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Light chain amyloidosis

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https://www.readbyqxmd.com/read/29142973/renal-amyloidosis-associated-with-5-novel%C3%A2-variants-in-the-fibrinogen-a-alpha-chain-protein
#1
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/29142197/proteotoxicity-in-cardiac-amyloidosis-amyloidogenic-light-chains-affect-the-levels-of-intracellular-proteins-in-human-heart-cells
#2
Esther Imperlini, Massimiliano Gnecchi, Paola Rognoni, Eduard Sabidò, Maria Chiara Ciuffreda, Giovanni Palladini, Guadalupe Espadas, Francesco Mattia Mancuso, Margherita Bozzola, Giuseppe Malpasso, Veronica Valentini, Giuseppina Palladini, Stefania Orrù, Giovanni Ferraro, Paolo Milani, Stefano Perlini, Francesco Salvatore, Giampaolo Merlini, Francesca Lavatelli
AL amyloidosis is characterized by widespread deposition of immunoglobulin light chains (LCs) as amyloid fibrils. Cardiac involvement is frequent and leads to life-threatening cardiomyopathy. Besides the tissue alteration caused by fibrils, clinical and experimental evidence indicates that cardiac damage is also caused by proteotoxicity of prefibrillar amyloidogenic species. As in other amyloidoses, the damage mechanisms at cellular level are complex and largely undefined. We have characterized the molecular changes in primary human cardiac fibroblasts (hCFs) exposed in vitro to soluble amyloidogenic cardiotoxic LCs from AL cardiomyopathy patients...
November 15, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29137218/importance-of-fish-genetics-in-light-chain-amyloidosis
#3
EDITORIAL
Morie A Gertz, Angela Dispenzieri, Eli Muchtar
No abstract text is available yet for this article.
October 10, 2017: Oncotarget
https://www.readbyqxmd.com/read/29136724/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-renal-impairment-of-multiple-myeloma
#4
(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/29135289/-what-should-we-know-about-cardiac-amyloidosis-from-clinical-signs-to-treatment
#5
Dóra Földeák, Attila Nemes, Anita Kalapos, Péter Domsik, Árpád Kormányos, László Krenács, Enikő Bagdi, Zita Borbényi
Systemic amyloidosis is a rare disease, in which the heart involvement is rather frequent and determines survival remarkably. Regarding the disease and organ involvement, new diagnostic procedures help to establish the diagnosis and to start the adequate treatment as soon as possible. Cardiac involvement is more likely to be characterised by monoclonal immunglobulin free light chain (AL amyloidosis) type and transthyretin type. In case of AL amyloidosis, heart involvement can lead to serious consequences. Biomarker assessments for cardiac function are important to determine disease severity at the beginning and to measure response to the treatment...
November 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/29133141/abdominal-fat-pad-excisional-biopsy-for-the-diagnosis-and-typing-of-systemic-amyloidosis
#6
Yessica Garcia, A Bernard Collins, James R Stone
In the past, the diagnosis and typing of amyloidosis often required an invasive biopsy of an internal organ, such as the heart or kidneys. Abdominal fat pad excisional biopsy (FPEB) offers a less invasive approach, but the sensitivity of this technique has been unclear. To determine the sensitivity of FPEB for immunoglobulin light chain (AL) and transthyretin (ATTR) amyloidosis, we performed a retrospective clinicopathologic analysis of 97 patients who had undergone FPEB, of which 16 were positive for amyloid...
November 10, 2017: Human Pathology
https://www.readbyqxmd.com/read/29130681/nodular-glomerulosclerosis-not-always-diabetic-nephropathy
#7
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/29121956/regional-differences-in-prognostic-value-of-cardiac-valve-plane-displacement-in-systemic-light-chain-amyloidosis
#8
Marco M Ochs, Thomas Fritz, Nisha Arenja, Johannes Riffel, Florian Andre, Derliz Mereles, Fabian Aus dem Siepen, Ute Hegenbart, Stefan Schönland, Hugo A Katus, Matthias G W Friedrich, Sebastian J Buss
BACKGROUND: To compare the prognostic value of cardiac valve plane displacement (CVPD) on various locations in cardiac light chain (AL) amyloidosis. METHODS: Consecutive patients with biopsy-proven cardiac involvement in AL amyloidosis who had undergone cardiovascular magnetic resonance (CMR) between 2005 and 2014 in our institution, were retrospectively identified and data analyzed. The primary combined endpoint was all-cause mortality or heart transplantation...
November 9, 2017: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/29112180/impaired-right-ventricle-function-as-predictor-of-early-mortality-in-patients-with-light-chain-cardiac-amyloidosis-assessed-in-the-cardiology-department
#9
Justyna Anna Szczygieł, Piotr Zbigniew Wieczorek, Joanna Drozd-Sokołowska, Piotr Michałek, Łukasz Mazurkiewicz, Marta Legatowicz-Koprowska, Ewa Walczak, Wiesław Wiktor Jędrzejczak, Jadwiga Dwilewicz-Trojaczek, Jacek Grzybowski
INTRODUCTION    Light-chain amyloidosis (AL amyloidosis) is the most common cardiac amyloidosis. Early mortality remains a substantial problem despite progress in treatment. OBJECTIVES    The aim of the study was to obtain a clinical profile of patients diagnosed with AL amyloidosis in the cardiology department, to define an early mortality cut-off point in this population, and to identify predictors of early mortality. PATIENTS AND METHODS    The analysis covered 30 patients (14 females, median age 61...
November 3, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29111123/prognostic-and-added-value-of-two-dimensional-global-longitudinal-strain-for-prediction-of-survival-in-patients-with-light-chain-amyloidosis-undergoing-autologous-hematopoietic-cell%C3%A2-transplantation
#10
Shawn C Pun, Heather J Landau, Elyn R Riedel, Jonathan Jordan, Anthony F Yu, Hani Hassoun, Carol L Chen, Richard M Steingart, Jennifer E Liu
BACKGROUND: Autologous hematopoietic cell transplantation (HCT) is a first-line therapy for prolonging survival in patients with light-chain (AL) amyloidosis. Cardiac involvement is the most important determinant of survival. However, patients with advanced cardiac involvement have often been excluded from HCT because of high risk for transplantation-related mortality and poor overall survival. Whether baseline left ventricular global longitudinal strain (GLS) can provide additional risk stratification and predict survival after HCT in this high-risk population remains unclear...
October 27, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29111120/abnormal-coronary-flow-velocity-reserve-and-decreased-myocardial-contractile-reserve-are-main-factors-in-relation-to-physical-exercise-capacity-in-cardiac-amyloidosis
#11
Tor Skibsted Clemmensen, Hans Eiskjær, Henning Mølgaard, Anders Hostrup Larsen, Jens Soerensen, Niels Frost Andersen, Lars Poulsen Tolbod, Hendrik J Harms, Steen Hvitfeldt Poulsen
BACKGROUND: The aim of the present study was to evaluate the clinical importance of echocardiographic coronary flow velocity reserve (CFVR), resting and exercise left ventricular global longitudinal strain (LVGLS), and myocardial work efficiency (MWE) in patients with cardiac amyloidosis (CA). METHODS: The study population comprised 69 subjects: group A, 27 patients with CA confirmed by endomyocardial biopsy (CA positive); group B, 42 healthy control subjects. The amyloid phenotype in group A was as follows: patients with wild-type transthyretin-related amyloidosis (n = 10), carriers of the Danish familial transthyretin amyloidosis mutation with cardiac involvement (n = 5), and patients with amyloid light chain amyloidosis with cardiac involvement (n = 12)...
October 27, 2017: Journal of the American Society of Echocardiography
https://www.readbyqxmd.com/read/29101236/presentation-and-outcome-with-second-line-treatment-in-al-amyloidosis-previously-sensitive-to-non-transplant-therapies
#12
Giovanni Palladini, Paolo Milani, Andrea Foli, Marco Basset, Francesca Russo, Stefano Perlini, Giampaolo Merlini
The management of light chain (AL) amyloidosis has improved in recent years thanks to accurate biomarker-based staging systems and response criteria and availability of novel effective therapies. However, previous studies have focused on newly diagnosed patients, and little is known on relapsed patients, despite trials of new agents are often performed in this setting. In the present study we report the outcome of 259 patients who responded to upfront therapy. Ninety-two patients (35%) needed second-line therapy after a median of 49 months...
November 3, 2017: Blood
https://www.readbyqxmd.com/read/29093431/increased-prognostic-value-of-query-amyloid-late-enhancement-score-in-light-chain-cardiac-amyloidosis
#13
Ke Wan, Jiayu Sun, Yuchi Han, Hong Liu, Dan Yang, Weihao Li, Jie Wang, Wei Cheng, Qing Zhang, Zhi Zeng, Yucheng Chen
BACKGROUND: Late gadolinium enhancement (LGE) pattern is a powerful imaging biomarker for prognosis of cardiac amyloidosis. It is unknown if the query amyloid late enhancement (QALE) score in light-chain (AL) amyloidosis could provide increased prognostic value compared with LGE pattern.Methods and Results:Seventy-eight consecutive patients with AL amyloidosis underwent contrast-enhanced cardiovascular magnetic resonance imaging. Patients with cardiac involvement were grouped by LGE pattern and analyzed using QALE score...
November 2, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/29093404/nationwide-survey-of-741-patients-with-systemic-amyloid-light-chain-amyloidosis-in-japan
#14
Chihiro Shimazaki, Hiroyuki Hata, Sinsuke Iida, Mitsuharu Ueda, Nagaaki Katoh, Yoshiki Sekishima, Shuichi Ikeda, Masahide Yazaki, Wakaba Fukushima, Yukio Ando
Objective To retrospectively investigate the clinical manifestations of systemic amyloid light-chain (AL) amyloidosis in Japanese patients and the treatment strategy for the condition. Methods We conducted a survey of Japanese AL patients, who were treated between January 1, 2012, and December 31, 2014. Results A total of 741 AL amyloidosis patients were included in this study (436 men and 305 women; median age: 65 years old, range: 31-93). The most frequently affected organ was the kidneys (n=542), followed by the heart (n=252), gastrointestinal (GI) tract (n=164), autonomic nervous system (n=131), liver (n=71), and peripheral nervous system (n=71)...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29068915/ocular-manifestations-of-systemic-amyloidosis
#15
Margaret M Reynolds, Kevin K Veverka, Morie A Gertz, Angela Dispenzieri, Steven R Zeldenrust, Nelson Leung, Jose S Pulido
PURPOSE: To describe ophthalmic manifestations of systemic amyloidosis, a group of devastating conditions. METHODS: A retrospective chart review including patients who had ocular examinations at Mayo Clinic between January 1, 1985, and April 1, 2014, and a diagnosis of light-chain (AL), secondary (AA), or nontransthyretin familial amyloidosis was undertaken. Sixty-eight patients with AL amyloidosis, eight patients with AA amyloidosis, and five patients with nontransthyretin familial amyloidosis were included...
October 24, 2017: Retina
https://www.readbyqxmd.com/read/29067795/intramyocardial-inflammation-predicts-adverse-outcome-in-patients-with-cardiac-al-amyloidosis
#16
Christine S Siegismund, Felicitas Escher, Dirk Lassner, Uwe Kühl, Ulrich Gross, Friedrich Fruhwald, Philip Wenzel, Thomas Münzel, Norbert Frey, Reinhold P Linke, Heinz-Peter Schultheiss
AIMS: To evaluate the influence of endomyocardial biopsy (EMB)-proven intramyocardial inflammation on mortality in patients with cardiac transthyretin amyloid (ATTR) or amyloid light-chain (AL) amyloidosis. METHODS AND RESULTS: We included 54 consecutive patients (mean age 68.83 ± 9.59 years; 45 men) with EMB-proven cardiac amyloidosis. We followed up patients from first diagnostic biopsy to as long as 36 months (mean 11.5 ± 12 months) and compared their outcome with information on all-cause mortality with or without proof of inflammation on EMB...
October 25, 2017: European Journal of Heart Failure
https://www.readbyqxmd.com/read/29065679/localized-gastric-amyloidosis-with-kappa-and-lambda-light-chain-co-expression
#17
Yong Hwan Ahn, Ye Young Rhee, Suck Chei Choi, Geom Seog Seo
Esophagogastroduodenoscopy for cancer screening was performed in a 55-year-old woman as part of a health screening program, and revealed a depressed lesion approximately 20 mm in diameter in the lesser curvature of the mid-gastric body. Several biopsy specimens were collected as the lesion resembled early gastric cancer; however, histopathologic evaluation revealed chronic active gastritis with an ulcer and amorphous eosinophilic material deposition. Congo red staining identified amyloid proteins, and apple-green birefringence was shown using polarized light microscopy...
October 26, 2017: Clinical Endoscopy
https://www.readbyqxmd.com/read/29053157/current-applications-of-multiparameter-flow-cytometry-in-plasma-cell-disorders
#18
REVIEW
T Jelinek, R Bezdekova, M Zatopkova, L Burgos, M Simicek, T Sevcikova, B Paiva, R Hajek
Multiparameter flow cytometry (MFC) has become standard in the management of patients with plasma cell (PC) dyscrasias, and could be considered mandatory in specific areas of routine clinical practice. It plays a significant role during the differential diagnostic work-up because of its fast and conclusive readout of PC clonality, and simultaneously provides prognostic information in most monoclonal gammopathies. Recent advances in the treatment and outcomes of multiple myeloma led to the implementation of new response criteria, including minimal residual disease (MRD) status as one of the most relevant clinical endpoints with the potential to act as surrogate for survival...
October 20, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/29052601/intratubular-amyloid-in-light-chain-cast-nephropathy-is-a-risk-factor-for-systemic-light-chain-amyloidosis
#19
Jean-Baptiste Gibier, Viviane Gnemmi, François Glowacki, Eileen M Boyle, Benjamin Lopez, Evelyne MacNamara, Maxime Hoffmann, Raymond Azar, Thomas Guincestre, Franck Bourdon, Marie-Christine Copin, David Buob
Light chain cast nephropathy is the most common form of kidney disease in patients with multiple myeloma. Light chain casts may occasionally show amyloid staining properties, that is, green birefringence after Congo red staining. The frequency and clinical significance of this intratubular amyloid are poorly understood. Here, we retrospectively assessed the clinicopathological features of 60 patients with histologically proven light chain cast nephropathy with a specific emphasis on intratubular amyloid, especially, its association with extrarenal systemic light chain amyloidosis...
October 20, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/29052436/prognostic-impact-of-immunoparesis-at-diagnosis-and-after-treatment-onset-in-patients-with-light-chain-amyloidosis
#20
Luis Gerardo Rodríguez-Lobato, Carlos Fernández de Larrea, Maria Teresa Cibeira, Natalia Tovar, Ignacio Isola, Juan I Aróstegui, Laura Rosiñol, Tania Díaz, Ester Lozano, Jordi Yagüe, Joan Bladé
OBJECTIVES: Immunoparesis (IP) is a risk factor associated with an unfavourable outcome in several plasma cell disorders. It has been suggested that its presence in light-chain (AL) amyloidosis could be associated with worse prognosis. However, the relevance of IP after treatment has not been evaluated to date. The aim of this study was to determine the prognostic impact of IP at diagnosis and one year after treatment onset in patients with AL amyloidosis. METHODS: The clinical records of 69 patients with AL amyloidosis treated at a single institution from January 2006 to January 2016 were included in the study...
October 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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