一例采用全肺灌洗治疗的肺泡蛋白沉积症病例。

Bansal Avdhesh, Sikri Vikas

Department of Respiratory, Critical Care and Sleep Medicine, Indraprastha Apollo Hospital, New Delhi, India.

Indian J Crit Care Med. 2013 Sep;17(5):314-7. doi: 10.4103/0972-5229.120327.

Pulmonary alveolar proteinosis represents a rare syndrome characterized by the accumulation of proteinaceous phospholipid-laden material in the alveoli. This leads to impaired gas exchange and arterial hypoxemia of varying degrees. The diagnosis is confirmed by lung biopsy. Sequential whole-lung lavage (WLL) first described in 1963 is the standard of care. We report a case of a male diagnosed of having pulmonary alveolar proteinosis (PAP) on transbroncial lung biopsy (TBLB). He was treated with sequential WLL (Left followed by right, Left being more involved on chest X-ray) followed by recombinant GM-CSF, with good result.

肺泡蛋白沉积症是一种罕见的综合征，其特征是肺泡内积聚含蛋白质的磷脂物质。这会导致气体交换受损和不同程度的动脉低氧血症。通过肺活检可确诊。1963年首次描述的序贯全肺灌洗（WLL）是标准治疗方法。我们报告一例男性病例，经支气管肺活检（TBLB）诊断为肺泡蛋白沉积症（PAP）。他接受了序贯WLL（先左后右，胸部X线显示左肺受累更重）治疗，随后使用重组粒细胞巨噬细胞集落刺激因子（GM-CSF），效果良好。