There is evidence of intestinal inflammation in patients with CF. Intestinal inflammation may negatively impact the nutritional status of patient with CF, which adversely affects pulmonary function and survival. This paper provides an up-to-date review of intestinal inflammation in CF and an evaluation of utility of two specific faecal inflammatory markers (S100A12 and calprotectin). 1. Introduction Cystic fibrosis (CF) is the most common, life-shortening, recessive disease in Caucasians with an average life expectancy of 40 years [1]. In the majority of cases, mortality in CF is due to respiratory failure. CF is caused by mutations in the gene that encodes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein [2, 3]. The CFTR protein functions on the apical surface of epithelial cells as a cyclic AMP-dependent chloride and a bicarbonate channel [4, 5]. Absent or defective CFTR leads to viscous luminal secretions in affected organs particularly in the lungs, intestines, and pancreas. The nutritional status of CF patients is a major determinant of pulmonary and survival outcomes. Longitudinal cohort studies in CF report a distinct survival advantage among patients with better nutritional status [6–8]. More specifically, poor nutritional status is not only strongly linked to poorer lung function but was also an independent risk factor for early death in children with CF. Several factors contribute to impaired nutritional status in CF. These include malabsorption, recurrent sinopulmonary infections, increased energy expenditure, and suboptimal intake [9]. The malabsorptive state in CF is likely multifactorial. The primary cause of malabsorption is due to maldigestion from pancreatic exocrine insufficiency. However, children with CF can continue to have malabsorption despite pancreatic enzyme replacement therapy (PERT) administration. It has been previously suggested that the presence of an acidic intestinal milieu, that impairs enzyme activity, contributes to the failure of PERT in nutrient assimilation in CF. More recently, intestinal inflammation has been hypothesized as another contributing factor. 2. Intestinal Inflammation in Cystic Fibrosis There is increasing evidence of intestinal inflammation in CF. Although the exact underlying mechanism is unknown, there have been several pathogenic mechanisms proposed (Figure 1). As a primary consequence of a defective CFTR, intestinal mucus secretions are viscid and inspissated due to dehydration, are in an acidic milieu, and have altered glycosylation of mucins [10, 11]. Such
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