Ju Renhua, Du Xiaonan, Yin Ling, Yu Yang
Department of Anesthesia, Tianjin Medical University General Hospital, PR China.
Tianjin Institute of Anesthesiology, Tianjin, 300052, PR China.
Respir Med Case Rep. 2025 Apr 17;55:102206. doi: 10.1016/j.rmcr.2025.102206. eCollection 2025.
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by the accumulation of surfactant-derived lipoproteins within alveoli and impaired macrophage function, leading to progressive dyspnea, hypoxemic respiratory failure, secondary infections, and pulmonary fibrosis. We report a case of a 43-year-old male with a history of occupational exposure to airborne dust from lathe work, presenting with exertional dyspnea. High-resolution computed tomography (HRCT) revealed bilateral patchy ground-glass opacities with interlobular septal thickening. Histopathological analysis of lung biopsy specimens showed eosinophilic amorphous material in alveolar spaces, which exhibited positive periodic acid-Schiff (PAS) staining with diastase resistance, confirming PAP. The patient underwent whole-lung lavage (WLL) of the right lung under general anesthesia. Severe baseline hypoxemia complicated intraoperative oxygen saturation maintenance. The intermittent use of high-frequency jet ventilation (HFJV) in the operative lung markedly improved oxygenation (SpO increased from 85 % to 96 %) while ensuring effective saline distribution into distal alveoli. The procedure was completed without complications, highlighting the efficacy and safety of HFJV in managing complex airway conditions during WLL for PAP.
肺泡蛋白沉积症(PAP)是一种罕见的疾病,其特征是肺泡内表面活性物质衍生的脂蛋白积聚以及巨噬细胞功能受损,导致进行性呼吸困难、低氧性呼吸衰竭、继发感染和肺纤维化。我们报告一例43岁男性病例,有车床作业空气传播粉尘职业暴露史,表现为劳力性呼吸困难。高分辨率计算机断层扫描(HRCT)显示双侧斑片状磨玻璃影伴小叶间隔增厚。肺活检标本的组织病理学分析显示肺泡腔内有嗜酸性无定形物质,对淀粉酶具有抗消化性,高碘酸-希夫(PAS)染色呈阳性,确诊为PAP。患者在全身麻醉下接受了右肺全肺灌洗(WLL)。严重的基线低氧血症使术中维持氧饱和度变得复杂。在手术肺中间歇性使用高频喷射通气(HFJV)显著改善了氧合(血氧饱和度从85%提高到96%),同时确保有效盐水分布到远端肺泡。手术顺利完成,无并发症,突出了HFJV在PAP患者WLL期间处理复杂气道情况时的有效性和安全性。