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Wilsons disease

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https://www.readbyqxmd.com/read/28650899/evaluation-of-a-digital-behavioral-counseling-program-for-reducing-risk-factors-for-chronic-disease-in-a-workforce
#1
Mark G Wilson, Cynthia M Castro Sweet, Michael D Edge, Erica N Madero, Megan McGuire, Megan Pilsmaker, Dan Carpenter, Scott Kirschner
OBJECTIVE: To evaluate a digitally delivered, intensive behavioral counseling program for a workforce at risk for obesity-related chronic disease. METHODS: Employees were offered a digital health program modeled after the diabetes prevention program (DPP). Annual workforce health assessments were used to examine changes in chronic disease risk factors between participants (n = 634) relative to a matched comparison group (n = 1268). RESULTS: Overall, employees were gaining an average of 3...
June 23, 2017: Journal of Occupational and Environmental Medicine
https://www.readbyqxmd.com/read/28649610/evaluation-of-the-safety-and-immunomodulatory-effects-of-sargramostim-in-a-randomized-double-blind-phase-1-clinical-parkinson-s-disease-trial
#2
Howard E Gendelman, Yuning Zhang, Pamela Santamaria, Katherine E Olson, Charles R Schutt, Danish Bhatti, Bhagya Laxmi Dyavar Shetty, Yaman Lu, Katherine A Estes, David G Standaert, Elizabeth Heinrichs-Graham, LuAnn Larson, Jane L Meza, Matthew Follett, Erica Forsberg, Gary Siuzdak, Tony W Wilson, Carolyn Peterson, R Lee Mosley
A potential therapeutic role for immune transformation in Parkinson's disease evolves from more than a decade of animal investigations demonstrating regulatory T cell (Treg) nigrostriatal neuroprotection. To bridge these results to human disease, we conducted a randomized, placebo-controlled double-blind phase 1 trial with a well-studied immune modulator, sargramostim (granulocyte-macrophage colony-stimulating factor). We enrolled 17 age-matched non-Parkinsonian subjects as non-treated controls and 20 Parkinson's disease patients...
2017: NPJ Parkinson's Disease
https://www.readbyqxmd.com/read/28649159/initial-simulated-ffr-investigation-using-flow-measurements-in-patient-specific-3d-printed-coronary-phantoms
#3
Lauren Shepard, Kelsey Sommer, Richard Izzo, Alexander Podgorsak, Michael Wilson, Zaid Said, Frank J Rybicki, Dimitrios Mitsouras, Stephen Rudin, Erin Angel, Ciprian N Ionita
PURPOSE: Accurate patient-specific phantoms for device testing or endovascular treatment planning can be 3D printed. We expand the applicability of this approach for cardiovascular disease, in particular, for CT-geometry derived benchtop measurements of Fractional Flow Reserve, the reference standard for determination of significant individual coronary artery atherosclerotic lesions. MATERIALS AND METHODS: Coronary CT Angiography (CTA) images during a single heartbeat were acquired with a 320×0...
February 11, 2017: Proceedings of SPIE
https://www.readbyqxmd.com/read/28648967/glans-necrosis-following-penile-prosthesis-implantation-prevention-and-treatment-suggestions
#4
Steven K Wilson, Cesar Mora-Estaves, Paulo Egydio, David Ralph, Mohamad Habous, Christopher Love, Ahmad Shamsodini, Robert Valenzuela, Faysal A Yafi
OBJECTIVE: To examine possible etiology and treatment outcomes in 21 patients with glans necrosis following penile prosthesis implantation. METHODS: Glans necrosis typically presented with a dusky glans on first post-operative day following prosthesis implantation. RESULTS: The blood supply to the glans penis consists of the dorsal arteries and the terminal branches of the spongiosal arteries. Using this cohort, we compiled pre-operative comorbidities and adjunctive surgical maneuvers that might compromise glans vascularity leading to glans necrosis...
June 22, 2017: Urology
https://www.readbyqxmd.com/read/28648536/outcomes-of-elective-aortic-hemiarch-reconstruction-for-aneurysmal-disease-in-the-elderly
#5
Arman Kilic, George J Arnaoutakis, Joseph E Bavaria, Ibrahim Sultan, Nimesh D Desai, Prashanth Vallabhajosyula, Matthew L Williams, Rita K Milewski, Wilson Y Szeto
BACKGROUND: This study evaluated outcomes of elective aortic hemiarch reconstruction for aneurysmal disease in the elderly. METHODS: Patients undergoing elective aortic hemiarch reconstruction for aneurysmal disease at a single institution between 2009 and 2014 were retrospectively reviewed. Patients were stratified into nonelderly (aged less than 75 years) versus elderly (aged 75 years or more). Outcomes included operative mortality and morbidity. RESULTS: In all, 629 patients (95 elderly; 15%) were included...
June 22, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28648494/characteristics-and-prevalence-of-wilson-s-disease-a-2013-observational-population-based-study-in-france
#6
Aurélia Poujois, France Woimant, Solène Samson, Pascal Chaine, Nadège Girardot-Tinant, Philippe Tuppin
BACKGROUND AND AIMS: Only a few epidemiological studies on the incidence and prevalence of Wilson's disease (WD) have been performed to date, and the results vary widely according to the reports. The aim of the study was to investigate the prevalence, ambulatory care and treatments of patients with WD in France. METHODS: Among the 58 million general health scheme beneficiaries (86% of the French population), people managed for WD in 2013 were identified using hospitalisation diagnosis in 2011-2013 or specific long-term disease status with a 100% reimbursement for specific healthcare in 2013...
June 22, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28647728/impact-of-five-tobacco-endgame-strategies-on-future-smoking-prevalence-population-health-and-health-system-costs-two-modelling-studies-to-inform-the-tobacco-endgame
#7
Frederieke S van der Deen, Nick Wilson, Christine L Cleghorn, Giorgi Kvizhinadze, Linda J Cobiac, Nhung Nghiem, Tony Blakely
OBJECTIVE: There is growing international interest in advancing 'the tobacco endgame'. We use New Zealand (Smokefree goal for 2025) as a case study to model the impacts on smoking prevalence (SP), health gains (quality-adjusted life-years (QALYs)) and cost savings of (1) 10% annual tobacco tax increases, (2) a tobacco-free generation (TFG), (3) a substantial outlet reduction strategy, (4) a sinking lid on tobacco supply and (5) a combination of 1, 2 and 3. METHODS: Two models were used: (1) a dynamic population forecasting model for SP and (2) a closed cohort (population alive in 2011) multistate life table model (including 16 tobacco-related diseases) for health gains and costs...
June 24, 2017: Tobacco Control
https://www.readbyqxmd.com/read/28647347/differential-activation-of-human-keratinocytes-by-leishmania-spp-causing-localized-or-disseminated-disease
#8
Breanna M Scorza, Mark A Wacker, Kelly Messingham, Peter Kim, Aloysius Klingelhutz, Janet Fairley, Mary E Wilson
All Leishmania spp. parasites are introduced into mammalian skin through a sand fly bite, but different species cause distinct clinical outcomes. Mouse studies suggest early responses are critical determinants of subsequent adaptive immunity in leishmaniasis, yet few studies address the role of keratinocytes, the most abundant cell in the epidermis. We hypothesized Leishmania infection causes keratinocytes to produce immunomodulatory factors that influence the outcome of infection. Incubation of primary or immortalized human keratinocytes with Leishmania infantum or Leishmania major, which cause visceral or cutaneous leishmaniasis, respectively, elicited dramatically different responses...
June 21, 2017: Journal of Investigative Dermatology
https://www.readbyqxmd.com/read/28645223/decline-in-literacy-and-incident-ad-dementia-among-community-dwelling-older-persons
#9
Lei Yu, Robert S Wilson, S Duke Han, Sue Leurgans, David A Bennett, Patricia A Boyle
OBJECTIVE: To quantify longitudinal change in financial and health literacy and examine the associations of declining literacy with incident Alzheimer's disease (AD) dementia and mild cognitive impairment (MCI). METHOD: Data came from 799 participants of an ongoing cohort study. Literacy was measured using a battery of 32 questions. Clinical diagnoses were made annually following uniform structured procedures. The associations of declining literacy with incident AD dementia and MCI were tested using a joint model for longitudinal and time-to-event data...
June 1, 2017: Journal of Aging and Health
https://www.readbyqxmd.com/read/28644185/a-simple-ultrasound-score-for-the-accurate-detection-of-inflammatory-activity-in-crohn-s-disease
#10
Kerri L Novak, Gilaad G Kaplan, Remo Panaccione, Elnaz Ehteshami Afshar, Divine Tanyingoh, Mark Swain, Amelia Kellar, Stephanie Wilson
BACKGROUND: Ultrasound is accurate in the detection of Crohn's disease. Our aim was to identify ultrasound parameters contributing to inflammatory disease activity, develop a simple score, and validate this score prospectively. METHODS: This study comprised 2 single-center investigations. The first was a retrospective study on a population that had received colonoscopies (as a gold-standard diagnostic) within 60 days of ultrasound. The second was a prospective study on 2 populations: patients requiring induction with adalimumab and patients on adalimumab maintenance therapy...
June 20, 2017: Inflammatory Bowel Diseases
https://www.readbyqxmd.com/read/28642861/reported-systems-changes-and-sustainability-perceptions-of-three-state-departments-of-health-implementing-multi-faceted-evidence-based-fall-prevention-efforts
#11
Matthew Lee Smith, Ellen C Schneider, Imani N Byers, Tiffany E Shubert, Ashley D Wilson, Samuel D Towne, Marcia G Ory
Although the concepts of systems change and sustainability are not new, little is known about the factors associated with systems change sustaining multi-state, multi-level fall prevention efforts. This exploratory study focuses on three State Departments of Health (DOH) that were awarded 5-year funding from the Centers for Disease Control and Prevention to simultaneously implement four separate yet related evidence-based fall prevention initiatives at the clinical, community, and policy level. The purpose of this study was to examine changes in partnerships and collaborative activities that occurred to accomplish project goals (examining changes in the context of "before funding" and "after funding was received")...
2017: Frontiers in Public Health
https://www.readbyqxmd.com/read/28642858/pathological-fracture-of-femoral-neck-leading-to-a-diagnosis-of-wilson-s-disease-a-case-report-and-review-of-literature
#12
Nishit Bhatnagar, Purushotham Lingaiah, Jeetendra Singh Lodhi, Yugal Karkhur
Wilson's disease (WD) is a rare inherited disorder of copper metabolism. It chiefly has hepatic, neurological and ophthalmic manifestations. Although osteoporosis, rickets and early arthritis are common features of WD, they are under-recognized. Musculoskeletal manifestations very rarely lead to diagnosis of the disease. Here we present a case of a 12-year-old girl who presented with a 3-month-old pathological fracture of neck of femur. WD was diagnosed on investigating the cause of the pathological fracture, which was managed by performing a conventional McMurray's intertrochanteric osteotomy...
May 2017: Journal of Bone Metabolism
https://www.readbyqxmd.com/read/28642109/neoaortic-xenoprosthetic-grafts-for-treatment-of-mycotic-aneurysms-and-infected-aortic-grafts
#13
C Anibueze, V Sankaran, U Sadat, K Tan, Y G Wilson, R E Brightwell, M S Delbridge, P W Stather
OBJECTIVE: There is no international consensus regarding the optimum management of infected aortae (mycotic aneurysms, infected aortic grafts). Neoaortoiliac reconstruction has advantages over extra anatomical bypass grafting; however use of autologous vein is associated with venous hypertension and compartment syndrome, alternatively cadaveric homografts are associated with high rates of perianastomotic haemorrhage, limb occlusion and pseudoaneurysm. Arterial repair using xenoprosthetic patches is associated with lower infection rates compared to the use of prosthetic material...
June 19, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28639726/trend-of-centenarian-deaths-in-hong-kong-between-2001-and-2010
#14
Ruby Yu, Wilson Tam, Jean Woo
AIM: To examine the trends in the leading causes of deaths in centenarians in Hong Kong. METHODS: Descriptive analyses of vital statistics data on mortality in Hong Kong from 2001 to 2010 were carried out. RESULTS: The number of centenarians' deaths increased by 136% in 10 years, from less than 28 men and 166 women in 2001 to 80 men and 378 women in 2010. During the study period, the top leading cause of death was pneumonia, accounting for 33...
June 2017: Geriatrics & Gerontology International
https://www.readbyqxmd.com/read/28639484/detection-test-for-language-impairments-in-adults-and-the-aged-a-new-screening-test-for-language-impairment-associated-with-neurodegenerative-diseases-validation-and-normative-data
#15
Joël Macoir, Marion Fossard, Laurent Lefebvre, Laura Monetta, Antoine Renard, Thi Mai Tran, Maximiliano A Wilson
To date, there is no quick screening test that could be used during routine office visits to accurately assess language disorders in neurodegenerative diseases. To fill this important gap, we developed the Detection Test for Language impairments in Adults and the Aged (DTLA), a quick, sensitive, standardized screening test designed to assess language disorders in adults and the elderly individuals. In Study 1, we describe the development of the DTLA. In Study 2, we report data on the DTLA's validity and reliability...
January 1, 2017: American Journal of Alzheimer's Disease and Other Dementias
https://www.readbyqxmd.com/read/28638514/internal-medicine-residents-ambulatory-management-of-core-geriatric-conditions
#16
Kathryn E Callahan, Lindsay A Wilson, Juliessa M Pavon, James F Lovato, Hal H Atkinson, Jan Busby-Whitehead, Thomas Dalton, Mitchell T Heflin, Patricia Iverson, Janice S Lawlor, Justin Marsden, William P Moran, Ellen Roberts, Jeff D Williamson
BACKGROUND: Adults aged 65 years and older account for more than 33% of annual visits to internal medicine (IM) generalists and specialists. Geriatrics experiences are not standardized for IM residents. Data are lacking on IM residents' continuity experiences with older adults and competencies relevant to their care. OBJECTIVE: To explore patient demographics and the prevalence of common geriatric conditions in IM residents' continuity clinics. METHODS: We collected data on age and sex for all IM residents' active clinic patients during 2011-2012...
June 2017: Journal of Graduate Medical Education
https://www.readbyqxmd.com/read/28633655/inhibition-of-mmp-2-and-mmp-9-decreases-cellular-migration-and-angiogenesis-in-in-vitro-models-of-retinoblastoma
#17
Anderson H Webb, Bradley T Gao, Zachary K Goldsmith, Andrew S Irvine, Nabil Saleh, Ryan P Lee, Justin B Lendermon, Rajini Bheemreddy, Qiuhua Zhang, Rachel C Brennan, Dianna Johnson, Jena J Steinle, Matthew W Wilson, Vanessa M Morales-Tirado
BACKGROUND: Retinoblastoma (Rb) is the most common primary intraocular tumor in children. Local treatment of the intraocular disease is usually effective if diagnosed early; however advanced Rb can metastasize through routes that involve invasion of the choroid, sclera and optic nerve or more broadly via the ocular vasculature. Metastatic Rb patients have very high mortality rates. While current therapy for Rb is directed toward blocking tumor cell division and tumor growth, there are no specific treatments targeted to block Rb metastasis...
June 20, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28631601/viral-genes-and-cellular-markers-associated-with-neurological-complications-during-herpesvirus-infections
#18
Carine L Holz, Rahul K Nelli, M Eilidh Wilson, Lila M Zarski, Walid Azab, Rachel Baumgardner, Nikolaus Osterrieder, Anthony Pease, Liangliang Zhang, Sarah Hession, Lutz S Goehring, Stephen B Hussey, Gisela Soboll Hussey
Despite the importance of neurological disorders associated with herpesviruses, the mechanism by which these viruses influence the central nervous system (CNS) has not been definitively established. Owing to the limitations of studying neuropathogenicity of human herpesviruses in their natural host, many aspects of their pathogenicity and immune response are studied in animal models. Here, we present an important model system that enables studying neuropathogenicity of herpesviruses in the natural host. Equine herpesvirus type 1 (EHV-1) is an alphaherpesvirus that causes a devastating neurological disease (EHV-1 myeloencephalopathy; EHM) in horses...
June 20, 2017: Journal of General Virology
https://www.readbyqxmd.com/read/28630323/role-of-the-cbp-catalytic-core-in-intramolecular-sumoylation-and-control-of-histone-h3-acetylation
#19
Sangho Park, Robyn L Stanfield, Maria A Martinez-Yamout, H Jane Dyson, Ian A Wilson, Peter E Wright
The histone acetyl transferases CREB-binding protein (CBP) and its paralog p300 play a critical role in numerous cellular processes. Dysregulation of their catalytic activity is associated with several human diseases. Previous work has elucidated the regulatory mechanisms of p300 acetyltransferase activity, but it is not known whether CBP activity is controlled similarly. Here, we present the crystal structure of the CBP catalytic core encompassing the bromodomain (BRD), CH2 (comprising PHD and RING), HAT, and ZZ domains at 2...
June 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28630076/what-are-the-primary-limitations-in-b-cell-affinity-maturation-and-how-much-affinity-maturation-can-we-drive-with-vaccination-is-affinity-maturation-a-self-defeating-process-for-eliciting-broad-protection
#20
Christopher T Stamper, Patrick C Wilson
Vaccinations are one of the greatest success stories of modern medicine, saving millions of lives since their widespread adoption. However, several diseases continue to elude highly effective vaccination strategies. Chief among these are human immunodeficiency virus (HIV) and influenza (flu), both of which will require vaccines that can guide the creation of highly mutated, broadly neutralizing antibodies (bnAbs). The generation of bnAbs is hindered by our inability to effectively drive the high levels of affinity maturation required to achieve them in a large number of cells...
June 19, 2017: Cold Spring Harbor Perspectives in Biology
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