川崎病患儿血浆内皮素检测与冠状动脉病变

目的　了解川崎病 (KD)患儿血浆内皮素 (ET)在急性期和亚急性期变化 ,及其与KD及并冠状动脉病变的关系。方法　检测 5 0例KD患儿急性期和亚急性期血浆ET含量 ,并以 30例急性感染及 30例健康儿童作对照组。结果　 1.KD组亚急性期血浆ET较急性期显著增高 ;2 .KD组与健康对照组比较 ,急性期和亚急性期血浆ET均显著增高 ;与感染对照组相比 ,仅亚急性期显著增高 ;3.彩超探及KD组 5 4 %患儿冠状动脉扩张(CAD) ,其亚急性期血浆ET较无CAD组显著增高。结论　KD患儿亚急性期ET进一步增高 ,对发生冠状动脉及其他心血管并发症有一定提示 ,同时应作相关检查     

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.     

川崎病患儿冠状动脉病变的随访及超声与造影的对照研究

川崎病是一种病因未明的婴幼儿高发的血管炎综合征 ,主要影响中小型动脉 ,特别是冠状动脉。多数可自然康复。但部分患儿可在急性期或以后产生冠状动脉瘤和多发性狭窄 ,导致心肌缺血、心肌梗塞等。目前国内许多医疗机构主要应用二维超声心动图 (2 ＤＥ)评价急性期的冠状动脉病变并作长期随访 ,而不进行选择性冠状动脉造影 (ＣＡＧ)。本研究主要探讨川崎病患儿冠状动脉病变及其转归 ,以及 2 ＤＥ和ＣＡＧ在冠状动脉病变判断和长期随访中的作用。对象及方法在 1979年～ 1997年 18年间因为川崎病在日本自治医科大学治疗和随访中发现有冠状动脉…     

川崎病冠状动脉病变与内皮功能障碍

<正> 川崎病(Kawasaki disease,KD)由日本川崎富作医师于1961年首次发现,随后在许多国家都有病例报导。起初认为本病是一种自限性的出疹性热病,随着临床研究的深入,许多研究者认为:KD是一种微生物毒素以超抗原介导机制所引起的免疫性血管炎综合征,幼儿高发。KD的主要危险是其心脏并发症。KD目前已取代风湿热,成为儿童后天性心脏病的主要病因。     

川崎病急性期血钠、钾、磷水平的变化

川崎病 (KD)是一种以免疫系统活化和内皮细胞广泛损害为主要特征的全身性血管炎 ,可引起多器官损害和代谢紊乱。为了解KD对血清钠、钾、磷水平的影响 ,我院监测了50例KD急性期患儿血清钠、钾、磷的变化 ,报告如下。对象与方法一、对象我院1998年1月至2001年1月收治KD患儿50例 ,男31例 ,女19例 ,其中45例根据是否有冠状动脉病变分为冠状动脉损害组 (CA组 )和非冠状动脉损害组 (NCA组 )。CA组10例 ,男6例 ,女4例 ,平均年龄2.7岁(5月至5.5岁) ;NCA组35例 ,男20例 ,女15例 ,平均年龄2.5岁 (4月至5岁 )。随机选择性别、年龄相当的健康体…     

川崎病冠状动脉病变的影像学诊断

川崎病的主要并发症与心血管,特别是冠状动脉系统有关.在未经治疗的川崎病病例中冠状动脉瘤发生率占20％～30％,在经过IVIG及阿司匹林治疗的病例中约占5％.超声心动图已成为川崎病急性期及恢复期诊断及随访评估冠状动脉扩张等心血管并发症的标准诊断技术.急性期冠状动脉病变的超声心动图表现有冠状动脉扩张、冠状动脉瘤及管壁周围回声增强.冠状动脉内径测值与正常值比较对川崎病的诊断与治疗起关键作用.冠状动脉内径的正常范围受年龄、体表面积的影响,并存在地区人群间差异.超声心动图诊断远端冠状动脉病变、冠状动脉狭窄或闭塞病变的敏感性不够高.近年来磁共振血管造影及CT技术迅速发展成为重要的心血管诊断方法.多层CT对川崎病冠状动脉病变诊断效果优于超声心动图及磁共振血管造影.多层CT显像受心率及呼吸影响.选择性冠状动脉造影仍然是显示冠状动脉病变的金标准,但有一定的风险.     

川崎病冠状动脉病变介入疗法的研究现状

川崎病 (Ｋａｗａｓａｋｉｄｉｓｅａｓｅ,ＫＤ)是一种病因不明 ,以全身中、小动脉炎性病变为主要病理改变的急性发热性疾病 ,常见于 5岁以下的小儿 ,以冠状动脉 (简称冠脉 )病变为其最严重的并发症。ＫＤ急性期冠脉瘤 (ｃｏｒｏｎａｒｙａｒｔｅｒｙａｎｅｕｒｙｓｍ ,ＣＡＡ)的发生率约为 10 %～ 2 0 %。随访研究表明 ,约 5 0 %的ＣＡＡ于病后 1～ 2年内自行消退 ,80 %的轻、中度ＣＡＡ于病后5年内消退。ＣＡＡ直径 >5ｍｍ者可能进展为狭窄 ,且瘤体越长可能性越大。巨大ＣＡＡ(直径≥ 8ｍｍ)常不能消退 ,内膜增生伴血栓形成 ,瘤出入口产生…     

川崎病患儿血清 TNF-α、IL-6 的变化及其与冠状动脉病变之间关系的探讨

为探讨肿瘤坏死因子α（ＴＮＦ－α）和白细胞介素６（ＩＬ－６）在川崎病（ＫＤ）发病中的作用以及其与ＫＤ冠状动脉病变（ＣＡＤ）之间的关系。采用ＥＬＩＳＡ测定ＫＤ患儿血清ＴＮＦ－α和ＩＬ－６水平,与正常对照组进行比较,并将ＫＤ并ＣＡＤ组与无ＣＡＤ组进行比较,结果ＫＤ患儿血清ＴＮＦ－α和ＩＬ－６皆明显高于正常对照组（Ｐ〈０．０１）,ＴＮＦ－α在ＫＤ并ＣＡＤ组患儿亦高于无ＣＡＤ组患儿（Ｐ〈０．０５）,而ＩＬ－６在两组中无显著差别（Ｐ〉０．０５）,提示ＴＮＦ－α和ＩＬ－６皆参与了ＫＤ的发生,而ＴＮＦ－α在ＫＤ患儿ＣＡＤ中,可能起到比ＩＬ－６更为重要的作用。     

Fatal Kawasaki disease with incomplete criteria: Correlation between optical coherence tomography and pathology

Coronary artery aneurysm is a serious complication of Kawasaki disease (KD). A 3‐month‐old infant presented with severe KD 27 days after onset of fever. The patient presented with shock, inferolateral ischemia on electrocardiogram and high troponin. Echocardiography showed severe myocardial dysfunction with diffuse coronary dilation and right coronary artery aneurysm. Arterial Doppler demonstrated thrombosis of aneurysmal axillary and iliac arteries. Withdrawal of support was implemented due to multi‐organ failure. Post‐mortem optical coherence tomography correlated with pathology. The pulmonary artery was normal on OCT and histology. Coronary arteries showed aneurysmal dilatation, with intimal hyperplasia and preserved media on OCT. Pathology confirmed these findings, with destruction of the internal elastic lamina, luminal myofibroblastic proliferation, neovascularization, and partial disappearance of the media. This is the first report of pathologic correlation in KD with OCT at the subacute stage, which adequately identified structural wall changes.     

Prevalence of coronary artery abnormality in incomplete Kawasaki disease

BACKGROUND: The aim of the present study was to determine the prevalence of coronary artery abnormality (CAA) and other clinical features in patients with incomplete Kawasaki disease (iKD) using the data from the 17th Japanese nationwide survey of KD. METHODS: iKD was defined as the presence of four or fewer of the principal symptoms of the Japanese diagnostic guidelines, regardless of whether the patient had CAA. A total of 15,857 cases were analyzed. RESULTS: Among 15,857 cases, 83.9% of patients had five to six principal symptoms (complete KD: cKD), and 16.1% had iKD. The prevalence of CAA in cKD was 14.2%, and 18.4% in iKD. The prevalence of CAA in patients with four principal symptoms was 18.1%, which was higher than in cKD cases (14.2%). Although the reliability of the data has some limitations, the prevalence of CAA among patients with one to three symptoms was 19.3%. Among all CAA patients, 14% had four symptoms, and 6% had only one to three symptoms. CONCLUSION: Incomplete KD should not be equated with mild KD. Patients with four principal symptoms were comparable to cKD with respect to CAA occurrence. In patients with one to three symptoms also, especially in those under 1 year and older than 4 years of age, other significant symptoms and laboratory findings of the guidelines are very important in making a correct and early diagnosis of iKD so as to prevent CAA.     

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.     

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??Objective To analyze the clinical characteristics of patients with CAL complicated by KD??and to improve the understanding of this disease. Methods Clinical data of 19 cases diagnosed as KD complicated by CAL from September 2008 to August 2015 in the Pediatric Department of First Hospital of China Medical University were retrospectively analyzed. Results KD children complicated with CAL was more in male than in female??male??female was 3.75??1??children with coronary aneurysm were more in male than in female??male??female was 8??1??it was more often in less than 1-year old children??accounting for 36.8%. The incidence rate of coronary artery lesions in this group was 16.4%??CAD was in 10 cases??CAA in 5 cases??GCAA in 4 cases??the incidence rate being 8.6%??4.3% and 3.5%??respectively??in terms of CAL time 73.7% was 2-3 weeks after the disease?? according to the number of branches involved??both coronary artery involved accounted for 68.4%??and according to the location involved??right coronary artery involvement accounted for 41.5%??left main coronary artery involvement accounted for 39.0%??the left anterior descending coronary artery involvement accounted for 17.1%??and left circumflex artery involvement accounted for 2.4%. The clinical grade of severity in CAL children were???? grade in 5 cases??accounting for 26.3%???? grade in 8 cases??accounting for 42.1%???? grade in 6 cases??accounting for 31.6%. During follow-up??71.4% patients had coronary artery aneurysm or dilatation significantly retracted??the average time of retraction being ??1.5±0.7??years??of which 1 case was completely dissipated. Conclusion The incidence rate of the KD patients with CAL is 16.4%??in which CAD has the highest incidence rate while GCAA the lowest. CAL mostly occurs at 2-3 weeks after the disease. Any branch can be damaged??with the right coronary artery to be most likely involved. As to coronary artery disease severity??grade ?? is of the highest proportion. Most coronary artery aneurysm or dilatation has remarkable retraction within 1-2 years.     

川崎病冠状动脉损伤机制免疫遗传研究进展

川崎病（KD）是以全身性血管炎为主要特征的儿童常见疾病,其最严重的并发症即累及冠状动脉的心血管疾病,即使治疗后,也有部分儿童遗留冠状动脉瘤等后遗症。川崎病已成为儿童获得性心脏病最常见原因之一。目前KD冠状动脉损伤的机制尚不清晰。文章从免疫遗传方向综述国内外KD冠状动脉损伤机制最新研究成果与进展。     

川崎病冠状动脉损伤机制及治疗新进展

川崎病也称为皮肤黏膜淋巴结综合征,是一种具有自限性、由免疫介导的全身中小血管的炎性综合征,其中冠状动脉最易受累。目前川崎病已逐渐成为发达国家儿童冠状动脉损伤的首位病因。冠状动脉的损伤机制及治疗是目前川崎病研究的热点。该文就川崎病引起冠状动脉损伤的机制及治疗最新进展作一综述。     

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.     

547例川崎病临床分析及伴冠脉病变高危因素探讨

目的 总结川崎病(Kawasaki disease,KD)的临床特征,探讨KD临床表现与预后的关系,了解KD并发冠状动脉病变(coronary artery lesion,CAL)的危险因素.方法 同顾性分析1990年1月至2006年10月间547例住院KD患儿的临床资料,比较典型与不典型KD的临床特征,并探讨KD并发冠状动脉病变的高危因素.结果 547例中典型KD 434例,不典型KD 113例,不典型KD CAL发生牢较高(P＜0.05);除肛周脱屑外,其他临床症状发生少,出现晚(P＜0.05).547例KD中并发CAL 103例(18.82%),相关因素分析显示发病年龄、性别、血沉、血小板、C-反应蛋白与CAL发生率有密切关系.结论 不典型KD的CAL发生率较高,肛周脱屑较其他临床症状出现早,可以作为早期诊断线索之一;3岁以下的男孩及血沉、血小板、C-反应蛋白明显升高是CAL的高危因素.