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1、KawasakiDisease,宋雷,川崎 富作 （1925.2.7 ）,First introduced in 1967,概念,原因未明全身性血管炎综合征，主要影响中小动脉全世界均有发病，以亚裔儿童多见 1976年我国首例报导5岁以下占80%，平均年龄1.5-2岁，男：女=1.5：1微生物超抗原激活具有遗传易感性患儿的T细胞，引发异常免疫反应，导致免疫性损伤,Jett Travolta, Son of John Travolta,Jett Travolta, 16 yrs old, died after suffering a seizure at the familys vacation
2、home in the Bahamas. Jett was suffering from Kawasaki Syndrome since he was 2-years-old,临床症状和诊断标准,发热5d球结膜充血口唇潮红、杨梅舌、黏膜充血不定形皮疹，多形红斑急性期手足硬肿，掌跖及肢端红斑；恢复期指趾端脱皮非化脓性颈淋巴结肿大6个主要症状满足5个即可诊断，如只有4个，但超声或造影证实冠状动脉瘤或扩张，也可确诊,心脏受累表现,典型病理表现,Discrete regions of myointimal proliferation associated with disrupted internal
3、 elastic lamina and medial smooth muscle cell necrosis with replacement by fibrosis and calcification,冠状动脉损害,20%未治疗及5%经IVIG治疗后的KD患者发生冠脉损害以冠状动脉瘤或扩张多见绝大多数冠状动脉瘤发生在冠状动脉主干近端，最常见为LM近端，其次为RCA近端、LAD、RCA中段小（Small）8mm除冠脉外，KD还可累及心脏瓣膜、心肌和心包,40 岁CAG患者261例冠状动脉瘤/扩张16 例4例确诊继发于KD，9例可以继发于KD,预后,急性和亚急性期出现的轻度冠脉扩张多可在40d内
4、恢复多数小到中等的冠状动脉瘤在发病后12年消失巨大冠状动脉瘤或多个呈串珠状中等冠状动脉瘤可能发生心肌梗死、破裂导致的猝死及狭窄导致的缺血性心脏病川崎病并发心肌梗死者约占0.1-1，多发生在第一年,并发心梗的高危因素,冠状动脉瘤最大内径8mm冠状动脉瘤形态为囊状、念珠状、香肠状急性期发热时间21 d急性期单独使用糖皮质激素发病年龄2岁,心脏超声显示冠脉近端病变,超声心动图是检测冠状动脉并发症的最重要的非侵人性方法，与冠状动脉造影和尸检结果符合率达86-100,CT在川崎病诊断中的价值,CTA showing extensive calcification of a LAD aneurysm in
5、 a 12-year-old male patient 7 years after acute Kawasaki disease.,Coronary artery aneurysms in a 9-year old boy, 3 years after diagnosis of Kawasaki Disease,Case 1,3-year-11-month-old boy with KD and giant coronary aneurysms . Coronary angiogram of left coronary artery shows aneurysms of LAD and LCX
6、.,Case 2,Occluded RCA aneurysm in a 33-year-old man who experienced a KD at 6 years old and was asymptomatic until his myocardial infarction. Injection of the occluded RCARCA after PTCA. Note smooth appearance of distal branches of the RCAPatent RCA aneurysm 1 month after PTCA, aspirin, and warfarin
7、 therapy maintaining an INR of 2-2.5,Case 3,A 33-year-old woman developed shock and died 16 hours after labor with moderate hemorrhage. When the patient was 16 years old, she was diagnosis as Kawasaki disease (KD). This womans son, the product of that pregnancy, was admitted 5 years later with class
8、ic KD.,成人疑诊川崎病,幼年病史：长期发热、皮疹、硬肿、结膜充血尚无特异性回顾性诊断指标影像学：胸片或CT示原动脉瘤形成部位动脉壁钙化鉴别诊断：病变多局限IVUS有助于观察增厚的动脉壁,治疗,急性期大剂量丙种球蛋白静脉滴注肠溶阿司匹林 3050mg/kgd， 热退后3天渐减为35mg/kgd，持续 68周，冠状动脉损害者应延长治疗糖皮质激素不宜单独使用，对IVIG无效者可考虑加用恢复期口服抗血小板或抗凝药物定期复查，避免过量运动必要时PTCA或CABG，主要取决于缺血症状,冠状动脉瘤的进展,50% 恢复正常25% 变小25% 无变化7-20% 发生动脉瘤相关的狭窄或心肌梗死,小或中等冠状
9、动脉瘤,药物治疗口服小剂量阿司匹林直到恢复正常体育活动11岁，在发病6-8周内避免体育活动11-20岁，基于2年1次的负荷试验/心肌灌注扫描结果不鼓励接触性/高负荷运动随访每年复查心脏超声及心电图如有缺血症状需冠脉造影,无狭窄的巨大冠状动脉瘤,药物治疗长期口服小剂量阿司匹林+华法林或低分子肝素体育活动避免接触性/高负荷运动其它运动基于负荷试验/心肌灌注结果随访每年2次复查心脏超声及心电图每年1次负荷试验/心肌灌注显像侵入性检查6-12月时冠脉造影，根据临床需要随时复查,伴狭窄的冠状动脉瘤,药物治疗长期口服小剂量阿司匹林+华法林或低分子肝素受体阻滞剂减低心肌氧耗体育活动禁止接触性或高负荷运动其它
10、运动基于负荷试验/心肌灌注结果随访每年2次复查心脏超声及心电图每年1次负荷试验/心肌灌注显像侵入性检查常规冠脉造影，症状变化随时复查,Coronary arteries aneurysms & ectasia,冠状动脉局部或弥漫的扩张直径超过正常参考血管的1.5倍,Fusiform & Saccular,冠状动脉瘤/扩张的形态学分型,鉴别诊断,川崎病： 病变多局限，管壁增厚，前后血管光滑正常动脉粥样硬化遗传性家族性高胆固醇血症结节性多动脉炎大动脉炎其它结缔组织病：狼疮、白塞、马凡感染：败血症、梅毒、真菌感染外伤或冠脉介入相关损伤,鉴别诊断,鉴别诊断,Coronary artery athero
11、sclerosis,55 years old , hypertensive, obese female, with infero-posterior and right STEMI.,Coronary fistula,An 11-year-old boy with a CT demonstrated giant left main coronary artery (15 mm) due to a congenital fistula with the right atrium. Reformatted image demonstrates the entire course of the fi
12、stula . The patient underwent surgical closure of the fistula and remains asymptomatic.,ALCAPA syndrome,A 25-year-old woman with chest pain who had positive findings from a nuclear medicine stress test. CT clearly shows diffuse coronary ectasia as a compensatory response that is due to an anomalous
13、origin of the left main coronary artery from the pulmonary artery (ALCAPA）,Heterozygous Familial Hypercholesterolemia,Coronary angiograms from a 43-year-old man with familial hypercholesterolemia (A) and a 37-year-old man with familial hypercholesterolemia (B) in the left anterior oblique projection
14、s, showing localized ectasia of the right coronary artery. Arrows indicate ectatic regions.,Polyarteritis Nodosa,This 25-year-old woman had been given a diagnosis of polyarteritis nodosa 3 years earlier and was receiving prednisolone maintenance therapy when she presented with cardiac arrest. Althou
15、gh severe cardiac involvement in polyarteritis nodosa is unusual, it can result in myocardial infarction and confers a poorer prognosis. Despite treatment, the patient died a few months later,Takayasu Disease,A 30 year old women was admitted for syncope. Blood pressure was 100/70 mm Hg in the left a
16、rm, while not recordable in the right. There was a blowing diastolic murmur over the left sternal border. Total occlusion of the right subclavian artery, narrowing of the proximal left subclavian artery, and stenosis of the supra renal aorta consistent with a diagnosis of TA. Coronary angiogram show
17、ed a large aneurysm of the LAD.,Systemic lupus erythematosus,A 29-year-old woman with active SLE sustained an anterior MI and angiographic evidence of multiple, diffuse coronary aneurysms.,Behests Syndrome,A 28 yrs old male patient presented with chest pain. There was history of pain in bilateral MCP, PIP joints on and off. He had penile and recurrent oral ulcers during the past 1 year, positive for HLA B27. Coronary angiogram and CT showing the aneurysm involving the mid LAD.,谢谢,
