川崎病并发巨噬细胞活化综合征27例的临床特征分析

Clinical features of Kawasaki disease complicated by macrophage activation syndrome: an analysis of 27 cases

摘要:

目的 分析川崎病（Kawasaki disease，KD）并发巨噬细胞活化综合征（macrophage activation syndrome，MAS）患儿的临床表现及实验室检查结果，为寻找早期诊断KD并发MAS（KD-MAS）的预警指标和早期治疗提供依据。方法 回顾性选择2014年1月—2022年1月在华中科技大学同济医学院附属武汉儿童医院诊治的27例KD-MAS患儿（KD-MAS组）及110例KD患儿（KD组）为研究对象，分析比较两组间的临床资料及实验室检查差异，采用受试者操作特征曲线分析差异有统计学意义的实验室指标对KD-MAS的诊断价值。结果 KD-MAS组患儿肝大、脾大、不完全KD、静脉注射免疫球蛋白无反应、冠状动脉受损、其他脏器受损、KD反复的发生率高于KD组，住院时长大于KD组（P<0.05）。KD-MAS组白细胞计数、中性粒细胞绝对计数、血红蛋白、血小板计数（platelet count，PLT）、红细胞沉降率、血清白蛋白、血清钠离子、前白蛋白、纤维蛋白原（fibrinogen，FIB）水平，以及非渗出性结膜炎发生率均低于KD组（P<0.05）；C反应蛋白、谷丙转氨酶、谷草转氨酶、乳酸脱氢酶（latate dehydrogenase，LDH）、血清铁蛋白（serum ferritin，SF）水平均高于KD组（P<0.05）。受试者操作特征曲线分析显示SF、PLT、FIB、LDH对KD-MAS的诊断有较高价值，曲线下面积分别为0.989、0.966、0.932、0.897（均P<0.001），其最佳诊断临界点分别为349.95 μg/L、159×10⁹/L、3.85 g/L、403.50 U/L。4个指标联合诊断KD-MAS的曲线下面积高于PLT、FIB和LDH（均P<0.05），与SF的曲线下面积比较差异无统计学意义（P>0.05）。结论 当KD患儿在治疗过程中出现肝脾大、静脉注射免疫球蛋白无反应、冠状动脉受损、KD反复等，需警惕KD-MAS的发生。SF、PLT、FIB、LDH对KD-MAS的诊断有较高价值，尤其SF对KD-MAS诊断有重要意义。

Abstract:

Objective To investigate the clinical manifestations and laboratory examination results of children with Kawasaki disease complicated by macrophage activation syndrome (KD-MAS), and to provide a basis for identifying early warning indicators for the early diagnosis and treatment of KD-MAS.Methods A retrospective study was performed on 27 children with KD-MAS (KD-MAS group) and 110 children with KD (KD group) who were admitted to Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science and Technology, from January 2014 to January 2022. Clinical and laboratory data were compared between the two groups. The receiver operating characteristic (ROC) curve was used to investigate the value of laboratory markers with statistical significance in the diagnosis of KD-MAS.Results Compared with the KD group, the KD-MAS group had significantly higher incidence rates of hepatomegaly, splenomegaly, incomplete KD, no response to intravenous immunoglobulin, coronary artery damage, multiple organ damage, and KD recurrence, as well as a significantly longer length of hospital stay (P<0.05). Compared with the KD group, the KD-MAS group had significantly lower levels of white blood cell count, absolute neutrophil count, hemoglobin, platelet count (PLT), erythrocyte sedimentation rate, serum albumin, serum sodium, prealbumin, and fibrinogen (FIB), a significantly lower incidence rate of non-exudative conjunctiva, and significantly higher levels of C-reactive protein, alanine aminotransferase, aspartate aminotransferase, lactate dehydrogenase (LDH), and serum ferritin (SF) (P<0.05). The ROC curve analysis showed that SF, PLT, FIB, and LDH had high value in the diagnosis of KD-MAS, with areas under the curve (AUC) of 0.989, 0.966, 0.932, and 0.897, respectively (P<0.001), and optimal cut-off values of 349.95 μg/L, 159×10⁹/L, 3.85 g/L, and 403.50 U/L, respectively. The combination of SF, PLT, FIB, and LDH had a larger AUC than PLT, FIB, and LDH alone in the diagnosis of KD-MAS (P<0.05), but there was no significant difference in the AUC between the combination of SF, PLT, FIB, and LDH and SF alone (P>0.05).Conclusions KD-MAS should be considered when children with KD have hepatosplenomegaly, no response to intravenous immunoglobulin, coronary artery damage, and KD recurrence during treatment. SF, PLT, FIB, and LDH are of high value in the diagnosis of KD-MAS, especially SF is of great significance in the diagnosis of KD-MAS.