Examination of the Lipid Metabolism of the Liver After Choline Substitution in Cystic Fibrosis

University Hospital Tuebingen

Status

Completed

Conditions

Cystic Fibrosis Liver Disease

Treatments

Drug: Choline Chloride

Study type

Interventional

Funder types

Other

Identifiers

NCT03312140

Phos-CF II V1.2

Details and patient eligibility

About

Pilot study to investigate the effect of choline chloride in cystic fibrosis patients with liver steatosis by comparing their status before and after the intervention

Full description

Within this single arm pilot study patients with cystic fibrosis and liver steatosis receive choline chloride (2x0.5 gram three times a day) as a food supply for 88 days. D9-choline metabolism is measured before and after the intervention phase. Hypothesis of the study is, that supplying choline augments the secretion of phosphatidyl choline and lipoprotein (VLDL) from the liver and therefore results in a measurable reduction of the triglyceride storage of the liver.

Enrollment

10 patients

Sex

Male

Ages

18+ years old

Volunteers

No Healthy Volunteers

Inclusion criteria

Age > 18 years
male
Cystic fibrosis is verified
pancreatic insufficiency
Decline of plasma phosphatidylcholine and steatosis of the liver
informed consent is given

Exclusion criteria

cirrhosis of the liver
Hyperreactivity to choline containing food
allergic bronchopulmonary aspergillosis or other acute exacerbations of pulmonary infections
FEV1 < 40%
smoker
chronic alcohol consume
clinically relevant diseases (for example thyroid dysfunction, disturbance of lipid metabolism, renal diseases, significant left or right ventricular functional restriction) which will change the lipid metabolism of the liver
Implants or other reasons which make magnetic resonance examinations impossible