Suppr超能文献

[成年患者的川崎病]

[Kawasaki disease in adult patients].

作者信息

Sève P, Lega J-C

机构信息

Service de médecine interne, hospices civils de Lyon, Hôtel-Dieu, 1 place de l'Hôpital, Lyon cedex 02, France.

出版信息

Rev Med Interne. 2011 Jan;32(1):17-25. doi: 10.1016/j.revmed.2010.04.006.

Abstract

Kawasaki disease (KD) is a multisystemic vasculitis affecting mainly the skin, mucosa, and lymph nodes. Coronary artery aneurysms occur in 25% of patients but their prevalence is reduced to 4% in those patients treated with intravenous immunoglobulin (IVIg) within 10 days of illness onset. Interesting data recently published relate to physiopathology and diagnosis of the disease. Investigations identified an antigen-driven IgA oligoclonal response directed against cytoplasmic inclusions in KD tissues. An algorithm using laboratory tests and echocardiography has been recently proposed to improve early detection of incomplete KD. Although KD predominantly affects children, it may be also of interest for adult physicians. First, patients may develop long-term cardiovascular event. Coronary artery aneurysms may lead to the development of coronary stenosis or thrombosis. Despite the absence of coronary lesions during the acute phase of the disease, patients may present morphological and functional sequelae of coronary and peripheral arteries at convalescent phase. These potential arterial sequelae require long-term follow-up and treatment of associated cardiovascular risk factors. Although the level of injury seems to be correlated with the severity of initial coronary lesions, long-term course of vascular injuries is poorly known. Second, KD may occur in adults with 91 cases reported in the literature. Twenty-one cases have been reported in HIV infected patients. Intravenous immunoglobulins appear to shorten the disease course. Recent studies highlight the existence of incomplete KD and symptomatic coronary aneurysms in adults. Overall, these data suggest that adult patients with biological or echocardiographic features suggestive of incomplete KD should receive prompt IVIg to prevent coronary artery sequelae.

摘要

川崎病(KD)是一种主要影响皮肤、黏膜和淋巴结的多系统血管炎。25%的患者会出现冠状动脉瘤,但在发病10天内接受静脉注射免疫球蛋白(IVIg)治疗的患者中,其患病率降至4%。最近发表的有趣数据与该疾病的病理生理学和诊断有关。研究发现了一种抗原驱动的针对KD组织中细胞质包涵体的IgA寡克隆反应。最近有人提出了一种使用实验室检查和超声心动图的算法,以改善不完全KD的早期检测。虽然KD主要影响儿童,但成年医生也可能对此感兴趣。首先,患者可能会发生长期心血管事件。冠状动脉瘤可能导致冠状动脉狭窄或血栓形成。尽管在疾病急性期没有冠状动脉病变,但患者在恢复期可能会出现冠状动脉和外周动脉的形态和功能后遗症。这些潜在的动脉后遗症需要长期随访以及对相关心血管危险因素进行治疗。虽然损伤程度似乎与初始冠状动脉病变的严重程度相关,但血管损伤的长期病程却鲜为人知。其次,KD也可能发生在成年人中,文献报道了91例。在HIV感染患者中报告了21例。静脉注射免疫球蛋白似乎可以缩短病程。最近的研究强调了成年人中存在不完全KD和有症状的冠状动脉瘤。总体而言,这些数据表明,具有提示不完全KD的生物学或超声心动图特征的成年患者应及时接受IVIg治疗,以预防冠状动脉后遗症。

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验