Background The epidemiologic pictures of Kawasaki disease （KD） in Shanghai from 1998 through 2002 were reported while the current status of KD in the following five years remains unknown. Methods A questionnaire for...Background The epidemiologic pictures of Kawasaki disease （KD） in Shanghai from 1998 through 2002 were reported while the current status of KD in the following five years remains unknown. Methods A questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited. Results In total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 （mean 46.32±6.51） per 100 000 children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of （27.32±7.11） per 100 000, （t=4.406, P=0.002）. Ages at onset ranged from 12 days to 13.6 years （median 1.8 years）. It occurred more frequently in summer and spring. Coronary arterial lesions （CAL）, defined as ectasia or aneurysm, accounted for 19.8% （232 cases）. Flattened or inverted T wave was the most frequent finding （194 cases, 20.5%） by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases （86.6%）. The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice. Conclusions The incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary seauelae.展开更多
Objective: High-dose intravenous immunoglobulin (IVIG) decreases the risk for development of coronary arterial lesions (CAL) in Kawasaki disease (KD) while patients resistant to IVIG have higher risk. This study is ai...Objective: High-dose intravenous immunoglobulin (IVIG) decreases the risk for development of coronary arterial lesions (CAL) in Kawasaki disease (KD) while patients resistant to IVIG have higher risk. This study is aimed to investigate an additional therapy affects the risk for CAL in IVIG resistant KD. Design: The information from 11,510 children with KD was collected by the nationwide survey (2003-2004) in Japan. The secondary therapeutic strategies for IVIG resistant KD were an additional IVIG dose, steroids or both. The incidence of CAL was compared among 4 groups: G1, children responded to initial IVIG;G2, IVIG resistant patients receiving an additional IVIG dose;G3, IVIG resistant patients receiving additional steroids;G4, IVIG resistant patients receiving an additional IVIG dose plus steroids. CAL was assessed at 30th day of illness using ultrasound echocardiography. Results: 2229 patients (19.4%) were resistant to initial IVIG. Incidence of CAL was significantly lower in children responding to initial IVIG than in IVIG resistant patients (1.9% and 11.0%, respectively;odd’s ratio 6.3). The incidences of CAL in an each additional therapy group were as follows: G2: 6.7%, G3: 9.7%, G4: 22.2%. The risk for CAL was significantly higher in G4 as compared with G2 and G3. Giant aneurysmal formation (GA) was more frequently developed in G3 and G4 consisting of the patients receiving steroids (G1: 0.03%, G2: 0.63%, G3: 4.3%, G4: 3.7%). Conclusion: The incidence of CAL was considerably high in IVIG resistant KD and an additional therapy by steroids may increase the risk for GA.展开更多
晚期糖基化终末产物受体(receptor for advanced glycation end products,RAGE)属于一种细胞表面免疫球蛋白超家族分子,表达于多种细胞表面,如单核巨噬细胞、血管内皮细胞、平滑肌细胞及神经元细胞等。目前川崎病(Kawasaki disease,KD)...晚期糖基化终末产物受体(receptor for advanced glycation end products,RAGE)属于一种细胞表面免疫球蛋白超家族分子,表达于多种细胞表面,如单核巨噬细胞、血管内皮细胞、平滑肌细胞及神经元细胞等。目前川崎病(Kawasaki disease,KD)的发病机制尚不明确,但近年来发现RAGE在川崎病循环内皮细胞高表达。在体内多种配体可与RAGE结合,通过介导炎症因子释放和炎症通路活化等作用来影响KD的发病。阻断RAGE的策略可能干预川崎病冠脉病变进程,以期待为川崎病提供新的治疗方法。本文对RAGE途径在川崎病中的研究进展及阻断RAGE的策略进行综述。展开更多
文摘Background The epidemiologic pictures of Kawasaki disease （KD） in Shanghai from 1998 through 2002 were reported while the current status of KD in the following five years remains unknown. Methods A questionnaire form and diagnostic guidelines for KD were sent to 50 hospitals providing pediatric medical care in Shanghai, China. All patients with KD diagnosed during January 2003 through December 2007 were recruited. Results In total, 1187 cases of KD were enrolled. The incidence of KD was 36.78 to 53.28 （mean 46.32±6.51） per 100 000 children under the age of 5 years between 2003 and 2007, which was higher than the year from 1998 to 2002 of （27.32±7.11） per 100 000, （t=4.406, P=0.002）. Ages at onset ranged from 12 days to 13.6 years （median 1.8 years）. It occurred more frequently in summer and spring. Coronary arterial lesions （CAL）, defined as ectasia or aneurysm, accounted for 19.8% （232 cases）. Flattened or inverted T wave was the most frequent finding （194 cases, 20.5%） by electrocardiogram. Intravenous gamma-globulin was administrated to 1028 cases （86.6%）. The occurrence of CAL seemed less frequent in the patients received gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice. Conclusions The incidence of KD was increasing in Shanghai. Treatment with intravenous gamma-globulin from day 5 to day 9 after the onset with the regimen of 1000 mg/kg once or 1000 mg/kg twice resulted in less coronary seauelae.
文摘Objective: High-dose intravenous immunoglobulin (IVIG) decreases the risk for development of coronary arterial lesions (CAL) in Kawasaki disease (KD) while patients resistant to IVIG have higher risk. This study is aimed to investigate an additional therapy affects the risk for CAL in IVIG resistant KD. Design: The information from 11,510 children with KD was collected by the nationwide survey (2003-2004) in Japan. The secondary therapeutic strategies for IVIG resistant KD were an additional IVIG dose, steroids or both. The incidence of CAL was compared among 4 groups: G1, children responded to initial IVIG;G2, IVIG resistant patients receiving an additional IVIG dose;G3, IVIG resistant patients receiving additional steroids;G4, IVIG resistant patients receiving an additional IVIG dose plus steroids. CAL was assessed at 30th day of illness using ultrasound echocardiography. Results: 2229 patients (19.4%) were resistant to initial IVIG. Incidence of CAL was significantly lower in children responding to initial IVIG than in IVIG resistant patients (1.9% and 11.0%, respectively;odd’s ratio 6.3). The incidences of CAL in an each additional therapy group were as follows: G2: 6.7%, G3: 9.7%, G4: 22.2%. The risk for CAL was significantly higher in G4 as compared with G2 and G3. Giant aneurysmal formation (GA) was more frequently developed in G3 and G4 consisting of the patients receiving steroids (G1: 0.03%, G2: 0.63%, G3: 4.3%, G4: 3.7%). Conclusion: The incidence of CAL was considerably high in IVIG resistant KD and an additional therapy by steroids may increase the risk for GA.
文摘晚期糖基化终末产物受体(receptor for advanced glycation end products,RAGE)属于一种细胞表面免疫球蛋白超家族分子,表达于多种细胞表面,如单核巨噬细胞、血管内皮细胞、平滑肌细胞及神经元细胞等。目前川崎病(Kawasaki disease,KD)的发病机制尚不明确,但近年来发现RAGE在川崎病循环内皮细胞高表达。在体内多种配体可与RAGE结合,通过介导炎症因子释放和炎症通路活化等作用来影响KD的发病。阻断RAGE的策略可能干预川崎病冠脉病变进程,以期待为川崎病提供新的治疗方法。本文对RAGE途径在川崎病中的研究进展及阻断RAGE的策略进行综述。
