Choline Supplementation in Cystic Fibrosis-The Metabolic and Clinical Impact.
Adolescent
Adult
Choline
/ blood
Choline Deficiency
/ blood
Cystic Fibrosis
/ blood
Dietary Supplements
Exocrine Pancreatic Insufficiency
/ blood
Fatty Liver
/ blood
Forced Expiratory Volume
/ drug effects
Humans
Lipid Metabolism
/ drug effects
Liver
/ drug effects
Lung
/ drug effects
Male
Middle Aged
Phosphatidylcholines
/ blood
Triglycerides
/ blood
Young Adult
choline deficiency
choline supplementation
cystic fibrosis
liver
lung function
magnetic resonance spectroscopy
stable isotope labeling
steatosis
Journal
Nutrients
ISSN: 2072-6643
Titre abrégé: Nutrients
Pays: Switzerland
ID NLM: 101521595
Informations de publication
Date de publication:
18 Mar 2019
18 Mar 2019
Historique:
received:
06
02
2019
revised:
08
03
2019
accepted:
12
03
2019
entrez:
21
3
2019
pubmed:
21
3
2019
medline:
18
7
2019
Statut:
epublish
Résumé
Choline is essential for the synthesis of liver phosphatidylcholine (PC), parenchymal maintenance, bile formation, and lipoprotein assembly to secrete triglycerides. In choline deficiency, the liver accretes choline/PC at the expense of lung tissue, thereby impairing pulmonary PC homoeostasis. In cystic fibrosis (CF), exocrine pancreas insufficiency results in impaired cleavage of bile PC and subsequent fecal choline loss. In these patients, the plasma choline concentration is low and correlates with lung function. We therefore investigated the effect of choline supplementation on plasma choline/PC concentration and metabolism, lung function, and liver fat. 10 adult male CF patients were recruited (11/2014⁻1/2016), and orally supplemented with 3 × 1 g choline chloride for 84 (84⁻91) days. Pre-/post-supplementation, patients were spiked with 3.6 mg/kg [methyl-D₉]choline chloride to assess choline/PC metabolism. Mass spectrometry, spirometry, and hepatic nuclear resonance spectrometry served for analysis. Supplementation increased plasma choline from 4.8 (4.1⁻6.2) µmol/L to 10.5 (8.5⁻15.5) µmol/L at d84 ( Choline supplementation normalized plasma choline concentration and increased choline-containing PC precursor pools in adult CF patients. Improved lung function and decreased liver fat suggest that in CF correcting choline deficiency is clinically important. Choline supplementation of CF patients should be further investigated in randomized, placebo-controlled trials.
Sections du résumé
BACKGROUND
BACKGROUND
Choline is essential for the synthesis of liver phosphatidylcholine (PC), parenchymal maintenance, bile formation, and lipoprotein assembly to secrete triglycerides. In choline deficiency, the liver accretes choline/PC at the expense of lung tissue, thereby impairing pulmonary PC homoeostasis. In cystic fibrosis (CF), exocrine pancreas insufficiency results in impaired cleavage of bile PC and subsequent fecal choline loss. In these patients, the plasma choline concentration is low and correlates with lung function. We therefore investigated the effect of choline supplementation on plasma choline/PC concentration and metabolism, lung function, and liver fat.
METHODS
METHODS
10 adult male CF patients were recruited (11/2014⁻1/2016), and orally supplemented with 3 × 1 g choline chloride for 84 (84⁻91) days. Pre-/post-supplementation, patients were spiked with 3.6 mg/kg [methyl-D₉]choline chloride to assess choline/PC metabolism. Mass spectrometry, spirometry, and hepatic nuclear resonance spectrometry served for analysis.
RESULTS
RESULTS
Supplementation increased plasma choline from 4.8 (4.1⁻6.2) µmol/L to 10.5 (8.5⁻15.5) µmol/L at d84 (
CONCLUSIONS
CONCLUSIONS
Choline supplementation normalized plasma choline concentration and increased choline-containing PC precursor pools in adult CF patients. Improved lung function and decreased liver fat suggest that in CF correcting choline deficiency is clinically important. Choline supplementation of CF patients should be further investigated in randomized, placebo-controlled trials.
Identifiants
pubmed: 30889905
pii: nu11030656
doi: 10.3390/nu11030656
pmc: PMC6471815
pii:
doi:
Substances chimiques
Phosphatidylcholines
0
Triglycerides
0
Choline
N91BDP6H0X
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Margarete Müller Bull Stiftung, Stuttgart, Gertmany
ID : Phos-CF II
Déclaration de conflit d'intérêts
The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, or in the decision to publish the results.
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