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Wilson disease

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https://www.readbyqxmd.com/read/28211072/the-diagnosis-of-pancreatic-mucinous-cystic-neoplasm-and-associated-adenocarcinoma-in-males-an-eight-institution-study-of-349-patients-over-15-years
#1
Cecilia G Ethun, Lauren M Postlewait, Mia R McInnis, Nipun Merchant, Alexander Parikh, Kamran Idrees, Chelsea A Isom, William Hawkins, Ryan C Fields, Matthew Strand, Sharon M Weber, Clifford S Cho, Ahmed Salem, Robert C G Martin, Charles R Scoggins, David Bentrem, Hong J Kim, Jacquelyn Carr, Syed A Ahmad, Daniel E Abbott, Gregory Wilson, David A Kooby, Shishir K Maithel
BACKGROUND: Per WHO, 2000 classification, pancreatic mucinous cystic neoplasms (MCN) are defined by presence of ovarian stroma, and are primarily located in the pancreatic body/tail of females. The incidence of MCN and associated malignancy in males, since, standardization of MCN diagnostic-criteria is unknown. METHODS: MCN resections from 2000 to 2014 at eight institutions of the Central-Pancreas-Consortium were included, and divided into early (2000-2007) and late (2008-2014) time-periods...
February 17, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28208074/cd47-limits-antibody-dependent-phagocytosis-against-non-malignant-b-cells
#2
Sandra Gallagher, Sean Turman, Kristen Lekstrom, Susan Wilson, Ronald Herbst, Yue Wang
Recent studies have demonstrated the importance of CD47 in protecting malignant B cells from antibody dependent cellular phagocytosis (ADCP). Combined treatment of anti-CD47 and -CD20 antibodies synergistically augment elimination of tumor B cells in xenograft mouse models. This has led to the development of novel reagents that can potentially enhance killing of malignant B cells in patients. B cell depleting therapy is also a promising treatment for autoimmune patients. In the current study, we aimed to investigate whether or not CD47 protects non-malignant B cells from ADCP...
February 13, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28207896/rna-seq-analysis-of-chikungunya-virus-infection-and-identification-of-granzyme-a-as-a-major-promoter-of-arthritic-inflammation
#3
Jane A C Wilson, Natalie A Prow, Wayne A Schroder, Jonathan J Ellis, Helen E Cumming, Linden J Gearing, Yee Suan Poo, Adam Taylor, Paul J Hertzog, Francesca Di Giallonardo, Linda Hueston, Roger Le Grand, Bing Tang, Thuy T Le, Joy Gardner, Suresh Mahalingam, Pierre Roques, Phillip I Bird, Andreas Suhrbier
: Chikungunya virus (CHIKV) is an arthritogenic alphavirus causing epidemics of acute and chronic arthritic disease. Herein we describe a comprehensive RNA-Seq analysis of feet and lymph nodes at peak viraemia (day 2 post infection), acute arthritis (day 7) and chronic disease (day 30) in the CHIKV adult wild-type mouse model. Genes previously shown to be up-regulated in CHIKV patients were also up-regulated in the mouse model. CHIKV sequence information was also obtained with up to ≈8% of the reads mapping to the viral genome; however, no adaptive viral genome changes were apparent...
February 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28206976/lipid-and-alzheimer-s-disease-genes-associated-with-healthy-aging-and-longevity-in-healthy-oldest-old
#4
Lauren C Tindale, Stephen Leach, John J Spinelli, Angela R Brooks-Wilson
Several studies have found that long-lived individuals do not appear to carry lower numbers of common disease-associated variants than ordinary people; it has been hypothesized that they may instead carry protective variants. An intriguing type of protective variant is buffering variants that protect against variants that have deleterious effects. We genotyped 18 variants in 15 genes related to longevity or healthy aging that had been previously reported as having a gene-gene interaction or buffering effect...
February 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28205040/clinical-phenotyping-of-heart-failure-with-biomarkers-current-and-future-perspectives
#5
REVIEW
Vichai Senthong, Jennifer L Kirsop, W H Wilson Tang
INTRODUCTION: Heart failure (HF) is a complex clinical syndrome with diverse risk factors and etiologies, differing underlying pathophysiology, and large phenotypic heterogeneity. RECENT FINDINGS: Advances in imaging techniques coupled with clinical trials that targeted only in those with impaired left ventricular ejection fraction (LVEF) have largely shaped the current management strategy for HF that focuses predominantly in patients with systolic HF. In contrast, there are no effective treatments for HF with preserved ejection fraction (HFpEF)...
February 15, 2017: Current Heart Failure Reports
https://www.readbyqxmd.com/read/28199755/incidence-and-risk-factor-of-hemorrhagic-cystitis-after-allogeneic-transplantation-with-fludarabine-busulfan-and-anti-thymocyte-globulin-myeloablative-conditioning
#6
Wilson Lam, Jan Storek, Haocheng Li, Michelle Geddes, Andrew Daly
BACKGROUND: Hemorrhagic cystitis (HC) is a complication of allogeneic stem cell transplantation (SCT), associated with factors such as BK polyomavirus reactivation, age, conditioning regimen, and presence of graft-versus-host disease (GVHD). The incidence and impact of HC in patients receiving fludarabine (Flu), busulfan (Bu), and anti-thymocyte globulin (ATG) conditioning is unknown. METHODS: We conducted a case-control study of patients undergoing SCT at our center between January 1, 2003 and Dec 31, 2012, to determine the incidence of HC and its effect on patient outcomes including overall survival (OS), relapse, non-relapse mortality, GVHD, and healthcare resource use...
February 15, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28199053/the-effect-of-tonsillectomy-on-the-morbidity-from-recurrent-tonsillitis
#7
C M Douglas, K Lang, W M Whitmer, J A Wilson, K Mackenzie
BACKGROUND: Tonsillitis is a common condition with an incidence in UK general practice of 37 per 1000 population a year(1) . Recurrent tonsillitis results in significant morbidity and impacts on individuals' quality of life. This study assesses the morbidity and quality of life of adults with recurrent tonsillitis, and the impact of surgical intervention on their health state. OBJECTIVES: 1.To describe disease specific and global quality of life for adults with recurrent tonsillitis six months after tonsillectomy, using two instruments: the Health Impact of Throat Problems (HITP) and EuroQol-visual analogue scale (EQ-VAS) questionnaire...
February 15, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28198956/evaluating-pharmaceutical-waste-disposal-in-pediatric-units
#8
Maria Angélica Randoli de Almeida, Ana Maria Miranda Martins Wilson, Maria Angélica Sorgini Peterlini
OBJECTIVE: To verify the disposal of pharmaceutical waste performed in pediatric units. METHOD: A descriptive and observational study conducted in a university hospital. The convenience sample consisted of pharmaceuticals discarded during the study period. Handling and disposal during preparation and administration were observed. Data collection took place at pre-established times and was performed using a pre-validated instrument. RESULTS: 356 drugs disposals were identified (35...
November 2016: Revista da Escola de Enfermagem da U S P
https://www.readbyqxmd.com/read/28197923/viral-interplay-with-the-host-sumoylation-system
#9
Van G Wilson
Viruses have evolved elaborate means to regulate diverse cellular pathways in order to create a cellular environment that facilitates viral survival and reproduction. This includes enhancing viral macromolecular synthesis and assembly, as well as preventing antiviral responses, including intrinsic, innate, and adaptive immunity. There are numerous mechanisms by which viruses mediate their effects on the host cell, and this includes targeting various cellular post-translational modification systems, including sumoylation...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28197914/sumoylation-in-development-and-differentiation
#10
Adeline F Deyrieux, Van G Wilson
Tissue morphogenesis is a fascinating aspect of both developmental biology and regeneration of certain adult organs, and timely control of cellular differentiation is a key to these processes. During development, events interrupting cellular differentiation and leading to organ failure are embryonic lethal; likewise, perturbation of differentiation in regenerating tissues leads to dysfunction and disease. At the molecular level, cellular differentiation is orchestrated by a well-coordinated cascade of transcription factors (TFs) and chromatin remodeling complexes that drive gene expression...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28197532/using-global-gene-expression-to-discriminate-thin-melanomas-with-poor-outcomes
#11
Zachary Hothem, Andrew Bayci, Bryan J Thibodeau, Billie E Ketelsen, Laura E Fortier, Alison F Uzieblo, Diane Cosner, Kristin Totoraitis, Richard D Keidan, George D Wilson
Most melanomas present as thin lesions (≤1.0 mm) with a good prognosis; however, a small percentage of patients with thin lesions experience recurrence or metastasis. The aim of our study was to identify a distinct pattern of gene expression within thin melanomas known to have eventually metastasized to regional lymph nodes or distant sites compared with those that followed the typical course with good response to wide local excision alone. Patients who were disease-free for a minimum of 10 y served as controls (n = 10) to the experimental group who developed metastasis (n = 9)...
2017: Molecular & Cellular Oncology
https://www.readbyqxmd.com/read/28197071/chemosensitivity-of-u251-cells-to-the-co-treatment-of-d-penicillamine-and-copper-possible-implications-on-wilson-disease-patients
#12
Meghri Katerji, Kassem Barada, Mustapha Jomaa, Firas Kobeissy, Ahmad-Kareem Makkawi, Wassim Abou-Kheir, Julnar Usta
D-Penicillamine (PA), a copper chelator, and one of the recommended drugs for treatment of Wilson disease (WD) has been reported to worsen the symptoms of patients with neurologic presentations. However, the cause of this paradoxical response has not been fully elucidated and requires further investigations. Accordingly, we have studied the in vitro effect of Copper (Cu) and/or PA treatment on human glioblastoma U251 cells as an in vitro model of Cu cytotoxicity. Treatment of U251 cells with either Cu or PA exerted no significant effect on their morphology, viability or ROS level...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28197013/adolescent-onset-wilson-s-disease-misdiagnosed-as-psychosis
#13
Kishore Dudani, Ram Kumar Solanki, Pradeep Sharma, Dharamdeep Singh
No abstract text is available yet for this article.
October 2016: Indian Journal of Psychiatry
https://www.readbyqxmd.com/read/28195551/b-type-natriuretic-peptide-and-echocardiography-reflect-volume-changes-during-pregnancy
#14
Janet M Burlingame, Kelly Yamasato, Hyeong Jun Ahn, Todd Seto, W H Wilson Tang
OBJECTIVE: To evaluate B-type natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) and cardiac structure and function in normal women through pregnancy and the postpartum. METHODS: In this prospective observational study, we obtained serial transthoracic echocardiograms, BNP, and NT-proBNP at seven intervals from 6 weeks' gestation through 12 months postpartum. Women with hypertension or cardiac disease were excluded. Using 6-12 months postpartum as reference for non-pregnant levels, echocardiogram measurements and BNP/NT-proBNP were compared over time using linear mixed models with Tukey-Kramer adjustment for multiple comparisons...
February 14, 2017: Journal of Perinatal Medicine
https://www.readbyqxmd.com/read/28194132/molecular-imaging-markers-to-track-huntington-s-disease-pathology
#15
REVIEW
Heather Wilson, Rosa De Micco, Flavia Niccolini, Marios Politis
Huntington's disease (HD) is a progressive, monogenic dominant neurodegenerative disorder caused by repeat expansion mutation in the huntingtin gene. The accumulation of mutant huntingtin protein, forming intranuclear inclusions, subsequently leads to degeneration of medium spiny neurons in the striatum and cortical areas. Genetic testing can identify HD gene carriers before individuals develop overt cognitive, psychiatric, and chorea symptoms. Thus, HD gene carriers can be studied in premanifest stages to understand and track the evolution of HD pathology...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28193762/teaching-neuroimages-ocular-findings-in-a-patient-with-wilson-disease-and-venous-sinus-thrombosis
#16
Ivana Vodopivec, Emer McGrath, Henrikas Vaitkevicius
No abstract text is available yet for this article.
February 14, 2017: Neurology
https://www.readbyqxmd.com/read/28192686/the-down-syndrome-brain-in-the-presence-and-absence-of-fibrillar-%C3%AE-amyloidosis
#17
Tiina Annus, Liam R Wilson, Julio Acosta-Cabronero, Arturo Cardenas-Blanco, Young T Hong, Tim D Fryer, Jonathan P Coles, David K Menon, Shahid H Zaman, Anthony J Holland, Peter J Nestor
People with Down syndrome (DS) have a neurodevelopmentally distinct brain and invariably developed amyloid neuropathology by age 50. This cross-sectional study aimed to provide a detailed account of DS brain morphology and the changes occuring with amyloid neuropathology. Forty-six adults with DS underwent structural and amyloid imaging-the latter using Pittsburgh compound B (PIB) to stratify the cohort into PIB-positive (n = 19) and PIB-negative (n = 27). Age-matched controls (n = 30) underwent structural imaging...
January 17, 2017: Neurobiology of Aging
https://www.readbyqxmd.com/read/28192629/the-effect-of-co-trimoxazole-on-serum-potassium-concentration-safety-evaluation-of-a-randomised-controlled-trial
#18
Wei Yee Chan, Allan B Clark, Andrew M Wilson, Yoon K Loke
AIMS: Co-trimoxazole maintains a well-established role in the treatment of Pneumocystis jirovecii and Toxoplasma gondii, as well as urinary tract infections. Observational studies report hyperkalemia associated with co-trimoxazole which may stem from an amiloride-like potassium sparing effect. Our study reports on changes in serum potassium on patients without acute infections, and the influence of concomitant anti-kaliuretic drugs on this effect. METHODS: Post-hoc analysis of a randomised controlled trial in patients with interstitial lung disease who were assigned to placebo or 960 mg twice daily co-trimoxazole...
February 13, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28190636/correlation-of-18-f-fluorodeoxyglucose-positron-emission-tomography-parameters-with-patterns-of-disease-progression-in-locally-advanced-pancreatic-cancer-after-definitive-chemoradiotherapy
#19
J M Wilson, S Mukherjee, T B Brunner, M Partridge, M A Hawkins
AIMS: A proportion of patients with pancreatic cancer never develop metastatic disease. We evaluated a role for (18)F-fluorodeoxyglucose positron emission tomography (FDG-PET) in identifying a subset of patients with locally advanced pancreatic cancer (LAPC) who never develop metastatic disease and only experience local disease and may therefore benefit from local treatment intensification. MATERIAL AND METHODS: Patients with histologically confirmed LAPC entered a single-centre phase II study of definitive upfront chemoradiotherapy (CRT)...
February 9, 2017: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/28188030/unveiling-of-hiv-dynamics-among-transgender-women-a-respondent-driven-sampling-study-in-rio-de-janeiro-brazil
#20
Beatriz Grinsztejn, Emilia M Jalil, Laylla Monteiro, Luciane Velasque, Ronaldo I Moreira, Ana Cristina F Garcia, Cristiane V Castro, Alícia Krüger, Paula M Luz, Albert Y Liu, Willi McFarland, Susan Buchbinder, Valdilea G Veloso, Erin C Wilson
BACKGROUND: The burden of HIV in transgender women (transwomen) in Brazil remains unknown. We aimed to estimate HIV prevalence among transwomen in Rio de Janeiro and to identify predictors of newly diagnosed HIV infections. METHODS: We recruited transwomen from Rio de Janeiro, Brazil, by respondent-driven sampling. Eligibility criteria were self-identification as transwomen, being 18 years of age or older, living in Rio de Janeiro or its metropolitan area, and having a valid peer recruitment coupon...
February 7, 2017: Lancet HIV
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