16例川崎病休克综合征临床分析

Clinical manifestations of 16 cases with Kawasaki disease shock syndrome

目的分析川崎病休克综合征（KDSS）的临床特点，提高临床医生的诊治水平及改善患儿预后。方法回顾分析我院2013年1月至2017年4月住院的川崎病（KD）患儿共924例，其中KDSS患儿16例。随机选取同期住院的KD患儿30例为对照组。对比分析两组患儿的临床特点、实验室检查和治疗措施。结果KDSS患儿男9例，女7例。平均年龄（3.95±2.56）岁。出现休克的平均时间（4.31±0.79）d。与KD组比较，KDSS组性别、年龄、发热持续时间差异无统计学意义（P〉0.05）。KDSS患儿更容易出现腹痛、肝功能异常（包括转氨酶升高和黄疸）、蛋白尿、腹腔积液、肺炎、冠状动脉瘤和IVIG抵抗，住院时间更长（P〈0.05）。KDSS组的外周血白细胞计数[（28.42±10.46）×10^9/L比（20.34±7.57）×10^9/L]、中性粒细胞比例[（89.86±7.00）%比（73.14±13.91）% ]、超敏C-反应蛋白（mg/L）[181.95（141.58，218.00）比94.65（55.33，10^9.50）]、降钙素原（ng/ml）[9.64（4.85，12.07）比0.09（0.04，0.37）]、血清铁蛋白（ng/ml）[（388.12±241.75）比（169.86±95.14）]等炎症指标均高于KD组患儿（均P〈0.05）。两组患儿的红细胞沉降率（mm/h）[（75.31±25.25）比（79.87±22.76）]、纤维蛋白原（g/L）[（6.17±1.45）比（6.03±1.47）]差异无统计学意义（P〉0.05）。KDSS组患儿血小板计数[（655.50±226.98）×10^9/L比（549.93±119.15）×10^9/L]更高；而白蛋白（g/L）[（22.54±5.13）比（33.32±3.18）]、血钠（mmol/L）[（130.47±2.79）比（134.77±2.81）]、血钾（mmol/L）[（2.89±0.74）比（4.04±0.43）]水平及心脏彩超射血分数[（60.16±6.18）%比（64.81±3.71）% ]均更低（均P〈0.05）。治疗过程中14例患儿进行了液体复苏，液体量平均（27.19±19.58）ml/kg。10例患儿使用了血管活性药物。6例患儿使用激素（甲基强的松龙）治疗。所有患儿均康复出院。结论KDSS是KD的一种严重表现形式，患儿炎症指标升高更明显；更容易出现脏器功能损害，常见腹痛等消化道表现；抗休克治疗常需液体复苏并使用血管活性药物。

Objective To analyze the clinical characteristics of Kawasaki disease shock syndrome （KDSS） and to improve the diagnosis, treatment and prognosis of patients.Methods A total of 924 cases of Kawasaki disease （KD） hospitalized from January 2013 to April 2017 in our hospital were retrospectively analyzed, including 16 children with KDSS.And 30 patients with KD were randomly selected as the control group.The clinical characteristics, laboratory examination and treatment of the two groups were compared and analyzed.Results There were 9 males and 7 females in KDSS group, and average age was （3.95±2.56） years.The average time to happen shock was （4.31±0.79） days.There were no significant differences in gender, age and duration of fever between KDSS group and KD group respectively （P〉0.05）. KDSS patients were more likely to develop abdominal pain, hepatic injury （including elevated transaminase and jaundice）, proteinuria, peritoneal effusion, pneumonia, coronary aneurysm, IVIG resistance and longer hospitalization （P〈0.05）. WBC[（28.42±10.46）×10^9/L vs.（20.34±7.57） ×10^9/L], the neutrophils [（89.86±7.00）% vs.（73.14±13.91）%], hsCRP （mg/L） [181.95（141.58, 218.00） vs.94.65 （55.33, 10^9.50）], PCT（ng/ml）[9.68 （4.85, 12.07） vs.0.09 （0.04, 0.37）] and serum ferritin （ng/ml） [（388.12± 241.75） vs.（169.86±95.14）] in the KDSS group was significantly higher than those in KD group （P〈0.05）. There were no differences in ESR（mm/h） [（75.71±25.25） vs.（79.87±22.76）], fibrinogen （g/L） [（6.17±1.45） vs.（6.03±1.47）] between two groups （P〉0.05）. The levels of platelet count[ （655.50±226.98）×10^9/L vs.（549.93±119.15）×10^9/L], the albumin （g/L） [（22.54±5.13） vs.（33.32±3.18）], serum sodium（mmol/L）[（130.47±2.79） vs.（134.77±2.81）] and serum potassium （mmol/L） [（4.59 ± 0.74） vs.（4.04 ± 0.43）] and ejection fraction [（60.16±6.18）% vs.（64.81±3.71）%] in KDSS group were lower than those in KD group （P〈0.05）. During the course of treatment, 14 patients were accepted fluid resuscitation and the average volume of fluid was （27.19 ± 19.58） ml/kg.Ten patients used vasoactive drugs.Six patients were treated with hormone （methylprednisolone）. All patients were discharged from hospital.Conclusion KDSS is a serious form of KD, the indicators of inflammatory response increased more obviously.Organ damage occurs more frequently, and often accompanies abdominal pain.Anti-shock treatment often requires fluid resuscitation and use of vasoactive drugs.