Published in:
01-12-2012 | Original Article
Variations in the Number of CCL3L1 Gene Copies and Kawasaki Disease in Korean Children
Authors:
Hye-Eun Kim, Jae-Jung Kim, Myung Ki Han, Kyung-Yil Lee, Min Seob Song, Hyoung-Doo Lee, Dong Soo Kim, Jeong Jin Yu, In-Sook Park, Sin Weon Yun, Young Mi Hong, Gi Young Jang, Jong-Keuk Lee, Korean Kawasaki Disease Genetics Consortium
Published in:
Pediatric Cardiology
|
Issue 8/2012
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Abstract
High-dose intravenous immunoglobulin (IVIG) therapy is the highly effective and standard treatment for Kawasaki disease (KD). However, ~20 % of KD patients have persistent fever or recurrence of fever after the initial IVIG treatment, which increases the risk for coronary artery lesions (CALs). Furthermore, the mechanism of IVIG resistance in KD patients still is unknown. The number of CC chemokine ligand 3-like 1 (CCL3L1) gene copies is reported to be associated with KD and IVIG resistance in Japanese patients. In addition, the authors observed significant upregulation of the CCL3L1 gene expression after in vitro immunoglobulin treatment in B cell lines derived from KD patients. Therefore, this study of 459 KD patients and 496 healthy control subjects tested whether the number of CCL3L1 gene copies is associated with a risk of KD, CALs, and/or IVIG resistance in Korean KD patients. However, the number of CCL3L1 gene copies was not associated with KD (P = 0.18), CAL formation (P = 0.062), or the IVIG resistance (P = 0.90). Therefore, the results indicate that the number of CCL3L1 gene copies does not have a role in susceptibility to KD or CALs nor with IVIG resistance in Korean KD patients.