环孢素治疗川崎病并发冠状动脉瘤或治疗抵抗。

IF 3.9 2区医学 Q1 PEDIATRICS

Journal of Pediatrics Pub Date : 2025-01-24 DOI:10.1016/j.jpeds.2025.114479

Irene Bellicini MD , Emelia Bainto BA , Chisato Shimizu MD , Jane C. Burns MD , Adriana H. Tremoulet MD, MAS

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引用次数: 0

摘要

目的：描述33例因冠状动脉异常（CAA）或治疗抵抗而应用环孢素（CSA）治疗川崎病（KD）的临床过程和预后。研究设计：单中心、回顾性研究2013 - 2023年间因CAA或治疗耐药而接受治疗的KD患者。分析了人口统计学、实验室研究、药物、不良事件和超声心动图数据。结果：33例接受CSA治疗的KD患者中，25例因CAA接受CSA治疗，8例因耐药接受CSA治疗。4例患者最初接受静脉注射CSA，随后口服治疗。自2014年起，所有患者仅口服CSA。给药后2小时的目标水平（300-600 ng/ml）最好是每12小时分5 mg/kg/天。根据我们对这类患者的治疗经验，我们制定了一个标准化的治疗方案。在2周和6周的随访中，接受CAA治疗的患者冠状动脉z评分均有所改善。2例患者出现了明显的不良事件。一名患者患有后部可逆性脑病综合征，而另一名患者发展为ebv相关的淋巴细胞增生性疾病。停用CSA导致两例患者完全康复。结论：对于KD和CAA合并的患者或治疗抵抗患者，CSA的耐受性一般良好。我们的研究有助于补充有关CSA在KD中使用的有限文献，提供剂量指导并倡导谨慎监测。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

Cyclosporine Treatment in Patients with Kawasaki Disease and Coronary Artery Aneurysms or Treatment Resistance

Objective

To describe the clinical course and outcome of 33 patients with Kawasaki disease (KD) treated with cyclosporine (CSA) for coronary artery abnormalities (CAA) or treatment resistance.

Study design

Single-center, retrospective study of patients with KD treated from 2013 through 2023 for CAA or treatment resistance. Demographics, laboratory studies, medications, adverse events, and echocardiographic data were analyzed.

Results

Of the 33 KD patients treated with CSA, 25 patients received CSA for CAA and 8 for treatment resistance. Four patients received CSA intravenously initially, followed by oral therapy. Since 2014, all patients received CSA orally only. The target 2-hour postdose level (300-600 ng/ml) was best achieved by dividing 5 mg/kg/day every 12 hours. We developed a standardized treatment protocol based on our experiences with this patient population. The patients treated for CAA all showed improved coronary artery Z-scores at both the 2-week and 6-week follow-up. Two patients experienced significant adverse events. One patient had posterior reversible encephalopathy syndrome, while the other developed Epstein-Barr virus-associated lymphoproliferative disorder. Discontinuation of CSA led to complete recovery in both cases.

Conclusions

CSA was generally well tolerated in patients with KD and CAA or treatment resistance. Our study contributes to the limited literature on CSA use in KD, providing dosing guidance and advocating for cautious monitoring.

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来源期刊

Journal of Pediatrics 医学-小儿科

CiteScore

6.00

自引率

2.00%

发文量

696

审稿时长

31 days

期刊介绍： The Journal of Pediatrics is an international peer-reviewed journal that advances pediatric research and serves as a practical guide for pediatricians who manage health and diagnose and treat disorders in infants, children, and adolescents. The Journal publishes original work based on standards of excellence and expert review. The Journal seeks to publish high quality original articles that are immediately applicable to practice (basic science, translational research, evidence-based medicine), brief clinical and laboratory case reports, medical progress, expert commentary, grand rounds, insightful editorials, “classic” physical examinations, and novel insights into clinical and academic pediatric medicine related to every aspect of child health. Published monthly since 1932, The Journal of Pediatrics continues to promote the latest developments in pediatric medicine, child health, policy, and advocacy. Topics covered in The Journal of Pediatrics include, but are not limited to: General Pediatrics Pediatric Subspecialties Adolescent Medicine Allergy and Immunology Cardiology Critical Care Medicine Developmental-Behavioral Medicine Endocrinology Gastroenterology Hematology-Oncology Infectious Diseases Neonatal-Perinatal Medicine Nephrology Neurology Emergency Medicine Pulmonology Rheumatology Genetics Ethics Health Service Research Pediatric Hospitalist Medicine.