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Assessment of risk factors for Korean children with Kawasaki disease
Jae-Jung Kim1, Young Mi Hong, Sin Weon Yun
1Asan Institute for Life Sciences, University of Ulsan College of Medicine, 388-1 Pungnap-2-Dong, Songpa-Gu, Seoul 138-736, Korea.
Insights
Kawasaki disease patients with incomplete symptoms, prolonged fever, or a specific PELI1 gene variant face higher risks of coronary artery lesions. Low serum albumin is linked to poor response to standard IVIG therapy.
Area of Science:
- Pediatric Cardiology
- Genetics
- Immunology
Background:
- Kawasaki disease (KD) is a leading cause of acquired heart disease in children.
- Standard treatment with intravenous immunoglobulin (IVIG) is ineffective in over 10% of patients, increasing their risk of coronary artery lesions (CALs).
Purpose of the Study:
- To identify clinical and genetic factors predicting CAL development and IVIG nonresponsiveness in Korean KD patients.
Main Methods:
- Analysis of clinical data from 478 Korean KD patients.
- Multivariate logistic regression to assess risk factors for CALs and IVIG nonresponsiveness.
Main Results:
- Incomplete KD, IVIG nonresponse, fever lasting ≥7 days, and PELI1 rs7604693 genotypes (CC/AC) were significant risk factors for CALs (ORs 2.06-3.04).
- Low serum albumin (<3.6 g/dL) was independently associated with IVIG nonresponsiveness (OR 2.76).
Conclusions:
- Incomplete KD, IVIG nonresponsiveness, prolonged fever, and the PELI1 rs7604693 variant are key predictors of CAL development.
- Low serum albumin is an independent risk factor for treatment failure in Kawasaki disease.
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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