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% ofKDpatients 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/ACgenotypes 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 serumalbumin concentration is an independent risk factor for IVIG nonresponsiveness.
Original language | English |
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Pages (from-to) | 513-520 |
Number of pages | 8 |
Journal | Pediatric Cardiology |
Volume | 33 |
Issue number | 4 |
DOIs | |
Publication status | Published - 2012 Apr |
Externally published | Yes |
Bibliographical note
Funding Information: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).
Keywords
- Coronary artery lesions
- Intravenous immunoglobulin
- Kawasaki disease
- Korean
- PELI1 gene
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Cardiology and Cardiovascular Medicine