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systemic amyloidosis

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https://www.readbyqxmd.com/read/28340285/the-six-minute-walk-test-in-patients-with-al-amyloidosis-a-single-centre-case-series
#1
Vina Pulido, Gheorghe Doros, John L Berk, Vaishali Sanchorawala
The six-minute walk test (6MWT) has been widely used as an objective evaluation of functional exercise capacity and response to medical intervention in cardiopulmonary diseases. However, little is known about the 6MWT in evaluating patients with AL amyloidosis. We performed a retrospective study of 120 adults with systemic AL amyloidosis (60 with cardiac involvement and 60 without cardiac involvement) who had their initial evaluation at the Amyloidosis Center between 2013 and 2015 and had undergone 6MWT as a measure of functional exercise capacity...
March 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28336182/light-chain-amyloidosis-where-are-the-light-chains-from-and-how-they-play-their-pathogenic-role
#2
REVIEW
Chunlan Zhang, Xufei Huang, Jian Li
Amyloid light-chain (AL) amyloidosis is a plasma-cell dyscrasia, as well as the most common type of systematic amyloidosis. Pathogenic plasma cells that have distinct cytogenetic and molecular properties secrete an excess amount of amyloidogenic light chains. Assisted by post-translational modifications, matrix components, and other environmental factors, these light chains undergo a conformational change that triggers the formation of amyloid fibrils that overrides the extracellular protein quality control system...
March 8, 2017: Blood Reviews
https://www.readbyqxmd.com/read/28326465/pet-ct-evaluation-of-amyloid-systemic-involvement-with-18-f-florbetaben-in-patient-with-proved-cardiac-amyloidosis-a-case-report
#3
Dario Genovesi, Giuseppe Vergaro, Michele Emdin, Assuero Giorgetti, Paolo Marzullo
BACKGROUND: One of the most frequent disorders which lead to cardiac amyloidosis is transthyretin-related amyloidosis (ATTR). Some PET radio-pharmaceuticals for the detection of beta-amyloid deposits within the brain have shown to be able to detect also cardiac amyloid deposits. We present a case of a man with ATTR studied with [(18)F]-florbetaben PET-CT. RESULTS: Total-body scan showed a moderate uptake in the bone marrow, especially in correspondence of the vertebral column, while no significant myocardial uptake was present...
March 21, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28326212/a-case-of-conjunctival-amyloidosis-with-repeated-subconjunctival-hemorrhage
#4
Takaaki Ando, Toshiyuki Oshitari, Mamiko Saito, Ayako Tawada, Takayuki Baba, Jiro Yotsukura, Shuichi Yamamoto
Conjunctival amyloidosis is a very rare disease, and its presence may be a sign of systemic amyloidosis. We present our ocular and systemic findings in a patient with conjunctival amyloidosis. A 43-year-old man had repeated subconjunctival hemorrhages (SCHs) for two years and was referred to the Chiba University Hospital. He had comprehensive ophthalmological and systemic examinations to determine the cause of the SCHs. His visual acuities were 1.2 OU, and the intraocular pressures were 13-14 mmHg OU. Magnetic resonance imaging was normal...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28321082/systemic-al-amyloidosis-with-colonic-submucosal-hematoma
#5
Sho Sasaki, Jun Nishikawa, Shinichi Hashimoto, Isao Sakaida
No abstract text is available yet for this article.
2017: Internal Medicine
https://www.readbyqxmd.com/read/28320296/biological-basis-for-amyloidogenesis-in-alzheimer-s-disease
#6
REVIEW
T V Andreeva, W J Lukiw, E I Rogaev
Certain cellular proteins normally soluble in the living organism under certain conditions form aggregates with a specific cross-β sheet structure called amyloid. These intra- or extracellular insoluble aggregates (fibers or plaques) are hallmarks of many neurodegenerative pathologies including Alzheimer's disease (AD), Huntington's disease, Parkinson's disease, prion disease, and other progressive neurological diseases that develop in the aging human central nervous system. Amyloid diseases (amyloidoses) are widespread in the elderly human population, a rapidly expanding demographic in many global populations...
February 2017: Biochemistry. Biokhimii︠a︡
https://www.readbyqxmd.com/read/28314084/treatment-patterns-and-outcome-following-initial-relapse-or-refractory-disease-in-patients-with-systemic-light-chain-amyloidosis
#7
Nidhi Tandon, Surbhi Sidana, Morie A Gertz, Angela Dispenzieri, Martha Q Lacy, Francis K Buadi, David Dingli, Amie L Fonder, Miriam A Hobbs, Suzanne R Hayman, Wilson I Gonsalves, Yi Lisa Hwa, Prashant Kapoor, Robert A Kyle, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, S Vincent Rajkumar, Shaji K Kumar
We analyzed the outcomes following initial relapse or refractory disease in systemic light chain amyloidosis (AL) and the impact of type of therapy employed.A total of 1327 patients with AL seen at Mayo Clinic within 90 days of diagnosis, between 2006 and 2015, were reviewed. The study included 366 patients experiencing a documented hematological or organ relapse or refractory disease requiring start of second line therapy. Overall survival (OS) and time to next treatment (TTNT) were calculated from start of second line treatment...
March 17, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28298791/amyloidosis-and-anesthesia
#8
Zara Wani, Dev Kumar Harkawat, Meenaxi Sharma
AIM: The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition. MATERIAL AND METHOD: Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste. RESULTS: Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e...
January 2017: Anesthesia, Essays and Researches
https://www.readbyqxmd.com/read/28290973/total-artificial-heart-implantation-as-a-bridge-to-heart-transplantation-in-an-active-duty-service-member-with-amyloid-cardiomyopathy
#9
Michael S Scully, Dylan E Wessman, James M McKee, Gregory M Francisco, Keshav R Nayak, Jon A Kobashigawa
INTRODUCTION: Cardiac involvement by light-chain (AL) amyloid occurs in up to 50% of patients with primary AL amyloidosis. The prognosis of amyloid heart disease is poor with 1-year survival rates of 35 to 40%. Historically, heart transplantation was considered controversial for patients with AL amyloid cardiomyopathy (CM) given the systemic nature of the disease and poor survival. We present a case report of an active duty service member diagnosed with advanced cardiac amyloid who underwent total artificial heart transplant as a bridge to heart transplant and eventual autologous stem cell transplant...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28286797/malabsorption-secondary-to-gout-induced-amyloidosis
#10
Ali Azzam, Renuka Balasubramaniam, Shahram Safa, Carolyn McIvor, Peter Mollee
Many chronic inflammatory conditions can lead to systemic amyloidosis. However, secondary amyloidosis has rarely been associated with gout, and the literature reports only a handful of cases, all presenting with renal disease. We report a patient with a history of poorly controlled gout who presented with malabsorption. Endoscopic biopsies confirmed a diagnosis of small intestinal amyloidosis. This was believed to be a consequence of gout. Interestingly, renal involvement was subclinical. Our case raises awareness of this rare association and highlights the importance of considering a diagnosis of amyloidosis in patients who present with the combination of gout and gastrointestinal symptoms...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28286788/primary-hepatic-amyloidosis-presenting-as-acute-on-chronic-liver-failure
#11
Madhumita Premkumar, Devaraja Rangegowda, Tanmay Vyas, Anand Kulkarni, Shrruti Grover, Rakhi Mahiwall, Shiv Kumar Sarin
Systemic amyloidosis of amyloid light chain associated protein (AL), also called primary amyloidosis, frequently involves the liver, but rarely causes clinically apparent liver disease. The more common presentation is with acute renal failure. Hepatomegaly and mild elevation of alkaline phosphatase are the most common clinical and biochemical findings, respectively. We report a case of systemic amyloidosis of AL that clinically presented as acute-on-chronic liver failure and resulted in a fatal clinical course in a 56-year-old man...
2017: ACG Case Reports Journal
https://www.readbyqxmd.com/read/28283460/anti-parkinsonian-l-dopa-can-also-act-as-anti-systemic-amyloidosis-a-mechanistic-exploration
#12
Saima Nusrat, Nida Zaidi, Mohammad Khursheed Siddiqi, Masihuz Zaman, Ibrar Ahmed Siddique, Mohammad Rehan Ajmal, Ali Saber Abdelhameed, Rizwan Hasan Khan
In spite of the fact that amyloid related neurodegenerative illnesses and non-neuropathic systemic amyloidosis have allured the research endeavors, as no cure has been announced yet apart from symptomatic treatment. Therapeutic agents which can reduce or disaggregate those toxic oligomers and fibrillar species have been studied with more compounds are on their way. The current research work describes comprehensive biophysical, computational and microscopic studies which reveal that L-3, 4-dihydroxyphenylalanine (L-Dopa) have indisputable efficacy to hinder the heat induced amyloid fibrillation of the human lysozyme (HL) and also preserve the fibril disaggregating potential...
March 7, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28282682/-angioneurotic-edema-as-a-differential-diagnosis-of%C3%A2-recurrent-abdominal-pain
#13
Claudia Schadow, Cora Wex, Christian Wybranski, Thomas Kalinski, Christian Schulz, Frank Meyer
History and admission findings A 46-year-old woman was admitted because of recurrent postprandial vomiting including weight loss of 30 kg within the last 5 - 6 months. The physical investigation was indicative of preileus. Investigations CT scan revealed an unclear thickening of the jejunum. Treatment and course Explorative laparotomy was performed because of the considerable degree of suffering and a tendency of worsening. Intraoperatively, the preoperatively observed thickening was confirmed at 3 subsequent segments of the jejunum (3 cm long each) prompting to segmental resection of the jejunum...
March 2017: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/28279425/current-concepts-of-cardiac-amyloidosis-diagnosis-clinical-management-and-the-need-for-collaboration
#14
REVIEW
Alexandra J Ritts, Robert F Cornell, Kris Swiger, Jai Singh, Stacey Goodman, Daniel J Lenihan
Cardiac amyloidosis is a complex and vexing clinical condition that requires a high degree of suspicion for the diagnosis with a substantial amount of discipline to discern the extent of disease and the best available therapy. There is a complex interplay between multiple organ systems, and the clinical presentation may involve a myriad of confusing clinical symptoms. The diagnosis of cardiac amyloidosis can be confirmed with a combination of physical findings, cardiac biomarkers, noninvasive testing, and, if necessary, myocardial biopsy...
April 2017: Heart Failure Clinics
https://www.readbyqxmd.com/read/28277869/disease-burden-of-systemic-light-chain-al-amyloidosis-a-systematic-literature-review
#15
Huamao Mark Lin, Xin Gao, Catherine E Cooke, Deborah Berg, Richard Labotka, Douglas V Faller, Brian Seal, Parameswaran Hari
A systematic literature review on AL amyloidosis was conducted in order to understand the disease burden, and identify unmet medical needs and knowledge gaps. MEDLINE, EMBASE and COCHRANE databases were searched for English language studies published in the last 10 years using search terms that focused on the clinical, economic, and patient-reported outcome (PRO) aspects of AL amyloidosis. There was a low yield of articles in the economic and PRO categories and additional searches were conducted in clinical conference proceedings, and using Google and Google Scholar...
February 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28271734/overuse-of-organ-biopsies-in-immunoglobulin-light-chain-al-amyloidosis-the-consequence-of-failure-of-early-recognition
#16
Eli Muchtar, Angela Dispenzieri, Martha Q Lacy, Francis K Buadi, Prashant Kapoor, Suzanne R Hayman, Wilson Gonsalves, Rahma Warsame, Taxiarchis V Kourelis, Rajshekhar Chakraborty, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, S Vincent Rajkumar, David Dingli, Nelson Leung, Robert A Kyle, Shaji K Kumar, Morie A Gertz
INTRODUCTION: The diagnosis of amyloidosis requires histological confirmation of Congo-red (CR) deposits. The tissue source is preferably fat aspiration and/or bone marrow biopsy, but at times organ biopsy is required. METHODS: We studied 612 patients with systemic immunoglobulin light chain amyloidosis to characterize the tissues used to establish the diagnosis. RESULTS: The median number of tissue samples was 3. 95% of bone marrow (BM) biopsies were stained for CR, while 79% of patients had fat aspiration CR-stained...
March 8, 2017: Annals of Medicine
https://www.readbyqxmd.com/read/28267610/vitamin-b12-offers-neuronal-cell-protection-by-inhibiting-a%C3%AE-42-amyloid-fibrillation
#17
Parvez Alam, Mohammad Khursheed Siddiqi, Sumit Kumar Chaturvedi, Masihuz Zaman, Rizwan Hasan Khan
Protein misfolding and aggregation has been implicated as the cause of more than 20 diseases in humans such as Alzheimer's and Parkinson's and systemic amyloidosis. Retardation of Aβ- 42 aggregation is considered as a promising and challenging strategy for developing effective therapeutics against Alzheimer's disease. Herein, we demonstrated the effect of vitamin B12 (VB) on inhibiting amyloid formation by employing ThT fluorescence assay, circular dichroism, ANS fluorescence assay, dynamic light scattering measurements and transmission electron microscopy and cell viability assay...
March 4, 2017: International Journal of Biological Macromolecules
https://www.readbyqxmd.com/read/28265615/citrate-stabilized-gold-nanoparticles-hinder-fibrillogenesis-of-a-pathological-variant-of-%C3%AE-2-microglobulin
#18
Cristina Cantarutti, Sara Raimondi, Giorgia Brancolini, Alessandra Corazza, Sofia Giorgetti, Maurizio Ballico, Stefano Zanini, Giovanni Palmisano, Paolo Bertoncin, Loredana Marchese, P Patrizia Mangione, Vittorio Bellotti, Stefano Corni, Federico Fogolari, Gennaro Esposito
Nanoparticles have repeatedly been shown to enhance fibril formation when assayed with amyloidogenic proteins. Recently, however, evidence casting some doubt about the generality of this conclusion started to emerge. Therefore, to investigate further the influence of nanoparticles on the fibrillation process, we used a naturally occurring variant of the paradigmatic amyloidogenic protein β2-microglobulin (β2m), namely D76N β2m where asparagine replaces aspartate at position 76. This variant is responsible for aggressive systemic amyloidosis...
March 7, 2017: Nanoscale
https://www.readbyqxmd.com/read/28264538/-a-case-of-hemodialysis-associated-pelvic-amyloidoma-mimicking-renal-cancer-associated-etiology
#19
Hideaki Takada, Kanji Nagahama, Yoshiharu Nakashima, Koji Yoshimura, Yasunori Nishio, Ken Matsuo, Noriko Mori
Patients with renal insufficiency receiving long-term hemodialysis often develop so-called hemodialysis amyloidosis characterized by systemic β 2-microglobulin amyloid lesions, while patients with renal cell carcinoma may develop amyloid A(AA) amyloidosis. Herein, we present a 67-year-old man on thirty-yearlong hemodialysis who was diagnosed to have left renal cell carcinoma coincident with a large spaceoccupying lesion adjacent to the psoas muscle in the pelvic cavity. An ultrasound-guided percutaneous needle biopsy was performed at the time of laparoscopic radical nephrectomy...
February 2017: Hinyokika Kiyo. Acta Urologica Japonica
https://www.readbyqxmd.com/read/28261692/immunoglobulin-light-chains-form-an-extensive-and-highly-ordered-fibril-involving-the-n-and-c-termini
#20
Dennis W Piehl, Luis M Blancas-Mejía, Jonathan S Wall, Stephen J Kennel, Marina Ramirez-Alvarado, Chad M Rienstra
Light-chain (AL)-associated amyloidosis is a systemic disorder involving the formation and deposition of immunoglobulin AL fibrils in various bodily organs. One severe instance of AL disease is exhibited by the patient-derived variable domain (VL) of the light chain AL-09, a 108 amino acid residue protein containing seven mutations relative to the corresponding germline protein, κI O18/O8 VL. Previous work has demonstrated that the thermodynamic stability of native AL-09 VL is greatly lowered by two of these mutations, Y87H and N34I, whereas a third mutation, K42Q, further increases the kinetics of fibril formation...
February 28, 2017: ACS Omega
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