WS12.06Exploring the impact of excessive BMI on metabolic health in patients with cystic fibrosis: a 2-year experience with CFTR modulators at the Warsaw CF Centre

IF 5.4 2区医学 Q1 RESPIRATORY SYSTEM

Journal of Cystic Fibrosis Pub Date : 2025-06-01 DOI:10.1016/j.jcf.2025.03.563

M. Mielus , K. Zybert , K. Walicka-Serzysko , L. Wozniacki , J. Milczewska , D. Sands

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引用次数: 0

Abstract

Objectives

CFTR modulator therapies have transformed cystic fibrosis (CF) management, improving nutritional status. The emergence of overweight and obesity (OW/OB) in people with CF (pwCF) raises concerns about complications associated with excessive weight. Data on the long-term consequences of OW/OB in pediatric pwCF remain scarce.

This study aimed to compare changes in body composition and metabolic parameters in pediatric pwCF who developed OW/OB with those who maintained normal nutritional status (NNS) over 2 years of CFTR modulator therapy.

Methods

Twenty pediatric pwCF aged 12-18 who completed 2 years of CFTR modulator therapy by November 2024 were included. Ten pwCF who became OW/OB were matched with 10 who maintained NNS status, based on BMI z-scores (Wilschanski et al., 2023). Body composition was assessed using bioelectrical impedance, focusing on fat-free mass (FFM) and fat mass (FM). Fasting metabolic parameters: glucose, insulin, HOMA-IR, HbA1c, total cholesterol, HDL, LDL, and triglycerides, were measured at baseline and after two years.

Results

At baseline, no statistically significant differences were observed between the OW/OB and NNS groups. After 2 years, the OW/OB group showed significantly greater increases in FM (median 6.95 kg vs. 1.8 kg) and FFM (11 kg vs. 2.65 kg) compared to the NNS group. Median weight gain in the OW/OB group was over 4-fold higher (21.7 kg vs. 5.1 kg, p<0.001). Glucose decreased substantially in the NNS group (-11 mg/dL vs. -2 mg/dL). Insulin levels and HOMA-IR significantly increased in the OW/OB group (+4 µIU/ml, +1.37; p=0.04, p=0.01). Lipid profiles and HbA1c showed no significant differences. Changes in FM did not correlate with any metabolic parameters in either group.

Conclusion

Pediatric pwCF with OW/OB after 2 years of CFTR modulator therapy exhibited greater FM and FFM increases and significant insulin metabolism disruptions, underscoring the importance of close monitoring and tailored nutrition.

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ws12.06探索过度BMI对囊性纤维化患者代谢健康的影响：华沙CF中心CFTR调节剂的2年经验

目的cftr调节疗法改变了囊性纤维化（CF）的治疗，改善了营养状况。CF （pwCF）患者超重和肥胖（OW/OB）的出现引起了人们对超重相关并发症的关注。关于OW/OB对儿童pwCF的长期影响的数据仍然很少。本研究旨在比较发生OW/OB的儿童pwCF与在CFTR调节剂治疗2年期间维持正常营养状态（NNS）的儿童pwCF的身体组成和代谢参数的变化。方法纳入20例12-18岁的pwCF患儿，均于2024年11月前完成2年CFTR调节治疗。根据BMI z-score，将10名成为OW/OB的pwCF患者与10名保持NNS状态的pwCF患者进行匹配（Wilschanski et al., 2023）。采用生物电阻抗法评估体成分，重点关注无脂质量（fat-free mass， FFM）和脂肪质量（fat mass， FM）。空腹代谢参数：葡萄糖、胰岛素、HOMA-IR、HbA1c、总胆固醇、HDL、LDL和甘油三酯，在基线和两年后测量。结果在基线上，OW/OB组与NNS组间无统计学差异。2年后，与NNS组相比，OW/OB组的FM（中位数为6.95 kg对1.8 kg）和FFM（中位数为11 kg对2.65 kg）显著增加。OW/OB组的中位体重增加超过4倍（21.7 kg对5.1 kg, p<0.001）。NNS组血糖显著降低（-11 mg/dL vs -2 mg/dL）。OW/OB组胰岛素水平和HOMA-IR显著升高(+4 μ IU/ml, +1.37；p = 0.04, p = 0.01)。脂质谱和HbA1c无显著差异。两组的FM变化与任何代谢参数无关。结论儿童pwCF合并OW/OB患者在接受CFTR调节剂治疗2年后，FM和FFM升高，胰岛素代谢明显中断，强调密切监测和量身定制营养的重要性。

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

Journal of Cystic Fibrosis 医学-呼吸系统

CiteScore

10.10

自引率

13.50%

发文量

1361

审稿时长

50 days

期刊介绍： The Journal of Cystic Fibrosis is the official journal of the European Cystic Fibrosis Society. The journal is devoted to promoting the research and treatment of cystic fibrosis. To this end the journal publishes original scientific articles, editorials, case reports, short communications and other information relevant to cystic fibrosis. The journal also publishes news and articles concerning the activities and policies of the ECFS as well as those of other societies related the ECFS.