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Al Amyloidosis

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https://www.readbyqxmd.com/read/29142197/proteotoxicity-in-cardiac-amyloidosis-amyloidogenic-light-chains-affect-the-levels-of-intracellular-proteins-in-human-heart-cells
#1
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/29136724/-expert-consensus-for-the-diagnosis-and-treatment-of-patients-with-renal-impairment-of-multiple-myeloma
#2
(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
#3
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
#4
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/29121956/regional-differences-in-prognostic-value-of-cardiac-valve-plane-displacement-in-systemic-light-chain-amyloidosis
#5
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
#6
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
#7
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/29107944/correction-utility-of-abdominal-skin-plus-subcutaneous-fat-and-rectal-mucosal-biopsy-in-the-diagnosis-of-al-amyloidosis-with-renal-involvement
#8
(no author information available yet)
[This corrects the article DOI: 10.1371/journal.pone.0185078.].
2017: PloS One
https://www.readbyqxmd.com/read/29101236/presentation-and-outcome-with-second-line-treatment-in-al-amyloidosis-previously-sensitive-to-non-transplant-therapies
#9
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
#10
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/29093408/the-long-term-outcomes-after-vad-plus-sct-therapy-in-a-patient-with-al-amyloidosis-and-severe-factor-x-deficiency
#11
Dosuke Iwadate, Eiko Hasegawa, Junichi Hoshino, Noriko Hayami, Keiichi Sumida, Masayuki Yamanouchi, Akinari Sekine, Masahiro Kawada, Rikako Hiramatsu, Tstsuya Suwabe, Naoki Sawa, Mitsuhiro Yuasa, Atsushi Waki, Takeshi Fujii, Kenichi Ohashi, Kenmei Takaichi, Yoshifumi Ubara
A 55-year-old man was admitted to our institute to undergo evaluation for proteinuria (5.4 g/day) with lambda-type Bence-Jones protein (BJP). Primary AL amyloidosis and acquired factor X deficiency were diagnosed. High-dose melphalan combined with autologous stem cell transplantation was performed. After three years, the patient's proteinuria normalized, he was negative for urinary BJP, and his factor X activity improved to 105%. Serial renal biopsy showed no progression of amyloid deposition at a biopsy after 5 years, but showed a slight increase in the amyloid deposition after 11 years...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29093404/nationwide-survey-of-741-patients-with-systemic-amyloid-light-chain-amyloidosis-in-japan
#12
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/29093392/atraumatic-splenic-rupture-due-to-ectopic-extramedullary-hematopoiesis-after-autologous-stem-cell-transplantation-in-a-patient-with-al-amyloidosis
#13
Shuku Sato, Yotaro Tamai, Satomi Okada, Emiko Kannbe, Kotaro Takeda, Eri Tanaka
A 50-year-old man was diagnosed with multiple myeloma complicating AL amyloidosis. Splenic rupture was complicated during autologous stem cell transplantation (auto-SCT). Granulocyte colony-stimulating factor (G-CSF) was not administered. A pathological examination of the spleen revealed that CD34-positive cells were concentrated in the ruptured part of the splenic capsule. Hematopoietic cells were engrafted in the small gap between the capsule and amyloid protein deposition area of the spleen, which might have caused the splenic rupture in the absence of G-CSF administration...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29068915/ocular-manifestations-of-systemic-amyloidosis
#14
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
#15
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/29064094/cardiac-amyloidosis-is-associated-with-increased-aortic-stiffness
#16
Attila Nemes, Dóra Földeák, Péter Domsik, Anita Kalapos, Árpád Kormányos, Zita Borbényi, Tamás Forster
OBJECTIVE: Cardiac amyloidosis (CA) is as an infiltrative disorder primarily caused by extracellular tissue deposition of amyloid fibrils in the myocardial interstitium. The current study was designed to test whether alterations in ascending aortic elastic properties could be detected by echocardiography in CA patients, and to compare their results to controls. PATIENTS AND METHODS: We included 19 CA patients from which CA proved to be AL amyloidosis in 17 cases and transthyretin (TTR) amyloidosis in 2 cases...
October 24, 2017: Journal of Clinical Ultrasound: JCU
https://www.readbyqxmd.com/read/29052436/prognostic-impact-of-immunoparesis-at-diagnosis-and-after-treatment-onset-in-patients-with-light-chain-amyloidosis
#17
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
https://www.readbyqxmd.com/read/29046749/an-unusual-case-of-chronic-lymphocytic-leukemia-multiple-myeloma-and-cardiac-amyloidosis
#18
Dongyan Liu, Hakim T Uqdah, Alisha D Gordy
Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted multiple myeloma with plasma cells causing AL amyloidosis involving the heart. While monoclonal immunoglobulins occasionallyproduced by CLL have previously been implicated in AL amyloidosis, there only a few cases reported of AL amyloidosis resulting from a distinct plasma cell dyscrasia that is not clonally related to the concurrent CLL...
October 2017: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/29037943/mesenchymal-stromal-cells-protect-human-cardiomyocytes-from-amyloid-fibril-damage
#19
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/29021469/elevation-of-plasmin-%C3%AE-2-plasmin-inhibitor-complex-predicts-the-diagnosis-of-systemic-al-amyloidosis-in-patients-with-monoclonal-protein
#20
Kazuya Ishiguro, Toshiaki Hayashi, Yoshihiro Yokoyama, Yuka Aoki, Kei Onodera, Hiroshi Ikeda, Tadao Ishida, Hiroshi Nakase
Objective The complication of systemic immunoglobulin light chain (AL) amyloidosis in patients with monoclonal immunoglobulin affects the prognosis, but amyloid deposition in tissues is sometimes difficult to detect due to bleeding tendencies and preferential distributions. However, fibrinolysis is known to be exacerbated in patients with systemic AL amyloidosis specifically. We therefore explored new biomarkers for predicting a diagnosis of systemic AL amyloidosis focusing on coagulation and fibrinolysis markers...
October 11, 2017: Internal Medicine
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