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Benseler, s

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...
November 3, 2016: Annals of the Rheumatic Diseases
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
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
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
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
J B Kümmerle-Deschner, J Thomas, S M Benseler
BACKGROUND: The primary vasculitides are rare conditions in childhood. The most common disease subtypes are Schönlein-Henoch purpura and Kawasaki's syndrome, which frequently have a self-limiting course. In the majority of vasculitides, the etiology remains unknown. Environmental exposure, including infections, is suspected to trigger an autoinflammatory response in predisposed individuals. GOAL: The aim of this review is to present the various aspects of childhood vasculitis...
December 2015: Zeitschrift Für Rheumatologie
Lucy McGeoch, Marinka Twilt, Leilani Famorca, Volodko Bakowsky, Lillian Barra, Susan M Benseler, David A Cabral, Simon Carette, Gerald P Cox, Navjot Dhindsa, Christine S Dipchand, Aurore Fifi-Mah, Michelle Goulet, Nader Khalidi, Majed M Khraishi, Patrick Liang, Nataliya Milman, Christian A Pineau, Heather N Reich, Nooshin Samadi, Kam Shojania, Regina Taylor-Gjevre, Tanveer E Towheed, Judith Trudeau, Michael Walsh, Elaine Yacyshyn, Christian Pagnoux
OBJECTIVE: The Canadian Vasculitis research network (CanVasc) is composed of physicians from different medical specialties and researchers with expertise in vasculitis. One of its aims is to develop recommendations for the diagnosis and management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) in Canada. METHODS: Diagnostic and therapeutic questions were developed based on the results of a national needs assessment survey. A systematic review of existing non-Canadian recommendations and guidelines for the diagnosis and management of AAV and studies of AAV published after the 2009 European League Against Rheumatism/European Vasculitis Society recommendations (publication date: January 2009) until November 2014 was performed in the Medline database, Cochrane library, and main vasculitis conference proceedings...
January 2016: Journal of Rheumatology
Gregory S Day, Harald Prüss, Susanne M Benseler, Tara A Paton, Andrew D Paterson, Danielle M Andrade
OBJECTIVE: To determine whether distinct single nucleotide polymorphisms (SNPs) within the glutamate receptor ionotropic NMDA 1 gene (GRIN1) are associated with NMDA receptor (NMDAR) encephalitis and whether these same variants are associated with variability in the clinical presentation and course of affected patients. METHODS: We performed clinical follow-up on 48 patients with NMDAR encephalitis and NMDAR autoantibodies detected in serum or CSF. All RefSeq GRIN1 coding exons were sequenced in 39 Caucasian-European patients, and the frequencies of SNPs were compared with those of an ethnically similar population using a case-control study design...
October 2015: Neurology® Neuroimmunology & Neuroinflammation
Nienke M ter Haar, Marlen Oswald, Jerold Jeyaratnam, Jordi Anton, Karyl S Barron, Paul A Brogan, Luca Cantarini, Caroline Galeotti, Gilles Grateau, Veronique Hentgen, Michael Hofer, Tilmann Kallinich, Isabelle Kone-Paut, Helen J Lachmann, Huri Ozdogan, Seza Ozen, Ricardo Russo, Anna Simon, Yosef Uziel, Carine Wouters, Brian M Feldman, Sebastiaan J Vastert, Nico M Wulffraat, Susanne M Benseler, Joost Frenkel, Marco Gattorno, Jasmin B Kuemmerle-Deschner
: Autoinflammatory diseases are characterised by fever and systemic inflammation, with potentially serious complications. Owing to the rarity of these diseases, evidence-based guidelines are lacking. In 2012, the European project Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate regimens for the management of children and young adults with rheumatic diseases, facilitating the clinical practice of paediatricians and (paediatric) rheumatologists...
September 2015: Annals of the Rheumatic Diseases
J Bichler, S M Benseler, M Krumrey-Langkammerer, J-P Haas, B Hügle
OBJECTIVES: Chronic anterior uveitis is a serious complication of juvenile idiopathic arthritis (JIA); disease flares are highly associated with loss of vision. Leflunomide (LEF) is used successfully for JIA joint disease but its effectiveness in uveitis has not been determined. The aim of this study was to determine whether LEF improves flare rates of uveitis in JIA patients compared to preceding methotrexate (MTX) therapy. METHOD: A single-centre retrospective study of consecutive children with JIA and chronic anterior uveitis was performed...
2015: Scandinavian Journal of Rheumatology
Jaime Guzman, Kiem Oen, Adam M Huber, Karen Watanabe Duffy, Gilles Boire, Natalie Shiff, Roberta A Berard, Deborah M Levy, Elizabeth Stringer, Rosie Scuccimarri, Kimberly Morishita, Nicole Johnson, David A Cabral, Alan M Rosenberg, Maggie Larché, Paul Dancey, Ross E Petty, Ronald M Laxer, Earl Silverman, Paivi Miettunen, Anne-Laure Chetaille, Elie Haddad, Kristin Houghton, Lynn Spiegel, Stuart E Turvey, Heinrike Schmeling, Bianca Lang, Janet Ellsworth, Suzanne E Ramsey, Alessandra Bruns, Johannes Roth, Sarah Campillo, Susanne Benseler, Gaëlle Chédeville, Rayfel Schneider, Shirley M L Tse, Roxana Bolaria, Katherine Gross, Brian Feldman, 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
OBJECTIVE: To describe probabilities and characteristics of disease flares in children with juvenile idiopathic arthritis (JIA) and to identify clinical features associated with an increased risk of flare. METHODS: We studied children in the Research in Arthritis in Canadian Children emphasizing Outcomes (ReACCh-Out) prospective inception cohort. A flare was defined as a recurrence of disease manifestations after attaining inactive disease and was called significant if it required intensification of treatment...
June 2016: Annals of the Rheumatic Diseases
Damien G Noone, Marinka Twilt, Wesley N Hayes, Paul S Thorner, Susanne Benseler, Ronald M Laxer, Rulan S Parekh, Diane Hebert
BACKGROUND AND OBJECTIVES: A proposed histopathologic classification for ANCA-associated GN is predictive of long-term renal outcome in adult populations. This study sought to validate this system in a pediatric cohort. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This was a retrospective, single-center, cohort study of 40 children diagnosed and followed until their transition to adult care at one institution between 1987 and 2012. Renal biopsy specimens were reviewed by a pathologist blinded to patient outcome and were classified using the new histopathologic classification system of focal, crescentic, mixed, and sclerotic groups...
October 7, 2014: Clinical Journal of the American Society of Nephrology: CJASN
Rachana P Hasija, Earl D Silverman, Stephanie Cho, Lillia Fung, Susanne M Benseler, Bonnie Cameron, Brian M Feldman, Ronald M Laxer, Deborah M Levy, Rayfel Schneider, Lynn R Spiegel, Rae Sm Yeung, Michelle Anderson, Audrey Bell-Peter, Holly Convery, Karen Queffelec, Megan Saunders, Shirley Ml Tse
BACKGROUND/PURPOSE: Biologic therapies have revolutionized the management of rheumatic diseases of childhood. However, these medications are associated with adverse effects including the possible development of neoplasms. The aim of our study was to determine the rate as well as risk factors for the development of neoplasms in patients with JIA treated with biologics. METHODS: We performed a retrospective review of the rheumatology biologic registry (RBR) at The Hospital for Sick Children (SickKids), Toronto, Canada, one of the largest quarternary pediatric referral centres in North America...
March 2014: Arthritis & Rheumatology
Joseph F Seay, Rebecca E Fellin, Shane G Sauer, Peter N Frykman, Carolyn K Bensel
The dose-response relationship between biomechanical variables and the magnitude of external loads is unclear. The use of different load distributions (e.g., pack types) may confound results because of changes in torso center of mass. Therefore, we examined the relationship between load magnitude and sagittal plane lower extremity mechanics of Soldiers walking with two symmetrically distributed loads. Fourteen Soldiers marched on a force-sensing treadmill at 1.34 m/s for 10 minutes with no load (BW_00) and while wearing vest-borne loads of 15 kg (BW_15) and 55 kg (BW_55)...
January 2014: Military Medicine
Gregory S Day, Susanne M Benseler
No abstract text is available yet for this article.
March 2014: Annals of Neurology
Szun S Tay, Bo Lu, Fred Sierro, Volker Benseler, Claire M McGuffog, G Alex Bishop, Peter J Cowan, Geoffrey W McCaughan, Karen M Dwyer, David G Bowen, Patrick Bertolino
Donor passenger leukocytes (PLs) from transplanted livers migrate to recipient lymphoid tissues, where they are thought to induce the deletion of donor-specific T cells and tolerance. Difficulties in tracking alloreactive T cells and PLs in rats and in performing this complex surgery in mice have limited progress in identifying the contribution of PL subsets and sites and the kinetics of T cell deletion. Here we developed a mouse liver transplant model in which PLs, recipient cells, and a reporter population of transgenic CD8 T cells specific for the graft could be easily distinguished and quantified in allografts and recipient organs by flow cytometry...
November 2013: Liver Transplantation
Lars Bräuer, Martin Schicht, Dieter Worlitzsch, Tobias Bensel, R Gary Sawers, Friedrich Paulsen
Surfactant proteins (SP), originally known from human lung surfactant, are essential to proper respiratory function in that they lower the surface tension of the alveoli. They are also important components of the innate immune system. The functional significance of these proteins is currently reflected by a very large and growing number of publications. The objective goal of this study was to elucidate whether Staphylococcus aureus and Pseudomonas aeruginosa is able to express surfactant proteins. 10 different strains of S...
2013: PloS One
L H Verhey, H M Branson, S Laughlin, M M Shroff, S M Benseler, B M Feldman, D L Streiner, J G Sled, B Banwell
BACKGROUND AND PURPOSE: The degree to which MR imaging is useful in the diagnosis of MS is predicated on standardized and reliable evaluation of MR imaging parameters. We aimed to devise items for an MR imaging scoring tool that would have high inter-rater agreement and would be straightforward to apply. MATERIALS AND METHODS: On the basis of a literature search and consensus of an expert panel, we identified 48 parameters that describe acute CNS demyelination, predict MS diagnosis, or characterize demyelinating disorder mimics...
June 2013: AJNR. American Journal of Neuroradiology
Maarten J Titulaer, Lindsey McCracken, Iñigo Gabilondo, Thaís Armangué, Carol Glaser, Takahiro Iizuka, Lawrence S Honig, Susanne M Benseler, Izumi Kawachi, Eugenia Martinez-Hernandez, Esther Aguilar, Núria Gresa-Arribas, Nicole Ryan-Florance, Abiguei Torrents, Albert Saiz, Myrna R Rosenfeld, Rita Balice-Gordon, Francesc Graus, Josep Dalmau
BACKGROUND: Anti-NMDA receptor (NMDAR) encephalitis is an autoimmune disorder in which the use of immunotherapy and the long-term outcome have not been defined. We aimed to assess the presentation of the disease, the spectrum of symptoms, immunotherapies used, timing of improvement, and long-term outcome. METHODS: In this multi-institutional observational study, we tested for the presence of NMDAR antibodies in serum or CSF samples of patients with encephalitis between Jan 1, 2007, and Jan 1, 2012...
February 2013: Lancet Neurology
Gordon S Soon, Lance H Rodan, Suzanne Laughlin, Ronald M Laxer, Susanne Benseler, Earl D Silverman
No abstract text is available yet for this article.
August 2012: Journal of Rheumatology
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