儿童特发性肺间质纤维化临床表现与辅助检查

Clinical Manifestations and Auxiliary Examination of Idiopathic Pulmonary Fibrosis in Children

目的探讨儿童特发性肺间质纤维化(IPF)的临床表现及辅助检查诊断价值。方法回顾性分析17例IPF患儿的临床资料及胸部X线、高分辨率CT(HRCT)、纤维支气管镜、肺功能、血气分析、肺活检等辅助检查资料。结果 IPF患儿临床以咳嗽、气促、发热、进行性呼吸困难伴肺底部Velcho啰音为主要表现;胸片/HRCT主要显示双肺斑片状、网格状或实变影、毛玻璃影、马赛克征。肺功能多数呈限制性通气功能和弥散障碍,血气分析提示低氧血症,17例中达呼吸衰竭诊断标准者5例。纤维支气管镜检查可见气管支气管内膜炎,支气管软化、扩张、狭窄等,无诊断特异性表现。1例患儿死亡后行肺活检,显示肺弥散性炎症伴灶型坏死,成人型小儿肺透明膜病。结论儿童IPF的诊断主要根据典型临床表现,结合胸片、HRCT、肺功能、血气分析等辅助检查,胸部影像学尤其HRCT是能反映IPF肺组织结构改变的敏感方法,纤维支气管镜检查有重要鉴别诊断作用,不典型病例确诊还需肺活检。

Objective To explore the diagnostic value of clinical manifestations and auxiliary examination of idiopathic pulmonary fibro- sis （IPF） in children. Methods The clinical manifestations,chest X - ray,high - resolution computed tomography （HRCT） ,fiberoptic bron- choscopy, pulmonary function, blood gas analysis, lung biopsy, et al,in 17 children with IPF, were retrospectively analyzed. Results The main clinical manifestations with presented with fever, cough, shortness of breath, progressive dyspnea and lung bottom with Velcho sound. Chest ra- diograph/HRCT mainly displayed patchy lungs, grid or consolidation shadow, ground glass shadow, and mosaic signs. Lung functions mainly re- vealed restriction in ventilation and diffusion barriers. Blood gas analysis suggested hypoxia, as 5 cases were up to respiratory failure diagnosis standard. Fiberoptic bronehoscopy displayed tracheal bronchial endometritis, bronchial softening, expansion, narrowness, and it had no specific diagnostic manifestations. One case after death had lung biopsy in this group, and it presented lung diffuse inflammation with kitchen type nec- rosis, with children transparent film lung disease in adult type. Conclusions Diagnosis of IPF in children is mainly based on the typical clini- cal manifestations, combined with the chest radiograph, HRCT, pulmonary function, blood gas analysis, et al. Chest imaging especially HRCT had importance in early diagnosis of IPF. Fiberoptic bronchoscopy had important differential diagnosis function. To diagnose non - typical cases still needs lun~ biopsy.