Vasculitis in Kawasaki disease : the molecular and vascular biological studies

冠状动脉病变川崎病（KD）患者外周血单个核细胞对细菌产物产生较高水平的肿瘤坏死因子(TNF)- α。tnf - α基因侧翼5个区域的多态性均与KD无关。这些结果表明，针对细菌产物产生tnf - α过量的遗传倾向可能参与了KD的发病机制（Kamizono S, Kato H, et al.）。儿科，1999)。我们通过冠状动脉内输注乙酰胆碱和硝酸异山梨酯来研究冠状动脉内皮功能，并通过血管内超声（IVUS）成像评估KD后长期血管壁形态。长期的冠状动脉病变，即使在动脉瘤消退后，也可能导致内皮功能受损和血管壁形态异常（Yamakawa R, Kato H，等）。李春华，李春华，李春华，等。心2000)。类固醇脉冲治疗可能对静脉注射免疫球蛋白（IVIG）抵抗的川崎病患者有帮助。然而，在类固醇脉冲治疗期间观察到冠状动脉的短暂扩张（Hashino K， Kato H等）。儿科，出版中)。我们研究了选择素家族可溶性形式（P-、E-和L-选择素）的血浆水平与KD患者CAL发病率之间的关系。血浆选择素水平的改变是KD患者重要的临床分子标志物。e -选择素分子可能有潜力作为KD患者CAL发病率的预测因子（Furui J Kurume Med J, in press）。血管内皮生长因子（Vascular endothelial growth factor， VEGF）参与了KD的发病机制，特别是在CAL的发展中，VEGF可能成为IVIG耐药KD患者CAL的有用预测因子（Furui J, Kato H, et al.）。JACC 2001, abstract)。

The peripheral blood mononuclear cells of Kawasaki disease (KD) patients with coronary artery lesions produced higher levels of tumor necrosis factor (TNF)-alpha in response to bacterial products. None of the polymorphisms in the 5 flanking region of the TNF-alpha gene were related to KD.These results suggest that a genetic disposition towards overproduction of TNF-alpha in response to bacterial products may be involved in the pathogenesis of KD (Kamizono S, Kato H, et al. Pediatr Int. 1999). We investigated the coronary endothelial function using intracoronary infusions of acetylcholine and isosorbide dinitrate and evaluated vascular wall morphology using intravasucular ultrasound (IVUS) imaging long-term after KD.Long-term coronary artery lesions, even after aneurysm regression, may have impaired endothelial function and abnormal vascular wall morphology (Yamakawa R, Kato H, et al. JACC, 1998, Iemura M, Kato H, et al. Heart 2000). The steroid pulse therapy migt be useful in patients with intravenous immune globulin (IVIG)-resistant Kawasaki disease who experience prolonged fever. However, transient dilatation of the coronary artery was observed during steroid pulse therapy (Hashino K, Kato H, et al. Pediatr Int, in press). We investigated the relationship between the plasma levels of soluble forms of selectin family (P-, E-, and L- selectin) and the incidence of CAL in KD patients. Alterations in plasma selectin levels are important clinical molecular markers KD patients. E-selectin molecules may have potential as a predictor of incidence of CAL in patients with KD (Furui J Kurume Med J, in press). Vascular endothelial growth factor (VEGF) was involved in the pathogenesis of KD, especially in the developing of CAL.The VEGF may have possibility to become useful predictor of CAL in patients with IVIG resistant KD (Furui J, Kato H, et al. JACC 2001, abstract).