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light chain deposition disease

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https://www.readbyqxmd.com/read/29773990/atorvastatin-inhibits-inflammatory-response-attenuates-lipid-deposition-and-improves-the-stability-of-vulnerable-atherosclerotic-plaques-by-modulating-autophagy
#1
Shi Peng, Long-Wei Xu, Xin-Yu Che, Qing-Qing Xiao, Jun Pu, Qin Shao, Ben He
Atherosclerosis is a chronic disease comprising intima malfunction and arterial inflammation. Recent studies have demonstrated that autophagy could inhibit inflammatory response in atherosclerosis and exert subsequent atheroprotective effects. Our previous study also demonstrated the role of autophagy in the inhibition of inflammation by atorvastatin in vitro . Therefore, in the present study, we aimed to determine whether atorvastatin could upregulate autophagy to inhibit inflammatory cytokines secretion, lipid accumulation, and improve vulnerable plaque stability, both in vitro and in vivo ...
2018: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/29757481/severe-white-matter-astrocytopathy-in-cadasil
#2
Yoshiki Hase, Aiqing Chen, Letitia L Bates, Lucinda J L Craggs, Yumi Yamamoto, Elizabeth Gemmell, Arthur E Oakley, Viktor I Korolchuk, Raj N Kalaria
Objectives Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterised by strategic white matter (WM) hyperintensities on MRI. Pathological features include WM degeneration, arteriolosclerosis, lacunar infarcts and the deposition of granular osmiophilic material. Based on the hypothesis that the gliovascular unit is compromised, we assessed the nature of astrocyte damage in the deep WM of CADASIL subjects. Methods We evaluated post-mortem brains from CADASIL, cerebral small vessel disease, similar age cognitively normal and older control subjects...
May 14, 2018: Brain Pathology
https://www.readbyqxmd.com/read/29748430/epidemiology-of-al-amyloidosis-a-real-world-study-using-us-claims-data
#3
Tiffany P Quock, Tingjian Yan, Eunice Chang, Spencer Guthrie, Michael S Broder
Amyloid light-chain (AL) amyloidosis is a rare disease caused by extracellular deposition of misfolded immunoglobulin light chains. This study aimed to provide an up-to-date estimate of prevalence and incidence of AL amyloidosis in the United States. Using claims databases from years 2007 to 2015, adults ≥18 years old with AL amyloidosis were included if they had (1) at least 1 inpatient or 2 outpatient claims consistent with AL amyloidosis and (2) received 1 AL-specific treatment. Prevalence was calculated as the number of AL patients divided by the number of enrollees on June 30th of each calendar year...
May 22, 2018: Blood Advances
https://www.readbyqxmd.com/read/29707360/wild-type-transthyretin-cardiac-amyloidosis-attrwt-ca-previously-known-as-senile-cardiac-amyloidosis-clinical-presentation-diagnosis-management-and-emerging-therapies
#4
REVIEW
Ilia G Halatchev, Jingsheng Zheng, Jiafu Ou
Cardiac amyloidosis is thought to be a rare group of diseases caused by extracellular deposition of misfolded proteins in the extracellular cardiac matrix resulting in heart failure with preserved ejection fraction (HFpEF). This review focuses on the similarities and differences between the pathophysiology, clinical presentation and diagnostic tests of wild-type transthyretin cardiac amyloidosis (ATTRwt-CA) compared to immunoglobulin light chain amyloidosis and hereditary cardiac amyloidosis. We address some obstacles to timely diagnosis and opportunities for management of the clinical symptoms as well as possibility of future novel disease modifying therapies...
March 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29703839/the-complexity-and-heterogeneity-of-monoclonal-immunoglobulin-associated-renal-diseases
#5
Sanjeev Sethi, S Vincent Rajkumar, Vivette D D'Agati
Monoclonal gammopathies are characterized by the overproduction of monoclonal Ig (MIg) detectable in the serum or urine resulting from a clonal proliferation of plasma cells or B lymphocytes. The underlying hematologic conditions range from malignant neoplasms of plasma cells or B lymphocytes, including multiple myeloma and B-cell lymphoproliferative disorders, to nonmalignant small clonal proliferations. The term MGUS implies presence of an MIg in the setting of a "benign" hematologic condition without renal or other end organ damage...
April 27, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29700090/cardiac-amyloidosis
#6
Ana Martinez-Naharro, Philip N Hawkins, Marianna Fontana
Systemic amyloidosis comprises an uncommon group of disorders caused by the extracellular deposition of misfolded proteins in various organs. Cardiac amyloid deposition, causing an infiltrative/restrictive cardiomyopathy, is a frequent feature of amyloidosis and a major determinant of survival. It may be the presenting feature of the disease or may be identified while investigating a patient presenting with other organ involvement. The need for a high index of suspicion and the critical importance of precise biochemical typing of the amyloid deposits is paramount in light of recent therapeutic advances that can significantly improve prognosis...
April 1, 2018: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/29696194/rationale-and-design-of-dual-study-doxycycline-to-upgrade-response-in-light-chain-al-amyloidosis-dual-a-phase-2-pilot-study-of-a-two-pronged-approach-of-prolonged-doxycycline-with-plasma-cell-directed-therapy-in-the-treatment-of-al-amyloidosis
#7
Anita D'Souza, Kathryn Flynn, Saurabh Chhabra, Binod Dhakal, Mehdi Hamadani, Kirsten Jacobsen, Marcelo Pasquini, Dorothee Weihrauch, Parameswaran Hari
Light chain (AL) amyloidosis is a plasma cell neoplasm associated with insoluble fibril deposition from clonal immunoglobulin chains systemically. The disease is associated with high early mortality and morbidity owing to advanced organ deposition as well as lack of proven de-fibrillogenic therapies. Pre-clinical and retrospective clinical data suggests that doxycycline has benefit in AL amyloidosis. The ongoing DUAL study is a single center, open label, phase 2 study in which patients with AL amyloidosis who are undergoing clone-directed therapy for the underlying neoplasm with oral doxycycline given for 1 year to test the hypothesis that prolonged doxycycline use will be safe, feasible, and lead to reduced early mortality in systemic AL amyloidosis and hasten organ amyloid response...
December 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/29672446/the-effects-of-treadmill-exercise-on-autophagy-in-hippocampus-of-app-ps1-transgenic-mice
#8
Na Zhao, Xianliang Zhang, Chenghui Song, Youcai Yang, Biao He, Bo Xu
The β-amyloid (Aβ) deposition is one of the major pathological hallmark of Alzheimer's disease. Dysfunction in autophagy has been reported to lead to the Aβ deposition. The current study aimed to investigate the effects of treadmill exercise on autophagy activity and the Aβ deposition and to demonstrate whether exercise-induced reduction in the Aβ deposition was associated with changes in autophagy activity. APP/PS1 transgenic mice were divided into transgenic sedentary (TG-SED, n=12) and transgenic exercise (TG-EXE, n=12) groups...
April 18, 2018: Neuroreport
https://www.readbyqxmd.com/read/29668344/phenotypic-plasticity-of-mesenchymal-stem-cells-is-crucial-for-mesangial-repair-in-a-model-of-immunoglobulin-light-chain-associated-mesangial-damage
#9
Guillermo A Herrera, Jiamin Teng, Chun Zeng, Hongzhi Xu, Man Liang, J Steven Alexander, Bing Liu, Chris Boyer, Elba A Turbat-Herrera
Mesangiopathies produced by glomerulopathic monoclonal immunoglobulin light chains (GLCs) acting on the glomerular mesangium produce two characteristic lesions: AL-amyloidosis (AL-Am) and light chain deposition disease (LCDD). In both cases, the pathology is centered in the mesangium, where initial and progressive damage occurs. In AL-Am the mesangial matrix is destroyed and replaced by amyloid fibrils and in LCDD, the mesangial matrix is increased and remodeled. The collagen IV rich matrix is replaced by tenascin...
April 18, 2018: Ultrastructural Pathology
https://www.readbyqxmd.com/read/29627865/advances-in-treatment-of-cardiac-amyloid
#10
REVIEW
Cherie N Dahm, R Frank Cornell, Daniel J Lenihan
Systemic amyloidosis represents a complex group of diseases with a common feature characterized by misfolded autologous proteins depositing into tissues or organs throughout the body. Light chain amyloidosis (AL) and transthyretin (TTR) amyloid are the two most prevalent forms of this disease that commonly results in cardiac amyloidosis. In both of these conditions, the myocardium is a frequent site of infiltration and end-organ involvement often with devastating consequences. With cardiac amyloidosis becoming an increasingly identified disease that has previously been under-recognized, the purpose of this comprehensive review is to focus on the diagnosis and treatment of these two types of cardiac amyloidosis including a contemporary update on currently available therapies being investigated in clinical trials...
April 7, 2018: Current Treatment Options in Cardiovascular Medicine
https://www.readbyqxmd.com/read/29622867/autologous-stem-cell-transplantation-for-multiple-myeloma-single-centre-experience-from-north-india
#11
Pankaj Malhotra, Uday Yanamandra, Alka Khadwal, Gaurav Prakash, Deepesh Lad, Arjun D Law, Harshit Khurana, M U S Sachdeva, Praveen Bose, Reena Das, Neelam Varma, Subhash Varma
Autologous stem cell transplantation (ASCT) is considered as standard of care in patients with multiple myeloma (MM) patients aged 65 years or younger. We analyzed data of 94 patients of plasma cell dyscrasias who underwent 95 autologous transplants at our institute from October 2003 to Aug 2016. Other than 76 patients of newly diagnosed multiple myeloma, we also transplanted two patients of POEMS syndrome, two patients of plasma cell leukemia, three patients of concurrent light chain deposition disease, three patients of multifocal plasmacytomas, and eight patients of isolated light chain myeloma...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29616329/proliferative-glomerulonephritis-with-monoclonal-igg-deposits-in-children-and-young-adults
#12
Guolan Xing, Robert Gillespie, Badreldin Bedri, Albert Quan, Pingchuan Zhang, Xin J Zhou
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age. METHODS: The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved...
April 3, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29589647/primary-systemic-amyloidosis-with-skin-and-cardiac-involvement-a-case-report
#13
Anja Trajber Horvat, Katarina Trčko, Vesna Jurčić, Pij Bogomir Marko
Primary systemic amyloidosis is characterized by the deposition of insoluble monoclonal immunoglobulin light chains in various tissues and is usually associated with an underlying plasma cell dyscrasia. In the early stage of the disease, dermatological findings can be the only manifestation, as opposed to organ involvement in the later stages. A dermatologist can diagnose amyloidosis early with a skin biopsy stained with Congo red dye and other appropriate investigations. This case report describes a female patient with primary systemic amyloidosis confirmed histologically from a skin biopsy...
March 2018: Acta Dermatovenerologica Alpina, Panonica, et Adriatica
https://www.readbyqxmd.com/read/29583159/-heart-transplantation-in-al-amyloidosis
#14
Pia Dahlberg, Sven-Erik Bartfay, Kristjan Karason, Ulf-Henrik Mellqvist, Entela Bollano
Amyloidosis is a disease complex characterized by the deposition of protein fibrils in various tissues, which leads to structural and functional derangement of the affected organ. There are different types of amyloidosis categorized on the basis of the type of protein fibrils deposited. Cardiac involvement has been predominantly noted in amyloid light chain (AL) amyloidosis and is the major prognostic determinant and influences the therapeutic strategy.  In AL amyloidosis, heart transplantation is generally not recommended because of a high risk of recurrence in the transplanted heart and poor survival rate...
March 26, 2018: Läkartidningen
https://www.readbyqxmd.com/read/29571269/a-functional-assay-to-identify-amyloidogenic-light-chains
#15
Emily B Martin, Angela D Williams, R Eric Heidel, James S Foster, Ronald H Lands, Stephen J Kennel, Jonathan S Wall
INTRODUCTION: Multiple myeloma (MM) and light chain monoclonal gammopathy of undetermined significance (LCMGUS) are plasma cell disorders associated with the secretion of monoclonal free light-chain (LC) proteins. Due to the high concentrations of LC in circulation, both of these populations are at risk for developing LC-associated amyloidosis (AL) - a protein misfolding disease characterized by the deposition of LC protein fibrils in organs and tissues, leading to dysfunction and significant morbidity...
March 23, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29551574/unilateral-localized-conjunctival-amyloidosis-in-a-patient-with-a-history-of-contralateral-orbit-eyelid-lymphoma
#16
Joshua T Byers, Xin Qing, Christopher Lo, Samuel W French, Ping Ji
Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy...
April 2018: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/29531659/light-chain-amyloidosis
#17
REVIEW
Paolo Milani, Giampaolo Merlini, Giovanni Palladini
Light chain (AL) amyloidosis is caused by a usually small plasma-cell clone that is able to produce the amyloidogenic light chains. They are able to misfold and aggregate, deposit in tissues in the form of amyloid fibrils and lead to irreversible organ dysfunction and eventually death if treatment is late or ineffective. Cardiac damage is the most important prognostic determinant. The risk of dialysis is predicted by the severity of renal involvement, defined by the baseline proteinuria and glomerular filtration rate, and by the response to therapy...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29520480/clinical-phenotyping-of-transthyretin-cardiac-amyloidosis-with-bone-seeking-radiotracers-in-heart-failure-with-preserved-ejection-fraction
#18
REVIEW
Wengen Chen, Van-Khue Ton, Vasken Dilsizian
PURPOSE OF REVIEW: The two most common types of cardiac amyloidosis are caused by fibril deposits of immunoglobulin light chains (AL) and transthyretin (TTR), each with distinct prognosis and clinical management. Cardiac amyloidosis is under-recognized among heart failure patients with preserved ejection fraction (HFpEF). Bone-seeking tracers like 99m Tc-PYP and 99m Tc-DPD have long been used to identify cardiac amyloidosis, and more recently, to differentiate TTR from AL cardiac amyloidosis in symptomatic patients...
March 8, 2018: Current Cardiology Reports
https://www.readbyqxmd.com/read/29507277/light-chain-deposition-disease-associated-with-multiple-myeloma-developing-in-late-pregnancy
#19
Min Jeong Kim, Joo Hui Kim, Il Young Kim, Soo Bong Lee, In Seong Park, Mi Yeun Han, Harin Rhee, Sang Heon Song, Eun Young Seong, Ihm Soo Kwak, Dong Won Lee
Preeclampsia is the most common cause of proteinuria with hypertension during pregnancy. Primary kidney disease and kidney disease secondary to systemic disorders may rarely occur during pregnancy, resulting in proteinuria. A 34-year-old woman was admitted to our hospital with abdominal distention and lower extremity edema. The pregnancy was terminated at the 24th week of gestation due to preterm labor. Even after the delivery, proteinuria and renal deterioration continued to progress. The M-peak was not found on serum and urine protein electrophoresis...
March 2018: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29441298/acquired-cutis-laxa-associated-with-light-and-heavy-chain-deposition-disease
#20
Reena A Majithia, Leni George, Meera Thomas, N A Fouzia
Acquired cutis laxa (ACL) is a rare connective tissue disorder characterized by pendulous and coarsely wrinkled skin. There have been few cases of its association to monoclonal immunoglobulin deposition disease (MIDD), which constitutes the light chain (LCDD), heavy chain (HCDD), and light and heavy chain (LHCDD) deposition disease. MIDD predominantly involves the kidney. Skin is the next common organ to be affected by HCDD, which presents as ACL. We report the case of a 40-year-old male who presented with ACL associated with LHCDD...
January 2018: Indian Dermatology Online Journal
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