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患有囊性纤维化的婴幼儿的表面活性剂成分

Surfactant composition in infants and young children with cystic fibrosis.

作者信息

Hull J, South M, Phelan P, Grimwood K

机构信息

Department of Thoracic Medicine, Royal Children's Hospital, Melbourne, Australia.

出版信息

Am J Respir Crit Care Med. 1997 Jul;156(1):161-5. doi: 10.1164/ajrccm.156.1.9609090.

DOI:10.1164/ajrccm.156.1.9609090
PMID:9230741
Abstract

We tested the hypothesis that the composition of bronchial surfactant is normal in infants with cystic fibrosis (CF) in the absence of active lung disease but that it is altered by lower respiratory tract infection and inflammation. We examined the total phospholipid (PL), disaturated phospholipid (DSP), surfactant protein-A (SP-A), surfactant protein B (SP-B), and surface activity in bronchoalveolar lavage fluid from 27 subjects with CF whose mean age was 22.7 (SD 14.5) mo. Six infants with stridor served as non-CF controls. Twelve of the subjects with CF (CF-I group) had evidence of active pulmonary infection or inflammation which was absent in the remaining 15 subjects (CF-NI group). We found no differences in the surfactant composition or activity between controls and the CF-NI group. In contrast, the DSP/PL ratio was lower in the CF-I subjects than in both the CF-NI subjects (p = 0.05) and controls (p < 0.01) suggesting a disturbance of surfactant function. SP-A concentrations were higher in the CF-I group compared to the other two groups (p < 0.05). These results suggest that the bronchial surfactant of infants with CF is altered following lower airway infection and inflammation and is not a primary abnormality associated with this disorder.

摘要

我们验证了以下假设

在无活动性肺部疾病的囊性纤维化(CF)婴儿中,支气管表面活性剂的组成是正常的,但下呼吸道感染和炎症会使其发生改变。我们检测了27名CF患儿支气管肺泡灌洗液中的总磷脂(PL)、二饱和磷脂(DSP)、表面活性蛋白A(SP-A)、表面活性蛋白B(SP-B)以及表面活性,这些患儿的平均年龄为22.7(标准差14.5)个月。6名喘鸣婴儿作为非CF对照。27名CF患儿中有12名(CF-I组)有活动性肺部感染或炎症的证据,其余15名(CF-NI组)则没有。我们发现对照组与CF-NI组之间的表面活性剂组成或活性没有差异。相比之下,CF-I组患儿的DSP/PL比值低于CF-NI组患儿(p = 0.05)和对照组(p < 0.01),提示表面活性剂功能受到干扰。与其他两组相比,CF-I组的SP-A浓度更高(p < 0.05)。这些结果表明,CF婴儿的支气管表面活性剂在下呼吸道感染和炎症后会发生改变,并非与该疾病相关的原发性异常。

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