Regelmann W E, Siefferman C M, Herron J M, Elliott G R, Clawson C C, Gray B H
Department of Pedatrics, University of Minnesota, Minneapolis 55455, USA.
Pediatr Pulmonol. 1995 Jan;19(1):1-9. doi: 10.1002/ppul.1950190102.
Patients with cystic fibrosis (CF) of the same age differ significantly in their degree of pulmonary disease. Based on preliminary observations, we postulated that the activity of myeloperoxidase would be significantly increased in patients with greater structural lung damage than in those with less lung damage. Acid extracts of weighed sputum samples were assayed for lactoferrin concentrations by ELISA. Activities of peroxidase, cathespsin G, and elastase (with and without proteinase 3) were determined by kinetic analysis using chromogenic substrates. The patients were divided into quartiles based on their Brasfield chest-radiograph score. Patients in the first quartile (least amount of structural lung abnormality) were compared to those in the fourth quartile. The concentration of lactoferrin, a specific (secondary) granule protein of neutrophils, did not differ between the two patient groups. However, the activities of the neutrophil primary granule proteins, peroxidase, elastase, and elastase plus proteinase 3, were significantly elevated in the group with the most structural lung abnormality. Sputum albumin concentration was used to estimate leakages of plasma proteins into the airways. Peroxidase activity, but not the activity of cathepsin G, of elastase, or of elastase plus proteinase 3, correlated significantly with albumin/g sputum in both quartile groups. To confirm the association of sputum peroxidase activity with differences in lung structure and to test its correlation with lung function, spirometry was performed in a second group of patients during the week prior to the time of sputum sampling. In this second group, increased sputum peroxidase activity was associated with worse Brasfield scores and with decreased percent-predicted forced expiratory volume in 1 sec.(ABSTRACT TRUNCATED AT 250 WORDS)
同龄的囊性纤维化（CF）患者肺部疾病程度差异显著。基于初步观察，我们推测与肺部损伤较轻的患者相比，肺部结构损伤较重的患者髓过氧化物酶活性会显著升高。通过酶联免疫吸附测定法（ELISA）检测称重后的痰液样本酸提取物中的乳铁蛋白浓度。使用显色底物通过动力学分析测定过氧化物酶、组织蛋白酶G和弹性蛋白酶（有或无蛋白酶3）的活性。根据患者的布拉斯菲尔德胸部X线评分将其分为四分位数。将第一四分位数（肺部结构异常最少）的患者与第四四分位数的患者进行比较。两组患者中性粒细胞特异性（二级）颗粒蛋白乳铁蛋白的浓度没有差异。然而，肺部结构异常最严重的组中性粒细胞初级颗粒蛋白过氧化物酶、弹性蛋白酶以及弹性蛋白酶加蛋白酶3的活性显著升高。痰液白蛋白浓度用于估计血浆蛋白向气道的渗漏情况。在两个四分位数组中，过氧化物酶活性与每克痰液中的白蛋白显著相关，而组织蛋白酶G、弹性蛋白酶或弹性蛋白酶加蛋白酶3的活性则不然。为了证实痰液过氧化物酶活性与肺部结构差异之间的关联，并测试其与肺功能的相关性，在第二组患者痰液采样前一周进行了肺活量测定。在这第二组患者中，痰液过氧化物酶活性升高与较差的布拉斯菲尔德评分以及一秒用力呼气量预测值百分比降低有关。（摘要截断于250字）
