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https://www.readbyqxmd.com/read/29778428/status-dystonicus-due-to-missense-variant-in-arx-diagnosis-and-management
#1
Kathleen M Gorman, Heather Cary, Laura Gaffney, Eva Forman, Dympna Waldron, Fowzy Al-Delami, Bryan J Lynch, Mary D King, Nicholas M Allen
Movement disorders are increasingly identified in infantile encephalopathies due to single gene disorders (e.g. SCN2A, CDKL5, ARX). The associated movement disorder can be challenging to recognise and treat. We report a 2 year-old boy with a background history of Ohtahara syndrome due to a missense variant in ARX (the aristaless-related homeobox gene) who subsequently developed status dystonicus. ARX is a transcription factor that plays a critical role in cortical neuronal development and is associated with a range of important neurodevelopmental disorders depending on the site of the pathogenic variant...
May 3, 2018: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/29775072/exploring-the-patients-experiences-of-living-with-a-subcutaneous-implantable-cardioverter-defibrillator
#2
Jacqueline Forman, Jennifer Baumbusch, Heather Jackson, Jen Lindenberg, Alyssa Shook, Jamil Bashir
BACKGROUND: The implantable cardioverter defibrillator provides effective treatment for the prevention of sudden cardiac arrest but significant risks associated with transvenous implantation persist. The subcutaneous implantable cardioverter defibrillator has proven to be an alternative and innovative treatment option for select patients to mitigate these risks. Obtaining the patients' perspectives can provide clinicians with essential information to guide implant selection, procedural decision-making, and support patient management...
May 1, 2018: European Journal of Cardiovascular Nursing
https://www.readbyqxmd.com/read/29769444/glioblastoma-targeted-cd4-car-t-cells-mediate-superior-antitumor-activity
#3
Dongrui Wang, Brenda Aguilar, Renate Starr, Darya Alizadeh, Alfonso Brito, Aniee Sarkissian, Julie R Ostberg, Stephen J Forman, Christine E Brown
Chimeric antigen receptor-modified (CAR-modified) T cells have shown promising therapeutic effects for hematological malignancies, yet limited and inconsistent efficacy against solid tumors. The refinement of CAR therapy requires an understanding of the optimal characteristics of the cellular products, including the appropriate composition of CD4+ and CD8+ subsets. Here, we investigated the differential antitumor effect of CD4+ and CD8+ CAR T cells targeting glioblastoma-associated (GBM-associated) antigen IL-13 receptor α2 (IL13Rα2)...
May 17, 2018: JCI Insight
https://www.readbyqxmd.com/read/29764969/cardiovascular-phenotype-and-prognosis-of-patients-with-heart-failure-induced-by-cancer-therapy
#4
Wilson Nadruz, Erin West, Morten Sengeløv, Gabriela L Grove, Mário Santos, John D Groarke, Daniel E Forman, Brian Claggett, Hicham Skali, Anju Nohria, Amil M Shah
OBJECTIVE: This study compared the clinical features, cardiac structure and function evaluated by echocardiography, cardiopulmonary response to exercise and long-term clinical outcomes between patients with heart failure (HF) induced by cancer therapy (CTHF) and heart failure not induced by cancer therapy (NCTHF). METHODS: We evaluated 75 patients with CTHF and 894 with NCTHF who underwent clinically indicated cardiopulmonary exercise testing, and followed these individuals for a median of 4...
May 15, 2018: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/29764112/human-rights-treaties-are-an-important-part-of-the-international-health-instrumentariam-comment-on-the-legal-strength-of-international-health-instruments-what-it-brings-to-global-health-governance
#5
Lisa Forman
In their commentary, Haik Nikogosian and Ilona Kickbusch argue for the necessity of new binding international legal instruments for health to address complex health determinants and offer a cogent analysis of the implications of such treaties for future global health governance. Yet in doing so they pay no attention to the existing instrumentarium of international legally binding treaties relevant to health, in the form of human rights treaties. International human rights law has entrenched individual entitlements and state obligations in relation to individual and public health through iterative human rights treaties since 1946...
October 2, 2017: International Journal of Health Policy and Management
https://www.readbyqxmd.com/read/29753309/endometriosis-and-adenomyosis-are-associated-with-increased-risk-of-preterm-delivery-and-a-small-for-gestational-age-child-a-systematic-review-and-meta-analysis
#6
Mette Rahbek Bruun, Linn Håkonsen Arendt, Axel Forman, Cecilia Høst Ramlau-Hansen
INTRODUCTION: The objective of this systematic review and meta-analysis was to evaluate the risk of preterm delivery and having a small for gestational age child in women with endometriosis and adenomyosis compared to women without these two diseases. MATERIAL AND METHODS: Studies on endometriosis or adenomyosis and risk of preterm delivery and/or small for gestational age were included. The systematic search was conducted for all published articles in PubMed and Embase published from 1950 to 2017 using specific search terms...
May 12, 2018: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/29753158/melphalan-based-reduced-intensity-conditioning-is-associated-with-favorable-disease-control-and-acceptable-toxicities-in-patients-older-than-70-with-hematologic-malignancies-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#7
Monzr M Al Malki, Nitya Nathwani, Dongyun Yang, Saro Armenian, Sanjeet Dadwal, Jaroslava Salman, Sally Mokhtari, Thai Cao, Karamjeet Sandhu, Michelle Rouse, Matthew Mei, Haris Ali, Pablo Parker, Joseph Alvarnas, Eileen Smith, Margaret O Donnell, Guido Marcucci, David Snyder, Auayporn Nademanee, Stephen J Forman, Anthony Stein, Ryotaro Nakamura
Allogeneic hematopoietic stem cell transplantation (AlloHCT) is offered increasingly to elderly patients with hematologic malignancies. However, outcome data in those who are 70 years or older are limited, and no standard conditioning regimen has been established for this population. In this retrospective study, we evaluated the outcome of 53 consecutive patients aged 70 years and older who underwent AlloHCT with melphalan (Mel)-based reduced-intensity conditioning (RIC) at City of Hope. Engraftment was prompt, with median time to neutrophil engraftment of 15 days...
May 9, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29750771/stemming-the-tide-of-abuse-clinicians-role-in-the-metoo-movement
#8
Sarah Pitts, Sara Forman
No abstract text is available yet for this article.
May 9, 2018: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/29747836/multimorbidity-in-older-adults-with-cardiovascular-disease
#9
REVIEW
Daniel E Forman, Mathew S Maurer, Cynthia Boyd, Ralph Brindis, Marcel E Salive, Frances McFarland Horne, Susan P Bell, Terry Fulmer, David B Reuben, Susan Zieman, Michael W Rich
Multimorbidity occurs in adults of all ages, but the number and complexity of comorbid conditions commonly increase with advancing age such that cardiovascular disease (CVD) in older adults typically occurs in a context of multimorbidity. Current clinical practice and research mainly target single disease-specific care that does not embrace the complexities imposed by concurrent conditions. In this paper, emerging concepts regarding CVD in combination with multimorbidity are reviewed, including recommendations for incorporating multimorbidity into clinical decision making, critical knowledge gaps, and research priorities to optimize care of complex older patients...
May 15, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29741802/deviant-peer-behavior-and-adolescent-delinquency-protective-effects-of-inhibitory-control-planning-or-decision-making
#10
J Benjamin Hinnant, Alissa B Forman-Alberti
We examined relations between adolescent perceptions of deviant peer behavior and delinquency as moderated by inhibitory control, planning, and decision making in the National Institute of Child Health and Human Development (NICHD) Study of Early Child Care and Youth Development at age 15 (N = 991). Adolescents reported perceptions of deviant peer behavior. Inhibitory control, planning, and decision making were assessed behaviorally. Delinquency was evaluated with a latent variable comprised of parent-guardian perceptions of adolescent delinquency and adolescent self-reports...
May 9, 2018: Journal of Research on Adolescence: the Official Journal of the Society for Research on Adolescence
https://www.readbyqxmd.com/read/29733063/adrenergic-signaling-molecular-complexes
#11
Rocío Alcántara-Hernández, Aurelio Hernández-Méndez
Adrenaline and noradrenaline bind to membrane receptors of the superfamily of G protein-coupled receptors (GPCR) in target cells, where they modulate physiological responses such as metabolism, vasoconstriction, vasodilation and proliferation. Alteration in their function is associated with conditions such as hypertension, benign prostatic hyperplasia and cardiac hypertrophy. In response to adrenaline, receptors form signaling complexes, which enables adrenergic action to be specific, rapid and efficient. These signaling complexes or signalosomes are composed of kinases, phosphatases, and adapter and scaffold proteins, which together modulate the receptor function...
2018: Gaceta Médica de México
https://www.readbyqxmd.com/read/29728514/positron-emission-tomography-of-adoptively-transferred-chimeric-antigen-receptor-t-cells-with-zirconium-89-oxine
#12
Michael Ryan Weist, Renate Starr, Brenda Aguilar, Junie Chea, Joshua Miles, Erasmus Poku, Ethan Gerdts, Xin Yang, Saul Priceman, Stephen Forman, David Colcher, Christine Brown, John Shively
CAR T cell therapy is a promising clinical approach for reducing tumor progression and prolonging patient survival. However, improvements in both the safety and potency of CAR T cell therapy demand quantitative imaging techniques to determine the distribution of cells following adoptive transfer. The purpose of this study is to optimize zirconium-89-oxine (89 Zr-oxine) labeling of chimeric antigen receptor (CAR) T cells and evaluate positron emission tomography (PET) as a platform for imaging adoptively transferred CAR T cells...
May 4, 2018: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
https://www.readbyqxmd.com/read/29728105/identifying-risk-factors-for-l-hermitte-s-sign-after-imrt-for-head-and-neck-cancer
#13
Hannah M Laidley, David J Noble, Gill C Barnett, Julia R Forman, Amy M Bates, Richard J Benson, Sarah J Jefferies, Rajesh Jena, Neil G Burnet
BACKGROUND: L'Hermitte's sign (LS) after chemoradiotherapy for head and neck cancer appears related to higher spinal cord doses. IMRT plans limit spinal cord dose, but the incidence of LS remains high. METHODS: One hundred seventeen patients treated with TomoTherapy™ between 2008 and 2015 prospectively completed a side-effect questionnaire (VoxTox Trial Registration: UK CRN ID 13716). Baseline patient and treatment data were collected. Radiotherapy plans were analysed; mean and maximum spinal cord dose and volumes receiving 10, 20, 30 and 40 Gy were recorded...
May 4, 2018: Radiation Oncology
https://www.readbyqxmd.com/read/29715502/a-multimodal-investigation-of-impulsivity-as-a-moderator-of-the-relation-between-momentary-elevations-in-negative-internal-states-and-subsequent-dietary-lapses
#14
Stephanie M Manasse, Rebecca J Crochiere, Diane H Dallal, Edward W Lieber, Leah M Schumacher, Ross D Crosby, Meghan L Butryn, Evan M Forman
Suboptimal outcomes from behavioral weight loss (BWL) treatments are partially attributable to accumulated instances of non-adherence to dietary prescriptions (i.e., dietary lapses). Results identifying negative internal triggers for dietary lapses are inconsistent, potentially due to individual differences that impact how individuals respond to cues. Impulsivity is one factor that likely influences reactivity to internal states. We examined three dimensions of impulsivity (delay discounting, inhibitory control, and negative urgency) as moderators of the relation between affective and physical states and subsequent dietary lapses at the beginning of BWL...
April 28, 2018: Appetite
https://www.readbyqxmd.com/read/29709705/aging-attenuates-redox-adaptive-homeostasis-and-proteostasis-in-female-mice-exposed-to-traffic-derived-nanoparticles-vehicular-smog
#15
Laura C D Pomatto, Mayme Cline, Nicholas Woodward, Payam Pakbin, Constantinos Sioutas, Todd E Morgan, Caleb E Finch, Henry Jay Forman, Kelvin J A Davies
Environmental toxicants are catalysts for protein damage, aggregation, and the aging process. Fortunately, evolution selected adaptive homeostasis as a system to mitigate such damage by expanding the normal capacity to cope with toxic stresses. Little is known about the subcellular degradative responses to proteins oxidatively damaged by air pollution. To better understand the impact of environmental toxicants upon the adaptive homeostatic response, female C57BL/6 mice were exposed for 10 weeks to filtered air or reaerosolized vehicular-derived nano-scale particulate matter (nPM), at which point tissues from young (6 month) and middle-aged (21 month) mice were studied...
April 27, 2018: Free Radical Biology & Medicine
https://www.readbyqxmd.com/read/29708978/frequency-and-distribution-of-152-genetic-disease-variants-in-over-100-000-mixed-breed-and-purebred-dogs
#16
Jonas Donner, Heidi Anderson, Stephen Davison, Angela M Hughes, Julia Bouirmane, Johan Lindqvist, Katherine M Lytle, Balasubramanian Ganesan, Claudia Ottka, Päivi Ruotanen, Maria Kaukonen, Oliver P Forman, Neale Fretwell, Cynthia A Cole, Hannes Lohi
Knowledge on the genetic epidemiology of disorders in the dog population has implications for both veterinary medicine and sustainable breeding. Limited data on frequencies of genetic disease variants across breeds exists, and the disease heritage of mixed breed dogs remains poorly explored to date. Advances in genetic screening technologies now enable comprehensive investigations of the canine disease heritage, and generate health-related big data that can be turned into action. We pursued population screening of genetic variants implicated in Mendelian disorders in the largest canine study sample examined to date by examining over 83,000 mixed breed and 18,000 purebred dogs representing 330 breeds for 152 known variants using a custom-designed beadchip microarray...
April 30, 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29699619/domain-management-approach-to-heart-failure-in-the-geriatric-patient-present-and-future
#17
REVIEW
Eiran Z Gorodeski, Parag Goyal, Scott L Hummel, Ashok Krishnaswami, Sarah J Goodlin, Linda L Hart, Daniel E Forman, Nanette K Wenger, James N Kirkpatrick, Karen P Alexander
Heart failure (HF) is a quintessential geriatric cardiovascular condition, with more than 50% of hospitalizations occurring in adults age 75 years or older. In older patients, HF is closely linked to processes inherent to aging, which include cellular and structural changes to the myocardium, vasculature, and skeletal muscle. In addition, HF cannot be considered in isolation of physical functioning, or without the social, psychological, and behavioral dimensions of illness. The role of frailty, depression, cognitive impairment, nutrition, and goals of care are each uniquely relevant to the implementation and success of medical therapy...
May 1, 2018: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/29697758/pathways-contributors-and-correlates-of-functional-limitation-across-specialties-workshop-summary
#18
Stephen B Kritchevsky, Daniel E Forman, Kathryn Callahan, E Wesley Ely, Kevin P High, Frances McFarland, Eliseo J Pérez-Stable, Kenneth Schmader, Stephanie Studenski, John Williams, Susan Zieman, Jack M Guralnik
Traditional clinical care models focus on the measurement and normalization of individual organ systems and de-emphasize aspects of health related to the integration of physiologic systems. Measures of physical, cognitive and sensory, and psychosocial or emotional function predict important health outcomes like death and disability independently from the severity of a specific disease, cumulative co-morbidity, or disease severity measures. A growing number of clinical scientists in several subspecialties are exploring the utility of functional assessment to predict complication risk, indicate stress resistance, inform disease screening approaches and risk factor interpretation, and evaluate care...
April 25, 2018: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
https://www.readbyqxmd.com/read/29696017/a-future-for-autologous-hematopoietic-stem-cell-transplantation-in-type-1-diabetes
#19
Kayleigh M van Megen, Ernst-Jan T van 't Wout, Stephen J Forman, Bart O Roep
No abstract text is available yet for this article.
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29694450/focused-ethnography-of-diagnosis-in-academic-medical-centers
#20
Vineet Chopra, Molly Harrod, Suzanna Winter, Jane Forman, Martha Quinn, Sarah Krein, Karen E Fowler, Hardeep Singh, Sanjay Saint
BACKGROUND: Approaches of trainees to diagnosis in teaching hospitals are poorly understood. Identifying cognitive and system-based barriers and facilitators to diagnosis may improve diagnosis in these settings. METHODS: We conducted a focused ethnography of trainees at 2 academic medical centers to understand the barriers and facilitators to diagnosis. Field notes regarding the diagnostic process (eg, information gathering, integration and interpretation, working diagnosis) and the work system (eg, team members, organization, technology and tools, physical environment, tasks) were recorded...
April 25, 2018: Journal of Hospital Medicine: An Official Publication of the Society of Hospital Medicine
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