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儿童低严重度囊性纤维化的呼吸系统参数:与健康同龄人相比,是否存在早期受累?

Respiratory system parameters in children with low severity cystic fibrosis: is there early involvement in relation to healthy peers?

机构信息

Universidade do Estado de Santa Catarina, Florianópolis, SC, Brasil.

出版信息

Rev Paul Pediatr. 2023 Dec 11;42:e2023030. doi: 10.1590/1984-0462/2024/42/2023030. eCollection 2023.

Abstract

OBJECTIVE

To compare and analyze pulmonary function and respiratory mechanics parameters between healthy children and children with cystic fibrosis.

METHODS

This cross-sectional analytical study included healthy children (HSG) and children with cystic fibrosis (CFG), aged 6-13 years, from teaching institutions and a reference center for cystic fibrosis in Florianópolis/SC, Brazil. The patients were paired by age and sex. Initially, an anthropometric evaluation was undertaken to pair the sample characteristics in both groups; the medical records of CFG were consulted for bacterial colonization, genotype, and disease severity (Schwachman-Doershuk Score - SDS) data. Spirometry and impulse oscillometry were used to assess pulmonary function.

RESULTS

In total, 110 children were included, 55 in each group. In the CFG group, 58.2% were classified as excellent by SDS, 49.1% showed the ΔF508 heterozygotic genotype, and 67.3% were colonized by some pathogens. Statistical analysis revealed significant differences between both groups (p<0.05) in most pulmonary function parameters and respiratory mechanics.

CONCLUSIONS

Children with cystic fibrosis showed obstructive ventilatory disorders and compromised peripheral airways compared with healthy children. These findings reinforce the early changes in pulmonary function and mechanics associated with this disease.

摘要

目的

比较和分析健康儿童与囊性纤维化儿童的肺功能和呼吸力学参数。

方法

本横断面分析性研究纳入了来自巴西弗洛里亚诺波利斯/南里奥格兰德州教学机构和囊性纤维化参考中心的健康儿童(HSG)和囊性纤维化儿童(CFG),年龄为 6-13 岁。患者按年龄和性别配对。首先,进行人体测量评估以匹配两组的样本特征;查阅 CFG 的病历以获取细菌定植、基因型和疾病严重程度(施瓦茨曼-多什评分-SDS)数据。使用肺量测定法和脉冲震荡法评估肺功能。

结果

共有 110 名儿童入组,每组 55 名。在 CFG 组中,58.2%的儿童根据 SDS 被评为优秀,49.1%的儿童携带 ΔF508 杂合基因型,67.3%的儿童被某些病原体定植。统计分析显示两组间大多数肺功能参数和呼吸力学均存在显著差异(p<0.05)。

结论

与健康儿童相比,囊性纤维化儿童表现出阻塞性通气障碍和外周气道功能受损。这些发现强调了与该疾病相关的肺功能和力学的早期变化。

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