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儿童间质性和弥漫性肺病患者的处理方法。

Approach to the patient with Childhood Interstitial and Diffuse Lung Disease.

机构信息

Northwell, New Hyde Park, New York, USA.

Departments of Science Education and Pediatrics, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York, USA.

出版信息

Pediatr Pulmonol. 2024 Sep;59(9):2267-2275. doi: 10.1002/ppul.27162. Epub 2024 Jul 26.

Abstract

Childhood Interstitial and Diffuse Lung Disease (chILD) encompasses a group of rare, chronic lung disorders in infants and children with overlapping clinical features but diverse etiologies. The clinical presentation of chILD is of chronic or recurring respiratory signs and symptoms, often including increased work of breathing and hypoxia, with diffuse radiographic abnormalities on chest imaging. Recognition can be challenging since some clinical features overlap with those of more common pediatric respiratory diseases including asthma and recurrent viral infections, among others. chILD should be considered as an underlying diagnosis when a patient's respiratory symptoms seem disproportionate to the clinical scenario and/or persist. The diagnostic process involves multiple steps and is tailored to the individual patient. Nearly all children will undergo imaging and pulmonary function testing, many will undergo bronchoscopy with bronchoalveolar lavage, many will receive genetic testing, and some will require lung biopsy. Treatment includes preventive care, evaluation for comorbidities, pharmacotherapy according to diagnosis, and ongoing disease surveillance, including revisiting genetic and histopathologic results as new clinical information becomes available and as our understanding of these rare disorders improves. The purpose of this review is to provide a broad approach to the diagnosis and management of patients with chILD.

摘要

儿童间质性和弥漫性肺疾病(chILD)涵盖了一组罕见的婴幼儿慢性肺部疾病,这些疾病具有重叠的临床特征,但病因不同。chILD 的临床表现为慢性或反复出现的呼吸道症状和体征,常伴有呼吸功增加和缺氧,胸部影像学显示弥漫性异常。由于一些临床特征与更常见的儿科呼吸道疾病(如哮喘和反复病毒感染等)重叠,因此识别可能具有挑战性。当患者的呼吸道症状与临床情况不成比例且/或持续存在时,应考虑 chILD 作为潜在诊断。诊断过程包括多个步骤,并根据个体患者进行调整。几乎所有儿童都会进行影像学和肺功能检查,许多儿童会进行支气管镜检查和肺泡灌洗,许多儿童会进行基因检测,一些儿童需要进行肺活检。治疗包括预防保健、合并症评估、根据诊断进行药物治疗以及持续的疾病监测,包括在获得新的临床信息和我们对这些罕见疾病的理解不断提高时重新评估基因和组织病理学结果。本综述的目的是提供一种广泛的方法来诊断和管理 chILD 患者。

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