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胰高血糖素样肽-1激动剂疗法用于成年囊性纤维化患者。

Glucagon-like-peptide-1 agonist therapy in adults with cystic fibrosis.

作者信息

Park Sanghoon, Jain Raksha, Mirfakhraee Sasan

机构信息

University of Texas Southwestern Medical Center, Department of Internal Medicine, Dallas, TX, United States.

University of Texas Southwestern Medical Center, Division of Pulmonary and Clinical Care Medicine, Dallas, TX, United States.

出版信息

J Cyst Fibros. 2025 Jan;24(1):40-46. doi: 10.1016/j.jcf.2024.08.005. Epub 2024 Aug 30.

DOI:10.1016/j.jcf.2024.08.005
PMID:39214747
Abstract

Glucagon-like-peptide-1 (GLP-1) agonists are commonly used to improve glycemic control and promote weight loss in individuals with type 2 diabetes mellitus (T2DM) and/or obesity. However, there is a paucity of evidence regarding GLP-1 agonist use in people with cystic fibrosis (pwCF). We present 11 people with CF (males: 3, females: 7; age range 24-47; BMI range 25.7-43.7) treated with GLP-1 agonists (semaglutide: 9,tirzepatide: 2) for variable duration (1-50 months). All experienced weight loss on GLP- 1 agonist therapy (median change in weight = -7.2 kg; change in BMI [kg/m2] = -0.9 to -8.1). Eight pwCF showed improvement in percent predicted forced expiratory volume in 1 second (ppFEV1) [change = -5 to + 18] and nine pwCF showed improvement in percent predicted forced vital capacity (ppFVC) [change= +1 to + 26]. Of the 7 pwCF with CFRD, all reduced their insulin quantity (mean, 31.5 % decrease in total daily insulin dose), and glucose time in range improved for most (mean, +11 % increase from baseline). Four pwCF stopped using GLP-1 agonists: 2 due to severe nausea/vomiting, 1 due to lack of perceived benefit, and 1 due to change in insurance coverage. This report is the largest published series to date of pwCF treated with GLP-1 agonist therapy. With the addition of GLP-1 agonists, all individuals experienced weight loss and a reduction in daily insulin dose, and most had improvement in pulmonary function. Future multi-center studies are needed to corroborate the efficacy and safety of these agents in the CF population.

摘要

胰高血糖素样肽-1(GLP-1)激动剂常用于改善2型糖尿病(T2DM)和/或肥胖患者的血糖控制并促进体重减轻。然而,关于GLP-1激动剂在囊性纤维化患者(pwCF)中的使用证据不足。我们报告了11例接受GLP-1激动剂(司美格鲁肽:9例,替尔泊肽:2例)治疗不同时长(1-50个月)的CF患者(男性:3例,女性:7例;年龄范围24-47岁;BMI范围25.7-43.7)。所有患者在接受GLP-1激动剂治疗后体重均减轻(体重中位数变化=-7.2 kg;BMI变化[kg/m²]=-0.9至-8.1)。8例pwCF的1秒用力呼气容积预测值百分比(ppFEV1)有所改善[变化=-5至+18],9例pwCF的用力肺活量预测值百分比(ppFVC)有所改善[变化=+1至+26]。在7例患有囊性纤维化相关糖尿病(CFRD)的pwCF中,所有患者的胰岛素用量均减少(每日总胰岛素剂量平均减少31.5%),大多数患者的血糖达标时间有所改善(平均较基线增加11%)。4例pwCF停止使用GLP-1激动剂:2例因严重恶心/呕吐,1例因未感觉到益处,1例因保险覆盖范围变更。本报告是迄今为止已发表的接受GLP-1激动剂治疗的pwCF患者的最大系列病例。加用GLP-1激动剂后,所有患者体重减轻且每日胰岛素剂量减少,大多数患者的肺功能有所改善。未来需要多中心研究来证实这些药物在CF人群中的疗效和安全性。

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引用本文的文献

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The State of Weight in Cystic Fibrosis: Understanding Nutritional Status and Individualizing Nutritional Care in the Modulator Era.囊性纤维化患者的体重状况:在调节剂时代理解营养状况并实现营养护理个体化
Nutrients. 2025 Jul 31;17(15):2533. doi: 10.3390/nu17152533.
2
Glucagon-Like Peptide 1 Agonist Use in an Adult With Cystic Fibrosis-Related Diabetes and Metabolic Syndrome.胰高血糖素样肽1激动剂在一名患有囊性纤维化相关糖尿病和代谢综合征的成人中的应用。
AACE Endocrinol Diabetes. 2025 Apr 11;12(2):67-70. doi: 10.1016/j.aed.2025.03.011. eCollection 2025 Jul-Aug.
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Evolving nutrition therapy in cystic fibrosis: Adapting to the CFTR modulator era.
囊性纤维化中不断发展的营养治疗:适应CFTR调节剂时代。
Nutr Clin Pract. 2025 Aug;40(4):816-828. doi: 10.1002/ncp.11332. Epub 2025 Jun 18.
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Glucagon-like peptide-1 receptor agonists in adults with cystic fibrosis-related diabetes: Rationale and emerging evidence.成人囊性纤维化相关糖尿病患者使用胰高血糖素样肽-1受体激动剂:理论依据与新证据
Diabetes Obes Metab. 2025 Sep;27(9):4621-4626. doi: 10.1111/dom.16516. Epub 2025 Jun 9.
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The Aging Patient with Cystic Fibrosis.患有囊性纤维化的老年患者
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