摘要目的:分析川崎病(KD)合并自发性寰枢椎半脱位(AARS)患儿的临床特点。方法:回顾性分析2010年12月至2019年12月在重庆医科大学附属儿童医院确诊为KD的住院患儿资料，合并AARS(脱位组)及未发生AARS(对照组)各60例，并采用 χ2检验及 t检验比较2组临床特点。 结果:KD患儿8 365例中合并AARS共60例(0.72%)，好发于急性期，好发年龄为3~6岁( P<0.001)，其首发临床表现为发热伴颈部活动受限(100.00%)、颈部包块(66.67%)、斜颈(21.67%)、颈痛(11.67%)。颈部CT或X线示AARS表现，部分伴颈部软组织增厚、肿胀。脱位组患儿唇红皲裂( P＝0.01)和颈淋巴结大( P<0.001)较对照组显著，中性粒细胞绝对和相对计数均高于对照组(均 P<0.05)，颈部B超较对照组颈软组织肿胀、增厚更明显( P<0.05)，但2组患儿在冠状动脉病变及静脉注射丙种球蛋白(IVIG)联合阿司匹林疗效方面，差异无统计学意义( P>0.05)，头颅牵引可一定程度缓解患儿颈部症状，但疗效差异无统计学意义( P>0.05)。 结论:颈淋巴结大、唇红皲裂、外周血中性粒细胞计数的增高和颈部软组织的肿胀、增厚是KD合并AARS的危险因素，但该合并症不增加冠状动脉损伤及IVIG无应答风险，KD抗炎治疗是关键，患儿多数预后良好。

abstractsObjective:To investigate the clinical characteristics of children with Kawasaki disease (KD) complicated with atlantoaxial rotatory subluxation (AARS).Methods:Clinical characteristics of 60 AARS patients complicated with KD (the atlantoaxial rotatory subluxation group) and 60 patients with KD only diagnosed (the control group)in the Children′s Hospital of Chongqing Medical University from December 2010 to December 2019 were retrospectively analyzed.Differences between groups were compared by the Chi- square test and the t test. Results:A total of 8 365 KD patients were diagnosed during the study period, involving 60 cases (0.72%) complicated with AARS.which usually occurred in the acute phase with the onset ages of 3 to 6 years ( P<0.001). Initial clinical symptoms of KD complicated with AARS included fever with restricted neck movement (100.00%), neck mass (66.67%), torticollis (21.67%) and neck pain (11.67%). CT or X-ray exa-mination of the neck indicated AARS, with thickening and swelling of the cervical soft tissues in some cases.Compared with those of control group, red, dry, cracked lips ( P=0.01) and cervical lymph node swollen ( P<0.001) were significantly pronounced in KD patients complicated with AARS.The absolute and relative count of neutrophils were significantly higher in KD patients complicated with AARS (all P< 0.05). Cervical soft tissue swelling and thickening in B-ultrasound were more obvious than those in the control group( P<0.05). However, there were no significant differences in coronary artery lesions and the response to intravenous immunoglobulin (IVIG) combined with Aspirin between groups ( P>0.05). Head traction could relieve neck symptoms to a certain extent, but there was no significant difference between groups ( P>0.05). Conclusions:Cervical lymphadenopathy, red, dry, cracked lips, increase of absolute and relative count of neutrophils, and swelling and thicke-ning of cervical soft tissues were the high-risk factors of KD complicated with AARS.The complication of AARS in KD patients did not increase the risk of coronary artery injury and IVIG resistance.IVIG combined with aspirin achieved a good prognosis in the majority of KD patients complicated with AARS.