King M B, Campbell E J, Gray B H, Hertz M I
Department of Internal Medicine, University of Minnesota Medical School, Minneapolis 55455.
Am J Respir Crit Care Med. 1994 Apr;149(4 Pt 1):966-71. doi: 10.1164/ajrccm.149.4.8143063.
We examined the proteinase-antiproteinase balance in the bronchoalveolar lavage (BAL) fluid from alpha-1-proteinase inhibitor (alpha 1PI)-deficient lung transplant recipients to determine whether they would derive benefit from intravenous augmentation therapy with alpha 1PI. BAL fluid from 11 alpha 1PI-deficient lung transplant recipients and eight control subjects was assayed for free neutrophil elastase activity, immunoreactive alpha 1PI, and elastase inhibitory capacity. Samples were obtained during intervals of health and respiratory illness. BAL fluid from healthy alpha 1PI-deficient lung transplant recipients had minimal or unmeasurable free elastase activity, which was not different from that of control subjects. alpha 1PI concentrations in BAL fluid from alpha 1PI-deficient lung transplant recipients were reduced when compared with those of control subjects. Despite this observation, all but one alpha 1PI-deficient patient had the ability to inhibit exogenous elastase. During respiratory illness, however, three of seven alpha 1PI-deficient lung transplant recipients had measurable free elastase activity, which was inhibited ex vivo by addition of alpha 1PI. We conclude that alpha 1PI-deficient lung transplant recipients demonstrate free elastase activity in BAL fluid during severe lower respiratory tract inflammation, which is not present during health. Intravenous supplementation of alpha 1PI-deficient lung transplant recipients with exogenous alpha 1PI during respiratory tract inflammation may be indicated to inhibit elastase-mediated injury to the transplanted lung.
我们检测了α1-抗胰蛋白酶（α1PI）缺乏的肺移植受者支气管肺泡灌洗（BAL）液中的蛋白酶-抗蛋白酶平衡，以确定他们是否能从静脉注射α1PI强化治疗中获益。检测了11名α1PI缺乏的肺移植受者和8名对照者的BAL液中的游离中性粒细胞弹性蛋白酶活性、免疫反应性α1PI和弹性蛋白酶抑制能力。在健康和呼吸道疾病期间采集样本。健康的α1PI缺乏的肺移植受者的BAL液中游离弹性蛋白酶活性极低或无法检测，这与对照者无异。与对照者相比，α1PI缺乏的肺移植受者的BAL液中α1PI浓度降低。尽管有此观察结果，但除一名α1PI缺乏的患者外，其他所有患者都有抑制外源性弹性蛋白酶的能力。然而，在呼吸道疾病期间，7名α1PI缺乏的肺移植受者中有3名的游离弹性蛋白酶活性可检测到，添加α1PI后可在体外抑制该活性。我们得出结论，α1PI缺乏的肺移植受者在严重下呼吸道炎症期间BAL液中表现出游离弹性蛋白酶活性，而在健康时不存在这种情况。在呼吸道炎症期间，可能需要对α1PI缺乏的肺移植受者静脉补充外源性α1PI，以抑制弹性蛋白酶介导的对移植肺的损伤。
