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amyloidosis guidelines

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https://www.readbyqxmd.com/read/27931749/-hereditary-epidermolysis-bullosa-french-national-guidelines-pnds-for-diagnosis-and-treatment
#1
C Chiaverini, E Bourrat, J Mazereeuw-Hautier, S Hadj-Rabia, C Bodemer, J-P Lacour
Hereditary epidermolysis bullosa (EB) is a heterogeneous group of rare genetic diseases characterized by fragile skin and/or mucous membrane, and it may be either local or generalized. It is caused by mutations in genes encoding different proteins involved mainly in the structure and function of the dermal-epidermal junction. Nineteen genes have so far been identified. They are classified by level of skin cleavage (from top to bottom) into four groups: EB simplex, junctional EB, dystrophic EB and Kindler syndrome...
December 5, 2016: Annales de Dermatologie et de Vénéréologie
https://www.readbyqxmd.com/read/27884064/amyloid-fibril-proteins-and-amyloidosis-chemical-identification-and-clinical-classification-international-society-of-amyloidosis-2016-nomenclature-guidelines
#2
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/27861403/localized-immunoglobulin-light-chain-amyloid-of-the-conjunctiva-confirmed-by-mass-spectrometry-without-evidence-of-systemic-disease
#3
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/27774327/poor-outcomes-in-hepatic-amyloidosis-a-report-of-2-cases
#4
Brandon Yim, Elizabeth Kertowidjojo, Yue Zhang, Pruthvi Patel
Hepatic amyloidosis is a rare disease entity that results from insoluble amyloid protein deposition in the liver. The disease often presents with vague, nonspecific clinical features. Currently, there is little literature describing treatment outcomes for biopsy-proven hepatic amyloidosis and current treatment guidelines recommend that patients enroll in a clinical trial due to insufficient evidence to suggest an optimal treatment regimen. Here, we present two cases of hepatic amyloidosis at an academic medical center and describe their presentation, treatment, and outcomes...
2016: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/27712634/systemic-immunoglobulin-light-chain-amyloidosis-associated-myopathy-presentation-diagnostic-pitfalls-and-outcome
#5
Eli Muchtar, Daniele Derudas, Michelle Mauermann, Teerin Liewluck, Angela Dispenzieri, Shaji K Kumar, David Dingli, Martha Q Lacy, Francis K Buadi, Suzanne R Hayman, Prashant Kapoor, Nelson Leung, Rajshekhar Chakraborty, Wilson Gonsalves, Stephen Russell, John A Lust, Yi Lin, Ronald S Go, Steven Zeldenrust, Robert A Kyle, S Vincent Rajkumar, Morie A Gertz
OBJECTIVE: To characterize the natural history of immunoglobulin light chain amyloidosis-associated myopathy and to provide guidelines for recognition. PATIENTS AND METHODS: Fifty-one patients with systemic immunoglobulin light chain amyloidosis and biopsy-confirmed muscle amyloid deposition diagnosed between January 1, 1995, and December 31, 2015, were included in this study. RESULTS: Common presenting symptoms were muscle weakness in 49 patients (96%), dysphagia in 23 (45%), myalgia in 17 (33%), macroglossia in 17 (33%), jaw claudication in 13 (25%), and hoarseness in 9 (18%)...
October 2016: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/27358783/long-term-outcomes-of-cardiac-transplant-for-immunoglobulin-light-chain-amyloidosis-the-mayo-clinic-experience
#6
Martha Grogan, Morie Gertz, Arleigh McCurdy, Lindsey Roeker, Robert Kyle, Sudhir Kushwaha, Richard Daly, Joseph Dearani, Richard Rodeheffer, Robert Frantz, Martha Lacy, Suzanne Hayman, Christopher McGregor, Brooks Edwards, Angela Dispenzieri
AIM: To determine the outcome of orthotopic heart transplantation (OHT) in immunoglobulin light chain (AL) amyloidosis. METHODS: The medical records of patients with AL who underwent orthotopic heart transplantation at the Mayo Clinic in Rochester Minnesota from 1992 to 2011 were reviewed. Patients met at least one of the following at: New York Heart Association class IV heart failure, ventricular thickness > 15 mm, ejection fraction < 40%. Selection guidelines for heart transplant included age < 60 years, absence of multiple myeloma and significant extra-cardiac organ involvement...
June 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/27156471/amyloid-pet-imaging-mci-and-ad
#7
Vladimir Kepe
PET with "β-amyloid-specific" molecular imaging probes is proposed for the measurement of brain β-amyloid protein amyloidosis in the new guidelines for diagnosis of Alzheimer disease (AD) at different levels of disease progression. This article discusses limitations of this proposed use pointing to unresolved issues and inconsistencies between PET scan results and correlation with other biomarkers, and with postmortem histopathological studies. These unresolved issues do not warrant the conclusion that PET imaging with "β-amyloid-specific" molecular imaging probes can be used as a biomarker in AD or in the various stages of disease progression...
October 2013: PET Clinics
https://www.readbyqxmd.com/read/26894382/smoldering-multiple-myeloma-emerging-concepts-and-therapeutics
#8
REVIEW
Srinath Sundararajan, Abhijeet Kumar, Neha Korde, Amit Agarwal
Smoldering multiple myeloma (SMM) is a pre-malignant condition with an inherent risk for progression to multiple myeloma (MM). The 2014 IMWG guidelines define smoldering multiple myeloma as a monoclonal gammopathy disorder with serum monoclonal protein (IgG or IgA) ≥30 g/L or urinary monoclonal protein ≥500 mg per 24 h and/or clonal bone marrow plasma cells 10-60 % without any myeloma-defining events or amyloidosis. The risk for progression of SMM to MM vary based on clinical, laboratory, imaging, and molecular characteristics...
April 2016: Current Hematologic Malignancy Reports
https://www.readbyqxmd.com/read/26557519/bacteriological-quality-of-treated-water-and-dialysate-in-haemodialysis-unit-of-a-tertiary-care-hospital
#9
Shiva Verma, V A Indumathi, K C Gurudev, Shalini Ashok Naik
INTRODUCTION: Haemodialysis is one of the treatment modalities for patients suffering from end stage renal disease (ESRD). Dialysis patients are exposed to large volumes of water for production of dialysis fluids. Treated water and dialysate come in direct contact with the patient's bloodstream. Such patients suffer from abnormalities of the immune system, making them more susceptible to infections. Microbial contamination of the treated water and dialysate can lead to biofilm formation and release of endotoxins in Haemodialysis system...
October 2015: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/26528443/pediatric-renal-biopsies-in-india-a-single-centre-experience-of-six-years
#10
Kamal V Kanodia, Aruna V Vanikar, Lovelesh K Nigam, Rashmi D Patel, Kamlesh S Suthar, Dinesh N Gera, Hargovind L Trivedi
BACKGROUND: Renal biopsy is a well-established diagnostic modality for the assessment of kidney diseases in children. It can provide diagnostic precision and prognostic value and guide in therapeutic options for many renal diseases. OBJECTIVES: This report describes the indication, histopathological patterns, and epidemiology of renal diseases in children in India. PATIENTS AND METHODS: This is a single-center study on renal biopsies performed between January 2008 and December 2013 in 346 children (age ≤ 14 years)...
July 2015: Nephro-urology Monthly
https://www.readbyqxmd.com/read/26498847/guidelines-for-the-management-and-treatment-of-periodic-fever-syndromes-familial-mediterranean-fever
#11
Maria Teresa R A Terreri, Wanderley Marques Bernardo, Claudio Arnaldo Len, Clovis Artur Almeida da Silva, Cristina Medeiros Ribeiro de Magalhães, Silvana B Sacchetti, Virgínia Paes Leme Ferriani, Daniela Gerent Petry Piotto, André de Souza Cavalcanti, Ana Júlia Pantoja de Moraes, Flavio Roberto Sztajnbok, Sheila Knupp Feitosa de Oliveira, Lucia Maria Arruda Campos, Marcia Bandeira, Flávia Patricia Sena Teixeira Santos, Claudia Saad Magalhães
OBJECTIVE: To establish guidelines based on scientific evidence for the management of familial Mediterranean fever. DESCRIPTION OF THE EVIDENCE COLLECTION METHOD: The Guideline was prepared from 5 clinical questions that were structured through PICO (Patient, Intervention or indicator, Comparison and Outcome), to search in key primary scientific information databases. After defining the potential studies to support the recommendations, these were graduated considering their strength of evidence and grade of recommendation...
October 9, 2015: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/26023828/acute-kidney-injury-kdigo-stage-2-to-3-in-hiv-positive-patients-treated-with-cart-a-case-series-over-11-years-in-a-cohort-of-1-153-patients
#12
Mario Kurz, Felix Burkhalter, Michael Dickenmann, Helmut Hopfer, Michael Mayr, Luigia Elzi, Manuel Battegay
OBJECTIVES: We aimed to explore acute kidney injury (AKI) Kidney Disease Improving Global Guidelines (KDIGO) stage 2 to 3 in a cohort of antiretroviral treated HIV-infected individuals. METHODS: HIV-infected individuals of the Swiss HIV Cohort Study (Basel site), treated with combination antiretroviral therapy (cART) 2002-2013, were included. AKI was defined and classified according to the KDIGO Clinical Practice Guidelines for AKI. Data were prospectively collected and reports of kidney biopsies obtained from records...
2015: Swiss Medical Weekly
https://www.readbyqxmd.com/read/25695244/alect2-amyloidosis-a-new-type-of-systemic-amyloid-highly-prevalent-in-the-hispanic-population
#13
Víctor H Jiménez-Zepeda, Nelson Leung
Amyloidosis results from extracellular deposition of fibril-forming proteins and currently ~30 different proteins have been found to be amyloidogenic. Recently, a novel type of amyloidosis with a high incidence on Hispanic population has been described to be derived from leukocyte chemotactic factor 2 (ALECT2). The objective of the present article is to raise awareness on the presence of this entity for the medical community in México. ALECT2 is a clinical entity characterized by deposition of the LECT2 protein mainly on liver and kidney...
May 2014: Revista de Investigación Clínica; Organo del Hospital de Enfermedades de la Nutrición
https://www.readbyqxmd.com/read/25628446/evidence-based-recommendations-for-genetic-diagnosis-of-familial-mediterranean-fever
#14
REVIEW
Gabriella Giancane, Nienke M Ter Haar, Nico Wulffraat, Sebastiaan J Vastert, Karyl Barron, Veronique Hentgen, Tilmann Kallinich, Huri Ozdogan, Jordi Anton, Paul Brogan, Luca Cantarini, Joost Frenkel, Caroline Galeotti, Marco Gattorno, Gilles Grateau, Michael Hofer, Isabelle Kone-Paut, Jasmin Kuemmerle-Deschner, Helen J Lachmann, Anna Simon, Erkan Demirkaya, Brian Feldman, Yosef Uziel, Seza Ozen
Familial Mediterranean fever (FMF) is a disease of early onset which can lead to significant morbidity. In 2012, Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched with the aim of optimising and disseminating diagnostic and management regimens for children and young adults with rheumatic diseases. The objective was to establish recommendations for FMF focusing on provision of diagnostic tools for inexperienced clinicians particularly regarding interpretation of MEFV mutations...
April 2015: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/25543267/consensus-statement-on-a-framework-for-the-management-of-comorbidity-and-extra-articular-manifestations-in-rheumatoid-arthritis
#15
REVIEW
Estíbaliz Loza, Cristina Lajas, Jose Luis Andreu, Alejandro Balsa, Isidoro González-Álvaro, Oscar Illera, Juan Ángel Jover, Isabel Mateo, Javier Orte, Javier Rivera, José Manuel Rodríguez Heredia, Fredeswinda Romero, Juan Antonio Martínez-López, Ana María Ortiz, Esther Toledano, Virginia Villaverde, Loreto Carmona, Santos Castañeda
The objective of the study was to develop evidence-based and practical recommendations for the detection and management of comorbidity in patients with rheumatoid arthritis (RA) in daily practice. We used a modified RAND/UCLA methodology and systematic review (SR). The process map and specific recommendations, based on the SR, were established in discussion groups. A two round Delphi survey permitted (1) to prioritize the recommendations, (2) to refine them, and (3) to evaluate their agreement by a large group of users...
March 2015: Rheumatology International
https://www.readbyqxmd.com/read/25535224/life-saving-implantable-cardioverter-defibrillator-therapy-in-cardiac-al-amyloidosis
#16
Ketna S Patel, Philip N Hawkins, Carol J Whelan, Julian D Gillmore
Cardiac involvement is the main determinant of prognosis in systemic monoclonal immunoglobulin light chain (AL) amyloidosis. Ventricular arrhythmias and sudden cardiac death are not uncommon. The electrical events that precede sudden death, and their potential to be treated effectively, remain undefined. There are no European guidelines for the use of implantable cardioverter defibrillator (ICD) in amyloidosis. ICDs in general are not usually offered to patients with a life expectancy of less than 1 year. We describe a patient who presented with cardiac AL amyloidosis who underwent prophylactic ICD implantation for the prevention of sudden cardiac death during treatment with chemotherapy, in whom life-threatening ventricular arrhythmia was successfully terminated over a 3-year period...
December 22, 2014: BMJ Case Reports
https://www.readbyqxmd.com/read/25312307/guidelines-on-the-diagnosis-and-investigation-of-al-amyloidosis
#17
Julian D Gillmore, Ashutosh Wechalekar, Jenny Bird, Jamie Cavenagh, Stephen Hawkins, Majid Kazmi, Helen J Lachmann, Philip N Hawkins, Guy Pratt
No abstract text is available yet for this article.
January 2015: British Journal of Haematology
https://www.readbyqxmd.com/read/25303672/guidelines-on-the-management-of-al-amyloidosis
#18
Ashutosh D Wechalekar, Julian D Gillmore, Jenny Bird, Jamie Cavenagh, Stephen Hawkins, Majid Kazmi, Helen J Lachmann, Philip N Hawkins, Guy Pratt
No abstract text is available yet for this article.
January 2015: British Journal of Haematology
https://www.readbyqxmd.com/read/25169210/management-of-systemic-al-amyloidosis-recommendations-of-the-myeloma-foundation-of-australia-medical-and-scientific-advisory-group
#19
REVIEW
N Weber, P Mollee, B Augustson, R Brown, L Catley, J Gibson, S Harrison, P J Ho, N Horvath, W Jaksic, D Joshua, H Quach, A W Roberts, A Spencer, J Szer, D Talaulikar, B To, A Zannettino, H M Prince
Systemic AL amyloidosis is a plasma cell dyscrasia with a characteristic clinical phenotype caused by multi-organ deposition of an amyloidogenic monoclonal protein. This condition poses a unique management challenge due to the complexity of the clinical presentation and the narrow therapeutic window of available therapies. Improved appreciation of the need for risk stratification, standardised use of sensitive laboratory testing for monitoring disease response, vigilant supportive care and the availability of newer agents with more favourable toxicity profiles have contributed to the improvement in treatment-related mortality and overall survival seen over the past decade...
April 2015: Internal Medicine Journal
https://www.readbyqxmd.com/read/24905633/acr-appropriateness-criteria%C3%A2-nonischemic-myocardial-disease-with-clinical-manifestations-ischemic-cardiomyopathy-already-excluded
#20
Leena Mammen, Pamela K Woodard, Suhny Abbara, Sharmila Dorbala, Cylen Javidan-Nejad, Paul R Julsrud, Jacobo Kirsch, Christopher M Kramer, Rajesh Krishnamurthy, Archana T Laroia, Amar B Shah, Jens Vogel-Claussen, Richard D White
Nonischemic myocardial disease or cardiomyopathy can present as arrhythmia, palpitations, heart failure, dyspnea, lower extremity edema, ascites, syncope, and/or chest discomfort and can be classified as either systolic, diastolic, or a combination of both. Echocardiography is the mainstay of evaluating left ventricular function. However, cardiac magnetic resonance imaging (MRI) is now considered the reference standard imaging technique to assess myocardial anatomy, function, and viability. Advanced MRI techniques with delayed myocardial enhancement, especially, can provide information beyond echocardiography for tissue characterization in CM and can assist in determining specific etiology or in narrowing the differential...
July 2014: Journal of Thoracic Imaging
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