Division of Pulmonary Medicine, Department of Pediatrics, Vanderbilt University School of Medicine, 2200 Children's Way, 11215 Doctor's Office Tower, Nashville, TN 37232-9500.
Pediatrics. 2013 Oct;132(4):684-91. doi: 10.1542/peds.2013-1780. Epub 2013 Sep 30.
Childhood interstitial lung diseases (ILD) occur in a variety of clinical contexts. Advances in the understanding of disease pathogenesis and use of standardized terminology have facilitated increased case ascertainment. However, as all studies have been performed at specialized referral centers, the applicability of these findings to general pulmonary practice has been uncertain. The objective of this study was to determine the historical occurrence of childhood ILD to provide information reflecting general pediatric pulmonary practice patterns.
Childhood ILD cases seen at Vanderbilt Children's Hospital from 1994 to 2011 were retrospectively reviewed and classified according to the current pediatric diffuse lung disease histopathologic classification system.
A total of 93 cases were identified, of which 91.4% were classifiable. A total of 68.8% (64/93) of subjects underwent lung biopsy in their evaluations. The largest classification categories were disorders related to systemic disease processes (24.7%), disorders of the immunocompromised host (24.7%), and disorders more prevalent in infancy (22.6%). Eight cases of neuroendocrine cell hyperplasia of infancy (NEHI) were identified, including 5 that were previously unrecognized before this review.
Our findings demonstrate the general scope of childhood ILD and that these cases present within a variety of pediatric subspecialties. Retrospective review was valuable in recognizing more recently described forms of childhood ILD. As a significant portion of cases were classifiable based on clinical, genetic, and/or radiographic criteria, we urge greater consideration to noninvasive diagnostic approaches and suggest modification to the current childhood ILD classification scheme to accommodate the increasing number of cases diagnosed without lung biopsy.
儿童间质性肺病（ILD）发生在多种临床情况下。对疾病发病机制的认识进步和标准化术语的使用促进了更多病例的确定。然而，由于所有研究都是在专门的转诊中心进行的，因此这些发现对一般肺部实践的适用性尚不确定。本研究的目的是确定儿童ILD 的历史发生率，以提供反映一般儿科肺部实践模式的信息。
对范德比尔特儿童医院 1994 年至 2011 年期间就诊的儿童ILD 病例进行回顾性分析，并根据当前儿科弥漫性肺疾病组织病理学分类系统进行分类。
共确定了 93 例病例，其中 91.4%可分类。在评估中，共有 68.8%（64/93）的患者进行了肺活检。最大的分类类别是与系统性疾病过程相关的疾病（24.7%）、免疫功能低下宿主的疾病（24.7%）和在婴儿期更常见的疾病（22.6%）。共发现 8 例婴儿期神经内分泌细胞增生（NEHI）病例，其中 5 例在此回顾之前未被识别。
我们的研究结果表明了儿童ILD 的一般范围，这些病例出现在各种儿科亚专业中。回顾性分析对于识别最近描述的儿童ILD 形式非常有价值。由于根据临床、遗传和/或影像学标准可对很大一部分病例进行分类，因此我们强烈主张更多地考虑非侵入性诊断方法，并建议对当前的儿童ILD 分类方案进行修改，以适应越来越多无需肺活检即可诊断的病例。
