Abstract
Cystic fibrosis (CF) is a life-limiting autosomal recessive multisystem disease. While its burden of morbidity and mortality is classically associated with pulmonary disease, CF also profoundly affects the gastrointestinal (GI) tract. Chronic low-grade inflammation and alterations to the gut microbiota are hallmarks of the CF intestine. The etiology of these manifestations is likely multifactorial, resulting from cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction, a high-fat CF diet, and the use of antibiotics. There may also be a bidirectional pathophysiological link between intestinal inflammation and changes to the gut microbiome. Additionally, a growing body of evidence suggests that these GI manifestations may have significant clinical associations with growth and nutrition, quality of life, and respiratory function in CF. As such, the potential utility of GI therapies and long-term GI outcomes are areas of interest in CF. Further research involving microbial modulation and multi-omics techniques may reveal novel insights. This article provides an overview of the current evidence, pathophysiology, and future research and therapeutic considerations pertaining to intestinal inflammation and alterations in the gut microbiota in CF.
Highlights
Cystic fibrosis (CF) is a life-limiting multisystem autosomal recessive disease most commonly occurring in Caucasian populations [1,2,3,4]
Some studies have reported that patients with exocrine pancreatic insufficiency have significantly higher fecal calprotectin than their pancreatic sufficient counterparts [40,42], whereas other studies have found no difference between the two groups [38,53]
Infants with CF who had been hospitalised for GI-related indications exhibited lower growth parameters than those who had not been hospitalised. These findings suggest that intestinal inflammation may relate to growth but may have associations with morbidity and wellbeing due to hospitalisations in infancy [45]
Summary
Cystic fibrosis (CF) is a life-limiting multisystem autosomal recessive disease most commonly occurring in Caucasian populations [1,2,3,4]. While the burden of morbidity and mortality in CF is classically associated with pulmonary disease, CFTR dysfunction has far-reaching extrapulmonary sequelae [8,9]. These include exocrine pancreatic insufficiency [10], pancreatitis [11,12], liver disease [13,14,15,16], CF-related diabetes [17,18], and male infertility [19,20].
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