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light chain deposition disease

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https://www.readbyqxmd.com/read/29155738/imaging-amyloid-tissues-stained-with-luminescent-conjugated-oligothiophenes-by-hyperspectral-confocal-microscopy-and-fluorescence-lifetime-imaging
#1
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
#2
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
#3
(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
#4
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
#5
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
#6
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: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29042965/overexpression-of-let-7a-increases-neurotoxicity-in-a-pc12-cell-model-of-alzheimer-s-disease-via-regulating-autophagy
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28954985/a-novel-method-of-dapi-staining-for-differential-diagnosis-of-renal-amyloidosis
#13
Motokazu Matsuura, Hideharu Abe, Tatsuya Tominaga, Akiko Sakurai, Taichi Murakami, Seiji Kishi, Yoshimi Bando, Jun Minakuchi, Kojiro Nagai, Toshio Doi
Amyloidosis is often overlooked because its clinical manifestations can mimic those of more-common diseases. It is important to get a precise diagnosis as early as possible for the prevention of further organ damages. Amyloidosis is a disorder caused by deposition of insoluble abnormal amyloid. The kidney is a frequent site of amyloid deposition. The amyloid fibrils have a characteristic appearance and generate birefringence under polarized light when stained with the Congo red dye. Classification of amyloidosis is based on the precursor protein that forms the amyloid fibrils and the distribution of amyloid deposits as either systemic or localized...
2017: Journal of Medical Investigation: JMI
https://www.readbyqxmd.com/read/28953013/cutaneous-light-chain-deposition-disease-a-report-of-2-cases-and-review-of-the-literature
#14
Carlo Hendricks, Maite T Fernández Figueras, Julia Liersch, Maria-Teresa Martin-Urdà, Dolores López, Christoph Brochhausen, Christoph Röcken, Jörg Schaller
Light chain deposition disease (LCDD) is a rare systemic disorder with deposition of mostly monoclonal amorphous nonamyloid light chains in multiple organs. Renal involvement with rapidly progressing renal failure presents the dominant manifestation of LCDD. Approximately 20%-30% of patients show symptomatic cardiac or liver involvement. Cutaneous manifestations are extremely rare with only a few published cases. We report 2 additional cases of cutaneous LCDD without detectable systemic disease.
September 20, 2017: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/28946960/monoclonal-gammopathies-of-renal-significance
#15
Fernando Caravaca-Fontán, Eduardo Gutiérrez, Ramón Delgado Lillo, Manuel Praga
The term monoclonal gammopathy of renal significance (MGRS) comprises a group of diseases pathogenetically characterised by proliferation of a B-cell or plasma cell clone that synthesises and secretes a monoclonal immunoglobulin or its components (light and/or heavy chains), that may deposit and cause glomerular, tubular, interstitial and/or vascular damage. The importance of differentiating the term MGRS from other monoclonal gammopathies lies in the fact that diagnostic and therapeutic procedures aimed at controlling monoclonal protein synthesis and secretion can be indicated, irrespective of the classic criteria based on malignant tumour expansion...
September 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28926601/utility-of-abdominal-skin-plus-subcutaneous-fat-and-rectal-mucosal-biopsy-in-the-diagnosis-of-al-amyloidosis-with-renal-involvement
#16
Ting Li, Xianghua Huang, Shuiqin Cheng, Liang Zhao, Guisheng Ren, Wencui Chen, Qingwen Wang, Caihong Zeng, Zhihong Liu
OBJECTIVES: Skin fat biopsy of the abdominal wall is a simple and safe method for detecting amyloidosis, and rectal mucosal biopsy is also frequently used for screening for the disease; however, the sensitivity of these approaches has not been fully studied. The aim of this study was to evaluate the efficacy of skin fat biopsy combined with rectal mucosal biopsy as a screening procedure for the diagnosis of systemic immunoglobulin light-chain (AL) amyloidosis. METHODS: We retrospectively analyzed 224 AL amyloidosis patients confirmed by renal biopsy, including a test group of 165 patients and validation group of 59 patients...
2017: PloS One
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#17
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28903572/biomarkers-in-immunoglobulin-light-chain-amyloidosis
#18
Z Kufová, T Sevcikova, K Growkova, P Vojta, J Filipová, Z Adam, L Pour, M Penka, R Rysava, P Němec, L Brozova, P Vychytilova, A Jurczyszyn, S Grosicki, A Barchnicka, M Hajdúch, M Simicek, R Hájek
Immunoglobulin light chain amyloidosis (AL amyloidosis - ALA) is a monoclonal gammopathy characterized by presence of aberrant plasma cells producing amyloidogenic immunoglobulin light chains. This leads to formation of amyloid fibrils in various organs and tissues, mainly in heart and kidney, and causes their dysfunction. As amyloid depositing in target organs is irreversible, there is a big effort to identify biomarker that could help to distinguish ALA from other monoclonal gammopathies in the early stages of disease, when amyloid deposits are not fatal yet...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28875827/multiple-myeloma-associated-intestinal-amyloidosis-intestinal-pseudo-obstruction-falsely-considered-as-an-ascites
#19
Vladimir Milivojevic, Maja Stojanovic, Ivan Rankovic, Marjan Micev, Rada Jesic Vukicevic, Tomica Milosavljevic
INTRODUCTION: Amyloidosis is a group of diseases pathohistologicaly diagnosed by characteristic extracellular deposition of an abnormal fibrillary protein (i.e. amyloid) into organs, leading to organ dysfunction secondary to destruction of normal tissue architecture. METHOD: Case-report of a 44 year-old female, presenting with massive abdominal distension clinically suspected of ascites. RESULTS: On admission, the patient was complaining of nausea, vomiting, abdominal pain, distension and bloating associated with weight loss and diarrhea...
August 29, 2017: Reviews on Recent Clinical Trials
https://www.readbyqxmd.com/read/28865972/high-dose-melphalan-and-stem-cell-transplantation-in-patients-on-dialysis-due-to-immunoglobulin-light-chain-amyloidosis-and-monoclonal-immunoglobulin-deposition-disease
#20
Felipe Batalini, Laura Econimo, Karen Quillen, J Mark Sloan, Shayna Sarosiek, Dina Brauneis, Andrea Havasi, Lauren Stern, Laura Dember, Vaishali Sanchorawala
The kidney is the most common organ affected by immunoglobulin light-chain (AL) amyloidosis and monoclonal immunoglobulin deposition disease (MIDD), often leading to end-stage renal disease (ESRD). High-dose melphalan and stem cell transplantation (HDM/SCT) is effective for selected patients with AL amyloidosis, with high rates of complete hematologic response and potential for improved organ dysfunction. Data on tolerability and response to HDM/SCT in patients with ESRD due to AL amyloidosis and MIDD are limited...
September 1, 2017: Biology of Blood and Marrow Transplantation
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