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

血浆血小板激活因子及血浆血小板激活因子乙酰水酶多态性在川崎病发生发展中的研究

• 批准号: 14570762
• 负责人: TAKEUCHI Takashi
• 金额: $ 2.18万
• 依托单位: Wakayama Medical University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2002
• 资助国家: 日本
• 起止时间: 2002 至 2004
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-14570762/
• 关键词: Kawasaki Disease; platelet-activating factor(PAF); PAF acetylhydrolase (PAF-AH); Immunoglobulin therapy; Immunoglobulin resistant case

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

目的：探讨是否血浆血小板活化因子乙酰水解酶（PAF-AH）水平降低，由于遗传多态性参与了川崎（KD）的发病机制。研究设计：在76名日本KD儿童和112名日本健康成人中，采用等位基因特异性聚合酶链反应定量分析PAF-AH基因V279 F多态性（G/T颠换）的频率。探讨KD患者基因型、临床特征与静脉免疫球蛋白（IVIG）耐药的关系。结果：PAF-AH基因型频率在两组间差异无统计学意义（P=0.51）。与GG（正常基因型）组相比，GT（杂合子）+TT（纯合子缺陷型）组中需要额外IVIG的患者明显增多（52%vs14%，P=0.001）。GT+TT组的发热持续时间和最大血清C反应蛋白水平也显著增加（P分别为0.012和0.036），而血浆PAF-AH活性显著降低（P<0.0001）。我们认为，血浆PAF-AH基因V279 F多态性和由此引起的酶缺乏是IVIG无效的因素之一。日本急性KD患者的反应

项目成果

Cerebrospinal fluid cytokines in Salmonella urbana encephalopathy

城市沙门氏菌脑病中的脑脊液细胞因子

• 发表时间: 2004
• 期刊: Tohoku J Exp Med 203(2)
• 作者: Minami K;et al.;Koichi Minami et al.;Koichi Minami et al.
• 通讯作者: Koichi Minami et al.

Yoshioka T, Matsutani T, Toyosaki-Maeda T, Suzuki H, et al.: "Relation of streptococcal pyrogenic exotoxin C as a causative superantigen for Kawasaki disease"Pediatr Res. 53(3). 403-410 (2003)

Yoshioka T、Matsutani T、Toyosaki-Maeda T、Suzuki H 等：“链球菌热原性外毒素 C 作为川崎病致病超抗原的关系”Pediatr Res。

Detection of auto-antibodies against a 70 kDa protein derived from vascularsmooth muscle cells in patients with Kawasaki disease.

检测源自川崎病患者血管平滑肌细胞的 70 kDa 蛋白自身抗体。

• 发表时间: 2002
• 期刊: Eur J Pediatr 161
• 作者: Suzuki H;Muragaki Y;Uemura S;et al.
• 通讯作者: et al.

南 孝臣, 鈴木啓之, 武内 崇 他: "急性期CRP低値で経過し、冠動脈瘤を形成した川崎病男児例"Prog Med. 23(7). 1737-1740 (2003)

Takaomi Minami、Hiroyuki Suzuki、Takashi Takeuchi 等人：“一名患有川崎病的男孩因急性期 CRP 低而发生冠状动脉瘤”Prog Med 23(7) (2003)。

子供の川崎病発症を契機に発見された川崎病親子例

儿童川崎病发病后发现川崎病亲子病例

• 发表时间: 2003
• 期刊: 日本小児科学会雑誌 107
• 作者: 花井直美;鈴木啓之;南 孝臣;武内 崇 他
• 通讯作者: 武内 崇 他