Effect of polycmorphism in plasminogen activate inhavitor on the occunence ofcardiac complication in Kawasaki disease

纤溶酶原激活因子多态性对川崎病心脏并发症发生的影响

• 批准号: 12670800
• 负责人: SHIRAHATA Akira
• 金额: $ 2.11万
• 依托单位: University of Occupational and Environmental Health
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2000
• 资助国家: 日本
• 起止时间: 2000 至 2001
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-12670800/
• 关键词: Kawasaki desease; polymorphism; tissue plasminogen activator; plasminogen activator; cardiac complication; プラスミノゲン・アクチベーター・インヒビター

1.Time course of fibrinolytic system in patients with Kawasaki disease (KD) during acute phase.To determine whether the fibrinolytic system is related to the occurrence of cardiac ccmplication in KD, we measured tissue plasminogen activator (1PA), plasminogen activator inhibitor-1 (PAI-1), and related factors in the plasma of children with KD. TPA and PAI-1 were increased in acute phase. Ch the other hand, TPA/lPAI-1 ratio was lower in the cardiac complication group than in the no ccmplication group throughout the observation period These results indicate that a low fibrinolytic activity may be related to the occurrence of cardiac Duplication in KD.2.Polymorphism in PAI-1 in KDPAI-1 polymorphismwas investigated in patients with KD and controls. Genotype (4G, 5G allele of PAI-1gene) was determined, using PCR assays based on published method The distribution of 4G/4G, 4G/5G and 5G/5G genotype was similar in KD and control. On the other hand, weak association of homozygote of 4G with no cardiac ccmplication were observed in KD. These results provide preliminary evidence that polymorphism in PAI-1 does not contribute to occurrence of KD and cardiac complication in KD.

1.为探讨川崎病（KD）患儿急性期纤溶系统的时程变化，测定了KD患儿血浆组织型纤溶酶原激活物（1 PA）、纤溶酶原激活物抑制物-1（派-1）及其相关因子的含量。TPA和派-1在急性期升高。心脏并发症组TPA/lPAI-1比值在观察期内均低于无并发症组，提示KD患者纤溶活性低下可能与心脏复制有关。采用PCR方法检测派-1基因4G、5G等位基因，4G/4G、4G/5G、5G/5G基因型在川崎病组和对照组中的分布相似。而在KD中，4G纯合子与无心脏并发症之间的相关性较弱。这些结果为派-1基因多态性与川崎病及其心脏并发症的发生无关提供了初步证据。

项目成果

Yoshioka A., Shima M., Fukutake K., Takamatsu J., Shirahata A., et al.: "Safety and efficacy of a new recombinant FVIII formulated with sucrose (rFVII-FS) in patients with haemophilia A : a long-term, multtcentre clinical study in Japan"Haemophilia. 7. 24

Yoshioka A.、Shima M.、Fukutake K.、Takamatsu J.、Shirahata A. 等人：“用蔗糖配制的新型重组 FVIII (rFVII-FS) 在血友病 A 患者中的安全性和有效性：

白幡 聡: "紫斑・出血傾向"小児科診療. 64・11. 1766-1772 (2001)

白畑聪：“紫癜/出血倾向”儿科 64・11（2001）。

Sakai M, Shirahata A et al.: "Low TPA relative to PAI-1 as a marker of cardiac complication in children with Kawasaki disease"Clin Appl Thrombosis/Hemostasis. 7・3. 214-218 (2001)

Sakai M、Shirahata A 等：“相对于 PAI-1 的低 TPA 作为川崎病儿童心脏并发症的标志物”Clin Appl Thrombosis/Hemostasis 7·3 (2001)。

酒井道生, 白幡 聡: "抗血小板療法の基礎と臨床"小児科診療. 64・8. 1175-1181 (2001)

Michio Sakai，Satoshi Shirahata：“抗血小板治疗的基础和临床实践”儿科学 64・8（2001）。

白幡 聡: "小児の出血傾向の診断と治療の進め方"日本医事新報. 4020. 19-26 (2001)

白畑聪：“如何进行儿童出血倾向的诊断和治疗”日本医学报。 4020. 19-26 (2001)。