Abstract

BackgroundGlucose abnormalities in cystic fibrosis (CF) are common, but there is limited evidence to guide their dietary management. Progressive impaired glucose tolerance eventually leads to cystic fibrosis-related diabetes (CFRD), the most prevalent complication of CF, which is associated with increased morbidity and mortality. Optimising glycaemic control improves clinical status and reduces mortality; insulin therapy is the primary means of controlling glycaemia in CFRD, but its role in managing pre-diabetes is less clear. CF dietary therapy requires a high calorie diet due to increased energy expenditure and malabsorption, but this energy-dense diet is typically high in fat and sugar, and high sugar intakes often result in hyperglycaemia in individuals who have impaired glucose handling. Current guidelines for the dietary management of glucose abnormalities in CF are based on clinical consensus rather than empirical evidence. A systematic review conducted in 2012 on the effects of low glycaemic index dietary intervention in CF concluded that there is a dearth of evidence in this area. This review will update the systematic review by Balzer et al. in 2012 and will broaden the scope of their review to include any type of dietary intervention for managing glucose abnormalities in CF.MethodsQuantitative studies of dietary interventions to manage glucose abnormalities in individuals aged over 5 years with CF and glucose abnormalities will be reviewed. No limits will be placed on language or study design. The comparator will be standard CF dietary therapy (energy dense, high-fat diet) in addition to insulin therapy for individuals with CFRD. Electronic databases will be searched for completed quantitative studies published in peer-review journals that focus on dietary interventions for managing glucose abnormalities in CF. Searches will be conducted from 2000 up to the present day to reflect the evolving improvements in CF management. No restrictions will be placed on study design or language. Duration of the dietary intervention must be a minimum of 2 months and only interventions in out-patient or community settings will be included. Studies must report on dietary intervention, glycaemic control, anthropometry and lung function. Evidence will be assessed for heterogeneity and a narrative review or meta-analysis conducted as appropriate.DiscussionThis systematic review will elucidate current knowledge of the effects of dietary interventions for managing glucose abnormalities in the vulnerable CF clinical population.Systematic review registrationPROSPERO registration number: CRD42018085569www.crd.york.ac.uk/prospero/

Highlights

  • Glucose abnormalities in cystic fibrosis (CF) are common, but there is limited evidence to guide their dietary management

  • Abnormalities in glucose metabolism are common and a spectrum of progressive glucose tolerance abnormalities occur as individuals grow older; indeterminate glycaemia is followed by impaired glucose tolerance (IGT), which affects 20% of 10-year-olds and 82% of the CF population by the age of 30 years [2], before CF-related diabetes (CFRD) eventually develops [3]

  • The aim of the current review is to update the 2012 systematic review by Balzer et al [20], to assess current understanding of glycaemic index dietary intervention in CF and to broaden the scope of their review to include any type of dietary intervention for managing glucose abnormalities in CF

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Summary

Introduction

Glucose abnormalities in cystic fibrosis (CF) are common, but there is limited evidence to guide their dietary management. Progressive impaired glucose tolerance eventually leads to cystic fibrosis-related diabetes (CFRD), the most prevalent complication of CF, which is associated with increased morbidity and mortality. There is limited evidence to guide dietary therapy for glucose abnormalities in cystic fibrosis (CF). Abnormalities in glucose metabolism are common and a spectrum of progressive glucose tolerance abnormalities occur as individuals grow older; indeterminate glycaemia is followed by impaired glucose tolerance (IGT), which affects 20% of 10-year-olds and 82% of the CF population by the age of 30 years [2], before CF-related diabetes (CFRD) eventually develops [3]. Fewer than a quarter of people with CFRD survive beyond the age of 30 years, compared with 60% of CF individuals without diabetes [12, 13]

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