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Primary amyloidosis

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https://www.readbyqxmd.com/read/29146130/corrigendum-to-familial-primary-localized-cutaneous-amyloidosis-in-a-japanese-family-j-dermatol-sci-83-2016-162-164
#1
D Ueo, A Utani, Y Okubo, M Yozaki, Y Mine, T Anan, H Nishida, D Takahashi, T Sakai, Y Hatano, S Fujiwara
No abstract text is available yet for this article.
November 13, 2017: Journal of Dermatological Science
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/29141071/usefulness-of-total-12-lead-qrs-voltage-as-a-clue-to-diagnosis-of-patients-with-cardiac-sarcoidosis-severe-enough-to-warrant-orthotopic-heart-transplant
#3
William C Roberts, Tiffany M Becker, Shelley A Hall
Importance: Severe heart failure caused by cardiac sarcoidosis is difficult to diagnosis without biopsy. Objective: To assess whether total electrocardiographic 12-lead QRS voltage may be a clue to diagnosis. Design, Setting, Participants: Case-series study with cases collected at Baylor University Medical Center at Dallas, Dallas, Texas, from January 13, 2005, to January 24, 2017. The clinical records of 16 patients with severe heart failure caused by cardiac sarcoidosis were studied...
November 15, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/29121956/regional-differences-in-prognostic-value-of-cardiac-valve-plane-displacement-in-systemic-light-chain-amyloidosis
#4
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/29097623/dnaj-heat-shock-protein-family-b-member-9-is-a-novel-biomarker-for-fibrillary-gn
#5
Surendra Dasari, Mariam P Alexander, Julie A Vrana, Jason D Theis, John R Mills, Vivian Negron, Sanjeev Sethi, Angela Dispenzieri, W Edward Highsmith, Samih H Nasr, Paul J Kurtin
Fibrillary GN (FGN) is a rare primary glomerular disease. Histologic and histochemical features of FGN overlap with those of other glomerular diseases, and no unique histologic biomarkers for diagnosing FGN have been identified. We analyzed the proteomic content of glomeruli in patient biopsy specimens and detected DnaJ heat shock protein family (Hsp40) member B9 (DNAJB9) as the fourth most abundant protein in FGN glomeruli. Compared with amyloidosis glomeruli, FGN glomeruli exhibited a >6-fold overexpression of DNAJB9 protein...
November 2, 2017: Journal of the American Society of Nephrology: JASN
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
#6
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/29077585/cardiovascular-complications-of-proteasome-inhibitors-used-in-multiple-myeloma
#7
Daniel C Cole, William H Frishman
The use of proteasome inhibitors (PI) as targeted chemotherapeutics have significantly improved survival in patients with multiple myeloma (MM). However, rare and serious cardiovascular complications have occurred as a result of their use, most commonly congestive heart failure, hypertension, and arrhythmias. MM occurs in an aged population with many concurrent cardiovascular risk factors. The primary disease process also contributes to cardiovascular complications. Furthermore, many MM patients have prior exposure to cardiotoxic chemotherapy such as anthracyclines...
October 25, 2017: Cardiology in Review
https://www.readbyqxmd.com/read/29070439/amyloid-and-immune-homeostasis
#8
REVIEW
Ying-Hui Wang, Yu-Gen Zhang
Extracellular amyloid deposition defines a range of amyloidosis and amyloid-related disease. Addition to primary and secondary amyloidosis, amyloid-related disease can be observed in different tissue/organ that sharing the common pathogenesis based on the formation of amyloid deposition. Currently, both Alzheimer's disease and type 2 diabetes can be diagnosed with certainly only based on the autopsy results, by which amyloidosis of the associative tissue/organ is observed. Intriguingly, since it demonstrated that amyloid deposits trigger inflammatory reaction through the activation of cascaded immune response, wherein several lines of evidence implies a protective role of amyloid in preventing autoimmunity...
October 16, 2017: Immunobiology
https://www.readbyqxmd.com/read/29058206/role-of-cardiovascular-magnetic-resonance-in-suspected-cardiac-amyloidosis-late-gadolinium-enhancement-pattern-as-mortality-predictor
#9
M Baroni, S Nava, G Quattrocchi, A Milazzo, C Giannattasio, A Roghi, P Pedrotti
BACKGROUND: Cardiac magnetic resonance (CMR) has gained a central role in the diagnosis of cardiac amyloidosis (CA). While the diagnostic role of a typical late gadolinium enhancement (LGE) pattern (global subendocardial enhancement coupled with accelerated contrast washout) has been identified, evidence is still conflicting regarding the prognostic role of such examination. METHODS AND RESULTS: We retrospectively analysed all patients referring for CMR at Niguarda Hospital (Milan, Italy) from January 2006 to January 2015 for suspected CA...
October 20, 2017: Netherlands Heart Journal
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
#10
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
https://www.readbyqxmd.com/read/29020719/diagnosis-and-management-of-nephrotic-syndrome
#11
Oonagh McCloskey, Alexander P Maxwell
Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L). It is often associated with hyperlipidaemia, thromboembolism and an increased risk of infection. Nephrotic syndrome develops following pathological injury to renal glomeruli. This may be a primary problem, with a disease specific to the kidneys, or secondary to a systemic disorder such as diabetes mellitus. The most common cause in children is minimal change glomerulonephritis...
February 2017: Practitioner
https://www.readbyqxmd.com/read/28970295/infectious-and-non-infectious-complications-in-primary-immunodeficiency-disorders-an-autopsy-study-from-north-india
#12
Kirti Gupta, Amit Rawat, Parimal Agrawal, Ankur Jindal, Ritambhra Nada, Biman Saikia, Koon Wing Chan, Yu Lung Lau, Ranjana Walker Minz, Surjit Singh
BACKGROUND: Primary immunodeficiency disorders (PID) include a wide spectrum of inherited disorders characterised by functional abnormalities of one or more components of the immune system. Recent updates from the genomic data have contributed significantly to its better understanding with identification of new entities. Diagnosis is always challenging due to their variable clinical presentation. With the evolution of molecular diagnosis, many of these children are being diagnosed early and offered appropriate therapy...
September 28, 2017: Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28969251/primary-cutaneous-amyloidosis-a-clinical-histopathological-and-immunofluorescence-study
#13
Krati Mehrotra, Rupali Dewan, Jagannath V Kumar, Abhinav Dewan
INTRODUCTION: Primary Localized Cutaneous Amyloidosis (PLCA) is a relatively rare chronic condition characterized by amyloid deposition in dermis without associated deposits in internal organs. Histopathology of cutaneous amyloidosis using Haematoxylin and Eosin (H&E) stain shows eosinophilic hyaline material in papillary dermis, which can be further confirmed by Congo Red (CR) staining or Direct Immunofluorescence (DIF) Test or immunohistochemistry. AIM: To assess the concordance between the clinical, histo pathological and DIF findings in various subtypes of (PLCA)...
August 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28935176/current-insight-in-the-localized-insulin-derived-amyloidosis-lida-clinico-pathological-characteristics-and-differential-diagnosis
#14
REVIEW
Amir Mehdi Ansari, Lais Osmani, Aerielle E Matsangos, Qing K Li
BACKGROUND: In diabetic patients, subcutaneous insulin injection may cause several types of injection site-related lesions, such as lipoatrophy, insulin-induced cutaneous lipohypertrophy (IICL), allergic reaction, and iatrogenic localized insulin-derived amyloidosis (LIDA). Among these complications, both IICL and LIDA present as tumor-like and slow growing lesions; and they may be confused with one another. The clinical implication and management of IICL and LIDA are different. LIDA causes poor blood glycemic controls due to inadequate absorption of the insulin...
October 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28912854/comparative-proteomics-analysis-of-primary-cutaneous-amyloidosis
#15
Daxing Cai, Yang Li, Chunlei Zhou, Yulin Jiang, Jian Jiao, Lin Wu
Primary cutaneous amyloidosis (PCA) is a localized skin disorder that is characterized by the abnormal deposition of amyloid in the extracellular matrix (ECM) of the dermis. The pathogenesis of PCA is poorly understood. The objective of the present study was to survey proteome changes in PCA lesions in order to gain insight into the molecular basis and pathogenesis of PCA. Total protein from PCA lesions and normal skin tissue samples were extracted and analyzed using the isobaric tags for relative and absolute quantitation technique...
October 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#16
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28903570/czech-registry-of-monoclonal-gammopathies-technical-solution-data-collection-and-visualisation
#17
L Brozova, D Schwarz, I Snabl, J Kalina, B Pavlickova, M Komenda, J Jarkovský, P Němec, D Horinek, Z Stefanikova, L Pour, R Hájek, V Maisnar
BACKGROUND: The Registry of Monoclonal Gammopathies (RMG) was established by the Czech Myeloma Group in 2007. RMG is a registry designed for the collection of clinical data concerning diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies. Data on patients with monoclonal gammopathy of undetermined significance (MGUS), Waldenström macroglobulinaemia (WM), multiple myeloma (MM) or primary AL ("amyloid light-chain") amyloidosis are collected in the registry...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28902953/dystrophic-calcification-in-a-patient-with-primary-localized-cutaneous-nodular-amyloidosis-an-uncommon-ultrasound-finding
#18
Sergi Planas Ciudad, Esther Roé Crespo, Juan Francisco Mir Bonafé, Fania Zamantta Muñoz-Garza, Luis Puig Sanz
No abstract text is available yet for this article.
September 13, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28894337/primary-tracheobronchial-amyloidosis
#19
Pedro Paulo Teixeira E Silva Torres, Matheus Rabahi, Sebastião Alves Pinto, Karla Cristina de Morais Arantes Curado, Marcelo Fouad Rabahi
No abstract text is available yet for this article.
July 2017: Radiologia Brasileira
https://www.readbyqxmd.com/read/28893208/trial-design-and-rationale-for-apollo-a-phase-3-placebo-controlled-study-of-patisiran-in-patients-with-hereditary-attr-amyloidosis-with-polyneuropathy
#20
David Adams, Ole B Suhr, Peter J Dyck, William J Litchy, Raina G Leahy, Jihong Chen, Jared Gollob, Teresa Coelho
BACKGROUND: Patisiran is an investigational RNA interference (RNAi) therapeutic in development for the treatment of hereditary ATTR (hATTR) amyloidosis, a progressive disease associated with significant disability, morbidity, and mortality. METHODS: Here we describe the rationale and design of the Phase 3 APOLLO study, a randomized, double-blind, placebo-controlled, global study to evaluate the efficacy and safety of patisiran in patients with hATTR amyloidosis with polyneuropathy...
September 11, 2017: BMC Neurology
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