Alexandre-Heymann Laure, Boudreau Valérie, Lim Dylan, Cepeda Danna, Girouard Heather, Lavoie Annick, Tremblay François, Rabasa-Lhoret Rémi, Coriati Adèle
Institut de Recherches Cliniques de Montréal, Montréal, QC, Canada.
Département de Médecine, Service de Pneumologie, Centre Hospitalier de l'Université de Montréal (CHUM), Montreal, QC, Canada.
Eur Respir Rev. 2025 May 14;34(176). doi: 10.1183/16000617.0220-2024. Print 2025 Apr.
The Montreal Cystic Fibrosis Related Diabetes Screening Cohort (MCFC) was established in 2004 to study the prevalence, risk factors and management of cystic fibrosis-related diabetes, a significant extrapulmonary complication of cystic fibrosis with an increasing prevalence due to improved cystic fibrosis survival rates. The aims of this review are to highlight the key insights gained from monitoring the MCFC over 20 years, and to discuss the challenges and advantages of establishing such a cohort in a rare disease like cystic fibrosis.
Adult people with cystic fibrosis were recruited from 2004 onward in Montreal, Canada, excluding those already diagnosed with cystic fibrosis-related diabetes. Clinical and biological results (including oral glucose tolerance tests) were recorded regularly.
Findings from the MCFC contributed to a better understanding of cystic fibrosis-related diabetes pathophysiology (in particular, the joint roles of reduced insulin secretion and added insulin resistance) and its relationship with lung function. Over the years, we observed a shift towards overweight and obesity among cystic fibrosis patients, along with improved lung function. This could be due to improved cystic fibrosis care and to the introduction of cystic fibrosis transmembrane conductance regulator modulators. We were also able to validate new, simplified screening modalities and management strategies ( physical activity) for cystic fibrosis-related diabetes.
The MCFC has contributed to the understanding of cystic fibrosis-related diabetes and informed best practice guidelines. Future research will focus on how cystic fibrosis transmembrane conductance regulator modulators influence glycaemic control and cardiometabolic health in people with cystic fibrosis.
蒙特利尔囊性纤维化相关糖尿病筛查队列（MCFC）于2004年建立，旨在研究囊性纤维化相关糖尿病的患病率、危险因素及管理情况。囊性纤维化相关糖尿病是囊性纤维化一种重要的肺外并发症，随着囊性纤维化生存率的提高，其患病率呈上升趋势。本综述的目的是强调在20年里监测MCFC所获得的关键见解，并讨论在囊性纤维化这种罕见病中建立这样一个队列的挑战和优势。
从2004年起，在加拿大蒙特利尔招募成年囊性纤维化患者，排除那些已被诊断患有囊性纤维化相关糖尿病的患者。定期记录临床和生物学结果（包括口服葡萄糖耐量试验）。
MCFC的研究结果有助于更好地理解囊性纤维化相关糖尿病的病理生理学（特别是胰岛素分泌减少和胰岛素抵抗增加的共同作用）及其与肺功能的关系。多年来，我们观察到囊性纤维化患者中超重和肥胖的情况有所增加，同时肺功能也有所改善。这可能归因于囊性纤维化护理的改善以及囊性纤维化跨膜传导调节因子调节剂的引入。我们还能够验证用于囊性纤维化相关糖尿病的新的、简化的筛查方式和管理策略（体育活动）。
MCFC有助于对囊性纤维化相关糖尿病的理解，并为最佳实践指南提供了依据。未来的研究将聚焦于囊性纤维化跨膜传导调节因子调节剂如何影响囊性纤维化患者的血糖控制和心脏代谢健康。
