儿童川崎病休克综合征35例分析

Clinical analysis of Kawasaki disease shock syndrome in 35 children

目的探讨儿童川崎病休克综合征(KDSS)的临床特征、治疗方案及预后。方法回顾性分析2014年1月至2020年12月浙江大学医学院附属儿童医院收治的35例KDSS患儿的临床资料。根据是否入儿童重症监护室(PICU),分为PICU组及非PICU组;根据是否合并冠状动脉病变(CALs),分为CALs组与无CALs组;根据首次丙种球蛋白(IVIG)输注后反应分为IVIG抵抗组和IVIG敏感组;比较不同组别间临床特征及实验室指标的差异。结果35例患儿中,不完全川崎病18例(51.43%),25例(71.43%)患儿转入PICU进一步治疗。心血管系统受累18例(51.43%),消化系统受累30例(85.71%),肾脏及泌尿系统受累27例(77.14%),呼吸系统受累24例(68.57%),神经系统受累6例(17.14%)。35例患儿均使用IVIG联合阿司匹林口服、液体复苏治疗,15例(42.86%)患儿IVIG抵抗,9例(25.71%)患儿接受第2剂IVIG,23例(65.71%)患儿使用糖皮质激素治疗,19例(54.29%)患儿使用血管活性药物,无死亡病例。PICU组及非PICU组患儿总住院时间、CALs例数、血管活性药物使用情况、血管活性药物评分比较差异均有统计学意义(均P<0.05),CALs组与无CALs组患儿总住院时间、入PICU例数比较差异均有统计学意义(均P<0.05),IVIG抵抗组及IVIG敏感组患儿扩容量比较差异有统计学意义(P<0.05)。结论KDSS是川崎病的一种严重表现形式,绝大部分患儿需入PICU治疗。KDSS患儿CALs、IVIG抵抗发病率更高。消化系统、呼吸系统、肾脏及心血管系统为常受累的脏器。大部分患儿对大剂量IVIG加糖皮质激素冲击治疗反应良好。

Objective To analyze the clinical features,treatment methods and prognosis of children with Kawasaki disease shock syndrome(KDSS).Methods The medical records of all consecutive inpatients with KDSS admitted in the Children's Hospital,Zhejiang University School of Medicine from January 2014 to December 2020 were retrospectively reviewed.Patients were divided into pediatric intensive unit(PICU)group or non-PICU group,coronary artery lesions(CALs)or non-CALs group,intravenous immunoglobulin(IVIG)resistance group or IVIG responder group.Clinical characteristics were analyzed and compared among groups.Results Thirty-five children met the inclusion criteria of KDSS,among whom 18 cases(51.43%)had incomplete Kawasaki disease(KD),25 cases(71.43%)were required PICU admission for severe manifestations.Cardiovascular system involvement was present in 18 cases(51.43%),digestive system involvement was present in 30 cases(85.71%),renal and urinary system involvement was documented in 27 cases(77.14%),respiratory system involvement was documented in 24 cases(68.57%),central nervous system involvement was documented in 6 cases(17.14%).All patients received IVIG combined with oral aspirin and liquid resuscitation.Fifteen cases(42.86%)had IVIG resistance,9(25.71%)cases needed a second dose of IVIG.Twenty-three cases(65.71%)received systemic steroids,19 cases(54.29%)were required inotropic treatment.No death occurred.PICU group had significantly higher vasoactive-inotropic score,longer length of hospital stay,higher number of CALs,higher use of vasoactive-inotropic(all P<0.05).The CALs group had longer length of hospital stay,higher proportion of PICU admission(both P<0.05).IVIG responder group had significantly higher fluid volumes(P<0.05).Conclusion KDSS is a severe condition related to KD,which requires critical care in PICU.KDSS patients have a higher incidence of CALs and IVIG resistance.They are more likely to have higher rats of digestive system,respiratory system,renal and cardiovascuar system involvement.For most patients,multiple doses of IVIG and corticosteroids are generally used and are sufficient to control the disease.