本文關(guān)鍵詞： 肺泡蛋白沉積癥 脂蛋白a　出處：《大連醫(yī)科大學(xué)》2013年碩士論文　論文類型：學(xué)位論文

【摘要】：目的：肺泡蛋白沉積癥（Pulmonary alveolar proteinosis，PAP）為彌漫性實(shí)質(zhì)性肺疾病中的一種，以肺泡及呼吸性終末細(xì)支氣管腔內(nèi)充滿不可溶性富磷脂蛋白樣物質(zhì)為特征，，導(dǎo)致氣體交換受損，出現(xiàn)限制性呼吸困難，嚴(yán)重者呼吸衰竭或重癥感染而死亡，臨床以隱襲性漸進(jìn)性呼吸困難為主要表現(xiàn)，屬于臨床少見病。肺臟是重要的內(nèi)分泌和代謝器官，其自身可以合成膽固醇、脂質(zhì)等，但大量的膽固醇來自血清脂蛋白。通過肺泡灌洗液及細(xì)胞學(xué)檢查已證實(shí)該病為肺泡腔內(nèi)脂質(zhì)和蛋白質(zhì)的異常積聚所致，屬于肺泡表面活性物質(zhì)自身代謝性疾病，但血清中的脂質(zhì)與肺泡蛋白沉積癥之間是否存在相關(guān)尚不明確。本文通過分析7例肺泡蛋白沉積癥患者臨床資料并結(jié)合相關(guān)文獻(xiàn)復(fù)習(xí)，總結(jié)該病的特點(diǎn)，進(jìn)一步提高臨床認(rèn)識。 方法：回顧性分析2000-2012年于大連醫(yī)科大學(xué)附屬第一醫(yī)院住院的7例經(jīng)病理確診為肺泡蛋白沉積癥患者的血脂、動(dòng)脈血?dú)獾扰R床結(jié)果，并結(jié)合Pub Med檢索包含pulmonary alveolar proteinosis和serum lipid的免費(fèi)全文進(jìn)行文獻(xiàn)復(fù)習(xí)并討論。 結(jié)果：7例患者男性6例、女性1例，平均年齡56.85±8.51歲；咳嗽5例（71.4%），咳痰3例（42.9%），進(jìn)行性呼吸困難7例（100%）；紫紺2例（28.6%）；杵狀指2例（28.6%）；肺部濕性Up音5例（71.4%）；經(jīng)實(shí)驗(yàn)室檢查均存在脂代謝紊亂：甘油三酯升高2例（160.86±66.22mg/dl）、總膽固醇升高5例（229.14±56.46mg/dl）、低密度脂蛋白升高5例(143.43±39.11mg/dl)，脂蛋白（a）升高3例(253.5±121.5mg/l)，2例患者經(jīng)腹部超聲證實(shí)存在脂肪肝。各變量服從正態(tài)分布，采用Pearman相關(guān)檢驗(yàn)：脂蛋白a分別與氧分壓、肺泡動(dòng)脈氧分壓差的相關(guān)性（r＝-0.792，p＝0.034＜0.05；r＝0.583，p＝0.169），脂蛋白每增加一個(gè)單位，氧分壓下降0.069mmHg；血清甘油三酯、膽固醇、低密度脂蛋白、高密度脂蛋白與動(dòng)脈血?dú)夥治鲋g無相關(guān)性。 結(jié)論：脂蛋白a水平與疾病程度呈負(fù)相關(guān)，有望成為評價(jià)疾病嚴(yán)重程度的指標(biāo)。
[Abstract]:Objective: pulmonary alveolar proteinosism (PAPs) is a diffuse pulmonary disease characterized by insoluble phosphatidylphospholipin-rich substances in the alveolar and respiratory terminal bronchioles, which results in impaired gas exchange. Restricted breathing difficulties, severe respiratory failure or severe infection, the main clinical manifestations of progressive dyspnea, belong to clinical rare disease. Lung is an important endocrine and metabolic organs, It can synthesize cholesterol, lipid, etc., but a large amount of cholesterol comes from serum lipoprotein. It has been proved by alveolar lavage fluid and cytology that the disease is caused by abnormal accumulation of lipid and protein in alveolar cavity. It is a metabolic disease of alveolar surfactant, but the correlation between serum lipid and alveolar proteinosis is not clear. The clinical data of 7 patients with alveolar proteinosis were analyzed and the related literatures were reviewed. Summarize the characteristics of the disease and further improve the clinical understanding. Methods: the clinical results of blood lipid and arterial blood gas in 7 patients with pulmonary alveolar proteinosis diagnosed pathologically from 2000 to 2012 in the first affiliated Hospital of Dalian Medical University were retrospectively analyzed. The free full text of pulmonary alveolar proteinosis and serum lipid is reviewed and discussed in combination with Pub Med search. Results there were 6 males and 1 female in 7 cases with an average age of 56.85 鹵8.51 years. There were 5 cases of cough, 3 cases of expectoration, 7 cases of progressive dyspnea, 100 cases of chronic dyspnea, 2 cases of cyanosis, 2 cases of cyanosis, 2 cases of clubbing finger, 2 cases of clubbing finger, 2 cases of clubbing finger and 2 cases of phlegm, 5 cases of pulmonary wet up sound, 5 cases of dyslipidemia, and 2 cases of elevated triglyceride in 2 cases (160.86 鹵66.22 mg 路dl-1), and the increase of total cholesterol, respectively. 5 patients (229.14 鹵56.46mg / dlg / dlg), 5 patients with elevated low density lipoprotein (LDL-C) (143.43 鹵39.11mg / dlg), 3 patients with elevated lipoprotein (253.5 鹵121.5mg / d) and 2 patients with fatty liver confirmed by abdominal ultrasound. Pearman correlation test was used: the correlation between lipoprotein a and oxygen partial pressure, alveolar arterial oxygen pressure difference was r = -0.792p ~ (0.034) < 0.05 ~ 0.583p ~ (-1) ~ 0.169ng. The oxygen partial pressure decreased by 0.069mmHg for every unit of lipoprotein, serum triglyceride, cholesterol, low density lipoprotein (LDL), and serum triglyceride (TG), cholesterol (TC), low density lipoprotein (LDL). There was no correlation between HDL and arterial blood gas analysis. Conclusion: the level of lipoprotein a is negatively correlated with the degree of disease, which may be an index to evaluate the severity of the disease.
【學(xué)位授予單位】：大連醫(yī)科大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2013
【分類號】：R563.8

【參考文獻(xiàn)】

相關(guān)期刊論文 前3條

1 楊光釗,葉曉雪,李森華;肺泡蛋白沉積癥的高分辨率CT表現(xiàn)[J];中華放射學(xué)雜志;2002年05期

2 徐凱峰,陳燕,郭子健,朱元玨;肺泡蛋白沉積癥患者血清中抗粒-巨噬細(xì)胞集落刺激因子抗體等血清學(xué)指標(biāo)的臨床意義[J];中華結(jié)核和呼吸雜志;2004年12期

3 王繼旺;殷凱生;;肺泡蛋白沉積癥的研究進(jìn)展[J];中華結(jié)核和呼吸雜志;2007年05期

本文編號：1529516