Hemoglobin A1c in youth and adults with cystic fibrosis related diabetes decreases after elexacaftor-tezacaftor-ivacaftor

IF 6 2区医学 Q1 RESPIRATORY SYSTEM Journal of Cystic Fibrosis Pub Date : 2025-07-01 DOI:10.1016/j.jcf.2025.03.007

Colleen Wood , Amir Moheet , Tim Vigers , Andrea Granados , Andrea Lorenz , Elinor Hanley , Edith Zemanick , Christine L. Chan

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Abstract

Background

Elexacaftor/tezacaftor/ivacaftor (ETI) has been highly effective for improving pulmonary disease and nutritional outcomes. However, the effect of this therapy on glycemic control in people with cystic fibrosis related diabetes (CFRD) is unclear. This study aimed to examine real-world effects of ETI on glycemia as captured by hemoglobin A1c (HbA1c) in people with pre-existing CFRD.

Methods

Retrospective chart review was performed at 4 US CF centers. Individuals with CFRD included in the study started ETI before December 2020, and had an HbA1c within 1 year before and up to 2 years after ETI initiation. A sub-analysis comparing CGM data and insulin dosing within the year before and after ETI was performed. Summary statistics were calculated and within-subject results compared.

Results

A total 175 individuals with CFRD had HbA1c data before and after ETI. Mean (±SD) age was 32.4 (±12.4) years, 49.1 % female. HbA1c were compared a median (IQR) of -40 (-93, 0) days before and 290 (107, 441) days after ETI initiation. Median (IQR) HbA1c decreased from 6.4 % (5.8, 7.2) to 6.0 % (5.5, 6.8), p<0.001. A subgroup of 13 individuals had CGM and basal insulin data for comparison. No changes were observed in CGM metrics, however, basal insulin dose in these patients decreased (p=0.03).

Conclusion

Findings suggest clinical improvements in glycemia following ETI initiation in people with CFRD. Further studies are required to better understand the mechanisms by which ETI may modulate insulin and glucose dynamics in individuals with existing CFRD.

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患有囊性纤维化相关性糖尿病的青年和成人患者的糖化血红蛋白（A1c）在使用溶出因子-溶出因子-溶出因子后降低。

背景：elexaftor /tezacaftor/ivacaftor （ETI）在改善肺部疾病和营养结局方面非常有效。然而，这种疗法对囊性纤维化相关性糖尿病（CFRD）患者血糖控制的影响尚不清楚。本研究旨在研究ETI对已有CFRD患者血红蛋白A1c （HbA1c）捕获的血糖的实际影响。方法：在4个美国CF中心进行回顾性图表回顾。研究中包括的CFRD患者在2020年12月之前开始ETI，并且在ETI开始前1年内和开始后2年内有HbA1c。对ETI前后一年内的CGM数据和胰岛素剂量进行亚分析比较。计算汇总统计数据并比较受试者内结果。结果：共有175名CFRD患者在ETI前后有HbA1c数据。平均（±SD）年龄为32.4（±12.4）岁，女性占49.1%。HbA1c的中位值（IQR）在ETI开始前为-40（- 93,0）天，在ETI开始后为290（- 107,441）天。中位（IQR） HbA1c从6.4%（5.8,7.2）降至6.0%（5.5,6.8）。结论：研究结果表明，CFRD患者开始ETI后，血糖有临床改善。需要进一步的研究来更好地了解ETI可能调节现有CFRD患者胰岛素和葡萄糖动力学的机制。

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.