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儿童支气管扩张症:使用巴哈拉评分对囊性纤维化和非囊性纤维化病因的比较分析

Bronchiectasis in Children: A Comparative Analysis of Cystic Fibrosis and Non-Cystic Fibrosis Etiologies Using the Bhalla Score.

作者信息

Kekeç Handan, Şişmanlar Eyüboğlu Tuğba, Tana Aslan Ayse, Duman Yasemin, Medeni Volkan, Yazol Merve, Boyunağa Öznur Leman

机构信息

Department of Pediatric Pulmonology, Gazi University Faculty of Medicine, Ankara, Türkiye

Department of Pediatrics, Gazi University Faculty of Medicine, Ankara, Türkiye

出版信息

Balkan Med J. 2025 Sep 1;42(5):429-439. doi: 10.4274/balkanmedj.galenos.2025.2025-6-247. Epub 2025 Aug 20.

DOI:10.4274/balkanmedj.galenos.2025.2025-6-247
PMID:40832796
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12402963/
Abstract

BACKGROUND

Childhood bronchiectasis (BE) is a chronic lung condition that remains under recognized, marked by irreversible widening of the bronchi, frequent respiratory infections, a persistent wet cough, and progressive lung damage, often leading to significant health burdens.

AIMS

To evaluate children with cystic fibrosis (CF) and non-CF BE and to examine the association between clinical presentation and radiological severity of BE using the Bhalla scoring system.

STUDY DESIGN

Retrospective observational study.

METHODS

Children aged 0-18 years with a CT-confirmed diagnosis of BE were enrolled. Data on demographics, clinical characteristics, and imaging results were collected retrospectively from medical records. The Bhalla score was used to assess BE severity. Patients were categorized into CF-related and non-CF BE groups. The two groups were compared with respect to clinical features, growth z-scores, hospitalization frequency, and pulmonary function test outcomes.

RESULTS

A total of 157 patients were analyzed. Among them, CF accounted for 23.6% of cases, and while the leading causes in the non-CF group were post-infectious BE (28%), immunodeficiency (19.8%), and primary ciliary dyskinesia (12.8%). The CF group presented at an earlier age, had a longer follow-up period, and experienced more frequent hospitalizations ( < 0.001). In the CF group, weight, height, and body mass index z-scores significantly improved from the initial to the final assessment ( = 0.010, = 0.006, and = 0.026, respectively), whereas no such improvement was observed in the non-CF group. Severe Bhalla scores were more frequently observed in the CF group (p < 0.001). Among CF patients, Bhalla scores showed a strong correlation with forced expiratory volume in one second (FEV) in univariate analysis (r = 0.846, < 0.001), though this was not significant in multivariable analysis ( = 0.434). In the non-CF group, there was no correlation between Bhalla scores and final FEV values ( = 0.148, r = 0.212).

CONCLUSION

The results underscore distinct clinical trajectories between CF and non-CF BE in children. Improvements in CF patients suggest the effectiveness of structured clinical management, whereas inconsistent outcomes in non-CF patients point to the need for standardized follow-up protocols. While the Bhalla score may indicate the extent of structural lung disease in CF, it does not independently predict lung function, and therefore should be used as a supplementary, not solitary, measure of disease severity.

摘要

背景

儿童支气管扩张症(BE)是一种尚未得到充分认识的慢性肺部疾病,其特征为支气管不可逆性增宽、频繁的呼吸道感染、持续性湿性咳嗽以及进行性肺损伤,常导致重大的健康负担。

目的

评估患有囊性纤维化(CF)和非CF支气管扩张症的儿童,并使用巴哈拉评分系统检查支气管扩张症的临床表现与放射学严重程度之间的关联。

研究设计

回顾性观察研究。

方法

纳入0至18岁经CT确诊为支气管扩张症的儿童。从病历中回顾性收集人口统计学、临床特征和影像学结果的数据。使用巴哈拉评分评估支气管扩张症的严重程度。患者分为CF相关组和非CF支气管扩张症组。比较两组的临床特征、生长z评分、住院频率和肺功能测试结果。

结果

共分析了157例患者。其中,CF占病例的23.6%,而非CF组的主要病因是感染后支气管扩张症(28%)、免疫缺陷(19.8%)和原发性纤毛运动障碍(12.8%)。CF组发病年龄更早,随访期更长,住院频率更高(<0.001)。在CF组中,从初始评估到最终评估,体重、身高和体重指数z评分显著改善(分别为=0.010、=0.006和=0.026),而非CF组未观察到此类改善。CF组更频繁地观察到严重的巴哈拉评分(p<0.001)。在CF患者中,单因素分析中巴哈拉评分与一秒用力呼气容积(FEV)呈强相关(r=0.846,<0.001),尽管在多变量分析中不显著(=0.434)。在非CF组中,巴哈拉评分与最终FEV值之间无相关性(=0.148,r=0.212)。

结论

结果强调了儿童CF和非CF支气管扩张症之间不同的临床轨迹。CF患者的改善表明结构化临床管理的有效性,而非CF患者结果不一致表明需要标准化的随访方案。虽然巴哈拉评分可能表明CF中结构性肺病的程度,但它不能独立预测肺功能,因此应用作疾病严重程度的补充指标,而非唯一指标。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1617/12402963/babd081504cc/BalkanMedJ-42-5-429-figure-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1617/12402963/babd081504cc/BalkanMedJ-42-5-429-figure-1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1617/12402963/babd081504cc/BalkanMedJ-42-5-429-figure-1.jpg

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