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https://www.readbyqxmd.com/read/29174857/the-utility-of-collaterals-as-a-biomarker-in-pediatric-unilateral-intracranial-arteriopathy
#1
Jorina Elbers, Derek Armstrong, Susanne M Benseler, Nomazulu Dlamini, Gary K Steinberg, Kristen W Yeom
BACKGROUND: Intracranial arteriopathies are frequent causes of pediatric stroke and important risk factors for stroke recurrence. Without tissue diagnosis, vascular imaging is relied upon to identify the underlying etiology and prognosis. We hypothesized that children with unilateral intracranial arteriopathy with lenticulostriate collaterals would demonstrate distinct vascular outcomes compared with children without collaterals. METHODS: We retrospectively identified children with unilateral intracranial arteriopathy from two institutions...
September 20, 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/29166923/childhood-takayasu-arteritis-disease-course-and-response-to-therapy
#2
Florence A Aeschlimann, Simon W M Eng, Shehla Sheikh, Ronald M Laxer, Diane Hebert, Damien Noone, Marinka Twilt, Christian Pagnoux, Susanne M Benseler, Rae S M Yeung
BACKGROUND: Takayasu arteritis (TAK) is a large vessel vasculitis that rarely affects children. Data on childhood TAK are scarce. The aim of this study was to analyze the presenting features, course and outcome of children with TAK, compare efficacy of treatment regimens and identify high-risk factors for adverse outcome. METHODS: A single-center cohort study of consecutive children fulfilling the EULAR/PRINTO/PReS criteria for childhood TAK between 1986 and 2015 was performed...
November 22, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29051063/defining-optimal-permeant-characteristics-for-ultrasound-mediated-gastrointestinal-delivery
#3
Carl M Schoellhammer, Yiyun Chen, Cody Cleveland, Daniel Minahan, Taylor Bensel, June Y Park, Sarah Saxton, Young-Ah Lucy Lee, Lucas Booth, Robert Langer, Giovanni Traverso
Ultrasound-mediated drug delivery in the gastrointestinal (GI) tract is a bourgeoning area of study. Localized, low-frequency ultrasound has recently been shown to enable significant enhancement in delivery of a broad set of active pharmaceutical ingredients including small molecules, proteins, and nucleic acids without any formulation or encapsulation of the therapeutic. Traditional chemical formulations are typically required to protect, stabilize, and enable the successful delivery of a given therapeutic...
October 16, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/29021237/recommendations-for-collaborative-paediatric-research-including-biobanking-in-europe-a-single-hub-and-access-point-for-paediatric-rheumatology-in-europe-share-initiative
#4
Jasmin B Kuemmerle-Deschner, Sandra Hansmann, Nico M Wulffraat, Sebastiaan J Vastert, Kristien Hens, Jordi Anton, Tadej Avcin, Alberto Martini, Isabelle Koné-Paut, Yosef Uziel, Angelo Ravelli, Carine Wouters, David Shaw, Seza Özen, Andreas Eikelberg, Berent J Prakken, Nicolino Ruperto, Gerd Horneff, Tamas Constantin, Michael W Beresford, Marijn Sikken, Helen E Foster, Iris Haug, Sabrina Schuller, Christine Jägle, Susanne M Benseler
Innovative research in childhood rheumatic diseases mandates international collaborations. However, researchers struggle with significant regulatory heterogeneity; an enabling European Union (EU)-wide framework is missing. The aims of the study were to systematically review the evidence for best practice and to establish recommendations for collaborative research. The Paediatric Rheumatology European Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) project enabled a scoping review and expert discussion, which then informed the systematic literature review...
October 11, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28870142/validation-of-the-standardized-universal-pain-evaluations-for-rheumatology-providers-for-children-and-youth-super-kidz
#5
Nadia J Luca, Jennifer N Stinson, Brian M Feldman, Susanne M Benseler, Dorcas Beaton, Sarah Campillo, Claire LeBlanc, Margaret van Wyk, Ahmed M Bayoumi
Study Design Longitudinal observational clinimetric study with repeated measures. Background No validated multidimensional pain measure for children and youth with juvenile idiopathic arthritis exists. Objective To determine the test-retest reliability, construct validity, and responsiveness of English and French versions of the Standardized Universal Pain Evaluations for Rheumatology Providers for Children and Youth (SUPER-KIDZ). Methods Measurement properties of the SUPER-KIDZ (older child, younger child, and parent versions) were prospectively evaluated in patients (aged 4 to 18 years) with juvenile idiopathic arthritis at 2 centers...
October 2017: Journal of Orthopaedic and Sports Physical Therapy
https://www.readbyqxmd.com/read/28830457/growth-and-weight-gain-in-children-with-juvenile-idiopathic-arthritis-results-from-the-reacch-out-cohort
#6
Jaime Guzman, Tristan Kerr, Leanne M Ward, Jinhui Ma, Kiem Oen, Alan M Rosenberg, Brian M Feldman, Gilles Boire, Kristin Houghton, Paul Dancey, Rosie Scuccimarri, Alessandra Bruns, Adam M Huber, Karen Watanabe Duffy, Natalie J Shiff, Roberta A Berard, Deborah M Levy, Elizabeth Stringer, Kimberly Morishita, Nicole Johnson, David A Cabral, Maggie Larché, Ross E Petty, Ronald M Laxer, Earl Silverman, Paivi Miettunen, Anne-Laure Chetaille, Elie Haddad, Lynn Spiegel, Stuart E Turvey, Heinrike Schmeling, Bianca Lang, Janet Ellsworth, Suzanne E Ramsey, Johannes Roth, Sarah Campillo, Susanne Benseler, Gaëlle Chédeville, Rayfel Schneider, Shirley M L Tse, Roxana Bolaria, Katherine Gross, Debbie Feldman, Bonnie Cameron, Roman Jurencak, Jean Dorval, Claire LeBlanc, Claire St Cyr, Michele Gibbon, Rae S M Yeung, Ciarán M Duffy, Lori B Tucker
BACKGROUND: With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort. METHODS: Canadian children newly-diagnosed with JIA 2005-2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years. These measurements were used to calculate mean age- and sex-standardized Z-scores, and estimate prevalence and cumulative incidence of growth impairments, and the impact of disease activity and corticosteroids on growth...
August 22, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28807967/l%C3%A2-acide-ac%C3%A3-tylsalicylique-pour-les-enfants-atteints-de-la-maladie-de-kawasaki
#7
Teeranai Sakulchit, Susanne M Benseler, Ran D Goldman
No abstract text is available yet for this article.
August 2017: Canadian Family Physician Médecin de Famille Canadien
https://www.readbyqxmd.com/read/28807954/acetylsalicylic-acid-for-children-with-kawasaki-disease
#8
Teeranai Sakulchit, Susanne M Benseler, Ran D Goldman
Question A 7-year-old child in my office was recently discharged from the hospital after receiving intravenous immunoglobulin for Kawasaki disease. Should I continue treatment with acetylsalicylic acid (ASA), and if so, what is the appropriate dose? Answer The role of ASA for Kawasaki disease during the acute febrile phase has recently been called into question. According to several studies, ASA might reduce the duration of fever but it does not appear to directly reduce the incidence of coronary artery complications...
August 2017: Canadian Family Physician Médecin de Famille Canadien
https://www.readbyqxmd.com/read/28729171/exercise-therapy-in-juvenile-idiopathic-arthritis-a-systematic-review-and-meta-analysis
#9
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 in 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 November 16, 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 18, 2017: Archives of Physical Medicine and Rehabilitation
https://www.readbyqxmd.com/read/28692792/clinical-and-molecular-phenotypes-of-low-penetrance-variants-of-nlrp3-diagnostic-and-therapeutic-challenges
#10
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
OBJECTIVE: Cryopyrin-associated periodic syndromes (CAPS) result from gain-of-function mutations in the NLRP3 gene, which causes excessive release of interleukin-1β (IL-1β) and systemic inflammation. While pathogenetic 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 biologic phenotype, and the effect of anti-IL-1 treatment in patients with low-penetrance NLRP3 variants...
November 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28556862/therapeutic-hypothermia-to-treat-a-newborn-with-perinatal-hypoxic-ischemic-encephalopathy
#11
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
#12
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
#13
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
#14
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
#15
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...
July 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28320817/symptomatic-adrenal-suppression-among-children-in-canada
#16
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
#17
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
#18
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
#19
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
#20
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
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