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

2017-10-23 20:44:13 | BioPortfolio

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

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 Design

Conditions

Cystic Fibrosis Liver Disease

Intervention

Choline Chloride

Status

Completed

Source

University Hospital Tuebingen

Results (where available)

View Results

Links

Published on BioPortfolio: 2017-10-23T20:44:13-0400

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Medical and Biotech [MESH] Definitions

A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)

An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.

A condition produced by a deficiency of CHOLINE in animals. Choline is known as a lipotropic agent because it has been shown to promote the transport of excess fat from the liver under certain conditions in laboratory animals. Combined deficiency of choline (included in the B vitamin complex) and all other methyl group donors causes liver cirrhosis in some animals. Unlike compounds normally considered as vitamins, choline does not serve as a cofactor in enzymatic reactions. (From Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984)

A strain of mice widely studied as a model for cystic fibrosis. These mice are generated from embryonic stem cells in which the CFTR (cystic fibrosis transmembrane conductance regulator) gene is inactivated by gene targeting. As a result, all mice have one copy of this altered gene in all their tissues. Mice homozygous for the disrupted gene exhibit many features common to young cystic fibrosis patients, including failure to thrive, meconium ileus, and alteration of mucous and serous glands.

Donor of choline in biosynthesis of choline-containing phosphoglycerides.

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