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

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

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

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.

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