Abstract

BackgroundKawasaki disease (KD) is a systemic vasculitis with unknown etiology mainly affecting children in Asian countries. Dendritic cell-specific intercellular adhesion molecule-3 grabbing non-integrin (DC-SIGN, CD209) in humans was showed to trigger an anti-inflammatory cascade and associated with KD susceptibility. This study was conducted to investigate the association between genetic polymorphisms of CD209 and the risk KD.MethodsA total of 948 subjects (381 KD and 567 controls) were recruited. Nine tagging SNPs (rs8112310, rs4804800, rs11465421, rs1544766, rs4804801, rs2287886, rs735239, rs735240, rs4804804) were selected for TaqMan allelic discrimination assay. Clinical phenotypes, coronary artery lesions (CAL) and intravenous immunoglobulin (IVIG) treatment outcomes were collected for analysis.ResultsSignificant associations were found between CD209 polymorphisms (rs4804800, rs2287886, rs735240) and the risk of KD. Haplotype analysis for CD209 polymorphisms showed that A/A/G haplotype (P = 0.0002, OR = 1.61) and G/A/G haplotype (P = 0.0365, OR = 1.52) had higher risk of KD as compared with G/G/A haplotype in rs2287886/rs735239/rs735240 pairwise allele analysis. There were no significant association in KD with regards to CAL formation and IVIG treatment responses.ConclusionCD209 polymorphisms were responsible for the susceptibility of KD, but not CAL formation and IVIG treatment responsiveness.

Highlights

  • Kawasaki disease (KD) is a systemic vasculitis which was first reported by Dr Kawasaki in 1974 in English from Japan [1]

  • coronary artery lesions (CAL) was defined as the internal diameter of the coronary artery greater than 3 mm (4 mm, if the subject was more than 5 year-old) or the internal diameter of a segment being at least 1.5 times than adjacent segment by echocardiogram [20,21,22]

  • Of the 381 KD patients, 126 (33.1%) patients had coronary artery lesion (CAL), and 49 (12.9%) patients suffered from persistent fever after they treated with intravenous immunoglobulin (IVIG)

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Summary

Introduction

Kawasaki disease (KD) is a systemic vasculitis which was first reported by Dr Kawasaki in 1974 in English from Japan [1]. It mainly affects children less than 5-years-old world widely, especially in Asia. The clinical characteristics and diagnosis criteria of KD include a prolonged fever (more than 5 days), bilateral non-purulent conjunctivitis, diffuse mucosal inflammation of oral cavity with strawberry tongue and fissure lips, polymorphous skin rashes over body surface, indurative angioedema of the hands and feet followed by desquamation in the sub-acute stage, and lymphadenopathy over neck [3,5]. This study was conducted to investigate the association between genetic polymorphisms of CD209 and the risk KD

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