PDF(Pubmed)
Abstract：
Kawasaki disease (KD) is an acute systemic vasculitis that predominantly afflicts children. KD development is known to be associated with an aberrant immune response and abnormal platelet activation, however its etiology is still largely unknown. Myosin light chain 9 (Myl9) is known to regulate cellular contractility of both non-muscle and smooth muscle cells, and can be released from platelets, whereas any relations of Myl9 expression to KD vasculitis have not been examined.
Plasma Myl9 concentrations in KD patients and children with febrile illness were measured and associated with KD clinical course and prognosis. Myl9 release from platelets in KD patients was also evaluated in vitro. Myl9 expression was determined in coronary arteries from Lactobacillus casei cell wall extract (LCWE)-injected mice that develop experimental KD vasculitis, as well as in cardiac tissues obtained at autopsy from KD patients.
Plasma Myl9 levels were significantly higher in KD patients during the acute phase compared with healthy controls or patients with other febrile illnesses, declined following IVIG therapy in IVIG-responders but not in non-responders. In vitro, platelets from KD patients released Myl9 independently of thrombin stimulation. In the LCWE-injected mice, Myl9 was detected in cardiac tissue at an early stage before inflammatory cell infiltration was observed. In tissues obtained at autopsy from KD patients, the highest Myl9 expression was observed in thrombi during the acute phase and in the intima and adventitia of coronary arteries during the chronic phase. Thus, our studies show that Myl9 expression is significantly increased during KD vasculitis and that Myl9 levels may be a useful biomarker to estimate inflammation and IVIG responsiveness to KD.
Plasma Myl9 concentrations in KD patients and children with febrile illness were measured and associated with KD clinical course and prognosis. Myl9 release from platelets in KD patients was also evaluated in vitro. Myl9 expression was determined in coronary arteries from Lactobacillus casei cell wall extract (LCWE)-injected mice that develop experimental KD vasculitis, as well as in cardiac tissues obtained at autopsy from KD patients.
Plasma Myl9 levels were significantly higher in KD patients during the acute phase compared with healthy controls or patients with other febrile illnesses, declined following IVIG therapy in IVIG-responders but not in non-responders. In vitro, platelets from KD patients released Myl9 independently of thrombin stimulation. In the LCWE-injected mice, Myl9 was detected in cardiac tissue at an early stage before inflammatory cell infiltration was observed. In tissues obtained at autopsy from KD patients, the highest Myl9 expression was observed in thrombi during the acute phase and in the intima and adventitia of coronary arteries during the chronic phase. Thus, our studies show that Myl9 expression is significantly increased during KD vasculitis and that Myl9 levels may be a useful biomarker to estimate inflammation and IVIG responsiveness to KD.
摘要：
未经证实：川崎病（KD）是一种急性系统性血管炎，主要困扰儿童。已知KD的发展与异常的免疫反应和异常的血小板活化有关。然而，其病因仍然很大程度上是未知的。已知肌球蛋白轻链9（Myl9）调节非肌肉和平滑肌细胞的细胞收缩性，可以从血小板中释放出来，而Myl9表达与KD血管炎的任何关系尚未研究。
UNASSIGNED：测量KD患者和发热患儿的血浆Myl9浓度，并与KD临床病程和预后相关。还在体外评价了KD患者中从血小板释放的Myl9。在注射干酪乳杆菌细胞壁提取物（LCWE）的小鼠发生实验性KD血管炎的冠状动脉中确定Myl9表达，以及从KD患者尸检中获得的心脏组织。
UNASSIGNED：KD患者急性期血浆Myl9水平明显高于健康对照组或其他发热性疾病患者，IVIG治疗后IVIG应答者下降，但非应答者没有下降。体外，KD患者的血小板释放Myl9独立于凝血酶刺激。在注射LCWE的小鼠中，在观察到炎性细胞浸润之前的早期阶段在心脏组织中检测到Myl9。在从KD患者尸检中获得的组织中，在急性期的血栓和慢性期的冠状动脉内膜和外膜中观察到最高的Myl9表达。因此，我们的研究表明,在KD血管炎期间Myl9表达显著增加,Myl9水平可能是评估炎症和IVIG对KD反应性的有用生物标志物.
UNASSIGNED：测量KD患者和发热患儿的血浆Myl9浓度，并与KD临床病程和预后相关。还在体外评价了KD患者中从血小板释放的Myl9。在注射干酪乳杆菌细胞壁提取物（LCWE）的小鼠发生实验性KD血管炎的冠状动脉中确定Myl9表达，以及从KD患者尸检中获得的心脏组织。
UNASSIGNED：KD患者急性期血浆Myl9水平明显高于健康对照组或其他发热性疾病患者，IVIG治疗后IVIG应答者下降，但非应答者没有下降。体外，KD患者的血小板释放Myl9独立于凝血酶刺激。在注射LCWE的小鼠中，在观察到炎性细胞浸润之前的早期阶段在心脏组织中检测到Myl9。在从KD患者尸检中获得的组织中，在急性期的血栓和慢性期的冠状动脉内膜和外膜中观察到最高的Myl9表达。因此，我们的研究表明,在KD血管炎期间Myl9表达显著增加,Myl9水平可能是评估炎症和IVIG对KD反应性的有用生物标志物.
