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Inclusion body disease

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https://www.readbyqxmd.com/read/29786894/the-prognostic-role-of-phase-angle-in-advanced-cancer-patients-a-systematic-review
#1
REVIEW
Mayane Marinho Esteves Pereira, Mariana Dos Santos Campello Queiroz, Nathália Masiero Cavalcanti de Albuquerque, Juliana Rodrigues, Emanuelly Varea Maria Wiegert, Larissa Calixto-Lima, Livia Costa de Oliveira
Phase angle (PA) is a ratio between the reactance and resistance obtained by bioelectric impedance analysis and has been interpreted as a cell membrane integrity indicator and a predictor of total body cell mass. A low PA may suggest deterioration of the cell membrane, which in advanced cancer patients may result in a reduced overall survival (OS). This systematic review sought to investigate the current evidence regarding whether there is an association between PA and OS in patients with advanced cancer (ie, metastatic disease)...
May 22, 2018: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/29786526/application-of-multiple-behaviour-change-models-to-identify-determinants-of-farmers-biosecurity-attitudes-and-behaviours
#2
I F Richens, J Houdmont, W Wapenaar, O Shortall, J Kaler, H O'Connor, M L Brennan
It has been recognised that few cattle farmers undertake biosecurity practices on their farms. Approaches that take into consideration individuals' preparedness for change, alongside beliefs thought to motivate the enactment of certain behaviours, may provide a framework for actuating tangible change. The aim of this study was to use a combination of behaviour change models to link beliefs with behaviour and identify possible key interventions to improve the uptake of biosecurity measures by dairy cattle farmers in Great Britain (GB)...
July 1, 2018: Preventive Veterinary Medicine
https://www.readbyqxmd.com/read/29783224/influence-of-risk-factors-on-insulin-resistance-in-patients-with-overweight-and-obesity
#3
Tetiana Maksymets, Natalia Karpyshyn, Taras Gutor, Helen Sklyarova, Eugen Sklyarov
OBJECTIVE: Introduction: Obesity is a multifactorial, heterogenic disease, associated with an increased risk of morbidity and mortality due to cardiovascular diseases, diabetes, cancer, chronic liver and kidney diseases. Excessive body weight and obesity are serious medical and social problems, since their incidence is constantly increasing and has reached global epidemic proportions. The aim: Determining the influence of risk factors on insulin resistance level in patients with overweight and obesity...
2018: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29780824/clinical-histological-and-immunohistochemical-findings-in-inclusion-body-myositis
#4
Leonardo Valente de Camargo, Mary Souza de Carvalho, Samuel Katsuyuki Shinjo, Acary Souza Bulle de Oliveira, Edmar Zanoteli
Sporadic inclusion body myositis (sIBM) is considered the most common acquired myopathy aged over 50 years. The disease is characterized by a particular process of muscle degeneration characterized by abnormal deposit of protein aggregates in association with inflammation. The aim of this study was to present clinical and muscle histopathological findings, including immunostaining for LC3B, p62, α -synuclein, and TDP-43, in 18 patients with sIBM. The disease predominated in males (61%) and European descendants, with onset of clinical manifestations around 59 years old...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29779724/the-emerging-use-of-social-media-for-health-related-purposes-in-low-and-middle-income-countries-a-scoping-review
#5
Emily Hagg, V Susan Dahinten, Leanne M Currie
BACKGROUND: Social media allows for instant access to, and dissemination of, information around the globe. Access to social media in low- and middle-income countries has increased exponentially in recent years due to technological advances. Despite this growth, the use of social media in low- and middle-income countries is less well-researched than in high-income countries. OBJECTIVE: To identify, explore and summarize the current state of the literature on the use of social media for health in low- and middle-income countries...
July 2018: International Journal of Medical Informatics
https://www.readbyqxmd.com/read/29775118/blood-flow-restricted-resistance-training-in-patients-with-sporadic-inclusion-body-myositis-a-randomized-controlled-trial
#6
A N Jørgensen, P Aagaard, U Frandsen, E Boyle, L P Diederichsen
OBJECTIVES: To investigate the effect of 12 weeks of low-load blood-flow restricted resistance (BFR) training on self-reported and objective physical function, and maximal muscle strength in patients with sporadic inclusion body myositis (sIBM). METHOD: Twenty-two patients with sIBM were randomized into a training group (BFR group) or a non-exercising control group, according to CONsolidated Standards Of Reporting Trials (CONSORT) guidelines. The BFR group performed 12 weeks of BFR training twice per week...
May 18, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29770361/differential-diagnosis-of-vacuolar-muscle-biopsies-use-of-p62-lc3-and-lamp2-immunohistochemistry
#7
Elisa Vittonatto, Silvia Boschi, Loredana CHIADò-Piat, Valentina Ponzalino, Sara Bortolani, Chiara Brusa, Innocenzo Rainero, Federica Ricci, Liliana Vercelli, Tiziana Mongini
Intrafibral vacuoles are the morphological hallmark in a wide variety of human skeletal muscle disorders with different etiology. In most cases, differential diagnosis is feasible with a routine histochemical work up of muscle biopsy. Ultrastructural analysis is an important confirmatory tool, but it is not widely available. Immunohistochemical stainings for p62, LAMP2 and LC3 are commonly available as tissutal marker for autophagy. We compared the immunohistochemical patterns for autophagic markers p62, LC3 and LAMP2 with routine histochemical markers in 39 biopsies from patients with definite diagnoses of glycogen storage disease type 2 (LOPD or Pompe disease, PD), sporadic inclusion body myositis (sIBM), oculo-pharyngeal muscular dystrophy (OPMD) and necrotizing myopathy (NM)...
December 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/29769092/mineralization-of-alpha-1-antitrypsin-inclusion-bodies-in-mmalton-alpha-1-antitrypsin-deficiency
#8
Francesco Callea, Isabella Giovannoni, Paola Francalanci, Renata Boldrini, Gavino Faa, Daniela Medicina, Valerio Nobili, Valeer J Desmet, Kamal Ishak, Kuniaki Seyama, Emanuele Bellacchio
BACKGROUND: Alpha-1-antitrypsin (AAT) deficiency (AATD) of Z, Mmalton, Siiyama type is associated with liver storage of the mutant proteins and liver disease. The Z variant can be diagnosed on isoelectric focusing (IEF) while Mmalton and Siiyama may be missed or misdiagnosed with this technique. Therefore, molecular analysis is mandatory for their characterization. In particular, that holds true for the Mmalton variant as on IEF profile it resembles the wild M2 subtype. METHODS: This is a retrospective analysis involving review of medical records and of liver biopsy specimens from a series of Mmalton, Z and Siiyama Alpha-1-antitrypsin deficiency patients...
May 16, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/29766941/idiopathic-inflammatory-myopathies-in-adults-a-comparative-study-of-bohan-and-peter-and-european-neuromuscular-center-2004-criteria
#9
Sundaram Challa, Saumya Jakati, Megha S Uppin, Meena A Kannan, Rajasekhar Liza, M K Murthy Jagarlapudi
Background: Bohan and Peter criteria are widely used for the diagnosis of idiopathic inflammatory myopathies (IIMs). Recently, European Neuromuscular Center (ENMC) formulated criteria to identify subgroups of IIMs. Aim: To compare the two diagnostic criteria in adult IIMs. Materials and Methods: This was a retrospective review of case records of histologically confirmed IIMs in adults between January 2014 and May 2015. Both the Bohan and Peter, and ENMC 2004 criteria were applied in the same group of patients to subgroup the IIMs...
May 2018: Neurology India
https://www.readbyqxmd.com/read/29766824/adipose-derived-mesenchymal-stem-cells-in-the-treatment-of-obesity-a-systematic-review-of-longitudinal-studies-on-preclinical-evidence
#10
Fatima Saleh, Leila Itani, Simona Calugi, Riccardo Dalle Grave, Marwan El Ghoch
BACKGROUND: Obesity is an increasing global health problem, but its treatment is not yet optimal, especially in the long term. For this reason, preclinical studies have been conducted relating to a new therapeutic strategy for obesity based on adipose-derived mesenchymal stem cells (AD-MSCs). The aim of our systematic review is to summarize these findings deriving from the animal model in order to establish whether there is sufficient evidence to justify going forward to clinical studies...
May 15, 2018: Current Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29764943/microscopic-and-molecular-evidence-of-the-first-elasmobranch-adomavirus-the-cause-of-skin-disease-in-a-giant-guitarfish-rhynchobatus-djiddensis
#11
Jennifer A Dill, Alvin C Camus, John H Leary, Terry Fei Fan Ng
Only eight families of double-stranded DNA (dsDNA) viruses are known to infect vertebrate animals. During an investigation of papillomatous skin disease in an elasmobranch species, the giant guitarfish ( Rhynchobatus djiddensis ), a novel virus, distinct from all known viral families in regard to particle size, morphology, genome organization, and helicase phylogeny was discovered. Large inclusion bodies containing 75-nm icosahedral viral particles were present within epithelial cell nuclei in the proliferative skin lesions...
May 15, 2018: MBio
https://www.readbyqxmd.com/read/29754758/novel-valosin-containing-protein-mutations-associated-with-multisystem-proteinopathy
#12
Sejad Al-Tahan, Ebaa Al-Obeidi, Hiroshi Yoshioka, Anita Lakatos, Lan Weiss, Marjorie Grafe, Johanna Palmio, Matt Wicklund, Yadollah Harati, Molly Omizo, Bjarne Udd, Virginia Kimonis
Over fifty missense mutations in the gene coding for valosin-containing protein (VCP) are associated with a unique autosomal dominant adult-onset progressive disease associated with combinations of proximo-distal inclusion body myopathy (IBM), Paget's disease of bone (PDB), frontotemporal dementia (FTD), and amyotrophic lateral sclerosis (ALS). We report the clinical, histological, and molecular findings in four new patients/families carrying novel VCP mutations: c.474 G > A (p.M158I); c.478 G > C (p...
April 17, 2018: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29751994/dietary-potential-renal-acid-load-and-risk-of-albuminuria-and-reduced-kidney-function-in-the-jackson-heart-study
#13
Tanushree Banerjee, Katherine Tucker, Michael Griswold, Sharon B Wyatt, Jane Harman, Bessie Young, Herman Taylor, Neil R Powe
OBJECTIVE: Diets high in sulfur-rich protein and low in fruit and vegetables affect human acid-base balance adversely and may have a harmful effect on progression of chronic kidney disease (CKD). Little is known about the relationship of participant characteristics, dietary acid load (DAL), and kidney injury in African-Americans with high risk of CKD progression. DESIGN AND METHODS: We examined the association of DAL with CKD in 3,257 African-Americans aged >20 years in Jackson Heart Study...
May 8, 2018: Journal of Renal Nutrition
https://www.readbyqxmd.com/read/29750538/stereotactic-body-radiation-therapy-for-liver-oligometastases-predictive-factors-of-local-response-by-18f-fdg-pet-ct
#14
Rosario Mazzola, Sergio Fersino, Pierpaolo Alongi, Gioacchino Di Paola, Fabiana Gregucci, Dario Aiello, Umberto Tebano, Stefano Pasetto, Ruggero Ruggieri, Matteo Salgarello, Filippo Alongi
OBJECTIVE: to investigate metabolic parameters as predictive of local response after Stereotactic Body Radiation Therapy (SBRT) for liver-oligometastases Methods: Inclusion criteria of the present retrospective study were: a) liver oligometastases with controlled primary tumor; b) absence of progressive disease ≥6 months; c) metastases ≤ 3; d) evaluation of SBRT-response by means of 18-FDG-PET/CT for at least two subsequent evaluations; e) Karnofsky performance status >80; f) life-expectancy >6 months...
May 11, 2018: British Journal of Radiology
https://www.readbyqxmd.com/read/29747823/gangliosides-%C3%AE-synuclein-and-parkinson-s-disease
#15
Robert W Ledeen, Gusheng Wu
This review addresses the role of α-synuclein (αSyn) in the etiopathology of Parkinson's disease (PD), with emphasis on its interaction with GM1 ganglioside. We begin with a brief review of some of the milestone discoveries that helped to elucidate PD neuropathology, including the fibrous inclusions of Lewy that characterize the degenerating dopaminergic neurons of the substantia nigra and the presence of αSyn as a major constituent of these Lewy bodies and neurites. This enabled Braak et al. to define the progressive nature of PD in developing their staging hypothesis which described the topographically predictable sequence of neuropathological changes giving rise to prodromal nonmotor symptoms that precede the classical motor dysfunctions...
2018: Progress in Molecular Biology and Translational Science
https://www.readbyqxmd.com/read/29746262/clinical-comparison-of-surgical-constructs-for-anterior-cervical-corpectomy-and-fusion-in-patients-with-cervical-spondylotic-myelopathy-or-ossified-posterior-longitudinal-ligament-a-systematic-review-and-meta-analysis
#16
Timothy R Niedzielak, Joseph Palmer, John P Malloy
STUDY DESIGN: This is a systematic review and meta-analysis. OBJECTIVE: To examine the differences in outcomes among current constructs and techniques for anterior cervical corpectomy and fusion (ACCF) in patients with single or multiple level cervical myelopathy (CM) secondary to cervical spondylosis or ossified posterior longitudinal ligament. SUMMARY OF BACKGROUND DATA: The natural history of CM can be a progressive disease process. In such cases, where surgical decompression is indicated to halt the progression, ACCF is typically chosen for pathology located posterior to the vertebral body...
May 9, 2018: Clinical Spine Surgery
https://www.readbyqxmd.com/read/29743672/cellular-milieu-imparts-distinct-pathological-%C3%AE-synuclein-strains-in-%C3%AE-synucleinopathies
#17
Chao Peng, Ronald J Gathagan, Dustin J Covell, Coraima Medellin, Anna Stieber, John L Robinson, Bin Zhang, Rose M Pitkin, Modupe F Olufemi, Kelvin C Luk, John Q Trojanowski, Virginia M-Y Lee
In Lewy body diseases-including Parkinson's disease, without or with dementia, dementia with Lewy bodies, and Alzheimer's disease with Lewy body co-pathology 1 -α-synuclein (α-Syn) aggregates in neurons as Lewy bodies and Lewy neurites 2 . By contrast, in multiple system atrophy α-Syn accumulates mainly in oligodendrocytes as glial cytoplasmic inclusions (GCIs) 3 . Here we report that pathological α-Syn in GCIs and Lewy bodies (GCI-α-Syn and LB-α-Syn, respectively) is conformationally and biologically distinct...
May 9, 2018: Nature
https://www.readbyqxmd.com/read/29743208/overlap-between-age-at-onset-and-disease-progression-determinants-in-huntington-disease
#18
N Ahmad Aziz, Jorien M M van der Burg, Sarah J Tabrizi, G Bernhard Landwehrmeyer
OBJECTIVE: A fundamental but still unresolved issue regarding Huntington disease (HD) pathogenesis is whether the factors that determine age at onset are the same as those that govern disease progression. Because elucidation of this issue is crucial for the development as well as optimal timing of administration of novel disease-modifying therapies, we aimed to assess the extent of overlap between age-at-onset and disease-progression determinants in HD. METHODS: Using observational data from Enroll-HD, the largest cohort of patients with HD worldwide, in this study we present, validate, and apply an intuitive method based on linear mixed-effect models to quantify the variability in the rate of disease progression in HD...
May 9, 2018: Neurology
https://www.readbyqxmd.com/read/29738880/the-interaction-of-%C3%AE-synuclein-and-tau-a-molecular-conspiracy-in-neurodegeneration
#19
REVIEW
Xu Yan, Riikka-Liisa Uronen, Henri J Huttunen
α-synuclein and Tau are proteins prone to pathological misfolding and aggregation that are normally found in the presynaptic and axonal compartments of neurons. Misfolding initiates a homo-oligomerization and aggregation cascade culminating in cerebral accumulation of aggregated α-synuclein and Tau in insoluble protein inclusions in multiple neurodegenerative diseases. Traditionally, α-synuclein-containing Lewy bodies have been associated with Parkinson's disease and Tau-containing neurofibrillary tangles with Alzheimer's disease and various frontotemporal dementia syndromes...
May 5, 2018: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/29738352/the-effect-of-obesity-on-the-quality-of-bowel-preparation-for-colonoscopy-results-from-a-large-observational-study
#20
Ava B Anklesaria, Elena A Ivanina, Kenechukwu O Chudy-Onwugaje, Kevin Tin, Chaya M Levine, Peter Homel, Mary Rojas, Ira E Mayer, Rabin Rahmani
BACKGROUND: Obesity has been linked to suboptimal bowel preparation but this association has not been conclusively investigated in prospective studies. GOALS: Our objective was to determine whether any relationship exists between obesity as measured by body mass index (BMI) and quality of bowel preparation. STUDY: Adult patients who presented for outpatient colonoscopy at a single urban ambulatory surgery center within a 6-month period and fulfilled inclusion criteria were prospectively enrolled for the study...
May 5, 2018: Journal of Clinical Gastroenterology
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