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.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
10
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
Time frame: 3 months
Lipid storage of the liver
Comparison of steatosis of the liver (measured by magnetic resonance spectroscopy or sonography) before to after the choline substitution
Time frame: 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
Time frame: 3 months
FVC
Comparison of FVC before to after the choline substitution
Time frame: 3 months
FEV1
Comparison of FEV1 before to after the choline substitution
Time frame: 3 months
MEF25
Comparison of MEF25 before to after the choline substitution
Time frame: 3 months
MMEF
Comparison of MMEF before to after the choline substitution
Time frame: 3 months
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