title: Assessment of risk factors for Korean children with 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.
authors: Asan Institute for Life Sciences, University of Ulsan College of Medicine, 388-1 Pungnap-2-Dong, Songpa-Gu, Seoul 138-736, Korea.
authors: Jae-Jung
authors: JJ
authors: Kim
authors: Young Mi
authors: YM
authors: Hong
authors: Sin Weon
authors: SW
authors: Yun
authors: Myung Ki
authors: MK
authors: Han
authors: Kyung-Yil
authors: KY
authors: Lee
authors: Min Seob
authors: MS
authors: Song
authors: Hyoung-Doo
authors: HD
authors: Lee
authors: Dong Soo
authors: DS
authors: Kim
authors: Sejung
authors: S
authors: Sohn
authors: Kee-Soo
authors: KS
authors: Ha
authors: Soo-Jong
authors: SJ
authors: Hong
authors: Kwi-Joo
authors: KJ
authors: Kim
authors: In-Sook
authors: IS
authors: Park
authors: Gi Young
authors: GY
authors: Jang
authors: Jong-Keuk
authors: JK
authors: Lee
mesh_terms: D002675:Child, Preschool; D003331:Coronary Vessels / Q000473:pathology*; D004247:DNA / Q000235:genetics; D005260:Female; D005500:Follow-Up Studies; D020022:Genetic Predisposition to Disease; D005838:Genotype; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use; D007155:Immunologic Factors / Q000008:administration & dosage / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D007275:Injections, Intravenous; D008297:Male; D009017:Morbidity / Q000639:trends; D009080:Mucocutaneous Lymph Node Syndrome / Q000188:drug therapy / Q000453:epidemiology* / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D020641:Polymorphism, Single Nucleotide; D056910:Republic of Korea / Q000453:epidemiology; D012189:Retrospective Studies; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D044767:Ubiquitin-Protein Ligases / Q000235:genetics
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D009687:Nuclear Proteins; D004247:DNA; C485959:PELI1 protein, human; D044767:Ubiquitin-Protein Ligases