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systemic amyloidosis

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https://www.readbyqxmd.com/read/28229334/specific-amyloid-binding-of-polybasic-peptides-in-vivo-is-retained-by-%C3%AE-sheet-conformers-but-lost-in-the-disrupted-coil-and-all-d-amino-acid-variants
#1
Jonathan S Wall, Angela Williams, Tina Richey, Alan Stuckey, Craig Wooliver, J Christopher Scott, Robert Donnell, Emily B Martin, Stephen J Kennel
PURPOSE: The heparin-reactive, helical peptide p5 is an effective amyloid imaging agent in mice with systemic amyloidosis. Analogs of p5 with modified secondary structure characteristics exhibited altered binding to heparin, synthetic amyloid fibrils, and amyloid extracts in vitro. Herein, we further study the effects of peptide helicity and chirality on specific amyloid binding using a mouse model of systemic inflammation-associated (AA) amyloidosis. PROCEDURES: Peptides with disrupted helical structure [p5(coil) and p5(Pro3)], with an extended sheet conformation [p5(sheet)] or an all-D enantiomer [p5(D)], were chemically synthesized, radioiodinated, and their biodistribution studied in WT mice as well as transgenic animals with severe systemic AA amyloidosis...
February 22, 2017: Molecular Imaging and Biology: MIB: the Official Publication of the Academy of Molecular Imaging
https://www.readbyqxmd.com/read/28225824/caloric-restriction-reduces-the-systemic-progression-of-mouse-aapoaii-amyloidosis
#2
Lin Li, Jinko Sawashita, Xin Ding, Mu Yang, Zhe Xu, Hiroki Miyahara, Masayuki Mori, Keiichi Higuchi
In mouse senile amyloidosis, apolipoprotein (Apo) A-II is deposited extracellularly in many organs in the form of amyloid fibrils (AApoAII). Reduction of caloric intake, known as caloric restriction (CR), slows the progress of senescence and age-related disorders in mice. In this study, we intravenously injected 1 μg of isolated AApoAII fibrils into R1.P1-Apoa2c mice to induce experimental amyloidosis and investigated the effects of CR for the next 16 weeks. In the CR group, AApoAII amyloid deposits in the liver, tongue, small intestine and skin were significantly reduced compared to those of the ad libitum feeding group...
2017: PloS One
https://www.readbyqxmd.com/read/28219562/isolated-pericardial-infiltration-without-myocardial-involvement-in%C3%A2-light-chain-related-amyloidosis
#3
Sophie Georgin-Lavialle, Aziz Guellich, Jean-François Deux, David Buob, Thibaud Damy
Light-chain-related amyloidosis is a systemic disease characterized by continuous accumulation of insoluble fibrillar proteins in different organs. Cardiac involvement is frequent in this condition. However, atypical presentations and unusual amyloid deposits localization may be encountered making the diagnosis challenging. We present here a case of a light-chain-related pericardial amyloidosis without evidence of myocardial involvement and emphasize the difficulty and importance of amyloidosis typing before starting treatment...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28217248/iatrogenic-amyloid-polyneuropathy-after-domino-liver-transplantation
#4
REVIEW
Diana Mnatsakanova, Saša A Živković
Liver transplantation has been used in treatment of transthyretin amyloidosis, and some patients undergo domino liver transplantation (DLT) with explanted liver being transplanted to another patient with liver failure as the liver is otherwise usually functionally normal. Until end of 2015, there were 1154 DLT performed worldwide. DLT for transthyretin amyloidosis is associated with the risk of developing de novo systemic amyloidosis and amyloid neuropathy, and the risk may be greater with some non-Val30Met mutations...
January 28, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28213380/complex-formation-with-pentraxin-2-regulates-factor-x-plasma-levels-and-macrophage-interactions
#5
Vincent Muczynski, Gabriel Aymé, Véronique Regnault, Marc Vasse, Delphine Borgel, Paulette Legendre, Amine Bazaa, Amélie Harel, Cécile Loubière, Peter J Lenting, Cécile V Denis, Olivier D Christophe
Recently, we have identified scavenger receptor-AI (SR-AI) as a receptor for coagulation factor X (FX), mediating the formation of a FX-reservoir at the macrophage-surface (Muczynski, Blood 2016 127:778). Here, we demonstrate that the FX/SR-AI-complex comprises a third protein, pentraxin-2. The presence of pentraxin-2 is essential to prevent internalization of FX by SR-AI, while the presence of FX is needed to interfere with internalization of pentraxin-2. Binding studies showed that FX, SR-AI and pentraxin-2 independently bind to each other (Kd,app: 0...
February 17, 2017: Blood
https://www.readbyqxmd.com/read/28202864/clinical-presentation-of-tubulointerstitial-nephritis-caused-by-amyloid-light-chain-amyloidosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome
#6
Reiko Inoue, Yoshihide Fujigaki, Kana Kobayashi, Yoshifuru Tamura, Tatsuru Ota, Shigeru Shibata, Tsuyoshi Ishida, Fukuo Kondo, Yutaka Yamaguchi, Shunya Uchida
We report a 70-year-old woman with Sjögren's syndrome who had severe renal dysfunction with mild proteinuria and elevated urinary low-molecular-weight proteins. Based on these clinical presentations, interstitial nephritis due to Sjögren's syndrome was strongly suspected. Unexpectedly, renal pathology revealed amyloid light-chain (AL) lambda-type depositions predominantly in the vasculatures with severe tubulointerstitial damage. Concentrated urine immunofixation was positive for Bence Jones lambda-type monoclonal proteins...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28188192/multiple-myeloma-version-3-2017-nccn-clinical-practice-guidelines-in-oncology
#7
Shaji K Kumar, Natalie S Callander, Melissa Alsina, Djordje Atanackovic, J Sybil Biermann, Jason C Chandler, Caitlin Costello, Matthew Faiman, Henry C Fung, Cristina Gasparetto, Kelly Godby, Craig Hofmeister, Leona Holmberg, Sarah Holstein, Carol Ann Huff, Adetola Kassim, Michaela Liedtke, Thomas Martin, James Omel, Noopur Raje, Frederic J Reu, Seema Singhal, George Somlo, Keith Stockerl-Goldstein, Steven P Treon, Donna Weber, Joachim Yahalom, Dorothy A Shead, Rashmi Kumar
Multiple myeloma (MM) is caused by the neoplastic proliferation of plasma cells. These neoplastic plasma cells proliferate and produce monoclonal immunoglobulin in the bone marrow causing skeletal damage, a hallmark of multiple myeloma. Other MM-related complications include hypercalcemia, renal insufficiency, anemia, and infections. The NCCN Multiple Myeloma Panel members have developed guidelines for the management of patients with various plasma cell dyscrasias, including solitary plasmacytoma, smoldering myeloma, multiple myeloma, systemic light chain amyloidosis, and Waldenström's macroglobulinemia...
February 2017: Journal of the National Comprehensive Cancer Network: JNCCN
https://www.readbyqxmd.com/read/28188164/peritoneal-amyloidosis-with-myopathy-in-primary-systemic-al-amyloidosis
#8
Ali Al-Adhami, Kate Steiner, Spencer Ellis
No abstract text is available yet for this article.
February 10, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28178435/an-up-to-date-approach-to-a-patient-with-a-suspected-autoinflammatory-disease
#9
REVIEW
Merav Lidar, Eitan Giat
Autoinflammatory diseases (AID) are characterized by seemingly unprovoked self-limited attacks of fever and systemic inflammation potentially leading to amyloidosis. Familial Mediterranean fever (FMF) is the most common AID and therefore the most studied. Besides FMF, the other main hereditary AID are tumor necrosis factor-associated periodic fever syndrome (TRAPS), mevalonate kinase deficiency (MKD), and cryopyrin-associated periodic fever syndrome (CAPS). These hereditary diseases result from a mutant gene that is involved in the regulation of inflammation, resulting in a characteristic clinical phenotype...
January 30, 2017: Rambam Maimonides Medical Journal
https://www.readbyqxmd.com/read/28160367/cerebral-amyloidoma-is-characterized-by-b-cell-clonality-and-a-stable-clinical-course
#10
Katharina Heß, Jan Purrucker, Ute Hegenbart, Benjamin Brokinkel, Rouven Berndt, Kathy Keyvani, Camelia M Monoranu, Mario Löhr, Guido Reifenberger, Christopher Munoz-Bendix, Jörg Kalla, Justus Groß, Uta Schick, Jennifer Kollmer, Wolfram Klapper, Christoph Röcken, Martin Hasselblatt, Werner Paulus
Amyloidomas are rare amyloid-containing lesions, which may also occur in the central nervous system. Etiology, pathogenesis and clinical course are poorly understood. To gain more insight into the biology of cerebral amyloidoma, we aimed to characterize its histopathological, molecular and clinical features in a retrospective series of seven patients. FFPE tissue specimens were examined using immunohistochemistry, chromogenic in situ hybridization (CISH) for light chains kappa and lambda as well as an IgH gene clonality analysis...
February 4, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28153537/quantitative-assessment-of-systolic-and-diastolic-function-in-patients-with-lge-negative-systemic-amyloidosis-using-cmr
#11
Daniel L R Kuetting, Rami Homsi, Alois M Sprinkart, Julian Luetkens, Daniel K Thomas, Hans H Schild, Darius Dabir
OBJECTIVES: In clinical routine myocardial affection in amyloidosis is assessed by qualitative late gadolinium enhancement (LGE). Recent studies suggest that early cardiac involvement in amyloidosis may be overlooked by qualitative LGE assessment. The aim of this study was to assess possible early cardiac involvement in LGE negative AL patients by means of systolic and diastolic strain assessment and quantitative enhancement. METHODS: 51 individuals (17 Patients with LGE positive light-chain amyloidosis (AL) (Group A), 17 Patients with LGE negative systemic AL (Group B), and 17 healthy controls (Group C)) were investigated...
December 22, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28145535/amyloid-cardiomyopathy
#12
Lucie Karafiatova, Tomas Pika
Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. It is caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, or familial amyloidosis. Amyloid deposition can occur in multiple organs (eg, heart, liver, kidney, skin, eyes, lungs, nervous system) resulting in a variety of clinical manifestations. Cardiac involvement can occur as part of a systemic disease or as a localized phenomenon...
February 1, 2017: Biomedical Papers of the Medical Faculty of the University Palacký, Olomouc, Czechoslovakia
https://www.readbyqxmd.com/read/28134587/amyloidosis-of-the-lung
#13
Andras Khoor, Thomas V Colby
CONTEXT: -Amyloidosis is a heterogeneous group of diseases characterized by the deposition of congophilic amyloid fibrils in the extracellular matrix of tissues and organs. To date, 31 fibril proteins have been identified in humans, and it is now recommended that amyloidoses be named after these fibril proteins. Based on this classification scheme, the most common forms of amyloidosis include systemic AL (formerly primary), systemic AA (formerly secondary), systemic wild-type ATTR (formerly age-related or senile systemic), and systemic hereditary ATTR amyloidosis (formerly familial amyloid polyneuropathy)...
February 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/28130214/the-amyloidogenic-light-chain-is-a-stressor-that-sensitizes-plasma-cells-to-proteasome-inhibitor-toxicity
#14
Laura Oliva, Ugo Orfanelli, Massimo Resnati, Andrea Raimondi, Andrea Orsi, Enrico Milan, Giovanni Palladini, Paolo Milani, Fulvia Cerruti, Paolo Cascio, Simona Casarini, Paola Rognoni, Thierry Touvier, Magda Marcatti, Fabio Ciceri, Silvia Mangiacavalli, Alessandro Corso, Giampaolo Merlini, Simone Cenci
Systemic light chain (AL) amyloidosis is caused by the clonal production of an unstable immunoglobulin light chain (LC), which affects organ function systemically. While pathogenic LCs have been characterized biochemically, little is known about the biology of amyloidogenic PCs. Intrigued by the unique response rates of AL amyloidosis patients to the first-in-class proteasome inhibitor (PI) bortezomib, we purified and investigated patient-derived AL PCs, in comparison with primary multiple myeloma (MM) PCs, the prototypical PI-responsive cells...
January 27, 2017: Blood
https://www.readbyqxmd.com/read/28110103/the-retromer-complex-system-in-a-transgenic-mouse-model-of-ad-influence-of-age
#15
Jin Chu, Domenico Praticò
Deficiencies of the retrograde transport mediated by the retromer complex have been described in Alzheimer's disease (AD). Genetic manipulation of retromer modulates brain amyloidosis in Tg2576 mice. However, whether the complex is altered during the development of the AD-like phenotype remains unknown. In this study we assayed the expression levels of the vacuolar sorting protein 35 (VPS35), VPS26, VPS29, and its cargo proteins, cation independent mannose 6-phosphate receptor, sortilin-related receptor in brains of Tg2576 and controls at the ages of 3, 8, and 14 months...
January 3, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28108533/noncerebral-amyloidoses-aspects-on-seeding-cross-seeding-and-transmission
#16
Gunilla T Westermark, Marcus Fändrich, Katarzyna Lundmark, Per Westermark
More than 30 proteins form amyloid in humans, most of them outside of the brain. Deposition of amyloid in extracerebral tissues is very common and seems inevitable for an aging person. Most deposits are localized, small, and probably without consequence, but in some instances, they are associated with diseases such as type 2 diabetes. Other extracerebral amyloidoses are systemic, with life-threatening effects on the heart, kidneys, and other organs. Here, we review how amyloid may spread through seeding and whether transmission of amyloid diseases may occur between humans...
January 20, 2017: Cold Spring Harbor Perspectives in Medicine
https://www.readbyqxmd.com/read/28095562/the-efficacy-of-q-switched-nd-yag-1064-nm-laser-in-recalcitrant-macular-amyloidosis-a-case-report
#17
Nevil Khurana, Christine Urman
Macular amyloidosis typically presents as small, dusky-brown or greyish pigmented macules, the result of altered keratin deposition. Treatment of these hyperpigmented regions with topical and systemic therapies remains challenging, however Q-Switched neodymi- um-doped yttrium aluminum garnet (ND:YAG) laser has proven to be an effective treatment modality to reduce hyperpigmentation. In this case report we investigated the ef cacy of Q-Switched Nd:YAG laser treatment on a 34-year old woman with recalcitrant macular amyloidosis who failed to respond to over-the-counter bleaching creams...
November 1, 2016: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28090046/an-isolated-case-of-late-onset-amyloidogenic-transthyretin-type-familial-amyloid-polyneuropathy-associated-with-a-mutant-transthyretin-substituting-methionine-for-valine-at-position-30-showing-latent-progressive-cardiac-involvement-confirmed-by-serial-annual
#18
Chikako Sato, Tomofumi Takaya, Shumpei Mori, Kohei Hasegawa, Fumitaka Soga, Hidekazu Tanaka, Yoshiaki Watanabe, Tatsuya Nishii, Atsushi K Kono, Yukiko Morinaga, Hatsue Ishibashi-Ueda, Ken-Ichi Hirata
Late-onset amyloidogenic transthyretin (ATTR) type familial amyloid polyneuropathy (FAP) shows features distinct from those of early-onset hereditary ATTR type FAP. We herein describe an asymptomatic 68-year-old man with late-onset ATTR type FAP whose serial annual electrocardiograms demonstrated progressive left bundle branch block. Latent but severe cardiac involvement seems to be one feature of late-onset ATTR type FAP, similar to senile systemic amyloidosis (SSA). Early differential diagnosis of late-onset ATTR type FAP from SSA is important because, currently, only the former has new therapeutic options available in Japan...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28079687/periocular-amyloidosis-manifesting-as-pseudopemphigoid-treated-with-mitomycin-c
#19
Kaveh Vahdani, Vladimir T Thaller, Giorgio Albanese, Andrew F Dean
PURPOSE: To describe an unusual cicatrizing manifestation of periocular amyloid and the utility of adjuvant antimetabolite therapy. METHODS: This is a case report of a 49-year old woman with progressive bilateral upper lid ptosis, who was found to have bilateral inferior forniceal masses with thickening of the conjunctiva. Conjunctival and eyelid biopsies showed evidence of amyloid deposition. Systemic evaluation did not reveal any evidence of systemic amyloidosis...
January 11, 2017: Cornea
https://www.readbyqxmd.com/read/28078835/pathological-characteristics-of-fifty-patients-with-renal-amyloidosis-in-sri-lanka
#20
D L Dasanayaka, S Wijetunge, N V Ratnatunga
Renal involvement is the most important cause of morbidity and mortality in systemic amyloidosis. This retrospective analysis was conducted to analyse the clinico-pathological characteristics of renal amyloidosis in a group of Sri Lankan patients undergoing renal biopsy. Renal amyloidosis was observed in 50/ 9712 (0.5%) renal biopsies. The underlying cause for amyloidosis was not known in most. Of the known causes multiple myeloma was the commonest. Nephrotic range proteinuria was the most common clinical outcome and most had grade I to III chronic kidney disease at the time of diagnosis...
30, 2016: Ceylon Medical Journal
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