Department of Pediatrics, University of Bologna, S. Orsola-Malpighi Hospital Bologna, Italy.
Early Hum Dev. 2013 Oct;89 Suppl 3:S39-43. doi: 10.1016/j.earlhumdev.2013.07.021. Epub 2013 Aug 22.
Children's interstitial lung disease (ILD) includes a wide range of rare respiratory disorders associated with high morbidity and mortality. Genetic factors, systemic disease processes, nonspecific inflammatory or fibrotic patterns of repair seen in a number of clinical settings are involved in the ILD pathogenesis. Specific disorders more prevalent in young children include diffuse developmental disorders, alveolar growth abnormalities, genetic surfactant disorders, pulmonary interstitial glycogenosis and neuroendocrine cell hyperplasia of infancy. It may be difficult to recognize these entities and this can lead to delayed treatment. The diagnostic approach is based on a combination of history/physical examinations, imaging studies, pulmonary function testing, genetic testing, bronchoalveolar lavage (BAL) and in most cases an open lung biopsy. Although some disease types overlap with those seen in adults, in this review emphasis is placed on entities unique to the pediatric population focusing on clinical characteristics, histologic definitions, radiologic-pathologic correlation and therapeutic strategies.
儿童间质性肺病（ILD）包括一系列与高发病率和死亡率相关的罕见呼吸系统疾病。遗传因素、系统性疾病过程、在许多临床情况下观察到的非特异性炎症或纤维修复模式都与ILD 的发病机制有关。在幼儿中更为常见的特定疾病包括弥漫性发育障碍、肺泡生长异常、遗传表面活性剂疾病、肺间质糖原病和神经内分泌细胞增生症。识别这些实体可能具有一定难度，这可能导致治疗延误。诊断方法基于病史/体格检查、影像学研究、肺功能测试、基因测试、支气管肺泡灌洗（BAL）以及在大多数情况下进行开胸肺活检的综合运用。虽然某些疾病类型与成人中所见类型重叠，但在本次综述中，重点放在了儿科人群中特有的实体上，重点关注临床特征、组织学定义、放射病理学相关性和治疗策略。
