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https://www.readbyqxmd.com/read/29303069/unfolding-cardiac-amyloidosis-from-pathophysiology-to-cure
#1
Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P Rainer
Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated...
January 4, 2018: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/29280764/primary-central-nervous-system-lymphoma-with-light-chain-deposition-disease-aggregoma
#2
Tyler D Boulter, Ama Sadaka, Mohammad Obadah Nakawah, Stacy V Smith, Nail Alouch, Shauna E Berry, Andrew T Whyte, Gregory N Fuller, Andrew G Lee
No abstract text is available yet for this article.
December 26, 2017: Journal of Neuro-ophthalmology: the Official Journal of the North American Neuro-Ophthalmology Society
https://www.readbyqxmd.com/read/29250142/cerebral-embolism-secondary-to-cardiac-amyloidosis-a-case-report-and-literature-review
#3
Xu-Dong Zhang, Ying-Xian Liu, Xiao-Wei Yan, Li-Gang Fang, Quan Fang, Da-Chun Zhao, Yi-Ning Wang
Cardiac amyloidosis (CA) describes a group of heterogeneous diseases that are characterized by the extracellular fibril deposition of amyloid protein in the myocardium. The abnormal protein is usually derived from light-chain amyloidosis, mutant transthyretin amyloidosis and wild-type transthyretin. Patients with ischemic strokes and amyloidosis have been sporadically reported, however, they are not well summarized. In the present study, a case of cerebral ischemic stroke, secondary to CA was described. This patient presented with dyspnea on exertion, without any evidence of atrial fibrillation...
December 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29233648/left-atrial-dysfunction-in-light-chain-cardiac-amyloidosis-and-hypertrophic-cardiomyopathy-a-comparative-three-dimensional-speckle-tracking-echocardiographic-analysis-from-the-magyar-path-study
#4
Dóra Földeák, Árpád Kormányos, Péter Domsik, Anita Kalapos, Györgyike Á Piros, Nóra Ambrus, Zénó Ajtay, Róbert Sepp, Zita Borbényi, Tamás Forster, Attila Nemes
INTRODUCTION: While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE)...
December 9, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29222231/al-amyloidosis-from-molecular-mechanisms-to-targeted-therapies
#5
REVIEW
Giampaolo Merlini
Systemic amyloidosis is caused by misfolding and extracellular deposition of circulating proteins as amyloid fibrils, resulting in the dysfunction of vital organs. The most common systemic amyloidosis, light-chain (AL) amyloidosis, is caused by misfolded light chains produced by a small, dangerous B-cell clone. The process of amyloid formation, organ targeting, and damage is multifaceted and, after disease initiation, the complexity of the downstream pathogenic cascade increases, rendering its control a challenge...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29212103/recurrent-diffuse-gastric-bleeding-as-a-leading-symptom-of%C3%A2-gastrointestinal-al-amyloidosis
#6
Caspar Franck, Marino Venerito, Jochen Weigt, Albert Roessner, Peter Malfertheiner
Amyloidosis is a rare disease (incidence about 0.8/100 000) characterized by extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of serum proteins. Clinical manifestations are largely determined by the type of precursor protein, the tissue distribution and the amount of amyloid deposition. Gastrointestinal (GI) manifestations of amyloidosis are even more uncommon (3 % of all amyloidosis patients). Symptoms of GI amyloidosis are nonspecific, heterogeneous, and include weight loss, GI bleeding, heartburn, early satiety, diarrhea and abdominal pain...
December 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29209544/a-case-of-proliferative-glomerulonephritis-with-monoclonal-igg-deposits-that-showed-predominantly-membranous-features
#7
Homare Shimohata, Kentaro Ohgi, Hiroshi Maruyama, Yasunori Miyamoto, Mamiko Takayashu, Kouichi Hirayama, Masaki Kobayashi
In 2004, the novel category of monoclonal IgG deposition disease has been proposed and termed "proliferative glomerulonephritis with monoclonal IgG deposits" (PGNMID). This disease is characterized by membranoproliferative glomerulonephritis and staining for a single light-chain isotype and gamma heavy-chain subclass. A 76-year-old male who had monoclonal gammopathy was referred to our hospital because of proteinuria. The renal biopsy showed diffuse thickening of the glomerular capillary walls with focal mesangial proliferation...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/29186589/detergent-insoluble-proteins-and-inclusion-body-like-structures-immunoreactive-for-prkdc-dna-pk-dna-pkcs-ftl-nnt-and-aifm1-in-the-amygdala-of-cognitively-impaired-elderly-persons
#8
Jozsef Gal, Jing Chen, Yuriko Katsumata, David W Fardo, Wang-Xia Wang, Sergey Artiushin, Douglas Price, Sonya Anderson, Ela Patel, Haining Zhu, Peter T Nelson
Misfolded protein in the amygdala is a neuropathologic feature of Alzheimer disease and many other neurodegenerative disorders. We examined extracts from human amygdala (snap-frozen at autopsy) to investigate whether novel and as yet uncharacterized misfolded proteins would be detectable. Polypeptides from the detergent-insoluble, urea-soluble protein fractions of amygdala were interrogated using liquid chromatography-electrospray ionization-tandem mass spectrometry. Among the detergent-insoluble proteins identified in amygdala of demented subjects but not controls were Tau, TDP-43, Aβ, α-synuclein, and ApoE...
November 24, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/29155738/imaging-amyloid-tissues-stained-with-luminescent-conjugated-oligothiophenes-by-hyperspectral-confocal-microscopy-and-fluorescence-lifetime-imaging
#9
Sofie Nyström, Marcus Bäck, K Peter R Nilsson, Per Hammarström
Proteins that deposit as amyloid in tissues throughout the body can be the cause or consequence of a large number of diseases. Among these we find neurodegenerative diseases such as Alzheimer's and Parkinson's disease afflicting primarily the central nervous system, and systemic amyloidosis where serum amyloid A, transthyretin and IgG light chains deposit as amyloid in liver, carpal tunnel, spleen, kidney, heart, and other peripheral tissues. Amyloid has been known and studied for more than a century, often using amyloid specific dyes such as Congo red and Thioflavin T (ThT) or Thioflavin (ThS)...
October 20, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29142973/renal-amyloidosis-associated-with-5-novel%C3%A2-variants-in-the-fibrinogen-a-alpha-chain-protein
#10
Dorota Rowczenio, Maria Stensland, Gustavo A de Souza, Erik H Strøm, Janet A Gilbertson, Graham Taylor, Nigel Rendell, Shane Minogue, Yvonne A Efebera, Helen J Lachmann, Ashutosh D Wechalekar, Philip N Hawkins, Ketil R Heimdal, Kristian Selvig, Inger K Lægreid, Nathalie Demoulin, Selda Aydin, Julian D Gillmore, Tale N Wien
Introduction: Fibrinogen A alpha chain amyloidosis is an autosomal dominant disease associated with mutations in the fibrinogen A alpha chain (FGA) gene, and it is the most common cause of hereditary renal amyloidosis in the UK. Patients typically present with kidney impairment and progress to end-stage renal disease over a median time of 4.6 years. Methods: Six patients presented with proteinuria, hypertension, and/or lower limb edema and underwent detailed clinical and laboratory investigations...
May 2017: KI Reports
https://www.readbyqxmd.com/read/29136724/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-renal-impairment-of-multiple-myeloma
#11
(no author information available yet)
Renal impairment (RI) is a common complication of multiple myeloma (MM), which is presented as chronic kidney disease (CKD) or acute kidney injury (AKI). The typical pathological feature is cast nephropathy. Presently international system staging (ISS) is used in evaluating MM. Although the classic Durie-Salmon staging system could be still used in clinical practice, it may miss out some patients with renal impairment. For evaluations of RI in MM patients with CKD, it's recommended to assess the estimated glomerular filtration rate (eGFR) by creatinine based formula CKD-epidemiology collaboration (EPI) or modification of diet in renal disease(MDRD) and to stage the renal injuries according to 2013 Kidney Disease Improving Global Outcomes (KDIGO) CKD guidelines...
November 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/29135016/light-chain-deposition-disease-with-bullous-skin-lesions-mimicking-atypical-bullous-pemphigoid
#12
Marion Malphettes, Pauline Bonnet, Pierre Schneider, Marguerite Vignon, Marine Baron, Jean-David Bouaziz, Maxime Battistella
No abstract text is available yet for this article.
August 23, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/29130681/nodular-glomerulosclerosis-not-always-diabetic-nephropathy
#13
Olga Balafa, Georgios Liapis
Nodular glomerulosclerosis -as it was originally described by Kimmelstiel and Wilson- is the main pathognomonic lesion of diabetic nephropathy. However, this particular pattern can also be seen in other conditions such as membranoproliferative glomerulonephritis, light or heavy chain deposition disease, amyloidosis, fibrillary and immunotactoid glomerulonephritis and Takayasu's arteritis. History of diabetes as well as other clinical and pathological features are of crucial importance in order to determine the precise cause of nodular glomerulosclerosis...
November 10, 2017: Minerva Medica
https://www.readbyqxmd.com/read/29043147/a-case-report-of-paraproteinemia-associated-pauci-immune-glomerulonephritis-a-new-form-of-monoclonal-gammopathy-of-renal-significance
#14
Robert Rope, Neeraja Kambham, Neiha Arora
BACKGROUND: Renal disease associated with paraproteinemias is classically predicated upon pathologic paraprotein deposition in the kidney. However, growing evidence suggests that paraproteins may be able to systemically activate complement or neutrophils to drive renal damage. This may provide an alternative pathologic mechanism for renal injury in rare cases. CASE REPORT: We report a case of a patient with crescentic pauci-immune glomerulonephritis presenting with rapidly progressive renal failure, polyarthropathy, and a purpuric rash in association with a monoclonal immunoglobulin G κ-light-chain producing multiple myeloma...
2017: Clinical Nephrology. Case Studies
https://www.readbyqxmd.com/read/29042965/overexpression-of-let-7a-increases-neurotoxicity-in-a-pc12-cell-model-of-alzheimer-s-disease-via-regulating-autophagy
#15
Huizi Gu, Lan Li, Chen Cui, Zihui Zhao, Guijun Song
Increased deposition of β-amyloid (Aβ) protein is one of the typical characteristics of Alzheimer's disease (AD). Recent evidence has demonstrated that the microRNA let-7 family, which is highly expressed in the central nervous system, participates in the regulation of pathologic processes of AD. In the present study, the effect of let-7a overexpression on Aβ1-40-induced neurotoxicity was evaluated in PC12 and SK-N-SH cells. The results indicated that overexpression of let-7a enhanced the neurotoxicity induced by Aβ1-40 in PC12 and SK-N-SH cells...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29037943/mesenchymal-stromal-cells-protect-human-cardiomyocytes-from-amyloid-fibril-damage
#16
Yi Lin, Marta Marin-Argany, Christopher J Dick, Keely R Redhage, Luis M Blancas-Mejia, Peggy Bulur, Greg W Butler, Michael C Deeds, Benjamin J Madden, Angela Williams, Jonathan S Wall, Allan Dietz, Marina Ramirez-Alvarado
BACKGROUND AIMS: Light chain (AL) amyloidosis is a protein misfolding disease characterized by extracellular deposition of immunoglobulin light chains (LC) as amyloid fibrils. Patients with LC amyloid involvement of the heart have the worst morbidity and mortality. Current treatments target the plasma cells to reduce further production of amyloid proteins. There is dire need to understand the mechanisms of cardiac tissue damage from amyloid to develop novel therapies. We recently reported that LC soluble and fibrillar species cause apoptosis and inhibit cell growth in human cardiomyocytes...
October 13, 2017: Cytotherapy
https://www.readbyqxmd.com/read/29023182/potential-clinical-utility-of-multiple-system-atrophy-biomarkers
#17
Kurt A Jellinger
Multiple system atrophy (MSA), an adult-onset, fatal disorder of uncertain etiology, characterized by parkinsonism, cerebellar, autonomic and motor dysfunctions, is an α-synucleinopathy with glioneuronal degeneration involving multiple parts of the nervous system. The clinical variants correlate with the morphological phenotypes of striatonigral degeneration (MSA-P), olivoponto-cerebellar atrophy (MSA-C), and mixed type MSA. Neuropathological hallmark is the deposition of aberrant α-synuclein in glia and neurons forming cytoplasmic inclusions that cause cell dysfunction/demise...
October 23, 2017: Expert Review of Neurotherapeutics
https://www.readbyqxmd.com/read/28985862/autophagy-negatively-regulates-pro-osteogenic-activity-in-human-aortic-valve-interstitial-cells
#18
Xin-Sheng Deng, Xianzhong Meng, Neil Venardos, Rui Song, Katsuhiro Yamanaka, David Fullerton, James Jaggers
BACKGROUND: Autophagy is a physiological process that plays an important role in maintaining cellular functions. When aortic valve interstitial cells (AVICs) are stimulated with inflammatory or mechanical stress, one response is elevated pro-osteogenic activity. We hypothesized that autophagy is important in the prevention or regulation of this pro-osteogenic activity in AVICs. MATERIALS AND METHODS: AVICs were isolated. Autophagy activity was examined and its role in AVIC's pro-osteogenic activity was determined using chemical inhibitors and genetic techniques...
October 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28984299/medical-renal-diseases-are-frequent-but-often-unrecognized-in-adult-autopsies
#19
Marie E Perrone, Anthony Chang, Kammi J Henriksen
Kidney diseases affect many hospitalized patients and contribute to morbidity and mortality. Therefore, kidney disease should be prevalent, but the frequency and spectrum of medical renal pathology in autopsy specimens has not been well documented. We sought to determine the spectrum of medical renal pathology in adult autopsy specimens and the frequency of overlooked diagnoses. We reviewed the hematoxylin- and eosin-stained kidney sections from 140 adult autopsies performed at a large teaching hospital over a 2-year period...
October 6, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28975104/proliferative-glomerulonephritis-with-monoclonal-igg-deposits-an-unusual-cause-of-de-novo-disease-in-kidney-allograft
#20
Sabiha M Hussain, Kalathil K Sureshkumar
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a newly described and rare entity that can develop in native and very rarely in transplanted kidneys. We present a patient who developed de novo PGNMID in the kidney allograft along with a review of the literature. CASE PRESENTATION: A 38-year old female with type 1 diabetes who underwent successful simultaneous pancreas-kidney (SPK) transplantation 6 years earlier presented with rising serum creatinine, nephrotic range proteinuria and microhematuria...
July 2017: Journal of Nephropathology
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