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Updated: Aug 1, 2026

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A New Murine Model of Endovascular Aortic Aneurysm Repair
Published on: July 7, 2013
气溶α1-抗素治疗囊性纤维化
N G McElvaney1, R C Hubbard, P Birrer
1Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, Maryland 20892.
Lancet (London, England)
|February 16, 1991
概括
在囊性纤维化患者中,空气注射的α1-抗素 (α1AT) 疗法降低了中性粒细胞弹性酶. 这种治疗恢复了保护性酶的能力,并改善了细菌的杀死,这表明囊性纤维化中增强了宿主防御能力.
科学领域:
- 肺部医学 肺部医学
- 酶抑制剂治疗疗法 酶抑制剂治疗
- 囊性纤维化研究 囊性纤维化研究
背景情况:
- 囊性纤维化包括呼吸道表面中性粒细胞弹性酶的慢性炎症.
- 中性粒细胞弹性酶是一种破坏性酶,会负担呼吸道上皮质.
- 阿尔法1抗素 (alpha1AT) 是肺中中性粒细胞弹性酶的主要抑制剂.
研究的目的:
- 在囊性纤维化患者中研究气溶式α1-抗素 (alpha 1AT) 的疗效.
- 评估α1AT对呼吸道上皮层层液 (ELF) 中中性粒细胞弹性酶水平的影响.
- 为了确定α1AT治疗是否可以恢复抗中性粒细胞弹性酶能力并改善宿主防御.
主要方法:
- 气溶性α1-抗素 (α1AT) 给了12名囊性纤维化患者.
- 在ELF中测量了中性粒细胞弹性酶和α1AT度.
- 囊性纤维化ELF对中性粒细胞介导的Pseudomonas杀死的影响在治疗前和治疗后进行了评估.
主要成果:
- 在呼吸道上皮质内膜液 (ELF) 中,空气溶解的α1AT抑制了中性粒细胞弹性酶.
- 当ELFα1AT达到8mmol/l时,ELF抗中性粒细胞弹性酶的能力得到恢复.
- 囊性纤维化ELF对中性粒细胞杀死Pseudomonas的抑制作用是逆转的.
结论:
- 气溶性α1-抗素在控制囊性纤维化中的中性粒细胞弹性酶方面是有效的.
- 恢复ELF抗中性粒细胞弹性酶的能力表明了潜在的治疗益处.
- 这种治疗可以增强囊性纤维化患者的宿主防御机制,改善Pseudomonas杀伤能力.
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