1、安 徽 医 药 Anhui Medical and Pharmaceutical Journal 2023 Feb,27(2)华产科急救电子杂志,2019,8(2):93-96.DOI:10.3877/cma.j.issn.2095-3259.2019.02.007.14陶亮,黄志.儿童重症肌无力治疗进展 J.儿科药学杂志,2019,25(12):59-63.15WOLFE GI,WARD ES,DE HAARD H,et al.IgG regulation through FcRn blocking:A novel mechanism for the treatment of myasthe
2、nia gravisJ.J Neurol Sci,2021,430:118074.DOI:10.1016/j.jns.2021.118074.16SHIGEO I.Clinical and pathophysiologic relevance of autoantibodies in neonatal myasthenia gravis J.Pediatrics and Neonatology,2021,62(6):581-590.17GILHUS NE,HONG Y.Maternal myasthenia gravis represents a risk for the child thro
3、ugh autoantibody transfer,immunosuppressive therapy and genetic influenceJ.Eur J Neurol,2018,25(12):1402-1409.(收稿日期:2021-11-04,修回日期:2021-12-26)肺泡蛋白沉着症1例并文献复习蒋慧,杨树坤,乔伟作者单位:皖北煤电集团总医院(蚌埠医学院第三附属医院)呼吸与危重症医学科,安徽 宿州234000摘要:目的 探讨肺泡蛋白沉着症(PAP)发病原因、临床特征、肿瘤标志物的动态变化、大容量全肺灌洗术的临床疗效及安全性、提高临床医生对肺泡蛋白沉着症的认识、减少漏诊、误诊。方
4、法 回顾性分析1例肺泡蛋白沉着症病人2020年9月至2021年3月共3次在皖北煤电集团总医院呼吸与危重医学科住院的临床资料(包括吸烟史、职业暴露、临床表现、胸部影像学、肺功能、电子支气管镜检查、实验室检查、大容量全肺灌洗的临床疗效及安全性)。结果 吸烟及职业暴露是PAP的易患因素之一,临床症状缺乏特异性;胸部高分辨率CT(HRCT)提示“铺路石征”,并呈“地图样”分布;肺功能提示一氧化碳(CO)弥散功能重度减低,限制性通气功能障碍;血气分析提示低氧血症并过度通气;肿瘤标志物癌胚抗原(CEA)轻度升高,细胞角蛋白19片段(CYFRA211)明显升高,随着病情好转,数值逐渐下降。共进行3次大容量双
5、肺同期灌洗术,病人临床症状及影像学均明显好转。结论 PAP是一种罕见的呼吸系统疾病,临床表现缺乏特异性,容易误诊,大容量全肺灌洗术治疗肺泡蛋白沉着症安全、有效、值得临床推广应用。关键词:肺泡蛋白沉积症;一氧化碳;临床表现;全肺灌洗术A case of alveolar protein deposition and literature reviewJIANG Hui,YANG Shukun,QIAO WeiAuthor Affiliation:Department of Respiratory and Critical Care Medicine,General Hospital of Wan
6、bei Coal and Electricity Group(The Third Affiliated Hospital of Bengbu Medical College),Suzhou,Anhui 234000,ChinaAbstract:Objective To investigate the pathogenesis,clinical characteristics,dynamic changes in tumor markers,clinical efficacy and safety of large-volume whole-lung lavage,improve clinici
7、ans understanding of alveolar proteinosis,and reduce missed diagnosis and misdiagnosis of pulmonary alveolar proteinosis(PAP).Methods Retrospective analysis of clinical data(including smoking history,occupational exposure,clinical manifestations,chest imaging,pulmonary function,2 electronic bronchos
8、copy,laboratory tests,clinical efficacy and safety of high volume whole lung lavage)of a patient with alveolar protein deposition who was admitted to the Department of Respiratory and Critical Care Medicine of the Wanbei Coal and Electric Power Group General Hospital on a total of three occasions fr
9、om September 2020 to March 2021.Results Smoking and occupational exposure are factors that predispose individuals to PAP,and clinical symptoms are not specific.High-resolution CT of the chest(HRCT)indicated paving stone signs with a map-like distribution.Lung function suggested a severe reduction in
10、 carbon monoxide(CO)diffusion and restrictive ventilation.Blood gas analysis suggested hypoxemia and hyperventilation.Tumor marker carcinoembryonic antigen(CEA)was slightly increased,and cytokeratin 19 fragment(CYFRA211)was significantly increased,with values gradually decreasing as the condition im
11、proved.A total of 3 simultaneous high-volume double lung lavage procedures were performed,and the patient showed significant improvement in clinical symptoms and imaging.Conclusion PAP is a rare respiratory disease with a lack of specificity in clinical manifestations that is easily misdiagnosed.Hig
12、h-volume whole lung lavage is safe,effective and worthy of clinical application in the treatment of alveolar proteinosis.Key words:Pulmonary alveolar proteinosis;Carbon monoxide;Clinical manifestations;Whole lung lavage引用本文:蒋慧,杨树坤,乔伟.肺泡蛋白沉着症1例并文献复习 J.安徽医药,2023,27(2):388-391.DOI:10.3969/j.issn.1009-6
13、469.2023.02.041.临床医学388安 徽 医 药 Anhui Medical and Pharmaceutical Journal 2023 Feb,27(2)肺泡蛋白沉着症(pulmonary alveolar proteinosis,PAP)是一种以肺泡及终末呼吸性细支气管内大量的表面活性蛋白及脂质沉积为特征的罕见疾病。文献报道此病发病率为0.360.49/100万,中位发病年龄为39岁,男女比例为2.1 11。PAP目前病因不明,可能的发病机制为肺表面活性物质增多,刺激巨噬细胞增生并大量吞噬表面活性物质,随之巨噬细胞崩解,表面活性物质再次释放入肺泡腔并堆积成团;或与粒细胞巨噬
14、细胞集落刺激因子(granulocyte-macrophage colony stimutaing factor,GM-CSF)的缺乏有关2-4。PAP 起病隐匿,临床表现缺乏特异性,所以误诊率极高。为提高对本病的认识和确诊率,本研究回顾性分析2020年9月至2021年3月皖北煤电集团总医院收治的1例PAP病人连续3次的住院临床、影像、肺功能、肿瘤标志物的动态变化及大容量肺灌洗的疗效,报告如下。1资料与方法1.1一般资料男,农民,47岁。“因为反复咳嗽咳痰 1年伴进行性加重活动后胸闷 2月”于 2020年 9月25日入院。病人1年前始出现咳嗽,咳少量白色黏痰,多家医院就诊拟诊肺炎,反复予抗感染
15、治疗无效。2月前始出现动则喘息,日常生活明显受限。病程中无发热、纳差、乏力、盗汗,进食可,体质量有所下降。既往无其他疾病史。吸烟30年,每天约20支。从事大理石切割打磨 5 年。体格检查:体温36.5;脉搏 100 次/分;呼吸 26 次/分;血压 125/85 mmHg。精神差,中度喘息貌,唇甲中度紫绀,心率100 次/分,律齐。双肺呼吸音粗,可闻及 Velcor 啰音。无杵状指(趾),外周指脉氧80%(未吸氧)。实验室检查:血常规示白细胞(WBC)7.46109/L,GR 62.7%,LY26.3%,红细胞(RBC)5.711012/L,血红蛋白(HB)169 g/L,血小板(PLT)28
16、8109/L;结核抗体阴性;肺炎支原体阴性;N-端脑钠肽(PBNP)10.4 ng/L;凝血测试正常;肝肾功能、血脂、血糖、电解质、C反应蛋白(CRP)正常;肿瘤标志物甲胎蛋白(AFP)、糖 类 抗 原 19-9(CA19-9)、糖 类 抗 原 125(CA125)、神经元特异性烯醇化酶(NSE)、鳞状细胞癌相关抗原(SCC)正常,癌胚抗原(CEA)6.5 g/L,轻度升高,但细胞角蛋白 19片段(CYFRA211)67.1 g/L,明显升高;甲、乙、丙、丁、戊肝、人类免疫缺陷病毒(HIV)、梅毒等传染病学筛查全阴性;自身抗体12项及血管炎3项检查全阴性;T-SPOT 阴性;真菌G实验 GM 实验阴性;血气分析(吸氧 4 L/min):pH 7.398、二氧化碳分压(PaCO2)20.6 mmHg、氧分压(PaO2)76.2 mmHg.降钙素原0.05 g/L。痰液涂片检查:未见细菌、真菌、抗酸杆菌、革兰阳性球菌少量;培养:阴性。结核菌素试验(PPD)阴性。超声及心电检查:肝、胆、胰腺、脾脏、双肾、输尿管彩超正常;心脏彩超正常;心电图:正常。影像检查:胸部高分辨率CT(HRCT)(20