两例肺泡蛋白沉积症病例报道并文献复习

发布时间：2018-04-30 16:54

本文选题：肺泡蛋白沉积症 + 支气管镜肺活检　；参考：《蚌埠医学院》2017年硕士论文

【摘要】：目的提高对肺泡蛋白沉积症的的临床特征的认识,减少误诊率及漏诊率,以提高临床医师对该病的临床诊断率及临床治疗疗效。方法对2014年至2015年在我科住院的2例肺泡蛋白沉积症的临床资料进行整理、分析,并回顾性复习2006至2015年10年间发表的国内有关本病的文献资料,分析该病的年龄分布、性别构成、危险因素、临床表现、误诊情况、诊断及鉴别诊断及治疗情况。结果肺泡蛋白沉积症可发生在任何年龄,男性发病多于女性,临床表现主要表现为活动后呼吸困难,咳嗽、咳痰,体征无或少,典型的影像学表现为“铺路石”、“地图样”改变,确诊方法主要为支气管镜肺活检或肺泡灌洗液检查,易误诊为特发性肺纤维化、肺泡细胞癌、肺炎等病,确诊患者多数经肺泡灌洗症状能好转。结论1.肺泡蛋白沉积症的临床表现的特异性不强,通常患者表现为临床症状不典型,体征相对较轻或无,而肺部影像学改变显著,最易误诊为特发性肺纤维化。如果临床上遇到肺部影像学改变明显,而临床表现不典型,且抗生素、激素治疗效果不佳的患者,需考虑到该病可能,需行支气管镜下肺活检或支气管镜下肺泡灌洗明确诊断。2.粉尘接触史及吸烟史可能是本病的危险因素。3.支气管镜下肺组织活检或支气管镜下肺泡灌洗液检查诊断率较高,如发现肺泡腔内出现大量过碘酸雪夫染色阳性的牛奶样或泥浆样物质沉积可明确诊断。4.绝大多数患者经肺泡灌洗治疗症状可得到缓解,且影像学、肺功能及血气分析结果较前好转。
[Abstract]:Objective to improve the understanding of the clinical features of pulmonary alveolar proteinosis and reduce the misdiagnosis rate and missed diagnosis rate so as to improve the clinical diagnosis rate and clinical treatment effect of pulmonary alveolar proteinosis. Methods the clinical data of 2 cases of pulmonary alveolar proteinosis hospitalized in our department from 2014 to 2015 were analyzed, and the data of domestic literature about the disease from 2006 to 2015 were reviewed retrospectively, and the age distribution of the disease was analyzed. Sex composition, risk factors, clinical manifestations, misdiagnosis, diagnosis and differential diagnosis and treatment. Results Pulmonary alveolar proteinosis can occur at any age. The incidence of pulmonary alveolar proteinosis is more in males than in females. The clinical manifestations are dyspnea, cough, expectoration, no or little signs. The typical imaging manifestations are "paving stone". "Map like" changes, the diagnosis method is mainly bronchoscopic lung biopsy or alveolar lavage fluid examination, easily misdiagnosed as idiopathic pulmonary fibrosis, alveolar cell carcinoma, pneumonia and other diseases, the diagnosis of most patients through alveolar lavage symptoms can be improved. Conclusion 1. The clinical manifestation of pulmonary alveolar proteinosis is not specific. Usually, the patients have atypical clinical symptoms, relatively mild or no signs, but the pulmonary imaging changes are significant, the most easily misdiagnosed as idiopathic pulmonary fibrosis. If the lung imaging changes are obvious and the clinical manifestation is not typical, and the effect of antibiotics and hormone therapy is not good, we should consider the possibility of the disease and make a clear diagnosis by bronchoscopic lung biopsy or bronchoscope alveolar lavage. The history of dust exposure and smoking may be the risk factors of the disease. The diagnostic rate of lung biopsy under bronchoscope or alveolar lavage fluid under bronchoscopy was higher. Most of the patients were relieved by alveolar lavage, and the results of imaging, pulmonary function and blood gas analysis were improved.
【学位授予单位】：蚌埠医学院
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R563

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