Belchior I, Cerdeira A S, Santos M, Braga J Sousa, Aragão I, Martins A
Pneumologia, Centro Hospitalar do Porto, Hospital de Santo António, Porto, Portugal.
Rev Port Pneumol. 2011 May-Jun;17(3):139-42. doi: 10.1016/j.rppneu.2011.01.005. Epub 2011 Apr 13.
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by abnormal accumulation of a lipoproteinaceous material in the alveoli, which may lead to respiratory failure and has an associated high risk for infections. The mainstay treatment for PAP is whole lung lavage. A pregnant woman, previously diagnosed with primary PAP, the most common form of PAP, was admitted with dyspnea and worsening respiratory function. In one month period, a whole-lung bronchopulmonary lavage was performed twice, with clinical and functional improvement. Pregnancy was carried to term and a healthy baby was delivered. The mechanisms of respiratory impairment are discussed as well as treatment options and response.
肺泡蛋白沉积症（PAP）是一种罕见的疾病，其特征是肺泡内脂蛋白物质异常积聚，这可能导致呼吸衰竭，并伴有较高的感染风险。PAP的主要治疗方法是全肺灌洗。一名先前被诊断为原发性PAP（PAP最常见的形式）的孕妇因呼吸困难和呼吸功能恶化入院。在一个月的时间里，进行了两次全肺支气管肺灌洗，临床症状和功能均有改善。妊娠足月并分娩出一个健康的婴儿。文中还讨论了呼吸功能损害的机制以及治疗选择和反应。
