Cystic Fibrosis Liver Disease
Conditions
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.
Interventions
DRUGCholine Chloride
Sponsors
University Hospital Tuebingen
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Intervention model description
Pilot study to evolve the effect ot the treatment by comparing data before and after the treatment within the same patients
Eligibility
Sex/Gender
MALE
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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
| Measure | Time frame | Description |
|---|---|---|
| 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 |
| 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 |
| FEV1 | 3 months | Comparison of FEV1 before to after the choline substitution |
| MMEF | 3 months | Comparison of MMEF before to after the choline substitution |
| FVC | 3 months | Comparison of FVC before to after the choline substitution |
Outcome results
None listed