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嗜酸性粒细胞增多性疾病:当前分类与诊断标准的评估,实用诊断算法的建议

Eosinophilic disorders: evaluation of current classification and diagnostic criteria, proposal of a practical diagnostic algorithm.

作者信息

Leru Polliana Mihaela

机构信息

1Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.

2Internal Medicine Department, Colentina Clinical Hospital, Sos. Stefan cel Mare, No. 19-21, District 2, 020125 Bucharest, Romania.

出版信息

Clin Transl Allergy. 2019 Jul 25;9:36. doi: 10.1186/s13601-019-0277-4. eCollection 2019.

Abstract

Eosinophilic disorders represent a group of pathologic conditions with highly heterogeneous pathophysiology and clinical presentation and variable prognosis, ranging from asymptomatic or mild, to severe and complex cases, with fatal outcome. Interest in this group of disorders has increased during the last two decades, with consistent progress made regarding understanding of molecular mechanisms, refining of diagnostic criteria, classification and evaluation of therapeutic options. There are still many gaps and difficulties in evaluating eosinophilic syndromes and diseases in medical practice. The disease prognosis depends mainly on the cause and mechanism of eosinophilia, on severity of organ dysfunction and on accurate diagnosis and response to treatment. Besides primary hypereosinophilic syndromes and secondary (reactive) eosinophilias, many associated or idiopathic forms have been described, making this topic a complex and difficult medical entity. An important aim of the experts in the field is to agree upon a more clear and practically useful classification, a better characterization of various phenotypes and endotypes of eosinophilic diseases and to identify novel biomarkers and more effective therapies. The aim of this paper is to review recent data from the literature regarding definition, classification and diagnosis criteria of eosinophilic diseases and to propose a revised and updated diagnostic algorithm useful in clinical practice.

摘要

嗜酸性粒细胞增多性疾病是一组病理状况,其病理生理学和临床表现高度异质,预后不一,从无症状或轻度病例到严重复杂病例,甚至可导致致命后果。在过去二十年中,人们对这组疾病的关注度有所增加,在分子机制的理解、诊断标准的完善、治疗方案的分类和评估方面都取得了持续进展。在医学实践中,评估嗜酸性粒细胞增多综合征和疾病仍存在许多差距和困难。疾病的预后主要取决于嗜酸性粒细胞增多的原因和机制、器官功能障碍的严重程度以及准确的诊断和对治疗的反应。除了原发性高嗜酸性粒细胞综合征和继发性(反应性)嗜酸性粒细胞增多外,还描述了许多相关或特发性形式,这使得该主题成为一个复杂且棘手的医学实体。该领域专家的一个重要目标是就更清晰、更实用的分类达成共识,更好地描述嗜酸性粒细胞增多性疾病的各种表型和内型,并确定新的生物标志物和更有效的治疗方法。本文的目的是回顾文献中关于嗜酸性粒细胞增多性疾病的定义、分类和诊断标准的最新数据,并提出一种经修订和更新的、对临床实践有用的诊断算法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7844/6657042/5f9cbc567625/13601_2019_277_Fig1_HTML.jpg

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