BACKGROUND Previous studies have suggested the existence of enteropathy in cystic fibrosis (CF), which may contribute to intestinal function impairment, a poor nutritional status and decline in lung function. This study evaluated enterocyte damage and intestinal inflammation in CF and studied its associations with nutritional status, CF-related morbidities such as impaired lung function and dia...

The cause of insulin insufficiency remains unknown in many diabetic cases. Up to 50% adult patients with cystic fibrosis (CF), a disease caused by mutations in the gene encoding the CF transmembrane conductance regulator (CFTR), develop CF-related diabetes (CFRD) with most patients exhibiting insulin insufficiency. Here we show that CFTR is a regulator of glucose-dependent electrical acitivitie...

The cause of cystic fibrosis-related diabetes (CFRD) remains unknown, but cystic fibrosis transmembrane conductance regulator (CFTR) mutations contribute directly to multiple aspects of the cystic fibrosis phenotype. We hypothesized that susceptibility to islet dysfunction in cystic fibrosis is determined by the lack of functional CFTR. To address this, glycemia was assessed in CFTR null (CFTR(...

Objectives: People with or without Cystic Fibrosis-Related Diabetes (CFRD) have increased prevalence of hypoglycaemia, related to insulin therapy and reactive hypoglycaemia. type 1 2 diabetes mellitus are at risk hypoglycaemia within 48hr after exercise, however this has not been objectively measured in people CF. We aimed investigate the using flash glucose monitoring, moderate intensity aerob...

Objectives: We assessed the impact of ELX/TEZ/IVA treatment on glucose tolerance in people with CF and either impaired (IGT) or CF-related diabetes (CFRD) over a 48-week period. Methods: VX19-445-117 (NCT04599465) was Phase 3b, single-arm, open-label study aged ≥12 years heterozygous for F508del minimal function mutation who had abnormal as determined by oral testing (OGTT), IGT CFRD at screeni...

The plinth elevation angle was related to the upstream slope and face slab shape of CFRD, as well dam axis, which very important in design. Based on space vector algorithm, function established consists three-dimensional coordinate key points line, taken unique input variable, then directly obtained. improved method with characteristics high efficiency, simple parameters clear spatial concept, ...