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https://www.readbyqxmd.com/read/28729171/exercise-therapy-in-juvenile-idiopathic-arthritis-a-systematic-review-and-meta-analysis
#1
REVIEW
Gregor Kuntze, Colleen Nesbitt, Jackie L Whittaker, Alberto Nettel-Aguirre, Clodagh Toomey, Shane Esau, Patricia K Doyle-Baker, Jena Shank, Julia Brooks, Susanne Benseler, Carolyn A Emery
OBJECTIVE: To conduct a systematic review to evaluate the efficacy of exercise interventions on improving outcomes across domains of functioning and disability in children and adolescents with Juvenile Idiopathic Arthritis (JIA). DATA SOURCES: Seven electronic databases were systematically searched up to December 2016. STUDY SELECTION: Original data, analytic prospective design, physical therapy led exercise intervention evaluation, children and adolescents with JIA, and assessment of functional, structural, activity, participation or quality of life outcomes...
July 17, 2017: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/28692792/clinical-and-molecular-phenotypes-of-low-penetrance-variants-in-nlrp3-diagnostic-and-therapeutic-challenges
#2
J B Kuemmerle-Deschner, D Verma, T Endres, L Broderick, A A de Jesus, F Hofer, N Blank, K Krause, C Rietschel, G Horneff, I Aksentijevich, P Lohse, R Goldbach-Mansky, H M Hoffman, S M Benseler
Background Cryopyrin associated periodic syndrome (CAPS) results from gain-of-function mutations in the NLRP3 gene causing excessive interleukin-1β (IL-1β) release and systemic inflammation. While pathogenic NLRP3 variant phenotypes are well characterized, low-penetrance NLRP3 variants represent a significant clinical challenge. The aims of this study were to determine the clinical phenotype, the in-vitro biological phenotype and the effect of anti-IL-1 treatment in patients with low-penetrance NLRP3 variants...
July 10, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28556862/therapeutic-hypothermia-to-treat-a-newborn-with-perinatal-hypoxic-ischemic-encephalopathy
#3
Todd R Fredricks, Christopher Gibson, Francis Essien, Jeffrey S Benseler
Hypoxic-ischemic encephalopathy is caused by neonatal asphyxia and can lead to mortality or long-term neurodevelopmental morbidity in neonates. Therapeutic hypothermia (TH) is one of the few effective ways to manage mitigating neurologic sequelae. The authors describe the case of a neonate who had a perinatal hypoxic insult and sustained no long-term sequelae after being treated with TH. It is important that osteopathic physicians who provide obstetric and gynecologic, perinatal, and emergency medical care are able to recognize a perinatal hypoxic event, understand the stratification of hypoxic-ischemic encephalopathy risk factors, and implement early TH protocols...
June 1, 2017: Journal of the American Osteopathic Association
https://www.readbyqxmd.com/read/28503078/variations-in-the-clinical-practice-of-physicians-managing-takayasu-arteritis-a-nationwide-survey
#4
Lillian Barra, Patrick Liang, Susanne M Benseler, David A Cabral, Aurore Fifi-Mah, Yueyang Li, Nataliya Milman, Marinka Twilt, Elaine Yacyshyn, Christian Pagnoux
OBJECTIVE: Takayasu arteritis (TAK) is a large vessel vasculitis that predominately affects young women and can cause severe ischemic complications. Given the rarity of TAK, the management of this condition is challenging. We aim to describe current rheumatologist practices for the management of TAK and identify discrepancies and gaps in knowledge. METHODS: An online survey (developed by the Canadian Vasculitis Network and approved by the Canadian Rheumatology Association) containing 48 questions with regard to the diagnosis, monitoring and treatment of TAK was distributed to 495 Canadian adult and pediatric rheu-matologists by email...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28484025/common-general-anesthetic-propofol-impairs-kinesin-processivity
#5
Brandon M Bensel, Stephanie Guzik-Lendrum, Erin M Masucci, Kellie A Woll, Roderic G Eckenhoff, Susan P Gilbert
Propofol is the most widely used i.v. general anesthetic to induce and maintain anesthesia. It is now recognized that this small molecule influences ligand-gated channels, including the GABAA receptor and others. Specific propofol binding sites have been mapped using photoaffinity ligands and mutagenesis; however, their precise target interaction profiles fail to provide complete mechanistic underpinnings for the anesthetic state. These results suggest that propofol and other common anesthetics, such as etomidate and ketamine, may target additional protein networks of the CNS to contribute to the desired and undesired anesthesia end points...
May 23, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28447624/wireless-power-transfer-to-millimeter-sized-gastrointestinal-electronics-validated-in-a-swine-model
#6
Abubakar Abid, Jonathan M O'Brien, Taylor Bensel, Cody Cleveland, Lucas Booth, Brian R Smith, Robert Langer, Giovanni Traverso
Electronic devices placed in the gastrointestinal (GI) tract for prolonged periods have the potential to transform clinical evaluation and treatment. One challenge to the deployment of such gastroresident electronics is the difficulty in powering millimeter-sized electronics devices without using batteries, which compromise biocompatibility and long-term residence. We examined the feasibility of leveraging mid-field wireless powering to transfer power from outside of the body to electronics at various locations along the GI tract...
April 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28371513/early-outcomes-in-children-with-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#7
Kimberly A Morishita, Lakshmi N Moorthy, Joanna M Lubieniecka, Marinka Twilt, Rae S M Yeung, Mary B Toth, Susan Shenoi, Goran Ristic, Susan M Nielsen, Raashid A Luqmani, Suzanne C Li, Tzielan Lee, Erica F Lawson, Mikhail M Kostik, Marisa Klein-Gitelman, Adam M Huber, Aimee O Hersh, Dirk Foell, Melissa E Elder, Barbara A Eberhard, Paul Dancey, Sirirat Charuvanij, Susanne M Benseler, David A Cabral
OBJECTIVE: To characterize the early disease course in childhood-onset antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and the 12-month outcomes in children with AAV. METHODS: Eligible subjects were children entered into the Pediatric Vasculitis Initiative study who were diagnosed before their eighteenth birthday as having granulomatosis with polyangiitis (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (Churg-Strauss), or ANCA-positive pauci-immune glomerulonephritis...
March 28, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28320817/symptomatic-adrenal-suppression-among-children-in-canada
#8
Ellen B Goldbloom, Arati Mokashi, Elizabeth A Cummings, Sharon Abish, Susanne M Benseler, Hien Q Huynh, Wade Watson, Alexandra Ahmet
BACKGROUND: Adrenal suppression (AS) is an under-recognised side effect of glucocorticoid (GC) use. AS may go undetected until a physiological stress precipitates an adrenal crisis. The incidence of AS has not been established. We sought to estimate the minimum national incidence and presenting features of paediatric symptomatic AS. METHODS: Through the established methodology of the Canadian Paediatric Surveillance Program, over 2500 paediatricians were surveyed monthly for 2 years (April 2010-March 2012) to report new cases of symptomatic AS...
April 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28320056/health-related-quality-of-life-in-an-inception-cohort-of-children-with-juvenile-idiopathic-arthritis-a-longitudinal-analysis
#9
Kiem Oen, Jaime Guzman, Brenden Dufault, Lori B Tucker, Natalie J Shiff, Karen Watanabe Duffy, Jennifer J Y Lee, Brian M Feldman, Roberta A Berard, Paul Dancey, Adam M Huber, Rosie Scuccimarri, David A Cabral, Kimberly A Morishita, Suzanne E Ramsey, Alan M Rosenberg, Gilles Boire, Susanne M Benseler, Bianca Lang, Kristin Houghton, Paivi M Miettunen, Gaëlle Chédeville, Deborah M Levy, Alessandra Bruns, Heinrike Schmeling, Elie Haddad, Rae S M Yeung, Ciarán M Duffy
OBJECTIVES: To describe changes in health-related quality of life (HRQL) over time in children with juvenile idiopathic arthritis (JIA), relative to other outcomes; to identify predictors of unfavourable HRQL trajectories. METHODS: Children with JIA in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) cohort were included. The Juvenile Arthritis Quality of Life Questionnaire (JAQQ, a standardized instrument), health-related Quality of My Life (HRQoML, an instrument based on personal valuations) and JIA core variables were completed serially...
March 20, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/27980015/predicting-which-children-with-juvenile-idiopathic-arthritis-will-have-a-severe-disease-course-results-from-the-reacch-out-cohort
#10
Jaime Guzman, Andrew Henrey, Thomas Loughin, Roberta A Berard, Natalie J Shiff, Roman Jurencak, Susanne M Benseler, Lori B Tucker
OBJECTIVE: We studied an inception cohort of children with juvenile idiopathic arthritis (JIA) to (1) identify distinct disease courses based on changes over 5 years in 5 variables prioritized by patients, parents, and clinicians; and (2) estimate the probability of a severe disease course for each child at diagnosis. METHODS: Assessments of quality of life, pain, medication requirements, patient-reported side effects, and active joint counts were scheduled at 0, 6, 12, 18, 24, 36, 48, and 60 months...
February 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/27856796/oral-ultra-long-lasting-drug-delivery-application-toward-malaria-elimination-goals
#11
Andrew M Bellinger, Mousa Jafari, Tyler M Grant, Shiyi Zhang, Hannah C Slater, Edward A Wenger, Stacy Mo, Young-Ah Lucy Lee, Hormoz Mazdiyasni, Lawrence Kogan, Ross Barman, Cody Cleveland, Lucas Booth, Taylor Bensel, Daniel Minahan, Haley M Hurowitz, Tammy Tai, Johanna Daily, Boris Nikolic, Lowell Wood, Philip A Eckhoff, Robert Langer, Giovanni Traverso
Efforts at elimination of scourges, such as malaria, are limited by the logistic challenges of reaching large rural populations and ensuring patient adherence to adequate pharmacologic treatment. We have developed an oral, ultra-long-acting capsule that dissolves in the stomach and deploys a star-shaped dosage form that releases drug while assuming a geometry that prevents passage through the pylorus yet allows passage of food, enabling prolonged gastric residence. This gastric-resident, drug delivery dosage form releases small-molecule drugs for days to weeks and potentially longer...
November 16, 2016: Science Translational Medicine
https://www.readbyqxmd.com/read/27811147/development-of-the-autoinflammatory-disease-damage-index-addi
#12
Nienke M Ter Haar, Kim V Annink, Sulaiman M Al-Mayouf, Gayane Amaryan, Jordi Anton, Karyl S Barron, Susanne M Benseler, Paul A Brogan, Luca Cantarini, Marco Cattalini, Alexis-Virgil Cochino, Fabrizio De Benedetti, Fatma Dedeoglu, Adriana A De Jesus, Ornella Della Casa Alberighi, Erkan Demirkaya, Pavla Dolezalova, Karen L Durrant, Giovanna Fabio, Romina Gallizzi, Raphaela Goldbach-Mansky, Eric Hachulla, Veronique Hentgen, Troels Herlin, Michaël Hofer, Hal M Hoffman, Antonella Insalaco, Annette F Jansson, Tilmann Kallinich, Isabelle Koné-Paut, Anna Kozlova, Jasmin B Kuemmerle-Deschner, Helen J Lachmann, Ronald M Laxer, Alberto Martini, Susan Nielsen, Irina Nikishina, Amanda K Ombrello, Seza Ozen, Efimia Papadopoulou-Alataki, Pierre Quartier, Donato Rigante, Ricardo Russo, Anna Simon, Maria Trachana, Yosef Uziel, Angelo Ravelli, Marco Gattorno, Joost Frenkel
OBJECTIVES: Autoinflammatory diseases cause systemic inflammation that can result in damage to multiple organs. A validated instrument is essential to quantify damage in individual patients and to compare disease outcomes in clinical studies. Currently, there is no such tool. Our objective was to develop a common autoinflammatory disease damage index (ADDI) for familial Mediterranean fever, cryopyrin-associated periodic syndromes, tumour necrosis factor receptor-associated periodic fever syndrome and mevalonate kinase deficiency...
May 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27707729/diagnostic-criteria-for-cryopyrin-associated-periodic-syndrome-caps
#13
REVIEW
Jasmin B Kuemmerle-Deschner, Seza Ozen, Pascal N Tyrrell, Isabelle Kone-Paut, Raphaela Goldbach-Mansky, Helen Lachmann, Norbert Blank, Hal M Hoffman, Elisabeth Weissbarth-Riedel, Boris Hugle, Tilmann Kallinich, Marco Gattorno, Ahmet Gul, Nienke Ter Haar, Marlen Oswald, Fatma Dedeoglu, Luca Cantarini, Susanne M Benseler
Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease entity associated with NLRP3 gene mutations and increased interleukin-1 (IL-1) secretion. Early diagnosis and rapid initiation of IL-1 inhibition prevent organ damage. The aim of the study was to develop and validate diagnostic criteria for CAPS. An innovative process was followed including interdisciplinary team building, item generation: review of CAPS registries, systematic literature review, expert surveys, consensus conferences for item refinement, item reduction and weighting using 1000Minds decision software...
June 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27698103/management-of-juvenile-idiopathic-arthritis-2015-a-position-statement-from-the-pediatric-committee-of-the-canadian-rheumatology-association
#14
EDITORIAL
Tania Cellucci, Jaime Guzman, Ross E Petty, Michelle Batthish, Susanne M Benseler, Janet E Ellsworth, Kristin M Houghton, Claire M A LeBLANC, Adam M Huber, Nadia Luca, Heinrike Schmeling, Natalie J Shiff, Gordon S Soon, Shirley M L Tse
No abstract text is available yet for this article.
October 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/27658465/arterial-dissection-in-childhood-takayasu-arteritis-not-as-rare-as-thought
#15
Florence A Aeschlimann, Lars Grosse-Wortmann, Susanne M Benseler, Ronald M Laxer, Diane Hebert, Rae S M Yeung
BACKGROUND: Arterial vessel wall dissection is a rare, life-threatening and rarely described complication in childhood Takayasu Arteritis (cTA). Prevalence and risk factors for arterial dissection in cTA are unknown. We sought to study the prevalence and analyse risk factors for arterial dissection in cTA. FINDINGS: A single center retrospective review of all children with cTA was performed. Patients with arterial dissection at cTA diagnosis were reported in detail and compared to the remaining single center retrospective cohort of children without dissection...
September 22, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27633024/inter-rater-reliability-of-the-cascade-criteria-challenges-in-classifying-arteriopathies
#16
Timothy J Bernard, Lauren A Beslow, Marilyn J Manco-Johnson, Jennifer Armstrong-Wells, Richard Boada, David Weitzenkamp, Amanda Hollatz, Sharon Poisson, Catherine Amlie-Lefond, Warren Lo, Gabrielle deVeber, Neil A Goldenberg, Michael M Dowling, E Steve Roach, Heather J Fullerton, Susanne M Benseler, Lori C Jordan, Adam Kirton, Rebecca N Ichord
BACKGROUND AND PURPOSE: There are limited data about the reliability of subtype classification in childhood arterial ischemic stroke, an issue that prompted the IPSS (International Pediatric Stroke Study) to develop the CASCADE criteria (Childhood AIS Standardized Classification and Diagnostic Evaluation). Our purpose was to determine the CASCADE criteria's reliability in a population of children with stroke. METHODS: Eight raters from the IPSS reviewed neuroimaging and clinical records of 64 cases (16 cases each) randomly selected from a prospectively collected cohort of 113 children with arterial ischemic stroke and classified them using the CASCADE criteria...
October 2016: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/27469076/vascular-imaging-outcomes-of-childhood-primary-angiitis-of-the-central-nervous-system
#17
Jorina Elbers, Derek Armstrong, Ivanna Yau, Susanne Benseler
BACKGROUND: Inflammation affecting cerebral blood vessels is a common cause of stroke in children. Arterial abnormalities on vascular imaging are an important risk factor for stroke recurrence. We aimed to describe the vascular imaging outcomes in children with primary angiitis of the central nervous system after 12 months and identify factors associated with vascular progression and stroke recurrence. METHODS: We retrospectively analyzed clinical and neuroimaging data from the BrainWorks Registry of children with large-vessel primary angiitis of the central nervous system...
October 2016: Pediatric Neurology
https://www.readbyqxmd.com/read/27112683/central-nervous-system-vasculitis-in-adults-and-children
#18
REVIEW
Marinka Twilt, Susanne M Benseler
Primary angiitis of the central nervous system (PACNS) is an inflammatory brain disease targeting the cerebral blood vessels, leading to a wide spectrum of signs and symptoms, including neurologic deficits, cognitive dysfunction, and psychiatric symptoms. The inflammation could be reversible if diagnosed and treated early. The diagnosis requires the careful consideration and rapid evaluation of systemic underlying conditions and disease mimics. The differential diagnosis is distinctly different for angiography-positive and -negative PACNS subtypes and differs depending on age, so there is childhood PACNS or adult PACNS...
2016: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/27111558/comparing-presenting-clinical-features-in-48-children-with-microscopic-polyangiitis-to-183-children-who-have-granulomatosis-with-polyangiitis-wegener-s-an-archive-cohort-study
#19
COMPARATIVE STUDY
David A Cabral, Debra L Canter, Eyal Muscal, Kabita Nanda, Dawn M Wahezi, Steven J Spalding, Marinka Twilt, Susanne M Benseler, Sarah Campillo, Sirirat Charuvanij, Paul Dancey, Barbara A Eberhard, Melissa E Elder, Aimee Hersh, Gloria C Higgins, Adam M Huber, Raju Khubchandani, Susan Kim, Marisa Klein-Gitelman, Mikhail M Kostik, Erica F Lawson, Tzielan Lee, Joanna M Lubieniecka, Deborah McCurdy, Lakshmi N Moorthy, Kimberly A Morishita, Susan M Nielsen, Kathleen M O'Neil, Andreas Reiff, Goran Ristic, Angela B Robinson, Angelyne Sarmiento, Susan Shenoi, Mary B Toth, Heather A Van Mater, Linda Wagner-Weiner, Jennifer E Weiss, Andrew J White, Rae S M Yeung
OBJECTIVE: To uniquely classify children with microscopic polyangiitis (MPA), to describe their demographic characteristics, presenting clinical features, and initial treatments in comparison to patients with granulomatosis with polyangiitis (Wegener's) (GPA). METHODS: The European Medicines Agency (EMA) classification algorithm was applied by computation to categorical data from patients recruited to the ARChiVe (A Registry for Childhood Vasculitis: e-entry) cohort, with the data censored to November 2015...
October 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27099088/aortic-root-aneurism-found-in-a-42-year-old-epitomizes-the-importance-of-auscultation-in-routine-exams
#20
Todd R Fredricks, Jeffrey S Benseler
BACKGROUND: Bicuspid aortic valve disease (BAV) is the most common cardiac valve pathology. BAV is associated with aortic root disorders. The literature has few case reports identifying this condition during routine physical exam. CASE REPORT: A 42-yr-old military reservist flight medic presented for his annual military flight physical. He was found to have a faint cardiac murmur. His past family and medical history were remarkable for familial essential hypertension and being told at age 9 that he had a "murmur...
May 2016: Aerospace Medicine and Human Performance
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