川崎病的冠状动脉损害研究概况

川崎病的冠状动脉损害研究概况广东工学院医院李洁综述武汉冶金高等医学专科学校张天民审校川崎病自１９６７年首次报告后，日本已有１０万例以上，全世界都有病例报道。川崎病患者中２０％～４０％有冠状动脉（管称冠脉）损害，可致心肌梗塞、心衰、猝死［１］，是川崎病...     

川崎病的心脏损害及治疗现状

川崎病,又称粘膜皮肤淋巴结综合征,1967年由日本儿科医生Tomisaku Kawasaki初次描述,多累及5岁以下儿童,是一种原因未明的以变态反应性全身血管炎为主要病理改变的急性发热出疹性疾病,具有自限性.川崎病可能由感染因素引起,超抗原介导了淋巴细胞激活及免疫应答,引起全身血管炎性损伤,导致血管的结构改变及功能障碍.川崎病在亚洲国家(尤其是日本)高发,提示遗传因素亦发挥了一定作用[1].川崎病血管炎主要累及中小动脉,可累及多个脏器,导致心肌炎、心包炎、冠状动脉病变、雷诺现象、关节炎、腹痛、肝功能异常、胆囊水肿、一过性感觉神经性耳聋、无菌性脑膜炎等.其中,最严重的并发症为冠状动脉损害,可导致儿童心肌梗死、心力衰竭及猝死的发生.在发达国家,川崎病已经取代风湿热成为儿童获得性心脏病的主要病因.在我国,川崎病并非少见,儿童川崎病患者成年后心血管系统后遗症的诊断和规范处理是成人心血管医生需要面临的问题,以下将对川崎病的心脏损害及治疗进行简要综述.     

川崎病患儿冠状动脉损害危险因素分析

目的探讨川崎病（KD）并发冠状动脉（冠脉）损害（CAL）的危险因素。方法将215例KD患儿按开始治疗时间分为早期治疗组（发热10d内进行治疗者）和延迟治疗组（发热10d,后进行治疗者）;按发病年龄分为婴儿组（≤12个月）及儿童组（〉12个月）;按CAL发生情况分为CAL组和NCAL组。收集所有KD患儿的心脏超声冠脉检查结果及各项实验室检查结果,进行相关分析。结果延迟治疗组冠脉病变发生率为40．3％,与早期治疗组的22．8％相比,P〈0．05;婴儿组冠脉病变发生率为35．5％,与儿童组的21．3％相比,P〈0．05;CAL组与NCAL组持续发热时间及血清C反应蛋白（CRP）、血清白蛋白、血钠水平相比,P均〈0．05。结论延迟治疗、1岁以下、热程〉10d、血清CRP水平升高、血钠及白蛋白水平降低是KD并发CAL的危险因素。     

氨基末端脑钠肽前体在川崎病心脏损害时的临床意义

目的分析氨基末端脑钠肽前体(NT-proNBP)在川崎病心脏损害时的临床意义。方法将在我院诊治的川崎病心脏损害患者40例作为观察组,选择同期35例健康儿童作为对照组,检测两组血浆NT-proNBP水平。结果观察组血浆NT-proNBP水平高于对照组,差异有统计学意义(P<0.05)。结论 NT-proNBP水平可作为辅助诊断川崎病心脏损伤的标志物,有助于疾病及时治疗,避免患者病情进一步加重。     

川崎病并发冠状动脉损害的诊断

川崎病(KD),又称皮肤粘膜淋巴结综合征,由日本学者川崎富作于1967年首次描述和报告.该病是一种好发于5岁以下儿童、病因未明的血管炎性疾病,以发热、双侧球结膜充血、口腔黏膜炎症、手足硬肿、皮疹以及颈部淋巴结肿大、血小板增高、血沉增快、C-反应蛋白增高为其临床特征.     

丹参酮ⅡA对川崎病并发冠状动脉损害患儿基质金属

目的　检测不同的治疗方案下川崎病（　Kawasaki　disease,　KD　）患儿治疗前后基质金属蛋白酶9　（matrix　metalloproteinase-9,　MMP-9）及组织型基质金属蛋白酶抑制剂1　（　tissue　inhibitor　of　metalloproteinase-1,　TIMP-1）表达的动态变化,分析丹参酮ⅡA（TanⅡA）对上述指标的影响以及对川崎病并发冠状动脉损害（coronary　artery　1esion,　CAL）的保护作用。方法　将2010　年4　月至2012　年9　月在南华大学附属第二医院住院治疗的川崎病患儿48例,包括无冠状动脉损害28例,有冠状动脉损害20例。随机分为TanⅡA加常规治疗组和常规治疗组,各组24例（无冠状动脉损害14例,有冠状动脉损害10例）,并以健康体检儿童作为对照组。分别采用酶联免疫吸附法（ELISA）检测治疗前后患儿血清MMP-9、TIMP-1变化表达变化。实时荧光定量PCR（qRT-PCR）检测治疗前后患儿外周血单个核细胞（peripheral　blood　mononuclear　cells,　PBMC）MMP-9、TIMP-1　mRNA表达水平。结果　川崎病患儿治疗前MMP-9、TIMP-1　mRNA和蛋白表达均较正常对照组显著升高,且有冠状动脉损害的川崎病患儿血清MMP-9、TIMP-1、MMP-9/TIMP-1比值明显高于无冠状动脉损害（no　coronary　artery　1esion,　NCAL）川崎病患儿（P<0.05）;治疗5～7　天后　MMP-9、TIMP-1　mRNA和蛋白表达显著降低（P<0.05）,其中TanⅡA加常规治疗组较常规治疗组MMP-9、TIMP-1　mRNA和蛋白表达下降更明显（P<0.05）,同时,在有冠状动脉损害的川崎病患儿中,　TanⅡA加常规治疗后,与常规治疗相比,其血清MMP-9、TIMP-1也明显降低（P<0.05）。结论　TanⅡA可以一定程度抑制川崎病患者MMP-9、TIMP-1　mRNA和蛋白表达,从而减轻血管炎性损伤,减少川崎病并发冠状动脉损害。     

215例川崎病的消化道损害及其治疗与护理

川崎病又名皮肤粘膜淋巴结综合征,其病因不明,但与感染有关,基本病理改变为全身广泛性血管炎。由于其发病率的增加及其对心血管系统损害的严重性,现已取代风湿热而成为小儿后天性心脏病的首要原因,日益受到国内外儿科界学者的重视。但在对川崎病的临床研究工作中,绝大多数文献报道的都是川崎病的心血管损害的情况,作者对川崎病的多系统损害进行研究之后,认为有必要对其消化道损害进行报道。     

川崎病患儿冠脉损害78例临床分析

川崎病（KD）是一种以全身血管炎为主要病变的儿童急性发热性、出疹性疾病,患儿常有冠状动脉（下称冠脉）受损。1978年8月-2004年2月,我院收治KD患儿78例,其中36例有冠脉损害。现将KD患儿的冠脉损害情况报告如下。     

川崎病心血管损害心电表现暨心肌梗死的心电评价

目的 了解川崎病（MCLS）对心肌损害,评价心电图（ECG）对该病继发心肌梗死的临床意义。方法 以常规12导联同步描记（必要者加做V3R～V6R、V7～V9导联）,对84例患儿作分析。结果 经积极治疗后,70例（83.3%）治愈出院,死亡2例（2.4%）〔其中：重症心肌炎併急性左心衰竭死亡1例（1.2%）,冠脉瘤继发血栓形成导致急性广泛性前壁及右室壁心肌梗死致猝死1例（1.2%）〕;12例病情不稳定,反复发作,心电图表现多样性、易变性,经半至1年的治疗,ECG逐渐转为正常,病情逐渐稳定。结论 ECG对MCLS所致心血管损害反应敏感,尤其对心肌缺血、损伤、梗死诊断及演变过程的观察、心律失常及预后评价具有重要意义。     

Anderson Fabry病的心脏损害及治疗

Anderson Fabry病(AFD)是X性染色体显性遗传疾病,国内未见报道。患者通常有多器官损害,心脏损害尤其常见,可发生进展性肥厚型心肌病、瓣膜病、心律失常,亦可导致冠心病。目前在各种原因所致的肥厚型心肌病中,只有AFD导致的肥厚型心肌病可进行病因治疗。现就AFD患者的心脏病变以及α-galA酶替代疗法作一概述。     

甘油三酯/高密度脂蛋白胆固醇比值对老年冠心病诊断价值的探讨

目的 :探讨甘油三酯 /高密度脂蛋白胆固醇 (TG/ HDL- C)比值对老年冠心病 (CHD)的诊断价值。方法 :分析 34 2例选择性冠状动脉造影 (冠脉造影 )确诊的老年 CHD患者 (CHD组 )及 96例冠脉造影阴性老年人 (对照组 )的 TG/ HDL - C比值水平与 CHD之间的关系。结果 :CHD组患者的 TG/ HDL - C比值水平及异常率均明显高于对照组 (2 .5 4± 1.2 9比 1.32± 1.16 ,P<0 .0 0 1,35 .6 %比 13.7% ,P<0 .0 0 1)。经多元逐步回归分析显示 :与 TG、HDL - C相比 ,该比值与 CHD之间显示了更有意义的回归关系 (β值 0 .32 ,P值 =0 .0 0 0 1,复相关系数为 0 .5 5 )。结论 :TG/ HDL- C比值对于老年 CHD诊断是一有临床使用价值的指标     

Intermittent heparin infusion in children with ischemic heart disease caused by Kawasaki disease

We determined change in serum hepatocyte growth factor (HGF), one of the most potent angiogenic factors, after intravenous infusion of heparin in children and reported successful improvement of cardiac ischemia after regular intravenous heparin infusion in an infant with ischemic heart disease (IHD) caused by Kawasaki disease (KD). Intravenous infusion of 50 units/kg of heparin significantly increased serum HGF from 0.8+/-0.6 to 8.4+/-3.4 at 5 min, 8.3+/-2.5 at 30 min, and 4.9+/-1.5 ng/ml at 60 min, respectively. Subsequently, a 16-month-old infant with IHD caused by KD had received regular intravenous infusion of heparin, 100 IU/kg twice a day for 1 month and once a week for subsequent 2 months. With this treatment, his perfusion defect on myocardial scintigraphy disappeared. In conclusion, regular heparin infusion alone can improve myocardial ischemia caused by KD, probably due to facilitated production of HGF.     

甘油三酯/高密度脂蛋白胆固醇比值对冠心病的诊断价值的探讨

目的　探讨甘油三酯 /高密度脂蛋白胆固醇 (TG/HDL C)比值对冠心病 (CHD)的诊断价值。方法　分析 173例选择性冠状动脉造影 (冠脉造影 )确诊的CHD患者 (CHD组 )及 118例冠脉造影阴性者 (对照组 )的TG/HDL C比值水平与CHD之间的关系。结果　CHD组患者的TG/HDL C比值水平及异常率均明显高于对照组 (2 0 2± 1 19vs 1 4 0± 0 97,P <0 0 1,2 8 9%比 11 8% ,P <0 0 1)。经多元逐步回归分析显示 :与TG、HDL C相比 ,该比值与CHD之间显示了更有意义的回归关系 (β值 0 2 5 ,P值 =0 0 0 0 1,复相关系数为 0 4 5 )。结论　TG/HDL C比值对于CHD诊断是一有临床使用价值的指标。     

运动平板试验对女性冠心病预测价值的相关性研究

运动平板试验（TET）是目前采用最广泛的心电图（ECG）负荷试验,是诊断女性冠心病（CHD）或判断预后的重要无创检查手段之一.     

Kawasaki disease in children

Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown aetiology. It is the most common cause of acquired heart disease in young children. The intense inflammatory process has a predilection for the coronary arteries, resulting in the development of aneurysmal lesions, arterial thrombotic occlusion or, potentially, sudden death. There is no specific diagnostic test; however, treatment with immunoglobulin and aspirin effectively reduces cardiac complications from 25% to 4.7% in the UK. Inflammation of the myocardium, endocardium or pericardium can occur early in the disease and endothelial dysfunction along with abnormalities of myocardial blood flow may require continuing medication, interventional catheterisation or even cardiac surgery. Several new pharmacological treatments may have important roles to play in managing KD in children and adolescents. This review discusses the history of the disease, the diagnostic challenges, epidemiology, aetiology, pathology, immunopathogenesis, treatment, genetic influences and the long-term cardiovascular sequelae.     

The cardiovascular risk in children with congenital heart disease

We aimed to study the prevalence of risk factors of atherosclerosis as defined in the INTERHEART study, in children with congenital heart disease. PATIENTS AND METHOD: a cross-sectional study performed in 262 children aged from 3 to 18 years old, with an operated or non-operated congenital heart disease. They all responded to a standardized questionnaire on their lifestyle and family history of atherosclerotic disease. RESULTS: A majority of patients presented risk factors: insufficient physical activity (89.0%), lack of daily intake of fruits and vegetables (43.1%), stress (31.3%), familial risk of hypercholesterolemia (14.1%), overweight (7.6%), active smoking (20.6% in teenagers after 14 years old), hypertension (2.3%). These children had a mean of 1.9 + 1.0 risk factors. CONCLUSION: A majority of children with congenital heart disease have risk factors of atherosclerosis. The results of this study highlight the importance of atherosclerotic cardiovascular diseases prevention in these young patients.     

Sensitivity, specificity and predictive value of two-dimensional echocardiography in detecting coronary artery aneurysms in patients with Kawasaki disease

Seventy-seven patients with Kawasaki disease were prospectively evaluated from 1978 to 1983 to determine the sensitivity, specificity and predictive value for detecting coronary artery aneurysms with two-dimensional echocardiography. Seventy (91%) underwent selective coronary arteriography and are included in this report. The study was divided into two periods because of increased experience and the use of a systematic approach with two-dimensional echocardiography in the second period as compared with the first. Aneurysms were demonstrated in nine patients (13%). The coronary artery system was divided into six regions: proximal third of the main right coronary artery, distal right coronary artery, left main coronary artery, left anterior descending coronary artery, circumflex coronary artery and distal left coronary artery. The sensitivity and specificity were high when imaging the proximal regions, and improved from the first period to the second. Both sensitivity and specificity were lower for the more distal regions of the right and left coronary arteries. Overall, the sensitivity of two-dimensional echocardiography was 100% because there were no patients in our study who had isolated distal coronary artery aneurysms. Two-dimensional echocardiography is a sensitive and specific test for detecting aneurysms in the proximal portions of both the right and left coronary arteries, and is useful in selecting patients for invasive investigation with selective coronary arteriography.     

川崎病合并肝损害的特点及相关因素分析

目的研究川崎病合并肝损害的特点,探讨可能的相关因素,提高对川崎病合并肝损害的认识。方法收集2008-2013年收治的75例川崎病患儿的临床资料,回顾性分析肝损害的临床特点及血常规、C反应蛋白(CRP)、血沉、肝功能检查、腹部B超等相关指标。计数资料采用χ2检验,计量资料组间差异采用t检验分析、多元逐步回归分析。结果 75例川崎病患儿ALT异常发生率32%,79.2%患儿血清ALT升高在200 IU/L以内,其中伴肝肿大的患儿3例,黄疸、脾大患儿各1例,分别占肝损害患儿的12.5%、4.2%和4.2%。在肝损害的相关因素分析中未发现CRP、血沉与肝损害有相关关系(t=1.560、1.291,P=0.123、0.201)。血小板在肝损害的研究中,与对照组差异有统计学意义(t=-3.228,P=0.003)。白细胞计数升高在肝损害组与对照组之间差异不明显,但是与血小板同时升高时肝损害几率明显升高(P0.05)。结论未发现CRP、血沉与肝损害的发生有明显相关关系,但是血小板的异常与肝损害的发生关系密切,白细胞计数也与肝损害的发生存在一定联系,应引起临床医生注意,及早干预。     

Relative atherogenicity and predictive value of non-high-density lipoprotein cholesterol for coronary heart disease

Although low-density lipoprotein cholesterol (LDL-C) is a well-established atherogenic factor for coronary heart disease, it does not completely represent the risk associated with atherogenic lipoproteins in the presence of high triglyceride (TG) levels. Constituent lipoproteins constituting non-high-density lipoprotein cholesterol (non-HDL-C) include atherogenic TG-rich lipoproteins, cholesteryl ester-enriched remnants of TG-rich lipoproteins, and lipoprotein(a). Recent observational and intervention studies suggest that the predictive value of non-HDL-C for cardiovascular risk and mortality is better than low-density lipoprotein cholesterol and that non-HDL-C correlates highly with plasma apolipoprotein B levels. Currently, the National Cholesterol Education Program Adult Treatment Panel III guidelines identify non-HDL-C as a secondary target of therapy in patients with TG elevation (> or =200 mg/dl) after the attainment of LDL-C target goals. In patients with coronary heart disease or coronary heart disease risk equivalents, an optional non-HDL-C goal is <100 mg/dl. To achieve the non-HDL-C goal, statin therapy may be intensified or combined with ezetimibe, niacin, a fibrate, or omega-3 fatty acids. In conclusion, non-HDL-C remains an important target of therapy for patients with elevated TGs, although its widespread adoption has yet to gain a foothold among health care professionals treating patients with dyslipidemia.