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Phleos的开发与实施,一种用于嗜酸性粒细胞增多综合征数据收集的基于网络的工具:意大利嗜酸性粒细胞增多综合征网络(INHES)研究方案。

Development and implementation of Phleos, a web-based tool for the data collection on Hypereosinophilic syndrome: the Italian Network on HES (INHES) study protocol.

作者信息

Nicola Stefania, Caminati Marco, Borrelli Richard, Lo Sardo Luca, Corradi Federica, Badiu Iuliana, Vacca Angelo, Carlucci Palma, De Paulis Amato, Mormile Ilaria, Zurlo Marco, Di Gioacchino Mario, Patella Vincenzo, Del Giacco Stefano, Costanzo Giulia, Negrini Simone, Rolla Giovanni, Brussino Luisa

机构信息

Immunology and Allergy Unit - AO Ordine Mauriziano di Torino, Turin, Italy.

Department of Medical Sciences, University of Turin, Turin, Italy.

出版信息

Front Immunol. 2025 Jul 16;16:1638798. doi: 10.3389/fimmu.2025.1638798. eCollection 2025.

Abstract

Hypereosinophilic syndrome (HES) is a heterogeneous group of disorders characterised by persistent hypereosinophilia associated with organ damage. Due to its rarity and heterogeneity in its clinical presentation, HES remains underdiagnosed or misdiagnosed, often leading to delayed diagnosis and irreversible organ damage. The complexity of HES diagnosis is even more complicated due to the absence of standardised criteria. Moreover, the lack of structured referral pathways among specialists, including allergists, clinical immunologists, haematologists, and rheumatologists, further hinders optimal patient care. To address these challenges, the Italian National Hypereosinophilic Syndrome (INHES) Network aimed to enhance the diagnosis, management, and research of HES. INHES objectives also include connecting specialised centres, facilitating data collection on HES and eosinophilic-associated conditions, and improving healthcare standards through consensus guidelines. To do this, INHES has created a web-based platform called "Phleos" to develop a comprehensive referral map, ensuring timely and appropriate patient access to expert care. Phleos is a web-based datasheet, per GDPR regulations, to systematically collect anonymised clinical data, including absolute eosinophil counts, organ involvement, laboratory parameters, instrumental assessments, and treatments. A structured classification system enables differentiation among idiopathic, lymphocytic, myeloid, familial, reactive, and overlap forms of HES. Moreover, the platform integrates standardised diagnostic pathways and treatment protocols to harmonise patient care across participating centres. The INHES Network aims to mitigate diagnostic delays, optimise therapeutic decision-making, and advance research in eosinophilic disorders by fostering interdisciplinary collaboration and establishing a unified framework. This initiative represents a crucial step toward a cohesive national strategy, ultimately improving clinical outcomes for HES patients in Italy.

摘要

高嗜酸性粒细胞综合征(HES)是一组异质性疾病,其特征为持续性嗜酸性粒细胞增多并伴有器官损害。由于其临床症状罕见且具有异质性,HES仍常被漏诊或误诊,常导致诊断延迟和不可逆的器官损害。由于缺乏标准化标准,HES诊断的复杂性更加突出。此外,包括过敏症专科医生、临床免疫学家、血液学家和风湿病学家在内的专科医生之间缺乏结构化的转诊途径,进一步阻碍了对患者的最佳治疗。为应对这些挑战,意大利国家高嗜酸性粒细胞综合征(INHES)网络旨在加强HES的诊断、管理和研究。INHES的目标还包括连接专业中心,促进关于HES和嗜酸性粒细胞相关疾病的数据收集,并通过共识指南提高医疗标准。为此,INHES创建了一个名为“Phleos”的基于网络的平台,以绘制全面的转诊地图,确保患者能及时、适当地获得专家治疗。根据通用数据保护条例(GDPR),Phleos是一个基于网络的数据表,用于系统收集匿名临床数据,包括绝对嗜酸性粒细胞计数、器官受累情况、实验室参数、仪器评估和治疗方法。一个结构化的分类系统能够区分HES的特发性、淋巴细胞性、髓细胞性、家族性、反应性和重叠性类型。此外,该平台整合了标准化诊断途径和治疗方案,以协调各参与中心的患者治疗。INHES网络旨在通过促进跨学科合作和建立统一框架,减少诊断延迟,优化治疗决策,并推动嗜酸性粒细胞疾病的研究。这一举措是朝着凝聚性国家战略迈出的关键一步,最终改善意大利HES患者的临床结局。

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Eosinophil-associated diseases: the Allergist's and Clinical Immunologist's perspective.嗜酸粒细胞相关疾病:过敏学家和临床免疫学家的视角。
Eur Ann Allergy Clin Immunol. 2024 Sep;56(5):195-209. doi: 10.23822/EurAnnACI.1764-1489.339. Epub 2024 Mar 28.

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