Persistent Fat Malabsorption in Cystic Fibrosis

Despite pancreas enzyme replacement therapy, intestinal fat absorption remains insufficient in most cystic fibrosis (CF) patients. We reviewed the potential causes of CF-related persistent intestinal fat malabsorption. Data are obtained from studies in patients and in CF experimental animal models. Based on the physiology of intestinal fat absorption, we discuss the pathology in CF conditions. The chapter covers CF-related alterations of intestinal pH and bicarbonate secretion, bile salt metabolism, and enterohepatic circulation and intestinal mucosal abnormalities.