肺泡蛋白沉积症的临床分析和诊治的探讨

Clinical analysis,research of diagnosis and treatment of pulmonary alveolar proteinosis

目的通过临床病例分析结合文献资料以提高对该病的诊断水平。方法回顾我院自2000年至2007年1月收治的6例肺泡蛋白沉积症(PAP)病人结合文献进行分析。结果6例患者中男性4例,女性2例。以30～50岁为主。渐进性呼吸困难是最主要的临床表现。胸片和CT表现为双肺弥漫性病变。高分辨CT上可有特征性的"铺路石样"或"地图样"改变。肺功能主要表现为限制性通气功能障碍、弥散障碍和低氧血症。6例均经纤维支气管镜下肺泡灌洗和肺活检获得确诊。全肺灌洗是治疗该病最有效的方法。结论PAP虽然是少见病,但由于其在HRCT上的特殊表现,以及通过纤维支气管镜下的灌洗液及肺活检对该病的重要诊断价值,提高对该病的认识可大大减少通过开胸肺活检诊断给病人带来的创伤和经济的负担。

Objective To elvaluate the clinical and conbine medical document of pulmonary alveolar proteinosis and improve the familiarity and diagnosis ability of this rare disease through olinical cases analysis. Methods Retrospectively analyzing 6 the clinical characteristics of patients with confirmed pulmonary alveolar proteinosis（PAP） involved from 2000 to January 2007 in affiliated Li-Hui-Li Hospital in Ningbo. Results There were 6 cases of PAP （ male ： female 4 ： 2） with an average age of 44.3 （ 30 - 50） years old. One case among them had history of dust exposure. Progressing dyspnea is the most common symptom, cough and expectoration are also common. Chest film or CT suggests diffuse consolidation. HRCT indicated the characteristic changes of lung marking and crazy paving appearance. Pulmonary function changes include restrictive pattern, decreased diffusion capacity and hypoxemia. There were 6 cases confirmed by diagnostic bronchoalveolar lavage and trausbronchial lung biopsy. The most effective measure of treatment for PAP is whole lung lavage. Conclusion PAP is rare, but as long as we improve the familiarity and through diagnostic bronchoalveolar lavage and transbronchial lung biopsy combined with the typical features on HRCT, Not only can we gain an accurate diagnosis, which greatly deduce surgical lung biopsy. but also can decrease the patient＇s expenditure ou medical treatment and alleviate the patient＇s suffering.