Examination of the Lipid Metabolism of the Liver After Choline Substitution in Cystic Fibrosis
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 10
- 主要终点
- Measurement of D9-Methyl-Choline Kinetic
研究概览
简要总结
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
详细描述
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Treatment
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- Male
- 接受健康志愿者
- 否
入选标准
- •Age > 18 years
- •Cystic fibrosis is verified
- •pancreatic insufficiency
- •Decline of plasma phosphatidylcholine and steatosis of the liver
- •informed consent is given
排除标准
- •cirrhosis of the liver
- •Hyperreactivity to choline containing food
- •allergic bronchopulmonary aspergillosis or other acute exacerbations of pulmonary infections
- •FEV1 < 40%
- •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
研究组 & 干预措施
Choline Chloride
Patients receive choline chloride (1g Choline) three times a day for 12.6 weeks as a food supply
干预措施: Choline Chloride (Drug)
结局指标
主要结局
Measurement of D9-Methyl-Choline Kinetic
时间窗: 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
次要结局
- Lipid storage of the liver(3 months)
- MMEF(3 months)
- Augmentation of creatinine concentration within the calf muscle(3 months)
- MEF25(3 months)
- FVC(3 months)
- FEV1(3 months)