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Assessment of Risk Factors for Korean Children with Kawasaki Disease

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Abstract

Kawasaki disease (KD) is the most common cause of acquired heart disease in children. Intravenous immunoglobulin (IVIG) is the standard therapy for KD, but more than 10% of KD patients do not respond to IVIG and are at high risk for the development of coronary artery lesions (CALs). To identify clinical and genetic risk factors associated with CAL development and IVIG nonresponsiveness, this study analyzed the clinical data for 478 Korean KD patients. Multivariate logistic regression analysis showed that incomplete KD, IVIG nonresponse, fever duration of 7 days or longer, and the CC/AC genotypes of the rs7604693 single nucleotide polymorphism (SNP) in the PELI1 gene were significantly associated with the development of CALs, with odds ratios (ORs) ranging from 2.06 to 3.04. The risk of CAL formation was synergistically increased by the addition of individual risk factors, particularly the genetic variant in the PELI1 gene. Multivariate analysis also showed that a serum albumin level of 3.6 g/dl or lower was significantly associated with nonresponsiveness to IVIG [OR, 2.76; 95% confidence interval (CI), 1.34–5.68; P = 0.006]. Conclusively, incomplete KD, IVIG nonresponsiveness, long febrile days, and the rs7604693 genetic variant in the PELI1 gene are major risk factors for the development of CALs, whereas low serum albumin concentration is an independent risk factor for IVIG nonresponsiveness.

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Acknowledgments

We thank all the patients with KD and their families for participating in this study. We also thank Dr. Kyu-Young Song and Dr. Eul-Joo Seo for critical review of the manuscript. This work was supported by a grant (A010384) from the Ministry of Health & Welfare of the Republic of Korea and a grant (no. 2011-419) from the Asan Institute for Life Sciences, Seoul, Korea. Jae-Jung Kim was supported by a National Research Foundation of Korea Grant funded by the Korean Government (Ministry of Education, Science and Technology; NRF-2011-355-C00109).

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Authors and Affiliations

  1. Asan Institute for Life Sciences, University of Ulsan College of Medicine, 388-1 Pungnap-2-Dong, Songpa-Gu, Seoul, 138-736, Korea

    Jae-Jung Kim & Jong-Keuk Lee

  2. Department of Pediatrics, Ewha Womans University Hospital, Seoul, Korea

    Young Mi Hong & Sejung Sohn

  3. Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea

    Sin Weon Yun

  4. Department of Pediatrics, University of Ulsan, Gangneung Asan Hospital, Gangneung, Korea

    Myung Ki Han

  5. Department of Pediatrics, The Catholic University of Korea, Daejeon St. Mary’s Hospital, Daejeon, Korea

    Kyung-Yil Lee

  6. Department of Pediatrics, Inje University Paik Hospital, Busan, Korea

    Min Seob Song

  7. Department of Pediatrics, Pusan National University Hospital, Busan, Korea

    Hyoung-Doo Lee

  8. Department of Pediatrics, Yonsei University College of Medicine, Severance Children’s Hospital, Seoul, Korea

    Dong Soo Kim

  9. Department of Pediatrics, Korea University Hospital, Seoul, 136-701, Korea

    Kee-Soo Ha & Gi Young Jang

  10. Department of Pediatrics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

    Soo-Jong Hong, Kwi-Joo Kim & In-Sook Park

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  1. Jae-Jung Kim
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Korean Kawasaki Disease Genetics Consortium

Corresponding authors

Correspondence to Gi Young Jang or Jong-Keuk Lee.

Additional information

J.-J. Kim, Y. M. Hong and S.W. Yun contributed equally to this work.

The members of Korean Kawasaki Disease Genetics Consortium are given in the Appendix.

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Appendix: Korean Kawasaki Disease Genetics Consortium

Appendix: Korean Kawasaki Disease Genetics Consortium

In-Sook Park, Jeong-Jin Yoo, Soo-Jong Hong, Kwi-Joo Kim (Department of Pediatrics, Asan Medical Center, Seoul, Korea); Jong-Keuk Lee, Jae-Jung Kim, Young-Mi Park (Asan Institute for Life Sciences, University of Ulsan College of Medicine, Seoul, Korea); Young Mi Hong, Sejung Sohn (Department of Pediatrics, Ewha Womans University Hospital, Seoul, Korea); Gi Young Jang, Kee-Soo Ha, Hyo-Kyoung Nam, Jung-Hye Byeon (Department of Pediatrics, Korea University Hospital, Seoul, Korea); Sin Weon Yun (Department of Pediatrics, Chung-Ang University Hospital, Seoul, Korea); Myung Ki Han (Department of Pediatrics, University of Ulsan, Gangneung Asan Hospital, Gangneung, Korea); Kyung-Yil Lee, Ja-Young Hwang, Jung-Woo Rhim (Department of Pediatrics, The Catholic University of Korea, Daejeon St. Mary’s Hospital, Daejeon, Korea); Min Seob Song (Department of Pediatrics, Inje University Paik Hospital, Busan, Korea); Hyoung-Doo Lee (Department of Pediatrics, Pusan National University Hospital, Busan, Korea); Dong Soo Kim (Department of Pediatrics, Yonsei University College of Medicine, Severance Children’s Hospital, Seoul, Korea); Jae-Moo Lee (Seoul Clinical Laboratories, Seoul, Korea).

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Kim, JJ., Hong, Y.M., Yun, S.W. et al. Assessment of Risk Factors for Korean Children with Kawasaki Disease. Pediatr Cardiol 33, 513–520 (2012). https://doi.org/10.1007/s00246-011-0143-1

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