The significant impact of meconium ileus on clinical outcomes among Brazilian individuals with cystic fibrosis—a retrospective analysis of a patient registry

IF 7 Q1 HEALTH CARE SCIENCES & SERVICES

Lancet Regional Health-Americas Pub Date : 2025-04-28 DOI:10.1016/j.lana.2025.101099

Luiz Vicente Ribeiro F. da Silva-Filho , Gianluca Belchior , Angela Tavares Paes , Nicole Costa Soriano Freire , Cintia Steinhaus , Matias Epifanio

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引用次数: 0

Abstract

Background

Meconium ileus (MI) is one of the earliest manifestations of cystic fibrosis (CF), affecting 15–20% of individuals. The impact of MI on health outcomes has yet to be elucidated and may vary based on the amount of health care resources. The aim of this study was to investigate the clinical impact of MI on outcomes among Brazilian CF individuals using data from the Brazilian Cystic Fibrosis Patient Registry.

Methods

This retrospective cohort study included data from individuals with CF from 53 reference centres in Brazil. Data from individuals with a history of MI during the neonatal period were compared to those of the non-MI individuals. Demographic data, genotype, lung function, nutritional data, microbiological data and survival data were compared between groups. The impact of MI on lung function and anthropometric outcomes was evaluated using mixed effects models after adjusting for age. Individual survival data were analyzed by Kaplan–Meier curves, log-rank tests and Cox proportional hazards models.

Findings

Among the 5128 individuals included in the registry, 369 (7·2%) were diagnosed with MI at birth. The occurrence of MI was associated with an earlier diagnosis of CF but a lower mean Z score for weight (−0·32, 95% CI −0·46 to −0·18, p < 0·0001) and height (−0·28 95% CI −0·40 to −0·15, p < 0·0001). Lung function was significantly lower among those affected by MI (reduction of −4·3% 95% CI −8·0 to −0·5, p = 0·028) up to the age of 18 years. A greater prevalence of Pseudomonas aeruginosa colonization was observed in the MI group (79·1% (272/344) versus 64·5% (2818/4367); p < 0·0001). Survival was significantly worse in the MI group, and the results of the Cox regression model revealed that the impact of MI on mortality was significant after controlling for other risk factors (HR = 1·84, 95% CI 1·50–2·25, p < 0·0001).

Interpretation

CF individuals affected by MI had more severe and earlier declines in lung function, slower rates of weight and height gain, and lower survival rates. These findings underscore the importance of early identification and tailored management strategies for this high-risk subgroup.

Funding

None.

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胎粪肠梗阻对巴西囊性纤维化患者临床结果的显著影响——对患者登记的回顾性分析

背景：肠便（MI）是囊性纤维化（CF）的早期表现之一，影响15-20%的个体。心肌梗死对健康结果的影响尚未阐明，可能因卫生保健资源的数量而异。本研究的目的是利用巴西囊性纤维化患者登记处的数据，调查心肌梗死对巴西CF患者结局的临床影响。方法本回顾性队列研究包括来自巴西53个参考中心的CF患者的数据。将新生儿期有心肌梗死史的个体与非心肌梗死个体的数据进行比较。组间比较人口学数据、基因型、肺功能、营养数据、微生物数据和生存数据。在调整年龄后，使用混合效应模型评估心肌梗死对肺功能和人体测量结果的影响。个体生存数据采用Kaplan-Meier曲线、log-rank检验和Cox比例风险模型进行分析。在纳入登记的5128人中，369人（7.2%）在出生时被诊断为心肌梗死。心肌梗死的发生与CF的早期诊断有关，但体重的平均Z评分较低(- 0.32,95% CI - 0.46至- 0.18,p <；0·0001)和高度(−0·28 95%可信区间40 0−−0··15日p & lt;0·0001)。18岁前，心肌梗死患者肺功能显著降低（降低幅度为- 4.3%，95% CI为- 8.0 ~ - 0.05,p = 0.028）。在心肌梗死组中，铜绿假单胞菌的定殖率更高(79.1% (272/344)vs . 64.5% (2818/4367)；p & lt;0·0001)。心肌梗死组的生存率明显较差，Cox回归模型结果显示，在控制其他危险因素后，心肌梗死对死亡率的影响显著(HR = 1.84, 95% CI 1.50 - 2.25, p <；0·0001)。解释：受心肌梗死影响的cf个体肺功能下降更严重，更早，体重和身高增加速度更慢，生存率更低。这些发现强调了对这一高风险亚群进行早期识别和量身定制的管理策略的重要性。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Lancet Regional Health-Americas Multiple-

CiteScore

8.00

自引率

0.00%

发文量

期刊介绍： The Lancet Regional Health – Americas, an open-access journal, contributes to The Lancet's global initiative by focusing on health-care quality and access in the Americas. It aims to advance clinical practice and health policy in the region, promoting better health outcomes. The journal publishes high-quality original research advocating change or shedding light on clinical practice and health policy. It welcomes submissions on various regional health topics, including infectious diseases, non-communicable diseases, child and adolescent health, maternal and reproductive health, emergency care, health policy, and health equity.