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静脉-静脉体外膜肺氧合辅助下全肺灌洗治疗重症肺泡蛋白沉积症:1例报告

Whole-lung lavage for severe pulmonary alveolar proteinosis assisted by veno-venous extracorporeal membrane oxygenation: a case report.

作者信息

Moreira João P, Ferraz Sofia, Freitas Cláudia, Morais António, Albuquerque Roberto R, Fiuza Carlos

机构信息

Department of Anesthesiology, Centro Hospitalar São João, Porto, Portugal.

Department of Pneumology, Centro Hospitalar São João, Porto, Portugal.

出版信息

Can J Respir Ther. 2018 Dec;55(1):9-12. doi: 10.29390/cjrt-2018-019. Epub 2018 Dec 5.

Abstract

Pulmonary alveolar proteinosis (PAP) is a rare pathology characterized by accumulation of phospholipoproteinaceous material within the alveoli. The evolution of PAP is variable and treatment modalities are limited. Pharmacological therapeutic targets are being actively developed, but whole-lung lavage (WLL), first described in the 1960s, remains the cornerstone of therapy. The preferential treatment for PAP in our center is sequential WLL, where each lung is separately and sequentially perfused with warmed saline. However, some patients do not tolerate single lung ventilation (SLV), as there is a greater risk of severe hypoxemia with this method. Extracorporeal membrane oxygenation (ECMO), referring to an extracorporeal circuit that directly oxygenates and removes carbon dioxide from the blood, may be considered in highly selected patients with severe respiratory failure who otherwise would not be able to undergo WLL. In this context, veno-venous ECMO is most often utilized. We describe a case of a 44-year-old male diagnosed with silicosis five years earlier who presented with severe hypoxemic respiratory failure not amenable to WLL under general anesthesia with SLV, which was successfully managed with ECMO-assisted WLL.

摘要

肺泡蛋白沉积症(PAP)是一种罕见的病理状况,其特征为肺泡内积聚磷脂蛋白样物质。PAP的病情发展具有变异性,治疗方式有限。目前正在积极研发药物治疗靶点,但20世纪60年代首次描述的全肺灌洗(WLL)仍是治疗的基石。我们中心对PAP的首选治疗方法是序贯WLL,即分别依次用温热盐水灌注每侧肺。然而,一些患者无法耐受单肺通气(SLV),因为这种方法导致严重低氧血症的风险更高。体外膜肺氧合(ECMO)是指一种直接对血液进行氧合并清除二氧化碳的体外循环装置,对于因严重呼吸衰竭而无法进行WLL的高度选择性患者,可考虑使用。在此情况下,最常采用的是静脉-静脉ECMO。我们报告一例44岁男性病例,该患者5年前被诊断为矽肺,现出现严重低氧性呼吸衰竭,在全身麻醉下单肺通气时无法耐受WLL,最终通过ECMO辅助WLL成功治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/71c7/6345579/a04ee128d54d/cjrt-2018-019-g001.jpg

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