Original Articles: Metabolic Liver DiseaseSupplementation of ursodeoxycholic acid improves fat digestion and absorption in cystic fibrosis patients with mild liver involvementDrzymała-Czyż, Sławomiraa; Jończyk-Potoczna, Katarzynab; Lisowska, Aleksandraa; Stajgis, Marekc; Walkowiak, Jarosława Author Information Departments of aPediatric Gastroenterology and Metabolic Diseases bPediatric Radiology cRadiology, Poznań University of Medical Sciences, Poznań, Poland Correspondence to Sławomira Drzymała-Czyż, PhD, Department of Gastroenterology & Metabolic Diseases, Poznań University of Medical Sciences, Szpitalna 27/33, 60-572 Poznań, Poland Tel: +48 61 849 1432; fax: +48 61 847 2685; e-mail: [email protected] European Journal of Gastroenterology & Hepatology: June 2016 - Volume 28 - Issue 6 - p 645-649 doi: 10.1097/MEG.0000000000000593 Buy Metrics Abstract Background Ursodeoxycholic acid (UDCA) supplementation is recommended for cystic fibrosis (CF) patients with associated liver disease. However, its effect on fat digestion and absorption is not known. Materials and methods In 23 patients with mild liver involvement, a 13C-mixed triglyceride breath test was performed on UDCA supplementation (with and without pancreatic enzymes – standard and increased dose) and after 1 month of UDCA withdrawal. Cumulative percentage dose recovery [CPDR; median (interquartile range)] has been considered to reflect lipid digestion and absorption. Results The enzyme supplementation resulted in a significant CPDR improvement [0% (0–0) vs. 4.6% (0.4–6.0); P<0.00046]. With the increased dose of enzymes in 16 patients with abnormal 13C-mixed triglyceride breath test results and lipase dose less than 3000 U/g of fat, higher CPDR values [8.6% (5.6–12.7); P<0.000027] were observed. However, a 1-month UDCA withdrawal resulted in a significant reduction in (P<0.000031) fat digestion and absorption [2.9% (0.7–5.8)]. Conclusion UDCA supplementation seems to enhance lipid digestion and absorption in pancreatic insufficient CF patients with mild liver involvement. This finding points toward the potential impact of UDCA supplementation on nutritional status in CF patients with liver disease and underscores the often overlooked role of factors other than pancreatic enzymes on digestion and absorption of fats in CF. Copyright © 2016 Wolters Kluwer Health, Inc. All rights reserved.