Cystic Fibrosis-Related Diabetes: Clinical approach and knowledge gaps

Bernadette J Prentice; Kathryn J Potter; Adèle Coriati; Valérie Boudreau; Leah Rusnell; Tamizan Kherani; Peter A Senior; Shihab Hameed; Rémi Rabasa-Lhoret

doi:10.1016/j.prrv.2022.10.001

Cystic Fibrosis-Related Diabetes: Clinical approach and knowledge gaps

Paediatr Respir Rev. 2023 Jun:46:3-11. doi: 10.1016/j.prrv.2022.10.001. Epub 2022 Oct 5.

Authors

Bernadette J Prentice¹, Kathryn J Potter², Adèle Coriati², Valérie Boudreau³, Leah Rusnell⁴, Tamizan Kherani⁴, Peter A Senior⁵, Shihab Hameed⁶, Rémi Rabasa-Lhoret⁷

Affiliations

¹ Discipline of Pediatrics, School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, New South Wales, Australia; Respiratory Department, Sydney Children's Hospital, Randwick, Sydney, New South Wales, Australia.
² Montreal Clinical Research Institute (IRCM), Montréal, Québec, Canada.
³ Montreal Clinical Research Institute (IRCM), Montréal, Québec, Canada; Department of Nutrition, Faculty of Medicine, Université de Montréal, Montréal, Québec, Canada.
⁴ Department of Pediatric Respirology, Stollery Children's Hospital, Edmonton, Alberta, Canada.
⁵ Alberta Diabetes Institute, University of Alberta, Edmonton, Alberta, Canada.
⁶ School of Women's and Children's Health, University of New South Wales, Sydney, Randwick, Australia; Faculty of Medicine, University of Sydney, Sydney, Australia; Dept of Endocrinology, Sydney Children's Hospital, Randwick, Australia; Dept of Paediatric Endocrinology Royal North Shore Hospital, Sydney, Australia.
⁷ Montreal Clinical Research Institute (IRCM), Montréal, Québec, Canada; Department of Nutrition, Faculty of Medicine, Université de Montréal, Montréal, Québec, Canada; Division of Experimental Medicine, Faculty of Medicine, McGill University, Montréal, Québec, Canada; Department of Medicine, Faculty of Medicine, Université de Montréal, Montréal, Québec, Canada; Cystic Fibrosis Clinic, Centre Hospitalier de l'Université de Montréal (CHUM), Montréal, Québec, Canada. Electronic address: remi.rabasa-lhoret@ircm.qc.ca.

PMID: 36376223
DOI: 10.1016/j.prrv.2022.10.001

Abstract

Cystic Fibrosis-Related Diabetes (CFRD) is a unique type of diabetes mellitus that shares some features with both type 1 and type 2 diabetes. Yet, its distinguishing feature of acute pulmonary complications associated with hyperglycemia and the catabolic metabolism associated with a relative insulin deficiency poses challenges to the application of traditional definitions and treatments for diabetes mellitus. People with CF (pwCF) undergo rigorous annual screening starting at age 10, a process that is challenging for patients and limited by sensitivity, specificity, and reproducibility. As pwCF continue to live longer, over 50% are expected to develop CFRD over their lifetime, including up to 20% of adolescents. Increasing numbers of people with CFRD will make this disease increasingly relevant to diabetes practitioners. Evidence-guided practice in CFRD care is limited by small and short studies. Our current understanding of CFRD may change significantly with the recent introduction of CF Transmembrane Regulator (CFTR) modulator medications. This review will explore current challenges in the diagnosis and management of CFRD, specifically highlighting knowledge gaps in the pathophysiology of CFRD, optimal screening methods, priorities for research and provide guidance with regards to screening, diagnosis, and treatment.

Keywords: Cystic Fibrosis related diabetes mellitus [CFRDM]; Management; Pathophysiology; Screening.

Publication types

Review

MeSH terms

Adolescent
Child
Cystic Fibrosis* / drug therapy
Cystic Fibrosis* / therapy
Diabetes Mellitus* / diagnosis
Diabetes Mellitus* / epidemiology
Diabetes Mellitus* / etiology
Diabetes Mellitus, Type 2* / complications
Diabetes Mellitus, Type 2* / drug therapy
Humans
Insulin / therapeutic use
Mass Screening
Reproducibility of Results

Substances

Insulin