Hasan Naveed, Bagga Sidharth, Monteagudo Julie, Hirose Hitoshi, Cavarocchi Nicholas C, Hehn Boyd T, Kavuru Mani S
Department of Pulmonary/Critical Care Medicine, Thomas Jefferson University, Philadelphia, PA, USA.
J Bronchology Interv Pulmonol. 2013 Jan;20(1):41-4. doi: 10.1097/LBR.0b013e31827ccdb5.
Pulmonary alveolar proteinosis is a rare lung disease characterized by accumulation of lipoproteinaceous material within the alveoli. Therapeutic whole-lung lavage (WLL) under general anesthesia is the standard treatment in patients with progressive symptomatic disease. Severe hypoxemic respiratory failure is uncommon, yet when present poses a technical challenge to performing WLL without further compromising respiratory status. Rarely, hyperbaric chamber or extracorporeal membrane oxygenation (ECMO) has been utilized to perform WLL to manage severe hypoxemia, with venovenous ECMO being used more often. We present a case of hypoxemic and hypercarbic respiratory failure from pulmonary alveolar proteinosis successfully managed by placing the patient on venoarterial ECMO to facilitate the performance of bilateral WLL.
肺泡蛋白沉积症是一种罕见的肺部疾病，其特征是肺泡内脂蛋白物质积聚。全身麻醉下的治疗性全肺灌洗（WLL）是进展性症状性疾病患者的标准治疗方法。严重低氧性呼吸衰竭并不常见，但一旦出现，在不进一步损害呼吸状态的情况下进行WLL会带来技术挑战。很少有情况下，会使用高压氧舱或体外膜肺氧合（ECMO）来进行WLL以处理严重低氧血症，其中静脉-静脉ECMO使用得更为频繁。我们报告一例肺泡蛋白沉积症导致的低氧性和高碳酸血症性呼吸衰竭病例，通过将患者置于静脉-动脉ECMO上以促进双侧WLL的实施而成功得到治疗。
