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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 investigate the clinical features of nonspecific interstitial pneumonia (NSIP) in children. Methods Two cases of NSIP diagnosed by thoracoscopic lung biopsy were retrospectively reviewed and reviewed for the clinical manifestations, imaging, lung function, pathological data and prognosis. Results Two cases of children and one male female each were aged 11 and 8 years old. The time from onset to treatment was 8 weeks to 2 years. 2 cases of children have cough, progressive dyspnea, intermittent low fever performance, during the inspiratory phase have wet rales, 1 case of clubbing. 2 cases had obvious hypoxemia, lung function tests were prompted mixed ventilation dysfunction. Chest high resolution CT (HRCT) both prompted a diffuse distribution of ground glass in both lungs, thickening of the interlobular septa, and patchy patches in both lungs, with diffuse grid and cable strokes in one case, none seen Cellular changes. One type of tissue was cell type and one type was mixed type. Two patients were given glucocorticoid treatment, the condition improved significantly. Conclusion In children, when the clinical manifestations, chest radiography and other tests suggest that interstitial lung disease can not be a clear cause, the condition to allow lung biopsy to be as much as possible to clear the pathological diagnosis of NSIP and histological types. NSIP patients diagnosed with glucocorticoid therapy can significantly improve the clinical treatment effect, but still need long-term follow-up, combined with history and related auxiliary examination to further identify possible underlying causes.