145 results match your criteria Amyloidosis Overview


Inhibition of amyloid fibrillation by small molecules and nanomaterials: Strategic development of pharmaceuticals against amyloidosis.

Protein Pept Lett 2019 Mar 7. Epub 2019 Mar 7.

Department of Chemistry, National Institute of Technology Hamirpur, Himachal Pradesh 177005. India.

Amyloid fibrils are a special class of self assembled protein molecules, which exhibit various toxic effects in cell. Different physiological disorders such as Alzheimer's, Parkinson's, Huntington's diseases etc. happen due to the lack of proper cellular mechanism for the removal of fibrils. Read More

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http://dx.doi.org/10.2174/0929866526666190307164944DOI Listing
March 2019
1 Read

The Case of Amyloidosis in Cardiological Practice.

Kardiologiia 2019 Jan 28;59(1):93-96. Epub 2019 Jan 28.

Kazan State Medical University.

In this article we present brief overview of the subject of amyloidosis and involvement of the cardiovascular system, the criteria for diagnosis, principles of treatment, and the clinical case of cardiac amyloidosis. Read More

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https://lib.ossn.ru/jour/article/view/437
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http://dx.doi.org/10.18087/cardio.2019.1.10221DOI Listing
January 2019
4 Reads

Imaging cardiac amyloidosis: Patient page.

J Nucl Cardiol 2019 Feb 4;26(1):217-221. Epub 2018 Dec 4.

Cardiac Amyloidosis Program, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, 02115, USA.

Amyloidosis is a disease that develops when abnormally folded proteins collect together to form amyloid fibrils, which deposit in various organs and cause organ damage. Amyloidosis in the heart can be identified by using various imaging tests. This patient page provides an overview of imaging in cardiac amyloidosis. Read More

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http://link.springer.com/10.1007/s12350-018-01551-5
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http://dx.doi.org/10.1007/s12350-018-01551-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6374189PMC
February 2019
19 Reads

Assays for Light Chain Amyloidosis Formation and Cytotoxicity.

Methods Mol Biol 2019 ;1873:123-153

Department of Biochemistry and Molecular Biology, Mayo Clinic, Rochester, MN, USA.

Common biophysical techniques like absorption and fluorescence spectroscopy, microscopy, and light scattering studies have been in use to investigate fibril assembly for a long time. However, there is sometimes a lack of consensus from the findings of an individual technique when compared in parallel with the other techniques. In this chapter, we aim to provide a concise compilation of techniques that can effectively be used to obtain a comprehensive representation of the structural, aggregation, and toxicity determinants in immunoglobulin light chain amyloidosis. Read More

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http://dx.doi.org/10.1007/978-1-4939-8820-4_8DOI Listing
January 2019
2 Reads

Waldenström macroglobulinemia: 2019 update on diagnosis, risk stratification, and management.

Authors:
Morie A Gertz

Am J Hematol 2019 Feb 17;94(2):266-276. Epub 2018 Oct 17.

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.

Diagnosis: Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. Read More

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http://doi.wiley.com/10.1002/ajh.25292
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http://dx.doi.org/10.1002/ajh.25292DOI Listing
February 2019
15 Reads

Cerebrovascular disorders associated with genetic lesions.

Cell Mol Life Sci 2019 Jan 16;76(2):283-300. Epub 2018 Oct 16.

Departments of Neurosurgery and Neuroscience, Program on Neurogenetics, Yale School of Medicine, P.O. Box 208082, New Haven, CT, 06520-8082, USA.

Cerebrovascular disorders are underlain by perturbations in cerebral blood flow and abnormalities in blood vessel structure. Here, we provide an overview of the current knowledge of select cerebrovascular disorders that are associated with genetic lesions and connect genomic findings with analyses aiming to elucidate the cellular and molecular mechanisms of disease pathogenesis. We argue that a mechanistic understanding of genetic (familial) forms of cerebrovascular disease is a prerequisite for the development of rational therapeutic approaches, and has wider implications for treatment of sporadic (non-familial) forms, which are usually more common. Read More

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http://dx.doi.org/10.1007/s00018-018-2934-5DOI Listing
January 2019
2 Reads

Histopathology of salivary glands.

Reumatismo 2018 Oct 3;70(3):146-154. Epub 2018 Oct 3.

Department of Medicine, ASL1 Avezzano Sulmona L'Aquila; Rheumatology Unit, Department of Biotechnological and Applied Clinical Science, University of L'Aquila.

Salivary gland (SG) biopsy is a technique broadly applied for the diagnosis of primary Sjögren's syndrome (pSS), lymphoma accompanying SS, sarcoidosis, amyloidosis, and IgG4-related disease The most peculiar feature of pSS on biopsy is focal lymphocytic sialadenitis. In the past, several histological scores have been reported in the literature to describe glandular involvement during pSS. However, the variability among centres in reporting glandular scores is one of the rationales behind the development of standardised consensus guidance. Read More

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https://reumatismo.org/index.php/reuma/article/view/1053
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http://dx.doi.org/10.4081/reumatismo.2018.1053DOI Listing
October 2018
8 Reads

Magnetic Nanoparticles Applications for Amyloidosis Study and Detection: A Review.

Nanomaterials (Basel) 2018 Sep 18;8(9). Epub 2018 Sep 18.

Laboratoire de Chimie et Biologie des Métaux, Université Grenoble Alpes, CNRS, CEA, 17 Rue des Martyrs, CEDEX 9, 38041 Grenoble, France.

Magnetic nanoparticles (MNPs) have great potential in biomedical and clinical applications because of their many unique properties. This contribution provides an overview of the MNPs mainly used in the field of amyloid diseases. The first part discusses their use in understanding the amyloid mechanisms of fibrillation, with emphasis on their ability to control aggregation of amyloidogenic proteins. Read More

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http://dx.doi.org/10.3390/nano8090740DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6164038PMC
September 2018
10 Reads

Targeting Amyloid Aggregation: An Overview of Strategies and Mechanisms.

Int J Mol Sci 2018 Sep 9;19(9). Epub 2018 Sep 9.

Centre for Misfolding Diseases, Department of Chemistry, University of Cambridge, Cambridge CB2 1EW, UK.

Amyloids result from the aggregation of a set of diverse proteins, due to either specific mutations or promoting intra- or extra-cellular conditions. Structurally, they are rich in intermolecular β-sheets and are the causative agents of several diseases, both neurodegenerative and systemic. It is believed that the most toxic species are small aggregates, referred to as oligomers, rather than the final fibrillar assemblies. Read More

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http://www.mdpi.com/1422-0067/19/9/2677
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http://dx.doi.org/10.3390/ijms19092677DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6164555PMC
September 2018
19 Reads

[Policy paper nuclear cardiology - update 2018 - Current status of clinical practice].

Nuklearmedizin 2018 Aug 24;57(4):146-152. Epub 2018 Jul 24.

The joint position paper of the working community "Cardiovascular Nuclear Medicine" of the German Society of Nuclear Medicine (DGN) and the working group "Nuclear Cardiology Diagnostics" of the German Cardiac Society (DKG) updates the former 2009 paper. It is the purpose of this paper to provide an overview about the application fields, the state-of-the-art and the current value of nuclear cardiology imaging. The topics covered are chronic coronary artery disease, including viability imaging, furthermore cardiomyopathies, infective endocarditis, cardiac sarcoidosis and amyloidosis. Read More

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http://dx.doi.org/10.3413/2018-06-0001DOI Listing
August 2018
6 Reads
1.671 Impact Factor

Immunoglobulin light chain amyloidosis: 2018 Update on diagnosis, prognosis, and treatment.

Authors:
Morie A Gertz

Am J Hematol 2018 Sep;93(9):1169-1180

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light or heavy chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic dysfunction, peripheral/autonomic neuropathy, and "atypical multiple myeloma."

Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple-green birefringence is required for diagnosis. Read More

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http://dx.doi.org/10.1002/ajh.25149DOI Listing
September 2018
38 Reads

Clinical application and technical considerations of T & T(*) mapping in cardiac, liver, and renal imaging.

Br J Radiol 2018 Dec 23;91(1092):20170825. Epub 2018 Jul 23.

1 Department of Radiology, Leiden University Medical Center , Leiden , The Netherlands.

Pathological tissue alterations due to disease processes such as fibrosis, edema and infiltrative disease can be non-invasively visualized and quantified by MRI using T and T relaxation properties. Pixel-wise mapping of T and T image sequences enable direct quantification of T, T(*), and extracellular volume values of the target organ of interest. Tissue characterization based on T and T(*) mapping is currently making the transition from a research tool to a clinical modality, as clinical usefulness has been established for several diseases such as myocarditis, amyloidosis, Anderson-Fabry and iron deposition. Read More

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https://www.birpublications.org/doi/10.1259/bjr.20170825
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http://dx.doi.org/10.1259/bjr.20170825DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6319842PMC
December 2018
22 Reads

Causes of mortality and morbidity in free-ranging mustelids in Switzerland: necropsy data from over 50 years of general health surveillance.

BMC Vet Res 2018 Jun 19;14(1):195. Epub 2018 Jun 19.

Centre for Fish and Wildlife Health (FIWI), Vetsuisse Faculty, University of Bern, Länggassstrasse 122, Postfach, 3001, Bern, Switzerland.

Background: Although mustelids occur worldwide and include a wide range of species, little is known about the diseases affecting them. Mustelids have regularly been submitted for post mortem investigation in the framework of the program for general wildlife health surveillance in Switzerland, which has been in place for nearly 60 years. We performed a retrospective analysis of the necropsy reports on mustelids submitted to the diagnostic service of the University of Bern. Read More

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http://dx.doi.org/10.1186/s12917-018-1494-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6009050PMC
June 2018
16 Reads

Current Challenges in Delivery and Cytosolic Translocation of Therapeutic RNAs.

Nucleic Acid Ther 2018 06;28(3):178-193

Institut Curie, PSL Research University , Cellular and Chemical Biology, U1143 INSERM, UMR3666 CNRS, Paris, France .

RNA interference (RNAi) is a fundamental cellular process for the posttranscriptional regulation of gene expression. RNAi can exogenously be modulated by small RNA oligonucleotides, such as microRNAs (miRNAs) and small interfering RNAs (siRNAs), or by antisense oligonucleotides. These small oligonucleotides provided the scientific community with powerful and versatile tools to turn off the expression of genes of interest, and hold out the promise of new therapeutic solutions against a wide range of gene-associated pathologies. Read More

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http://dx.doi.org/10.1089/nat.2017.0716DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6000866PMC
June 2018
5 Reads

Updated overview of molecular pathways involved in the most common monogenic autoinflammatory diseases.

Clin Exp Rheumatol 2018 Jan-Feb;36 Suppl 110(1):3-9. Epub 2018 May 3.

Research Centre of Systemic Autoinflammatory Diseases, Behçet's Disease Clinic and Rheumatology-Ophthalmology Collaborative Uveitis Centre, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.

An apparently unprovoked recurrent inflammation is the quintessential hallmark of autoinflammatory diseases (AIDs), a large and heterogeneous group of disorders in which there is poor regulation of the innate immune system with no clearly demonstrated autoimmune machinery involvement. Innate immunity pathways are diverse and our understanding of their molecular composition and function is continuously expanding. The impaired immune responses we observe in monogenic AIDs, mostly in the hereditary periodic fever syndromes, is officiated by target molecules of microbial origin (pathogen-associated molecular patterns) and also host molecules (danger-associated molecular patterns). Read More

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July 2018
10 Reads

The prevention of hepatitis C virus transmission to hemodialysis patients and staff members.

Authors:
Michel Jadoul

Hemodial Int 2018 04;22 Suppl 1:S104-S109

Department of Nephrology, Cliniques universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium.

In this review article, we cover 4 topics very relevant to those involved in the care of hemodialysis (HD) patients and in the management of a HD unit. We start with an overview of the progress made over the last 25 years to reduce hepatitis C virus (HCV) transmission in HD. Next, we discuss the potential strategies to prevent the residual ongoing transmission of HCV to HD patients. Read More

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http://dx.doi.org/10.1111/hdi.12652DOI Listing
April 2018
5 Reads

Advances in Treatment of Cardiac Amyloid.

Curr Treat Options Cardiovasc Med 2018 Apr 7;20(5):37. Epub 2018 Apr 7.

Cardiovascular Division, Cardio-Oncology Center of Excellence, Washington University in St Louis, 660 South Euclid Avenue, Campus Box 8086, St Louis, MO, 63110, USA.

Systemic amyloidosis represents a complex group of diseases with a common feature characterized by misfolded autologous proteins depositing into tissues or organs throughout the body. Light chain amyloidosis (AL) and transthyretin (TTR) amyloid are the two most prevalent forms of this disease that commonly results in cardiac amyloidosis. In both of these conditions, the myocardium is a frequent site of infiltration and end-organ involvement often with devastating consequences. Read More

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http://dx.doi.org/10.1007/s11936-018-0631-1DOI Listing
April 2018
7 Reads

Genetic Infiltrative Cardiomyopathies.

Heart Fail Clin 2018 Apr;14(2):215-224

Adult Medical Genetics Program, Cardiovascular Institute, University of Colorado Anschutz, 12700 East 19th Avenue, Aurora, CO 80045, USA. Electronic address:

Infiltrative cardiomyopathies are characterized by abnormal accumulation or deposition of substances in cardiac tissue leading to cardiac dysfunction. These can be inherited, resulting from mutations in specific genes, which engender a diverse array of extracardiac features but overlapping cardiac phenotypes. This article provides an overview of each inherited infiltrative cardiomyopathy, describing the causative genes, the pathologic mechanisms involved, the resulting cardiac manifestations, and the therapies currently offered or being developed. Read More

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http://dx.doi.org/10.1016/j.hfc.2017.12.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5894511PMC
April 2018
9 Reads

New concepts in the treatment and diagnosis of amyloidosis.

Expert Rev Hematol 2018 02 10;11(2):117-127. Epub 2018 Jan 10.

a Amyloidosis Research and Treatment Center, Foundation 'Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Policlinico San Matteo', and Department of Molecular Medicine , University of Pavia , Pavia , Italy.

Introduction: The most common form of systemic amyloidosis in Western countries is light chain amyloidosis. It is characterized by the deposition of a misfolded light chain in target organs. This amyloid precursor is produced by a usually small but dangerous B-cell clone. Read More

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http://dx.doi.org/10.1080/17474086.2018.1424534DOI Listing
February 2018
14 Reads

Unfolding Cardiac Amyloidosis - From Pathophysiology to Cure.

Curr Med Chem 2018 Jan 4. Epub 2018 Jan 4.

Division of Cardiology, Medical University of Graz, Graz. Austria.

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. Read More

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http://dx.doi.org/10.2174/0929867325666180104153338DOI Listing
January 2018
11 Reads

Cardiovascular complications of multiple myeloma in the elderly.

Expert Rev Cardiovasc Ther 2017 Dec 27;15(12):933-943. Epub 2017 Nov 27.

b Department of Cardiovascular Medicine , Department of Medicine University of Arkansas Medical Sciences , Little Rock , AR , USA.

Introduction: Multiple myeloma is a malignant neoplasm characterized by plasma cell proliferation in blood and bone marrow. Approximately two-thirds of the patients with multiple myeloma are >65 years at the time of diagnosis. Patients in this age group often have co-existing cardiovascular diseases. Read More

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http://dx.doi.org/10.1080/14779072.2017.1409114DOI Listing
December 2017
14 Reads

Recent advances in diagnosis and treatment of cardiac amyloidosis.

J Cardiol 2018 Feb 5;71(2):135-143. Epub 2017 Nov 5.

Department of Cardiovascular Medicine, Faculty of Life Sciences, Kumamoto University, Kumamoto, Japan.

Cardiac amyloidosis (CA) has been believed to be a rare disease for a long time, but recent sophisticated diagnostic modalities demonstrate that a considerable number of CA patients are hidden among those diagnosed with heart failure. Prognosis of CA was poor, but recent developments in therapeutic interventions have improved survival in these patients. Therefore, early detection and precise diagnosis is clinically important. Read More

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http://dx.doi.org/10.1016/j.jjcc.2017.10.003DOI Listing
February 2018
6 Reads

Common molecular pathogenesis of disease-related intrinsically disordered proteins revealed by NMR analysis.

J Biochem 2018 Jan;163(1):11-18

Laboratory of Structural and Molecular Pharmacology, Graduate School of Pharmaceutical Sciences, Nagoya University, Aichi 464-8601, Japan.

Intrinsically disordered proteins (IDPs) are either completely unstructured or contain large disordered regions in their native state; they have drawn much attention in the field of molecular pathology. Some of them substantially tend to form protein self-assemblies, such as toxic or non-toxic aggregates and fibrils, and have been postulated to relate to diseases. These disease-related IDPs include Aβ(1-42) [Alzheimer's disease (AD)], Tau (AD and tauopathy), α-synuclein (Parkinson's disease) and p53 (cancer). Read More

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http://dx.doi.org/10.1093/jb/mvx056DOI Listing
January 2018
12 Reads

Tumor necrosis factor-associated periodic syndrome in adults.

Rheumatol Int 2018 Jan 23;38(1):3-11. Epub 2017 Sep 23.

NYU Langone Medical Center, New York, NY, USA.

Tumor necrosis factor-associated periodic syndrome is an autoinflammatory disorder classified under hereditary periodic fever syndromes. Mutations in the tumor necrosis factor receptor contribute to tumor necrosis factor-associated periodic syndrome. Decreased shedding of receptors and increased mitochondrial reactive oxygen species production leading to elevated proinflammatory cytokines are documented. Read More

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http://dx.doi.org/10.1007/s00296-017-3820-4DOI Listing
January 2018
7 Reads

Restrictive Cardiomyopathy: Genetics, Pathogenesis, Clinical Manifestations, Diagnosis, and Therapy.

Circ Res 2017 Sep;121(7):819-837

From the Division of Hematology (E.M., M.A.G.) and Department of Cardiovascular Medicine (L.A.B.), Mayo Clinic, Rochester, MN.

Restrictive cardiomyopathy (RCM) is characterized by nondilated left or right ventricle with diastolic dysfunction. The restrictive cardiomyopathies are a heterogenous group of myocardial diseases that vary according to pathogenesis, clinical presentation, diagnostic evaluation and criteria, treatment, and prognosis. In this review, an overview of RCMs will be presented followed by a detailed discussion on 3 major causes of RCM, for which tailored interventions are available: cardiac amyloidosis, cardiac sarcoidosis, and cardiac hemochromatosis. Read More

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http://dx.doi.org/10.1161/CIRCRESAHA.117.310982DOI Listing
September 2017
6 Reads

PERK as a hub of multiple pathogenic pathways leading to memory deficits and neurodegeneration in Alzheimer's disease.

Authors:
Masuo Ohno

Brain Res Bull 2018 07 10;141:72-78. Epub 2017 Aug 10.

Center for Dementia Research, Nathan Kline Institute, Orangeburg, NY, USA; Department of Psychiatry, New York University Langone Medical Center, New York, NY, USA. Electronic address:

Cell signaling in response to an array of diverse stress stimuli converges on the phosphorylation of eukaryotic initiation factor-2α (eIF2α). In the brain, eIF2α is a hub for controlling learning and memory function and for maintaining neuronal integrity in health and disease. Among four eIF2α kinases, PERK is emerging as a key regulator for memory impairments and neurodegeneration in Alzheimer's disease (AD). Read More

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http://dx.doi.org/10.1016/j.brainresbull.2017.08.007DOI Listing
July 2018
22 Reads

Tau imaging with PET: an overview of challenges, current progress, and future applications.

Q J Nucl Med Mol Imaging 2017 Dec 27;61(4):405-413. Epub 2017 Jul 27.

The Florey Institute of Neuroscience and Mental Health, The University of Melbourne, Melbourne, Australia -

Folded and misfolded tau is common to many neurodegenerative conditions, collectively termed "tauopathies". In recent years, many efforts have contributed toward development of tau imaging agents to allow measurement of tau deposits in vivo using positron emission tomography (PET). The particularities of tau present some unique challenges for the development of tau imaging tracers. Read More

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http://dx.doi.org/10.23736/S1824-4785.17.03012-6DOI Listing
December 2017
10 Reads

Mining databases for protein aggregation: a review.

Amyloid 2017 Sep 18;24(3):143-152. Epub 2017 Jul 18.

a Section of Cell Biology and Biophysics, Department of Biology, School of Sciences , National and Kapodistrian University of Athens , Athens , Greece.

Protein aggregation is an active area of research in recent decades, since it is the most common and troubling indication of protein instability. Understanding the mechanisms governing protein aggregation and amyloidogenesis is a key component to the aetiology and pathogenesis of many devastating disorders, including Alzheimer's disease or type 2 diabetes. Protein aggregation data are currently found "scattered" in an increasing number of repositories, since advances in computational biology greatly influence this field of research. Read More

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https://www.tandfonline.com/doi/full/10.1080/13506129.2017.1
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http://dx.doi.org/10.1080/13506129.2017.1353966DOI Listing
September 2017
16 Reads

Design and Rationale of the Phase 3 ATTR-ACT Clinical Trial (Tafamidis in Transthyretin Cardiomyopathy Clinical Trial).

Circ Heart Fail 2017 Jun;10(6)

From the Center for Advanced Cardiac Care, Columbia University College of Physicians and Surgeons, NY (M.S.M); Cardiovascular Medicine, University College London, United Kingdom (P.E.); IRCCS Policlinico San Matteo, University of Pavia, Italy (G.M.); Division of Cardiology, Northwestern University, Chicago, IL (S.J.S.); National Amyloidosis Referral Center, Centro de Estudos em Paramiloidose Antônio Rodrigues de Mello, Federal University of Rio de Janeiro, Brazil (M.W.C.); Pfizer, Inc, Collegeville, PA (A.F., C.H.); inVentiv Health, Burlington, MA (B.G.); Pfizer, Inc, Groton, CT (S.R., J.S.); Pfizer, Inc, NY (M.B.S.); and Department of Experimental, Diagnostic and Specialty Medicine, University of Bologna, Italy (C.R.).

Transthyretin amyloidosis is a rare, life-threatening disease resulting from aggregation and deposition of transthyretin amyloid fibrils in various tissues. There are 2 predominate phenotypic presentations of the disease: transthyretin familial amyloid polyneuropathy, which primarily affects the peripheral nerves, and transthyretin cardiomyopathy (TTR-CM), which primarily affects the heart. However, there is a wide overlap with symptoms at presentation and disease course being highly variable and influenced by the underlying transthyretin mutation, age of the affected individual, sex, and geographic location. Read More

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http://dx.doi.org/10.1161/CIRCHEARTFAILURE.116.003815DOI Listing
June 2017
56 Reads

Role of Imaging in Evaluating Infiltrative Heart Disease.

Curr Treat Options Cardiovasc Med 2017 Jan;19(1)

Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women's Hospital, 75 Francis Street, Boston, MA, 02115, USA.

Opinion Statement: Infiltrative heart disease is caused by the deposition of abnormal substances in the heart and can lead to abnormalities in cardiac function and electrical conduction. Advances in non-invasive cardiovascular imaging have allowed for improved diagnosis of infiltrative heart disease, as well as ways to track disease progression or regression, thus enabling a mechanism to follow response to therapy. In this review, we provide an overview of the role of imaging in the diagnosis and management of cardiac sarcoidosis (CS) and cardiac amyloidosis (CA), as well as outline a proposed algorithm for using non-invasive cardiovascular imaging for evaluating these conditions. Read More

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http://dx.doi.org/10.1007/s11936-017-0500-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5460755PMC
January 2017
9 Reads

Amyloid cardiomyopathy.

Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2017 Jun 1;161(2):117-127. Epub 2017 Feb 1.

Department of Hemato-Oncology, University Hospital Olomouc, Czech Republic.

Amyloidosis is a heterogeneous group of diseases characterized by the deposition of amyloid. It is caused by extracellular deposition of insoluble fibrils with beta-pleated sheet configuration. The protein misfolding abnormalities result in amyloid fibrils and may manifest as primary, secondary, or familial amyloidosis. Read More

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http://dx.doi.org/10.5507/bp.2017.001DOI Listing
June 2017
19 Reads

[The benefit of magnetic resonance for diagnosing cardiomyopathy and myocarditis].

Vnitr Lek Winter 2016;62(12):976-984

Magnetic resonance is becoming an increasingly used examination in cardiology, since it greatly improves the accuracy of diagnosing of many heart diseases. At present magnetic resonance is the gold standard in assessing the volumes of the heart chambers and the systolic function of both ventricles. The possibility of detecting tissue characteristics to refine the diagnostics of different types of myocardial pathology is of essential importance. Read More

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June 2017
10 Reads

Coexistance of Amyloidosis and Primary Sjögren's Syndrome: An Overview.

Curr Rheumatol Rev 2018 ;14(3):231-238

Deparment of Pathology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran. Vasco de Quiroga 15, Col. Belisario Dominguez Seccion XVI, CP 14080, Mexico City, Mexico.

Background: The association of primary Sjogren´s Syndrome (SS) and amyloidosis has been recognized but scarcely assessed.

Objective: Herein we analyzed the literature regarding this association in order to describe a SS phenotype prone to both conditions.

Methods: PubMed and CINAHL databases were searched from inception until April 2016 for the keywords: Amyloidosis, amyloid, Sjögren's syndrome and their combinations. Read More

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http://dx.doi.org/10.2174/1573397113666170103152537DOI Listing
January 2019
54 Reads

Primarily isolated hepatic involvement of amyloidosis: A case report and overview.

Medicine (Baltimore) 2016 Dec;95(52):e5645

Department of Gastroenterology and Hepatology, Jinling Hospital, Medical School of Nanjing University, Nanjing, Jiangsu Province, China.

Background: Amyloidosis is particularly difficult to diagnose because the signs and symptoms are subtle. Additionally, there are no specific imaging or laboratory tests, except histopathology. Although it is considered to be a systemic disorder, a small portion of cases may be localized. Read More

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http://dx.doi.org/10.1097/MD.0000000000005645DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5207551PMC
December 2016
5 Reads

[Cutaneous manifestations in renal diseases].

Hautarzt 2016 Dec;67(12):960-969

Dermatologische Abteilung, Donauspital, Langobardenstr. 122, 1220, Wien, Österreich.

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. Read More

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http://dx.doi.org/10.1007/s00105-016-3890-3DOI Listing
December 2016
15 Reads

Dermoscopy in General Dermatology: A Practical Overview.

Dermatol Ther (Heidelb) 2016 Dec 9;6(4):471-507. Epub 2016 Sep 9.

Department of Experimental and Clinical Medicine, Institute of Dermatology, University of Udine, Udine, Italy.

Over the last few years, dermoscopy has been shown to be a useful tool in assisting the noninvasive diagnosis of various general dermatological disorders. In this article, we sought to provide an up-to-date practical overview on the use of dermoscopy in general dermatology by analysing the dermoscopic differential diagnosis of relatively common dermatological disorders grouped according to their clinical presentation, i.e. Read More

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http://dx.doi.org/10.1007/s13555-016-0141-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5120630PMC
December 2016
65 Reads

[Sporadic Cerebral Amyloid Angiopathy: An Overview with Clinical Cases].

Fortschr Neurol Psychiatr 2016 Sep 8;84(9):534-41. Epub 2016 Sep 8.

Neurologische Klinik und Poliklinik & Deutsches Schwindel- und Gleichgewichtszentrum DSGZ, Klinikum der Universität München.

Sporadic cerebral amyloid angiopathy (CAA) is a cerebral small vessel disease in the elderly. Neuropathologically, it is characterized by deposition of amyloid-ß (Aß) in the wall of small to medium-sized arteries, capillaries and venules of the cerebral cortex and leptomeninges. Over the last years it was recognized as an important cause of spontaneous intracerebral hemorrhage and cognitive deficits in the elderly. Read More

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http://dx.doi.org/10.1055/s-0042-109537DOI Listing
September 2016
5 Reads

Prion-like disorders and Transmissible Spongiform Encephalopathies: An overview of the mechanistic features that are shared by the various disease-related misfolded proteins.

Biochem Biophys Res Commun 2017 02 30;483(4):1125-1136. Epub 2016 Aug 30.

CIC bioGUNE, Parque Tecnológico de Bizkaia, 48160, Derio, Spain; IKERBASQUE, Basque Foundation for Science, Bilbao, 48011, Bizkaia, Spain. Electronic address:

Prion diseases or Transmissible Spongiform Encephalopathies (TSEs) are a group of fatal neurodegenerative disorders affecting several mammalian species. Its causative agent, disease-associated prion protein (PrP), is a self-propagating β-sheet rich aberrant conformation of the cellular prion protein (PrP) with neurotoxic and aggregation-prone properties, capable of inducing misfolding of PrP molecules. PrP is the major constituent of prions and, most importantly, is the first known example of a protein with infectious attributes. Read More

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http://dx.doi.org/10.1016/j.bbrc.2016.08.166DOI Listing
February 2017
8 Reads

Immunoglobulin light chain amyloidosis: 2016 update on diagnosis, prognosis, and treatment.

Authors:
Morie A Gertz

Am J Hematol 2016 09;91(9):947-56

Division of Hematology, Mayo Clinic, Rochester, Minnesota.

Disease Overview: Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/autonomic neuropathy, and atypical multiple myeloma.

Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with applegreen birefringence is required for diagnosis. Read More

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http://dx.doi.org/10.1002/ajh.24433DOI Listing
September 2016
15 Reads

Paraprotein-Related Kidney Disease: Glomerular Diseases Associated with Paraproteinemias.

Clin J Am Soc Nephrol 2016 12 15;11(12):2260-2272. Epub 2016 Aug 15.

Division of Renal Medicine, Department of Medicine, Brigham and Women's Hospital, Boston, Massachusetts;

Paraproteins are monoclonal Igs that accumulate in blood as a result of abnormal excess production. These circulating proteins cause a diversity of kidney disorders that are increasingly being comanaged by nephrologists. In this review, we discuss paraprotein-related diseases that affect the glomerulus. Read More

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http://dx.doi.org/10.2215/CJN.02980316DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5142064PMC
December 2016
61 Reads

Does AL amyloidosis have a unique genomic profile? Gene expression profiling meta-analysis and literature overview.

Gene 2016 Oct 7;591(2):490-8. Epub 2016 Jun 7.

Department of Haematooncology, Faculty of Medicine, University of Ostrava, Czech Republic; Department of Haematooncology, University Hospital Ostrava, Czech Republic.

Immunoglobulin light chain amyloidosis (ALA) is a plasma cell dyscrasia characterized by deposition of amyloid fibrils in various organs and tissues. The current paper is devoted to clarify if ALA has a unique gene expression profile and to its pathogenetic argumentation. The meta-analysis of ALA patients vs. Read More

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http://dx.doi.org/10.1016/j.gene.2016.06.017DOI Listing
October 2016
24 Reads

Exercise-induced haemoptysis as a rare presentation of a rare lung disease.

Thorax 2016 09 7;71(9):865-8. Epub 2016 Jun 7.

Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute (NHLBI), National Institutes of Health (NIH), Bethesda, Maryland, USA Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, Maryland, USA.

Amyloid primarily affecting the lungs is a seldom seen clinical entity. This case discusses the work-up of a patient presenting with exercise-induced haemoptysis and diffuse cystic lung disease on radiographic imaging. The common clinical and radiographic findings of diffuse cystic lung diseases as well as a brief overview of pulmonary amyloid are presented. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2015-208020DOI Listing
September 2016
10 Reads

Systemic and organ involvement in monogenic autoinflammatory disorders: a global review filtered through internists' lens.

Intern Emerg Med 2016 Sep 25;11(6):781-91. Epub 2016 May 25.

Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.

Monogenic autoinflammatory disorders (AIDs) are rare diseases driven by cytokine-mediated extraordinary sterile inflammation that results from the activation of innate immune pathways. The clinical hallmark of these diseases is the recurrence of stereotyped episodes of systemic- and organ-specific inflammation; the most common systems involved being the skin, musculoskeletal system, gastrointestinal tract, and central nervous system. The autoinflammatory disorders may have a profound impact on the quality of life of the affected patients, and a delayed diagnosis may lead to severe complications, the most dreadful of which is AA-Amyloidosis. Read More

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http://dx.doi.org/10.1007/s11739-016-1466-yDOI Listing
September 2016
34 Reads

Exploring Big Data in Hematological Malignancies: Challenges and Opportunities.

Curr Hematol Malig Rep 2016 08;11(4):271-9

Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Secondary analysis of large datasets has become a useful alternative to address research questions outside the reach of clinical trials. It is increasingly utilized in hematology and oncology. In this review, we provided an overview of some examples of commonly used large datasets in the USA and described common research themes that can be pursued using such a methodology. Read More

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http://dx.doi.org/10.1007/s11899-016-0331-4DOI Listing
August 2016
13 Reads

Measurement of free light chains with assays based on monoclonal antibodies.

Clin Chem Lab Med 2016 Jun;54(6):1005-14

Recently, serum free light chain (FLC) assays incorporating anti-kappa (κ) and anti-lambda (λ) FLC monoclonal antibodies have become available: N Latex FLC assay (Siemens) and Seralite® (Abingdon Health). The purpose of this review is to provide an overview of these two new monoclonal antibody-based methods. In doing so, the review will outline the performance characteristics of each method, including a summary of: assay principles, antibody specificity, analytical performance and assay performance in disease. Read More

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http://dx.doi.org/10.1515/cclm-2015-0963DOI Listing
June 2016
7 Reads

Amyloid A amyloidosis secondary to hyper IgD syndrome and response to IL-1 blockage therapy.

Neth J Med 2016 Jan;74(1):43-6

Department of Nephrology, Leiden University Medical Center, Leiden, the Netherlands.

A 62-year-old woman with a history of genetically confirmed hyperimmunoglobulinaemia D and periodic fever syndrome (HIDS) was admitted because of chronic diarrhoea. During admission she developed a rapidly progressive nephrotic syndrome. Reactive amyloid A (AA) amyloidosis was confirmed after colonic and renal biopsy which showed deposition of amyloid. Read More

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January 2016
15 Reads

Drugs for the treatment of peripheral neuropathies.

Expert Opin Pharmacother 2016 5;17(3):381-94. Epub 2015 Dec 5.

a Experimental Neurology Unit, School of Medicine and Surgery and Milan Center for Neuroscience , University of Milano-Bicocca , Monza , Italy.

Peripheral neuropathies are frequent in association with systemic diseases as well as isolated disorders. Recent advances in the therapy of specific neuropathies led to the approval of new drugs/treatments. This review selected those peripheral neuropathies where the most recent approvals were provided and revised the potential future developments in diabetic and toxic-induced neuropathies, although they do not have a currently available causal therapy in view of their epidemiological and social relevance. Read More

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http://www.tandfonline.com/doi/full/10.1517/14656566.2016.11
Publisher Site
http://dx.doi.org/10.1517/14656566.2016.1120719DOI Listing
July 2016
6 Reads

Flow cytometry in immunoglobulin light chain amyloidosis: Short review.

Leuk Res 2015 Jul 13. Epub 2015 Jul 13.

Department of Haematooncology, University Hospital Ostrava and Faculty of Medicine OU, Ostrava, Czech Republic. Electronic address:

Flow cytometry (FCM) has found its application in clinical diagnosis and evaluation of monoclonal gammopathies (MG). Although, research has been mainly focused on multiple myeloma (MM), nowadays FCM becomes to be potential tool in the field of AL amyloidosis. Clonal plasma cells identification and specific phenotype profile detection is important for diagnosis, monitoring and prognosis of AL amyloidosis. Read More

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http://dx.doi.org/10.1016/j.leukres.2015.07.002DOI Listing
July 2015
10 Reads

Pathology in Captive Wild Felids at German Zoological Gardens.

PLoS One 2015 18;10(6):e0130573. Epub 2015 Jun 18.

Department of Pathology, University of Veterinary Medicine, Hannover, Lower Saxony, Germany; Center for Systems Neuroscience, Hannover, Lower Saxony, Germany.

This retrospective study provides an overview on spontaneous diseases occurring in 38 captive wild felids submitted for necropsy by German zoological gardens between 2004 and 2013. Species included 18 tigers, 8 leopards, 7 lions, 3 cheetahs and 2 cougars with an age ranging from 0.5 to 22 years. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0130573PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4472349PMC
April 2016
18 Reads

Diffuse Cystic Lung Disease. Part II.

Am J Respir Crit Care Med 2015 Jul;192(1):17-29

1 Division of Pulmonary, Critical Care, and Sleep Medicine and.

The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Read More

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http://www.atsjournals.org/doi/10.1164/rccm.201411-2096CI
Publisher Site
http://dx.doi.org/10.1164/rccm.201411-2096CIDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5447298PMC
July 2015
16 Reads