Cheah Chooi San, Lee Wendy Wei Li, Suhaini Siti Aisyah, Azidin Abdullah Harith, Khoo Mohammad Shukri, Ismail Noor Akmal Shareela, Ali Adli
Department of Pediatric, Faculty of Medicine, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Department of Pediatric, Universiti Kebangsaan Malaysia Specialist Children's Hospital (HPKK), Kuala Lumpur, Malaysia.
Front Pediatr. 2023 Jan 13;10:1090928. doi: 10.3389/fped.2022.1090928. eCollection 2022.
Kawasaki disease (KD) is an acute idiopathic systemic vasculitis with a self- limiting course that predominantly affects children under 5 years old, particularly in the East Asian countries. Nevertheless, to date, the data on KD in Malaysia are limited. This study aimed to evaluate the epidemiology, clinical features, treatment, and outcomes of KD among the pediatric patients admitted to Hospital Canselor Tunku Muhriz (HCTM), Kuala Lumpur, Malaysia.
A retrospective cohort study of 66,500 pediatric patients presented at HCTM from the year 2004 to 2021 was conducted.
62 KD cases out of 66,500 pediatric admissions were reported, with a male-to-female ratio of 1.58 to 1. Majority of KD patients (95.0%) were younger than 5 years old. Prior infection was reported in 5 KD patients (8.1%). Apart from the classical features, manifestations of various organ systems including cardiovascular (16.1%), gastrointestinal (43.5%), neurological (1.61%), musculoskeletal (1.61%), and genitourinary (17.7%) systems were observed. There was a significant association between sterile pyuria and coronary artery aneurysm (CAA) ( < 0.05). Interestingly, abnormal liver parameters ( < 0.05) and incomplete KD ( < 0.05) were significantly related to IVIG resistance.
The presence of family history, immunological disorder, and previous infection in our KD patients suggested that there is a possibility of genetic, immunological, and infectious roles in the pathophysiology of KD. IVIG resistance is more likely to occur in KD patients with hepatic dysfunction or incomplete KD presentation. These findings highlighted the significant contribution of laboratory parameters to the prognosis of KD, prompting more in-depth research on the KD scoring systems and their relevance in this country.
川崎病(KD)是一种急性特发性全身性血管炎,病程呈自限性,主要影响5岁以下儿童,在东亚国家尤为常见。然而,迄今为止,马来西亚关于KD的数据有限。本研究旨在评估马来西亚吉隆坡敦库·穆赫里兹总理医院(HCTM)收治的儿科患者中KD的流行病学、临床特征、治疗及预后情况。
对2004年至2021年在HCTM就诊的66500名儿科患者进行回顾性队列研究。
在66500例儿科住院病例中报告了62例KD病例,男女比例为1.58比1。大多数KD患者(95.0%)年龄小于5岁。5例KD患者(8.1%)报告有先前感染史。除了典型特征外,还观察到包括心血管系统(16.1%)、胃肠道系统(43.5%)、神经系统(1.61%)、肌肉骨骼系统(1.61%)和泌尿生殖系统(17.7%)在内的各个器官系统的表现。无菌性脓尿与冠状动脉瘤(CAA)之间存在显著关联(<0.05)。有趣的是,肝功能异常(<0.05)和不完全性KD(<0.05)与静脉注射免疫球蛋白(IVIG)抵抗显著相关。
我们的KD患者中存在家族史、免疫紊乱和先前感染,这表明在KD的病理生理学中可能存在遗传、免疫和感染因素。肝功能不全或不完全性KD表现的KD患者更易发生IVIG抵抗。这些发现突出了实验室参数对KD预后的重要贡献,促使对该国的KD评分系统及其相关性进行更深入的研究。