大容量全肺灌洗联合粒细胞-巨噬细胞集落刺激因子吸入治疗重度肺泡蛋白沉积症1例并文献复习

Wang Yun , Wu Xue-ling , Wang Qian-yu , Jiang Peng , Liao Jiang-rong
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引用次数: 0

摘要

肺泡蛋白沉积症(PAP)是一种罕见的肺部疾病,其特征是肺泡间隙积聚大量磷脂蛋白样表面活性剂物质,导致严重低氧血症和呼吸衰竭。这种疾病起病隐匿,早期难以诊断。其临床表现主要包括进行性呼吸困难和咳嗽。目前,全肺灌洗(WLL)是PAP的首选且有效的治疗方法,因为它可以改善肺泡通气功能。粒细胞集落刺激因子在临床上被用于治疗多种疾病。雾化粒细胞-巨噬细胞集落刺激因子(GM-CSF)对PAP有一定的治疗作用。在此,我们报告了一例PAP患者,WLL联合雾化GM-CSF治疗,在4年的随访期内取得了良好的治疗效果,以增强对该疾病的了解。
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Large volume whole lung lavage combined with granulocyte-macrophage colony-stimulating factor inhalation in the treatment of severe pulmonary alveolar proteinosis: A case report and literature review

Pulmonary alveolar proteinosis (PAP) is a rare lung disease characterized by the accumulation of large amounts of phospholipid protein-like surfactant material in the alveolar spaces, leading to severe hypoxemia and respiratory failure. The disease has an insidious onset and is difficult to diagnose in the early stages. Its clinical manifestations primarily include progressive dyspnea and coughing. Currently, whole lung lavage (WLL) is the preferred and effective treatment for PAP, as it improves alveolar ventilation function. Granulocyte colony-stimulating factor is used clinically in the treatment of a variety of disease. Nebulized granulocyte–macrophage colony-stimulating factor (GM-CSF) has a certain therapeutic effect on PAP. Here, we report a case of PAP treated with WLL combined with nebulized GM-CSF, which achieved good therapeutic effects during a 4-year follow-up period, to enhance understanding of the disease.

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