Choline Supplementation Improves Lung Function, Liver Health in CF Patients, Study Shows

Choline supplementation improves lung function and liver health in people with cystic fibrosis (CF), and should be considered as a complementary treatment to current standard care therapies, a study suggests.

The findings of the study, “Choline Supplementation in Cystic Fibrosis — The Metabolic and Clinical Impact,” were published in the journal Nutrients.

CF is a genetic disorder caused by mutations in the CFTR gene, which provides instructions to make the cystic fibrosis transmembrane conductance regulator (CFTR) protein.

This protein works as a channel that transports in and out of cells electrically charged molecules, such as chloride, which will have a direct impact on the regulation of water transport and the production of mucus in lung airways and other organs. In patients with CF, ion transport is impaired, leading to a buildup of fluid in the lungs and other organs.

Previous studies have reported that people with CF lack an essential nutrient called choline, mainly due to an excessive expulsion of the nutrient in the stools triggered by pancreatic insufficiency.

This vitamin-like nutrient is required for the production of liver phosphatidylcholine (PC, a component of cell membranes) and other elements that are important for liver function. It is known that in CF patients, low levels of choline in the plasma are directly associated with lung dysfunction and liver disease.

Researchers from Germany’s Eberhard-Karls-University and their collaborators set out to evaluate the effects of oral choline supplements on lung function and liver health in a group of patients with CF (NCT03312140).

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Ten male CF patients (ages 18–45) with different CFTR mutations were recruited between November 2014 and January 2016 to participate in the study.  They all received 1g of oral choline chloride supplements three times a day for 84 days.

Patients’ clinical data, including plasma levels of choline and PC, lung function (measured by spirometry parameters), and liver health (assessed by the amount of liver fat detected by magnetic resonance spectroscopy) were assessed before and after treatment. Spirometry parameters included forced vital capacity (FVC), percent of predicted forced expiratory volume in one second relative to FVC (ppFEV1), and forced expiratory flow at 25–75% of the pulmonary volume (FEF 25–75).

Oral supplementation significantly increased the levels of choline in the plasma after 84 days of treatment compared to baseline levels — 10.5 vs 4.8 μmol/L. Conversely, PC plasma levels remained unchanged after treatment with oral supplements.

The experimental therapy also improved lung function, which was reflected by a significant increase in ppFEV1 from 70% to 78.3%, as well as 5.4% and 7.6% improvements in FVC and FEF 25–75, respectively. Liver health also improved, with a significant reduction in the amount of fat present in the liver before (1.58%) and after treatment (0.84%).

“Choline supplementation normalizes the decreased concentration of plasma choline, (…) improves lung function and [fatty liver] in these patients, complementary to standard care and, possibly, other therapeutic innovations,” researchers said. “The relevance of choline as an essential nutrient” for normal tissue function and growth, as well as normal body metabolism “suggests its supplementation in children,” they added.

Researchers also reported that the levels of choline in patients’ plasma decreased to baseline levels within 60 hours after the patients took their last dose of oral supplements, suggesting a rapid clearance of choline from the body.

“The rapid return of plasma choline to baseline concentration suggests further efforts to optimize the [therapeutic formulation] of choline and to assess the duration of clinical improvements in randomized trials,” they said.