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一名患有嗜酸性肉芽肿性多血管炎的儿科患者发生大面积肺血栓栓塞:基于病例的综述,重点强调管理。

Massive pulmonary thromboembolism in a pediatric patient with eosinophilic granulomatosis with polyangiitis: a case-based review emphasizing management.

作者信息

Küçükali Batuhan, Yazol Merve, Yıldız Çisem, Acun Büşra, Belder Nuran, Karaçayır Nihal, Kutlar Merve, Esmeray Şenol Pelin, Kaya Zühre, Gezgin Yıldırım Deniz, Bakkaloğlu Sevcan A

机构信息

Department of Pediatric Rheumatology, Faculty of Medicine, Gazi University, Ankara, Besevler, 06500, Turkey.

Department of Pediatric Radiology, Faculty of Medicine, Gazi University, Ankara, Turkey.

出版信息

Pediatr Rheumatol Online J. 2025 Jan 3;23(1):1. doi: 10.1186/s12969-024-01054-3.

Abstract

BACKGROUND

Pediatric patients with Eosinophilic Granulomatosis with Polyangiitis (EGPA) are at an increased risk of arterial and venous thromboembolism (AVTE). Although the exact mechanisms underlying AVTE remain unclear, eosinophils play a pivotal role in AVTE.

MAIN BODY

Current guidelines lack evidence-based recommendations, particularly concerning anticoagulant and antiplatelet treatments for this condition. Herein, we document a pediatric EGPA patient with deep venous thrombosis presenting with massive pulmonary thromboembolism during a relapse, treated with immunosuppressive and anticoagulant therapy to raise awareness among clinicians. Additionally, we performed a literature review to highlight various aspects of pediatric AVTE. Moreover, we evaluated the management strategies employed for the patients identified in the literature review and summarized the current practice guidelines regarding pediatric EGPA patients with AVTE to provide recommendations to clinicians on the management of this challenging complication.

CONCLUSIONS

Most AVTE events occur during periods of high disease activity. Notably, EGPA patients with VTE often present with thrombocytopenia due to consumption, a finding not typically expected during disease exacerbation. Venous thrombosis generally requires both anticoagulation and immunosuppressive treatment. Although our review indicates a favorable prognosis for AVTE, the small number of reported cases prevents us from drawing definitive conclusions. Future studies should explore the efficacy of mepolizumab and other eosinophil-targeted therapies for AVTE, in addition to investigating the roles of anticoagulation and antiplatelet treatments.

摘要

背景

嗜酸性肉芽肿性多血管炎(EGPA)儿科患者发生动脉和静脉血栓栓塞(AVTE)的风险增加。尽管AVTE的确切机制尚不清楚,但嗜酸性粒细胞在AVTE中起关键作用。

主体

当前指南缺乏基于证据的建议,尤其是关于这种情况的抗凝和抗血小板治疗。在此,我们记录了1例儿科EGPA患者,其在复发期间出现深静脉血栓形成并伴有大面积肺血栓栓塞,接受了免疫抑制和抗凝治疗,以提高临床医生的认识。此外,我们进行了文献综述,以突出儿科AVTE的各个方面。此外,我们评估了文献综述中确定的患者所采用的管理策略,并总结了目前关于儿科EGPA合并AVTE患者的实践指南,为临床医生管理这一具有挑战性的并发症提供建议。

结论

大多数AVTE事件发生在疾病活动度高的时期。值得注意的是,EGPA合并VTE患者常因消耗出现血小板减少,这在疾病加重时通常不是预期的发现。静脉血栓形成一般需要抗凝和免疫抑制治疗。尽管我们的综述表明AVTE预后良好,但报告的病例数量较少,使我们无法得出明确结论。未来的研究应探索美泊利单抗和其他嗜酸性粒细胞靶向疗法对AVTE的疗效,此外还应研究抗凝和抗血小板治疗的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e760/11697824/cd549266d9ac/12969_2024_1054_Fig1_HTML.jpg

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