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. 2025 Apr 28:46:101099.
doi: 10.1016/j.lana.2025.101099. eCollection 2025 Jun.

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

Luiz Vicente Ribeiro F da Silva-Filho  1   2 , Gianluca Belchior  2 , Angela Tavares Paes  2 , Nicole Costa Soriano Freire  2 , Cintia Steinhaus  3 , Matias Epifanio  3 ; Brazilian CF Registry Contributors Team
Collaborators, Affiliations

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

Luiz Vicente Ribeiro F da Silva-Filho et al. Lancet Reg Health Am. .

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.

Keywords: Anthropometric variables; Cystic fibrosis; Meconium ileus; Nutritional status; Pulmonary function.

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Conflict of interest statement

Luiz Vicente Silva-Filho has received research grants from Vertex Pharmaceuticals, TimPel and Fundación Infant; consulting fees from Vertex Pharmaceuticals and Omron, honoraria for lectures from Astra-Zeneca, Sanofi, Vertex Pharmaceuticals and Omron, and participate in advisory boards of Vertex Pharmaceuticals; Matias Epifanio has received consulting fees from Danone, and honoraria for lectures from Danone, Vertex Pharmaceuticals Cellera and EMS.

Figures

Fig. 1
Fig. 1
Kaplan–Meier curve demonstrating the survival of CF individuals up to 18 years of age according to the meconium ileus status.
See this image and copyright information in PMC

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