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中华诊断学电子杂志 ›› 2024, Vol. 12 ›› Issue (02) : 95 -100. doi: 10.3877/cma.j.issn.2095-655X.2024.02.005

儿科疾病诊治

儿童川崎病休克综合征合并可逆性胼胝体压部病变综合征的诊断学特征并文献复习
轩欢欢1, 刘凤麟1, 李伟1, 李自普2, 贾宝俊1, 王金菊1, 满宜刚1,()   
  1. 1. 266034 北京大学人民医院青岛医院 青岛大学附属妇女儿童医院(城阳院区)儿童内科
    2. 266034 青岛大学附属妇女儿童医院重症医学中心
  • 收稿日期:2024-03-28 出版日期:2024-05-26
  • 通信作者: 满宜刚

Diagnostic features of Kawasaki disease shock syndrome complicated with reversible splenial lesion syndrome in children and literature review

Huanhuan Xuan1, Fenglin Liu1, Wei Li1, Zipu Li2, Baojun Jia1, Jinju Wang1, Yigang Man1,()   

  1. 1. Pediatric Ward, Qingdao Hospital of Peking University People′s Hospital, Women and Children′s Hospital, Qingdao University, Qingdao 266034, China
    2. Intensive Care Unit, Women and Children′s Hospital, Qingdao University, Qingdao 266034, China
  • Received:2024-03-28 Published:2024-05-26
  • Corresponding author: Yigang Man
引用本文:

轩欢欢, 刘凤麟, 李伟, 李自普, 贾宝俊, 王金菊, 满宜刚. 儿童川崎病休克综合征合并可逆性胼胝体压部病变综合征的诊断学特征并文献复习[J]. 中华诊断学电子杂志, 2024, 12(02): 95-100.

Huanhuan Xuan, Fenglin Liu, Wei Li, Zipu Li, Baojun Jia, Jinju Wang, Yigang Man. Diagnostic features of Kawasaki disease shock syndrome complicated with reversible splenial lesion syndrome in children and literature review[J]. Chinese Journal of Diagnostics(Electronic Edition), 2024, 12(02): 95-100.

目的

探讨川崎病休克综合征(KDSS)合并可逆性胼胝体压部病变综合征(RESLES)的诊断学特征。

方法

分析2022年5月25日青岛大学附属妇女儿童医院儿童内科收治的1例合并RESLES的KDSS患儿的临床资料,并复习文献,总结KDSS合并RESLES的诊断学特征。

结果

患儿男,11岁,头晕、发热2 d,谵妄1次。入院时血压正常,颈部淋巴结肿大,颈抵抗阳性。实验室检查C反应蛋白、血沉水平升高,血钠水平降低。入院后予抗感染、营养神经等治疗;第3天出现球结膜充血、皮疹、草莓舌、口唇红、手足硬肿等川崎病(KD)相关症状,头颅MRI示胼胝体压部稍低T1稍高T2信号,DWI呈明显高信号,提示RESLES;第4天出现低血压,同时超声示心脏左室壁整体运动幅度略减低、多浆膜腔积液,考虑KDSS,予多巴酚丁胺升血压、糖皮质激素抗炎及液体治疗后,休克纠正,KD相关症状10 d内渐消失;第14天头颅MRI示胼胝体压部异常信号影消失。检索中国知网、万方、维普、PubMed数据库建库至2023年12月31日收录的文献,共检索到14篇文献,其中外文期刊12篇,中文期刊2篇;共报道19例患儿,其中仅2例为国内病例,而明确报道出现低血压者共1例。

结论

KD合并RESLES儿童少见,临床症状多样且不典型,胼胝体损伤具有可逆性,积极治疗后预后良好。提高对本病的认识,有助于提高临床医师的诊断水平及给予患儿早期规范的治疗。

Objective

To explore diagnostic features of Kawasaki disease shock syndrome (KDSS) complicated with reversible splenial lesion syndrome (RESLES).

Methods

The clinical data of a pediatric patient with KDSS complicated with RESLES diagnosed in Women and Children′s Hospital, Qingdao University in May 25, 2022 were retrospectively analyzed. The diagnostic features of KDSS with RESLES were summarized and relevant literatures were reviewed.

Results

The 11-year-old boy was admitted to hospital because of dizziness, fever for 2 days and delirium once. On admission, his blood pressure was normal, but his cervical lymph nodes were enlarged, and he had positive neck resistance. In terms of laboratory tests, C-reactive protein level and erythrocyte sedimentation rate increased while serum sodium level decreased. Following admission, he was treated with anti-infection and nerve nourishment medications. On the third day, conjunctival congestion, rashes, bayberry tongue, red lip chapping, red swelling of hands and feet were appeared, which were associated with Kawasaki disease (KD). MRI of the head showed the splenium of the corpus callosum with slightly decreased T1 and slightly increased T2 signals in the pressure area, with high signal intensity on DWI. Based on these findings, he was diagnosed with RESLES. On the fourth day, hypotension was observed. Meanwhile, ultrasonography revealed that the motion of the left ventricular wall of the heart was slightly diminished, with multiple serosal effusions. KDSS was diagnosed and treated with dobutamine to raise blood pressure and glucocorticoid for anti-inflammatory, and fluid for resuscitation, after which shock was reversed and KD-related symptoms gradually faded within ten days. On the 14th day, cranial MRI revealed that the aberrant signal had disappeared. Literatures were searched from CNKI, Wanfang, VIP and PubMed databases until December 31, 2023, and a total of 14 literatures were retrieved, including 12 foreign literatures and 2 Chinese literatures. A total of 19 children were reported, of which 2 were Chinese. One was definitely reported to have hypotension.

Conclusions

KD complicated with RESLES is rare in children, the clinical symptoms are varied and atypical, the corpus callosum injury is reversible, and the prognosis is good after active treatment. Increasing awareness of this condition can improve physicians′ diagnostic abilities and provide early standardized treatment for the patient.

图1 川崎病休克综合征合并可逆性胼胝体压部病变综合征患儿治疗前后头颅MRI图像注:a~d图示治疗前胼胝体压部T1WI呈稍低信号(a)、T2WI呈稍高信号(b)、弥散加权成像序列(DWI)呈高信号(c、d) (箭头所示);e~h图示治疗后异常信号影消失
表1 20例川崎病合并RESLES患儿的临床资料
病例 发病年龄(岁) 性别 血压(mmHg) 神经系统症状 病程中首次头颅MRI检查时间(d) 病程中复查头颅MRI时间(d) 脑电图 脑脊液 血清钠离子值(mmol/L) 冠状动脉内径(mm) 治愈 参考文献
例1 14 50/37 头痛、幻视、意识障碍 第12天 第20天 NP NP 128 RCA 8 mm,LAD 5.1 mm [3]
例2 7 105/72 嗜睡、幻觉、性格改变 第3天 第10天 弥散慢波 NP 131 NP [4]
例3 2 NA 嗜睡、幻视 第10天 第17天 NP NP 134 NP [5]
例4 7 NA 嗜睡、幻视、言语及行为改变 第4天 第10天 弥散慢波 NP 129 NP [5]
例5 8 NA 嗜睡、言语改变、反应迟钝 第7天 第15天 额叶慢波 NP 119 直径5 mm [5]
例6 10 NA 嗜睡、幻视、性格改变 第3天 第7天 弥散慢波 NP 127 NP [5]
例7 2 86/58 抽搐、意识障碍 第3天 NA 慢波 NP 133 NP [6]
例8 2 NA 抽搐、意识障碍 第4天 第9天 慢波 压力升高,IL-6水平升高 133 NP [7]
例9 2 104/36 抽搐、意识障碍 第4天 第11天 棘波 NP 125 NP [8]
例10 5 NA 意识障碍 第7天 第20天 弥散慢波 NA NA NA [9]
例11 8 NA 意识障碍 第7天 第14天 慢波 NA NA RCA 3 mm,LAD 5 mm [9]
例12 11 NA 言语改变、共济失调 NA NA NA NA 143 NP [10]
例13 2 NA 意识障碍 NA NA NA NA NA 有(具体不详) [10]
例14 2 NA 意识障碍、言语改变 第5天 NA NA IL-6水平升高 131 NP [11]
例15 10 NA 幻视、言语及行为改变 4天内 NA 弥散慢波 NP 127 NA [12]
例16 6 NA 幻视、情绪、言语及行为改变 4天内 NA NP 129 NA [12]
例17 6 NA 情绪改变、嗜睡 NA NA NA NA NA NA [13]
例18 7 NA 头痛、嗜睡 第8天 第18天 128 NP [10]
例19 6 96/62 发热、头痛、呕吐 NA 第23天 慢波 137 NP [14]
本例 11 85/51 头晕、谵妄 第5天 第16天 总蛋白水平升高 133 NP  
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