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一名患有静脉注射免疫球蛋白抵抗性川崎病且需反复使用糖皮质激素治疗的婴儿:病例报告及文献综述

An infant with IVIG-resistant Kawasaki disease requiring repeated glucocorticoids therapy: A case report and literature review.

作者信息

Ren Yifan

机构信息

Department of Pediatrics, Shaoxing Keqiao Women and Children's Hospital, Shaoxing, PR China.

出版信息

Medicine (Baltimore). 2025 May 23;104(21):e42572. doi: 10.1097/MD.0000000000042572.

Abstract

RATIONALE

Kawasaki disease (KD) is an acute systemic vasculitis predominantly affecting children, characterized by fever, chapped lips, strawberry tongue, conjunctival hyperemia, rash, and cervical lymphadenopathy. The most severe complication associated with KD is the development of coronary artery abnormalities.

PATIENT CONCERNS

A 1-year and 2-month-old girl was admitted to the hospital with persistent fever, rash, and elevated inflammatory markers.

DIAGNOSES

Despite 36 hours of intravenous immunoglobulin (IVIG) therapy, she remained febrile and was classified as IVIG-resistant.

INTERVENTIONS

Methylprednisolone was administered for 13 days; however, tapering to a single daily dose resulted in symptom recurrence.

OUTCOMES

A subsequent course of intravenous methylprednisolone, administered over 42 days, resolved her symptoms, with follow-up evaluations demonstrating normal echocardiograms and electrocardiograms. The duration of methylprednisolone treatment exceeded standard guideline recommendations for KD; however, the patient achieved full recovery without adverse effects.

LESSONS

This case highlights the potential efficacy of glucocorticoids as a treatment option for IVIG-resistant KD and emphasizes the necessity for further research into their role as first-line therapy, particularly in patients with IVIG intolerance.

摘要

理论依据

川崎病(KD)是一种主要影响儿童的急性全身性血管炎,其特征为发热、嘴唇干裂、草莓舌、结膜充血、皮疹和颈部淋巴结病。与KD相关的最严重并发症是冠状动脉异常的发生。

患者情况

一名1岁2个月大的女孩因持续发热、皮疹和炎症标志物升高入院。

诊断

尽管进行了36小时的静脉注射免疫球蛋白(IVIG)治疗,但她仍持续发热,被归类为IVIG抵抗型。

干预措施

给予甲泼尼龙治疗13天;然而,减至每日单剂量导致症状复发。

结果

随后进行了为期42天的静脉注射甲泼尼龙疗程,症状得以缓解,随访评估显示超声心动图和心电图正常。甲泼尼龙治疗的持续时间超过了KD的标准指南建议;然而,患者完全康复且无不良反应。

经验教训

该病例突出了糖皮质激素作为IVIG抵抗型KD治疗选择的潜在疗效,并强调了进一步研究其作为一线治疗的作用的必要性,特别是在IVIG不耐受的患者中。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6975/12113926/a82a4e3ce519/medi-104-e42572-g001.jpg

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