Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease.

IF 9 1区 医学 Q1 RESPIRATORY SYSTEM
Thorax Pub Date : 2024-10-03 DOI:10.1136/thorax-2024-221772
Matthias Griese, Geoffrey Kurland, Michal Cidon, Robin R Deterding, Ralph Epaud, Nadia Nathan, Nicolaus Schwerk, David Warburton, Jason P Weinman, Lisa R Young, Gail H Deutsch
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引用次数: 0

Abstract

Background: Childhood interstitial lung disease (chILD) encompasses a group of rare heterogeneous respiratory conditions associated with significant morbidity and mortality. Reports suggest that many patients diagnosed with chILD continue to have potentially progressive or fibrosing disease into adulthood. Over the last decade, the spectrum of conditions within chILD has widened substantially, with the discovery of novel entities through advanced genetic testing. However, most evidence is often limited to small case series, with reports disseminated across an array of subspecialty, clinical and molecular journals. In particular, the frequency, management and outcome of paediatric pulmonary fibrosis is not well characterised, unlike in adults, where clear diagnosis and treatment guidelines are available.

Methods and results: This review assesses the current understanding of pulmonary fibrosis in chILD. Based on registry data, we have provisionally estimated the occurrence of fibrosis in various manifestations of chILD, with 47 different potentially fibrotic chILD entities identified. Published evidence for fibrosis in the spectrum of chILD entities is assessed, and current and future issues in management of pulmonary fibrosis in childhood, continuing into adulthood, are considered.

Conclusions: There is a need for improved knowledge of chILD among pulmonologists to optimise the transition of care from paediatric to adult facilities. Updated evidence-based guidelines are needed that incorporate recommendations for the diagnosis and management of immune-mediated disorders, as well as chILD in older children approaching adulthood.

肺纤维化可能始于婴儿期:从儿童期到成年期的间质性肺病。
背景:儿童间质性肺病(childhood interstitial lung disease,chILD)是一组罕见的异质性呼吸系统疾病,发病率和死亡率都很高。报告显示,许多确诊为儿童间质性肺病的患者在成年后仍有潜在的进展性或纤维化疾病。在过去的十年中,通过先进的基因检测发现了一些新的实体,从而大大拓宽了 chILD 的病症范围。然而,大多数证据往往局限于小型病例系列,报告散见于一系列亚专科、临床和分子杂志。特别是,儿科肺纤维化的发病率、管理和治疗效果还没有得到很好的描述,不像成人有明确的诊断和治疗指南:本综述评估了目前对儿童期肺纤维化的认识。根据登记数据,我们初步估算了各种表现形式的儿童慢性阻塞性肺疾病的肺纤维化发生率,共发现了47种不同的可能发生肺纤维化的儿童慢性阻塞性肺疾病。我们评估了已发表的有关chILD实体纤维化的证据,并考虑了儿童期肺纤维化管理的当前和未来问题,这些问题将持续到成年期:结论:需要提高肺科医生对chILD的认识,以优化从儿科到成人设施的护理过渡。需要更新以证据为基础的指南,将免疫介导疾病的诊断和管理建议以及即将成年的大龄儿童的 chILD 纳入其中。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Thorax
Thorax 医学-呼吸系统
CiteScore
16.10
自引率
2.00%
发文量
197
审稿时长
1 months
期刊介绍: Thorax stands as one of the premier respiratory medicine journals globally, featuring clinical and experimental research articles spanning respiratory medicine, pediatrics, immunology, pharmacology, pathology, and surgery. The journal's mission is to publish noteworthy advancements in scientific understanding that are poised to influence clinical practice significantly. This encompasses articles delving into basic and translational mechanisms applicable to clinical material, covering areas such as cell and molecular biology, genetics, epidemiology, and immunology.
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