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当α1 抗胰蛋白酶(α1 AT)缺乏时,常规疗法是恢复蛋白酶-抗蛋白酶系统的平衡。Wewers 等以混合血浆中制备的人α1 AT 每周注入60mg/kg(低剂量),而Hubbard 等最近每4周输注250 mg/kg(高剂量).Wewers 等发现21例患者血清α1 AT最高含量达300mg/dl 以上,其后迅速下降,与α1 AT正常清除率一致,并达到预定的80mg/dl 水平。作者根据流行病学调查结果,选择80mg/dl 作为α1 AT 阈值.在低剂量α1AT 研究组中所有病例的血清抗中性白细胞弹性蛋白酶均超出此值,8例支气管肺泡灌洗(BAL)液中α1 AT 亦超过此阈值,更重要的是抗中性白细胞弹性硬蛋白酶增加至正常范围。较大剂
When α1 antitrypsin (α1 AT) is deficient, conventional therapies restore the balance of the protease-resistant protease system. Wewers et al., Injected 60 mg / kg (low dose) weekly with human α1 AT prepared in pooled plasma, while Hubbard et al recently injected 250 mg / kg (high dose) every 4 weeks. Wewers et al found 21 patients with the highest serum α1 AT Content of 300mg / dl above, then decreased rapidly, with the normal clearance of α1 AT consistent and reach the predetermined level of 80mg / dl. The authors selected 80 mg / dl as the threshold for the [alpha] 1 AT, based on epidemiological findings, and all of the patients in the low-dose [alpha] 1 AT group exceeded this value for serum anti-neutrophil elastase and in 8 patients with bronchoalveolar lavage α1 AT also exceeds this threshold, more importantly, anti-neutrophil elastase increased to the normal range. Larger agent