川崎病合并冠状动脉瘤患儿应用华法林治疗的安全性

目的探讨川崎病合并冠状动脉瘤患儿应用华法林治疗的安全性和转归。方法选择2011至2020年湖南省人民医院收治的川崎病合并巨大冠状动脉瘤、1支冠状动脉血管内发生多个冠状动脉瘤或冠状动脉内发生血栓的患儿共21例进行前瞻性研究,应用华法林治疗,将国际标准化比值(INR)目标范围控制在2.0~3.0。在治疗开始时,治疗后1、2、3、4周及2、3、6、12个月,观察治疗前后冠状动脉瘤直径、数量、部位和冠状动脉内血栓的变化,并监测INR、心电图、肌酸激酶同工酶(CK-MB)、肌钙蛋白I。实施规范的华法林出血风险培训与管理,并根据家长实际落实情况,分为落实组和未落实组,比较2组患儿出血的发生情况。组间比较采用秩和检验或Fisher确切概率法。结果21例患儿男15例、女6例,发病年龄为2月龄至6岁。入组时评估冠状动脉病变为Ⅱ级有4例、Ⅲ级有7例、Ⅳ级有7例、Ⅴ级有3例。10例发生血栓的患儿临床发现血栓的时间为病程第4天至4个月。应用华法林治疗后,其剂量分布在0.06~0.10 mg/(kg·d),INR为1.80~2.59,10例有血栓的患儿有8例消失,2例未消失的Ⅴ级患儿的血栓有不同程度机化。治疗后,4例Ⅱ级病变患儿的冠状动脉均恢复正常;7例Ⅲ级病变患儿中有3例冠状动脉瘤消失,4例无改变;7例Ⅳ级病变患儿中5例冠状动脉瘤缩小为Ⅲ级,2例无改变;3例Ⅴ级病变患儿瘤体无改变。治疗后均没有新发血栓及新冠状动脉瘤出现。21例患儿治疗前后心电图无特殊改变,治疗前后肌钙蛋白I和CK-MB差异均无统计学意义[0.07(0~3.01)比0.04(0~0.29)μg/L,20.6(11.2~58.2)比29.0(16.7~47.0)U/L,Z=0.932、1.906,均P>0.05]。华法林出血风险管理落实组患儿出血发生率明显低于未落实组,差异有统计学意义(2/15比4/6,Fisher值=5.689,P=0.031)。结论应用INR 2.0~3.0为目标范围,结合川崎病并冠状动脉瘤的严重程度来调整华法林剂量,并予以规范而严格的管理与培训,可增加川崎病患儿华法林治疗的安全性,改善患儿的冠状动脉病变,治疗血栓及预防新血栓,降低出血风险。     

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。     

合并冠状动脉瘤的非典型川崎病5例

非典型川崎病（KD）病例近年来有增多趋势。对于合并冠状动脉瘤（CAA）的KD患者延误诊断和治疗可能导致严重的后果。本文报道5例会并冠状动脉瘤的非典型川崎病，以引起临床医师重视。临床资料一、一股资料男3例，女2例，年龄4个月～1.5岁3例，4岁、5岁各1例。临床表现：发热≥5     

川崎病冠状动脉病变的手术治疗

川崎病是一种急性自限性全身血管炎性综合征。该病好发于儿童,主要侵犯全身中小动脉,以冠状动脉最为显著,早期主要表现为冠状动脉扩张、冠状动脉瘤形成,后期随病程进展,形成血栓、内膜增生、钙化,进一步可发展为慢性缺血性心脏病,少数可发生心肌梗死。川崎病已成为发达国家儿童获得性心脏病的首要病因。目前,针对川崎病所致冠状动脉病变的...     

川崎病的冠状动脉损害

川崎病 (ＫＤ)又称皮肤粘膜淋巴结综合征 (ＭＣＬＳ) ,临床上以持续 5ｄ以上的发热、球结膜充血、皮肤粘膜弥漫性潮红、颈部淋巴结肿大、指 (趾 )端的硬性水肿及膜样脱皮等为主要表现 ,主要危及生命的损害是冠状动脉损害 ,如冠状动脉狭窄、血栓、梗死、瘤破裂等 ,可导致猝死。此病由日本的川崎教授在 196 7年首先报道[1] ,1972年草川教授对两名有心肌梗死症状患儿进行了冠状动脉造影 ,首次发现了冠状动脉瘤的改变 ,当时其发生率为 2 4.4% ,近年来由于丙种球蛋白等药物的早期应用 ,其发病率已降低为 13% [2 ] 。一、病因与发病情况病因不详 ,…     

体表面积纠正的冠状动脉内径测量在川崎病随访中的意义

目的：探讨体表面积纠正的冠状动脉内径在川崎病随访中的意义。方法：58例川崎病患者发作期和随访期分别进行超声心动图检查,测量其左右冠状动脉内径,比较体表面积纠正的冠状动脉内径及冠状动脉内径绝对值的变化。结果：按传统标准全组发作期无冠状动脉扩张,与发作期相比随访期平均冠状动脉内径无显著变化。随访期平均体表面积纠正的左右冠状动脉内径显著缩小,(sLCA：4.73±1.40) mm/m2 vs (3.88±1.28) mm/m2,P<0.01;sRCA：(4.51±1.28) mm/m2 vs (3.71±1.10) mm/m2,P<0.01),缩小率与随访时间和发病年龄显著相关。结论：体表面积纠正的冠状动脉内径是反映川崎病发作期后冠状动脉内径动态变化的敏感指标。     

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。     

川崎病冠状动脉病变的治疗现状和进展

川崎病（Kawasaki disease,KD）属于急性血管炎性综合征,冠状动脉病变是其最严重的并发症,发生率达20％-25％。尽管应用静脉注射用丙种球蛋白（IVIG）后冠状动脉瘤的发生率已降至5％以下,但在发达国家,川崎病所致的冠状动脉病变也已经成为小儿最常见的获得性心脏病。冠状动脉瘤,尤其是巨大瘤（直径≥8mm）常不能消退,血管内膜增生及血栓形成,造成冠状动脉管腔狭窄,加之瘤内血液滞留,易形成血栓性栓塞,由此通过冠状动脉的血流明显减少,从而导致心肌梗死或猝死。近年来,随着对川崎病研究的深入,冠状动脉瘤的治疗也取得了一定的进展,在药物治疗、外科及介入技术上均积累了一定的经验。现对川崎病冠状动脉病变的治疗现状及进展进行综述。     

冠状动脉造影追踪观察川崎病引起的巨大冠状动脉瘤

目的 探讨川崎病(KD)导致的巨大冠状动脉瘤(GCAA)的中远期变化特点,为此类儿童进入青少年期后心血管意外事件的防治提供依据.方法 采用冠状动脉造影(CAG)、超声心动图及心电图检查对12例急性期合并GCAA的KD患儿[男10例,女2例;发病年龄1～10(4.05±2.50)岁]进行2～10年追踪观察,分析该组患儿冠状动脉损害的变化特点.结果 本组患儿除2例急性期超声心动图诊断为单发右冠状动脉巨大瘤外,其余10例均为双侧多发冠状动脉瘤.随访CAG发现8例出现右冠状动脉节段性狭窄,其中右冠状动脉闭塞并桥样新生血管形成3例,多节段局域性狭窄4例,右冠状动脉全程编织样损害(血栓性闭塞并簇状新生血管)1例.本组9例冠状动脉瘤经治疗后仍有不同程度的存在,其中4例左前降支瘤远端血流充盈明显减慢.仅1例患儿(1/12) CAG见双侧冠状动脉瘤回缩至轻度扩张,未见狭窄及其他改变.本组12例患儿随访2 ～ 10年,均无自觉症状,活动未受限.静息心电图1例发现病理Q波,其余11例结果正常.静息心电图正常的11例患儿行药物负荷心电图检查仅1例出现多导联S-T段下移及T波低平;与同期CAG比较,超声心动图检出12例患儿冠状动脉近段形态改变,5例发现可疑室壁运动异常,但对观察冠状动脉中、远段狭窄欠敏感;对4例冠状动脉闭塞并新生血管形成的病例,超声观察均未能提示.结论 KD引起的GCAA损害可在急性期后长期存在.表现为GCAA持续存在、回缩、冠状动脉闭塞、局部狭窄及血管再形成等.本病缺乏临床表现,常规心电图及超声心动图对KD后遗症期冠状动脉形态及功能损害检查欠敏感,CAG对本病的追踪观察有重要价值.     

Thrombocytopenia in Kawasaki disease: a risk factor for the development of coronary artery aneurysms

The authors report on a 2-year-old boy with atypical Kawasaki disease (KD) and thrombocytopenia who developed 3 coronary artery aneurysms. The mechanism of thrombocytopenia in KD seems to be consumption coagulopathy. Review of the 30 reported cases of KD and thrombocytopenia revealed that this combination is more common in girls and in young age groups, and is associated with an increased risk of coronary artery aneurysm and myocardial infarction.     

Assessment of coronary artery aneurysms in paediatric patients with Kawasaki disease by multidetector row CT angiography: feasibility and comparison with 2D echocardiography

Background Transthoracic ECHO is the locally accepted method for coronary surveillance of patients with Kawasaki disease but it may have limited visualization in the older child. Objective To assess the feasibility of multidetector CT (MDCT) angiography in the follow-up of coronary artery aneurysms in children with previous Kawasaki disease. Materials and methods Six children (5 boys, 1 girl; mean age 11.5 years) with known Kawasaki disease and coronary artery involvement underwent CT coronary angiography using 16-detector MDCT. The visualized lengths and diameter of all coronary segments were measured. The number, size and location of coronary artery aneurysms were recorded and compared with recent ECHO. Results Twelve coronary artery aneurysms (seven saccular, five fusiform) were identified by MDCT angiography. One saccular aneurysm at the junction of the distal right coronary artery and posterior descending artery was not detected by ECHO while the remaining six in proximal segments were detected by both modalities. Two of five fusiform aneurysms were not detected by ECHO due to their small sizes. Excellent agreement was found between CT and ECHO for maximal diameter and length of the visualized aneurysms. Conclusions MDCT angiography accurately defines coronary artery aneurysms. It is more sensitive for detecting aneurysms at distal coronary segments and fusiform aneurysms of small size. Presented at the 5th International Pediatric Radiology Conjoint Meeting, Montreal, Canada, May 2006.     

A case of Kawasaki disease with coronary artery aneurysms documenting Yersinia pseudotuberculosis infection

A case of a 5-year-old boy who fulfilled all the criteria for Kawasaki disease (KD) was described. He had associated bilateral coronary artery aneurysms. Our study revealed the isolation of Yersinia pseudotuberculosis in stool cultures, and the elevation and seroconversion of the agglutination antibody titres, and hence he was diagnosed as Y. pseudotuberculosis infection-positive. We also demonstrated the positive mitogenic activity of the culture supernatant of the isolated bacterium from the patient and detected Y. pseudotuberculosis-derived mitogen by PCR. This case therefore suggests that Y. pseudotuberculosis might be closely related to the cause of KD.     

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。     

A sibship with recurrent Kawasaki disease and coronary artery lesion

Although epidemiologic studies of Kawasaki disease suggest an infectious etiology, the cause of this mysterious disease remains unclear. We describe the occurrence of five episodes of Kawasaki disease over a six-year period in three siblings. Two of the three children experienced recurrent Kawasaki disease and developed coronary artery lesions, which included giant coronary artery aneurysms in the youngest child. The non-contemporaneous occurrence of the disease in these three children emphasizes the importance of a genetic basis and/or environmental factors in the etiology of Kawasaki disease.     

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.     

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目的探讨川崎病(KD)患儿合并冠状动脉损伤的危险因素。方法按照KD诊断标准,对北京儿童医院2000-01-01—2004-12-31收治的644例KD患儿临床资料、治疗方法以及实验室资料进行分析。结果Pear-son卡方检验显示,患儿性别、发热持续时间、丙种球蛋白使用时间、丙种球蛋白使用剂量、血沉及C反应蛋白与KD合并冠状动脉损伤有关(P<0·05);进一步多因素Logistic回归分析显示,性别、发热持续时间、丙种球蛋白使用剂量与冠状动脉扩张显著独立相关(P<0·05)。结论对男性、发热持续时间长的KD患儿应予足够重视,早期足量使用丙种球蛋白以减少或减轻冠状动脉并发症。     

Effect of dipyridamole on the blood flow in coronary aneurysms resulting from Kawasaki disease

Summary Dipyridamole has been widely used in Japan to treat patients with a coronary aneurysm resulting from Kawasaki disease, but its effect in these patients has not been established. In the present study we assessed the effect of dipyridamole on the coronary arteries of patients with a history of Kawasaki disease by measuring the diameter of the coronary arteries and by quantifying the disappearance time of contrast medium (runoff time) on coronary angiography. Intravenous injection of dipyridamole increased the diameter of nondilated arteries by 7.9%. Its effect on the diameter of dilated coronary arteries (coronary aneurysm) was less than 3% (p<0.01). Runoff time of dilated coronary arteries was significantly (p<0.01) greater than that of nondilated coronary arteries. Dipyridamole accelerated runoff time not only in nondilated coronary arteries (p<0.01) but also in coronary arteries with various degrees of dilatation (p<0.01). We conclude that dipyridamole increases blood flow in coronary arteries without dilating the proximal aneurysm in children with a history of Kawasaki disease.