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肺纤维化合并肺气肿(CPFE)综合征7例病例报告并文献复习

发布时间:2018-10-17 09:27
【摘要】:目的 1.分析肺纤维化合并肺气肿(CPFE)综合征患者的一般资料、临床表现及体征、肺功能、肺动脉压、肺癌标志物、CT表现及预后等临床资料,为临床诊断及治疗提供参考。 2.通过对相关文献的系统复习,综合分析近十年来国内外对肺气肿合并肺纤维化综合征的认识。 方法 1.收集山东省千佛山医院2010年1月到2012年12月诊断的7例CPFE综合征的患者。 2.分析所收集患者的一般资料、临床表现及体征、肺功能、肺动脉压力、肺癌标志物及CT表现。 3.结合临床文献分析进行归纳、总结肺纤维化合并肺气肿(CPFE)综合征的临床特点。 结果 1.7例患者中6例为男性,1例为女性,年龄62-84岁,中位年龄77.4岁;6例有吸烟史,其中有1例从事酒渣运输工作40余年。全部病例均有活动后呼吸困难,双下肺均可闻及吸气相VelcroUp音。 2.肺功能结果[中位数(范围)]:FEVl%pred平均为73%(39%~98%),FVC%pred平均为73%(54%~101%),FEV1/FVC%平均为81%(61%~96%),VC%pred平均为79%(57%~86%),TLC%pred平均为85%(53%~110%),RV/TLC%平均为41%(21%~58%)。DLco%pred平均为46%(20%~58%),DLco/VA%平均为53%(19%~98%)。 3.有4例有肺动脉高压,平均为45mmHg(32mmHg~69mmHg)。肺癌标志物中有2例出现NSE升高。 4.胸部CT可见双上肺肺气肿及双下肺磨玻璃样、网格状、蜂窝样及牵拉性支气管扩张的肺纤维化表现。 结论 1. CPFE综合征患者好发于男性有吸烟史的患者。 2.临床特征包括呼吸困难、低氧血症、肺动脉高压。 3.肺功能表现为肺容积相对正常而弥散能力显著下降。 4.高分辨率CT的特点为同时存在上肺野肺气肿和下肺野纤维化。 5.其常见并发症为肺动脉高压、急性肺损伤、肺癌。
[Abstract]:Objective 1. To analyze the general data, clinical manifestations and signs, pulmonary function, pulmonary artery pressure, lung cancer markers, CT manifestations and prognosis in patients with pulmonary fibrosis complicated with emphysema (CPFE) syndrome, and to provide reference for clinical diagnosis and treatment. 2. Based on the systematic review of related literature, the understanding of emphysema complicated with pulmonary fibrosis syndrome in recent ten years was analyzed. Method 1. A total of 7 patients with CPFE syndrome diagnosed from January 2010 to December 2012 in Qianfushan Hospital of Shandong Province were collected. The general data, clinical manifestations and signs, pulmonary function, pulmonary artery pressure, lung cancer markers and CT findings were analyzed. The clinical features of pulmonary fibrosis combined with emphysema (CPFE) syndrome were summarized. Results among the 1.7 patients, 6 were male and 1 female, aged from 62 to 84 years, with a median age of 77.4 years, and 6 cases had a history of smoking, of which 1 case was engaged in the transport of wine residue for more than 40 years. All of the patients had dyspnea after movement, and both lower lungs could hear and inhale VelcroUp 2. 2. 鑲哄姛鑳界粨鏋淸涓綅鏁,

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