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To investigate whether reduced levels of plasma platelet-activating factor acetylhydrolase (PAF-AH) as a result of 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 (PCR). Associations between genotype, clinical features, and resistance to intravenous immunoglobulin (IVIG) were investigated in the patients with KD. 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 = .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 = .001). The duration of fever and maximum serum C-reactive protein (CRP) levels also were significantly increased in the GT+ TT group (P = .012 and .036, respectively), whereas plasma PAF-AH activity was significantly lower (P < .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 nonresponse in Japanese patients with acute KD.
To investigate whether reduced levels of plasma platelet-activating factor acetylhydrolase (PAF-AH) as a result of 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 (PCR). Associations between genotype, clinical features, and resistance to intravenous immunoglobulin (IVIG) were investigated in The patients with KD. 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 = .51) ) group, significantly more patients in the GT (heterozygous) + TT (homozygous deficient) group required additional IVIG (52% vs 14%, P = .001). The duration of fever and maximum serum C-reactive protein a Conclusion: We conclude that the V279F polymorphism in the plasma PAF-AH activity was significantly lower (P <.0001) AH gene and consequent enzymatic deficiency is one of the factors for IVIG nonresponse in Japanese patients with acute KD.