弥漫性肺部囊性病变病因分布及鉴别诊断

发布时间：2018-01-02 04:13

本文关键词：弥漫性肺部囊性病变病因分布及鉴别诊断　出处：《北京协和医学院》2015年硕士论文　论文类型：学位论文

【摘要】：第一部分弥漫性肺部囊性病变病因分布及鉴别诊断研究背景：弥漫性肺部囊性病变是指肺内多发薄壁含气空腔病变,病因多样,鉴别诊断复杂。研究目的：探讨弥漫性肺部囊性病变患者病因分布,描述疾病临床特征,总结鉴别诊断要点,提出合理的诊断流程。研究方法：回顾性分析北京协和医院肺淋巴管肌瘤病门诊收集的患者临床资料,根据引起弥漫性肺部囊性病变诊断标准对患者进行诊断分类,总结病因分布,描述各疾病人口学、临床表现、实验室检查、病理学、影像学等特点,总结鉴别诊断及诊断流程。研究结果：共398位患者肺CT表现为弥漫性肺部囊性病变,肺淋巴管肌瘤病287人,在其他111位患者中,病因不明弥漫性肺部囊性病变54人,干燥综合征17人,其中合并轻链沉积病2人,Birt-Hogg-Dube综合征16人,肺朗格汉斯组织细胞增生症7人,肺肿瘤6人,支气管扩张症5人,肺纤维化2人,马凡综合征1人,淀粉样变1人,先天性肺囊性腺瘤样畸形1人,显微镜下多血管炎1人。各疾病人口学、临床表现、实验室检查、病理学、影像学等特点以及鉴别诊断要点,并提出弥漫性肺部囊性病变基线评估资料和诊断流程。结论：北京协和医院肺淋巴管肌瘤病门诊主要的弥漫性肺部囊性病变病因为肺淋巴管肌瘤病,其他主要病因包括干燥综合征、Birt-Hogg-Dube综合征、肺朗格汉斯组织细胞增生症、肺肿瘤,部分患者病因不明。可通过人口学特征、血清学检查、影像学检查、肺部病理、基因等方法鉴别。第二部分经支气管镜肺活检在肺淋巴管肌瘤病中的诊断价值研究目的：经支气管镜肺活检在弥漫性肺实质病变中应用广泛,但在肺淋巴管肌瘤病的应用尚未被广泛认可。本研究通过分析北京协和医院肺淋巴管肌瘤病门诊收集的临床资料,探讨肺淋巴管肌瘤病患者行支气管镜肺活检的阳性率和安全性。研究方法：通过回顾已有病历资料、电话随访、网络调查问卷和电子邮件收集患者支气管镜肺活检资料,并由病理医师重新核实部分病理切片。研究结果：共47位患者有经支气管镜肺活检病理资料,总体来看,经支气管镜肺活检在肺淋巴管肌瘤病中的阳性率为42.6%,北京协和医院和其他医院的阳性率分别为63.2%及28.6%(p0.05)。每位患者需活检3块肺组织用以诊断。气胸的发生率是10%,没有严重出血的患者。结论：在有经验的肺疾病中心经支气管镜肺活检可以安全地用于肺淋巴管肌瘤病的诊断,阳性率为63.2%。
[Abstract]:The first part of the study on the etiological distribution and differential diagnosis of diffuse pulmonary cystic disease: diffuse pulmonary cystic disease refers to multiple pulmonary thin-walled air-filled cavity lesions, the etiology of which is diverse. Objective: to investigate the etiological distribution of patients with diffuse pulmonary cystic disease, to describe the clinical features of the disease, and to summarize the main points of differential diagnosis. Methods: the clinical data collected from the outpatient clinic of pulmonary lymphangiomyomatosis in Peking Union Hospital were analyzed retrospectively. According to the diagnostic criteria of diffuse pulmonary cystic disease, the patients were classified, the etiological distribution was summarized, and the characteristics of demography, clinical manifestation, laboratory examination, pathology, imaging and so on were described. Results: a total of 398 patients presented with diffuse pulmonary cystic lesions, 287 patients with pulmonary lymphangiomyomatosis, and 111 patients with pulmonary lymphangiomyomatosis. There were 54 patients with diffuse pulmonary cystic disease of unknown etiology and 17 patients with Sjogren's syndrome, of whom 2 were complicated with light chain deposition disease and 16 were complicated with Birt-Hogg-Dube syndrome. Lung Langerhans histiocytosis was found in 7 patients, lung tumor in 6, bronchiectasis in 5, pulmonary fibrosis in 2, Marfan's syndrome in 1, amyloidosis in 1, and congenital cystic adenomatoid malformation in 1. Multiple vasculitis under the microscope 1 person. Demography, clinical manifestation, laboratory examination, pathology, imaging and other characteristics of the disease, as well as the main points of differential diagnosis. The baseline evaluation data and diagnostic procedure of diffuse pulmonary cystic disease were put forward. Conclusion: pulmonary lymphangiomyomatosis is the main cause of diffuse pulmonary cystic disease in the clinic of Beijing Union Hospital. Other major causes include Sjogren's syndrome, Birt-Hogg-Dube syndrome, lung Langerhans histiocytosis, and lung tumors. Serology, imaging, lung pathology. Study on the diagnostic value of transbronchial lung biopsy in pulmonary lymphangiomyomatosis objective: transbronchial lung biopsy is widely used in diffuse pulmonary parenchyma lesions. However, the application in pulmonary lymphangiomyomatosis has not been widely recognized. This study analyzed the clinical data collected from the clinic of pulmonary lymphangiomyomatosis in Peking Union Hospital. Objective: to investigate the positive rate and safety of bronchoscopic lung biopsy in patients with pulmonary lymphangiomyomatosis. The data of bronchoscopic lung biopsy were collected by network questionnaire and email, and some pathological sections were re-verified by pathologist. Results: 47 patients had pathological data of bronchoscopic lung biopsy. Overall, the positive rate of transbronchial lung biopsy in pulmonary lymphangiomyomatosis was 42.6%. The positive rates of Beijing Union Hospital and other hospitals were 63.2% and 28.6% respectively. Each patient needed to biopsy 3 pieces of lung tissue for diagnosis. The incidence of pneumothorax was 10%. Conclusion: transbronchial lung biopsy can be safely used in the diagnosis of pulmonary lymphangiomyomatosis with a positive rate of 63.2%.
【学位授予单位】：北京协和医学院
【学位级别】：硕士
【学位授予年份】：2015
【分类号】：R563

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