KD RESEARCH

Kawasaki disease research center at UCSD and Rady Children's hospital San Diego, CA
KAWASAKI DISEASE RESEARCH CENTER AT UCSD AND
RADY CHILDREN’S HOSPITAL SAN DIEGO-2008
Ø For more information on Kawasaki Disease, visit our website at:http://www.pediatrics.ucsd.edu/kawasaki
Statement of Purpose:
The Kawasaki Disease Research Center is a joint collaboration between the Departments of Pediatrics and Sociology at UCSD, the Pediatric Pharmacology Research Unit, the Scripps Institute of Oceanography, and the Rady Children’s Hospital San Diego. The Center was created to help foster excellence in care for patients with Kawasaki Disease (KD) and to support clinical, laboratory, and epidemiologic investigation into the etiology, pathophysiology, genetics, treatment, and natural history of the disease. The Center brings together investigators from ..Japan.. and the ....United States.... with diverse research interests and expertise to work together to further our understanding of this enigmatic disease
Recent Studies Completed by the KD ....Research.. ..Center....:....
q New treatment for children who fail to respond to IVIG infusion....
Approximately 20% of children with KD fail to respond to the first dose of gamma globulin (IVIG). Infliximab (Remicade®) is an antibody that binds TNF-α, a pro-inflammatory molecule that is elevated in the blood of children with KD. This therapeutic antibody may help turn off the inflammation in children who do not completely respond to IVIG.....
q Burns JC, Mason WH, Hauger SB, Janai H, Bastian JF, Wohrley JD, Balfour I, Shen CA, Michel ED, Shulman ST, Melish ME. Infliximab treatment for refractory ....Kawasaki.... syndrome. J Pediatr. 2005;146:662-667.....
q Burns JC, Best BM, Mejias A, Mahony L, ..Fixler.. ..DE.., Jafri HS, Melish ME, Jackson MA, Asmar B, Lang D, Connor JD, Capparelli EV, Keen ML, Mamun K, Keenan G, Ramilo O. Infliximab treatment of IVIG-resistant ....Kawasaki.... disease. J Pediatr 2008;....
q Tremoulet AH, Best BM, Song S, Wang S, Corinaldesi E, Eichenfield J, Burns JC. Resistance to intravenous immunoglobulin in children with Kawasaki Disease. J Pediatr. 2008;153:117-121.....
q Epidemiology of KD in ....San Diego.. ..County...., Dr. Annie Kao....
Reporting of KD cases to the county health department is a requirement in ....California..... This study was performed in collaboration with the San Diego Dept. of Health Services and describes all patients diagnosed with KD in San Diego County during the 5-yr. study period from 1998-2003. For the first time, clustering of KD cases in time and space was documented, which further suggests an infectious disease trigger for KD. The manuscript describing this study is in press.....
q Genetics and KD....
Regions of the DNA that may influence susceptibility to KD and outcome were examined in a mixed ethnic/racial cohort of KD patients and their families from ..North America... Polymorphisms in the genes for Interleukin-4 and the C-C chemokine receptor 5 and copy number variation for CCL3L1 influence KD susceptibility. Families with multiple affected members are now being recognized in ..North America.......
q Dergun M, Kao A, Hauger SB, Newburger JW, Burns JC. Familial occurrence of Kawasaki syndrome in North America. Arch Pediatr Adolesc Med. 2005;159:876-881.....
q Burns JC, Shimizu C, Gonzalez E, Kulkarni H, Patel S, Shike J, Sundel RS, Newburger JW, Ahuja SK. Genetic variations in the receptor-ligand pair, CCR5 and CCL3L1, are important determinants of susceptibility to ....Kawasaki.... disease. J Infect Dis. 2005;192:344-349.....
q Burns JC, Shimizu C, Shike H, Newburger JW, Sundel RS, Matsubara T, Ishikawa Y, Cheng S, Grow MA, Steiner L, Brophy VA, Cantor R. Family-based association analysis of genes in inflammatory and cardiovascular pathways implicates IL-4 in Kawasaki Disease. Genes and Immunity 2005;6:438-444.....
q Onouchi Y, Gunji T, Burns JC, Shimizu C, Newburger JW, Yashiro M, Nakamura Y, Yanagawa H, Wakui K, Fukushima Y, Kishi F, Hamamoto K, Terai M, Sato Y, Ouchi K, Saji T, Narai A, Kaburagi Y, Yoshikawa T, Suzuki K, Tanaka T, Nagai T, Cho H, Fujino A, Sekine A, Nakmuchi R, Tatsuhiko T, Kawasaki T, Nakamura Y, Hata A. IPTKC functional polymorphism associated with Kawasaki disease susceptibility and formation of coronary artery aneurysms. Nature Genetics 2008 Jan;40(1):35-42. ....
.. ..
q Climate Study....
In collaboration with ..Dan Cayan.., Director, ....Climate.. ..Center...., Scripps Instiution of Oceanography, we studied possible links between KD and seasons of the year. In ....Japan...., there are two peaks, one in the winter months (Jan.-March) and a second in mid-summer (July/August). This pattern for KD also is evident in ....San Diego.. ..County..... Analysis of databases of KD patients from all over the world is in progress.....
.. ..
Current Studies in Progress:....
q New clinical trial for primary treatment for children with KD....
About 20% of children will be resistant to their first infusion of IVIG. In addition, despite timely treatment, approximately 5% will go on to develop coronary artery aneurysms. In an effort to increase the effectiveness of anti-inflammatory therapy, we are enrolling patients in a double-blind, placebo-controlled trial of IVIG plus Remicade vs. IVIG plus placebo. This clinical trial is sponsored by the FDA and will evaluate which treatment is best for children with KD.....
.. ..
q Diagnostic test for KD....
Despite several years of research, the cause of KD remains a mystery. This study uses innovative techniques to examine KD patients’ RNA (genetic information) and protein levels in the blood to look for patterns that may be used as a diagnostic test....
.. ..
q Genetic polymorphisms study....
An examination of KD patients’ DNA and comparison with DNA from their biologic parents can reveal the ways in which children are genetically predisposed to develop ....Kawasaki.... disease and coronary artery aneurysms. We are currently enrolling patients and their parents in this study. Please contact us if you would like to participate (858-246-0157 or e-mail jcburns@ucsd.edu).....
.. ..
q Gene expression in KD....
This study uses microarray analysis of whole blood RNA to understand pathways of gene expression in acute KD....
.. ..
q Proteomic analysis of acute KD plasma and urine....
This study is a collaboration with Dr. Harvey Cohen and the proteomics unit at ....Stanford.. ..University..... Analysis of peptide fragments in the plasma and urine of patients with acute KD may lead to novel diagnostic markers for the development of a diagnostic test for KD.....