Clement A, Eber E
Respiratory and Allergic Disease Division, Paediatric Dept, Medical University of Graz, Auenbruggerplatz 30, A-8036 Graz, Austria.
Eur Respir J. 2008 Mar;31(3):658-66. doi: 10.1183/09031936.00004707.
Interstitial lung disease (ILD) in infants and children represents a heterogeneous group of respiratory disorders that are mostly chronic and associated with high morbidity and mortality. Typical features of ILD include dyspnoea, the presence of diffuse infiltrates on chest radiographs and abnormal pulmonary function tests with evidence of a restrictive ventilatory defect (in older children) and/or impaired gas exchange. ILD is difficult to diagnose, as no classification scheme is entirely satisfactory. Recently, it has been proposed to categorise paediatric ILD as either primary pulmonary disorders or systemic disorders with pulmonary involvement. However, this classification leaves the difficult problem of which clinical conditions should be included or excluded in infants and children. The present article reviews interstitial lung disease in infants and children, placing emphasis on current concepts of pathophysiological mechanisms and approaches to treatment.
婴幼儿间质性肺疾病（ILD）是一组异质性的呼吸系统疾病，大多为慢性疾病，且发病率和死亡率较高。ILD的典型特征包括呼吸困难、胸部X线片上出现弥漫性浸润以及肺功能测试异常，表现为限制性通气功能障碍（大龄儿童）和/或气体交换受损。ILD难以诊断，因为没有一种分类方案是完全令人满意的。最近，有人提议将儿童ILD分为原发性肺部疾病或累及肺部的全身性疾病。然而，这种分类留下了一个难题，即哪些临床情况应纳入或排除在婴幼儿和儿童ILD中。本文综述了婴幼儿间质性肺疾病，重点介绍了当前病理生理机制的概念和治疗方法。
