儿童肺泡蛋白沉积症

Pediatric pulmonary alveolar proteinosis

肺泡蛋白沉积症是一种儿科罕见病，因肺泡腔内充满大量脂质蛋白物质为而造成的逐渐加重的呼吸困难和低氧血症。经肺高分辨CT和病理检查不难做出诊断。在全肺灌洗基础上，结合疾病分型给予相应治疗可有效改善病情。现将对儿童肺泡蛋白沉积症的病因、发病机制、临床特征、治疗和预后等方面内容进行综述。

Pulmonary alveolar proteinosis is a rare disease in children. The accumulation of massive amount of lipid and protein in the alveolar can lead to the progressive dyspnea and hypoxemia. The diagnosis could be made with the high resolution computed tomography and pathological findings. Appropriate treatments according to the pathogene- sis ,together with whole lung lavage ,can significantly improve disease outcome. This article will provide a review of the pathogenesis, clinical features, therapeutic approach and disease prognosis of pediatric pulmonary alveolar proteinosis.