Pediatric Pulmonology, Journal Year: 2024, Volume and Issue: unknown
Published: Nov. 28, 2024
Cystic fibrosis transmembrane conductance regulator modulator therapies (CFTR-MT) have altered management, reducing exacerbations and slowing pulmonary function decline. Nevertheless, it is still uncertain if the benefits of CFTR-MTs last when they are stopped. This study aimed to assess changes, exacerbation rates during after CFTR-MT use in adult cystic patients.
Language: Английский
European Respiratory Review, Journal Year: 2025, Volume and Issue: 34(176), P. 240220 - 240220
Published: April 1, 2025
Introduction 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 fibrosis with an increasing prevalence due improved survival rates. aims this review are highlight key insights gained from monitoring MCFC over 20 years, discuss challenges advantages establishing such cohort rare disease like fibrosis. Methods Adult people were recruited onward Montreal, Canada, excluding those already diagnosed diabetes. Clinical biological results (including oral glucose tolerance tests) recorded regularly. Results Findings contributed better understanding diabetes pathophysiology (in particular, joint roles reduced insulin secretion added resistance) its relationship lung function. Over we observed shift towards overweight obesity among patients, along This could be care introduction transmembrane conductance regulator modulators. We also able validate new, simplified screening modalities strategies ( e.g. physical activity) for Conclusion has informed best practice guidelines. Future research will focus on how modulators influence glycaemic control cardiometabolic health
Language: Английский
Citations
0
Pediatric Pulmonology, Journal Year: 2024, Volume and Issue: unknown
Published: Nov. 28, 2024
Cystic fibrosis transmembrane conductance regulator modulator therapies (CFTR-MT) have altered management, reducing exacerbations and slowing pulmonary function decline. Nevertheless, it is still uncertain if the benefits of CFTR-MTs last when they are stopped. This study aimed to assess changes, exacerbation rates during after CFTR-MT use in adult cystic patients.
Language: Английский
Citations
0