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systemic amyloidosis

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https://www.readbyqxmd.com/read/27911082/necrotizing-and-haemorrhagic-hepatitis-and-enteritis-in-commercial-layer-pullets
#1
Yuko Sato, William L Wigle, Tsang Long Lin, Eric Gingerich, Patricia S Wakenell
This case report describes an episode of recurring severe necrotizing and haemorrhagic hepatitis and enteritis experienced in a flock of commercial layer pullets at 12 weeks of age and again at 18 weeks of age in Indiana. Pullets had been vaccinated at 10 weeks old using a trivalent Salmonella Enteritidis (SE)/Newcastle disease/infectious bronchitis oil-emulsion-inactivated vaccine. The pullets were found dead at 12 weeks with firm but friable, enlarged, haemorrhagic livers, enlarged spleens, and necrohaemorrhagic intestines...
December 2, 2016: Avian Pathology: Journal of the W.V.P.A
https://www.readbyqxmd.com/read/27900617/new-and-evolving-concepts-regarding-the-prognosis-and-treatment-of-cardiac-amyloidosis
#2
REVIEW
Stefano Perlini, Roberta Mussinelli, Francesco Salinaro
Systemic amyloidoses are rare and proteiform diseases, caused by extracellular accumulation of insoluble misfolded fibrillar proteins. Prognosis is dictated by cardiac involvement, which is especially frequent in light chain (AL) and in transthyretin variants (ATTR, both mutated, (ATTRm), and wild-type, (ATTRwt)). Recently, ATTRwt has emerged as a potentially relevant cause of a heart failure with preserved ejection fraction (HFpEF). Cardiac amyloidosis is an archetypal example of restrictive cardiomyopathy, with signs and symptoms of global heart failure and diastolic dysfunction...
November 29, 2016: Current Heart Failure Reports
https://www.readbyqxmd.com/read/27884064/amyloid-fibril-proteins-and-amyloidosis-chemical-identification-and-clinical-classification-international-society-of-amyloidosis-2016-nomenclature-guidelines
#3
Jean D Sipe, Merrill D Benson, Joel N Buxbaum, Shu-Ichi Ikeda, Giampaolo Merlini, Maria J M Saraiva, Per Westermark
The Nomenclature Committee of the International Society of Amyloidosis (ISA) met during the XVth Symposium of the Society, 3 July-7 July 2016, Uppsala, Sweden, to assess and formulate recommendations for nomenclature for amyloid fibril proteins and the clinical classification of the amyloidoses. An amyloid fibril must exhibit affinity for Congo red and with green, yellow or orange birefringence when the Congo red-stained deposits are viewed with polarized light. While congophilia and birefringence remain the gold standard for demonstration of amyloid deposits, new staining and imaging techniques are proving useful...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27867938/a-mutation-in-npas3-that-segregates-with-schizophrenia-in-a-small-family-leads-to-protein-aggregation
#4
Leslie G Nucifora, YeeWen Candace Wu, Brian J Lee, Li Sha, Russell L Margolis, Christopher A Ross, Akira Sawa, Frederick C Nucifora
Schizophrenia and other major mental illnesses result from a complex interplay of genetic and environmental factors. We previously identified a mutation in NPAS3 that results in a valine to isoleucine (V304I) amino acid substitution segregating with schizophrenia in a small family. The amino acid change occurs in a potentially critical region for protein function. Furthermore, the same amino acid substitution in proteins related to familial Alzheimer's disease and transthyretin amyloidosis has been associated with protein aggregation...
October 2016: Molecular Neuropsychiatry
https://www.readbyqxmd.com/read/27862787/two-diseases-one-remedy-systemic-amyloidosis-secondary-to-hidradenitis-suppurativa-treatment-with-infliximab
#5
İlkay Özer, Cengiz Karaçin, Esra Adışen, Galip Güz, Mehmet Ali Gürer
Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Herein, we present such a patient that had developed AA amyloidosis during the course of hidradenitis suppurativa...
November 12, 2016: Dermatologic Therapy
https://www.readbyqxmd.com/read/27861403/localized-immunoglobulin-light-chain-amyloid-of-the-conjunctiva-confirmed-by-mass-spectrometry-without-evidence-of-systemic-disease
#6
Eric B Hamill, Preeti J Thyparampil, Michael T Yen
Current guidelines recommend a thorough systemic workup in patients with newly diagnosed immunoglobulin amyloid light chain (AL) amyloidosis because of a close association with an underlying plasma cell proliferative disorder. Rarely, however, AL amyloidosis may present as a localized, benign disease without systemic involvement. Few previous reports have documented localized AL amyloid of the conjunctiva, and all these used immunohistochemistry for amyloid typing, which can be inaccurate. The authors identified 2 cases of AL amyloidosis confirmed by mass spectrometry localized to the conjunctiva and without evidence of systemic disease as determined by thorough systemic workup...
November 15, 2016: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/27856462/clarifying-immunoglobulin-gene-usage-in-systemic-and-localized-immunoglobulin-light-chain-amyloidosis-by-mass-spectrometry
#7
Taxiarchis V Kourelis, Surendra Dasari, Jason D Theis, Marina Ramirez-Alvarado, Paul J Kurtin, Morie A Gertz, Steven R Zeldenrust, Roman M Zenka, Ahmet Dogan, Angela Dispenzieri
The goal of this study was to investigate the frequency of use of light chain variable region (IGVL) genes among patients with systemic (ALS) and localized (ALL) amyloidosis and to assess for associations between IGVL gene usage and organ tropism. We evaluated clinic charts from 821 AL patients seen at the Mayo Clinic who had bone marrow, fat pad and solid organ tissue samples typed by liquid chromatography tandem mass spectrometry (LC-MS). We identified 701 patients with ALs and 120 with ALL. Overall, we were able to identify an IGVL gene in 87 (72%) patients with ALL and 573 (82%) patients with ALS...
November 16, 2016: Blood
https://www.readbyqxmd.com/read/27832515/bortezomib-dexamethasone-versus-high-dose-melphalan-for-japanese-patients-with-systemic-light-chain-al-amyloidosis-a-retrospective-single-center-study
#8
Nagaaki Katoh, Akihiro Ueno, Takuhiro Yoshida, Ko-Ichi Tazawa, Yasuhiro Shimojima, Takahisa Gono, Yoshiki Sekijima, Masayuki Matsuda, Shu-Ichi Ikeda
Bortezomib-dexamethasone (BD) and high-dose melphalan (HDM) are effective for systemic light-chain (AL) amyloidosis, but have not been compared in detail. We retrospectively investigated patients treated with BD or HDM at our center between September 2001 and June 2016. Among 234 patients, 20 were treated with BD and 30 received HDM. With the exception of age, transplant eligibility, and previous history of other chemotherapy, there were no significant differences in most background parameters between the two groups...
November 10, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27828646/primary-localized-cutaneous-amyloidosis-with-lichen-and-poikiloderma-like-lesions-and-an-excellent-response-to-systemic-acitretin
#9
Han Ma, Xiangyang Su, Guoxing Zhu, Songchao Yin, Chun Lu, Wei Lai
Primary localized cutaneous amyloidosis is a skin-limited amyloidosis that does not involve internal organs. It is clinically subclassified into 3 general categories and some rare variants. However, there is considerable overlap within the classification. Though there are a variety of therapeutic measures, the treatment is often unsatisfactory, particularly when the disease is severe and extensive. We describe a rare case of primary localized cutaneous amyloidosis with lichen and poikiloderma-like lesions that showed an excellent response to systemic acitretin...
September 2016: Anais Brasileiros de Dermatologia
https://www.readbyqxmd.com/read/27825470/monoclonal-igm-related-al-amyloidosis
#10
REVIEW
Paolo Milani, Giampaolo Merlini
Monoclonal immunoglobulin M (IgM)-related light chain (AL) amyloidosis, which accounts for 5%-7% of all AL amyloidosis cases, is a distinct clinical entity that poses specific challenges to clinicians. Several studies reported that although there is a substantial overlap, the pattern of organ involvement is peculiar, with higher frequencies of lung, lymph nodes, and peripheral nervous system involvement. A recent collaborative study from three European referral centers, defined that cardiac involvement, advanced Mayo disease stage, neuropathic, and liver involvement were independent factors that had impact on survival in IgM-AL amyloidosis patients...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27825464/prognostic-factors-and-indications-for-treatment-of-waldenstr%C3%A3-m-s-macroglobulinemia
#11
REVIEW
Robert A Kyle, Stephen M Ansell, Prashant Kapoor
Waldenström's Macroglobulinemia (WM) is characterized by the presence of an IgM monoclonal protein regardless of its size, 10% or more bone marrow infiltration by small lymphocytes with a plasmacytoid or plasma cell differentiation. These cells usually have the following markers: IgM+, CD5(-), CD10(-), CD19(+), CD20(+) and CD23(-). Chronic lymphocytic leukemia as well as other lymphoproliferative disorders such as mantle cell, marginal zone and mucosa-associated lymphoid tissue (MALT) lymphoma must be excluded...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27822733/-cutaneous-manifestations-in-renal-diseases
#12
M Schmid-Simbeck, A Udvardi, B Volc-Platzer
In addition to general skin changes like pallor or dryness and the frequent, often excruciating nephrogenic pruritus, specific diseases in patients with renal failure may occur. Acquired perforating dermatoses are usually also highly pruritic. Calciphylaxis is a severe disease with poor prognosis. Nonhealing wounds with superinfection and progression to sepsis are characteristic. Bullous lesions can be caused by disturbances in porphyrin metabolism. Nephrogenic systemic fibrosis is a disease which was first described in 2000...
December 2016: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/27814585/mechanisms-of-protein-aggregation-and-inhibition
#13
Mohammad Khursheed Siddiqi, Parvez Alam, Sumit Kumar Chaturvedi, Yasser E Shahein, Rizwan Hasan Khan
Protein and peptide aggregation raises keen interest due to their involvement in number of pathological conditions ranging from neurodegenerative disorders to systemic amyloidosis. Here, we have reviewed recent advances in mechanisms of aggregation, emerging technologies towards exploration, characterization of aggregate structures, detection at molecular level and the strategies to combat the phenomenon of aggregation both in cellular and in vitro conditions. In consistence, we have illustrated almost all factors that influence the protein aggregation both in vitro and in vivo environments...
January 1, 2017: Frontiers in Bioscience (Elite Edition)
https://www.readbyqxmd.com/read/27811147/development-of-the-autoinflammatory-disease-damage-index-addi
#14
Nienke M Ter Haar, Kim V Annink, Sulaiman M Al-Mayouf, Gayane Amaryan, Jordi Anton, Karyl S Barron, Susanne M Benseler, Paul A Brogan, Luca Cantarini, Marco Cattalini, Alexis-Virgil Cochino, Fabrizio De Benedetti, Fatma Dedeoglu, Adriana A De Jesus, Ornella Della Casa Alberighi, Erkan Demirkaya, Pavla Dolezalova, Karen L Durrant, Giovanna Fabio, Romina Gallizzi, Raphaela Goldbach-Mansky, Eric Hachulla, Veronique Hentgen, Troels Herlin, Michaël Hofer, Hal M Hoffman, Antonella Insalaco, Annette F Jansson, Tilmann Kallinich, Isabelle Koné-Paut, Anna Kozlova, Jasmin B Kuemmerle-Deschner, Helen J Lachmann, Ronald M Laxer, Alberto Martini, Susan Nielsen, Irina Nikishina, Amanda K Ombrello, Seza Ozen, Efimia Papadopoulou-Alataki, Pierre Quartier, Donato Rigante, Ricardo Russo, Anna Simon, Maria Trachana, Yosef Uziel, Angelo Ravelli, Marco Gattorno, Joost Frenkel
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency...
November 3, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27809600/plasma-hepatocyte-growth-factor-is-a-novel-marker-of-al-cardiac-amyloidosis
#15
Kristopher J Swiger, Eitan A Friedman, Evan L Brittain, Kelsey A Tomasek, Shi Huang, Yan R Su, Douglas B Sawyer, Daniel J Lenihan
BACKGROUND: Cardiac amyloidosis is an infiltrative cardiomyopathy that is challenging to diagnose. We hypothesized that the novel biomarkers hepatocyte growth factor (HGF), galectin-3 (GAL-3), interleukin-6 (IL-6), and vascular endothelial growth factor (VEGF) would be elevated in cardiac amyloidosis and may be able to discriminate from non-cardiac systemic amyloidosis or other cardiomyopathies with similar clinical or morphologic characteristics. METHODS: Patients were selected from the Vanderbilt Main Heart Registry according to the following groups: (1) amyloid light-chain (AL) cardiac amyloidosis (n = 26); (2) transthyretin (ATTR) cardiac amyloidosis (n = 7); (3) left ventricular hypertrophy (LVH) (n = 45); (4) systolic heart failure (n = 42); and (5) non-cardiac systemic amyloidosis (n = 7)...
December 2016: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/27806283/biochemical-and-electrophysiological-modification-of-amyloid-transthyretin-on-cardiomyocytes
#16
Laura Sartiani, Monica Bucciantini, Valentina Spinelli, Manuela Leri, Antonino Natalello, Daniele Nosi, Silvia Maria Doglia, Annalisa Relini, Amanda Penco, Sofia Giorgetti, Elisabetta Gerace, Guido Mannaioni, Vittorio Bellotti, Stefania Rigacci, Elisabetta Cerbai, Massimo Stefani
Transthyretin (TTR) amyloidoses are familial or sporadic degenerative conditions that often feature heavy cardiac involvement. Presently, no effective pharmacological therapy for TTR amyloidoses is available, mostly due to a substantial lack of knowledge about both the molecular mechanisms of TTR aggregation in tissue and the ensuing functional and viability modifications that occur in aggregate-exposed cells. TTR amyloidoses are of particular interest regarding the relation between functional and viability impairment in aggregate-exposed excitable cells such as peripheral neurons and cardiomyocytes...
November 1, 2016: Biophysical Journal
https://www.readbyqxmd.com/read/27804150/progressive-refractory-light-chain-amyloidosis-and-multiple-myeloma-patients-are-responsive-to-the-addition-of-clarithromycin-to-imid-based-therapy
#17
Adir Shaulov, Chezi Ganzel, Noam Benyamini, Yossef Barshay, Neta Goldschmidt, David Lavie, Diana Libster, Alex Gural, Batia Avni, Moshe E Gatt
Multiple myeloma (MM) and primary systemic light chain amyloidosis (AL) are both chronic plasma cell dyscrasias with different clinical expression but limited treatment options for relapsed refractory disease. We report the effect of the addition of clarithromycin on 31 MM and 17 AL with relapsed or refractory disease who had an insufficient response or disease progression while on an IMiD based therapy. In this high risk population, hematological response was reported in 48% of MM patients and 94% of AL patients...
November 2, 2016: American Journal of Hematology
https://www.readbyqxmd.com/read/27801739/18f-florbetaben-a-new-tool-for-amyloidosis-staging
#18
Emmanuel D'Estanque, Benjamin Chambert, Olivier Moranne, Pierre Olivier Kotzki, Vincent Boudousq
We report the case of a 73-year-old man with a documented (renal biopsy) light-chain amyloidosis (AL) imaged with F-AV-1 (F-florbetaben) compared with a volunteer. A cardiac amyloidosis was suspected. As it was an AL and not a transthyretin amyloidosis, F-FDG and F-florbetaben PET/CT were preferred to bone scan. F-FDG scintigraphy showed a focal cardiac hypermetabolism. In addition of the heart, F-florbetaben scintigraphy showed an intense spleen and thyroid pathologic uptake and a moderate salivary gland and kidney uptake...
October 31, 2016: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27796618/distinct-secretory-activity-and-clinical-impact-of-subcutaneous-abdominal-adipose-tissue-in-women-with-rheumatoid-arthritis-and-osteoarthritis
#19
Ewa Kontny, Agnieszka Zielińska, Urszula Skalska, Krystyna Księżopolska-Orłowska, Piotr Głuszko, Włodzimierz Maśliński
In the general population, low-grade inflammation of adipose tissue accompanies obesity and contributes to cardiovascular disease (CVD) development, but the implication of this tissue in rheumatic disease pathology is unclear. Therefore, we characterized the secretory activity of subcutaneous abdominal adipose tissue (SAAT) of females with rheumatoid arthritis (RA) and osteoarthritis (OA) and searched for its relationship with intensity of systemic inflammation, body composition and comorbidity. The secretion of classical adipokines (leptin, adiponectin), pro- and anti-inflammatory factors, i...
October 28, 2016: Inflammation
https://www.readbyqxmd.com/read/27795698/implementation-and-evaluation-of-amyloidosis-subtyping-by-laser-capture-microdissection-and-tandem-mass-spectrometry
#20
Peter Mollee, Samuel Boros, Dorothy Loo, Jayde E Ruelcke, Vanessa A Lakis, Kim-Anh Lê Cao, Patricia Renaut, Michelle M Hill
BACKGROUND: Correct identification of the amyloidosis-causing protein is crucial for clinical management. Recently the Mayo Clinic reported laser-capture microdissection (LCM) with liquid chromatography-coupled tandem mass spectrometry (MS/MS) as a new diagnostic tool for amyloid diagnosis. Here, we report an independent implementation of this proteomic diagnostics method at the Princess Alexandra Hospital Amyloidosis Centre in Brisbane, Australia. RESULTS: From 2010 to 2014, 138 biopsies received from 35 different organ sites were analysed by LCM-MS/MS using Congo Red staining to visualise amyloid deposits...
2016: Clinical Proteomics
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