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目的 :提高对特发性肺间质纤维化临床、实验室所见的认识及其诊断水平。方法 :回顾性分析 18例特发性肺间质纤维化的临床资料 ,列举和分析常见症状、体征及实验室检查。结果 :肺功能检查示 ,弥散功能障碍、限制性通气功能障碍和小气道功能障碍分别占 85 .8%、71.4 %和 14 .3%。X线胸片表现为肺间质纤维化者占 87.5 % ,其余只表现为肺纹理增粗、模糊或斑片阴影。动脉血氧分压 (PaO2 )减低者占 5 5 .6 % ,PaO2 减低者病死率为 80 % ,PaO2 正常者病死率为 14 .3%。主要症状为活动后胸闷、气短及咳嗽者 ,分别占 88.9%和 77.8%。有肺部Velcro音及杵状指者分别占 88.9%和 5 5 .6 %。本组病例误诊率高达 10 0 %。结论 :了解特发性肺间质纤维化临床表现和实验室所见 ,对降低误诊率、提高本病诊断意识是重要的
Objective: To improve the understanding of idiopathic pulmonary fibrosis clinical and laboratory findings and their diagnostic level. Methods: A retrospective analysis of 18 cases of idiopathic pulmonary fibrosis clinical data, enumeration and analysis of common symptoms, signs and laboratory tests. Results: Pulmonary function tests showed diffuse dysfunction, restrictive ventilatory dysfunction and small airway dysfunction accounted for 85.8%, 71.4% and 14.3% respectively. X-ray showed pulmonary interstitial fibrosis who accounted for 87.5%, the rest showed only lung thickening, fuzzy or patch shadow. Arterial oxygen partial pressure (PaO2) decreased accounted for 55.6%, PaO2 reduced mortality was 80%, PaO2 normal mortality was 14.3%. The main symptoms of chest tightness after the event, shortness of breath and cough, accounted for 88.9% and 77.8%. There were 88.9% and 55.6% of patients with lung Velcro and clubbing respectively. Misdiagnosis rate of this group of patients as high as 10%. CONCLUSIONS: Understanding the clinical manifestations and laboratory findings of idiopathic pulmonary fibrosis is important for reducing the rate of misdiagnosis and improving the diagnosis of this disease