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primary studies published RCT

Ursodeoxycholic acid improves the hepatic metabolism of essential fatty acids and retinol in children with cystic fibrosis.

  • Year: 1997
  • Date: 1997
  • Author: Lepage G
Source

The Journal of pediatrics YR: 1997 VL: 130 NO: 1

Study design (if review, criteria of inclusion for studies)

Double-blind, placebo-controlled, randomised cross-over trial. 1 year duration (2 periods of 6 months each).

Participants

19 (13 males; 6 girls) children with CF and liver dysfunction, aged 7 - 17 years (mean (SD) 11. 9 (0. 6) years). 6 withdrawals (1 died, 4 moved away, 1 discontinued medication).

Interventions

UDCA (15 mg/kg/day) versus placebo.

Outcome measures

Liver function tests (AST, ALT, GGT), plasma lipid levels (total fatty acids, triglycerides, cholesterol), plasma RBP, transthyretin, retinol, retinyl ester levels.

Main results

At entry, all patients had biochemical evidence of EFA deficiency. The lipid profiles during an average period of 25 months of follow-up showed a significant decrease in triglycerides (p <0.002), cholesterol (p <0.02), and total fatty acids (p <0.006). In addition, UDCA therapy led to an improvement in EFA status, as indicated by an increase (p <0.05) in the n-6 fatty acid concentration and a reduction (p <0.04) in the 20:3n-9/20:4n-6 fatty acid ratio. Although no change in vitamin E levels was observed, retinol metabolism was altered. There was an increase (p <0.02) in the unesterified retinol/retinol binding protein molar ratio in the absence of a difference in retinol binding protein concentration. Furthermore, retinyl esters, which normally account for less than 3% of circulating retinol, decreased (p <0.05) from 13.7% +/- 3.6% to 8.1% +/- 1.7%.

Authors' conclusions

This study confirms that UDCA alters lipoprotein metabolism and shows that it improves the EFA and retinol status of patients with CF and liver disease.

Keywords: Adolescent; Child; Cholagogues and Choleretics; non pharmacological intervention - diet; pharmacological_intervention; UDCA; essential fatty acids; Liver Diseases; Gastrointestinal Diseases; Gastrointestinal Agents;