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土耳其儿童川崎病:单中心经验,重点关注静脉注射免疫球蛋白抵抗和巨大冠状动脉瘤

Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms.

作者信息

Yılmazer Murat Muhtar, Özdemir Rahmi, Meşe Timur, Küçük Mehmet, Öner Taliha, Devrim İlker, Bayram Nuri, Güven Barış, Tavlı Vedide

机构信息

Departments of Pediatric Cardiology, Dr. Behcet Uz Children's Hospital, University of Health Sciences, Izmir, Turkey.

Departments of Pediatric Infectious Diseases, Dr. Behcet Uz Children's Hospital, University of Health Sciences, Izmir, Turkey.

出版信息

Turk J Pediatr. 2019;61(5):648-656. doi: 10.24953/turkjped.2019.05.002.

Abstract

Yılmazer MM, Özdemir R, Meşe T, Küçük M, Öner T, Devrim İ, Bayram N, Güven B, Tavlı V. Kawasaki disease in Turkish children: a single center experience with emphasis on intravenous immunoglobulin resistance and giant coronary aneurysms. Turk J Pediatr 2019; 61: 648-656. Prompt diagnosis and the administration of intravenous immunoglobulin (IVIG) has reduced the incidence of coronary artery abnormalities (CAA) in Kawasaki Disease (KD). The resistance to treatment and development of the coronary sequelae remain the most important problems in KD. We aimed to determine the predicting factors of nonresponse to initial IVIG therapy and to analyze the cases who had giant coronary aneurysms. A total of 120 KD cases, including 61 children fulfilling the criteria for KD and 59 with incomplete KD were enrolled into this study. Demographic, laboratory, clinical, echocardiographic characteristics, and treatment regimens were reviewed, retrospectively. The median age of the patients was 33.5 months (range: 3-168 months). Coronary artery aneurysms were detected in 35 patients (29%) at the time of diagnosis. Twenty-eight patients had coronary aneurysms small or medium in size, one had a large, and seven had giant coronary aneurysms. CAA persisted in 8 cases in the follow-up, all of which were large or giant aneurysms. A ten month-old girl with a giant coronary aneurysm was referred to coronary bypass surgery in the subacute phase of follow-up, due to myocardial ischemia. Eighteen patients were unresponsive to the initial IVIG therapy (%15), of whom 10 were diagnosed as cKD and 8 were iKD. Patients who did not respond to initial IVIG therapy, had higher white blood cell (WBC) count, higher C-reactive protein (CRP) and lower albumin levels than those who did (P < 0.05). In univarite analysis; CRP, WBC and albumin were found to be significant predictors of nonresponse to initial IVIG therapy, while a stepwise multiple linear regression analysis showed that WBC count and albumin levels were significantly correlated with nonresponse to initial treatment with IVIG. Our study showed that WBC count and albumin levels might be used as predictors of nonresponse to the IVIG therapy in Turkish children with KD.

摘要

伊尔马泽尔·MM、厄兹德米尔·R、梅塞·T、库库克·M、厄纳·T、德夫林·İ、巴伊拉姆·N、居文·B、塔夫利·V。土耳其儿童川崎病:单中心经验,重点关注静脉注射免疫球蛋白抵抗和巨大冠状动脉瘤。《土耳其儿科学杂志》2019年;61: 648 - 656。及时诊断和静脉注射免疫球蛋白(IVIG)的应用降低了川崎病(KD)中冠状动脉异常(CAA)的发生率。治疗抵抗和冠状动脉后遗症的发生仍然是KD中最重要的问题。我们旨在确定初始IVIG治疗无反应的预测因素,并分析患有巨大冠状动脉瘤的病例。本研究共纳入120例KD病例,其中61例儿童符合KD标准,59例为不完全KD。回顾性分析了人口统计学、实验室、临床、超声心动图特征及治疗方案。患者的中位年龄为33.5个月(范围:3 - 168个月)。诊断时35例患者(29%)检测到冠状动脉瘤。28例患者有小或中等大小的冠状动脉瘤,1例有大的冠状动脉瘤,7例有巨大冠状动脉瘤。随访中有8例CAA持续存在,均为大或巨大动脉瘤。一名患有巨大冠状动脉瘤的10个月大女孩在随访亚急性期因心肌缺血接受了冠状动脉搭桥手术。18例患者对初始IVIG治疗无反应(15%),其中10例诊断为cKD,8例为iKD。对初始IVIG治疗无反应的患者白细胞(WBC)计数、C反应蛋白(CRP)水平较高,白蛋白水平低于有反应的患者(P < 0.05)。单因素分析中;CRP、WBC和白蛋白被发现是初始IVIG治疗无反应的显著预测因素,而逐步多元线性回归分析表明WBC计数和白蛋白水平与初始IVIG治疗无反应显著相关。我们的研究表明,WBC计数和白蛋白水平可作为土耳其KD儿童IVIG治疗无反应的预测指标。

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