丙种球蛋白防治川崎并发冠状动脉病变的机理探讨

目的 观察丙种球蛋白对川崎病患儿血清诱导人单核细胞表达血小板源生长因子（ＰＤＧＦ）Ｂ链蛋白ＰＤＧＦ－Ｂ的影响；观察丙种球蛋白作用后的单核细胞条件２基（ＭＣＭ）对内皮细胞凋亡的影响。方法 应用免疫细胞化学和流式细胞术方法。结果 川崎病患儿血清有强烈诱导单核细胞表达ＰＤＧＦ－Ｂ，川崎病患儿血清作用后的ＭＣＭ则能显著促进内皮细胞凋亡。川崎病患儿血清的上述作用能够被丙种球蛋白丙种球蛋白作用后的ＭＣＭ所抑制     

不同IVIG治疗方案对川崎病冠状动脉病变影响的多中心回顾性研究

目的 探讨静脉丙种球蛋白(IVIG)的不同治疗方案对川崎病(KD)患儿发生冠状动脉病变(CAL)的影响,提出最佳的IVIG治疗建议方案.方法 回顾性分析1998-2007年上海地区住院KD患儿1 682例的临床资料,男1 064例,女618例;发病年龄0.1～18.8岁,平均(2.57±2.33)岁.其中1 533例(91.1%)使用IVIG治疗,方案包括①1 g/kg×1次;②2 g/kg×1次;③0.4～0.5 g/kg×5次;④1 g/kg×2次;⑤2 g/kg×2次;⑥不规则治疗.IVIG治疗的时间窗包括:①病程1～4d;②病程5～10 d;③病程>10 d.结果 在1 533例IVIC治疗的KD患儿中,CAL总发生率为20.74%.与此相比较,CAL发生率明显降低者如下:①在病程5～10 d应用IVIG治疗1 g/kg×2次的KD患儿,其CAL发生率为12.06%;②病程5～10 d应用1 g/kg×1次者CAL发生率为15.00%;⑧病程1～4 d应用1 g/kg×1次者CAL发生率为16.28%;④病程5～10 d应用2 g/kg×1次者CAL发生率为16.71%.相反,以下几种情况CAL发生率明显高于CAL总发生率:①在病程10 d以上应用IVIC者;②使用IVIG 2 g/kg×2次(因使用1次后效果不佳而再次使用);③其他不规律使用IVIC者.结论 在IVIC治疗KD、减少CAL方面,以病程5～10 d使用IVIG 1 g/kg×2次效果最佳;但考虑经济因素和大剂量IVIG潜在的风险,在病程10 d以内使用IVIG 1 g/kg×1次亦是不错的选择.     

川崎病冠状动脉病变九年的回顾分析

川崎病是儿科常见的后天性心血管病之一 ,因其对心脏的损害 ,受到了普遍的关注。其中冠状动脉病变最为严重 ,多发生在疾病的急性期。我们回顾了 1992年 1月～ 2 0 0 0年12月在我院住院的川崎病患儿 875例 ,其中 313例患儿有川崎病急性期冠状动脉病变 ,初步分析如下。方法及诊断标准本组病例诊断均符合 1984年日本川崎病研究委员会修订的诊断标准。采用日本东芝 14 0Ａ及美国ＡＬＴ公司Ｕ 9超声诊断仪 ,探头频率 3或 5ＭＨｚ。冠状动脉 (ＣＡ)内径正常值采用我院资料[1] ,内径超过该年龄的正常值为冠状动脉扩张(ＣＡＤ)。左冠状动脉 /主动脉…     

川崎病20例

川崎病 (ＫＤ)是一种以全身血管炎性改变为主要病理改变的急性发热性出疹性疾病。其冠状动脉病变发生率可达15 %～ 3 0 % ,成为该病死亡的主要原因。现将我科应用大剂量丙种球蛋白治疗且长期随访的 2 0例ＫＤ报告如下。资料与方法一、一般资料　 2 0例均符合 1984年日本ＫＤ研究委员会提出的临床诊断标准。男 12例 ,女 8例。～ 1岁 4例 ,～ 3岁 11例 ,～ 4岁 3例 ,4岁 2例 ,平均发病年龄 2 .9岁。二、临床表现　 2 0例均有持续发热 ,体温最高达 40℃ ,平均热程 1～2周 ,热程最长 3 0ｄ ;皮疹 17例 (85 % ) ;结膜充血 16例 (80 % ) ;四肢末端…     

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。     

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.     

川崎病静脉注射丙种球蛋白无反应预测模型研究现状

川崎病(Kawasaki disease，KD)是一种急性自身免疫性系统性血管炎，是发达国家儿童获得性心脏病的主要病因。KD最严重的后果是冠状动脉病变(coronary artery lesions，CALs)，与KD的预后相关。临床研究证实静脉注射丙种球蛋白(IVIG)耐药是CALs的独立危险因素。近年来，一系列的预测模型已被开发来评估IVIG耐药的风险。然而，目前基于KD儿童人口学特征、临床表现、实验室检查及遗传特性的IVIG耐药性预测评分系统在不同民族和同一民族不同地区的人群中存在显著差异，尚未建立适用普遍人群的预测模型。     

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。     

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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.     

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.     

The pathophysiology of coronary artery aneurysms in Kawasaki disease: role of matrix metalloproteinases

Kawasaki disease is an acute inflammatory syndrome that takes the form of systemic vasculitis, and predominantly affects children. Important complications of this disease are coronary artery dilation and aneurysm formation. Recent studies indicate that Kawasaki disease patients have elevated expression, activity, or protein levels of matrix metalloproteinases (MMPs), and suggest that imbalances in MMPs or MMP/tissue inhibitor of MMP (TIMP) play important pathophysiological roles in the development of coronary artery lesions in this disease. However, it remains unclear whether MMP activities at the site of coronary artery lesions are indeed increased. Further studies on the effects of MMP inhibition on coronary outcome are needed to define the roles of MMPs and TIMPs in the formation of coronary artery lesions in Kawasaki disease; findings of such studies may support the use of MMP inhibitors for the prevention of coronary artery complications in patients with this disease.     

川崎病冠状动脉损害相关危险因素分析

目的探讨川崎病(KD)并发冠状动脉损害(CAL)的相关危险因素。方法回顾性分析110 例KD患儿的临床资料,根据超声心动图检查结果分为冠脉损害组(CAL组)40例,无冠脉损害组(NCAL组)70例,进行统计分析。结果 110例KD患儿的中位发病年龄为1.58(0.92～3.00)岁,其中男67例、女43例。CAL组和NCAL组间年龄分布、不典型KD、静脉用丙种球蛋白(IVIG)前发热持续时间6天比例差异均有统计学意义(P0.05)。CAL组患儿C反应蛋白(CRP)、白细胞(WBC)、白介素-6(IL-6)、N 端脑钠肽前体(NT-proBNP)水平均明显高于NCAL组,差异均有统计学意义(P0.05)。IVIG使用前发热持续时间、WBC、IL-6、NT-proBNP预测KD患儿并发CAL的受试者工作特征曲线(ROC)下面积(AUC)分别为0.97、0.69、0.76和0.87;最佳临界值分别为6.5天、19.85×10~9/L、92.19 pg/mL和1 122.5 pg/mL,预测KD并发CAL的灵敏度分别为84.6%、46.2%、100%和100%,特异度分别为91.4%、91.4%、57.1%和71.4%。结论年龄3岁,IVIG前发热持续时间6.5天,不典型KD,以及WBC、IL-6和NT-proBNP明显升高,是KD发生CAL的危险因素。     

川崎病患儿血红蛋白及血钾变化与冠状动脉病变的关系研究

川崎病(Kawasaki disease,KD)又称皮肤粘膜淋巴结综合征(mucocutaneous lymphnode syndrome,MCLS),是一种以全身血管炎性病变为主的儿童急性发热出疹性疾病,主要病理改变为全身性非特异性血管炎,可引起多脏器受累,以心血管病变最为严重,多侵犯冠状动脉[1],川崎病的早期诊治具有十分重要的意义。本研究通过观察川崎病患儿治疗前后血红蛋白和血钾的变化,结合冠状动脉受累情况,探讨血红蛋白和血钾在川崎病并发冠状动脉病变中的意义。1对象与方法1.1对象2000年1月～2004年1月我科收治川崎病急性期(≤11d)住院患儿50例,均符合第3届国际川崎病…     

肺炎支原体与肺炎衣原体感染在川崎病冠状动脉病变中的作用

目的探讨肺炎支原体(MP)和肺炎衣原体(CP)感染在川崎病(KD)冠状动脉病变(CAD)中的作用。方法用ELISA方法检测115例KD患儿和50名对照组儿童血浆MP和CPIgG抗体。①按CAD发生与否分组,行组间MP和CP感染率的比较;②先按是否CP感染分组,然后再按伴CAD与否分组,行组间MP感染率的比较。结果KD组与对照组比较,MP和CP感染率差异均无统计学意义(P>0.05);CAD组MP感染率高于非CAD组(61.9%∶26%,P<0.05),但CP的感染情况在两组间差异无统计学意义(69%:67.1%,P>0.05)。在CP感染的KD患儿中,CAD组MP感染率高于非CAD组(51.7%:16.3%,P<0.01);相反,在无CP感染的KD患儿中,CAD组与非CAD组间MP感染率差异无统计学意义(38.5%∶33.3%,P>0.05)。结论MP感染可能与CAD有关,但MP和CP的共同感染或许在其中起着更重要作用。