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Inclusion body disease

Giuliano Tosello, Maria Regina Torloni, Bruna S Mota, Teresa Neeman, Rachel Riera
BACKGROUND: Metastatic breast cancer is not a curable disease, but women with metastatic disease are living longer. Surgery to remove the primary tumour is associated with an increased survival in other types of metastatic cancer. Breast surgery is not standard treatment for metastatic disease, however several recent retrospective studies have suggested that breast surgery could increase the women's survival. These studies have methodological limitations including selection bias. A systematic review mapping all randomised controlled trials addressing the benefits and potential harms of breast surgery is ideal to answer this question...
March 15, 2018: Cochrane Database of Systematic Reviews
Sara Ekmark-Lewén, Veronica Lindström, Astrid Gumucio, Elisabeth Ihse, Anish Behere, Philipp J Kahle, Eva Nordström, Maria Eriksson, Anna Erlandsson, Joakim Bergström, Martin Ingelsson
Introduction: Intraneuronal inclusions of alpha-synuclein are commonly found in the brain of patients with Parkinson's disease and other α-synucleinopathies. The correlation between alpha-synuclein pathology and symptoms has been studied in various animal models. In (Thy-1)-h[A30P] alpha-synuclein transgenic mice, behavioral and motor abnormalities were reported from 12 and 15 months, respectively. The aim of this study was to investigate whether these mice also display symptoms at earlier time points...
March 2018: Brain and Behavior
Shelly Popowich, Ashish Gupta, Betty Chow-Lockerbie, Lisanework Ayalew, Neil Ambrose, Davor Ojkic, Thushari Gunawardana, Shanika Kurukulasuriya, Philip Willson, Suresh K Tikoo, Susantha Gomis
Historically, fowl adenovirus (FAdV) associated inclusion body hepatitis (IBH) was considered a secondary disease in broiler chickens associated with immunosuppression. However, we previously reported the occurrence of IBH as a primary disease in the broiler chicken industry in Canada as a result of infections with various FAdV serotypes. Therefore, the objectives of this study were to develop an immunization strategy in broiler breeders using live FAdV 11-1047 and FAdV8a-TR59 to confer homologous and heterologous protection in broiler progeny against IBH and to study the efficacy of natural exposure of naïve broiler breeders to a vaccine virus from live FAdV vaccinated birds as an immunization technique...
March 5, 2018: Research in Veterinary Science
Roshan Kumar, Raniki Kumari, Sanjay Kumar, Deepak Kumar Jangir, Tushar Kanti Maiti
α-Synuclein, a major constituent of proteinaceous inclusions named Lewy body, has been shown to be released and taken up by cells, which may facilitate its progressive pathological spreading and neuronal cell death in Parkinson's disease. However, the pathophysiological effect and signalling cascade initiated by extracellular α-synuclein in cellular milieu are not well understood. Herein we have investigated the perturbations induced by low molecular weight α-synuclein and different types of α-synuclein oligomers in the neuroblastoma SH-SY5Y cells...
March 14, 2018: Biomacromolecules
Mattia Laffranchi, Romina Berardelli, Riccardo Ronzoni, David A Lomas, Annamaria Fra
The most common genotype associated with severe α1-antitrypsin deficiency (AATD) is the Z homozygote. The Z variant (Glu342Lys) of α1-antitrypsin (AAT) undergoes a conformational change and is retained within the endoplasmic reticulum (ER) of hepatocytes leading to the formation of ordered polymeric chains and inclusion bodies. Accumulation of mutated protein predisposes to cirrhosis whilst plasma AAT deficiency leads to emphysema. Increased risk of liver and lung disease has also been reported in heterozygous subjects who carry Z in association with the milder S allele (Glu264Val) or even with wild-type M...
March 10, 2018: Human Molecular Genetics
Ivan Martinez-Valbuena, Irene Amat-Villegas, Rafael Valenti-Azcarate, Maria Del Mar Carmona-Abellan, Irene Marcilla, Maria-Teresa Tuñon, Maria-Rosario Luquin
Parkinson's disease patients experience a wide range of non-motor symptoms that may be provoked by deposits of phosphorylated α-synuclein in the peripheral nervous system. Pre-existing diabetes mellitus might be a risk factor for developing Parkinson's disease, and indeed, nearly 60% of Parkinson's disease patients are insulin resistant. Thus, we have investigated whether phosphorylated α-synuclein is deposited in pancreatic tissue of subjects with synucleinopathies. We studied pancreatic tissue from 39 subjects diagnosed with Parkinson's disease, Lewy body Dementia or incidental Lewy bodies disease, as well as that from 34 subjects with diabetes mellitus and a normal neuropathological examination, and 52 subjects with a normal neuropathological examination...
March 13, 2018: Acta Neuropathologica
Geneviève Huard, M Isabel Fiel, Jang Moon, Kishore Iyer, Thomas D Schiano
INTRODUCTION: Intestinal failure-associated liver disease (IFALD) occurs commonly in intestinal transplant (ITx) candidates receiving parenteral nutrition (PN). The aim of this study is to establish the prevalence and risk factors for advanced liver fibrosis in adults at the time of ITx. METHODS: Retrospective chart review of all ITx was performed in adults between January 2000 and May 2014. Advanced liver fibrosis was defined as stage 3 or stage 4 fibrosis. RESULTS: Fifty-three patients met the inclusion criteria...
March 13, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
Désirée van der Heijde, David I Daikh, Neil Betteridge, Gerd R Burmester, Afton L Hassett, Eric L Matteson, Ronald van Vollenhoven, Sharad Lakhanpal
A European League Against Rheumatism-American College of Rheumatology working group consisting of practicing and academic rheumatologists, a rheumatology researcher, and a patient representative created a succinct general statement describing rheumatic and musculoskeletal diseases (RMDs) in adults and children in language that can be used in conversations with the lay public, media, healthcare providers, and other stakeholders. Based on the literature review, several elements were deemed important for inclusion in the description of RMDs...
March 13, 2018: Arthritis & Rheumatology
Désirée van der Heijde, David I Daikh, Neil Betteridge, Gerd R Burmester, Afton L Hassett, Eric L Matteson, Ronald van Vollenhoven, Sharad Lakhanpal
A European League Against Rheumatism-American College of Rheumatology working group consisting of practising and academic rheumatologists, a rheumatology researcher and a patient representative created a succinct general statement describing rheumatic and musculoskeletal diseases (RMDs) in adults and children in language that can be used in conversations with the lay public, media, healthcare providers and other stakeholders. Based on the literature review, several elements were deemed important for inclusion in the description of RMDs...
March 10, 2018: Annals of the Rheumatic Diseases
Cheryl L Maier, Phillip J Gross, Christina L Dean, Satheesh Chonat, Andrew Ip, Morgan McLemore, Fuad El Rassi, Sean R Stowell, Cassandra D Josephson, Ross M Fasano
BACKGROUND: Fever accompanying vaso-occlusive crisis is a common presentation in patients with sickle cell disease (SCD) and carries a broad differential diagnosis. Here, we report a case of transfusion-transmitted malaria in a patient with SCD presenting with acute vaso-occlusive crisis and rapidly decompensating to multisystem organ failure (MSOF). CASE REPORT: An 18-year-old African American male with SCD was admitted after multiple days of fever and severe generalized body pain...
March 9, 2018: Transfusion
Ying Hou, Yue-Bei Luo, Tingjun Dai, Kai Shao, Wei Li, Yuying Zhao, Jian-Qiang Lu, Chuanzhu Yan
The European Neuromuscular Centre (ENMC) pathological classification criteria of idiopathic inflammatory myopathies (IIMs) are debatable. The aim of this study was to explore their practicability and reproducibility. We conducted a retrospective analysis of 57 cases of IIMs excluding dermatomyositis (DM) and sporadic inclusion body myositis (sIBM) by in-depth analysis of muscle biopsies and comparisons of the clinical characteristics among polymyositis (PM), non-specific myositis (NSM) and necrotizing autoimmune myopathy (NAM)...
March 7, 2018: Journal of Neuropathology and Experimental Neurology
Angelo Antonini, Michele Tinazzi, Giovanni Abbruzzese, Alfredo Berardelli, K Ray Chaudhuri, Giovanni Defazio, Joaquim Ferreira, Pablo Martinez-Martin, Claudia Trenkwalder, Olivier Rascol
BACKGROUND: Pain is one of the most common and troublesome non-motor symptoms of Parkinson's disease; it can appear at any time during the disease, and it is often present before diagnosis. However, there is little or no consensus on its definition. METHODS: An expert group of clinicians with relevant research experience met to review the existing evidence, and to identify gaps in our understanding towards an optimized therapy of pain in Parkinson's disease. RESULTS: Key findings from epidemiologic, neurophysiologic, neuroimaging and clinical studies are reviewed...
March 9, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
Ivy N Haskins, Julietta Chang, Zubaidah Nor Hanipah, Tavankit Singh, Neal Mehta, Arthur J McCullough, Stacy A Brethauer, Philip R Schauer, Ali Aminian
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) increases the risk of liver cirrhosis and hepatocellular carcinoma and is also strongly correlated with extrahepatic diseases, including cardiovascular disease and type 2 diabetes. This risk of NAFLD among obese individuals who are otherwise metabolically healthy is not well characterized. OBJECTIVES: To determine the prevalence and characteristics of NAFLD in individuals with metabolically healthy obesity. SETTING: A tertiary, academic, referral hospital...
March 2018: Surgery for Obesity and related Diseases: Official Journal of the American Society for Bariatric Surgery
Clayton C Clark, Jennifer Hunter
Heart failure is a chronic and terminal disease that affects a significant portion of the U.S. POPULATION: It is marked by considerable suffering, for which palliative care has been recommended. Palliative care standards require the inclusion of spiritual care, but there is a paucity of literature supporting effective spiritual interventions for the heart failure population. A literature search resulted in 30 articles meeting the criteria for review of spirituality and spiritual coping in the heart failure population...
March 1, 2018: Journal of Holistic Nursing: Official Journal of the American Holistic Nurses' Association
Kittikorn Boonsri, Chaleamchat Somgird, Pheudphol Noinafai, Kidsadagon Pringproa, Thittaya Janyamethakul, Taweepoke Angkawanish, Janine L Brown, Pallop Tankaew, Saralee Srivorakul, Chatchote Thitaram
Elephant endotheliotropic herpesvirus (EEHV) is an infection associated with fatal hemorrhagic disease in young Asian elephants ( Elephas maximus). This brief communication describes the postmortem evaluation of two Asian elephant calves diagnosed with EEHV4 and EEHV1A in conjunction with Clostridium perfringens infection. Case 1 was a 7-mo-old, male captive-born Asian elephant that developed diarrhea and died 2 days after clinical presentation. Examination of the heart, lungs, liver, and spleen revealed predominantly basophilic intranuclear inclusion bodies in the endothelial cells of the blood vessels...
March 2018: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Fabia Wyss, Martin Schneiter, Udo Hetzel, Fta Pathologie, Saskia Keller, Martin Frenz, Jaroslav Rička, Jean-Michel Hatt
Pneumonia is a common complication of boid inclusion body disease (BIBD) in snakes. The tracheal mucociliary apparatus of eight boas ( Boa constrictor) and two pythons ( Python regius, Morelia viridis) was examined to assess whether absent or reduced mucociliary clearance could be a predisposing factor. Nine of the examined snakes were positive for BIBD by detection of inclusion bodies and three had lung pathologies other than the formation of inclusion bodies. A considerable individual variation of ciliary beat frequency (CBF, 3...
March 2018: Journal of Zoo and Wildlife Medicine: Official Publication of the American Association of Zoo Veterinarians
Sandrine Herbelet, Elly De Vlieghere, Amanda Gonçalves, Boel De Paepe, Karsten Schmidt, Eline Nys, Laurens Weynants, Joachim Weis, Gert Van Peer, Jo Vandesompele, Jens Schmidt, Olivier De Wever, Jan L De Bleecker
Aims: Regeneration in skeletal muscle relies on regulated myoblast migration and differentiation, in which the transcription factor nuclear factor of activated T-cells 5 (NFAT5) participates. Impaired muscle regeneration and chronic inflammation are prevalent in myositis. Little is known about the impact of inflammation on NFAT5 localization and expression in this group of diseases. The goal of this study was to investigate NFAT5 physiology in unaffected myoblasts exposed to cytokine or hyperosmolar stress and in myositis...
2018: Frontiers in Physiology
Lukas Streese, Arne Deiseroth, Juliane Schäfer, Arno Schmidt-Trucksäss, Henner Hanssen
Background: Age is a key determinant for the development of cardiovascular disease and higher age coincides with an increased prevalence of obesity and physical inactivity. The study examines the influence of physical activity on aging processes of physiological systems focusing on the mechanisms of vascular aging. Methods/Design: The study consists of two parts. The cross-sectional approach aims at examining the association of physical fitness and cardiovascular risk with large and small artery function in healthy older active (HOA, n = 40) and sedentary (HOS, n = 40) persons as well as older sedentary individuals with increased cardiovascular risk (OSR, n = 80) aged 50-80 years...
2018: Frontiers in Physiology
Jessika C Bridi, Frank Hirth
Parkinson's disease (PD) is characterized by intracellular inclusions of aggregated and misfolded α-Synuclein (α-Syn), and the loss of dopaminergic (DA) neurons in the brain. The resulting motor abnormalities mark the progression of PD, while non-motor symptoms can already be identified during early, prodromal stages of disease. Recent studies provide evidence that during this early prodromal phase, synaptic and axonal abnormalities occur before the degenerative loss of neuronal cell bodies. These early phenotypes can be attributed to synaptic accumulation of toxic α-Syn...
2018: Frontiers in Neuroscience
Jordan F Hastings, Joanna N Skhinas, Dirk Fey, David R Croucher, Thomas R Cox
The extracellular matrix (ECM) is a salient feature of all solid tissues within the body. This complex, acellular entity is composed of hundreds of individual molecules whose assembly, architecture and biomechanical properties are critical to controlling the behaviour and phenotype of the different cells types residing within tissues. Cells are the basic unit of life and the core building block of tissues and organs. At their simplest, they follow a set of rules, governed by their genetic code, and actioned through the complex protein signalling networks that these genes encode...
March 6, 2018: British Journal of Pharmacology
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