Flinn A M, Gavin P J, McMahon C J, Oslizok P, Butler K M
Our Lady's Children's Hospital, Dublin, Ireland.
Health Intelligence Unit, Health and Wellbeing Directorate, HSE, Dublin 8.
Ir Med J. 2018 Feb 9;111(2):691.
Diagnosis of Kawasaki Disease (KD) can be challenging due to lack of a diagnostic test, and some children present with 'incomplete' KD when not all diagnostic criteria are met. Treatment with intravenous immunoglobulin (IVIG) and aspirin reduces the risk of coronary artery complications. There is sub-group of patients who are resistant to IVIG/aspirin therapy and are at increased risk of complications. Recent evidence suggests that additional treatment of this high-risk group with corticosteroids is beneficial in reducing this risk. We examine the treatment and coronary artery outcomes, by retrospective review of medical records, of a cohort of 32 paediatric patients with KD admitted to a single Irish tertiary centre from January 2010-December 2014. Twenty-eight percent of patients (9/32) had an incomplete diagnosis of KD; these patients received IVIG later compared to those with a complete KD diagnosis. 15/32 (47%) had abnormal echocardiogram findings in the acute phase, 8/32 (25%) had echocardiogram abnormalities at 6-week follow-up, and 4/32 (12.5%) had persisting abnormalities. This study highlights the potential for adverse outcome in KD, the difficulty in diagnosis in 'incomplete' cases, and the need to identify children at higher risk for adverse outcome where adjunctive therapies would be most beneficial.
由于缺乏诊断性检测，川崎病（KD）的诊断可能具有挑战性，并且当并非所有诊断标准都满足时，一些儿童会出现“不完全”KD。静脉注射免疫球蛋白（IVIG）和阿司匹林治疗可降低冠状动脉并发症的风险。有一部分患者对IVIG/阿司匹林治疗耐药，并发症风险增加。最近的证据表明，用皮质类固醇对这一高危组进行额外治疗有助于降低这种风险。我们通过回顾性查阅病历，研究了2010年1月至2014年12月入住爱尔兰一家单一三级中心的32例KD儿科患者的治疗及冠状动脉结局。28%的患者（9/32）KD诊断不完全；与完全诊断为KD的患者相比，这些患者接受IVIG的时间较晚。15/32（47%）在急性期超声心动图检查结果异常，8/32（25%）在6周随访时超声心动图有异常，4/32（12.5%）有持续性异常。本研究强调了KD出现不良结局的可能性、“不完全”病例诊断的困难，以及识别最能从辅助治疗中获益的不良结局高危儿童的必要性。
