中国儿童间质性肺疾病的临床研究

Clinical study on interstitial lung disease in children of China

目的了解中国儿童间质性肺疾病（ILD）的病因谱。方法以2009年来自全国11家医院就诊的93例儿童ILD患者为研究对象，采用统一登记表格，详细记录其病史、症状、体征；所有病例均进行胸CT或高分辨率CT；住院期间常规检查项目包括血常规、血沉、C反应蛋白、血气分析，据病情轻重和年龄尽可能进行肺功能检查；具有诊断意义的项目包括呼吸道分泌物细菌培养、呼吸道病毒抗原检测、血清的病毒和支原体抗体检查，以及血清学的免疫抗体检查，痰、胃液、肺泡灌洗液涂片找含铁血黄素细胞等；对25例病例进行了支气管镜检，对14例病例进行了肺活检。由全国儿童弥漫性实质性肺疾病／间质性肺疾病协作组成员对每一病例进行集体讨论诊断。14例病例按临床一放射．病理（C—R—P）诊断模式达成诊断共识，79例病例根据临床及典型影像学改变、辅助检查做出最后诊断。结果93例ILD患儿年龄8个月～14岁，平均年龄为4岁11个月，其中男53例，女40例。（1）93例ILD的病因谱为：闭塞性细支气管炎39例；特发性肺含铁血黄素沉着症39例；特发性间质性肺炎7例（其中非特异性间质性肺炎4例，急性间质性肺炎1例，淋巴细胞间质性肺炎1例，特发性肺纤维化1例）；继发性的间质性肺炎2例（其中继发于系统性红斑狼疮的急性问质性肺炎1例，HIV感染引起的淋巴细胞间质性肺炎1例）；外源性过敏性肺泡炎2例；闭塞性细支气管炎伴机化性肺炎1例，肺泡微石症1例，弥漫性泛细支气管炎1例，类脂性肺炎1例。（2）临床特点：72例有咳嗽，24例有气促，5例有三凹征，43例肺内可闯及湿哕音，8例有杵状指趾。（3）影像学特征：肺CT显示56例肺部有磨玻璃影；30例肺部有马赛克灌注征，其中11例伴有支气管扩张，8例伴有支气管壁增厚；l例弥漫性小叶中心性结节，伴小支气管和细支气管扩张；1例为弥漫性的细小结节影；1例为弥漫性网格影和泡性肺气肿。结论儿童ILD是一组异质性疾病，本组共诊断出11种疾病，其中例数最多的前三位疾病为闭塞性细支气管炎、特发性肺含铁血黄素沉着症、特发性间质性肺炎。

Objective Interstitial lung disease in children represents a heterogeneous group of disorders of both known and unknown causes. This study aimed to understand better the causes of the disease in children and to provide information on the current approach to diagnosis and management of the disease. Method Through the Pediatric Diffuse Parenchymal Lung Disease/Pediatric Interstitial Lung Disease Cooperative Group of China, data of 93 cases of interstitial lung disease of children from 11 hospitals werecollected with the same questionnaire in 2009. Respiratory tract secretions were obtained for bacterial culture. Respiratory virus antigen examination, mycoplasma antibody, EB virus, cytomegalovirus, and herpes simplex viruses antibody detection were performed. Cells in the sputum, gastric juice and bronchoalveolar lavage fluid（BALF） were tested for hemosiderin. The CT or high resolution CT （HRCT） of the lung and blood- gas analysis were also performed. Fourteen cases underwent lung biopsy and 25 cases underwent bronchomicrocopy. Data were then pooled and discussed through a series of meetings. Result Fifty-three cases were male, 40 were female and their age ranged from 8 months to 14 years. Thirty-nine cases were diagnosed as bronchiolitis obliterans （BO） ;39 as idiopathic pulmonary hemosiderosis （IPH） ;7 as idiopathic interstitial pneumonia （IIP） of unknown causes, of whom 4 cases had non specific interstitial pneumonia, 1 case as acute interstitial pneumonia and 1 case as lymphocytic interstitial pneumonia, l case as idiopathic pulmonary fibrosis;2 cases as secondary interstitial lung disease, one was secondary to SLE, one to human immunodeficiency virus （HIV） infection; 2 cases had hypersensitive pneumonitis;2 cases had pulmonary alveolar proteinosis ; 1 case had bronchiolitis obliterans organizing pneumonia ; 1 case had lipoid pneumonia ; 1 case of diffuse panbronchiolitis ; 1 case of microlithiasis alveolaris pulmonum. Forty two cases had cough,24 of them also had tachypnea, 8 cases had clubbing. HRCT showed that 56 cases had groundglass-like opacification;30 cases had mosaic appearance;1 case had diffuse micronodular opacities, 1 case had diffuse reticulonodular opacities and cysts. Conclusion Interstitial lung disease in children is a heterogeneous group of disorders. In this study diagnoses of 11 diseases were made, the top third diagnoses were BO,IPH and liP, respectively.