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基因分型囊性纤维化患者呼吸道黏液的物理化学和传输特性再探讨

Revisited physicochemical and transport properties of respiratory mucus in genotyped cystic fibrosis patients.

作者信息

Deneuville E, Perrot-Minot C, Pennaforte F, Roussey M, Zahm J M, Clavel C, Puchelle E, de Bentzmann S

机构信息

INSERM Unité 314, CHR Maison Blanche, Reims, France.

出版信息

Am J Respir Crit Care Med. 1997 Jul;156(1):166-72. doi: 10.1164/ajrccm.156.1.9606123.

Abstract

We investigated the physicochemical and transport properties of sputum samples collected in physiotherapy from a well-documented group of 27 cystic fibrosis (CF) patients with identified CF genotypes. Sputum samples were characterized ex vivo for their water content, surface properties (surface tension and contact angle), rheologic properties (viscosity and elastic modulus), and transport properties (mucociliary and cough transport). These data were analyzed in relation to the clinical status of the patients (FEV1, FVC, Shwachman score, Brasfield score, nutritional status), their genotype, and the degree of infection of their sputa (leukocyte and Pseudomonas aeruginosa counts). We observed negative and significant correlations between mucociliary transport and elastic modulus of the patients' sputum (r = -0.63, p < 0.01), and between the cough transport and contact angle of the sputum (r = -0.81, p < 0.0001), respectively. The P. aeruginosa count was also significantly correlated with the sputum water content (r = -0.53, p < 0.02) as well as with the cough transport of the sputum (r = -0.62, p < 0.01). In CF patients with a sputum leukocyte count > 2,000/mm3, the sputum water content (p < 0.02), FEV1 (p < 0.05) and FVC (p < 0.02) were significantly lower than those of CF patients with a leukocyte count < or = 2,000/mm3. CF patients with a homozygous delta F 508 genotype had significantly greater values of sputum water content (p < 0.05), and cough-transport capacity (p < 0.05) than did heterozygous patients. No correlation was observed between the sputum properties and any of the clinical data. These results suggest that the control of infection should be emphasized in CF, since it can directly or indirectly modulate the degree of hydration, and therefore the physicochemical and transport properties, of airway secretions.

摘要

我们调查了从一组有详细记录的27名已确定CF基因型的囊性纤维化(CF)患者身上收集的痰液样本的物理化学及传输特性。对痰液样本进行了离体表征,分析其含水量、表面特性(表面张力和接触角)、流变特性(粘度和弹性模量)以及传输特性(黏液纤毛传输和咳嗽传输)。将这些数据与患者的临床状况(第一秒用力呼气容积、用力肺活量、舒瓦茨曼评分、布拉斯菲尔德评分、营养状况)、基因型以及痰液的感染程度(白细胞和铜绿假单胞菌计数)进行关联分析。我们分别观察到患者痰液的黏液纤毛传输与弹性模量之间呈负相关且具有显著性(r = -0.63,p < 0.01),以及咳嗽传输与痰液接触角之间呈负相关且具有显著性(r = -0.81,p < 0.0001)。铜绿假单胞菌计数还与痰液含水量显著相关(r = -0.53,p < 0.02),以及与痰液的咳嗽传输显著相关(r = -0.62,p < 0.01)。在痰液白细胞计数>2,000/mm³的CF患者中,痰液含水量(p < 0.02)、第一秒用力呼气容积(p < 0.05)和用力肺活量(p < 0.02)显著低于白细胞计数≤2,000/mm³的CF患者。具有纯合子ΔF 508基因型的CF患者的痰液含水量(p < 0.05)和咳嗽传输能力(p < 0.05)显著高于杂合子患者。未观察到痰液特性与任何临床数据之间存在相关性。这些结果表明,在CF中应强调控制感染,因为它可直接或间接调节气道分泌物的水合程度,进而调节其物理化学及传输特性。

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