川崎病的诊断和治疗

川崎病的诊断和治疗梁翊常川崎病（ＫＤ）是一种急性血管炎。婴幼儿发病多。其特征为发热、粘膜炎、皮疹、颈淋巴结肿大和肢端改变。本病心血管受累最著，早期包括全心炎，冠状动脉炎及动脉瘤，后期表现为心肌梗塞及缺血性心脏病。１９６７年日本川崎医师首次报道本病，称...     

静脉注射免疫球蛋白治疗川崎病用法探讨

自Newburger等报道静注丙种球蛋白(IVIG)对小儿川畸病的治疗效果与剂量和用法有关以来，国内外围绕小儿川崎病静注丙种球蛋白优选方法的研究尚未取得一致的结论，我们对应用典型的IVIG常规剂量多日静注或单日大剂量静注的小儿川畸病进行临床疗效和不良反应观察，现将结果报告如下。     

静脉注射免疫球蛋白治疗川崎病的临床观察

静脉注射免疫球蛋白治疗川崎病的临床观察孙涛李永柏刘淑英陈维金涂文伟杨红育聂荣国谢依群尹秀兰杨锡强川崎病（ＫＤ）是一种原因不明的小儿急性发热性疾病。以前采用阿司匹林（ＡＳＰ）为主的抗凝、抗炎和对症治疗。近年国外报道静脉注射免疫球蛋白（ＩＶＩＧ）可迅速控...     

川崎病的早期诊断

川崎病的早期诊断郑州铁路局中心医院儿科（４５００５２）张梅梅本文总结了我院自１９８９年７月－１９９６年２月间收治的川崎病１４例，对其临床经过及主要特点加以分析，以期达到提高早期诊断丰的目的。临床资料一、一般情况１４例中，男１１例，女８例，男性明显高于...     

川崎病致心脏瓣膜病变的诊断及治疗

川崎病 (ＫＤ)是一种原因不明的全身性血管炎 ,主要影响婴幼儿 ,冠状动脉瘤 (ＣＡＡ)为其最严重的并发症 ,急性期ＣＡＡ的发生率约 2 0 %。心脏瓣膜病变为ＫＤ另一重要并发症 ,可导致充血性心力衰竭 ,甚至死亡。在日本、美国等国家 ,随着风湿热发病率进一步降低 ,ＫＤ已成为儿童后天性心脏瓣膜病变的一个重要原因[1,2 ] 。 1973年Ｈａｍａａｄａ等首先报道 3例ＫＤ患儿冠状动脉造影 (ＣＡＧ)时发现冠状动脉 (简称冠脉 )病变并二尖瓣返流 (ＭＲ)。此后 ,ＫＤ致心脏瓣膜病变受到广泛关注。Ａｋａｇｉ等[1] 对 12 15例ＫＤ患儿从急性期开始随访…     

川崎病延迟诊断的危险因素分析

背景 目前对川崎病(KD)尤其是不典型KD的及时诊断仍面临困难.目的 分析KD患儿延迟诊断的影响因素.设计病例对照研究.方法 采用日本川崎病研究委员会2005年发布的第5版KD诊断标准,6条主要诊断标准中满足≥5条即可诊断KD.纳入2009年1月至2014年12月在浙江大学医学院附属儿童医院住院的KD患儿,发病10 d...     

静脉注射丙种球蛋白无反应型川崎病治疗进展

川崎病(KD)是一种病因未明、以全身血管炎性病变为主要病理改变的儿童急性发热性疾病。KD常累及冠状动脉,若未及时治疗,患儿可发生冠脉损伤(CAL)。目前急性期KD标准治疗方案为静脉用丙种球蛋白(IVIG)联合口服阿司匹林,但仍有10%~20%的患儿对IVIG无反应,并且这部分患儿发生CAL的风险增高。文献报道有多种IVIG无反应型KD的治疗方法,如二次IVIG治疗、糖皮质激素、英夫利昔单抗、免疫抑制剂和血浆置换等,然而具体治疗方案仍无定论。文章综述IVIG无反应型KD的治疗。     

川崎病的诊断治疗现状

川崎病(KD)目前已成为最常见的小儿后天性心脏病之一.尽管近年来取得了诸多进展,但仍有众多悬而未决的问题有待于探讨.本病病因末明,目前趋向于感染、遗传易感及细菌超抗原介导学说.诊断标准几经修订,目前多沿用日本2002年修订的第5版诊断标准.关于不完全性KD的诊断、超声心动图对冠状动脉病变的诊断标准也做了阐述.治疗目的是控制全身血管炎性反应,防止冠状动脉瘤形成及血栓性阻塞.阿司匹林仍为本病首选药物,静脉注射丙种球蛋白国际上推荐单剂量为2 g/kg,国内有报道单剂量1 g/kg也可达到同样效果.关于糖皮质激素治疗,目前尚有不同看法,虽不是治疗禁忌,但也不是首选用药.就KD的预后、随访及展望也做了阐述.     

静脉注射免疫球蛋白治疗川崎病的机制

目的观察静脉注射免疫球蛋白（IVIG）对川崎病（KD）患儿外用血淋巴细胞凋亡的影响，进一步探讨IVIG的治疗机制。方法观察26例KD患儿IVIG治疗前后外周血单个核细胞（PBMC）经anti-CD3单抗刺激培养不同时间后计数凋亡细胞百分率和DNA梯度分析及临床症状改善。结果KD患儿PBMC体外培养凋亡细胞百分率较正常对照低，DNA梯度出现延迟；IVIG治疗后，PBMC凋亡细胞百分率提高（P＜0．001），DNA梯度出现时间提前，与单用阿司匹林组比较，有显著差异（P＜0．005），临床症状明显改善。结论IVIG治疗KD的机制可能部分归于逆转被抑制的淋巴细胞凋亡。     

川崎病诊治研究进展

2003(01)

Zaitsu M.Hamasaki Y.Tashiro K Ulinastalin,an elastase inhibitor,inhibits the increased mRNA expression of prostaglandin H2 synthase-type2 in Kawasaki disease 2000(03)

Williams RV.Wilke VM.Tani LY Does abciximab enhance regression of coronary aneurysms resulting from Kawasaki disease 2002(01)

Wallace CA.French JW.Kahn SJ Initial intravenous gammaglobulin treatment failure in Kawasaki disease 2000(06)

Sundel RP.Baker AL.Fulton DR Corticosteroids in the initial treatment of Kawasaki disease:report of a randomized treal 2003(06)

Hashino K.Ishii M.Iemura M Re-treatment for immune globulinresistant Kawasaki disease:a comparative study of additional immune globulin and steroid pulse therapy 2001(03)

小林徹.井上佳也.森川昭廣 γ-グロブリン不応例の予測因子とステロイド治療 2006

Coskun KO,C.Coskun ST.El Arousy M Pediatric Patients with Kawasaki disease and a case report of Kitamura operation 2006(06)

>>更多...     

静脉注射丙种球蛋白无反应型川崎病的诊治进展

川崎病(Kawasaki disease,KD)是一种病因未明的以全身性血管炎为主要病变的儿童急性发热出疹性疾病,大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,IVIG)对改善临床症状、抑制冠状动脉损害(coronary artery lesions,CAL)的发生发展起着重要作用.目前标准化治疗已广泛应用,但仍有10%-15%的病例对IVIG无反应[1],且这部分患儿并发CAL的风险升高[2].因此,早期识别及有效治疗此类无反应型病例显得十分重要.     

川崎病临床诊治的新观念

川崎病导致的心血管并发症目前已经成为儿童获得性心脏病的主要病因。尽管川崎病的诊治研究已经历了数十年,其具体病因仍不完全明确。部分川崎病患者对丙种球蛋白治疗没有反应,激素的应用目前仍然存在争议。临床中一些患者可出现严重的全身症状,甚至死亡。冠状动脉病变严重程度与预后判断并非易事。该文对近年来川崎病诊治中提出的新观点重点阐述。     

Kawasaki disease: an update on diagnosis and treatment

Kawasaki disease (KD) is an acute multi-system vasculitis syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age. In developed countries, it is the leading cause of acquired heart disease in children. However, KD remains a mysterious disease. Some viruses potentially causing the condition have been isolated, but the results have not been able to be reproduced. This article reviews and summarizes different aspects of KD and provides updated information on diagnosis and treatment. The supplementary criteria for incomplete presentation of KD patients suggested by the American Heart Association, treatment (including tumor necrosis factor-alpha antagonist, methylprednisolone pulse therapy, statins, plasma exchange, and cytotoxic agents) for those with intravenous immunoglobulin treatment failure, and other experiences are also included in this review.     

不完全川崎病的诊治现状

川崎病（Kawasaki disease,KD）是一种急性、自限性且病因不明的血管炎,最常见于婴幼儿。目前已经成为最常见的小儿获得性心脏病。该病于1967年由日本学者Kawasaki Tomisaku博士首先描述。以后在全世界各地及各种族均有报道。随着人们对该病认识的不断深入,一些表现不典型或不完全符合川崎病诊断标准的病例在国内外的报道越来越多。日本晚近的研究表明,不完全KD占KD患儿的13．8％。国内一项研究发现,19．4％的KD患儿的表现不典型或不完全（atypical or incomplete）。因此,了解国内外对不完全KD诊断及治疗的新进展具有重要意义。     

关于川崎病诊疗问题的答复

崔兴杰医师（453731河南省新乡县人民医院儿科）问：1．怎样才能早期诊断不完全性川崎病？2．治疗川崎病能用糖皮质激素吗？3．如果川崎病对IVIG治疗无反应时,应如何处理？     

Issues in the diagnosis of Kawasaki disease

The diagnosis of Kawasaki disease (KD) is based on clinical features and supporting laboratory and echocardiographic findings. Diagnosis is often difficult as the etiology remains unknown and no specific diagnostic assay exists, and many of the clinical features mimic common childhood viral illnesses. Incomplete (or atypical) KD is being recognized more frequently, and diagnosis may be difficult because fewer clinical features are present; however, diagnosis remains essential as these patients develop coronary artery sequelae at least as frequently as children with classic KD. Infants are at particular risk for coronary artery sequelae and often have incomplete KD features requiring astute clinical acumen. Revised guidelines for the diagnosis of incomplete KD are being developed, but until a diagnostic assay is available, a low threshold for theraphy is required to avoid potentially devastating sequelae.     

Difficulties in the diagnosis of Kawasaki disease

This study aims to highlight the difficulties faced in the clinical diagnosis of Kawasaki Disease (KD) presenting beyond the first week. This is a retrospective study of 25 cases of which only 36% met the criteria for classical and 8% was incomplete KD. Majority (56%) did not meet the criteria for classical KD; at the same time they were not incomplete / atypical cases. Difficulties arise in diagnosis of the cases presenting in the second week, as by that time many of the classical findings disappear or probably have not been present at all. In this scenario high index of suspicion for KD in a child presenting with fever, looking not that sick (Non toxic look) with bulbar conjunctivitis and oral mucositis helped us to reach the diagnosis. We incidentally observed Hyperemia of the Upper Eyelids in 32% of our cases, which might assist in the diagnosis.