Abstract: Objective    To analyze the clinical data of interstitial lung disease （ILD） in children， in order to improve the understanding of this disease among clinical doctors. Methods    Retrospective analysis of clinical data of pediatric patients with ILD who were hospitalized in the First Affiliated Hospital of Xiamen University  from December 2015 to April 2023， including gender， age， symptoms， bronchoscopic manifestations， imaging manifestations， lung function， primary disease， concomitant diease， disease burden and outcomes.Results    A total of 198 children were diagnosed， with 110 males and 88 females; The age of onset was 31.00 months （1.43-156.00 months）.The most common respiratory symptoms in children with ILD were cough in 130 cases （65.7%）， expectoration in 117 cases （59.1%）.The most common systemic symptoms were fever in 72 cases （36.4%）; 31 cases （15.7%） had different degrees of malnutrition. Among the etiology，134 cases（67.7%）were related to alveolar structure disorder. 59 cases（29.8%） were associated with systemic dieases. 3 cases（1.5%） were unigue to infancy. 2 cases（1.0%） were related to environmental exposure.Among them， 129 cases （96.3%） of pulmonary infection were the most common cases related to alveolar structural disorders; Systemic diseases were common in 25 cases （42.4%） of juvenile idiopathic arthritis， followed by 10 cases （16.9%） of systemic lupus erythematosus.The HRCT manifestations of the chest were ground glass shadow in 192 cases （97.0%）， subpleural patchy shadow in 162 cases （81.8%）.Under bronchoscopy， various degrees of bronchial intimal inflammation were observed in all 111 children. Among them， 16 cases （8.1%） were accompanied by tracheomalacia in different parts， 3 cases （1.5%） were accompanied by laryngeal cleft， 4 cases （2.0%） had tracheal bronchi. A total of 77 cases （38.9%） completed lung function examinations， 26 cases （33.8%） were normal， 28 cases （36.3%） had varying degrees of ventilation dysfunction， 8 cases （10.4%） had varying degrees of diffusion dysfunction， and 15 cases （19.5%） had mixed ventilation dysfunction. Conclusion    The age distribution of children with ILD is wide， and only some children have respiratory symptoms. HRCT manifestations of the chest are diverse， which helps in the early diagnosis of ILD，the control of the primary disease， and regular respiratory follow-up are the key to the diagnosis and treatment of ILD.

Key words:  , interstitial lung disease, child, infection, connective tissue disease

摘要： 目的    分析儿童间质性肺疾病（ILD）的临床资料，以提高对本病的认识。方法    回顾性分析2015年12月至 2023年4月在厦门大学附属第一医院儿科住院并确诊为ILD患儿的临床资料，包括性别、年龄、症状、支气管镜下表现、影像学表现、肺功能、原发病、伴随疾病、疾病负担、转归等。结果    198例患儿中，男性110例，女性88例；发病年龄31.00月龄（1.43～156.00月龄）；ILD患儿呼吸系统最常见的症状为咳嗽130例（65.7%），咳痰117例（59.1%）；全身症状最常见为发热72例（36.4%）；31例（15.7%）患儿存在不同程度的营养不良。病因中与肺泡结构紊乱相关134例（67.7%），与全身疾病相关59例（29.8%），婴儿期特有3例（1.5%），与环境暴露相关2例（1.0%）；其中肺泡结构紊乱相关以肺部感染129例（96.3%）最为常见；全身疾病以幼年特发性关节炎25例（42.4%）常见，其次为系统性红斑狼疮10例（16.9%）；胸部高分辨率CT（HRCT）表现为磨玻璃影192例（97.0%）、胸膜下斑片影162例（81.8%）；111例患儿支气管镜镜下均可见不同程度的支气管内膜炎症，其中16例（8.1%）伴有不同部位的气管软化，3例（1.5%）伴有喉裂，4例（2.0%）存在气管性支气管。共77例（38.9%）完善肺功能检查，其中26例（33.8%）正常，28例（36.3%）存在不同程度的通气功能障碍，8例（10.4%）存在不同程度的弥散功能障碍，15例（19.5%）存在混合通气功能障碍。结论    儿童ILD 发病年龄分布范围广，仅部分患儿存在呼吸道症状，胸部HRCT表现多样，有助于ILD的早期诊断，原发病的控制，定期呼吸专科随访是ILD诊治的关键。

关键词: 间质性肺疾病, 儿童, 感染, 结缔组织疾病