Cystic Fibrosis in the Intestine and the Influence on Digestion

Diagnosis and Management of Gastrointestinal Manifestations in Children with Cystic Fibrosis

“…CF exerts its influence on the G-I tract from the uterine and neonatal stages, persisting throughout an individual's life [1,9]. G-I symptoms, observed in approximately 85% of cases, tend to be more prevalent in patients with severe disease or genotypes associated with moderate or severe abdominal involvement, potentially contributing to heightened morbidity and mortality among CF patients [10].…”

“…The CF gut operates within a deleterious cycle involving impaired luminal flux due to the viscous mucus layer, epithelial inflammation, infection, and/or dysbiosis [13]. The G-I damage observed in CF is attributed to the alteration of intestinal secretion, absence of pancreatic fluids containing enzymes, dysbiosis, and intestinal inflammation, as illustrated in Figure 2 [1,8,14,15]. The dehydration of secretions leads to intraductal blockage, inflammation, fibrosis, and potential organic destruction in the presence of digestive enzymes [16][17][18][19].…”

“…Inflammation further enhances Escherichia coli colonization of the intestinal mucosa. Conversely, E. coli can contribute to inflammation, impairing metabolism and lipid absorption, leading to malnutrition and symptom exacerbation [1]. Antibiotic use is recognized as an iatrogenic factor in CF associated with gut inflammation.…”

“…Cystic fibrosis (CF) is an intricate multiorgan disorder affecting epithelial organs, including the respiratory tract, exocrine pancreas, intestine, hepatobiliary system, sweat glands, and, more recently, myeloid cells in secretory vesicle membranes [1][2][3]. It stands as the most prevalent autosomal recessive monogenic disease, exhibiting a chronic, progressive, and potentially fatal course in the Caucasian population.…”

Role of innate immunity and systemic inflammation in cystic fibrosis disease progression

“…Loss of CFTR also affects normal functioning of the digestive system. Modulation of Cl − concentration is vital for maintaining the composition of saliva, gastric juice, mucus, and commensal microbiota as well as processes, like food digestion, nutrient absorption, and excretion [ [68] , [69] , [70] , [71] ]. And since Cl − transport is predominantly controlled by CFTR, complications due to an ineffective digestive system is quite inevitable in CF ( Fig.…”