首页 | 本学科首页   官方微博 | 高级检索  
检索        

儿童特发性肺间质纤维化临床表现与辅助检查
引用本文:代丽,黄英,舒畅,代继宏,李渠北,王莹.儿童特发性肺间质纤维化临床表现与辅助检查[J].实用儿科临床杂志,2012,27(16):1236-1238.
作者姓名:代丽  黄英  舒畅  代继宏  李渠北  王莹
作者单位:重庆医科大学附属儿童医院呼吸中心,儿童发育疾病研究省部共建教育部重点实验室,重庆400014
摘    要: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。

关 键 词:特发性肺间质纤维化  临床表现  辅助检查  儿童

Clinical Manifestations and Auxiliary Examination of Idiopathic Pulmonary Fibrosis in Children
DAI Li , HUANG Ying , SHU Chang , DAI Ji-hong , LI Qu-bei , WANG Ying.Clinical Manifestations and Auxiliary Examination of Idiopathic Pulmonary Fibrosis in Children[J].Journal of Applied Clinical Pediatrics,2012,27(16):1236-1238.
Authors:DAI Li  HUANG Ying  SHU Chang  DAI Ji-hong  LI Qu-bei  WANG Ying
Institution:(Respiratory Center,Children′s Hospital of Chongqing Medical University,Key Laboratory of Developmental Diseases in Childhood,Chongqing Medical University,Ministry of Education,Chongqing 400014,China)
Abstract:Objective To explore the diagnostic value of clinical manifestations and auxiliary examination of idiopathic pulmonary fibrosis(IPF) in children.Methods The clinical manifestations,chest X-ray,high-resolution computed tomography(HRCT),fiberoptic bronchoscopy,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 radiograph/HRCT mainly displayed patchy lungs,grid or consolidation shadow,ground glass shadow,and mosaic signs.Lung functions mainly revealed restriction in ventilation and diffusion barriers.Blood gas analysis suggested hypoxia,as 5 cases were up to respiratory failure diagnosis standard.Fiberoptic bronchoscopy 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 necrosis,with children transparent film lung disease in adult type.Conclusions Diagnosis of IPF in children is mainly based on the typical clinical 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 lung biopsy.
Keywords:idiopathic pulmonary fibrosis  clinical manifestations  auxiliary examination  child
本文献已被 CNKI 万方数据 等数据库收录!
设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号