Ursodeoxycholic acid treatment is associated with improvement of liver stiffness in cystic fibrosis patients

Cathelijne van der Feen; Hubert P J van der Doef; Cornelis K van der Ent; Roderick H J Houwen

doi:10.1016/j.jcf.2016.07.009

Ursodeoxycholic acid treatment is associated with improvement of liver stiffness in cystic fibrosis patients

J Cyst Fibros. 2016 Nov;15(6):834-838. doi: 10.1016/j.jcf.2016.07.009.

Authors

Cathelijne van der Feen¹, Hubert P J van der Doef², Cornelis K van der Ent³, Roderick H J Houwen⁴

Affiliations

¹ Department of Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, The Netherlands.
² Department of Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, The Netherlands; Department of Pediatric Gastroenterology, University Medical Center Groningen, Groningen, The Netherlands.
³ Department of Pediatric Pulmonology, University Medical Center Utrecht, Utrecht, The Netherlands.
⁴ Department of Pediatric Gastroenterology, University Medical Center Utrecht, Utrecht, The Netherlands. Electronic address: [email protected].

PMID: 27481229
DOI: 10.1016/j.jcf.2016.07.009

Abstract

Background: Ursodeoxycholic acid (UDCA) might prevent progression of cystic fibrosis liver disease, but objective parameters for its effect are lacking.

Methods: We used liver stiffness measurements to evaluate the effect of Ursodeoxycholic acid.

Results: Paired measurements of liver stiffness were done in 73 patients without UDCA and in 32 patients with UDCA. In the latter group, 6 patients had cirrhosis; in 15 patients, UDCA was started based on Colombo criteria, and in 11 patients for other reasons. In patients without UDCA, liver stiffness increased: 0.19 (-0.03 to 0.59)kPa/year. Liver stiffness also increased in patients with cirrhosis: 4.6 (0.67-12.4)kPa/year. In patients who had UDCA based on Colombo criteria, a decrease of liver stiffness was observed: 0.70 (-1.6 to 0.55)kPa/year (P=0.01). In patients on UDCA for other reasons, liver stiffness increased: 0.23 (-0.20 to 0.51)kPa/year.

Conclusion: UDCA reduced liver stiffness in patients with well-defined, mild liver disease.

Keywords: Cystic fibrosis liver disease; Fibroscan; Liver stiffness measurement; Ursodeoxycholic acid.

Publication types

Observational Study
Research Support, Non-U.S. Gov't

MeSH terms

Adolescent
Child
Cholagogues and Choleretics / administration & dosage
Cystic Fibrosis* / complications
Cystic Fibrosis* / epidemiology
Drug Monitoring / methods
Elasticity Imaging Techniques / methods
Female
Humans
Liver Cirrhosis* / diagnosis
Liver Cirrhosis* / drug therapy
Liver Cirrhosis* / etiology
Liver Function Tests / methods
Liver* / diagnostic imaging
Liver* / drug effects
Liver* / pathology
Male
Netherlands / epidemiology
Treatment Outcome
Ultrasonography / methods
Ursodeoxycholic Acid / administration & dosage*

Substances

Cholagogues and Choleretics
Ursodeoxycholic Acid