2004 Fiscal Year Final Research Report Summary
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
| Project/Area Number |
14570762
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Wakayama Medical University |
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Principal Investigator |
TAKEUCHI Takashi Wakayama Medical University, Pediatrics, Assistant Professor, 医学部, 講師 (10246522)
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| Co-Investigator(Kenkyū-buntansha) |
YOSHIKAWA Norishige Wakayama Medical University, Pediatrics, Professor, 医学部, 教授 (10158412)
SUZUKI Hiroyuki Wakayama Medical University, Pediatrics, Assistant Professor, 医学部, 講師 (80196865)
UEMURA Shigeru Wakayama Medical University, Pediatrics, Associate Professor, 医学部, 助教授 (50137262)
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| Project Period (FY) |
2002 – 2004
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| Keywords | Kawasaki Disease / platelet-activating factor(PAF) / PAF acetylhydrolase (PAF-AH) / Immunoglobulin therapy / Immunoglobulin resistant case |
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| Research Abstract |
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
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