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

Not Applicable

Completed

• Conditions: Cystic Fibrosis Liver Disease
• Interventions: Drug: Choline Chloride

• Registration Number: NCT03312140
• Lead Sponsor: University Hospital Tuebingen

Brief Summary

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

Detailed 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.

Study Timeline

• Study Start: 2014-11-06
• Primary Completion: 2016-04-24
• Study Completion: 2017-02-08
• Status Verified: 2017-10
• Study First Submitted: 2017-10-04
• Study First Submitted QC: 2017-10-16
• Study First Posted: 2017-10-17
• Last Update Submitted: 2017-11-29
• Last Update Posted: 2017-11-30

Recruitment & Eligibility

• Status: COMPLETED
• Sex: Male
• Target Recruitment: 10

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: SINGLE_GROUP

Arm && Interventions

Group	Intervention	Description
Choline Chloride	Choline Chloride	Patients receive choline chloride (1g Choline) three times a day for 12.6 weeks as a food supply

Primary Outcome Measures

Name	Time	Method
Measurement of D9-Methyl-Choline Kinetic	3 months	The substitution of choline as a food supplement will augment the phosphatidylcholine and lipoprotein (VLDL) secretion of the liver into the blood and will therefore result in a reduction of the triglyceride retention in the liver

Secondary Outcome Measures

Name	Time	Method
Lipid storage of the liver	3 months	Comparison of steatosis of the liver (measured by magnetic resonance spectroscopy or sonography) before to after the choline substitution
MMEF	3 months	Comparison of MMEF before to after the choline substitution
Augmentation of creatinine concentration within the calf muscle	3 months	Augmentation of creatine concentration within the calf muscle due to the choline substitution measured by magnetic resonance spectroscopy
MEF25	3 months	Comparison of MEF25 before to after the choline substitution
FVC	3 months	Comparison of FVC before to after the choline substitution
FEV1	3 months	Comparison of FEV1 before to after the choline substitution