Kawasaki disease in New Zealand

AIM: To determine the epidemiology, management and outcome of Kawasaki disease (KD) in New Zealand. DESIGN: Prospective audit using New Zealand Paediatric Surveillance Unit (NZPSU) Reports. SETTING: Single country 2-year epidemiological study. Patients: All patients diagnosed with KD in New Zealand reported to the NZPSU from January 2001 to December 2002. MAIN OUTCOME MEASURES: Incidence of KD; time to diagnosis; use of intravenous immunoglobulin; cardiac features and outcome. RESULTS: Forty-nine new cases were identified. The annual incidence was 8.0 cases/100,000 children aged less than 5 years. Age at onset was less than 5 years in 86% of cases. Incidence was 4.6/100,000 for children of European origin, 9.6 for Maori, 12.2 for Pacific Islanders and 32.2 for children of East Asian origin. KD was diagnosed at a median of 6 days from onset of illness. 89% had fever and four or more diagnostic features. All patients had at least one echocardiogram: There was one small (2%) coronary artery aneurysm only; 13 (26%) had mild coronary artery dilatation. Thirty-five per cent did not have an echocardiogram performed four or more weeks from illness onset. 45 (92%) cases received intravenous immunoglobulin at median day six. There was one death due to occlusive coronary artery disease in a 3-month-old boy with atypical symptoms in whom KD was diagnosed at post-mortem. CONCLUSIONS: The incidence of KD in New Zealand is defined with significantly variable risk according to ethnicity. Most patients received appropriate rapid diagnosis and treatment but there was considerable variation in practice in regard to number and timing of echocardiograms. There was a low coronary artery aneurysm rate (2%). Accelerated vaso-occlusive disease was responsible for the single fatality in an atypical case.     

川崎病合并冠状动脉损害的远期转归与随访

近年对川崎病的追踪随访发现,大部分患儿预后良好,但仍有少数患儿长期遗留心血管后遗症,可发展为缺血性心脏病,一旦发生心肌梗死,病死率高.因此,美国、日本及我国等均制订了相应的川崎病患儿长期随访方案,应用各种随访手段以观察其冠状动脉损害及转归,预防川崎病引起的心血管意外发生.     

川崎病合并冠状动脉损害的远期转归与随访

近年对川崎病的追踪随访发现,大部分患儿预后良好,但仍有少数患儿长期遗留心血管后遗症,可发展为缺血性心脏病,一旦发生心肌梗死,病死率高.因此,美国、日本及我国等均制订了相应的川崎病患儿长期随访方案,应用各种随访手段以观察其冠状动脉损害及转归,预防川崎病引起的心血管意外发生.     

川崎病合并冠状动脉损害的远期转归与随访

近年对川崎病的追踪随访发现,大部分患儿预后良好,但仍有少数患儿长期遗留心血管后遗症,可发展为缺血性心脏病,一旦发生心肌梗死,病死率高.因此,美国、日本及我国等均制订了相应的川崎病患儿长期随访方案,应用各种随访手段以观察其冠状动脉损害及转归,预防川崎病引起的心血管意外发生.     

School-aged children with Kawasaki disease: high incidence of cervical lymphadenopathy and coronary artery involvement

OBJECTIVE: We describe 10 school-aged children with Kawasaki disease (KD) with a high incidence of cervical lymphadenopathy and coronary abnormality. METHODS: Based on a database of 1002 children with KD in Chang Gung Children's Hospital from January 1983 to March 2001, 10 (1%) school-aged patients (five boys, five girls) who met the diagnostic criteria of KD were included for analysis. RESULTS: Cervical lymphadenopathy was noted in all (100%) of these patients. Unilateral neck mass mimicking acute suppurative infections not responding to antibiotic therapy was the initial presentation in nine (90%) of the 10 patients. The mean interval between disease onset and diagnosis was 9.9 +/- 3.3 days (range, 6-15 days). Seven (70%) of these patients responded to one course of high-dose intravenous immunoglobulin (IVIG) therapy (2 g/kg) and oral aspirin (80-100 mg/kg per day), two (20%) required a second course of IVIG, and one (10%) responded to high-dose aspirin treatment only. Coronary artery abnormality (dilatation or aneurysm) was documented by echocardiography in seven (70%) patients (four boys, three girls). In six patients, the coronary artery abnormalities resolved in 1 year, while one patient had persistent right coronary artery aneurysm, which necessitated continued anticoagulant and low-dose aspirin therapy. CONCLUSION: The incidence of school-aged children among patients with KD is about 1% in our hospital. These patients are notable for the high incidence of initial manifestations of unilateral neck mass and coronary artery involvement. This disease should be listed as the differential diagnosis in school-aged children presenting with fever and neck mass that do not respond to antibiotic therapy.     

川崎病合并冠状动脉瘤63例临床分析

目的分析川崎病(KD)合并冠状动脉(以下简称冠脉)瘤患儿的临床特点。方法对首都医科大学附属北京儿童医院2000—2007年收治的63例超声心动图诊断为冠脉瘤的KD患儿临床资料、实验室检查、超声及心电图检查结果、治疗情况及随诊资料进行回顾性分析。结果(1)冠脉瘤患儿男性明显多于女性,男∶女为5.3∶1;冠脉巨大瘤男女比例为8.3∶1;<1岁患儿多发,占28.6%。(2)本组患儿中不完全KD、静脉注射丙种球蛋白(IVIG)抵抗以及KD复发的发生率均较高,分别为36.5%、30.2%和7.9%;急性期57例(90.5%)患儿使用IVIG冲击治疗,3例未用,3例使用情况不详;36例(57.1%)患儿发病至丙种球蛋白应用的时间间隔大于10 d。(3)超声检查发现小冠脉瘤患儿7例,中等冠脉瘤19例,巨大瘤37例,左冠脉受累者占76.2%,其中58.3%发生在前降支;右冠脉受累者达87.3%,其中47.3%发生在右冠Ⅱ段;双侧冠脉同时受累者占63.5%。(4)随诊发现71.4%冠脉瘤呈现回缩趋势,45.2%的受累分支冠脉瘤消退,平均消退时间为(2.1±1.5)年。结论对于男性、发病年龄<1岁、不完全KD、发生IVIG抵抗、复发患儿及应用IVIG治疗较晚患儿要警惕冠脉瘤的发生;左冠前降支及右冠脉瘤样病变最多见,多数冠脉瘤在恢复期发生回缩。     

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??ObjectiveTo investigate the clinical characteristics and prognosis of coronary aneurysm in children with Kawasaki disease.MethodsCoronary aneurysm was identified by using echocardiography in 63 children with Kawasaki disease from 2000 to 2007.Their gender,age,clinical presentations,use of intravenous gamma immunoglobulin (IVIG),laboratory study results and echocardiographic study results in the acute and convalescent phase were examined retrospectively.Results(1) Boys and infants were more likely to develop coronary aneurysms,the male to female ratio being 5.3??1,and for patients with giant aneurysms the male to female ratio was 8.3??1.(2)The rate of incomplete KD,IVIG non-responders and recurrence in this group of patients were 36.5%,30.2% and 7.9%,respectively,which were higher than the whole KD group.In the acute phase,57 of 63 patients were treated by IVIG with different doses,3 of them did not receive IVIG treatment and the other 3 patients?? IVIG treatments were unknown.The duration from onset to IVIG treatment was longer than 10 days in 36 of the 63 (57.1%) children.(3) Small aneurysms were detected by 2-D echo in 7 patients,medium aneurysms in 19 patients and giant aneurysm in 37 patients.In all of these patients,76.2% of them had left coronary artery aneurysms,and 87.3% of them had right coronary artery aneurysms;47.3% of them had both left and right aneurysms.In the patients with left coronary aneurysms,58.3% of them have left anterior descending branch aneurysms; and in patients with right coronary aneurysms,47.3% of them were located in the right coronary artery segment II.(4) Coronary aneurysm regressed in 71.4 % of the branches.The lumen diameter of the affected branches returned to normal in 45.2% of them,with a mean regression time of 2.1??1.5 years.ConclusionBeing male,younger than 1 year old,incomplete KD,IVIG non-responder,recurrence and later IVIG treatment are possible risk factors of coronary aneurysms in children with Kawasaki disease.Most children have multi-coronary artery aneurysms and left anterior descending branch and right coronary artery are the most commonly affected branches??and most of the coronary aneurysms have the tendency to regress during the convalescent phase.     

Septated pericarditis associated with Kawasaki disease: a brief case report

Kawasaki disease (KD) is primarily the systemic vasculitis of childhood that affects mainly the medium-sized arteries, such as the coronary arteries. KD is the leading cause of acquired heart disease, whereas the incidence of rheumatic fever has declined. The most serious complication is coronary artery involvement. Among the children with KD who developed cardiac complications, pericarditis is a rare complication, with an incidence of 0.07%. We report our experience in a 5.5-year-old child with KD complicated with aneurysm of the left anterior descendant coronary artery and septated pericardial effusion, which has not been reported in the literature. The pericardial effusion disappeared very dramatically with intravenous immunoglobulin (IVIG) therapy. We would like to point out that septated pericardial effusion in cases of KD do not need any further therapy other than IVIG and high-dose acetylsalicylic acid.     

Clinical responses of patients with Kawasaki disease to different brands of intravenous immunoglobulin

OBJECTIVE: To determine whether different brands of intravenous immunoglobulin (IVIG) administered to children with Kawasaki disease (KD) result in different outcomes. STUDY DESIGN: We analyzed children with KD and divided them into 4 groups according to the brand of IVIG. A coronary artery abnormality (CAA) was defined as having a lumen diameter (inner border to inner border) of > or =3 mm in KD cases <5 years old and > or =4 mm in cases > or =5 years old, and giant aneurysm was defined as a lumen diameter > or =8 mm. Patients were considered nonresponsive to IVIG therapy if fever persisted longer than 2 days after completion of treatment and needed retreatment with IVIG. RESULTS: We collected 437 cases, 29 (6.6%) were nonresponsive, 17 (3.9%) had CAA at convalescence, and 3 (0.7%) had giant aneurysm, 2 of whom had development of myocardial infarcts. Patients receiving Brand C IVIG, prepared with beta-propiolactone, had higher rates (10%, 9/93, P = .01) of CAA at convalescence and nonresponsiveness (13%, 12/93, P = .001); giant aneurysm occurred in 3/93 (3%) receiving Brand C IVIG and in 0/344 who received the other 3 brands (P = .008). CONCLUSIONS: IVIG, prepared with beta-propiolactone, was most significantly associated with nonresponsiveness, CAA at convalescence, and giant aneurysm. Physicians should be cautious when using IVIG prepared with beta-propiolactone or enzyme digestion to treat KD.     

川崎病治疗前发热时间与丙种球蛋白耐药的相关性临床研究北大核心CSCD

目的 探讨川崎病(Kawasaki disease,KD)患儿丙种球蛋白(intravenous immunoglobulin,IVIG)治疗前发热时间与IVIG耐药的关系。方法 回顾性收集2018年1月至2020年12月收治KD患儿317例的病例资料,根据IVIG治疗前发热时间分为短热程组(发热时间≤4 d,n=92)和长热程组(发热时间>4 d,n=225),根据是否发生IVIG耐药将每组再分为耐药组和非耐药组。分析比较不同热程耐药组及非耐药组的基线资料及实验室结果,并采用多因素logistic回归分析IVIG耐药的影响因素。结果 短热程组中IVIG耐药19例(20.7%),并发冠状动脉瘤5例(5.4%);长热程组中IVIG耐药22例(9.8%),并发冠状动脉瘤19例(8.4%);短热程组IVIG耐药率明显高于长热程组(P<0.05),而冠状动脉瘤发生率在两组间差异无统计学意义(P>0.05)。短热程组中,耐药患儿治疗前血钠水平低于非耐药患儿,而降钙素原、C反应蛋白及N末端B型利钠肽原水平则明显高于非耐药患儿(P<0.05)。长热程组中,耐药患儿治疗前血钠及肌酸激酶水平低于非耐药患儿(P<0.05)。多因素logistic回归分析显示,血钠水平降低与长热程组KD患儿IVIG耐药有关(P<0.05)。结论 KD患儿IVIG耐药因IVIG治疗前发热时间不同而异。在治疗前发热时间>4 d的KD患儿中,血钠降低与IVIG耐药具有相关性。     

川崎病并冠状动脉损害的危险因素

目的探讨川崎病(KD)并冠状动脉损害(CAL)的危险因素。方法收集1999年1月-2001年12月住院的145例KD患儿的临床资料,分析无CAL93例和CAL52例患儿的实验室检查资料及治疗、随访结果。结果无CAL组发热日程(8.7±3.4)d.血沉(79.5±34.6)mm/1h,CAL组发热日程(11.5±4.8)d,血沉(91.9±36.6)mm/1h,两组比较差异有显著性(P<0.01,0.05)。发病10d内接受静脉注射人血丙种球蛋白(IVIG)治疗患儿CAL发生率为18.8%(18/96),未予IVIG治疗患儿CAL发生率为69.4%(34/49),两者比较差异有显著性(P<0.001)。随访并CAL患儿38例,冠状动脉扩张20例均恢复正常;14例冠状动脉瘤中12例恢复正常,2例缩小;4例巨大冠状动脉瘤2例缩小,另2例超过2年仍未恢复。结论KD并CAL与热程过长及血沉显著增快密切相关,病程早期未予IVIG治疗的患儿并CAL的危险性明显增高,应用IVIG治疗对防治冠状动脉损害有重要作用。     

川崎病并发严重冠状动脉病变患儿旁路移植术术前评估及术后随访

目的回顾性总结川崎病(KD)并发严重冠状动脉病变患儿的影像学诊断、冠状动脉旁路移植术结果及随访情况。方法复旦大学附属儿科医院2006年8月至2008年3月收治5例(男3例,女2例)KD并发严重冠状动脉病变患儿,年龄12个月至10岁4个月。所有患儿均在KD急性期予IVIG治疗,其中3例予2次。病程第15～21天均行超声心动图(ECHO)检查发现冠状动脉病变;之后常规随访ECG和ECHO,并口服阿司匹林和双嘧达莫或氯吡格雷。1例患儿行64排螺旋CT(MSCT)检查,2例行99Tcm-MIBI心肌灌注显像检查。在病程6～65个月行选择性冠状动脉造影检查,并行冠状动脉旁路移植术及冠状动脉成形术。结果 1/5例在病程中有心绞痛发作,3/5例有心功能不全,1/5例无症状。1/5例有心肌梗死发生并在恢复过程有ECG记录;2/5例ECG有ST-T改变;2/5例ECG未见异常。ECHO除均显示多发性冠状动脉瘤(CAA)外,3/5例还显示左心房和左心室增大,左室射血分数(LVEF)和短轴缩短率的降低,2/5例左室壁运动不协调,与99Tcm-MIBI检查结果一致。冠状动脉造影均可见多发性CAA且至少一处为中等或巨大CAA,并伴有血栓形成和远端闭塞。MSCT与冠状动脉造影检查结果一致。年龄最小的1例患儿(手术时22个月)在冠状动脉移植术中死亡,其余4例术后即刻效果满意。在8～24个月的随访中,1例LVEF较低,随访1年未达到正常;1例术后18个月复发KD,治疗顺利,复查ECHO和MSCT未见冠状动脉进一步损害。4例患儿目前仍在随访中。结论 KD并发冠状动脉病变应定期随访ECG、ECHO,必要时进行99Tcm-MIBI和MSCT检查;如考虑手术需行冠状动脉造影仔细评估冠状动脉病变部位和程度。有心肌缺血表现需及时行冠状动脉旁路移植术和冠状动脉成形术,手术的近期效果显著,远期疗效有待长期随访结果。     

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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...     

A Case of Refractory Kawasaki Disease Complicated by Peripheral Ischemia

Kawasaki disease (KD), an acute systemic vasculitis that primarily affects infants and young children, is occasionally refractory to initial high-dose intravenous immunoglobulin (IVIG). Some patients with KD develop peripheral gangrene as a fatal complication, though this is rare. There has been no case reported in the literature of complicated peripheral gangrene that completely resolved without sequelae. Recently, we encountered a patient with KD which was refractory to IVIG, later developing peripheral gangrene. For the treatment of a peripheral gangrene dexamethasone, methotrexate, and an anticoagulant were administered, resulting in resolution of the gangrenous lesions without sequelae. Early diagnosis and treatment can reduce permanent sequelae and mortality from refractory KD complicated by peripheral ischemia.     

激素与静脉丙种球蛋白及其联合应用治疗静脉丙种球蛋白无反应川崎病的回顾性对照研究

目的 探讨激素对静脉丙种球蛋白（IVIG）无反应川崎病（KD）患儿的治疗价值。方法 回顾性收集重庆医科大学附属儿童医院IVIG无反应KD住院患儿,根据再治疗情况分为IVIG组、激素组和IVIG+激素组;将应用激素者根据激素应用途径分为静脉滴注序贯口服激素组和口服激素组。统计各组患儿的总热程、平均热退时间、治疗前后实验室检查结果并计算治疗前后的差值与治疗前值的比值（即差值比△）、急性期及出院后6、12、24个月冠状动脉病变和随访中的血栓形成情况。结果 IVIG无反应KD患儿143例进入本文分析。IVIG组107例,激素组12例, IVIG+激素组24例,3组性别、年龄、体重差异无统计学意义。IVIG+激素组治疗后WBC高于IVIG组,△WBC、△PLT均低于IVIG组,△CRP高于IVIG组,总热程长于IVIG组;急性期冠状动脉瘤发生率及随访至6个月时冠状动脉扩张发生率高于IVIG组,差异均有统计学意义;激素组△WBC低于IVIG组,总热程长于IVIG组,差异均有统计学意义。静脉滴注序贯口服激素组和口服激素组各18例,2组性别、年龄、体重差异无统计学意义,口服激素组再次治疗前和再次治疗后CRP均低于静脉滴注序贯口服激素组, 口服激素组△PLT高于静脉滴注序贯口服激素组,2组随访时点冠状动脉扩张和冠状动脉瘤发生率差异均无统计学意义。随访病例中,静脉滴注序贯口服激素组有2例出现血栓,经积极抗凝治疗后血栓消失。结论 IVIG无反应KD患儿再治疗时应用激素或再次IVIG无反应后应用激素,与单纯IVIG相比急性期治疗效果相近,且均不增加远期冠状动脉损伤的发生率;选择普通剂量口服或者静脉滴注序贯口服疗法临床效果相近,但静脉滴注序贯口服激素较口服激素有更高的血栓形成风险。     

川崎病患儿冠状动脉血流储备测定与影像形态学诊断的对照研究

目的评价持续冠状动脉病变对川崎病（KD）患儿冠状动脉血液动力学的影响, 探讨冠状动脉血流储备（CFR）检测在KD患儿冠状动脉病变远期随访中的应用价值。方法选择因胸痛、心悸以及各类偶发早搏的患儿为对照组，KD并发持续左冠状动脉病变患儿为KD组，均予以ATP 160 μg· kg-1·min-1持续静脉注射，共6 min。运用经胸多普勒超声心动图（TTDE）检测冠状动脉左前降支(LAD) 在ATP注射前后的舒张期峰值流速(PDV)及平均流速(MDV)，并通过计算比值得出相应CFR（PDV）和CFR（MDV）值。结果 对照组纳入25例，CFR（PDV）和CFR（MDV）值均与年龄呈显著正相关(r=0.596, 0.591, P=0.002, 0.002)；<2岁、～8岁和>8岁组的CFR (PDV) 95%参考值范围分别为≥1.22、≥1.94和≥2.22。KD组纳入8例，静息状态下PDV和MDV值分别为（50.0±15.07） cm· s-1和(35.16±11.52) cm· s-1，显著高于对照组(P=0.004, 0.015)；而CFR（PDV）和CFR（MDV）值明显降低，分别为（1.67±0.38） cm· s-1和（1.69±0.47） cm· s-1(P=0.002,0.018)。与对照组比较，KD组7/8例(87.5%)患儿CFR（PDV）值显著降低。结论持续冠状动脉病变可导致KD患儿CFR值显著降低，TTDE检测患儿CFR具无创、安全和便捷的优点，对于KD冠状动脉病变的长期随访与临床诊断治疗策略的选择具有指导意义。     

Long‐term efficacy of plasma exchange treatment for refractory Kawasaki disease

Background: The treatment of Kawasaki disease patients who fail to respond to initial i.v. immunoglobulin (IVIG) therapy is controversial. The aim of the present study was to investigate the long‐term efficacy of plasma exchange (PE) treatment for refractory Kawasaki disease. Methods: A total of 125 Kawasaki disease patients refractory to IVIG were treated with PE. Coronary artery lesions (CAL) before PE, in the acute period, and during the late period were examined retrospectively. Results: Residual sequelae requiring medical treatment occurred in six cases in the late period. The outcomes of treatment tended to be better when PE was begun in the early stage. Sequelae remained in 2.8% of patients in whom PE was initiated prior to day 9 after onset, and were present in 15% of patients in whom PE was started on or after day 10. The 105 patients whose coronary arteries were normal before PE had no sequelae (residual sequelae: 0%). Dilatation was present before PE in 14 patients, but remained in only two patients in the late period (residual sequelae, 14.3%). In four of the six patients in whom aneurysms had already formed before PE, the lesions had advanced into giant aneurysms, but in the other two patients they returned to the normal range (residual sequelae, 66.6%). Conclusions: The outcomes of PE for Kawasaki disease refractory to IVIG are favorable, and the effectiveness of this treatment is excellent, particularly if it is initiated before CAL arise.     

感染因素对川崎病及其冠状动脉病变的影响

目的 探讨感染因素对川崎病(KD)及其冠状动脉病变的影响。 方法 回顾性收集首都医科大学附属北京儿童医院2005年1月至2014年12月出院诊断中包含KD的病历,截取人口学信息、临床发现、急性期和后遗症期超声心动图发现、转归,对每份病历重新行典型KD、不完全KD、IVIG抵抗、冠状动脉病变及其程度和合并感染的甄别。分为KD感染组、KD非感染组分析组间临床发现的差异,分为冠状动脉病变组、非冠状动脉病变组分析感染因素的差异。 结果 2 331例KD患儿进入分析。KD合并感染的发生率为57.1% (1 331/2 331),KD并发冠状动脉病变的发生率为36.0%(840/2 331)。KD感染组与KD非感染组年龄、发热天数及不完全KD比例差异有统计学意义(P均<0.05);冠状动脉病变组与非冠状动脉病变组肺炎支原体感染及消化系统感染伴发腹泻比例差异有统计学意义(P均<0.05)。 结论 合并感染的KD患儿年龄多<1岁,易表现为不完全KD,合并肺炎支原体感染及消化系统感染伴发腹泻的KD患儿易发生冠状动脉病变。     

The Fate and Observed Management of Giant Coronary Artery Aneurysms Secondary to Kawasaki Disease in the Province of Quebec: The Complete Series Since 1976

Most population-based series reporting on the coronary artery complications after Kawasaki disease (KD) originate from Japan. This study aimed to describe the complete series of KD patients from the province of Quebec in Canada, a predominantly Caucasian population. This retrospective case series was conducted by the Quebec Kawasaki Disease Registry, a multi-institutional collaboration reviewing 89.8 % of all KD cases identified by the Ministry of Health records of hospitalization for KD from the first recognized case in 1976 until 2008. This report describes the course of 38 patients (95 % Caucasians) with a diagnosis of giant coronary artery aneurysms, which represent 1.9 % of all reviewed cases and 26.2 % of those with a coronary aneurysm 5 mm or larger. The age at diagnosis was 5.52 ± 4.04 years, and the follow-up period was 9.26 ± 6.9 years. The KD diagnosis was retrospective at autopsy in two cases and via echocardiography in four cases. The overall freedom from coronary thrombi, coronary intervention, or death was respectively 63.9, 67.5, and 85.1 %. Five deaths occurred as follows: 21 days after onset of fever (2 cases), 1.8 months after onset of fever (1 case), 1 year after retrospectively presumed but previously undiagnosed KD (1 case), and 5.7 years after a KD diagnosis (1 case of sudden cardiac death). Percutaneous transluminal coronary revascularization was attempted in four cases (1 requiring cardiac transplantation), and two other cases underwent primary bypass graft surgery. Whereas this study investigated cases of KD with severe coronary sequelae in the Province of Quebec, larger collaborative studies should be conducted for further understanding of the disease in predominantly non-Asian populations.