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systemic amyloidosis

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https://www.readbyqxmd.com/read/28937388/undiagnosed-senile-systemic-amyloidosis-with-mesenteric-angiopathy-leading-to-fatal-outcomes-two-case-series-with-a-review-of-literature
#1
Avneesh Gupta, Maryna Tarbunova, Anil Aggrawal, Anges E Aysola
Amyloid mesenteric angiopathy is difficult to diagnose as gastrointestinal (GI) signs are nonspecific and radiological studies rarely identify amyloid deposits with certainty. The objective of this study was to highlight the pathological findings of amyloid mesenteric angiopathy in cases of senile systemic amyloidosis. Literature review, author's research data, and practice experience were used. Systemic amyloidosis should be considered in the differential diagnoses when evaluating nonspecific and unexplained GI in patient population aged> 55 years to decrease the morbidity and fatal outcomes...
July 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28935176/current-insight-in-the-localized-insulin-derived-amyloidosis-lida-clinico-pathological-characteristics-and-differential-diagnosis
#2
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...
September 1, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28928748/a-peptide-fc-opsonin-with-pan-amyloid-reactivity
#3
James S Foster, Angela D Williams, Sallie Macy, Tina Richey, Alan Stuckey, Daniel Craig Wooliver, Richa Koul-Tiwari, Emily B Martin, Stephen J Kennel, Jonathan S Wall
There is a continuing need for therapeutic interventions for patients with the protein misfolding disorders that result in systemic amyloidosis. Recently, specific antibodies have been employed to treat AL amyloidosis by opsonizing tissue amyloid deposits thereby inducing cell-mediated dissolution and organ improvement. To develop a pan-amyloid therapeutic agent, we have produced an Fc-fusion product incorporating a peptide, p5, which binds many if not all forms of amyloid. This protein, designated Fcp5, expressed in mammalian cells, forms the desired bivalent dimer structure and retains pan-amyloid reactivity similar to the p5 peptide as measured by immunosorbent assays, immunohistochemistry, surface plasmon resonance, and pulldown assays using radioiodinated Fcp5...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28926601/utility-of-abdominal-skin-plus-subcutaneous-fat-and-rectal-mucosal-biopsy-in-the-diagnosis-of-al-amyloidosis-with-renal-involvement
#4
Ting Li, Xianghua Huang, Shuiqin Cheng, Liang Zhao, Guisheng Ren, Wencui Chen, Qingwen Wang, Caihong Zeng, Zhihong Liu
OBJECTIVES: Skin fat biopsy of the abdominal wall is a simple and safe method for detecting amyloidosis, and rectal mucosal biopsy is also frequently used for screening for the disease; however, the sensitivity of these approaches has not been fully studied. The aim of this study was to evaluate the efficacy of skin fat biopsy combined with rectal mucosal biopsy as a screening procedure for the diagnosis of systemic immunoglobulin light-chain (AL) amyloidosis. METHODS: We retrospectively analyzed 224 AL amyloidosis patients confirmed by renal biopsy, including a test group of 165 patients and validation group of 59 patients...
2017: PloS One
https://www.readbyqxmd.com/read/28924445/the-first-argentinian-family-with-familial-amyloidosis-of-the-finnish-type
#5
Francisco Lucero Saá, Federico Andrés Cremona, Natalia Ximena Mínguez, María Laura Igarzabal, Pablo Chiaradía
Familial amyloidosis of the Finnish type or Meretoja syndrome is a rare autosomic dominant inherited systemic condition. It was first described by Meretoja in Finland in 1969. It is a disease produced by a single mutation in the gene coding for gelsolin, which generates an abnormal protein that cumulates in tissues and leads to various signs. Obtaining an early diagnosis can be challenging, as the first manifestations of the disease are ophthalmological and may only be seen with slit-lamp biomicroscopy. We present the first 3 cases diagnosed in Argentina, confirmed by genetic molecular testing...
May 2017: Case Reports in Ophthalmology
https://www.readbyqxmd.com/read/28912154/inhibition-of-p25-cdk5-attenuates-tauopathy-in-mouse-and-ipsc-models-of-frontotemporal-dementia
#6
Jinsoo Seo, Oleg Kritskiy, L Ashley Watson, Scarlett J Barker, Dilip Dey, Waseem K Raja, Yuan-Ta Lin, Tak Ko, Sukhee Cho, Jay Penney, M Catarina Silva, Steven D Sheridan, Diane Lucente, James F Gusella, Bradford C Dickerson, Stephen J Haggarty, Li-Huei Tsai
Increased p25, a proteolytic fragment of the regulatory subunit p35, is known to induce aberrant activity of cyclin-dependent kinase 5 (Cdk5), which is associated with neurodegenerative disorders including Alzheimer's disease (AD). Previously, we showed that replacing endogenous p35 with the non-cleavable mutant p35 (Δp35) attenuated amyloidosis and improved cognitive function in a familial AD mouse model. Here, to address the role of p25/Cdk5 in tauopathy, we generated double transgenic mice by crossing mice overexpressing mutant human tau (P301S) with Δp35KI mice...
September 14, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28911993/ocular-manifestations-of-familial-transthyretin-amyloidosis
#7
Margaret M Reynolds, Kevin K Veverka, Morie A Gertz, Angela Dispenzieri, Steven R Zeldenrust, Nelson Leung, Jose S Pulido
PURPOSE: Among patients with familial amyloidosis, mutation in the transthyretin (TTR) protein is the most common type. Patients with TTR amyloidosis have been noted to have ocular, especially vitreous, involvement. In this report, an analysis of the types and frequency of ocular manifestations in TTR amyloidosis is presented. DESIGN: Observational case series. METHODS: Two hundred and sixty-three patients who presented to Mayo Clinic with TTR amyloidosis between January 1, 1970, and November 1, 2014, consented to be included in the Mayo Clinic amyloidosis database maintained by the Department of Hematology...
September 11, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#8
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...
September 15, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28903570/czech-registry-of-monoclonal-gammopathies-technical-solution-data-collection-and-visualisation
#9
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/28900607/de-novo-intraocular-amyloid-deposition-after-hepatic-transplantation-in-familial-amyloidotic-polyneuropathy
#10
Ivo Filipe Gama, Leonor Duarte Almeida
The familiar amyloid polyneuropathy (FAP) is a rare autosomal-dominant systemic amyloidosis. Amyloid deposition occurs more frequently and extensively in the vitq. The increase in intraocular pressure (IOP) is a result of deposition of transthyretin (TTR) in trabecular meshwork. Rarely, the amyloid deposition in anterior segment can be more exuberant than in posterior segment. A 42 years old man, with FAP (Val30Met mutation), liver transplantation in 1997. He was asymptomatic, without any significant ocular abnormality until 2011...
August 24, 2017: World Journal of Transplantation
https://www.readbyqxmd.com/read/28900341/hepatic-amyloidosis-something-that-can-camouflage-and-deceive-our-perception
#11
Anirudh V Nair, Manish Kumar Yadav, Madhavan N Unni, C M Simi, K A Biji, K S Manoj, Shabeer Ali, Ajith K Nair
Amyloidosis is a multi-systemic diffusely infiltrating disease due to extracellular deposition of protein-mucopolysaccharide complexes. The type of protein deposited determines the subgroup of amyloid. Hepatic amyloidosis is a rare infiltrating disease affecting the hepatic parenchyma. A wide range of clinical presentation and atypical imaging findings delay the diagnosis of amyloidosis, while tissue biopsy demonstrating amyloid deposits is vital for a definitive diagnosis.
April 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28894888/-posttherapeutic-changes-in-bone-marrow
#12
REVIEW
T Geith, A-C Stellwag, A Baur-Melnyk
The bone marrow basically consists of red blood-forming bone marrow and yellow fat. In the skeleton, there is an age-dependent distribution of these two parts. In the context of medical interventions or therapies, bone marrow changes can occur, whereby the normal bone marrow can basically be replaced by fat, edema, or fibrosis/sclerosis. Here, specific signal intensities and patterns are shown in imaging. After irradiation therapies, edematous changes, hemorrhages, and osteoradionecroses are observed. Likewise, insufficiency fractures, impairment of the growth gaps, or the development of tumors is possible...
September 11, 2017: Der Radiologe
https://www.readbyqxmd.com/read/28891012/visualization-of-multiple-organ-amyloid-involvement-in-systemic-amyloidosis-using-11-c-pib-pet-imaging
#13
Naoki Ezawa, Nagaaki Katoh, Kazuhiro Oguchi, Tsuneaki Yoshinaga, Masahide Yazaki, Yoshiki Sekijima
PURPOSE: To investigate the utility of Pittsburgh compound B (PiB) positron emission tomography (PET) imaging for evaluating whole-body amyloid involvement in patients with systemic amyloidosis. METHODS: Whole-body (11)C-PiB PET was performed in seven patients with systemic immunoglobulin light-chain (AL) amyloidosis, seven patients with hereditary transthyretin (ATTRm) amyloidosis, one asymptomatic TTR mutation carrier and three healthy controls. The correlations between clinical organ involvement, radiological (11)C-PiB uptake and histopathological findings were analysed for each organ...
September 10, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/28888072/characterization-of-isolated-amyloid-myopathy
#14
Teerin Liewluck, Margherita Milone
BACKGROUND AND PURPOSE: Amyloid myopathy frequently occurs in the setting of systemic amyloidosis and less commonly in isolation (isolated amyloid myopathy). Anoctaminopathy-5 and dysferlinopathy were recently recognized as causes of isolated amyloid myopathy. The present study aimed to characterize the isolated amyloid myopathy and to compare it with amyloid myopathy associated with systemic amyloidosis. METHODS: We searched Muscle Laboratory database to identify patients with pathologically-confirmed amyloid myopathy and were seen in neurology clinics between January 1998 and September 2016...
September 9, 2017: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/28887775/imaging-cardiac-innervation-in-amyloidosis
#15
Riemer H J A Slart, Andor W J M Glaudemans, Bouke P C Hazenberg, Walter Noordzij
Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. The use of iodine-123 labelled metaiodobenzylguanidine ([I-123]MIBG), a chemical modified analogue of norepinephrine, is well established in patients with heart failure and plays an important role in evaluation of sympathetic innervation in cardiac amyloidosis...
September 8, 2017: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
https://www.readbyqxmd.com/read/28887204/structural-determinants-in-apoa-i-amyloidogenic-variants-explain-improved-cholesterol-metabolism-despite-low-hdl-levels
#16
Rita Del Giudice, Joan Domingo-Espín, Ilaria Iacobucci, Oktawia Nilsson, Maria Monti, Daria Maria Monti, Jens O Lagerstedt
Twenty Apolipoprotein A-I (ApoA-I) variants are responsible for a systemic hereditary amyloidosis in which protein fibrils can accumulate in different organs, leading to their failure. Several ApoA-I amyloidogenic mutations are also associated with hypoalphalipoproteinemia, low ApoA-I and high-density lipoprotein (HDL)-cholesterol plasma levels; however, subjects affected by ApoA-I-related amyloidosis do not show a higher risk of cardiovascular diseases (CVD). The structural features, the lipid binding properties and the functionality of four ApoA-I amyloidogenic variants were therefore inspected in order to clarify the paradox observed in the clinical phenotype of the affected subjects...
September 5, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28877975/the-lung-in-amyloidosis
#17
REVIEW
Paolo Milani, Marco Basset, Francesca Russo, Andrea Foli, Giovanni Palladini, Giampaolo Merlini
Amyloidosis is a disorder caused by misfolding of autologous protein and its extracellular deposition as fibrils, resulting in vital organ dysfunction and eventually death. Pulmonary amyloidosis may be localised or part of systemic amyloidosis.Pulmonary interstitial amyloidosis is symptomatic only if the amyloid deposits severely affect gas exchange alveolar structure, thus resulting in serious respiratory impairment. Localised parenchymal involvement may be present as nodular amyloidosis or as amyloid deposits associated with localised lymphomas...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28871434/cutaneous-and-mucosal-manifestations-of-sj%C3%A3-gren-s-syndrome
#18
REVIEW
Elena Generali, Antonio Costanzo, Carlo Mainetti, Carlo Selmi
Sjögren's syndrome is currently considered an "autoimmune epithelitis," as exocrine glands, especially salivary and lacrimal, are progressively destructed by an immune-mediated process associated with specific serum autoantibodies and local lymphocyte infiltrate. Xerostomia remains a key complain in patients with Sjögren's syndrome but should be evaluated also for other causes such as xerogenic medications, followed by radiation and chemotherapy for head and neck cancers, hormone disorders, infections, or other connective tissue diseases...
September 4, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28868800/clinical-characteristics-of-laryngeal-versus-nonlaryngeal-amyloidosis
#19
Shannon F Rudy, Caroline C Jeffery, Edward J Damrose
OBJECTIVES/HYPOTHESIS: Amyloidosis represents a heterogeneous group of disorders marked by abnormal protein formation and deposition. Laryngeal amyloidosis is rare and classically thought to remain isolated with little risk of systemic involvement or associated malignancy. This study sought to further characterize differences in clinical characteristics between patients with laryngeal and nonlaryngeal amyloidosis. STUDY DESIGN: Retrospective case-control study. METHODS: The Stanford Translational Research Integrated Database Environment was searched to identify patients with biopsy-confirmed laryngeal amyloidosis and patients with amyloidosis without laryngeal involvement on endoscopy...
September 4, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28861330/a-plasma-cell-dyscrasia-presenting-as-amyloid-cardiomyopathy-and-autonomic-dysfunction-in-a-healthy-patient
#20
Rabih Tabet, Julie Zaidan, Boutros Karam, Samer Saouma, Foad Ghavami
Systemic amyloidosis is a rare multisystem disease caused by incorrectly folded proteins that deposit pathologically in different tissues and organs of the human body. It has a very wide spectrum of clinical presentations according to the affected organ(s), and its diagnosis is commonly delayed. Cardiac involvement is the leading cause of morbidity and mortality and carries a poor prognosis, especially in primary light chain amyloidosis. Therefore any delay in the diagnosis can result in devastating outcomes for the patient...
June 29, 2017: Curēus
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