title: Supplementation of ursodeoxycholic acid improves fat digestion and absorption in cystic fibrosis patients with mild liver involvement.
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.
  
  
  METHODS
  In 23 patients with mild liver involvement, a C-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 C-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)].
  
  
  CONCLUSIONS
  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.
authors.affiliation: Departments of aPediatric Gastroenterology and Metabolic Diseases bPediatric Radiology cRadiology, Poznań University of Medical Sciences, Poznań, Poland.
authors.forename: Sławomira
authors.initials: S
authors.lastname: Drzymała-Czyż
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Jończyk-Potoczna
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Lisowska
authors.forename: Marek
authors.initials: M
authors.lastname: Stajgis
authors.forename: Jarosław
authors.initials: J
authors.lastname: Walkowiak
mesh_terms: D000293:Adolescent; D000328:Adult; D001944:Breath Tests; D002247:Carbon Isotopes; D002648:Child; D002675:Child, Preschool; D002756:Cholagogues and Choleretics / Q000627:therapeutic use*; D003550:Cystic Fibrosis / Q000150:complications / Q000378:metabolism*; D004041:Dietary Fats / Q000378:metabolism*; D019587:Dietary Supplements; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000188:drug therapy* / Q000209:etiology / Q000378:metabolism; D008297:Male; D014280:Triglycerides / Q000378:metabolism; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D002247:Carbon Isotopes; D002756:Cholagogues and Choleretics; D004041:Dietary Fats; D014280:Triglycerides; D014580:Ursodeoxycholic Acid