Biochemical assessment of the nutritional status of cystic fibrosis patients treated with pancreatic enzyme extracts

Benabdeslam, Hassiba; Garcia, Isabelle; Bellon, G.; Gilly, R; Revol, A

doi:10.1093/ajcn/67.5.912

Cited by 56 publications

(31 citation statements)

References 51 publications

(67 reference statements)

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“…The discrepancies in AA and DHA plasma concentrations, from the previous studies, can be explained in part by the low number of CF patients enrolled (8 ). Because of the size of our cohort of CF patients, we were able to find significant decreases in AA and DHA concentrations in plasma as often observed when these PUFAs were analyzed from plasma phospholipids instead of total plasma lipids (8,26 ). However, the low DHA concentrations seem not to depend on pancreatic status.…”

Section: Discussionmentioning

confidence: 65%

Influence of Pancreatic Status and Sex on Polyunsaturated Fatty Acid Profiles in Cystic Fibrosis

Coste¹,

Deumer²,

Reychler

et al. 2008

Clinical Chemistry

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Background: Some but not all studies have reported abnormal polyunsaturated fatty acid composition in cystic fibrosis (CF) patients. We investigated the influence of pancreatic status and sex on the fatty acid profile in plasma and erythrocyte membranes in patients with CF. Methods: After a 1-step transesterification with acetyl chloride on plasma and washed erythrocyte membranes, we quantified fatty acid methyl esters by use of GC-MS in 124 CF patients and 80 age-matched healthy controls. In the CF group, mean (SD) age was 17.5 (11.3) years, and 51.6% were male. Pancreatic insufficiency was diagnosed in 78% of the CF population. Results: A decrease in docosahexaenoic acid concentrations was observed in CF patients independently of pancreatic status. Pancreatic insufficient CF patients displayed lower concentrations of linoleic acid and arachidonic acid and higher concentrations of dihomo-γ-linolenic acid and eicosatrienoic acid (mead acid) in plasma and erythrocyte membranes compared with healthy controls and pancreatic sufficient CF patients. Male CF patients had significantly lower docosahexaenoic acid and higher eicosatrienoic acid in plasma and erythrocyte membranes compared with female CF patients. Conclusions: These results support the concept that multiple abnormalities of polyunsaturated fatty acid composition participate in the CF disease phenotype and that pancreatic status plays a major role in such abnormalities. Moreover, patient sex influences the polyunsaturated fatty acid spectrum in CF, with more marked abnormalities in males.

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Section: Discussionmentioning

confidence: 65%

Influence of Pancreatic Status and Sex on Polyunsaturated Fatty Acid Profiles in Cystic Fibrosis

Coste¹,

Deumer²,

Reychler

et al. 2008

Clinical Chemistry

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“…The data in Fig. 2 show 3 paired comparisons between a CF group and a control group, including comparison of data from total plasma fatty acid analysis in CF patients and controls using our data and data from the study by Benabdeslam et al (22 ). The (18:2 n-6) ϫ (22:6 n-3) value separates CF patients from non-CF controls in all 3 paired comparisons.…”

Section: Discussionmentioning

confidence: 74%

Potential Utility of Plasma Fatty Acid Analysis in the Diagnosis of Cystic Fibrosis

et al. 2007

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Background: An altered distribution of fatty acids in cells and tissues is found in patients with cystic fibrosis (CF). In this study, we assessed the potential role of plasma fatty acid analysis in the diagnosis of CF. Methods: In this 2-part study, we first used gas chromatography–mass spectrometry to analyze fatty acids in plasma from 13 CF patients and 11 controls without CF. We then used the fatty acid distribution data to identify the fatty acids or multiple fatty acid calculations most effective in identifying CF patients. Part 2 of the study was a blinded analysis of 10 CF patients and 9 controls to directly test the effectiveness of the diagnostic parameters for CF identified from the plasma fatty acid analysis. Results: In the nonblinded trial, the multiplication product of (18:2 n-6) × (22:6 n-3) (each as percentage of total plasma fatty acid) was the most effective indicator for distinguishing patients with CF from controls (P = 0.0003). In part 2 (the blinded trial), this multiplication product was also the most effective indicator for distinguishing CF patients from controls (P = 0.0008). Conclusions: The product of (18:2 n-6) × (22:6 n-3) is effective for distinguishing CF patients from persons without CF. This diagnostic marker may have value as an alternative to the sweat chloride test in selected patients being evaluated for CF.

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“…Current high-fat, hypercaloric nutritional strategies and improved pancreas enzyme replacement therapies can usually maintain patients in optimal nutritional status, thus normalizing EFA status in many CF patients (5). Nonetheless, several reports still indicate the occurrence of EFA deficiency in CF (6)(7)(8). Although some authors have suggested that residual fat malabsorption and increased EFA turnover in CF may compromise EFA status (9,10), the exact pathophysiology of EFA deficiency in CF patients has not been elucidated.…”

mentioning

confidence: 99%

No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis

Werner¹,

Bongers²,

Bijvelds

et al. 2004

Journal of Lipid Research

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A deficiency of essential fatty acids (EFA) is frequently described in cystic fibrosis (CF), but whether this is a primary consequence of altered EFA metabolism or a secondary phenomenon is unclear. It was suggested that defective long-chain polyunsaturated fatty acid (LCPUFA) synthesis contributes to the CF phenotype. To establish whether cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction affects LCPUFA synthesis, we quantified EFA metabolism in cftr ؊ / ؊ CAM and cftr ؉ / ؉ CAM mice. Effects of intestinal phenotype, diet, age, and genetic background on EFA status were evaluated in cftr ؊ / ؊ CAM mice, ⌬ F508/ ⌬ F508 mice, and littermate controls. EFA metabolism was measured by 13 C stable isotope methodology in vivo. EFA status was determined by gas chromatography in tissues of cftr ؊ / ؊ CAM mice, ⌬ F508/ ⌬ F508 mice, littermate controls, and C57Bl/6 wild types fed chow or liquid diet. After enteral administration of [ 13 C]EFA, arachidonic acid (AA) and docosahexaenoic acid (DHA) were equally 13 C-enriched in cftr ؊ / ؊ CAM and cftr ؉ / ؉ CAM mice, indicating similar EFA elongation/desaturation rates. LA, ALA, AA, and DHA concentrations were equal in pancreas, lung, and jejunum of chow-fed cftr ؊ / ؊ CAM and ⌬ F508/ ⌬ F508 mice and controls. LCPUFA levels were also equal in liquid diet-weaned cftr ؊ / ؊ CAM mice and littermate controls, but consistently higher than in age-and diet-matched C57Bl/6 wild types. We conclude that cftr ؊ / ؊ CAM mice adequately absorb and metabolize EFA, indicating that CFTR dysfunction does not impair LCPUFA synthesis. A membrane EFA imbalance is not inextricably linked to the CF genotype. EFA status in murine CF models is strongly determined by genetic background. -Werner, A., M. E. J. Bongers, M. J. Bijvelds, H. R. de Jonge, and H. J. Verkade. No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis.

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Biochemical assessment of the nutritional status of cystic fibrosis patients treated with pancreatic enzyme extracts

Cited by 56 publications

References 51 publications

Influence of Pancreatic Status and Sex on Polyunsaturated Fatty Acid Profiles in Cystic Fibrosis

Influence of Pancreatic Status and Sex on Polyunsaturated Fatty Acid Profiles in Cystic Fibrosis

Potential Utility of Plasma Fatty Acid Analysis in the Diagnosis of Cystic Fibrosis

No indications for altered essential fatty acid metabolism in two murine models for cystic fibrosis

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