title: Persistent fat malabsorption in cystic fibrosis; lessons from patients and mice.
abstract: Fat malabsorption in pancreatic insufficient cystic fibrosis (CF) patients is classically treated with pancreatic enzyme replacement therapy (PERT). Despite PERT, intestinal fat absorption remains insufficient in most CF patients. Several factors have been suggested to contribute to the persistent fat malabsorption in CF (CFPFM). We reviewed the current insights concerning the proposed causes of CFPFM and the corresponding intervention studies. Most data are obtained from studies in CF patients and CF mice. Based on the reviewed literature, we conclude that alterations in intestinal pH and intestinal mucosal abnormalities are most likely to contribute to CFPFM. The presently available data indicate that acid suppressive drugs and broad spectrum antibiotics could be helpful in individual CF patients for optimizing fat absorption and/or nutritional status.
authors: Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, The Netherlands.
authors: M
authors: M
authors: Wouthuyzen-Bakker
authors: F A J A
authors: FA
authors: Bodewes
authors: H J
authors: HJ
authors: Verkade
mesh_terms: D000818:Animals; D000863:Antacids / Q000627:therapeutic use; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001647:Bile Acids and Salts / Q000378:metabolism; D003550:Cystic Fibrosis / Q000150:complications*; D010188:Exocrine Pancreatic Insufficiency / Q000209:etiology*; D005223:Fats / Q000378:metabolism*; D005228:Fatty Acids, Essential / Q000172:deficiency; D005772:Gastrointestinal Transit; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007408:Intestinal Absorption; D007413:Intestinal Mucosa / Q000378:metabolism; D008286:Malabsorption Syndromes / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism / Q000503:physiopathology; D051379:Mice; D009752:Nutritional Status
chemical_list: D000863:Antacids; D000900:Anti-Bacterial Agents; D001647:Bile Acids and Salts; D005223:Fats; D005228:Fatty Acids, Essential