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

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Cystic Fibrosis Liver Disease

Intervention / Treatment
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* Drug: Choline Chloride


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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Male

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Interventional Model Description: Pilot study to evolve the effect ot the treatment by comparing data before and after the treatment within the same patients
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Other: Choline Chloride<br>Patients receive choline chloride (1g Choline) three times a day for 12.6 weeks as a food supply | Drug: Choline Chloride<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Measurement of D9-Methyl-Choline Kinetic | 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 | 3 months | 

Secondary Outcome Measures

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