cellular prion protein (PrP), prevent the acute inhibition of long-term potentiation (LTP) by exogenous Aβ...
April 5, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28370245/renal-outcomes-in-patients-with-al-amyloidosis-prognostic-factors-renal-response-and-the-impact-of-therapy
#13
Efstathios Kastritis, Maria Gavriatopoulou, Maria Roussou, Magdalini Migkou, Despina Fotiou, Dimitrios C Ziogas, Nikos Kanellias, Evangelos Eleutherakis-Papaiakovou, Ioannis Panagiotidis, Stavroula Giannouli, Erasmia Psimenou, Smaragdi Marinaki, Theofanis Apostolou, Hariklia Gakiopoulou, Anna Tasidou, Ioannis Papassotiriou, Evangelos Terpos, Meletios A Dimopoulos
A staging system for patients with renal AL amyloidosis, based on eGFR (<50 ml/min/1.73 m(2) ) and proteinuria (≥5gr/day) at diagnosis, as well as criteria for renal progression (≥25% eGFR reduction) and response (≥30% reduction of proteinuria without renal progression) were recently proposed. We validated these criteria in a cohort of 125 patients with renal AL amyloidosis, mostly treated with bortezomib or lenalidomide. We confirmed the prognostic value of the renal staging system but also identified the limitations of renal progression criteria which are based only on eGFR reduction...
March 28, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28367947/a-case-of-amyloid-myopathy-diagnosed-during-the-treatment-of-myopathy-associated-with-anti-signal-recognition-particle-antibodies
#14
Nobuko Kawakami, Yusuke Katsuyama, Yuka Hagiwara, Hidefumi Yoshida, Kang Kim, Kiyoshi Harada
A 78-year-old man presented with subacute progressive proximal weakness and dysphagia. A biopsy specimen from the left biceps femoris revealed evidence of necrotic and regenerating muscle fibers, but lymphocyte infiltration was not noted. The patient was diagnosed with necrotizing myopathy with anti-signal recognition particle (SRP) antibodies. Concomitant therapy with prednisolone and azathioprine caused the serum CK level to return to normal and it caused clinical manifestations to abate. One year later, however, muscle weakness worsened...
March 30, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28367859/identification-and-assessment-of-cardiac-amyloidosis-by-myocardial-strain-analysis-of-cardiac-magnetic-resonance-imaging
#15
Seitaro Oda, Daisuke Utsunomiya, Takeshi Nakaura, Hideaki Yuki, Masafumi Kidoh, Kosuke Morita, Seiji Takashio, Megumi Yamamuro, Yasuhiro Izumiya, Kyoko Hirakawa, Toshifumi Ishida, Kenichi Tsujita, Mitsuharu Ueda, Taro Yamashita, Yukio Ando, Hiroyuki Hata, Yasuyuki Yamashita
BACKGROUND: We explored the usefulness of myocardial strain analysis on cardiac magnetic resonance imaging (CMR) scans for the identification of cardiac amyloidosis.Methods and Results:The 61 patients with systemic amyloidosis underwent 3.0-T CMR, including CMR tagging and late-gadolinium enhanced (LGE) imaging. The circumferential strain (CS) of LGE-positive and LGE-negative patients was measured on midventricular short-axis images and compared. Logistic regression modeling of CMR parameters was performed to detect patients with LGE-positive cardiac amyloidosis...
March 31, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28366333/high-prevalence-of-recurrent-nocturnal-desaturations-in-systemic-al-amyloidosis-a-cross-sectional-pilot-study
#16
Shameem Mahmood, Milind Sovani, Peter Smith, Leena George, Christina C Quarta, Sajitha Sachchithanantham, Marianna Fontana, Carol J Whelan, Helen J Lachmann, Julian D Gillmore, Philip N Hawkins, Ashutosh D Wechalekar
OBJECTIVES: Cardiac involvement and/or macroglossia with soft tissue deposits are risk factors for central sleep apnoea (CSA) and obstructive sleep apnoea (OSA), and common features of systemic AL amyloidosis. Little data exist on the occurrence of sleep-disordered breathing (SDB) or recurrent nocturnal hypoxia in amyloidosis, which this study sought to investigate. METHODS: A total of 72 consecutive patients with systemic amyloidosis (mean age 69 years and mean BMI 25) were evaluated for occurrence of SDB, by overnight continuous pulse oximetry, and completed Epworth Sleepiness Score (ESS) and STOPBANG questionnaires...
April 2017: Sleep Medicine
https://www.readbyqxmd.com/read/28364177/magnetic-resonance-imaging-for-characterizing-myocardial-diseases
#17
REVIEW
Maythem Saeed, Hui Liu, Chang-Hong Liang, Mark W Wilson
The National Institute of Health defined cardiomyopathy as diseases of the heart muscle. These myocardial diseases have different etiology, structure and treatment. This review highlights the key imaging features of different myocardial diseases. It provides information on myocardial structure/orientation, perfusion, function and viability in diseases related to cardiomyopathy. The standard cardiac magnetic resonance imaging (MRI) sequences can reveal insight on left ventricular (LV) mass, volumes and regional contractile function in all types of cardiomyopathy diseases...
March 31, 2017: International Journal of Cardiovascular Imaging
https://www.readbyqxmd.com/read/28361953/cell-to-cell-transfer-of-saa1-protein-in-a-cell-culture-model-of-systemic-aa-amyloidosis
#18
Stephanie Claus, Ioana Puscalau-Girtu, Paul Walther, Tatiana Syrovets, Thomas Simmet, Christian Haupt, Marcus Fändrich
Systemic AA amyloidosis arises from the misfolding of serum amyloid A1 (SAA1) protein and the deposition of AA amyloid fibrils at multiple sites within the body. Previous research already established that mononuclear phagocytes are crucial for the formation of the deposits in vivo and exposure of cultures of such cells to SAA1 protein induces the formation of amyloid deposits within the culture dish. In this study we show that both non-fibrillar and fibrillar SAA1 protein can be readily transferred between cultured J774A...
March 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28352636/unicentric-castleman-s-disease-associated-with-end-stage-renal-disease-caused-by-amyloidosis
#19
Eray Eroglu, Ismail Kocyigit, Aydin Unal, Murat Hayri Sipahioglu, Hulya Akgun, Leylagul Kaynar, Bulent Tokgoz, Oktay Oymak
Castleman's disease (CD), also known as angiofolicular lymph node hyperplasia, is a rare heterogenous group of lymphoproliferative disorders. Histologically, it can be classified as hyaline vascular type, plasma cell type, or mixed type. Clinically two different subtypes of the CD are present: Unicentric and multicentric. Unicentric CD is generally asymptomatic and associated with hyaline vascular type, and its diagnoses depend on the localized lymphadenopathy on examination or imaging studies. However, multicentric CD presents with generalized lymphadenopathy and systemic symptoms including malaise, fever, night sweats, weight loss, and it is associated with the plasma cell type and mix type...
March 16, 2017: World Journal of Clinical Cases
https://www.readbyqxmd.com/read/28350808/differential-recruitment-efficacy-of-patient-derived-amyloidogenic-and-myeloma-light-chain-proteins-by-synthetic-fibrils-a-metric-for-predicting-amyloid-propensity
#20
Emily B Martin, Angela Williams, Craig Wooliver, R Eric Heidel, Sarah Adams, John Dunlap, Marina Ramirez-Alvarado, Luis M Blancas-Mejia, Ronald H Lands, Stephen J Kennel, Jonathan S Wall
BACKGROUND: Monoclonal free light chain (LC) proteins are present in the circulation of patients with immunoproliferative disorders such as light chain (AL) amyloidosis and multiple myeloma (MM). Light chain-associated amyloid is a complex pathology composed of proteinaceous fibrils and extracellular matrix proteins found in all patients with AL and in ~10-30% of patients who presented with MM. Amyloid deposits systemically in multiple organs and tissues leading to dysfunction and ultimately death...
2017: PloS One
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