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Burns and kawasaki

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https://www.readbyqxmd.com/read/28065902/princess-privacy-protecting-rare-disease-international-network-collaboration-via-encryption-through-software-guard-extensions
#1
Feng Chen, Shuang Wang, Xiaoqian Jiang, Sijie Ding, Yao Lu, Jihoon Kim, S Cenk Sahinalp, Chisato Shimizu, Jane C Burns, Victoria J Wright, Eileen Png, Martin L Hibberd, David D Lloyd, Hai Yang, Amalio Telenti, Cinnamon S Bloss, Dov Fox, Kristin Lauter, Lucila Ohno-Machado
MOTIVATION: We introduce PRINCESS, a privacy-preserving international collaboration framework for analyzing rare disease genetic data that are distributed across different continents. PRINCESS leverages Software Guard Extensions (SGX) and hardware for trustworthy computation. Unlike a traditional international collaboration model, where individual-level patient DNA are physically centralized at a single site, PRINCESS performs a secure and distributed computation over encrypted data, fulfilling institutional policies and regulations for protected health information...
January 8, 2017: Bioinformatics
https://www.readbyqxmd.com/read/28002448/unique-molecular-patterns-uncovered-in-kawasaki-disease-patients-with-elevated-serum-gamma-glutamyl-transferase-levels-implications-for-intravenous-immunoglobulin-responsiveness
#2
Yue Wang, Zhen Li, Guang Hu, Shiying Hao, Xiaohong Deng, Min Huang, Miao Ren, Xiyuan Jiang, John T Kanegaye, Kee-Soo Ha, JungHwa Lee, Xiaofeng Li, Xuejun Jiang, Yunxian Yu, Adriana H Tremoulet, Jane C Burns, John C Whitin, Andrew Y Shin, Karl G Sylvester, Doff B McElhinney, Harvey J Cohen, Xuefeng B Ling
BACKGROUND: Resistance to intravenous immunoglobulin (IVIG) occurs in 10-20% of patients with Kawasaki disease (KD). The risk of resistance is about two-fold higher in patients with elevated gamma glutamyl transferase (GGT) levels. We sought to understand the biological mechanisms underlying IVIG resistance in patients with elevated GGT levels. METHOD: We explored the association between elevated GGT levels and IVIG-resistance with a cohort of 686 KD patients (Cohort I)...
2016: PloS One
https://www.readbyqxmd.com/read/27923814/mir-483-targeting-of-ctgf-suppresses-endothelial-to-mesenchymal-transition-therapeutic-implications-in-kawasaki-disease
#3
Ming He, Zhen Chen, Marcy Martin, Jin Zhang, Panjamaporn Sangwung, Brian Woo, Adriana Tremoulet, Chisato Shimizu, Mukesh K Jain, Jane C Burns, John Shyy
RATIONALE: Endothelial-to-mesenchymal transition (EndoMT) is implicated in myofibroblast-like cell-mediated damage to the coronary arterial wall in acute Kawasaki disease (KD) patients, as evidenced by positive staining for connective tissue growth factor (CTGF) and EndoMT markers in KD autopsy tissues. However, little is known about the molecular basis of EndoMT involved in KD. OBJECTIVE: We investigated the microRNA (miRNA) regulation of CTGF and the consequent EndoMT in KD pathogenesis...
December 6, 2016: Circulation Research
https://www.readbyqxmd.com/read/27918378/safety-of-infliximab-following-live-virus-vaccination-in-kawasaki-disease-patients
#4
Aaron M Lee, Jane C Burns, Adriana H Tremoulet
No published data are available regarding infliximab use in Kawasaki Disease (KD) patients with recent live virus vaccination. We investigated 38 acute KD patients who received infliximab within 90 days of receiving MMR, VZV, or Rotavirus vaccines. There were no adverse events or serious infections, which suggests that infliximab use in acute KD patients with recent live viral vaccinations is safe.
December 1, 2016: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/27879314/genetic-variation-in-the-slc8a1-calcium-signaling-pathway-is-associated-with-susceptibility-to-kawasaki-disease-and-coronary-artery-abnormalities
#5
Chisato Shimizu, Hariklia Eleftherohorinou, Victoria J Wright, Jihoon Kim, Martin P Alphonse, James C Perry, Rolando Cimaz, David Burgner, Nagib Dahdah, Long T Hoang, Chiea Chuen Khor, Andrea Salgado, Adriana H Tremoulet, Sonia Davila, Taco W Kuijpers, Martin L Hibberd, Todd A Johnson, Atsushi Takahashi, Tatsuhiko Tsunoda, Michiaki Kubo, Toshihiro Tanaka, Yoshihiro Onouchi, Rae S M Yeung, Lachlan J M Coin, Michael Levin, Jane C Burns
BACKGROUND: Kawasaki disease (KD) is an acute pediatric vasculitis in which host genetics influence both susceptibility to KD and the formation of coronary artery aneurysms. Variants discovered by genome-wide association studies and linkage studies only partially explain the influence of genetics on KD susceptibility. METHODS AND RESULTS: To search for additional functional genetic variation, we performed pathway and gene stability analysis on a genome-wide association study data set...
December 2016: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/27851085/1449-clinical-comparison-of-kawasaki-disease-shock-syndrome-and-septic-shock-in-the-picu
#6
Joseph Treister, Jennifer Foley, Helen Harvey, Adriana Tremoulet, Susan Duthie, Jane Burns
No abstract text is available yet for this article.
December 2016: Critical Care Medicine
https://www.readbyqxmd.com/read/27816942/extreme-giant-aneurysms-of-three-coronary-arteries-causing-heart-failure-as-late-sequelae-of-kawasaki-disease
#7
Enrico Ammirati, Jane C Burns, Antonella Moreo, Lori B Daniels, Fabrizio Oliva
No abstract text is available yet for this article.
November 5, 2016: European Heart Journal
https://www.readbyqxmd.com/read/27792871/found-in-translation-international-initiatives-pursuing-interleukin-1-blockade-for-treatment-of-acute-kawasaki-disease
#8
REVIEW
Jane C Burns, Isabelle Koné-Paut, Taco Kuijpers, Chisato Shimizu, Adriana Tremoulet, Moshe Arditi
The decision to move forward with three clinical trials of IL-1 blockade for treatment of acute Kawasaki disease is a case study in translational science. These trials were born on the one hand from transcriptome studies of host response during the acute disease coupled with animal model investigations of key immune signaling pathways and, on the other hand, out of clinical desperation to intervene in patients with severe inflammation in the setting of acute Kawasaki disease. The convergence of laboratory science and clinical observations led to the clinical trials described here and serves as a model for how such observations can be translated into new therapies...
October 28, 2016: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/27694492/inositol-triphosphate-3-kinase-c-mediates-inflammasome-activation-and-treatment-response-in-kawasaki-disease
#9
Martin Prince Alphonse, Trang T Duong, Chisato Shumitzu, Truong Long Hoang, Brian W McCrindle, Alessandra Franco, Stéphane Schurmans, Dana J Philpott, Martin L Hibberd, Jane Burns, Taco W Kuijpers, Rae S M Yeung
Kawasaki disease (KD) is a multisystem vasculitis that predominantly targets the coronary arteries in children. Phenotypic similarities between KD and recurrent fever syndromes point to the potential role of inflammasome activation in KD. Mutations in NLRP3 are associated with recurrent fever/autoinflammatory syndromes. We show that the KD-associated genetic polymorphism in inositol-triphosphate 3-kinase C (ITPKC) (rs28493229) has important functional consequences, governing ITPKC protein levels and thereby intracellular calcium, which in turn regulates NLRP3 expression and production of IL-1β and IL-18...
September 30, 2016: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/27633390/coronary-artery-aneurysms-in-kawasaki-disease-risk-factors-for-progressive-disease-and-adverse-cardiac-events-in-the-us-population
#10
Kevin G Friedman, Kimberly Gauvreau, Akiko Hamaoka-Okamoto, Alexander Tang, Erika Berry, Adriana H Tremoulet, Vidya S Mahavadi, Annette Baker, Sarah D deFerranti, David R Fulton, Jane C Burns, Jane W Newburger
BACKGROUND: The natural history of coronary artery aneurysms (CAA) after intravenous immunoglobulin (IVIG) treatment in the United States is not well described. We describe the natural history of CAA in US Kawasaki disease (KD) patients and identify factors associated with major adverse cardiac events (MACE) and CAA regression. METHODS AND RESULTS: We evaluated all KD patients with CAA at 2 centers from 1979 to 2014. Factors associated with CAA regression, maximum CA z-score over time (zMax), and MACE were analyzed...
September 15, 2016: Journal of the American Heart Association
https://www.readbyqxmd.com/read/27596163/differences-in-glyca-and-lipoprotein-particle-parameters-may-help-distinguish-acute-kawasaki-disease-from-other-febrile-illnesses-in-children
#11
Margery A Connelly, Chisato Shimizu, Deborah A Winegar, Irina Shalaurova, Ray Pourfarzib, James D Otvos, John T Kanegaye, Adriana H Tremoulet, Jane C Burns
BACKGROUND: Glycosylation patterns of serum proteins, such as α1-acid glycoprotein, are modified during an acute phase reaction. The response of acute Kawasaki disease (KD) patients to IVIG treatment has been linked to sialic acid levels on native IgG, suggesting that protein glycosylation patterns vary during the immune response in acute KD. Additionally, the distribution and function of lipoprotein particles are altered during inflammation. Therefore, the aim of this study was to explore the potential for GlycA, a marker of protein glycosylation, and the lipoprotein particle profile to distinguish pediatric patients with acute KD from those with other febrile illnesses...
September 5, 2016: BMC Pediatrics
https://www.readbyqxmd.com/read/27496266/kawasaki-disease-and-exposure-to-fine-particulate-air-pollution
#12
Andrew S Zeft, Jane C Burns, Rae S Yeung, Brian W McCrindle, Jane W Newburger, Samuel R Dominguez, Marsha S Anderson, Cammon Arrington, Stanford T Shulman, Jeein Yoon, Helen Tewelde, Carter Mix, C Arden Pope
OBJECTIVE: To analyze associations of short-term exposure to fine particulate matter (diameter ≤ 2.5 µm [PM2.5]), a measurable component of urban pollution, with the event date of fever onset for patients with Kawasaki disease (KD) residing in 7 metropolitan regions. STUDY DESIGN: A case-crossover study design was used. Time trends, seasonality, month, and weekday were controlled for by matching. We assembled PM2.5 exposure measurements from urban monitors and imputed PM2...
October 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27344221/a-classification-tool-for-differentiation-of-kawasaki-disease-from-other%C3%A2-febrile-illnesses
#13
Shiying Hao, Bo Jin, Zhou Tan, Zhen Li, Jun Ji, Guang Hu, Yue Wang, Xiaohong Deng, John T Kanegaye, Adriana H Tremoulet, Jane C Burns, Harvey J Cohen, Xuefeng B Ling
OBJECTIVE: To develop and validate a novel decision tree-based clinical algorithm to differentiate Kawasaki disease (KD) from other pediatric febrile illnesses that share common clinical characteristics. STUDY DESIGN: Using clinical and laboratory data from 801 subjects with acute KD (533 for development, and 268 for validation) and 479 febrile control subjects (318 for development, and 161 for validation), we developed a stepwise KD diagnostic algorithm combining our previously developed linear discriminant analysis (LDA)-based model with a newly developed tree-based algorithm...
September 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27271757/a-novel-truncated-form-of-serum-amyloid-a-in-kawasaki-disease
#14
John C Whitin, Tom To-Sang Yu, Xuefeng Bruce Ling, John T Kanegaye, Jane C Burns, Harvey J Cohen
BACKGROUND: Kawasaki disease (KD) is an acute vasculitis in children that can cause coronary artery abnormalities. Its diagnosis is challenging, and many cytokines, chemokines, acute phase reactants, and growth factors have failed evaluation as specific biomarkers to distinguish KD from other febrile illnesses. We performed protein profiling, comparing plasma from children with KD with febrile control (FC) subjects to determine if there were specific proteins or peptides that could distinguish the two clinical states...
2016: PloS One
https://www.readbyqxmd.com/read/27241708/adjunctive-therapies-for-kawasaki-disease
#15
Anita J Campbell, Jane C Burns
Kawasaki disease (KD) is the most common cause of acquired heart disease in children in developed countries.(1,2) The primary goal of treatment is to prevent coronary artery aneurysms (CAA). Between 10 and 20% of KD patients are resistant to treatment with intravenous immunoglobulin (IVIG) and have an almost nine-fold increased risk of developing CAA.(3) In addition, approximately 80-90% of patients who go on to develop CAA have abnormal coronary artery dimensions on their first echocardiogram and can therefore be identified as high-risk patients...
July 5, 2016: Journal of Infection
https://www.readbyqxmd.com/read/27131643/design-and-burn-up-analyses-of-new-type-holder-for-silicon-neutron-transmutation-doping
#16
Masao Komeda, Masaji Arai, Kazuo Tamai, Kozo Kawasaki
We have developed a new silicon irradiation holder with a neutron filter to increase the irradiation efficiency. The neutron filter is made of an alloy of aluminum and B4C particles. We fabricated a new holder based on the results of design analyses. This filter has limited use in applications requiring prolonged use due to a decrease in the amount of (10)B in B4C particles. We investigated the influence of (10)B reduction on doping distribution in a silicon ingot by using the Monte Carlo Code MVP.
July 2016: Applied Radiation and Isotopes
https://www.readbyqxmd.com/read/27080929/rationale-and-study-design-for-a-phase-i-iia-trial-of-anakinra-in-children-with-kawasaki-disease-and-early-coronary-artery-abnormalities-the-anakid-trial
#17
Adriana H Tremoulet, Sonia Jain, Susan Kim, Jane Newburger, Moshe Arditi, Alessandra Franco, Brookie Best, Jane C Burns
BACKGROUND: Although Kawasaki disease (KD) is the most common cause of acquired heart disease in children and may result in coronary artery aneurysms (CAA) with an attendant risk of myocardial infarction, there is no recommended therapy to halt progression of arterial wall damage and prevent aneurysm formation in the acute phase of the vasculitis. While intravenous immunoglobulin (IVIG) reduces the risk of CAA, up to 20% of KD patients are IVIG resistant and have a higher risk for developing CAA...
May 2016: Contemporary Clinical Trials
https://www.readbyqxmd.com/read/27056781/kawasaki-disease
#18
REVIEW
Jane W Newburger, Masato Takahashi, Jane C Burns
Kawasaki disease is an acute, self-limited vasculitis of unknown etiology that occurs predominantly in infants and children. If not treated early with high-dose intravenous immunoglobulin, 1 in 5 children develop coronary artery aneurysms; this risk is reduced 5-fold if intravenous immunoglobulin is administered within 10 days of fever onset. Coronary artery aneurysms evolve dynamically over time, usually reaching a peak dimension by 6 weeks after illness onset. Almost all the morbidity and mortality occur in patients with giant aneurysms...
April 12, 2016: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/27056307/the-spectrum-of-cardiovascular-lesions-requiring-intervention-in-adults-after-kawasaki-disease
#19
John B Gordon, Lori B Daniels, Andrew M Kahn, Susan Jimenez-Fernandez, Matthew Vejar, Fujito Numano, Jane C Burns
OBJECTIVES: The aim of this study was to characterize the range of management issues raised by adults with cardiovascular sequelae from Kawasaki disease (KD) in childhood. BACKGROUND: Aneurysms resulting from vascular inflammation associated with KD in childhood may remain clinically silent until adulthood. Adults with large aneurysms, unstable angina, or myocardial infarction following KD in childhood present unique challenges to interventional cardiologists and cardiothoracic surgeons...
April 11, 2016: JACC. Cardiovascular Interventions
https://www.readbyqxmd.com/read/26946987/psoriasiform-eruptions-during-kawasaki-disease-kd-a-distinct-phenotype
#20
Ellen S Haddock, Antoanella Calame, Chisato Shimizu, Adriana H Tremoulet, Jane C Burns, Wynnis L Tom
BACKGROUND: A psoriasis-like eruption develops in a subset of patients with Kawasaki disease (KD). OBJECTIVE: We sought to systematically compare KD-associated psoriasiform eruptions with classic psoriasis and the outcomes of KD in children with and without this rash. METHODS: This was a retrospective study of 11 KD cases with a psoriasiform eruption matched 1:2 by age, gender, and ethnicity with psoriasis-only and KD-only controls. Genotyping was performed in 10 cases for a deletion of 2 late cornified envelope (LCE) genes, LCE3C_LCE3B-del, associated with increased risk for pediatric-onset psoriasis...
July 2016: Journal of the American Academy of Dermatology
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