儿童非特异性间质性肺炎2例分析  

Clinical Report of 2 Cases of Nonspecific Interstitial Pneumonia in Children and Review of Related Literatures

作　　者：耿凌云[1] 曹玲[1] 黄荣妍[1] 邹继珍[1] 李競贤 康小会[1] 陈慧中[1] 

机构地区：[1]首都儿科研究所附属儿童医院,北京100020

出　　处：《中国医刊》2010年第3期47-50,共4页Chinese Journal of Medicine

摘　　要：目的探讨儿童非特异性间质性肺炎(NSIP)的临床特征。方法对2例经胸腔镜肺活检诊断的儿童NSIP的临床表现、影像学、肺功能、病理资料及预后进行回顾分析,并复习相关文献。结果 2例患儿,女男各1例,年龄分别为11岁和8岁,发病至就诊时间为8周至2年。2例患儿均有咳嗽、进行性呼吸困难、间断低热表现,病程中均曾有吸气相细湿啰音,1例有杵状指。2例均有明显的低氧血症,肺功能检查均提示混合性通气功能障碍。胸部高分辨率CT(HRCT)均提示两肺有弥漫分布的磨玻璃影、小叶间隔增厚及两肺内带的斑片影,1例还有弥漫网格影及索条影,均未见蜂窝样变。组织类型1例为细胞型,1例为混合型。2例患儿均给予糖皮质激素治疗,病情明显好转。结论对儿童患者,当临床表现、胸部影像学等检查提示肺间质病变而不能明确病因时,病情允许情况下要尽可能行肺活检检查,以明确NSIP的病理诊断及组织学类型。确诊NSIP患者,加用糖皮质激素治疗可明显提高临床治疗效果,但仍需长期随诊,结合病史和相关辅助检查进一步查找可能的潜在病因。Objective To explore the clinical features of nonspecific interstitial pneumonia in children. Methods The clinical, radiological, histologic manifestations, pulmonary function and prognosis of two cases with NSIP confirmed by video-assisted thoracoscopic lung biopsy were analyzed retrospectivedly. Results Two cases, one girl and one boy, aged 11 and 8 respectively, presented with a duration of symptoms on diagnosis of 8 weeks to 2 years. Cough, worsening dyspnea,intermittent low grade fever and inspiratory crackles were presented in 2 cases and digital club- bing in one. Both of them showed evident hypoxemia and mixed ventilatory dysfunction. The chest high-resolution CT scans showed diffuse ground glass opacities, thickened interlobular septa, patchy parenchymal infiltrate in the in- ner band of both lungs in 2 cases and diffuse reticular opacities, bundle opacities in 1, while no honeycombing was found in 2. In the tissue type, 1 case was cellular pattern, the other was cellular and fibrosing one. The 2 cases both showed better response to glucocorticoids. Conclusion When clinical manifestations,chest radiological features and so on suggest interstitial lung diseases while the causes were unknown in children, surgical lung biopsy should be performed to explicit their pathologic diagnosis and subgroup of NSIP if possible according to their situations. When the diagnosis of NSIP was confirmed, glucocorticoid should be given to raise the therapeutic effectiveness, but a fol- low-up for a long time was still necessary to further look for the possible underlying cause combined with medical history and related assistant tests.

关 键 词：非特异性间质性肺炎 肺活检 儿童 

分 类 号：R725.6[医药卫生—儿科]