Binder Elisabeth, Griesmaier Elke, Giner Thomas, Sailer-Höck Michaela, Brunner Juergen
Clinic of Pediatrics I, Department of Pediatrics, Medical University Innsbruck, Anichstrasse 35, 6020, Innsbruck, Austria.
Clinic of Pediatrics II, Department of Pediatrics, Medical University Innsbruck, Anichstrasse 35, 6020, Innsbruck, Austria.
Pediatr Rheumatol Online J. 2014 Sep 2;12(1):37. doi: 10.1186/1546-0096-12-37.
Kawasaki disease (KD) is a rare vasculitis seen predominantly in children. In developing countries, it is the leading cause of childhood-acquired heart disease. Besides a case report from 1981 there have been no data published dealing with the epidemiology and clinical aspects of KD in Austria.
The purpose of the present study was to investigate the clinical spectrum of KD in a geographically determined cohort of infants, children, and adolescents that were diagnosed and treated at the University Hospital of Innsbruck from 2003-2012.
Thirty-two patients were included in the study with a median age of 32.96 months (2-192). 59.4% of the patients were aged between six months and four years. The male-to-female ratio was 1:1.13. Clinical examination revealed non-purulent conjunctivitis and exanthema as the most common symptoms (84.4%). 75% showed oropharyngeal changes, 21.9% had gastrointestinal complaints such as diarrhoe, stomachache or vomiting prior to diagnosis. One third of the patients were admitted with a preliminary diagnosis, whereas 78.1% were pre-treated with antibiotics. The median fever duration at the time of presentation was estimated with 4.96 days (1-14), at time of diagnosis 6.76 days (3-15).75% were diagnosed with complete KD, and 25% with an incomplete form of the disease. There was no significant difference in the duration of fever neither between complete and incomplete KD, nor between the different age groups. Typical laboratory findings included increased C-reactive protein (CRP) (80.6%) and erythrocyte sedimentation rate (ESR) (96%),leukocytosis (48.4%) and thrombocytosis (40.6%) without any significant quantitative difference between complete and incomplete KD. Coronary complications could be observed in six patients: one with a coronary aneurysm and five with tubular dilatation of the coronary arteries. Our patient cohort represents the age distribution as described in literature and emphasizes that KD could affect persons of any age. The frequency of occurrence of the clinical symptoms differs from previous reports - in our study, we predominantly observed non-purulent conjunctivitis and exanthema.
KD should always be considered as a differential diagnosis in a child with fever of unknown origin, as treatment can significantly decrease the frequency of coronary complications.
川崎病（KD）是一种罕见的血管炎，主要见于儿童。在发展中国家，它是儿童后天性心脏病的主要病因。除了1981年的一篇病例报告外，奥地利尚未发表有关KD流行病学和临床方面的数据。
本研究的目的是调查2003年至2012年在因斯布鲁克大学医院诊断和治疗的一个地理区域确定的婴儿、儿童和青少年队列中KD的临床谱。
32例患者纳入研究，中位年龄为32.96个月（2至192个月）。59.4%的患者年龄在6个月至4岁之间。男女比例为1:1.13。临床检查显示非脓性结膜炎和皮疹是最常见的症状（84.4%）。75%有口咽改变，21.9%在诊断前有胃肠道症状，如腹泻、腹痛或呕吐。三分之一的患者以初步诊断入院，而78.1%曾接受抗生素预处理。就诊时发热的中位持续时间估计为4.96天（1至14天），诊断时为6.76天（3至15天）。75%被诊断为完全性KD，25%为不完全性KD。发热持续时间在完全性和不完全性KD之间以及不同年龄组之间均无显著差异。典型的实验室检查结果包括C反应蛋白（CRP）升高（80.6%）、红细胞沉降率（ESR）升高（96%）、白细胞增多（48.4%）和血小板增多（40.6%），完全性和不完全性KD之间在任何定量方面均无显著差异。6例患者观察到冠状动脉并发症：1例有冠状动脉瘤，5例有冠状动脉管状扩张。我们的患者队列代表了文献中描述的年龄分布，并强调KD可影响任何年龄的人。临床症状的发生频率与先前报告不同——在我们的研究中，我们主要观察到非脓性结膜炎和皮疹。
对于不明原因发热的儿童，应始终将KD视为鉴别诊断，因为治疗可显著降低冠状动脉并发症的发生率。
