肺泡蛋白沉着症1例并文献复习

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。

A case of alveolar protein deposition and literature review

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.