ArticleScientific reports2017
Structural abnormalities in islets from very young children with cystic fibrosis may contribute to cystic fibrosis-related diabetes.
Article in Scientific reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 45 papers.
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Who cites it
45 citing papers in PubMed, 70 citations in OpenAlex.
- Unravelling the developmental origins of cystic fibrosis-related diabetes.Diabetologia · 2026Review
- Review
- The endocrine complications of cystic fibrosis.Nature reviews. Endocrinology · 2026Review
- Cystic Fibrosis of the Pancreas: In Vitro Duct Models for CFTR-Targeted Translational Research.International journal of molecular sciences · 2026Review
- Relationship between impaired glucose metabolism and bone mineral density in patients with cystic fibrosis.European journal of pediatrics · 2026Article
- Elexacaftor-tezacaftor-ivacaftor enhances first-phase insulin secretion and improves glucose control in cystic fibrosis.Endocrine connections · 2026Article
- Article
- Single cell and spatial characterization of the human pancreas reveals drivers of beta cell dysfunction in cystic fibrosis.bioRxiv : the preprint server for biology · 2025Article
- SGLT2 inhibitor therapy in overweight and obese patients with cystic fibrosis-related diabetes: Case series.Journal of clinical & translational endocrinology · 2025Review
- Utility of Continuous Glucose Monitors for Improved Detection of Cystic Fibrosis-Related Diabetes.Pediatric pulmonology · 2025Article
- Identification of a vimentin-expressing α-cell phenotype in CF and normal pancreas.The Journal of endocrinology · 2025Article
- Pancreatic endocrine and exocrine signaling and crosstalk in physiological and pathological status.Signal transduction and targeted therapy · 2025Review
- E3 ligase substrate adaptor SPOP fine-tunes the UPR of pancreatic β cells.Genes & development · 2025Article
- Cystic fibrosis-related diabetes is associated with reduced islet protein expression of GLP-1 receptor and perturbation of cell-specific transcriptional programs.Scientific reports · 2024Article
- Pancreatic Crosstalk in the Disease Setting: Understanding the Impact of Exocrine Disease on Endocrine Function.Comprehensive Physiology · 2024Review
- Diagnosis and Management of Gastrointestinal Manifestations in Children with Cystic Fibrosis.Diagnostics (Basel, Switzerland) · 2024Review
- Preservation of β-cell Function in Pancreatic Insufficient Cystic Fibrosis With Highly Effective CFTR Modulator Therapy.The Journal of clinical endocrinology and metabolism · 2023Article
- Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes.Journal of clinical & translational endocrinology · 2023Article
- CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes.Scientific reports · 2023Article
- Cystic Fibrosis-Related Diabetes Workshop: Research Priorities Spanning Disease Pathophysiology, Diagnosis, and Outcomes.Diabetes care · 2023Review
Corrections and comments
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Authors and funding
6 authors at 6 institutions in 3 countries.
Funding
Abstract
Cystic fibrosis (CF)-related diabetes (CFRD) is thought to result from beta-cell injury due in part to pancreas exocrine damage and lipofibrosis. CFRD pancreata exhibit reduced islet density and altered cellular composition. To investigate a possible etiology, we tested the hypothesis that such changes are present in CF pancreata before the development of lipofibrosis. We evaluated pancreas and islet morphology in tissues from very young CF children (<4 years of age), and adult patients with CF and CFRD. The relative number of beta-cells in young CF tissues was reduced by 50% or more when compared to age-matched controls. Furthermore, young CF tissues displayed significantly smaller insulin-positive areas, lower proportion of beta-cells positive for the proliferation marker Ki67 or the ductal marker CK19 vs. control subjects, and islet inflammatory cell infiltrates, independently of the severity of the exocrine lesion and in the absence of amyloid deposits. CFRD pancreata exhibited greater islet injury with further reduction in islet density, decreased relative beta-cell number, and presence of amyloid deposits. Together, these results strongly suggest that an early deficiency in beta-cell number in infants with CF may contribute to the development of glucose intolerance in the CF pediatric population, and to CFRD, later in life.
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Registered trials
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