Awab Ahmed, Khan Muhammad S, Youness Houssein A
University of Oklahoma Health Sciences Center, Pulmonary and Critical Care Section, Oklahoma, USA.
J Thorac Dis. 2017 Jun;9(6):1697-1706. doi: 10.21037/jtd.2017.04.10.
Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with resulting hypoxemia and reduced lung function. Whole lung lavage (WLL) to physically remove the proteinaceous material from the affected lung is the standard treatment. Since its original description in 1964, there have been increasing numbers of WLL procedures done worldwide and the technique has been variously refined and modified. When done in experienced centers, WLL provides long lasting benefit in the majority of patients. It is considered safe and effective. There are no guidelines standardizing the procedure. Our preferred method is to lavage one lung at a time, with the patient supine, filling to functional residual capacity (FRC) and repeating cycles of drainage and instillation with chest percussion until the effluent is clear. The aim of this article is to provide a detailed description of the technique, equipment needed and logistic considerations as well as providing a physiologic rationale for each step of WLL. We will also review the available data concerning variations of the technique described in the literature.
肺泡蛋白沉积症（PAP）是一种罕见疾病，其特征为肺泡表面活性物质积聚，导致低氧血症和肺功能下降。通过全肺灌洗（WLL）从患肺中物理清除蛋白质物质是标准治疗方法。自1964年首次描述以来，全球范围内进行的WLL手术数量不断增加，该技术也得到了各种改进和改良。在经验丰富的中心进行时，WLL能为大多数患者带来持久益处。它被认为是安全有效的。目前尚无规范该手术的指南。我们首选的方法是让患者仰卧，每次灌洗一侧肺，灌至功能残气量（FRC），并重复进行引流和滴注循环，同时进行胸部叩击，直至流出液清澈。本文旨在详细描述该技术、所需设备和后勤考虑因素，并为WLL的每一步提供生理学依据。我们还将回顾文献中有关该技术变体的现有数据。
