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病(chILD)包括一组罕见的,婴儿和儿童的慢性肺部疾病具有重叠的临床特征,但病因不同。chILD的临床表现是慢性或反复出现的呼吸体征和症状,通常包括增加呼吸和缺氧的工作,胸部影像学弥漫性影像学异常。识别可能是具有挑战性的,因为一些临床特征与更常见的儿科呼吸系统疾病(包括哮喘和复发性病毒感染)重叠。在其他人中。当患者的呼吸道症状似乎与临床情况不成比例和/或持续存在时,chILD应被视为基础诊断.诊断过程涉及多个步骤并且是针对个体患者定制的。几乎所有儿童都将接受影像学和肺功能检查,许多人将接受支气管镜检查与支气管肺泡灌洗,许多人将接受基因检测,有些需要肺活检.治疗包括预防性护理,合并症的评估,根据诊断进行药物治疗,和持续的疾病监测,包括随着新的临床信息的获得以及我们对这些罕见疾病的理解的提高,重新审视遗传和组织病理学结果.这篇综述的目的是为child患者的诊断和治疗提供一种广泛的方法。