A polymorphism in plasma platelet-activating factor acetylhydrolase is involved in resistance to immunoglobulin treatment in Kawasaki disease |
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Authors: | Minami Takaomi Suzuki Hiroyuki Takeuchi Takashi Uemura Shigeru Sugatani Junko Yoshikawa Norishige |
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Affiliation: | Department of Pediatrics, Wakayama Medical University, Wakayama, Japan. takaomi@wakayama-med.ac.jp |
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Abstract: | OBJECTIVE: 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. |
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Keywords: | CAL, Coronary artery lesion CRP, C-reactive protein IL, Interleukin IVIG, Intravenous immunoglobulin KD, Kawasaki disease PAF, Platelet-activating factor PAF-AH, Platelet-activating factor acetylhydrolase PCR, Polymerase chain reaction TNF, Tumor necrosis factor WBC, White blood cell |
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