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Sperling M

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https://www.readbyqxmd.com/read/28804536/combined-respiratory-chain-deficiency-and-uqcc2-mutations-in-neonatal-encephalomyopathy-defective-supercomplex-assembly-in-complex-iii-deficiencies
#1
René G Feichtinger, Michaela Brunner-Krainz, Bader Alhaddad, Saskia B Wortmann, Reka Kovacs-Nagy, Tatjana Stojakovic, Wolfgang Erwa, Bernhard Resch, Werner Windischhofer, Sarah Verheyen, Sabine Uhrig, Christian Windpassinger, Felix Locker, Christine Makowski, Tim M Strom, Thomas Meitinger, Holger Prokisch, Wolfgang Sperl, Tobias B Haack, Johannes A Mayr
Vertebrate respiratory chain complex III consists of eleven subunits. Mutations in five subunits either mitochondrial (MT-CYB) or nuclear (CYC1, UQCRC2, UQCRB, and UQCRQ) encoded have been reported. Defects in five further factors for assembly (TTC19, UQCC2, and UQCC3) or iron-sulphur cluster loading (BCS1L and LYRM7) cause complex III deficiency. Here, we report a second patient with UQCC2 deficiency. This girl was born prematurely; pregnancy was complicated by intrauterine growth retardation and oligohydramnios...
2017: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/28799444/the-msoac-approach-to-developing-performance-outcomes-to-measure-and-monitor-multiple-sclerosis-disability
#2
Nicholas G LaRocca, Lynn D Hudson, Richard Rudick, Dagmar Amtmann, Laura Balcer, Ralph Benedict, Robert Bermel, Ih Chang, Nancy D Chiaravalloti, Peter Chin, Jeffrey A Cohen, Gary R Cutter, Mat D Davis, John DeLuca, Peter Feys, Gordon Francis, Myla D Goldman, Emily Hartley, Raj Kapoor, Fred Lublin, Gary Lundstrom, Paul M Matthews, Nancy Mayo, Richard Meibach, Deborah M Miller, Robert W Motl, Ellen M Mowry, Rob Naismith, Jon Neville, Jennifer Panagoulias, Michael Panzara, Glenn Phillips, Ann Robbins, Matthew F Sidovar, Kathryn E Smith, Bjorn Sperling, Bernard Mj Uitdehaag, Jerry Weaver
BACKGROUND: The Multiple Sclerosis Outcome Assessments Consortium (MSOAC) was formed by the National MS Society to develop improved measures of multiple sclerosis (MS)-related disability. OBJECTIVES: (1) To assess the current literature and available data on functional performance outcome measures (PerfOs) and (2) to determine suitability of using PerfOs to quantify MS disability in MS clinical trials. METHODS: (1) Identify disability dimensions common in MS; (2) conduct a comprehensive literature review of measures for those dimensions; (3) develop an MS Clinical Data Interchange Standards Consortium (CDISC) data standard; (4) create a database of standardized, pooled clinical trial data; (5) analyze the pooled data to assess psychometric properties of candidate measures; and (6) work with regulatory agencies to use the measures as primary or secondary outcomes in MS clinical trials...
August 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28791443/outcomes-of-shoulder-arthroplasty-in-haematopoietic-stem-cell-transplant-patients
#3
Joseph M Statz, Brian P Chalmers, Cameron K Ledford, Joaquin Sanchez-Sotelo, John W Sperling, Mark E Morrey
BACKGROUND: With the growing population of patients undergoing haematopoietic stem cell transplants (HSCTs), the demand for shoulder arthroplasty (SA) in this population can be expected to increase. No studies in the literature have examined the outcomes of SA in HSCT patients. PATIENTS AND METHODS: A retrospective review of 11 SAs in 10 patients with previous HSCT was performed. We characterized the clinical outcomes, survivorship, and peri-operative complication rates in patients who underwent SA after receiving a HSCT...
August 9, 2017: International Orthopaedics
https://www.readbyqxmd.com/read/28752121/docetaxel-extravasation-pathologic-correlation-and-treatment-with-intralesional-steroids
#4
John W Roman, Michelle A Ojemuyiwa, Lisa M Cordes, Ravi A Madan, Leonard Sperling
No abstract text is available yet for this article.
July 2017: JAAD Case Reports
https://www.readbyqxmd.com/read/28751093/two-stage-reimplantation-for-the-treatment-of-deep-infection-after-shoulder-arthroplasty
#5
Andrew T Assenmacher, Eduard Alentorn-Geli, Taylor Dennison, Yaser M K Baghdadi, Robert H Cofield, Joaquín Sánchez-Sotelo, John W Sperling
BACKGROUND: Two-stage reimplantation is the most common treatment modality considered for periprosthetic shoulder infection (PSI). Most studies to date have reported on a relatively small number of shoulders. The purpose of this study was to determine the outcome of 2-stage reimplantation for PSI in terms of both eradication of infection and restoration of function. METHODS: Between 1980 and 2010, 40 shoulders (39 patients) underwent a 2-stage reimplantation for PSI; 35 shoulders (34 patients) met inclusion criteria (10 hemiarthroplasties, 24 anatomic total shoulder arthroplasties, 1 reverse total shoulder arthroplasty)...
July 24, 2017: Journal of Shoulder and Elbow Surgery
https://www.readbyqxmd.com/read/28738187/presymptomatic-atrophy-in-autosomal-dominant-alzheimer-s-disease-a%C3%A2-serial-mri-study
#6
Kirsi M Kinnunen, David M Cash, Teresa Poole, Chris Frost, Tammie L S Benzinger, R Laila Ahsan, Kelvin K Leung, M Jorge Cardoso, Marc Modat, Ian B Malone, John C Morris, Randall J Bateman, Daniel S Marcus, Alison Goate, Stephen Salloway, Stephen Correia, Reisa A Sperling, Jasmeer P Chhatwal, Richard Mayeux, Adam M Brickman, Ralph N Martins, Martin R Farlow, Bernardino Ghetti, Andrew J Saykin, Clifford R Jack, Peter R Schofield, Eric McDade, Michael W Weiner, John M Ringman, Paul M Thompson, Colin L Masters, Christopher C Rowe, Martin N Rossor, Sebastien Ourselin, Nick C Fox
INTRODUCTION: Identifying at what point atrophy rates first change in Alzheimer's disease is important for informing design of presymptomatic trials. METHODS: Serial T1-weighed magnetic resonance imaging scans of 94 participants (28 noncarriers, 66 carriers) from the Dominantly Inherited Alzheimer Network were used to measure brain, ventricular, and hippocampal atrophy rates. For each structure, nonlinear mixed-effects models estimated the change-points when atrophy rates deviate from normal and the rates of change before and after this point...
July 21, 2017: Alzheimer's & Dementia: the Journal of the Alzheimer's Association
https://www.readbyqxmd.com/read/28728756/circulating-soluble-urokinase-plasminogen-activator-receptor-levels-and-peripheral-arterial-disease-outcomes
#7
Ayman Samman Tahhan, Salim S Hayek, Pratik Sandesara, Jamal Hajjari, Muhammad Hammadah, Wesley T O'Neal, Heval M Kelli, Ayman Alkhoder, Nima Ghasemzadeh, Yi-An Ko, Hiroshi Aida, Mohamad Mazen Gafeer, Naser Abdelhadi, Kareem Hosny Mohammed, Keyur Patel, Shipra Arya, Jochen Reiser, Viola Vaccarino, Laurence Sperling, Arshed Quyyumi
BACKGROUND AND AIMS: Circulating soluble urokinase plasminogen activator receptor (suPAR) is a marker of immune activation associated with atherosclerosis. Whether suPAR levels are associated with prevalent peripheral arterial disease (PAD) and its adverse outcomes remains unknown and is the aim of the study. METHODS: SuPAR levels were measured in 5810 patients (mean age 63 years, 63% male, 77% with obstructive coronary artery disease [CAD]) undergoing cardiac catheterization...
June 8, 2017: Atherosclerosis
https://www.readbyqxmd.com/read/28726693/nitrogen-removal-in-a-shallow-maturation-pond-with-sludge-accumulated-during-10-years-of-operation-in-brazil
#8
V A J Rodrigues, E F A Mac Conell, D F C Dias, M von Sperling, J C de Araújo, J L Vasel
Accumulated sludge in polishing (maturation) ponds reduces the hydraulic retention time (smaller useful volume), and this could potentially lead to a decrease in performance. However, settled biomass, present in the sediments, can contribute to nitrogen removal by different mechanisms such as nitrification and denitrification. This study investigated the influence of the bottom sludge present in a shallow maturation pond treating the effluent from an anaerobic reactor on the nitrification and denitrification processes...
July 2017: Water Science and Technology: a Journal of the International Association on Water Pollution Research
https://www.readbyqxmd.com/read/28719863/evaluation-of-platelet-adhesion-and-activation-on-polymers-round-robin-study-to-assess-inter-center-variability
#9
S Braune, C Sperling, M F Maitz, U Steinseifer, J Clauser, B Hiebl, S Krajewski, H P Wendel, F Jung
The regulatory agencies provide recommendations rather than protocols or standard operation procedures for the hemocompatibility evaluation of novel materials e.g. for cardiovascular applications. Thus, there is a lack of specifications with regard to test setups and procedures. As a consequence, laboratories worldwide perform in vitro assays under substantially different test conditions, so that inter-laboratory and inter-study comparisons are impossible. Here, we report about a prospective, randomized and double-blind multicenter trial which demonstrates that standardization of in vitro test protocols allows a reproducible assessment of platelet adhesion and activation from fresh human platelet rich plasma as possible indicators of the thrombogenicity of cardiovascular implants...
July 3, 2017: Colloids and Surfaces. B, Biointerfaces
https://www.readbyqxmd.com/read/28697559/depressive-symptoms-and-tau-accumulation-in-the-inferior-temporal-lobe-and-entorhinal-cortex-in-cognitively-normal-older-adults-a-pilot-study
#10
Jennifer R Gatchel, Nancy J Donovan, Joseph J Locascio, Aaron P Schultz, J Alex Becker, Jasmeer Chhatwal, Kathryn V Papp, Rebecca E Amariglio, Dorene M Rentz, Deborah Blacker, Reisa A Sperling, Keith A Johnson, Gad A Marshall
BACKGROUND: Depressive symptoms are common in older adults and associated with increased morbidity and cognitive decline. These symptoms occur during preclinical and prodromal stages of Alzheimer's disease (AD), but their relationship to tau, one of the main AD proteinopathies, is poorly understood. OBJECTIVE: The objective of this study was to investigate the cross-sectional association between depressive symptoms and cerebral tau [18F T807 (also known as 18F-AV-1451) tau positron emission tomography (PET) imaging] in cognitively normal (CN) older adults...
2017: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/28673897/an-aggregate-biomarker-risk-score-predicts-high-risk-of-near-term-myocardial-infarction-and-death-findings-from-bari-2d-bypass-angioplasty-revascularization-investigation-2-diabetes
#11
Nima Ghasemzadeh, Maria M Brooks, Helen Vlachos, Regina Hardison, Sergey Sikora, Laurence Sperling, Arshed A Quyyumi, Stephen E Epstein
BACKGROUND: In a previous study, we found that a biomarker risk score (BRS) comprised of C-reactive protein, fibrin-degradation products, and heat shock protein-70 predicts risk of myocardial infarction and death in coronary artery disease patients. We sought to: (1) validate the BRS in the independent BARI 2D (Bypass Angioplasty Revascularization Investigation 2 Diabetes) cohort, (2) investigate whether 1 year of intensive medical therapy is associated with improved BRS, and (3) elucidate whether an altered BRS parallels altered risk...
July 3, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/28656019/characterization-of-elite-suppressors-cell-associated-hiv-1-mrna-at-baseline-and-with-t-cell-activation%C3%A2-%C3%A2
#12
Christopher W Pohlmyer, C Korin Bullen, Alyssa R Martin, Gregory M Laird, Stanley U Chioma, Victoria E K Walker-Sperling, Joel N Blankson
Objective: Elite Controllers or Suppressors (ES) are patients who control HIV replication without antiretroviral therapy. In this study, we compared baseline and inducible HIV-1 mRNA levels in CD4+ T cells from ES and chronic progressors (CPs) receiving suppressive antiretroviral therapy. Methods: We quantified basal levels of cell associated HIV-1 mRNA in CD4+ T cells isolated from CPs and ES. Additionally, we measured the fold upregulation of intracellular HIV-mRNA after stimulation of CD4+ T cells with phorbol 12-myristate 13-acetate (PMA) and ionomycin, and quantified the amount of HIV-mRNA levels released into culture supernatant...
June 2017: Yale Journal of Biology and Medicine
https://www.readbyqxmd.com/read/28616381/anosognosia-for-memory-deficits-in-mild-cognitive-impairment-insight-into-the-neural-mechanism-using-functional-and-molecular-imaging
#13
Patrizia Vannini, Bernard Hanseeuw, Catherine E Munro, Rebecca E Amariglio, Gad A Marshall, Dorene M Rentz, Alvaro Pascual-Leone, Keith A Johnson, Reisa A Sperling
Anosognosia, or loss of insight of memory deficits, is a common and striking symptom in Alzheimer's disease (AD). Previous findings in AD dementia patients suggest that anosognosia is due to both functional metabolic changes within cortical midline structures involved in self-referential processes, as well as functional disconnection between these regions. The present study aims to extend these findings by investigating the neural correlates of anosognosia in the prodromal stage of AD. Here, we used regional brain metabolism (resting state 18-F fluorodeoxyglucose positron emission tomography (FDG-PET)) to unravel the metabolic correlates of anosognosia in subjects with amnestic mild cognitive impairment (aMCI) and subsequently resting state functional magnetic resonance imaging (rs-fMRI) to investigate the intrinsic connectivity disruption between brain regions...
2017: NeuroImage: Clinical
https://www.readbyqxmd.com/read/28592457/functional-network-integrity-presages-cognitive-decline-in-preclinical-alzheimer-disease
#14
Rachel F Buckley, Aaron P Schultz, Trey Hedden, Kathryn V Papp, Bernard J Hanseeuw, Gad Marshall, Jorge Sepulcre, Emily E Smith, Dorene M Rentz, Keith A Johnson, Reisa A Sperling, Jasmeer P Chhatwal
OBJECTIVE: To examine the utility of resting-state functional connectivity MRI (rs-fcMRI) measurements of network integrity as a predictor of future cognitive decline in preclinical Alzheimer disease (AD). METHODS: A total of 237 clinically normal older adults (aged 63-90 years, Clinical Dementia Rating 0) underwent baseline β-amyloid (Aβ) imaging with Pittsburgh compound B PET and structural and rs-fcMRI. We identified 7 networks for analysis, including 4 cognitive networks (default, salience, dorsal attention, and frontoparietal control) and 3 noncognitive networks (primary visual, extrastriate visual, motor)...
July 4, 2017: Neurology
https://www.readbyqxmd.com/read/28590378/increasing-body-mass-index-is-associated-with-worse-outcomes-after-shoulder-arthroplasty
#15
Eric R Wagner, Matthew T Houdek, Cathy Schleck, William S Harmsen, Joaquin Sanchez-Sotelo, Robert Cofield, John W Sperling, Bassem T Elhassan
BACKGROUND: Although obesity is associated with increased complication rates after lower-extremity arthroplasty, there is a relative paucity of studies examining the effect of body mass index (BMI) on shoulder arthroplasty. The purpose of this investigation was to evaluate the effect of BMI on implant survival and the rate of complications after shoulder arthroplasty. METHODS: Using an institutional total joint registry, 4,567 consecutive shoulder arthroplasty cases from 1970 to 2013 were studied...
June 7, 2017: Journal of Bone and Joint Surgery. American Volume
https://www.readbyqxmd.com/read/28554264/health-variables-are-informative-in-screening-for-mild-cognitive-impairment-among-elderly-african-americans
#16
Siny Tsang, Scott A Sperling, Moon-Ho Park, Ira M Helenius, Ishan C Williams, Carol Manning
To aid primary care providers in identifying people at increased risk for cognitive decline, we explored the relative importance of health and demographic variables in detecting potential cognitive impairment using the Mini-Mental State Examination (MMSE). Participants were 94 older African Americans coming to see their primary care physicians for reasons other than cognitive complaints. Education was strongly associated with cognitive functioning. Among those with at least 9 years of education, patients with more vascular risk factors were at greater risk for mild cognitive impairment...
May 1, 2017: Journal of Applied Gerontology: the Official Journal of the Southern Gerontological Society
https://www.readbyqxmd.com/read/28504297/elemental-bioimaging-by-means-of-la-icp-oes-investigation-of-the-calcium-sodium-and-potassium-distribution-in-tobacco-plant-stems-and-leaf-petioles
#17
G M Thyssen, M Holtkamp, H Kaulfürst-Soboll, C A Wehe, M Sperling, A von Schaewen, U Karst
Laser ablation-inductively coupled plasma-optical emission spectroscopy (LA-ICP-OES) is presented as a valuable tool for elemental bioimaging of alkali and earth alkali elements in plants. Whereas LA-ICP-OES is commonly used for micro analysis of solid samples, laser ablation-inductively coupled plasma-mass spectrometry (LA-ICP-MS) has advanced to the gold standard for bioimaging. However, especially for easily excitable and ubiquitous elements such as alkali and earth alkali elements, LA-ICP-OES holds some advantages regarding simultaneous detection, costs, contamination, and user-friendliness...
June 21, 2017: Metallomics: Integrated Biometal Science
https://www.readbyqxmd.com/read/28491280/ar2-a-novel-automatic-muscle-artifact-reduction-software-method-for-ictal-eeg-interpretation-validation-and-comparison-of-performance-with-commercially-available-software
#18
Shennan Aibel Weiss, Ali A Asadi-Pooya, Sitaram Vangala, Stephanie Moy, Dale H Wyeth, Iren Orosz, Michael Gibbs, Lara Schrader, Jason Lerner, Christopher K Cheng, Edward Chang, Rajsekar Rajaraman, Inna Keselman, Perdro Churchman, Christine Bower-Baca, Adam L Numis, Michael G Ho, Lekha Rao, Annapoorna Bhat, Joanna Suski, Marjan Asadollahi, Timothy Ambrose, Andres Fernandez, Maromi Nei, Christopher Skidmore, Scott Mintzer, Dawn S Eliashiv, Gary W Mathern, Marc R Nuwer, Michael Sperling, Jerome Engel, John M Stern
Objective: To develop a novel software method (AR2) for reducing muscle contamination of ictal scalp electroencephalogram (EEG), and validate this method on the basis of its performance in comparison to a commercially available software method (AR1) to accurately depict seizure-onset location. Methods: A blinded investigation used 23 EEG recordings of seizures from 8 patients. Each recording was uninterpretable with digital filtering because of muscle artifact and processed using AR1 and AR2 and reviewed by 26 EEG specialists...
2017: F1000Research
https://www.readbyqxmd.com/read/28488693/icos-protects-against-mortality-from-acute-lung-injury-through-activation-of-il-5-ilc2s
#19
C L Hrusch, S T Manns, D Bryazka, J Casaos, C A Bonham, M R Jaffery, K M Blaine, K A M Mills, P A Verhoef, A O Adegunsoye, J W Williams, M Y Tjota, T V Moore, M E Strek, I Noth, A I Sperling
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease causing irreversible lung scarring and loss of pulmonary function. IPF Patients suffer from a high rate of pulmonary infections and acute exacerbations of disease that further contribute to pulmonary decline. Low expression of the inducible T-cell costimulatory molecule (ICOS) in peripheral blood mononuclear cells predicts decreased survival of IPF patients, but the mechanisms by which ICOS protects are unclear. Using a model of bleomycin-induced lung injury and fibrosis, we now demonstrate that ICOS expression enhances survival from lung injury rather than regulating fibrogenesis...
May 10, 2017: Mucosal Immunology
https://www.readbyqxmd.com/read/28474918/detector-independent-verification-of-quantum-light
#20
J Sperling, W R Clements, A Eckstein, M Moore, J J Renema, W S Kolthammer, S W Nam, A Lita, T Gerrits, W Vogel, G S Agarwal, I A Walmsley
We introduce a method for the verification of nonclassical light which is independent of the complex interaction between the generated light and the material of the detectors. This is accomplished by means of a multiplexing arrangement. Its theoretical description yields that the coincidence statistics of this measurement layout is a mixture of multinomial distributions for any classical light field and any type of detector. This allows us to formulate bounds on the statistical properties of classical states...
April 21, 2017: Physical Review Letters
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