Pediatric Pulmonary Section, Department of Pediatrics, USA.
Curr Opin Pediatr. 2011 Jun;23(3):325-31. doi: 10.1097/MOP.0b013e3283464a37.
In this review, we discuss the recent advances in our understanding of the cause, pathogenesis, presentation, diagnosis, treatment, and prognosis of interstitial lung disease (ILD) in children.
The classification of ILD syndromes in children greater than 2 years of age is based largely on adult classification schemes. In children less than 2 years of age, classification has been developed and evaluated pathologically. Entities can be categorized into developmental disorders, growth abnormalities, and surfactant dysfunction disorders based on pathologic findings. Two distinctive entities, neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis, present early in life with characteristic findings. These two disorders appear to have a favorable prognosis. Diagnosis of ILD syndromes is based on the summation of history and physical findings and both noninvasive and invasive studies. Newer approaches are being evaluated to decrease the need for lung biopsy.
Children's interstitial lung diseases are rare diffuse lung diseases resulting from a variety of pathogenic processes that include genetic factors, association with systemic disease processes, and inflammatory or fibrotic responses to stimuli. There are unique causes and presentations seen in infancy. Diagnosis in these disorders is made by the summation of clinical, radiologic, and pathologic findings.
在这篇综述中，我们讨论了近年来我们对儿童间质性肺疾病（ILD）病因、发病机制、表现、诊断、治疗和预后的理解的最新进展。
2 岁以上儿童的ILD 综合征分类主要基于成人分类方案。2 岁以下儿童的分类是基于病理发现而制定和评估的。根据病理发现，实体可分为发育障碍、生长异常和表面活性剂功能障碍。两种独特的实体，婴儿期神经内分泌细胞增生和肺间质糖原沉积症，在生命早期出现特征性表现。这两种疾病的预后良好。ILD 综合征的诊断基于病史和体格检查以及非侵入性和侵入性研究的总和。正在评估新的方法以减少肺活检的需要。
儿童间质性肺疾病是由多种致病过程引起的罕见弥漫性肺部疾病，包括遗传因素、与全身性疾病过程的关联以及对刺激的炎症或纤维化反应。在婴儿期有独特的病因和表现。这些疾病的诊断是通过临床、影像学和病理学表现的综合得出的。
