Abstract
Cystic fibrosis (CF) is a life-shortening genetic disorder that affects the cystic fibrosis transmembrane conductance regulator (CFTR) protein. In the gastrointestinal (GI) tract, CFTR dysfunction results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, and reduced motility. These mechanisms, combined with antibiotic therapies, drive GI inflammation and significant alteration of the GI microbiota (dysbiosis). Dysbiosis and inflammation are key factors in systemic inflammation and GI complications including malignancy. The following review examines the potential for probiotic and prebiotic therapies to provide clinical benefits through modulation of the microbiome. Evidence from randomised control trials suggest probiotics are likely to improve GI inflammation and reduce the incidence of CF pulmonary exacerbations. However, the highly variable, low-quality data is a barrier to the implementation of probiotics into routine CF care. Epidemiological studies and clinical trials support the potential of dietary fibre and prebiotic supplements to beneficially modulate the microbiome in gastrointestinal conditions. To date, limited evidence is available on their safety and efficacy in CF. Variable responses to probiotics and prebiotics highlight the need for personalised approaches that consider an individual’s underlying microbiota, diet, and existing medications against the backdrop of the complex nutritional needs in CF.
Highlights
Cystic fibrosis (CF) is a genetic condition of autosomal recessive inheritance related to mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein [1]
This review describes the physiology of the GI tract in CF and the clinical relevance of GI microbiome dysbiosis and inflammation
While a clear causative mechanism has yet to be established in the context of CF, it is well recognised from studies pertaining to inflammatory bowel disease (IBD) that chronic inflammation poses a significant risk for the development of GI cancers [139,140]
Summary
Cystic fibrosis (CF) is a genetic condition of autosomal recessive inheritance related to mutations in the gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein [1]. Dysfunction of the CFTR protein in the GI system results in low intestinal pH, thick and inspissated mucus, a lack of endogenous pancreatic enzymes, reduced motility, and possibly an impaired innate immunity [5,6,7] (Figure 1). These mechanisms are proposed drivers of local GI inflammation and contribute to a range of intestinal morbidities, including an increased risk of early-onset adult GI cancer [8,9,10,11]. We discuss the current understanding of probiotic and prebiotic mechanisms of action, provide important examples of clinical studies examining probiotic and prebiotic applications in CF, and discuss considerations for clinical translation
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