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Direct hemoperfusion with polymyxin B-immobilized fiber column in a patient with acute exacerbation of idiopathic pulmonary fibrosis

机译:用急性发作性肺纤维化的患者用多粘菌素B固定化纤维柱直接血液灌注

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Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing interstitial lung disease characterized by dyspnea and a worsening of the lung function. Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF) are defined by a clinically significant respiratory deterioration, that typically develops in less than 1 month, accompanied by new radiologic abnormalities on HRCT, including diffused and bilateral ground-glass opacification, along with an absence of other obvious clinical etiologies. Recently, AE-IPF has gained significant importance as a major cause of mortality and morbidity. However, despite the extremely poor prognosis of the condition, no well-validated therapeutic interventions are currently available. Therefore, novel treatment modalities are being investigated and applied in addition to conventional treatments. Among them, several studies have reported that a direct hemoperfusion with a polymyxin B-immobilized fiber column (PMX-DHP), developed for endotoxin removal in septic shock, has an effect on AE-IPF. We describe two cases of PMX-DHP treatment with conflicting results. One patient successfully recovered via a PMX-DHP in severe AE-IPF that required extracorporeal membrane oxygenation (ECMO). PMX-DHP subsequently improved oxygenation (PaOsub2/sub/FiOsub2/sub ratio) and decreased the levels of inflammatory markers (interleukin-6, C-reactive protein, and white blood cells). The patient dramatically recovered without the need for ECMO. PMX-DHP may be considered an alternative therapy in AE-IPF patients requiring mechanical ventilation or ECMO.
机译:特发性肺纤维化(IPF)是一种渐进式抗体肺病,其特征在于呼吸困难和肺功能恶化。特发性肺纤维化(AE-IPF)的急性发作由临床显着的呼吸衰退定义,通常在不到1个月内开发,伴随着HRCT的新放射学异常,包括扩散和双侧玻璃渗透率,以及缺失其他明显的临床病因。最近,AE-IPF作为死亡率和发病率的主要原因获得了重大重要性。但是,尽管病症预后极差,但目前没有验证良好的治疗干预措施。因此,除常规治疗外,还研究了新的治疗方式。其中,几项研究报道说,用多种多辛B-固定化的纤维柱(PMX-DHP)直接血液灌注,用于在脓毒症休克中用于内毒素去除,对AE-IPF具有作用。我们描述了两种患者PMX-DHP治疗突破性效果。一名患者通过在严重的AE-IPF中成功地回收,所述严重AE-IPF,需要体外膜氧合(ECMO)。 PMX-DHP随后改善氧合(Pao 2 / fio 2 比率)并降低炎症标记物的水平(白细胞介素-6,C反应蛋白和白细胞) 。患者在不需要Ecmo的情况下显着恢复。 PMX-DHP可以被认为是需要机械通气或ECMO的AE-IPF患者的替代治疗。

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