普拉斯丁C (prolastin c)

Prolastin c

Mechanism of Action — Alpha1-antitrypsin (AAT) is the principle protease inhibitor in serum. Its major physiologic role is to render proteolytic enzymes (secreted during inflammation) inactive. A decrease in AAT, as seen in congenital AAT deficiency, leads to increased elastic damage in the lung, causing emphysema.  Labeled Indications —Alpha1-proteinase inhibitor deficiency: Long-term augmentation and maintenance therapy in adults with severe hereditary deficiency of alpha1-antitrypsin (AAT) with clinically evident emphysema.  The effect of augmentation therapy with any alpha1-proteinase inhibitor on pulmonary exacerbations has not been demonstrated in randomized, controlled clinical trials. However, one randomized controlled trial showed a reduction in emphysema progression with alpha1-proteinase inhibitor augmentation therapy when CT lung density was measured at total lung capacity (Chapman 2015).

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普拉斯丁C (prolastin c)

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作用机制 — α1-抗胰蛋白酶（AAT）是血清中主要的蛋白酶抑制剂。它的主要生理作用是使蛋白分解酶（在炎症期间分泌）失去活性。AAT的减少(如先天性AAT缺乏症)会导致肺部的弹性损伤增加引起肺气肿。 适应症— α-1蛋白酶抑制剂缺乏症。患有严重遗传性α1-抗胰蛋白酶（AAT）缺乏症并伴有临床明显肺气肿的成年人的长期增强和维持治疗。一项随机对照试验显示，当CT肺密度按总肺活量测量时，α1-蛋白酶抑制剂的增强治疗可减少肺气肿的发展（Chapman 2015）。然而任何α1-蛋白酶抑制剂的增强治疗对肺部恶化的影响尚未在随机、对照的临床试验中得到证实。所以FDA批准的适应症只有α-1蛋白酶抑制剂缺乏症。