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不完全川崎病的非典型表现:周围性面神经麻痹

Atypical Presentation of Incomplete Kawasaki Disease: A Peripheral Facial Nerve Palsy.

作者信息

Rodriguez-Gonzalez Moises, Castellano-Martinez Ana, Perez-Reviriego Alvaro A

机构信息

Pediatric Cardiology Department, Hospital Universitario Puerta del Mar, Cadiz, Spain.

Pediatric Department of Hospital Universitario Puerta del Mar, Cadiz, Spain.

出版信息

J Emerg Med. 2018 Jul;55(1):118-120. doi: 10.1016/j.jemermed.2018.04.013. Epub 2018 May 3.

Abstract

BACKGROUND

Kawasaki disease (KD) is a multisystem vasculitic disease. Coronary artery aneurysms (CAAs) are the most important and life-threatening complication of KD. Various neurologic complications have been described to occur in 1-30% of patients with KD, but peripheral facial nerve palsy (FNP) is rare (0.9%).

CASE REPORT

We describe a 5-month-old male infant who presented to us with unilateral left infranuclear FNP in the convalescent phase (day 18 of illness) of incomplete KD. The initial diagnosis was not made during the first 10 days of illness (therapeutic window for immunoglobulin treatment) as he was suspected to have hand-mouth-foot disease. We believe that both the delay in diagnosis and treatment of an atypical presentation of KD, combined with the more severe vasculitis and inflammatory burden reported in these cases, contributed to the development of CAA in our patient. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the importance of considering KD diagnosis in children with prolonged unexplained fever, even with incomplete diagnostic features, as well as the need to be aware of unusual manifestations, such as FNP. Atypical cases like this may be at increased risk of CAA because of delayed diagnosis and a higher inflammatory burden; therefore, a more aggressive treatment approach may be necessary.

摘要

背景

川崎病(KD)是一种多系统血管炎性疾病。冠状动脉瘤(CAA)是KD最重要且危及生命的并发症。已有报道称,1%至30%的KD患者会出现各种神经系统并发症,但周围性面神经麻痹(FNP)较为罕见(0.9%)。

病例报告

我们描述了一名5个月大的男婴,他在不完全KD的恢复期(发病第18天)出现左侧核下性单侧FNP。在发病的前10天(免疫球蛋白治疗的治疗窗)未做出初步诊断,因为他被怀疑患有手足口病。我们认为,KD非典型表现的诊断和治疗延迟,再加上这些病例中报道的更严重的血管炎和炎症负担,导致了我们患者CAA的发生。

急诊医生为何应知晓此事?:本病例强调了在不明原因长期发热的儿童中考虑KD诊断的重要性,即使诊断特征不完全,同时也需要意识到FNP等不寻常表现。像这样的非典型病例可能因诊断延迟和更高的炎症负担而增加CAA风险;因此,可能需要采取更积极的治疗方法。

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