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儿童间质性肺疾病的诊断与治疗进展

An update on diagnosis and treatments of childhood interstitial lung diseases.

作者信息

Marczak Honorata, Krenke Katarzyna, Griese Matthias, Carlens Julia, Seidl Elias, Gilbert Carlee, Emiralioglu Nagehan, Torrent-Vernetta Alba, Willemse Brigitte, Epaud Ralph, Delestrain Celine, Louvrier Camille, Koucký Václav, Nathan Nadia

机构信息

Department of Pediatric Pneumology and Allergy, Medical University of Warsaw, Warsaw, Poland.

Department of Paediatric Pneumology, Dr von Hauner Children's Hospital, German Centre for Lung Research, University of Munich, Munich, Germany.

出版信息

Breathe (Sheff). 2025 May 13;21(2):250004. doi: 10.1183/20734735.0004-2025. eCollection 2025 Apr.

Abstract

Childhood interstitial lung diseases (chILDs) are rare and heterogeneous disorders associated with significant morbidity and mortality. The clinical presentation of chILD typically includes chronic or recurrent respiratory signs and symptoms with diffuse radiographic abnormalities on chest imaging. Diagnosis requires a structured, multi-step approach. Treatment options are limited, with disease-specific therapies available only in selected cases and management relying primarily on supportive care. Awareness of chILDs has been steadily increasing. New diagnoses, advanced diagnostic tests, and novel treatments are emerging each year, highlighting the importance of collaborative, multidisciplinary teams in providing comprehensive care for children and families affected by these complex conditions. On behalf of the European Respiratory Society Clinical Research Collaboration for chILD (ERS CRC chILD-EU), this review provides an updated overview of the diagnostic approach and management strategies for chILDs.

摘要

儿童间质性肺疾病(chILDs)是一类罕见且异质性的疾病,与显著的发病率和死亡率相关。chILD的临床表现通常包括慢性或反复出现的呼吸道症状和体征,胸部影像学检查显示弥漫性异常。诊断需要采用结构化的多步骤方法。治疗选择有限,仅在特定病例中有针对疾病的疗法,管理主要依赖支持性治疗。对chILDs的认识一直在稳步提高。每年都有新的诊断、先进的诊断测试和新的治疗方法出现,这凸显了协作性多学科团队在为受这些复杂疾病影响的儿童和家庭提供全面护理方面的重要性。代表欧洲呼吸学会儿童临床研究协作组(ERS CRC chILD-EU),本综述提供了chILDs诊断方法和管理策略的最新概述。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1588/12070201/c6b979c1ec84/EDU-0004-2025.01.jpg

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