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一种通过黏液黏弹性特性改善囊性纤维化患者临床评估的新方法。

A new method to improve the clinical evaluation of cystic fibrosis patients by mucus viscoelastic properties.

机构信息

Dipartimento di Ingegneria Chimica, dei Materiali e della Produzione Industriale, Università di Napoli Federico II, Napoli, Italy ; CEINGE-Advanced Biotechnologies, Napoli, Italy.

Dipartimento di Fisica, Università di Napoli Federico II, Complesso Universitario M.S. Angelo, Napoli, Italy.

出版信息

PLoS One. 2014 Jan 3;9(1):e82297. doi: 10.1371/journal.pone.0082297. eCollection 2014.

DOI:10.1371/journal.pone.0082297
PMID:24404129
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3880261/
Abstract

In cystic fibrosis (CF) patients airways mucus shows an increased viscoelasticity due to the concentration of high molecular weight components. Such mucus thickening eventually leads to bacterial overgrowth and prevents mucus clearance. The altered rheological behavior of mucus results in chronic lung infection and inflammation, which causes most of the cases of morbidity and mortality, although the cystic fibrosis complications affect other organs as well. Here, we present a quantitative study on the correlation between cystic fibrosis mucus viscoelasticity and patients clinical status. In particular, a new diagnostic parameter based on the correlation between CF sputum viscoelastic properties and the severity of the disease, expressed in terms of FEV1 and bacterial colonization, was developed. By using principal component analysis, we show that the types of colonization and FEV1 classes are significantly correlated to the elastic modulus, and that the latter can be used for CF severity classification with a high predictive efficiency (88%). The data presented here show that the elastic modulus of airways mucus, given the high predictive efficiency, could be used as a new clinical parameter in the prognostic evaluation of cystic fibrosis.

摘要

在囊性纤维化 (CF) 患者中,由于高分子量成分的浓度,气道粘液表现出增加的粘弹性。这种粘液变厚最终导致细菌过度生长并阻止粘液清除。粘液流变行为的改变导致慢性肺部感染和炎症,这导致了大多数发病率和死亡率的病例,尽管囊性纤维化并发症也会影响其他器官。在这里,我们对囊性纤维化粘液粘弹性与患者临床状况之间的相关性进行了定量研究。特别是,基于 CF 痰粘弹性特性与疾病严重程度(以 FEV1 和细菌定植表示)之间的相关性,开发了一种新的诊断参数。通过主成分分析,我们表明,定植类型和 FEV1 类别与弹性模量显著相关,并且后者可用于 CF 严重程度分类,具有较高的预测效率(88%)。这里呈现的数据表明,气道粘液的弹性模量,由于其高预测效率,可以用作囊性纤维化预后评估的新临床参数。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/76b2/3880261/0120db65226e/pone.0082297.g008.jpg
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Molecular and functional analysis of the large 5' promoter region of CFTR gene revealed pathogenic mutations in CF and CFTR-related disorders.对 CFTR 基因的大 5'启动子区域进行的分子和功能分析揭示了 CF 和 CFTR 相关疾病中的致病突变。
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