川崎病丙种球蛋白治疗策略与心脏损害及冠状动脉病变的相关性研究(1998-2008上海地区调查报告)(英文)

目的 川崎病(Kawasaki disease,KD)是一种病因未明的全身血管炎性综合征,伴冠状动脉病变(coronary artery lesion,CAL);大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,IVIG)治疗KD的临床疗效肯定,但目前IVIG的用法和用量尚存在争议.该研究主要为评价不同IVIG方法治疗.KD的效果,探讨最佳治疗方案.方法 由上海市儿科心血管学组制定统一的KD调查表,发放到上海提供儿科服务的50家医院.回顾性分析1998-2008年上海地区住院KD患者的临床资料.共收集完全符合要求的KD患者资料表格1682例,其中男性1064例(63.3%),女性618例(36.7%);发病年龄(2.57±2.33)岁(0.1～18.8岁).治疗KD的IVIG方案包括1 g/ks×1次、2 g/kg×1次、0.4～0.5 g/kg×5次、1 g/kg×2次、2 g/kg×2次及其他.采用SAS 6.12统计软件包进行统计分析,计数资料采用X2检验计算;计量资料数据以X-±s表示,采用t检验.结果 在KD病程的5～10 d应用IVIG有助于最大化降低KD患者的CAL发生率;所有IVIG的KD患者中,应用方案1g/kg×2次治疗者心脏损害、冠脉病变的发生率均为最低,差异有统计学意义(P<0.05).结论 在KD病程5～10 d以IVIG 1g/kg×2次的剂量,有助于最大化降低KD患者的CAL发生率.

Cardiac/coronary artery lesion in Kawasaki disease treated with different kind of intravenous immunoglobulin therapy in Shanghai from 1998-2008

Objective To evaluate the effect of different kind of intravenous immunoglobulin (IVIG) therapy in treating Kawasaki disease (KD) and preventing cardiac consequences (coronary artery lesion, CAL). Methods A questionnaire form and guideline for KD diagnosis were sent to 50 hospitals providing pediatric medical care in Shanghai. The data from a total of 1 682 KD patients were collected. It included 1 064 males and 618 females from 1998 through 2008 in Shanghai. The average age of the KD patients was (2.57±2.33) years old (0.1-18.8 years).The patients had been divided into 6 groups for different IVIG therapy, which included 1 g/kg once, 2 g/kg once, 0.4-0.5 g/kg five times, 1 g/kg twice, 2 g/kg twice and others. SAS 6.12 software was used for statistical analysis. Results In all KD patients, the patients treated with IV1G in 5th-10th day of illness has the least cardiac complication and CAL incidence and the group with IVIG therapy of 1 g/kg twice also has the least cardiac complication and CAL incidence. Conclusions The best doses of IVIG in treating KD is 1 g/kg twice and the IVIG therapy should be used in 5th-10th day of KD illness.