川崎病并发巨大冠状动脉瘤的远期预后研究

目的探讨川崎病(KD)并发GCAA患儿的远期预后,为进一步探索KD引起心血管损害后期的诊断治疗提供依据。方法选取2001年5月至2006年5月在广州市妇女儿童医疗中心儿童医院诊断为KD并发冠状动脉损害的患儿为研究对象。依据冠状动脉损害严重程度分为冠状动脉扩张(CAD)组、中小冠状动脉瘤(CAA)组和巨大冠状动脉瘤(GCAA)组。随访起止时间为急性期出院后至2009年5月。所有纳入随访的患儿均于急性期出院后6个月、1年、2年和3年各随访超声心动图1次,观察冠状动脉形态;对急性期3个月后常规超声心动图提示冠状动脉形态未恢复患儿行ATP负荷超声心动图及冠状动脉造影检查,观察心脏室壁运动、左右冠状动脉血流储备和冠状动脉分支病变情况。死亡患儿行尸解及冠状动脉病理学检查。结果CAD组纳入166例,中小CAA组纳入59例,GCAA组纳入15例。①随访3年时超声心动图显示GCAA组2/15例(13.3%)恢复正常,显著低于CAD组和中小CAA组(P均0.001);GCAA组1例(6.7%)显示冠状动脉扩张;10例(66.7%)表现为CAA,其中3例为GCAA,3例合并冠状动脉狭窄或闭塞。②ATP负荷超声心动图显示,GCAA组室壁运动异常的发生率显著高于CAD组(6/9例vs8/55例,P0.001),与CAA组差异无统计学意义(6/9例vs17/31例,P0.05);非参数检验提示,3组间室壁运动异常发生率有统计学差异,以GCAA组发生率较高(Z=-4.2,P0.001)。③GCAA组6/15例行冠状动脉造影,4/6例仍存在CAA,1/6例GCAA退缩为轻度CAD。5/6例出现冠状动脉狭窄或(和)闭塞,其中1例闭塞后再通,1例合并侧支血管形成。④GCAA组死亡2例,尸解冠状动脉大体可见,左右冠状动脉各分支呈串珠样膨大,瘤样扩张的冠状动脉管腔内可见充满暗红色血栓样物质,管壁显著增厚。冠状动脉分支和前降支病理学检查均显示:内膜增厚,炎症细胞浸润。结论KD并发GCAA患儿的预后较差,冠状动脉损害可持续存在;后期可发生冠状动脉狭窄或闭塞,导致缺血性心脏病;常规超声心动图与ATP负荷超声心动图、冠状动脉造影结合应用,对KD并发GCAA的远期随访有重要意义。     

川崎病冠状动脉病变远期随访评估:二维超声与冠状动脉造影对照观察

目的探讨体表十二导联心电图(ECG)、二维经胸超声心动图(2DE)、64排螺旋CT冠脉成像技术(MDCT)和选择性冠状动脉造影(CAG)在川崎病(KD)严重冠状动脉病变远期随访中的应用价值。方法回顾性分析2006年7月至2010年9月于复旦大学附属儿科医院随访的15例KD并发严重冠状动脉病变(美国冠状动脉分级Ⅲ～Ⅴ级)患儿的临床特征、治疗方案以及ECG、2DE、MDCT和CAG检查结果。结果 15例KD患儿进入分析,KD发病年龄为5个月至13.5岁,平均(4.8±1.1)岁;病程3～64个月,平均(17.4±13.0)个月。2例主诉反复心绞痛发作,2例表现为充血性心力衰竭,其余患儿无明显临床症状。ECG检查发现异常Q波及ST-T波改变3例;MDCT与CAG检查结果显示,在单支、两支和三支冠状动脉中发生巨大瘤样病变的患儿分别为5例、5例和3例;6例合并远端冠状动脉瘤;2例可见冠状动脉瘤内血栓形成,但未形成狭窄;4例存在冠状动脉严重梗阻性病变,其中右冠状动脉合并左回旋支完全闭塞1例,右冠状动脉合并左前降支严重狭窄2例,右回旋支完全闭塞1例。冠状动脉病变Ⅲ级1例,Ⅳ级10例,Ⅴ级4例。与MDCT、CAG结果对照,2DE诊断完全符合率为46.7%(7/15例),其中遗漏诊断远端冠状动脉瘤6例,冠状动脉严重狭窄和(或)闭塞4例,冠状动脉内附壁血栓1例。结论对于2DE诊断为严重冠状动脉病变的KD患儿,应予以进一步MDCT或CAG检查以了解冠状动脉病变全貌,为治疗策略的制定提供更多信息。     

川崎病并发冠状动脉病变的超声心动图表现的动态观察

目的 探讨川崎病(KD)所引起的冠状动脉扩张(CAD)以及冠状动脉瘤(CAA)的超声心动图特点.方法 用彩色多普勒超声心动图检测KD患儿冠状动脉开口和内径,并动态观察静脉免疫球蛋白治疗前后冠状动脉内径的变化情况.结果 本组46例KD患儿,41例患儿行超声心动图检查,合并CAD 12例,左冠状动脉(LCA)较右冠状动脉(RCA)更易受累及(P<0.05);CAA 4例,均为双侧冠状动脉病变,最大内径10 mm.治疗后6～18 d复查超声心动图,CAD组LCA较治疗前明显回缩(P<0.05),RCA内径无变化(P>0.05);CAA组LCA和RCA内径均无明显变化(P>0.05).对10例冠状动脉病变患儿进行随访,其中8例CAD冠状动脉内径均恢复正常,1例CAA冠状动脉病变加重,1例CAA完全恢复正常.结论 (1)在KD所引起的CAD中,LCA比RCA更易受累及,静脉免疫球蛋白治疗后短期内LCA明显回缩.(2)超声心动图是动态观察KD并发CAD和CAA的无创手段.     

多层螺旋计算机断层照像术在评价川崎病冠状动脉损伤中的意义

目的探讨多层螺旋CT(MSCT)在诊断川崎病(KD)并冠状动脉损伤(CALs)中的作用。方法选择门诊随访的KD并CALs患儿15例,采用MSCT和二维超声心动图(TDE)检查冠状动脉,并分析二者结果。结果MSCT诊断CALs阳性率与TDE比较有显著差异(P<0.05)。MSCT和TDE呈显著正相关性(r=0.975P<0.01)。结论MSCT可清晰显示冠状动脉近端及远端病变情况,精确测量和了解冠状动脉扩张程度、钙化部位,对评价KD患儿并CALs治疗疗效的随访有主要意义。     

冠状动脉造影在川崎病冠状动脉损害检查中的作用

目的　通过对疑有冠状动脉损害川崎病 (KD) 1 2例患儿行经皮冠状动脉造影 ,旨在准确了解冠状动脉损害情况 ,以指导临床对KD患儿的后期治疗及管理。方法　在静脉麻醉下采用Sedinger法穿刺右股动脉 ,采用Judkin法将冠状动脉造影管送达冠状动脉口 ,推注非离子造影剂 ,同时行电影摄影记录。结果　接受冠状动脉造影检查 1 2例患儿中 9例冠状动脉异常 ,其中左冠状动脉和 (或 )其分支受累 5例 ,右冠状动受累、左右冠状动脉同时受累各 2例。与超声心动图结果不尽一致。结论　冠状动脉造影可明确显示冠状动脉损害位置、形状、数目及病变程度 ,是一种安全、有效的检查方法。     

放射性核素心肌灌注显像评价川崎病恢复期心肌缺血的意义

目的探讨放射性核素锝甲氧基异丁基异腈(99Tcm-MIBI)心肌灌注显像评价川崎病(KD)恢复期心肌缺血的意义。方法对21例KD恢复期患儿行超声心动图检查。根据超声检查结果分为冠状动脉扩张和无冠状动脉扩张组。各组均行双嘧达莫负荷心肌灌注显像,显像阳性病例隔日再次接受静息心肌显像。结果21例KD恢复期中9例阳性,阳性率为42.8%。超声心动图发现4例冠状动脉扩张,单光子发射计算断层显像仪(SPECT)显像均显示心肌缺血。17例无冠脉扩张患者SPECT显像5例缺血,阳性率29.4%。结论99Tcm-MIBI负荷心肌灌注显像较超声心动图更能客观评价心肌缺血部位、范围和程度,可作为KD随访过程中判断心肌缺血的常规检查方法。     

川崎病合并冠状动脉病变患儿117例冠状动脉造影结果分析

目的分析川崎病(KD)患儿冠状动脉造影(CAG)资料中冠状动脉病变(CAL)的特点, 探讨CAG在KD合并CAL患儿诊治中的重要性。方法回顾性分析2013年1月至2022年8月于上海交通大学医学院附属上海儿童医学中心行CAG检查的KD合并CAL患儿的临床资料、心电图、心脏超声及CAG检查时间及结果, 分析CAL病变的分布、病变类型、严重程度及预后。结果共117例KD合并CAL患儿被纳入分析, 患儿KD发病年龄为2个月至12.8岁, 造影年龄为8个月至18.1岁。共234支冠状动脉发生病变, 其中, 右冠状动脉(RCA)96支(41.1%), 左冠状动脉前降支(LAD)78支(33.3%), 左冠状动脉主干44支(18.8%), 左回旋支16支(6.8%)。单侧冠状动脉受累43例患儿, 占36.8%, 以LAD受累最显著;双侧冠状动脉受累74例, 占63.2%, 以LAD与RCA同时受累占比最多。从冠状动脉受累的严重程度来看, RCA与LAD大型冠状动脉瘤与严重冠状动脉狭窄的发生率最高;管腔内病变在小型、中型、大型冠状动脉瘤、狭窄或闭塞部位分别检出10例(13.6%)、20例(24.3%...     

川崎病冠状动脉病变的远期预后观察

目的观察川崎病(KD)伴冠状动脉病变(CAD)的远期恢复情况.方法对262例确诊KD住院患儿,于急期或亚急期行心电图及超声心动图检查,并于病程不同时期(6个月至10年)进行复查.结果急期和亚急期行心电图检查262例异常74例.随访中低电压全部恢复,3例重度冠状动脉瘤患儿心肌缺血持续存在.超声心动图检查发现CAD50例,随访6个月至10年,33例恢复,6例未愈,11例失访.无CAD的KD患儿随访结果正常.结论CAD的KD患儿约60%在10年内痊愈,阿斯匹林需持续用至痊愈.     

川崎病所致严重冠状动脉病变单中心队列研究

背景：川崎病（KD）所致严重冠状动脉病变（CAL）容易导致患儿发生冠状动脉事件或死亡,对远期预后及生存质量具有重要影响。 目的：总结分析伴严重CAL的KD患儿的中远期临床转归,为KD患儿长期随访管理及预后评估提供依据。 设计：队列研究。 方法：检索复旦大学附属儿科医院（我院）HIS系统,纳入2008年1月至2019年12月出院诊断为KD,经我院冠状动脉造影（CAG）确诊为KD所致严重CAL的＜18岁患儿。排除合并其他引起严重CAL和影响严重CAL预后疾病者。所有患儿在门诊及病房由资深心血管专科医生随访,均以药物治疗为基础,伴冠状动脉严重狭窄及心肌缺血的患儿经多学科讨论后予手术治疗。以确诊及随访期间出现心肌缺血、心肌梗死或死亡的患儿为预后不良组,反之则为预后良好组,观察出现不良预后及CAL的演变情况,分析不良预后的影响因素。 主要结局指标：不良预后发生情况和CAL的恢复情况。 结果：144例KD所致严重CAL患儿纳入本文分析。其中男117例（81.2%）,女27例;KD中位发病年龄2.2（1.0,4.5）岁,中位随访时间1.6（0,3.4）年,确诊为严重CAL时病程为5.0（3.0,21.3）月。144例KD所致严重CAL患儿共累及276支冠状动脉,56例（38.9%）累及单支冠状动脉,88例（61.1%）累及≥2支冠状动脉。发生巨大冠状动脉瘤（GCAA)122例,单支冠状动脉多发中小型冠状动脉瘤（CAA)8例,GCAA合并单支冠状动脉多发中小型CAA 6例,受累冠状动脉均狭窄8例;62例发生冠状动脉狭窄,其中冠状动脉闭塞31例。预后不良组54例（37.5%）,均存在心肌缺血,其中14例患儿缺血心肌处发生过有记录的急性心肌梗死,1例患儿因心肌梗死所致严重心力衰竭死亡。多因素分析结果显示,≥2支冠状动脉受累的患儿是单支冠状动脉受累的患儿发生不良预后风险的2.6倍（OR=2.602,95%CI：1.201~5.634）,合并冠状动脉狭窄的患儿是未合并冠状动脉狭窄的患儿发生不良预后风险的3倍（OR=3.055,95%CI：1.450~6.437）。共行179次CAG,114例（208支受累冠状动脉）行1次CAG,30例（68支受累冠状动脉）行≥2次CAG。68支受累冠状动脉首次确诊时,51支为GCAA,末次随访时好转率为4.0%;3支为多发中小型CAA,末次随访时好转率为66.6%;2支为单个中小型CAA,至末次随访均发生狭窄（狭窄程度约50%）;12支为狭窄,至末次随访时均未好转。 结论：GCAA是KD所致严重CAL的主要类型,且难以恢复,单支冠状动脉多发中小型CAA好转率较高。冠状动脉狭窄及多支冠状动脉受累是KD所致严重CAL患儿发生不良预后的影响因素。     

川崎病并发冠状动脉损害19例临床分析

目的分析川崎病(KD)并发冠状动脉损害(CAL)患儿的临床特点,提高对本病的认识。方法对中国医科大学附属第一医院儿科2008年9月至2015年8月收治的19例诊断为KD并发CAL患儿的临床资料进行回顾性分析。结果 KD患儿并发CAL男性多于女性,男∶女为3.75∶1,其中冠状动脉瘤(CAA)患儿男性明显多于女性,男∶女为8∶1;小于1岁患儿多发,占36.8%。患儿CAL的发生率为16.4%;冠状动脉扩张(CAD)10例,CAA5例,巨大冠状动脉瘤(GCAA)4例,其发生率分别为8.6%、4.3%、3.5%;73.7%的患儿为病后2~3周发生CAL;按累及分支数计,累及双支冠状动脉占68.4%;按累及部位计,右冠状动脉受累占41.5%,左主干受累占39.0%,左前降支受累占17.1%,左回旋支受累占2.4%。患儿CAL的严重程度临床分级为:Ⅱ级病变5例,占26.3%;Ⅲ级病变8例,占42.1%;IV级病变6例,占31.6%。随访发现71.4%的患儿CAA或扩张呈明显回缩,回缩时间平均(1.5±0.7)年,其中1例CAA完全消退。结论 KD患儿并发CAL的发生率为16.4%,其中CAD比例最高,GCAA比例最低;CAL多发生在病后2~3周,各分支均可受损,最易累及右冠状动脉,CAL严重程度Ⅲ级的比例最高,多数CAA或扩张在1~2年内呈明显回缩。     

川崎病患儿外周血基质金属蛋白酶-9的表达及其与冠状动脉损伤的关系(英文)

目的　观察MMP 9在川崎病 (KD)患儿不同时期表达水平 ,探讨其在冠状动脉损伤中的可能作用。方法　选择KD患儿 2 7例为研究对象 (无冠脉损伤组 10例 ,冠脉损伤组 17例 ) ,取年龄相仿的 10例败血症患儿、10例健康儿童为发热对照组和正常对照组。分别应用明胶酶谱法、酶联免疫吸附法检测血清MMP 9的活性和蛋白浓度 ,半定量RT PCR的方法检测外周血白细胞MMP 9mRNA表达水平。结果　 (1)冠脉损伤组急性期血清MMP 9活性和蛋白水平 (10 .2± 2 .2 ,114 6± 6 91ng/ml)较无冠脉损伤组显著增高 (6 .2± 2 .1,4 5 7± 133ng/ml,P <0 .0 5 )。两组均较正常对照组 (0 .1± 0 .0 ,72± 2 4ng/ml)和发热对照组 (3.1± 1.4 ,2 2 1± 15 4ng/ml)显著增高 (P <0 .0 1或P <0 .0 5 )。 (2 )KD患儿急性期血清MMP 9蛋白水平与外周血白细胞计数显著正相关 (r =0 .4 80 ,P <0 .0 5 )。 (3)两组KD患儿急性期外周血白细胞MMP 9mRNA表达水平无显著性差异 ,但均显著高于发热对照组和正常对照组 (P <0 .0 1)。 (4)川崎病患儿急性期血清MMP 9活性、蛋白浓度和白细胞表达MMP 9mRNA水平 ,在亚急性期、恢复期均依次明显降低 (P <0 .0 1)。结论　MMP 9在川崎病患儿急性期 ,尤其在伴冠状动脉损伤时表达明显升高 ;MMP 9可能参与了川崎病冠状     

Kawasaki disease with severe cardiac sequelae: lessons from recent New Zealand experience

OBJECTIVES: To review recent cases of Kawasaki disease (KD) with significant cardiac sequelae in New Zealand. It is known that intravenous immunoglobulin (IVIG) reduces the risk of coronary artery aneurysm formation if given within 8-10 days of onset of KD. METHODS: Retrospective review of medical course, criteria for KD, laboratory and cardiac findings for six children identified with KD and significant coronary artery sequelae. RESULTS: There was delay in diagnosis of KD in three of the six children. Three cases were atypical by extremes of age (2 months, 10 years, 14 years). By definition all six children had significant coronary artery involvement. One patient had a thrombus detected in a coronary aneurysm 3 weeks after KD. One patient underwent coronary artery bypass grafting for unstable angina 2 years after KD. One patient developed coronary artery aneurysms after an initial 'toxic shock' type illness evolving to KD. Three patients died, one due to rupture of a coronary artery aneurysm, two from rapid early coronary artery obstruction occurring at three and 4 months after initial KD. CONCLUSIONS: Kawasaki disease remains an important cause of mortality and morbidity for children. Diagnostic delay beyond 8 days reduces the chances of successful IVIG therapy in KD. Current studies supported by the Paediatric Surveillance Unit should establish the epidemiology of KD in New Zealand.     

Soluble forms of the selectin family in children with Kawasaki disease: prediction for coronary artery lesions

AIM: To investigate the relationship between the plasma levels of soluble forms of the selectin family and the incidence of coronary artery lesions (CALs) in patients with Kawasaki disease (KD). METHODS: Thirty-three patients with KD, including group A patients (n = 22) who had no CALs and group B patients (n = 11) who had CALs, as well as age-matched febrile (n = 10) and afebrile controls (n = 11), were studied. RESULTS: Peak plasma E-selectin levels (172.0 +/- 58.6 ng ml(-1)) occurred during the acute phase of KD, while peak plasma P-selectin levels (260.3 +/- 43.2 ng ml(-1)) occurred during the subacute phase of the illness (p<0.05). Plasma L-selectin levels (1757.3 +/- 244.3 ng ml(-1)) during the convalescent phase tended to be higher than in either the acute or the subacute phase (not significant). Before intravenous immunoglobulin treatment, the plasma levels of E- (225.1 +/- 46.8 ng ml(-1)) and P-selectin (259.4 +/- 76.2 ng ml(-1)) of patients with CALs (n = 11) were significantly higher than those of patients (n = 22) with no CALs (E-selectin, 131.6 +/- 36.9 ng ml(-1); P-selectin, 184.9 +/- 84.6 ng ml(-1); p < 0.05). When a plasma E-selectin value before immunoglobulin treatment of >184.7 ng ml(-1) was used as the cut-off point, the sensitivity and specificity for the incidence of CALs were 81.8% and 90.9%, respectively. These findings demonstrate the relationship between plasma levels of selectins and disease severity of Kawasaki vasculitis. CONCLUSION: Higher plasma levels of E-selectin may have potential as a predictor of the incidence of coronary artery lesions in Kawasaki disease patients.     

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.     

State-of-the-art acute phase management of Kawasaki disease after 2017 scientific statement from the American Heart Association

Kawasaki disease (KD) has become the most common form of pediatric systemic vasculitis. Although patients with KD received intravenous immunoglobulin (IVIG) therapy, coronary arterial lesions (CALs) still occurred in 5%–10% of these patients during the acute stage. CALs may persist and even progress to stenosis or obstruction. Therefore, CALs following KD are currently the leading cause of acquired heart diseases in children. The etiology of CALs remains unknown despite more than four decades of research. Two unsolved problems are IVIG unresponsiveness and the diagnosis of incomplete KD. The two subgroups of KD patients with these problems have a high risk of CAL. In April 2017, the American Heart Association (AHA) updated the guidelines for the diagnosis, treatment, and long-term management of KD. Compared with the previous KD guidelines published in 2004, the new guidelines provide solutions to the aforementioned two problems and emphasize risk stratification by using coronary artery Z score systems, as well as coronary severity–based management and long-term follow-up. Therefore, in this study, we merged the AHA Scientific Statement in 2017 with recent findings for Taiwanese KD patients to provide potential future care directions for Taiwanese patients with KD.     

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.     

Successful pregnancy after coronary artery bypass grafting for Kawasaki disease

Abstract A 13-year-old girl with a history of Kawasaki disease underwent coronary artery bypass grafting because of angina pectoris due to a giant coronary artery aneurysm on the left main trunk artery. Nine years after the operation, the patient had an uneventful pregnancy followed by a normal vaginal delivery. This is the first case of a successful pregnancy after coronary artery bypass grafting for Kawasaki coronary artery disease.     

Competition Between Native Flow and Graft Flow After Coronary Artery Bypass Grafting. Impact on Indications for Coronary Artery Bypass Grafting for Localized Stenosis with Giant Aneurysms Due to Kawasaki Disease

We report the postoperative course of native and graft flow after coronary artery bypass grafting (CABG) in two patients with giant aneurysms and localized stenosis due to Kawasaki disease (KD). Although both patients had undergone CABG to the left anterior descending artery (LAD) with the left internal thoracic artery (ITA), at 5 and 10 years old, respectively, the ITA grafts were occluded 1 month postsurgery. However, when the two patients suffered complete occlusion of the native LAD more than 10 years after surgery, angiograms showed that the ITA grafts had reopened. We believe that this postoperative course reflects competition between the native artery flow and graft flow after CABG. CABG in patients with severely delayed coronary flows or recurrence of thrombus in giant aneurysms was ineffective in preventing myocardial infarction or damage. We conclude that CABG in giant aneurysm without significant localized stenosis should be avoided.