| 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. |
| 肺泡蛋白沉着症 1例并文献复习 |
| A case of alveolar protein deposition and literature review |
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| DOI：10.3969/j.issn.1009-6469.2023.02.041 |
| 中文关键词: 肺泡蛋白沉积症 一氧化碳 临床表现 全肺灌洗术 |
| 英文关键词: Pulmonary alveolar proteinosis Carbon monoxide Clinical manifestations Whole lung lavage |
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| 中文摘要: |
| 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。 |
| 英文摘要: |
| Objective To investigate the pathogenesis, clinical characteristics, dynamic changes in tumor markers, clinical efficacyand safety of large-volume whole-lung lavage, improve clinicians' understanding of alveolar proteinosis, and reduce missed diagnosisand misdiagnosis of pulmonary alveolar proteinosis (PAP).Methods Retrospective analysis of clinical data (including smoking histo-ry, occupational exposure, clinical manifestations, chest imaging, pulmonary function,2 electronic bronchoscopy, laboratory tests, clini-cal efficacy and safety of high volume whole lung lavage) of a patient with alveolar protein deposition who was admitted to the Depart-ment of Respiratory and Critical Care Medicine of the Wanbei Coal and Electric Power Group General Hospital on a total of three occa-sions from 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 carbon monoxide (CO) diffusion and restrictive ventilation. Blood gas analy-sis suggested hypoxemia and hyperventilation.Tumor marker carcinoembryonic antigen (CEA) was slightly increased, and cytokeratin19 fragment (CYFRA211) was significantly increased,with values gradually decreasing as the condition improved. A total of 3 simulta-neous high-volume double lung lavage procedures were performed,and the patient showed significant improvement in clinical symptomsand imaging.Conclusion PAP is a rare respiratory disease with a lack of specificity in clinical manifestations that is easily misdiag-nosed.High-volume whole lung lavage is safe, effective and worthy of clinical application in the treatment of alveolar proteinosis. |
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