肺泡蛋白沉积症3例临床分析

Clinical features of three pulmonary alveolar proteinosis patients

目的探讨肺泡蛋白沉积症(PAP)的诊断和治疗。方法回顾分析2010年至2016年收治的3例PAP患儿的临床资料。结果 3例患儿均为女性,分别为3岁1个月、3岁9个月、6岁4个月,均以咳嗽为主要表现,伴或不伴气促,其中2例病程较长,常规治疗疗效欠佳,胸部CT均提示明显病变,行纤维支气管镜检查PAS染色可见阳性反应,经肺泡灌洗治疗后好转。结论 PAP以咳嗽、气促等为主要临床表现,缺乏特异性,PAS染色及肺泡灌洗对其诊断及治疗有重要价值。

Objective To investigate the diagnosis and treatment of pulmonary alveolar proteinosis （PAP）. Methods Clinical data of 3 children with PAP admitted from 2010 to 2016 were retrospectively analyzed. Results Three children were female and aged 3 years and 1 month, 3 years and 9 months, and 6 years and 4 months, respectively. The main symptom of the three children was cough along with or without anhelation. Two of them had longer course of disease. The therapeutic effect of conventional treatment was poor, and their chest CT indicated significant lesions. Fiberoptic bronchoscopy showed positive reaction by PAS staining, and the treatment effect was improved after bronchoalveolar lavage. Conclusions The main clinical symptoms of PAP are non-specific cough and shortness of breath. PAS staining and bronchoalveolar lavage are of great value in the diagnosis and treatment of PAP.