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systematic amyloidosis

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https://www.readbyqxmd.com/read/28526873/role-of-s-palmitoylation-by-zdhhc13-in-mitochondrial-function-and-metabolism-in-liver
#1
Li-Fen Shen, Yi-Ju Chen, Kai-Ming Liu, Amir N Saleem Haddad, I-Wen Song, Hsiao-Yuh Roan, Li-Ying Chen, Jeffrey J Y Yen, Yu-Ju Chen, Jer-Yuarn Wu, Yuan-Tsong Chen
Palmitoyltransferase (PAT) catalyses protein S-palmitoylation which adds 16-carbon palmitate to specific cysteines and contributes to various biological functions. We previously reported that in mice, deficiency of Zdhhc13, a member of the PAT family, causes severe phenotypes including amyloidosis, alopecia, and osteoporosis. Here, we show that Zdhhc13 deficiency results in abnormal liver function, lipid abnormalities, and hypermetabolism. To elucidate the molecular mechanisms underlying these disease phenotypes, we applied a site-specific quantitative approach integrating an alkylating resin-assisted capture and mass spectrometry-based label-free strategy for studying the liver S-palmitoylome...
May 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28373528/addressing-common-questions-encountered-in-the-diagnosis-and-management-of-cardiac-amyloidosis
#2
REVIEW
Mathew S Maurer, Perry Elliott, Raymond Comenzo, Marc Semigran, Claudio Rapezzi
Advances in cardiac imaging have resulted in greater recognition of cardiac amyloidosis in everyday clinical practice, but the diagnosis continues to be made in patients with late-stage disease, suggesting that more needs to be done to improve awareness of its clinical manifestations and the potential of therapeutic intervention to improve prognosis. Light chain cardiac amyloidosis, in particular, if recognized early and treated with targeted plasma cell therapy, can be managed very effectively. For patients with transthyretin amyloidosis, there are numerous therapies that are currently in late-phase clinical trials...
April 4, 2017: Circulation
https://www.readbyqxmd.com/read/28342017/primary-localized-cutaneous-amyloidosis-a-systematic-treatment-review
#3
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/28336182/light-chain-amyloidosis-where-are-the-light-chains-from-and-how-they-play-their-pathogenic-role
#4
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/28277869/disease-burden-of-systemic-light-chain-al-amyloidosis-a-systematic-literature-review
#5
Huamao Mark Lin, Xin Gao, Catherine E Cooke, Deborah Berg, Richard Labotka, Douglas V Faller, Brian Seal, Parameswaran Hari
A systematic literature review on AL amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and knowledge gaps. MEDLINE, EMBASE and COCHRANE databases were searched for English language studies published in the last 10 years using search terms that focused on the clinical, economic, and patient-reported outcome (PRO) aspects of AL amyloidosis. There was a low yield of articles in the economic and PRO categories and additional searches were conducted in clinical conference proceedings, and using Google and Google Scholar...
February 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28254515/hereditary-cerebral-small-vessel-disease-and-stroke
#6
REVIEW
Christian Baastrup Søndergaard, Jørgen Erik Nielsen, Christine Krarup Hansen, Hanne Christensen
Cerebral small vessel disease is considered hereditary in about 5% of patients and is characterized by lacunar infarcts and white matter hyperintensities on MRI. Several monogenic hereditary diseases causing cerebral small vessel disease and stroke have been identified. The purpose of this systematic review is to provide a guide for determining when to consider molecular genetic testing in patients presenting with small vessel disease and stroke. CADASIL, CARASIL, collagen type IV mutations (including PADMAL), retinal vasculopathy with cerebral leukodystrophy, Fabry disease, hereditary cerebral hemorrhage with amyloidosis, and forkhead box C1 mutations are described in terms of genetics, pathology, clinical manifestation, imaging, and diagnosis...
April 2017: Clinical Neurology and Neurosurgery
https://www.readbyqxmd.com/read/28231451/disaggregation-of-human-islet-amyloid-polypeptide-fibril-formation-by-ruthenium-polypyridyl-complexes
#7
Dengsen Zhu, Gehui Gong, Wenji Wang, Weihong Du
The toxicity of amyloid proteins is associated with many degenerative and systematic diseases. The aggregation of human islet amyloid polypeptide may induce pancreatic β-cell death, which is linked to type II diabetes. Ruthenium complexes are inhibitors of various proteins and potential anticancer metallodrugs, which can also be used to disaggregate amyloid proteins. This work reported that several ruthenium polypyridyl complexes remarkably affected the peptide aggregation by predominant hydrophobic interaction and metal coordination, as reflected by thermodynamic parameters and mass spectrometry analysis...
February 13, 2017: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/28226286/peripheral-nerve-ultrasonography-in-patients-with-transthyretin-amyloidosis
#8
Simon Podnar, Stayko Sarafov, Ivailo Tournev, Gregor Omejec, Janez Zidar
OBJECTIVE: To systematically study peripheral nerve morphology in patients with transthyretin (TTR) amyloidosis and TTR gene mutation carriers using high-resolution ultrasonography (US). METHODS: In this prospective cross-sectional study we took a structured history, performed neurological examination, and measured peripheral nerve cross-sectional areas (CSAs) bilaterally at 28 standard locations using US. Demographic and US findings were compared to controls. RESULTS: Peripheral nerve CSAs were significantly larger in 33 patients with familial amyloid polyneuropathy (FAP) compared to 50 controls, most dramatically at the common entrapment sites (median nerve at the wrist, ulnar nerve at the elbow), and in the proximal nerve segments (median nerve in the upper arm, sciatic nerve in the thigh)...
April 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
https://www.readbyqxmd.com/read/28188032/preclinical-alzheimer-s-disease-a-systematic-review-of-the-cohorts-underlying-the-concept
#9
REVIEW
Stéphane Epelbaum, Rémy Genthon, Enrica Cavedo, Marie Odile Habert, Foudil Lamari, Geoffroy Gagliardi, Simone Lista, Marc Teichmann, Hovagim Bakardjian, Harald Hampel, Bruno Dubois
Preclinical Alzheimer's disease (AD) is a relatively recent concept describing an entity characterized by the presence of a pathophysiological biomarker signature characteristic for AD in the absence of specific clinical symptoms. There is rising interest in the scientific community to define such an early target population mainly because of failures of all recent clinical trials despite evidence of biological effects on brain amyloidosis for some compounds. A conceptual framework has recently been proposed for this preclinical phase of AD...
February 7, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28073516/early-diagnosis-of-systemic-amyloidosis-by-means-of-a-transverse-carpal-ligament-biopsy-carried-out-during-carpal-tunnel-syndrome-surgery
#10
Judit Fernández Fuertes, Óscar Rodríguez Vicente, Sergio Sánchez Herráez, Luis Rafael Ramos Pascua
INTRODUCTION AND OBJECTIVE: The systematic analysis of a carpal transverse ligament (CTL) sample obtained during routine carpal tunnel syndrome (CTS) surgery may constitute a method of early diagnosis for systemic amyloidosis. MATERIAL AND METHODS: Prospective study carried out on 147 consecutive CTL samples collected from patients intervened for CTS at the University Hospital of León from April 2006 to May 2007. In those cases in which amyloid deposition was observed in the CTL sample, the study was completed with a fine needle aspiration biopsy (FNAB) of the subcutaneous fascia, using the Red Congo stain in both cases...
March 3, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28065238/selection-of-biopsy-site-for-patients-with-systematic-amyloidosis
#11
Chun-Lan Zhang, Jun Feng, Xin-Xin Cao, Cong-Li Zhang, Kai-Ni Shen, Xu-Fei Huang, Lu Zhang, Dao-Bin Zhou, Jian Li
Objective To evaluate the sensitivities of various biopsy methods for the diagnosis of systematic amyloidosis (SA). Methods The clinical data and biopsy results of 194 SA patients who were treated in Peking Union Medical College Hospital from January 2009 to June 2015 were retrospectively analyzed. Results The highest sensitivity was achieved by biopsy of affected organs,with renal biopsy 97.4%,heart biopsy 95.0% and liver biopsy 87.5%. Among non-invasive biopsy methods,tongue biopsy was found to be 75% sensitive,followed by gingiva biopsy at 57%,abdominal fat pad aspiration at 57%,rectum biopsy at 16%,and bone marrow examination at 8%...
December 20, 2016: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28031465/aggregation-of-full-length-immunoglobulin-light-chains-from-systemic-light-chain-amyloidosis-al-patients-is-remodeled-by-epigallocatechin-3-gallate
#12
Kathrin Andrich, Ute Hegenbart, Christoph Kimmich, Niraja Kedia, H Robert Bergen, Stefan Schönland, Erich Wanker, Jan Bieschke
Intervention into amyloid deposition with anti-amyloid agents like the polyphenol epigallocatechin-3-gallate (EGCG) is emerging as an experimental secondary treatment strategy in systemic light chain amyloidosis (AL). In both AL and multiple myeloma (MM), soluble immunoglobulin light chains (LC) are produced by clonal plasma cells, but only in AL do they form amyloid deposits in vivo We investigated the amyloid formation of patient-derived LC and their susceptibility to EGCG in vitro to probe commonalities and systematic differences in their assembly mechanisms...
February 10, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28018795/nephrotoxicity-of-methadone-a-systematic-review
#13
REVIEW
Samira Alinejad, Kazem Ghaemi, Mohammad Abdollahi, Omid Mehrpour
BACKGROUND: Methadone is commonly administered for chronic pain relief and treatment of opioid dependence. Concurrent with its increased consumption, toxicities and fatalities have increased. One of the adverse effects of opioid analgesics, including methadone, is that of nephrotoxicity. Opioids can have an effect on renal function through several different mechanisms. METHODS: We searched common bibliographical databases for the terms methadone, toxicity, poisoning, kidney, renal, and nephrotoxicity and summarize our findings in this review...
2016: SpringerPlus
https://www.readbyqxmd.com/read/27760547/secondary-bladder-amyloidosis-with-familial-mediterranean-fever-in-a-living-donor-kidney-transplant-recipient-a-case-report
#14
Sentaro Imamura, Shintaro Narita, Ryuta Nishikomori, Hiroshi Tsuruta, Kazuyuki Numakura, Atsushi Maeno, Mitsuru Saito, Takamitsu Inoue, Norihiko Tsuchiya, Hiroshi Nanjo, Toshio Heike, Shigeru Satoh, Tomonori Habuchi
BACKGROUND: Secondary bladder amyloidosis is an extremely rare disease, resulting from a chronic systematic inflammatory disorder associated with amyloid deposits. Although uncommon in Japan, familial Mediterranean fever (FMF) is a hereditary autoinflammatory disease characterized by recurrent episodes of fever of short duration and serositis and is frequently associated with systemic amyloidosis. Here, we present a case of a Japanese patient complaining of fever and macroscopic hematuria after a living donor renal transplantation...
October 19, 2016: BMC Research Notes
https://www.readbyqxmd.com/read/27568115/prognostic-value-of-late%C3%A2-gadolinium%C3%A2-enhancement-cmr-in%C3%A2-systemic-amyloidosis
#15
Sameer Raina, Shelly Y Lensing, Ramez S Nairooz, Naga Venkata K Pothineni, Abdul Hakeem, Sabha Bhatti, Tarun Pandey
OBJECTIVES: The aim of this study was to access the prognostic implication of late gadolinium enhancement (LGE) in patients with systemic amyloidosis undergoing cardiac magnetic resonance (CMR). BACKGROUND: Cardiac amyloidosis confers significantly worse prognosis in patients with systemic amyloidosis. CMR imaging has emerged as an attractive noninvasive modality to diagnose cardiac involvement in patients with systemic amyloidosis. We performed a systemic review and meta-analysis to evaluate the prognostic role of LGE-CMR imaging in patients with systemic amyloidosis...
November 2016: JACC. Cardiovascular Imaging
https://www.readbyqxmd.com/read/27567877/a%C3%AE-pp-ps1-transgenic-mice-show-sex-differences-in-the-cerebellum-associated-with-aging
#16
Lara Ordoñez-Gutierrez, Ivan Fernandez-Perez, Jose Luis Herrera, Marta Anton, Irene Benito-Cuesta, Francisco Wandosell
Cerebellar pathology has been related to presenilin 1 mutations in certain pedigrees of familial Alzheimer's disease. However, cerebellum tissue has not been intensively analyzed in transgenic models of mutant presenilins. Furthermore, the effect of the sex of the mice was not systematically analyzed, despite the fact that important gender differences in the evolution of the disease in the human population have been described. We analyzed whether the progression of amyloidosis in a double transgenic mouse, AβPP/PS1, is susceptible to aging and differentially affects males and females...
September 6, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27513652/outcome-and-incidence-of-appropriate-implantable-cardioverter-defibrillator-therapy-in-patients-with-cardiac-amyloidosis
#17
David Hamon, Vincent Algalarrondo, Estelle Gandjbakhch, Fabrice Extramiana, Eloi Marijon, Nathalie Elbaz, Dounia Selhane, Jean-Luc Dubois-Rande, Emmanuel Teiger, Violaine Plante-Bordeneuve, Thibaud Damy, Nicolas Lellouche
BACKGROUND: Cardiac amyloidosis (CA) is associated with a poor prognosis with the proposed mechanism of sudden cardiac death in the majority of patients being pulseless electrical activity. However, the incidence of ventricular arrhythmias (VA) and implantable cardioverter-defibrillator (ICD) indications in CA patients are unclear. We performed a detailed evaluation of our CA population undergoing ICD implantation and assessed appropriate ICD therapy and survival predictors. METHODS: We included consecutive patients from June 2008 to November 2014 in five centers...
November 1, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/27422051/transthyretin-amyloid-neuropathy-has-earlier-neural-involvement-but-better-prognosis-than-primary-amyloid-counterpart-an-answer-to-the-paradox
#18
Adam J Loavenbruck, Wolfgang Singer, Michelle L Mauermann, Paola Sandroni, P James B Dyck, Morie Gertz, Christopher J Klein, Phillip A Low
OBJECTIVE: To systematically compare transthyretin with primary amyloid neuropathy to define their natural history and the underlying mechanisms for differences in phenotype and natural history. METHODS: All patients with defined amyloid subtype and peripheral neuropathy who completed autonomic testing and electromyography at Mayo Clinic Rochester between 1993 and 2013 were included. Medical records were reviewed for time of onset of defined clinical features. The degree of autonomic impairment was quantified using the composite autonomic severity scale...
September 2016: Annals of Neurology
https://www.readbyqxmd.com/read/27328652/the-incidence-of-senile-cataract-and-glaucoma-is-increased-in-patients-with-plasma-cell-dyscrasias-etiologic-implications
#19
Kari Hemminki, Asta Försti, Raimo Tuuminen, Otto Hemminki, Hartmut Goldschmidt, Kristina Sundquist, Jan Sundquist, Xinjun Li
Plasma cell dyscrasias, including monoclonal gammopathy of undetermined significance (MGUS), multiple myeloma (MM), Waldenström macroglobulinemia (WM) and light chain AL amyloidosis, are characterized by clonal expansion of plasma cells which produce a vast amount of an immunoglobulin-derived M-protein. We noted that MGUS diagnosis often coincided with diagnoses of senile cataract and glaucoma and tested the associations of MGUS, MM, WM and AL amyloidosis with subsequent eye diseases identified from the Swedish patient registers between 1997 and 2012...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27267362/diagnostic-accuracy-of-cardiovascular-magnetic-resonance-for-patients-with-suspected-cardiac-amyloidosis-a-systematic-review-and-meta-analysis
#20
Lei Zhao, Zhuang Tian, Quan Fang
BACKGROUND: This study is a systematic review and meta-analysis of the diagnostic value of cardiovascular magnetic resonance (CMR) in cardiac amyloidosis (CA). METHODS: A wide variety of electronic databases were searched for studies of CMR that reported the diagnostic accuracy in patients with suspected CA. Research manuscripts were subjected to further systematic review and meta-analysis. Methodological evaluation was performed under the guidance of the Quality Assessment of Diagnostic Accuracy Studies -2 (QUADAS-2)...
2016: BMC Cardiovascular Disorders
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