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一例表现为咽后累及的不完全和非典型川崎病。

A Case of Incomplete and Atypical Kawasaki Disease Presenting with Retropharyngeal Involvement.

机构信息

Department of Surgical and Biomedical Sciences, University of Perugia, 06132 Perugia, Italy.

Department of Medical and Surgical Sciences, University of Parma, 43126 Parma, Italy.

出版信息

Int J Environ Res Public Health. 2019 Sep 5;16(18):3262. doi: 10.3390/ijerph16183262.

DOI:10.3390/ijerph16183262
PMID:31491922
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6765912/
Abstract

Kawasaki disease (KD) is a childhood acute febrile vasculitis of unknown aetiology. The diagnosis is based on clinical criteria, including unilateral cervical lymphadenopathy, which is the only presenting symptom associated with fever in 12% of cases. A prompt differential diagnosis distinguishing KD from infective lymphadenitis is therefore necessary to avoid incorrect and delayed diagnosis and the risk of cardiovascular sequelae. We describe the case of a 4 years old boy presenting with febrile right cervical lymphadenopathy, in which the unresponsiveness to broad-spectrum antibiotics, the following onset of other characteristic clinical features and the evidence on the magnetic resonance imaging (MRI) of retropharyngeal inflammation led to the diagnosis of incomplete and atypical KD. On day 8 of hospitalisation (i.e., 13 days after the onset of symptoms), one dose of intravenous immunoglobulins (IVIG; 2 g/kg) was administered with rapid defervescence, and acetylsalicylic acid (4 mg/kg/day) was started and continued at home for a total of 8 weeks. Laboratory examinations revealed a reduction in the white blood cell count and the levels of inflammatory markers, thrombocytosis, and persistently negative echocardiography. Clinically, we observed a gradual reduction of the right-side neck swelling. Fifteen days after discharge, the MRI of the neck showed a regression of the laterocervical lymphadenopathy and a resolution of the infiltration of the parapharyngeal and retropharyngeal spaces. Head and neck manifestations can be early presentations of KD, which is frequently misdiagnosed as suppurative lymphadenitis or retropharyngeal infection. A growing awareness of the several possible presentations of KD is therefore necessary. Computed tomography (CT) or MRI can be utilised to facilitate the diagnosis.

摘要

川崎病(KD)是一种病因不明的儿童急性发热性血管炎。该诊断基于临床标准,包括单侧颈淋巴结肿大,这是 12%发热病例的唯一表现症状。因此,需要及时做出鉴别诊断,将 KD 与感染性淋巴结炎区分开来,以避免误诊和延误诊断,并降低心血管并发症的风险。

我们描述了一例 4 岁男孩,表现为右侧颈淋巴结发热,广谱抗生素治疗无反应,随后出现其他特征性临床症状,以及颈后咽部炎症的磁共振成像(MRI)证据,导致不完全和非典型 KD 的诊断。在住院第 8 天(即症状出现后第 13 天),给予单剂量静脉注射免疫球蛋白(IVIG;2 g/kg),迅速退热,开始在家中服用乙酰水杨酸(4 mg/kg/天),共 8 周。实验室检查显示白细胞计数和炎症标志物水平降低、血小板增多,以及持续阴性的超声心动图。临床观察到右侧颈部肿胀逐渐减轻。出院后第 15 天,颈部 MRI 显示颈侧淋巴结病消退,咽旁和咽后间隙浸润减轻。

头颈部表现可作为 KD 的早期表现,常被误诊为化脓性淋巴结炎或咽后感染。因此,需要提高对 KD 多种可能表现形式的认识。CT 或 MRI 可用于辅助诊断。

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