The study of the plasma platelet-activating factor and a Porimorphism in plasma platelet-activating factor acetylhydrase in the development and progression in Kawasaki disease

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Objective : To investigate whether reduced levels of plasma platelet-activating factor acetylhydrolase(PAF-AH) due to a genetic polymorphism are involved in the pathogenesis of Kawasaki disease(KD).Study design : The frequency of a V279F polymorphism (G/T transversion) in the PAF-AH gene was quantified in 76 Japanese children with KD and 112 healthy Japanese adults using the allele-specific polymerase chain reaction. Associations between genotype, clinical features and resistance to intravenous immunoglobulin(IVIG) were investigated in the KD patients. Plasma PAF-AH activity was measured by using [^3H]-acetyl-PAF.Results : There were no significant differences in genotype frequency between patients and controls (P=0.51). Compared with the GG (normal genotype) group, significantly more patients in the GT (heterozygous)+TT (homozygous deficient) group required additional IVIG (52% vs 14%, P=0.001). The duration of fever and maximum serum C-reactive protein levels were also significantly increased in the GT+TT group (P=0.012 and 0.036, respectively), while plasma PAF-AH activity was significantly lower (P<0.0001).Conclusion : We conclude that the V279F polymorphism in the plasma PAF-AH gene and consequent enzymatic deficiency is one of the factors for IVIG non-response in Japanese patients with acute KD