cystic fibrosis/triacylglycerol

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Bioavailability and safety of a high dose of docosahexaenoic acid triacylglycerol of algal origin in cystic fibrosis patients: a randomized, controlled study.

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OBJECTIVE Several studies have reported omega-3 and omega-6 fatty acid imbalances in patients with cystic fibrosis (CF). Whether these imbalances contribute to or are manifestations of the pathophysiology of CF is unknown. The study objective was to determine bioavailability, tissue accretion, and

Liver disease does not affect lipolysis as measured with the 13C-mixed triacylglycerol breath test in children with cystic fibrosis.

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BACKGROUND Liver disease associated with cystic fibrosis may not only limit the solubilisation and absorption of the products of fat digestion, but also may depress the activity of pancreatic lipase. The purpose of this study was to measure the effect of liver disease on triacylglycerol lipolysis

Improving the specificity of the [13C]mixed triacylglycerol breath test by estimating carbon dioxide production from heart rate.

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BACKGROUND The [13C]mixed triacylglycerol (MTG) breath test is a non-invasive measure of fat digestion and can be used to assess the need for enzyme replacement therapy in children with cystic fibrosis (CF). However, it lacks specificity. Quantitation of cumulative percent dose recovered (cPDR)

Plasma lipid concentrations in children with cystic fibrosis: the value of a high-fat diet and pancreatic supplementation.

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Impaired digestion of dietary fat is an almost universal feature of cystic fibrosis (CF) which results in low concentrations of essential fatty acids in plasma lipids. We have evaluated the effect of a high-lipid diet and pancreatic enzyme supplementation, using enteric-coated microsphere

Acetate correction for postabsorption metabolism does not improve the [13C]mixed triacylglycerol breath test.

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OBJECTIVE The [C]mixed triacylglycerol (MTG) breath test is a noninvasive measure of fat digestion. After absorption and oxidation, C appears in breath CO2. Recovery is no more than 50% in healthy subjects because of sequestration of acetate in intermediary metabolism. The aims of this study were to

Effect of an organized lipid matrix on lipid absorption and clinical outcomes in patients with cystic fibrosis.

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OBJECTIVE To compare the absorption of a lysophosphatidylcholine, monoglyceride, and fatty acid matrix (organized lipid matrix, OLM) with that of a triacylglycerol (TG)-based fat meal in patients with cystic fibrosis (CF). METHODS Five adolescents with CF and 3 control patients were given fat meals

Bulk and compound specific analysis of stool lipid confirm that the "missing" 13C in the mixed triacylglycerol breath test is not in the stool.

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The 13C-mixed triacylglycerol (MTG) breath test is used to measure intraluminal fat digestion. In normal digestion 20% to 40% of the ingested 13C label is recovered in breath CO2. We aimed to identify the proportions of ingested label excreted in stool as well as breath following ingestion of

Plasma lipidomics reveals potential prognostic signatures within a cohort of cystic fibrosis patients.

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Cystic fibrosis (CF) is associated with abnormal lipid metabolism. We have recently shown variations in plasma levels of several phosphatidylcholine (PC) and lysophopshatidylcholine (LPC) species related to disease severity in CF patients. Here our goal was to search for blood plasma lipid

Abnormal lipid concentrations in cystic fibrosis.

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BACKGROUND Concentrations of cholesterol and triacylglycerol are commonly believed to be low in persons with cystic fibrosis and thus not of concern. OBJECTIVE The goal was to determine whether concentrations of cholesterol and triacylglycerol are related to glucose tolerance or nutritional status

Abnormal intracellular lipid processing contributes to fat malabsorption in cystic fibrosis patients.

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A common feature of cystic fibrosis (CF) is the functional derangement of the exocrine pancreas, which affects output of pancreatic lipase. This condition results in severe dietary malabsorption due to the poor hydrolysis of triacylglycerol (TG) in the lumen of the small intestine. Despite the

Fat malabsorption in cystic fibrosis patients receiving enzyme replacement therapy is due to impaired intestinal uptake of long-chain fatty acids.

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BACKGROUND Pancreatic enzyme replacement therapy frequently fails to correct intestinal fat malabsorption completely in cystic fibrosis (CF) patients. The reason for this failure is unknown. OBJECTIVE We investigated whether fat malabsorption in CF patients treated with pancreatic enzymes is caused

Choline-related supplements improve abnormal plasma methionine-homocysteine metabolites and glutathione status in children with cystic fibrosis.

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BACKGROUND Liver triacylglycerol accumulation and oxidative stress are common in cystic fibrosis (CF) and also occur in choline deficiency. Previously, we showed an association between elevated plasma homocysteine, reduced ratios of S-adenosylmethionine to S-adenosylhomocysteine (SAM:SAH) and of

The deltaF508 mutation in the cystic fibrosis transmembrane conductance regulator alters control of essential fatty acid utilization in epithelial cells.

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Essential fatty acid (EFA) incorporation into phospholipid is influenced by chloride channels, suggesting that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) may regulate aspects of EFA metabolism. The objective of this study was to determine whether the DeltaF508 mutation in

Monoacylglycerol Form of Omega-3s Improves Its Bioavailability in Humans Compared to Other Forms.

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Numerous benefits are attributed to omega-3 fatty acids (OM3) especially in cardiovascular health. However, bioavailability and clinical efficacy depend on numerous factors, including OM3 form, food matrix effects (especially the lipid content of the diet), and metabolic capacity. Here, we show in

CFTR knockdown stimulates lipid synthesis and transport in intestinal Caco-2/15 cells.

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Cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel highly expressed in epithelial cells of the gastrointestinal tract. Mutations in the CFTR gene cause cystic fibrosis (CF), a disease characterized by pancreatic insufficiency, fat malabsorption, and steatorrhea. Despite

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