肺蛋白沉著症病患接受肺灌洗治療之氧合成效

Pulmonary alveolar proteinosis is a rare syndrome of unclear etiology, which is characterized by intra-alveolar accumulation of lipoproteinaceous material with minimal interstitial inflammation or fibrosis and resulted in decreasing lung compliance and impairing gas exchange. It may relate to congenital abnormality of granulocyte-macrophage colony-stimulating factor (GM-CSF) pathway activity, inhaled toxic substance, and certain hematological or pulmonary infectious diseases. The patients usually present short of breath, cough, progressive exertional dyspnea, and cyanosis. Whole lung lavage is the only effective treatment but unable to cure the disease.
This patient was diagnosed as PAP for 2 years and received several times of whole lung lavage. The acute exacerbation was induced by influenza this time. In this report, we will discuss the procedures of whole lung lavage and its effects on oxygenation, and the importance of long term respiratory care for PAP patients.