Kim Hye-Eun, Kim Jae-Jung, Han Myung Ki, Lee Kyung-Yil, Song Min Seob, Lee Hyoung-Doo, Kim Dong Soo, Yu Jeong Jin, Park In-Sook, Yun Sin Weon, Hong Young Mi, Jang Gi Young, Lee Jong-Keuk
Asan Institute for Life Sciences, University of Ulsan College of Medicine, 388-1 Pungnap-2-Dong, Songpa-Gu, Seoul, Korea.
Pediatr Cardiol. 2012 Dec;33(8):1259-63. doi: 10.1007/s00246-012-0289-5. Epub 2012 Mar 27.
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.
大剂量静脉注射免疫球蛋白（IVIG）疗法是川崎病（KD）的高效标准治疗方法。然而，约20%的KD患者在初次IVIG治疗后仍持续发热或发热复发，这增加了冠状动脉病变（CALs）的风险。此外，KD患者IVIG抵抗的机制仍不清楚。据报道，在日本患者中，CC趋化因子配体3样1（CCL3L1）基因拷贝数与KD及IVIG抵抗有关。此外，作者观察到在来自KD患者的B细胞系中，体外免疫球蛋白治疗后CCL3L1基因表达显著上调。因此，本研究对459例KD患者和496例健康对照受试者进行检测，以确定CCL3L1基因拷贝数是否与韩国KD患者的KD风险、CALs和/或IVIG抵抗有关。然而，CCL3L1基因拷贝数与KD（P = 0.18）、CAL形成（P = 0.062）或IVIG抵抗（P = 0.90）均无关联。因此，结果表明CCL3L1基因拷贝数在韩国KD患者对KD或CALs的易感性以及IVIG抵抗方面不起作用。
