【医院】川崎病的诊治进展及其心血管并发症

1、http:/ Dec. Beijinghttp:/ Diseases, KD)又称皮肤粘膜淋巴结综合征(Mucocutaneous lymph node syndrome，MCLS)主要发生在5岁以下婴幼儿的急性发热出疹性疾病病因、发病机制不明发病时伴全身性血管炎，属血管炎综合征，所致冠状动脉并发症已经是最常见的儿童后天性心脏病http:/ Kawasaki) 首次报道，日文文献;1961 至 1967 的50例病例，皮肤黏膜淋巴结综合症；最初认为是一种良性疾病。http:/ Shigematsu等进行了日本第一次全国川崎病普查，结果10例2yr的患儿在症状好转时死亡；1976年美国Hawai

2、i大学的Maria Melish在日本外首次报道了16例MCLS患儿 ，称Kawasaki Disease；在世界范围内都有报道，发病率逐年增高趋势。http:/ http:/ Y, Yanagawa H. Prog Ped Cardiol, 2004,19:99108 美国KD发病率报道资料 研究者研究者地区地区调查年限调查年限发病率发病率调查方法调查方法Holman全国全国2000199717.117.6全国儿童住院病人数据库全国儿童住院病人数据库Chang 加州加州1995-199915.3州住院系统数据库州住院系统数据库Gibbons佐治亚州佐治亚州1997-199814.0住院病历调

3、查住院病历调查BronsteinSan Diego1994-19988.0-15.4住院病历调查住院病历调查Holman全国全国199717.3全国儿童住院系统数据库全国儿童住院系统数据库Holman夏威夷州夏威夷州康乃迪克州康乃迪克州1994-19971993-199647.718.8各州住院系统数据库各州住院系统数据库Belay 西海岸四州西海岸四州 1993-19969.0-19.1 保险公司住院系统数据库保险公司住院系统数据库Holman阿拉斯加州阿拉斯加州1980-19954.8阿拉斯加美国印第安医疗系统阿拉斯加美国印第安医疗系统Taubert美国美国33个州个州1984-19939

4、.87住院病历调查住院病历调查Davis西雅图地区西雅图地区1987-198915.2住院病历调查住院病历调查北京川崎病发病率010203040506070801995 1996 1997 1998 1999 2000 2001 2002 2003 2004YearIncidence (/100000 children5year)BoysGirlsTotalDu Zhong-Dong, et al. Pediatr Infect Dis J. 2002,21(2):103107http:/ 1545天天北京近北京近10年急性死亡率年急性死亡率0远期死亡因为冠状动脉并发症远期死亡因为冠状动脉并发

5、症http:/ 40年来很多学者筛选了无数种微生物的感染证据但到目前仍未找到明确的病因(coravirus):培养培养血清学血清学动物接种动物接种http:/ (Rowley 2008)?可能的发病机制可能的发病机制: 川崎病是由一种或多种感染因素引起的川崎病是由一种或多种感染因素引起的一种强烈的免疫反应，这种反应只发一种强烈的免疫反应，这种反应只发生于本身基因有易感性的个体，如亚生于本身基因有易感性的个体，如亚裔人群裔人群http:/ 陌及指趾端充血；恢复期指趾端甲床皮肤 移行处有膜状脱皮6、急性期出现非化脓性颈部淋巴结肿胀http:/ 心外膜炎：发生率1517%；心肌炎：发生率约30%；大

6、多数患儿没有症状，偶尔有心包填塞或心功能不全；心内膜炎和/或二尖瓣返流：2.5%；多轻度，中度或重度1.5倍倍; 或者冠状动脉内膜呈明显不规整。或者冠状动脉内膜呈明显不规整。分型分型:小瘤：内径小瘤：内径 8 mmhttp:/ of coronary http:/ 20%男男 24%，女，女15%（P0.001)IVIG治疗后治疗后: 56%未用未用IVIG治疗冠状动脉瘤治疗冠状动脉瘤: 4%IVIG治疗后治疗后: 0.05）RCALCA第一次冠脉造影第一次冠脉造影(发发病病40 d)RCALCA第二次冠脉造影第二次冠脉造影 (发发病病6个月个月http:/ 2000.4 2000.12 20

7、02.3冠脉狭窄及再通冠脉狭窄及再通http:/ http:/ http:/ H, Akagi T, et al. Circulation 1996; 94: 13790 01010202030304040505060600 05 51010151520201 13 35 57 79 91111131315151717(years)http:/ CAL 2 年消退年消退 n血管中层平滑肌细胞增生，内膜增厚血管中层平滑肌细胞增生，内膜增厚n冠状动脉狭窄发生约占全部冠状动脉狭窄发生约占全部KD的的4%， 合合并冠状动脉并发症患儿的并冠状动脉并发症患儿的20%n没有报道冠状动脉扩张回复后发生冠状动没

8、有报道冠状动脉扩张回复后发生冠状动脉狭窄？脉狭窄？http:/ (10 - 20 年年)临床表现缺乏或不典型临床表现缺乏或不典型http:/ ASP, 华法林？冠状动脉搭桥手术冠状动脉经导管介入性治疗其它? Arterial graftVenous graftYoshikawa, et al. Eur J Cardio Surg 2000http:/ 经皮腔内冠状动脉旋切术经皮腔内冠状动脉旋切术经皮腔内冠状动脉血运重建术经皮腔内冠状动脉血运重建术日本介入性治疗效果http:/ 经导管介入性治疗的未来干预的时间选择？干预的时间选择？治疗器械的选择治疗器械的选择提高远期效果：提高远期效果：再狭窄再

9、狭窄新瘤形成新瘤形成治疗指导原则的建立与更新治疗指导原则的建立与更新病人及家长的教育病人及家长的教育http:/ T, Kato H, Inoue O, et al. The Journal of Pediatrics 1992;121.684-8 http:/ YF, Wong SJ, Ho MH. Archives of Disease in Childhood 2007;92:43-7 川崎病冠状动脉并发症与冠脉粥样硬化的关系？http:/ DISEASE AND ITS CORONARY COMPLICATIONZhong-Dong Du, MD, PhDProfessor of Pe

10、diatricsBeijing Childrens HospitalCapital Medical Universityduzhongdongvip.http:/ BRIEFSDefinition and historyEpidemiologic featuresEtiology and mechanismClinical pictures and diagnosisTreatmentLong-term management of coronary complicationhttp:/ disease (KD) is an acute systemic febrile illness pred

11、ominantly affecting children 5 yrs of age. Initially described in Japan, KD has been reported worldwideKD is associated with coronary complication that might be life-threatening, and has become the leading cause of acquired heart diseaseMay be a risk factor for adult ischaemic heart disease Its etio

12、logy remains unknown.http:/ HISTORYFirst described by Tomisaku Kawasaki in Japanese literature in 1967. He reported on 50 children who presented from 1961 to 1967 with symptoms distinct from other known childhood illnesses, and termed the disorder mucocutaneous lymph node syndrome.Originally thought

13、 it was a benign self-limited disease. http:/ HISTORYBy late 1970, Itsuzo Shigematsu organized the first Japan nation wide KD Survey. Up to 10 deaths had occurred in children under age 2 years with KS in Japan.All the death was found to be caused by coronary aneurysm and thrombus in the coronary art

14、ery. In 1976, Melish described the same illness in 16 children in Hawaii. Reported world wide. http:/ BRIEFSDefinition and historyEpidemiologic featuresEtiology and mechanism Clinical pictures and diagnosisTreatmentLong-term management of coronary complicationhttp:/ http:/ OF KD IN JAPANNakamura Y,

15、Yanagawa H. Prog Ped Cardiol, 2004,19:99108 http:/ OF KD IN USAAuthorAreasStudy yearRatesStudy methodsHolmanAll states2000199717.117.6National in-pts data baseChang California1995-199915.3States in-pt data baseGibbonsGeorgia1997-199814.0SurveyBronsteinSan Diego1994-19988.0-15.4SurveyHolmanAll states

16、199717.3Kids in-pt data baseHolmanHI, CO1994-19971993-199647.718.8States in-pt data basesBelay West costal 1993-19969.0-19.1 Data base of health insuranceHolmanAlaska1980-19954.8Data base Taubert33 states1984-19939.87In-pt chart surveyDavisSeatle1987-198915.2In-pa chart surveyhttp:/ OF KD IN BEIJING

17、010203040506070801995 1996 1997 1998 1999 2000 2001 2002 2003 2004YearIncidence (/100000 children3 mm in children 4 mm in children =5 years oldBeijing Childrens Hospital (1988): 2.5 mm if age 3 mm if age 3-9 yr 4 mm if age 9yrhttp:/ Coronary Artery Diameter corrected by BSA (Zorzi)http:/ LCX LADRCA

18、http:/ aneurysm:Definition: if the internal diameter of a segment measures 1.5 times that of an adjacent segment; or if the coronary lumen is clearly irregular.Classification:small 8-mm internal diameterhttp:/ of coronary http:/ OF CORONARY COMPLICATIONSUntreated children with KD: 20%Boys 24%，Girl15

19、%（P0.001)After IVIG treatment: 56%.Coronary aneurysm untreated pts: 4%After IVIG: 0.05）http:/ Angiography (40 days after the onset)http:/ Angiography (6 months after the onset)http:/ 2000.4 2000.12 2002.3Coronary artery obstruction and recanalizationhttp:/ in VRRCA in VRhttp:/ in MPRRCA in MPRhttp:/

20、 H, Akagi T, et al. Circulation 1996; 94: 13790 01010202030304040505060600 05 51010151520201 13 35 57 79 91111131315151717(years)http:/ FEATURE OF CORONARY ARTERY LESION IN KDnAbout 50% of CAL regress 2 years. nIntimal proliferation derived from the smooth muscle cells of media and regenerated endot

21、helium.nCoronary stenosis occurred in 4% of all patients, or in 20% with CAL in 10-22 years follow-up.nNo coronary stenosis has developed in 10-20 years follow-up in patients with regressed aneurysms.http:/ ARTERIAL LESION IN KDPediatric OnsetHigh Incidence of Calcified LesionMixed Lesions (Stenosis & Aneurysm)Progressive (10 to 20 years interval)Less Clinical Symptoms of Ischemiahttp:/ OF CORONARY STENOSIS OR OCCLUSIONMedicine: ASP, WarfarinCoronary by-pass surgeryTranscatheter interventionOther? http:/ graftVenous g