Abstract
Cystic fibrosis (CF) is the most common lethal, multisystemic genetic disorder in Caucasians. Mutations in the gene encoding the cystic fibrosis transmembrane regulator (CFTR) protein are responsible for impairment of epithelial anionic transport, leading to impaired fluid regulation and pH imbalance across multiple organs. Gastrointestinal (GI) manifestations in CF may begin in utero and continue throughout the life, resulting in a chronic state of an altered intestinal milieu. Inherent dysfunction of CFTR leads to dysbiosis of the gut. This state of dysbiosis is further perpetuated by acquired factors such as use of antibiotics for recurrent pulmonary exacerbations. Since the gastrointestinal microbiome and their metabolites play a vital role in nutrition, metabolic, inflammatory, and immune functions, the gut dysbiosis will in turn impact various manifestations of CF—both GI and extra-GI. This review focuses on the consequences of gut dysbiosis and its metabolic implications on CF disease and possible ways to restore homeostasis.
Highlights
Manuel Suárez Recio and Cystic fibrosis (CF) is the most common autosomal recessive disease among Caucasians, affecting more than 30,000 patients in the United States and almost 2.5 times this number worldwide [1]. It is characterized by mutations in the gene coding for the cystic fibrosis transmembrane regulator (CFTR) protein
This review focuses on the cuses on the implications of gut dysbiosis in CF
Along with reduced gut microbial diversity, there is a significant alteration of microbial composition in patients with CF [9,26,30,37,39,40,50,55] (Table 4 and Figure 1), For example, an increase in Firmicutes, a reduction in Bacteroidetes, along with a higher abundance of pro-inflammatory microbiota such as those belonging to Enterobacteriaceae, Streptococcus, and Veillonella are consistently reported in patients with CF [6,45]
Summary
Manuel Suárez Recio and Cystic fibrosis (CF) is the most common autosomal recessive disease among Caucasians, affecting more than 30,000 patients in the United States and almost 2.5 times this number worldwide [1]. Reduced gut microbial diversity is observed in several i metabolic, immune-related, and systemic diseases [8,9,10]. Non-homeostatic imbalance metabolic, immune-related, Host–microbe interactions host entities such as imm in the composition, diversity, or function of theseimpact residentvarious microorganisms is termed metabolic pathways, growth, and development, yet many of the mechanism dysbiosis [7,11,12,13,14,15]. About 80% of gut microorganisms c temporal changestured and clinical significance of gut dysbiosis and itsTechnologic effects on theadvancements disease proby standard culture methods [14,17].
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