本文關(guān)鍵詞：彌漫性肺部囊性病變病因分布及鑒別診斷　出處：《北京協(xié)和醫(yī)學(xué)院》2015年碩士論文　論文類型：學(xué)位論文

  更多相關(guān)文章： 彌漫性肺部囊性病變 病因 鑒別診斷 經(jīng)支氣管鏡肺活檢 肺淋巴管肌瘤病

【摘要】：第一部分 彌漫性肺部囊性病變病因分布及鑒別診斷研究背景：彌漫性肺部囊性病變是指肺內(nèi)多發(fā)薄壁含氣空腔病變,病因多樣,鑒別診斷復(fù)雜。研究目的：探討彌漫性肺部囊性病變患者病因分布,描述疾病臨床特征,總結(jié)鑒別診斷要點(diǎn),提出合理的診斷流程。研究方法：回顧性分析北京協(xié)和醫(yī)院肺淋巴管肌瘤病門診收集的患者臨床資料,根據(jù)引起彌漫性肺部囊性病變?cè)\斷標(biāo)準(zhǔn)對(duì)患者進(jìn)行診斷分類,總結(jié)病因分布,描述各疾病人口學(xué)、臨床表現(xiàn)、實(shí)驗(yàn)室檢查、病理學(xué)、影像學(xué)等特點(diǎn),總結(jié)鑒別診斷及診斷流程。研究結(jié)果：共398位患者肺CT表現(xiàn)為彌漫性肺部囊性病變,肺淋巴管肌瘤病287人,在其他111位患者中,病因不明彌漫性肺部囊性病變54人,干燥綜合征17人,其中合并輕鏈沉積病2人,Birt-Hogg-Dube綜合征16人,肺朗格漢斯組織細(xì)胞增生癥7人,肺腫瘤6人,支氣管擴(kuò)張癥5人,肺纖維化2人,馬凡綜合征1人,淀粉樣變1人,先天性肺囊性腺瘤樣畸形1人,顯微鏡下多血管炎1人。各疾病人口學(xué)、臨床表現(xiàn)、實(shí)驗(yàn)室檢查、病理學(xué)、影像學(xué)等特點(diǎn)以及鑒別診斷要點(diǎn),并提出彌漫性肺部囊性病變基線評(píng)估資料和診斷流程。結(jié)論：北京協(xié)和醫(yī)院肺淋巴管肌瘤病門診主要的彌漫性肺部囊性病變病因?yàn)榉瘟馨凸芗×霾?其他主要病因包括干燥綜合征、Birt-Hogg-Dube綜合征、肺朗格漢斯組織細(xì)胞增生癥、肺腫瘤,部分患者病因不明�？赏ㄟ^人口學(xué)特征、血清學(xué)檢查、影像學(xué)檢查、肺部病理、基因等方法鑒別。第二部分經(jīng)支氣管鏡肺活檢在肺淋巴管肌瘤病中的診斷價(jià)值研究目的：經(jīng)支氣管鏡肺活檢在彌漫性肺實(shí)質(zhì)病變中應(yīng)用廣泛,但在肺淋巴管肌瘤病的應(yīng)用尚未被廣泛認(rèn)可。本研究通過分析北京協(xié)和醫(yī)院肺淋巴管肌瘤病門診收集的臨床資料,探討肺淋巴管肌瘤病患者行支氣管鏡肺活檢的陽性率和安全性。研究方法：通過回顧已有病歷資料、電話隨訪、網(wǎng)絡(luò)調(diào)查問卷和電子郵件收集患者支氣管鏡肺活檢資料,并由病理醫(yī)師重新核實(shí)部分病理切片。研究結(jié)果：共47位患者有經(jīng)支氣管鏡肺活檢病理資料,總體來看,經(jīng)支氣管鏡肺活檢在肺淋巴管肌瘤病中的陽性率為42.6%,北京協(xié)和醫(yī)院和其他醫(yī)院的陽性率分別為63.2%及28.6%(p0.05)。每位患者需活檢3塊肺組織用以診斷。氣胸的發(fā)生率是10%,沒有嚴(yán)重出血的患者。結(jié)論：在有經(jīng)驗(yàn)的肺疾病中心經(jīng)支氣管鏡肺活檢可以安全地用于肺淋巴管肌瘤病的診斷,陽性率為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%.
【學(xué)位授予單位】：北京協(xié)和醫(yī)學(xué)院
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2015
【分類號(hào)】：R563

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