背景:川崎病(KD)是一种急性全身性免疫性血管炎,影响儿童的多个器官和系统,并且在5岁以下的儿童中普遍存在。肌无力是KD的一种罕见表现,仅有11例KD合并肌无力的儿科患者被报道,其中三分之二的患者发现了肌炎的证据,1/3不能用肌炎来解释,其机制尚不清楚。KD合并膀胱潴留的病例更为罕见,仅有1例报告KD合并膀胱潴留的儿童既往无基础疾病。
方法:我们报告了一名22个月大的亚洲儿童,患有不完全性川崎病(IKD),最初表现为发烧和下肢进行性肌无力,其次是膀胱和肠潴留异常和心力衰竭的快速发作,呼吸衰竭和休克。患儿出现冠状动脉扩张症(CAA),但没有出现皮疹等KD的主要临床特征,结膜充血,末端的剥脱,口面部改变和颈部淋巴结肿大。肌酸激酶和肌电图正常。静脉注射免疫球蛋白后,温度逐渐恢复正常,肌力略有恢复。阿司匹林联合类固醇治疗1周后,可以帮助孩子走路。
结论:我们介绍了一个22个月大的儿童IKD的病例。孩子从四肢进行性肌肉无力开始,其次是膀胱和肠潴留异常,并迅速发展为心力衰竭,呼吸衰竭,和震惊。尽管早期未能发现这种疾病,患儿恢复迅速,预后良好.以肌肉无力为主要表现的KD合并症并不常见。这是首例报告的IKD同时伴有肌无力和膀胱和肠潴留,这可以为临床医生提供诊断和治疗思路,以及未来探索KD合并肌无力或膀胱和肠潴留异常的机制的基础。
BACKGROUND: Kawasaki disease (KD) is an acute systemic immune vasculitis affecting multiple organs and systems in children, and is prevalent in children under 5 years of age. Muscular weakness is a rare manifestation of KD, and only 11 pediatric patients with KD combined with muscular weakness have been reported, of which evidence of myositis was found in 2/3 of the patients, and 1/3 could not be explained by myositis, the mechanism of which is still unclear. Cases of KD combined with bladder retention are even more rare, and there has been only 1
case report of KD combined with bladder retention in a child with no previous underlying disease.
METHODS: We report a 22-month-old Asian child with incomplete Kawasaki disease (IKD) who initially presented with fever and progressive muscular weakness in the lower extremities, followed by the bladder and bowel retention abnormalities and rapid onset of heart failure, respiratory failure and shock. The child developed coronary artery ectasia (CAA) without the main clinical features of KD such as rash, conjunctival congestion, desquamation of the extremity endings, orofacial changes and enlarged lymph nodes in the neck. Creatine kinase and electromyography were normal. Temperature gradually normalized and muscle strength recovered slightly after intravenous immunoglobulin. The child could be helped to walk after 1 week of aspirin combined with steroid therapy.
CONCLUSIONS: We present the
case of a 22-month-old child with IKD. The child began with progressive muscular weakness in the extremities, followed by the bladder and bowel retention abnormalities, and rapidly developed heart failure, respiratory failure, and shock. Despite early failure to detect the disease, the child recovered rapidly and had a favorable prognosis. KD comorbidities with muscular weakness as the main manifestation are uncommon. This is the first
case report of IKD combined with both muscular weakness and bladder and bowel retention, which may provide clinicians with diagnostic and therapeutic ideas, as well as a basis for future exploration of the mechanisms of KD combined with muscular weakness or bladder and bowel retention abnormalities.