血脂水平在肺泡蛋白沉积症中的临床意义

发布时间：2018-02-24 08:15

本文关键词： 肺泡蛋白沉积症脂蛋白a　出处：《大连医科大学》2013年硕士论文　论文类型：学位论文

【摘要】：目的：肺泡蛋白沉积症（Pulmonary alveolar proteinosis，PAP）为弥漫性实质性肺疾病中的一种，以肺泡及呼吸性终末细支气管腔内充满不可溶性富磷脂蛋白样物质为特征，，导致气体交换受损，出现限制性呼吸困难，严重者呼吸衰竭或重症感染而死亡，临床以隐袭性渐进性呼吸困难为主要表现，属于临床少见病。肺脏是重要的内分泌和代谢器官，其自身可以合成胆固醇、脂质等，但大量的胆固醇来自血清脂蛋白。通过肺泡灌洗液及细胞学检查已证实该病为肺泡腔内脂质和蛋白质的异常积聚所致，属于肺泡表面活性物质自身代谢性疾病，但血清中的脂质与肺泡蛋白沉积症之间是否存在相关尚不明确。本文通过分析7例肺泡蛋白沉积症患者临床资料并结合相关文献复习，总结该病的特点，进一步提高临床认识。方法：回顾性分析2000-2012年于大连医科大学附属第一医院住院的7例经病理确诊为肺泡蛋白沉积症患者的血脂、动脉血气等临床结果，并结合Pub Med检索包含pulmonary alveolar proteinosis和serum lipid的免费全文进行文献复习并讨论。结果：7例患者男性6例、女性1例，平均年龄56.85±8.51岁；咳嗽5例（71.4%），咳痰3例（42.9%），进行性呼吸困难7例（100%）；紫绀2例（28.6%）；杵状指2例（28.6%）；肺部湿性Up音5例（71.4%）；经实验室检查均存在脂代谢紊乱：甘油三酯升高2例（160.86±66.22mg/dl）、总胆固醇升高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例患者经腹部超声证实存在脂肪肝。各变量服从正态分布，采用Pearman相关检验：脂蛋白a分别与氧分压、肺泡动脉氧分压差的相关性（r＝-0.792，p＝0.034＜0.05；r＝0.583，p＝0.169），脂蛋白每增加一个单位，氧分压下降0.069mmHg；血清甘油三酯、胆固醇、低密度脂蛋白、高密度脂蛋白与动脉血气分析之间无相关性。结论：脂蛋白a水平与疾病程度呈负相关，有望成为评价疾病严重程度的指标。
[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 卤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 卤66.22 mg 路dl-1), and the increase of total cholesterol, respectively. 5 patients (229.14 卤56.46mg / dlg / dlg), 5 patients with elevated low density lipoprotein (LDL-C) (143.43 卤39.11mg / dlg), 3 patients with elevated lipoprotein (253.5 卤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.
【学位授予单位】：大连医科大学
【学位级别】：硕士
【学位授予年份】：2013
【分类号】：R563.8

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