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Research updates in cystic fibrosis related diabetes: Understanding pathophysiology, expanding animal and human islet models, and advancing clinical and translational research

  • Melissa S. Putman*
  • , Rebecca L. Hull-Meichle*
  • , Shafagh A. Waters
  • , Katherine Tuggle
  • , Paula Sommer
  • , Rebecca Conway
  • , Lee A. Borthwick
  • , Jakob G. Knudsen
  • , E. Danielle Dean
  • , Sarah A. Stanley
  • , Xingshen Sun
  • , John F. Engelhardt
  • , Shih Hsing Leir
  • , Lena Eliasson
  • , Bibi Uhre Nielsen
  • , Darko Stefanovski
  • , Amanda Brennan
  • , Charlotte K. Boughton
  • , Andrea Kelly
  • , Amir Moheet
  • James A.M. Shaw
*Corresponding author af dette arbejde
1 Citationer (Scopus)

Abstract

In 2024–2025, the Cystic Fibrosis Foundation (US) and Cystic Fibrosis Trust (UK) hosted an International CFRD Consortium round-table webinar series for basic science, translational, and clinical researchers with the goal of sharpening mechanistic understanding of CFRD pathogenesis and prioritizing therapeutic development. This review summarizes the research priorities identified in the International CFRD Consortium, including (i) further investigation into the role of pancreatic fibrosis, vascular abnormalities, and α-cell dysfunction in the development of CFRD; (ii) the creation and refinement of novel animal and human cell- and tissue-based models to understand the complex interplay of exocrine and endocrine cells in the CF pancreas; (iii) development and validation of circulating and imaging biomarkers, together with dynamic glucose testing to explore β-cell function and kinetics in people with CF across the dysglycemia spectrum; and (iv) prospective clinical studies to guide CFRD treatment options and investigate the changing landscape of aging, increasing prevalence of obesity and diabetes and their complications in the era of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Collectively, these priorities aim to accelerate transition from mechanism to intervention and expand evidence-based care for people with CF at risk of, or living with, CFRD.

OriginalsprogEngelsk
TidsskriftJournal of Cystic Fibrosis
Vol/bind25
Udgave nummer3
Sider (fra-til)393-405
Antal sider13
ISSN1569-1993
DOI
StatusUdgivet - maj 2026

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