儿童川崎病100例临床特征及随访分析

The clinical features and follow-up study of 100 cases of Kawasaki disease

目的探讨儿童川崎病(KD)的临床、治疗和预后特点。方法对2000—2005年广东省中山市博爱医院儿科收治的100例KD患儿进行随访,对其临床特征、治疗方案以及预后进行回顾分析。结果 (1)临床特点:发病年龄(2.01±1.35)岁,随访年龄(8.2±1.7)岁;男女之比为1.7∶1。典型KD88例,其中再发病例2例;不完全KD12例。心血管系统并发症25例,包括冠状动脉改变22例,心包积液2例,心肌炎1例,其中一过性冠脉扩张18例,冠脉瘤形成4例;非心血管系统并发症包括肝损害34例,胆囊积液3例,麻痹性肠梗阻1例,无菌性脑膜炎3例,面神经麻痹5例,肺炎53例,尿道炎12例,关节炎6例。(2)治疗和疗效:89例病程10d内给予丙种球蛋白(IVIG)2g/kg,12例无效,需追加第2次IVIG,其中2例加用肾上腺皮质激素。11例亚急性期确诊者给予IVIG1～2g/kg,其中3例并发冠脉瘤(P<0.05)。无冠脉损害者病程6～8周停药,18例有冠脉扩张者跟踪至半年至1年后停药,3例中小型冠脉瘤者在病程1～3年停药,1例巨大冠脉瘤者服用阿司匹林至今已7年。(3)随访和预后:56例追踪随访至病程≥5年,最长10年。随访心脏彩超异常10例(17.8%),其中1例巨大冠脉瘤者仍然呈冠脉瘤样扩张,其余为肺动脉瓣或三尖瓣反流,心功能均正常。随访运动负荷心电图(EECG)异常17例(30.3%),仅5例起病时有心血管并发症(P>0.05)。4例起病时并发冠脉瘤者EECG均无明显异常;非心血管并发症均恢复正常;无死亡病例。结论 (1)KD冠脉并发症多以一过性冠脉扩张为主;冠脉瘤的发生与确诊及IVIG使用时机相关。(2)发病时有心血管并发症者与5年后随访EECG异常无相关性,但远期EECG改变多,需要长期随访。(3)非心血管并发症涉及多系统,但预后良好。

Objective To explore the clinical features, etiological factors, treatments and prognosis of 100 cases of Kawasaki disease （KD）. Methods Totally 100 patients with KD in our department treated from 2000 to 2005 was included in the study. A retrospective study was carried out to analyze clinical characteristics, treatment and outcomes of these patients. Results Clinical features： age of onset （2.01 ± 1.35） years, the age of follow-up （8.2 ± 1.68） years;male to female ratio was 1.7 ： 1. Tipieal KD （88）, including recurrence KD （2）, incomplete KD （12）. Cardiova-scular complications existed in 25 patients, including coronary artery complications （22）, pericardial effusion （ 2 ）, myocarditis （ 1 ）, transient coronary artery ectasia （18） and coronary aneurysm formation （4）, （small tumors in 1 case, medlum-sized tumor in 2 cases and a huge tumor in 1 ease ）. Non-cardiovascular complications cases included hepatitis or impaired liver function （ 34）, gallbladder hydrops （ 3 ）, paralytic intestinal obstruction （ 1 ）, aseptic meningitis （ 3 ）, facial palsy （ 5 ）, pneumonia （53）, urethritis （12） and arthritis （6）. Treatment and efficacy： 89 patients within 10 days of the course were given IVIG 2 g/kg; 12 patients without response were given a 2nd IVIG; 2 IVIG non-responsive patients were added the use of glucoeorticoid; 11 cases diagnosed as subacute were given IVIG 1 -2 g/（kg, d） , 3 cases with coronary aneurysm formation （P 〈 0.05）.Those without coronary artery lesions withdrawed ihe treatment at the course of 6 to 8 weeks; 18 patients with coronary artery dilation were tracked from 6 months to 1 year to discontinuation; 3 cases of small and medium-sized coronary aneurysm withdrawed in the course of 1 to 3 years; 1 case of giant coronary aneurysm was given aspirin for 7 years. Follow-up and prognosis ： 56 patients were followed up to ≥ 5 years, up to 10 years. Abnormal follow-up eehocardiography occurred in 10 cases （17.8%）.Among them, 1 case of giant coronary artery aneurysms was currently still on the expansion of tumor-like coronal＇y artery, for the others the pulmonary valve or tricuspid valve regurgitation and cardiac function were normal. Exetvise EECG abnormalities existed in 17 cases （30.3%）. Among them, 5 cases were with cardiovascular complications（5 ）（P 〉 0.05 ）. The course of 4 cases with coronary aneurysm had no obvious abnormalities of exercise ECG. Non-cardiovascular complication cases recovered completely. No deaths occurred. Conclusion Most of KD coronary complications are transient coronary artery ectasia. The occurrence of coronary aneurysm is related to the time of diagnosis and use of IVIG. No specific changes of the exercise ECG exist in the patients with coronary after7 complication who are in the acute period and have been followed for five years. The patients with more EECG abnormalities need long-time follow-up. Non-cardiovascular complications involve muhiple systems, but the prognosis is good.