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systemic amyloidosis

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https://www.readbyqxmd.com/read/29311044/multiple-myeloma-presenting-with-acquired-cutis-laxa-and-primary-systemic-amyloidosis
#1
Min Young Lee, Ji Yeon Byun, You Won Choi, Hae Young Choi
No abstract text is available yet for this article.
December 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/29303358/treatment-patterns-and-health-care-resource-utilization-among-patients-with-relapsed-refractory-systemic-light-chain-amyloidosis
#2
Parameswaran Hari, Huamao Mark Lin, Carl V Asche, Jinma Ren, Candice Yong, Katarina Luptakova, Douglas V Faller, Vaishali Sanchorawala
BACKGROUND: Treatment for patients with systemic light chain (AL) amyloidosis remains challenging. Our study aims to describe treatment patterns for both newly diagnosed and relapsed/refractory AL (RRAL) amyloidosis, and to assess clinical outcomes, healthcare costs, and resource utilization during the first year following a diagnosis of RRAL amyloidsis. METHODS: This was a retrospective observational study of adult patients with AL amyloidosis using the US Optum administrative claims data during 1/1/2008 to 6/30/2015...
January 5, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29303069/unfolding-cardiac-amyloidosis-from-pathophysiology-to-cure
#3
Klemens Ablasser, Nicolas Verheyen, Theresa Glantschnig, Giulio Agnetti, Peter P Rainer
Deposition of amyloidogenic proteins leading to the formation of amyloid fibrils in the myocardium cause cardiac amyloidosis. Although any form of systemic amyloidosis can affect the heart, light-chain (AL) or transthyretin amyloidosis (ATTR) account for the majority of diagnosed cardiac amyloid deposition. The extent of cardiac disease independently predicts mortality. The reversal or arrest of adverse cardiac remodeling is the target of current therapies, as cardiac-related mortality worsens prognosis in patients where the underlying systemic amyloidosis was successfully treated...
January 4, 2018: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/29298867/repeat-doses-of-antibody-to-serum-amyloid-p-component-clear-amyloid-deposits-in-patients-with-systemic-amyloidosis
#4
Duncan B Richards, Louise M Cookson, Sharon V Barton, Lia Liefaard, Thirusha Lane, David F Hutt, James M Ritter, Marianna Fontana, James C Moon, Julian D Gillmore, Ashutosh Wechalekar, Philip N Hawkins, Mark B Pepys
Systemic amyloidosis is a fatal disorder caused by pathological extracellular deposits of amyloid fibrils that are always coated with the normal plasma protein, serum amyloid P component (SAP). The small-molecule drug, miridesap, [(R)-1-[6-[(R)-2-carboxy-pyrrolidin-1-yl]-6-oxo-hexanoyl]pyrrolidine-2-carboxylic acid (CPHPC)] depletes circulating SAP but leaves some SAP in amyloid deposits. This residual SAP is a specific target for dezamizumab, a fully humanized monoclonal IgG1 anti-SAP antibody that triggers immunotherapeutic clearance of amyloid...
January 3, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29280335/amyloidosis-diagnosed-in-cytology-specimen-of-pleural-effusion-a-case-report
#5
Rashmi Manur, Ihab Lamzabi
Amyloidosis is a rare condition resulting from extracellular deposition of amyloid, a fibrillary material derived from various precursor proteins. Involvement of the pleura by amyloidosis is a rare but serious complication. Pleural amyloidosis is primarily diagnosed by identifying amyloid deposition by histology on pleural biopsy specimens. Hereby, we report a case of systemic amyloidosis where we were able to identify amyloid in a pleural effusion specimen sent for cytopathology evaluation. A 59-year-old male with newly diagnosed multiple myeloma and systemic amyloidosis underwent therapeutic thoracentesis...
December 27, 2017: Diagnostic Cytopathology
https://www.readbyqxmd.com/read/29277593/oculoleptomeningeal-amyloidosis-secondary-to-the-rare-transthyretin-c-381t-g-p-ile127met-mutation
#6
Francois Mathieu, Erin Morgan, Joyce So, David G Munoz, Warren Mason, Paul Kongkham
BACKGROUND: Oculoleptomeningeal amyloidosis (OLMA) represents a rare subtype of familial transthyretin (TTR) amyloidosis, characterized by deposition of amyloid in cranial and spinal leptomeninges along with ocular involvement. Of more than 100 TTR mutations identified, few have been associated with OLMA. Herein we describe the first report of leptomeningeal amyloidosis associated with the c.381T>G (p.Ile127Met) TTR mutation, linking this variant in the OLMA phenotype. CASE DESCRIPTION: A 53 year-old male presented with a 2-year history of progressive symptoms including upper and lower limb weakness, ataxia, peripheral and autonomic neuropathy...
December 22, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29241665/structure-and-energetic-basis-of-overrepresented-%C3%AE-light-chain-in-systemic-light-chain-amyloidosis-patients
#7
Jun Zhao, Baohong Zhang, Jianwei Zhu, Ruth Nussinov, Buyong Ma
No abstract text is available yet for this article.
December 11, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29239128/efficacy-of-anakinra-in-a-patient-with-systemic-amyloidosis-presented-with-amyloidoma
#8
Hulya Nalcacioglu, Ozan Ozkaya, Gurkan Genc, Suat Ayyildiz, Mehmet Kefeli, Murat Elli, Oguz Aydin, Meltem Ceyhan Bilgici
Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of unique protein fibrils. The least common presentation of an amyloid deposition is as a discrete mass called amyloidoma or amyloid tumor. We report a case of a soft tissue amyloidoma in the abdomen of a 16-year-old girl who was diagnosed as having systemic amyloidosis. A girl aged 16 years was referred to our hospital with a pre-diagnosis of a retroperitoneal mass documented with abdominal ultrasonography and tomography...
December 13, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29233677/alzheimer-s-disease-and-gut-microbiota-modifications-the-long-way-between-preclinical-studies-and-clinical-evidence
#9
REVIEW
Cesare Mancuso, Rosaria Santangelo
Recent studies have suggested the role of an infectious component in the pathogenesis of Alzheimer's disease (AD). In light of this, research has focused on some bacteria constituting the intestinal microbial flora which can produce amyloid. Once generated, the latter hypothetically triggers a systemic inflammatory response which compromises complex brain functions, such as learning and memory. Clinical studies have shown that, in cognitively impaired elderly patients with brain amyloidosis, there is lower abundance in the gut of E...
December 9, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/29233648/left-atrial-dysfunction-in-light-chain-cardiac-amyloidosis-and-hypertrophic-cardiomyopathy-a-comparative-three-dimensional-speckle-tracking-echocardiographic-analysis-from-the-magyar-path-study
#10
Dóra Földeák, Árpád Kormányos, Péter Domsik, Anita Kalapos, Györgyike Á Piros, Nóra Ambrus, Zénó Ajtay, Róbert Sepp, Zita Borbényi, Tamás Forster, Attila Nemes
INTRODUCTION: While cardiac amyloidosis (CA) is a rare systemic disease characterized by extracellular deposition of protein-derived fibrils, hypertrophic cardiomyopathy (HCM) is histopathologically characterized by myocyte hypertrophy and disarray, interstitial fibrosis, and small intramural coronary arteriole dysplasia. The aim of the present study was to compare left atrial (LA) volumetric and functional characteristics between light-chain (AL) CA and HCM by three-dimensional (3D) speckle-tracking echocardiography (STE)...
December 9, 2017: Portuguese Journal of Cardiology: An Official Journal of the Portuguese Society of Cardiology
https://www.readbyqxmd.com/read/29228957/evaluation-of-the-effect-of-d-amino-acid-incorporation-into-amyloid-reactive-peptides
#11
Emily B Martin, Angela Williams, Tina Richey, Craig Wooliver, Alan Stuckey, James S Foster, Stephen J Kennel, Jonathan S Wall
BACKGROUND: Systemic amyloidoses comprise diseases characterized by the deposition of proteinaceous material known as amyloid. Currently, without performing multiple biopsies, there is no way to ascertain the extent of amyloid deposition in patients-a critical piece of information that informs prognosis and therapeutic strategies. We have developed pan-amyloid-targeting peptides for imaging amyloid and recently have adapted these for use as pre-targeting agents in conjunction with immunotherapy...
December 11, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/29222231/al-amyloidosis-from-molecular-mechanisms-to-targeted-therapies
#12
REVIEW
Giampaolo Merlini
Systemic amyloidosis is caused by misfolding and extracellular deposition of circulating proteins as amyloid fibrils, resulting in the dysfunction of vital organs. The most common systemic amyloidosis, light-chain (AL) amyloidosis, is caused by misfolded light chains produced by a small, dangerous B-cell clone. The process of amyloid formation, organ targeting, and damage is multifaceted and, after disease initiation, the complexity of the downstream pathogenic cascade increases, rendering its control a challenge...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29217108/improved-outcomes-after-heart-transplantation-for-cardiac-amyloidosis-in-the-modern-era
#13
Arnt V Kristen, Michael M Kreusser, Patrick Blum, Stefan O Schönland, Lutz Frankenstein, Andreas O Dösch, Benjamin Knop, Matthias Helmschrott, Bastian Schmack, Arjang Ruhparwar, Ute Hegenbart, Hugo A Katus, Philip W J Raake
BACKGROUND: Cardiac amyloidosis, caused most commonly by deposition of light chain (AL) or transthyretin (ATTR) type fibrils, has an extremely poor prognosis. In this retrospective single-center study, we evaluated temporal trends in survival after heart transplantation for cardiac amyloidosis. METHODS: We analyzed 48 patients with cardiac amyloidosis (AL, n = 32; familial ATTR, n = 16) who underwent heart transplantation from May 2002 to March 2017. Patients were analysed in 2 periods, Era 1 (2002- 2007) and Era 2 (2008- 2017), separated by altered patient selection in both, AL and ATTR amyloidosis, and changed chemotherapy regimens for AL amyloidosis...
November 15, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/29212103/recurrent-diffuse-gastric-bleeding-as-a-leading-symptom-of%C3%A2-gastrointestinal-al-amyloidosis
#14
Caspar Franck, Marino Venerito, Jochen Weigt, Albert Roessner, Peter Malfertheiner
Amyloidosis is a rare disease (incidence about 0.8/100 000) characterized by extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of serum proteins. Clinical manifestations are largely determined by the type of precursor protein, the tissue distribution and the amount of amyloid deposition. Gastrointestinal (GI) manifestations of amyloidosis are even more uncommon (3 % of all amyloidosis patients). Symptoms of GI amyloidosis are nonspecific, heterogeneous, and include weight loss, GI bleeding, heartburn, early satiety, diarrhea and abdominal pain...
December 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29209781/age-dependent-cognitive-dysfunction-in-untreated-hereditary-transthyretin-amyloidosis
#15
Ana Martins da Silva, Sara Cavaco, Joana Fernandes, Raquel Samões, Cristina Alves, Márcio Cardoso, Jeffery W Kelly, Cecília Monteiro, Teresa Coelho
Central nervous system (CNS) involvement in hereditary transthyretin (TTR) amyloidosis has been described in patients whose disease course was modified by liver transplant. However, cognitive dysfunction has yet to be investigated in those patients. Moreover, CNS involvement in untreated patients or asymptomatic mutation carriers remains to be studied. A series of 340 carriers of the TTRVal30Met mutation (180 symptomatic and 160 asymptomatic) underwent a neuropsychological assessment, which included the Dementia Rating Scale-2 (DRS-2), auditory verbal learning test, semantic fluency, phonemic fluency, and trail making test...
December 5, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/29202802/histopathology-microbiology-and-the-inflammatory-process-associated-with-sarcoptes-scabiei-infection-in-the-iberian-ibex-capra-pyrenaica
#16
José Espinosa, Arián Ráez-Bravo, Jorge R López-Olvera, Jesús M Pérez, Santiago Lavín, Asta Tvarijonaviciute, Francisco J Cano-Manuel, Paulino Fandos, Ramón C Soriguer, José Enrique Granados, Diego Romero, Roser Velarde
BACKGROUND: Sarcoptic mange has been identified as the most significant infectious disease affecting the Iberian ibex (Capra pyrenaica). Despite several studies on the effects of mange on ibex, the pathological and clinical picture derived from sarcoptic mange infestation is still poorly understood. To further knowledge of sarcoptic mange pathology, samples from ibex were evaluated from histological, microbiological and serological perspectives. METHODS: Samples of skin, non-dermal tissues and blood were collected from 54 ibex (25 experimentally infected, 15 naturally infected and 14 healthy)...
December 4, 2017: Parasites & Vectors
https://www.readbyqxmd.com/read/29200282/incomplete-refolding-of-antibody-light-chains-to-non-native-protease-sensitive-conformations-leads-to-aggregation-a-mechanism-of-amyloidogenesis-in-patients
#17
Gareth J Morgan, Grace A Usher, Jeffery W Kelly
Genetic, biochemical, and pharmacologic evidence supports the hypothesis that conformationally altered or misfolded protein states enable aggregation and cytotoxicity in the systemic amyloid diseases. Reversible structural fluctuations of natively folded proteins are involved in the aggregation of many degenerative disease associated proteins. Herein, we use antibody light chains (LCs) that form amyloid fibrils in AL amyloidosis to consider an alternative hypothesis of amyloidogenesis: that transient unfolding and incomplete extracellular refolding of secreted proteins can lead to metastable, alternatively folded states that are more susceptible to aggregation or to endoproteolysis that can release aggregation-prone fragments...
December 19, 2017: Biochemistry
https://www.readbyqxmd.com/read/29199213/variation-of-amino-acid-sequences-of-serum-amyloid-a-saa-and-immunohistochemical-analysis-of-amyloid-a-aa-in-japanese-domestic-cats
#18
Meina Tei, Kazuyuki Uchida, James K Chambers, Ken-Ichi Watanabe, Takashi Tamamoto, Koichi Ohno, Hiroyuki Nakayama
Amyloid A (AA) amyloidosis, a fatal systemic amyloid disease, occurs secondary to chronic inflammatory conditions in humans. Although persistently elevated serum amyloid A (SAA) levels are required for its pathogenesis, not all individuals with chronic inflammation necessarily develop AA amyloidosis. Furthermore, many diseases in cats are associated with the elevated production of SAA, whereas only a small number actually develop AA amyloidosis. We hypothesized that a genetic mutation in the SAA gene may strongly contribute to the pathogenesis of feline AA amyloidosis...
December 4, 2017: Journal of Veterinary Medical Science
https://www.readbyqxmd.com/read/29199167/which-diseases-are-risk-factors-for-developing-gastroesophageal-reflux-disease
#19
Filiz Akyüz, Özlem Mutluay Soyer
Although the pathophysiology of gastroesophageal reflux disease (GERD) remains unclear, it is accepted as a multifactorial disease. It is thought that some of the interventions that might cause alterations in the normal gastrointestinal tract anatomy and diseases that affect the lower esophageal sphincter, esophageal clearance, and stomach motility (gastric emptying) might lay a foundation for GERD development. Moreover, it is common knowledge that GERD might cause various extraesophageal symptoms and complications...
December 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/29197924/echocardiography-as-the-first-diagnostic-clue-to-rapidly-progressive-systemic-al-amyloidosis-associated-with-multiple-site-thrombosis-a-case-report
#20
Bogdan Caloian, Dumitru Zdrenghea, Sorin Claudiu Man, Simona Costea, Mihnea Zdrenghea, Dana Pop
AL-amyloidosis is a rare, but complex disease, with a severe prognosis, cardiac involvement being found in half of the patients. The rapid increase of the LV wall thickness predicts an unfavorable evolution. We report the case of a 63-year-old man diagnosed with AL-amyloidosis, with cardiac involvement, associated with multiple site thrombosis.  Specific echocardiographic methods like tissue Doppler imaging and speckle tracking provided crucial diagnostic and prognostic information.
November 29, 2017: Medical Ultrasonography
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