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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
Institution:1. Asan Institute for Life Sciences, University of Ulsan College of Medicine, 388-1 Pungnap-2-Dong, Songpa-Gu, Seoul, Korea
2. Department of Pediatrics, University of Ulsan, Gangneung Asan Hospital, Gangneung, Korea
3. Department of Pediatrics, The Catholic University of Korea, Daejeon St. Mary??s Hospital, Daejeon, Korea
4. Department of Pediatrics, Inje University Paik Hospital, Busan, Korea
5. Department of Pediatrics, Pusan National University Hospital, Busan, Korea
6. Department of Pediatrics, Yonsei University College of Medicine, Severance Children??s Hospital, Seoul, Korea
7. Department of Pediatrics, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea
8. Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea
9. Department of Pediatrics, Ewha Womans University Hospital, Seoul, Korea
10. Department of Pediatrics, Korea University Hospital, Seoul, Korea
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.
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