Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation

Lloyd-Still, John D.; Johnson, Susan B.; Holman, R T

doi:10.1093/ajcn/34.1.1

Cited by 105 publications

(74 citation statements)

References 22 publications

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“…Abnormalities in cord blood EFA in infants with CF suggest that a fundamental defect of fat metabolism may be in part responsible for the clinical manifestations of this disease (22). Several investigators have reported abnormalities of prostaglandin metabolism in CF patients (2,4).…”

Section: Discussionmentioning

confidence: 99%

Adipose Tissue Abnormalities in Cystic Fibrosis: Noninvasive Determination of Mono- and Polyunsaturated Fatty Acids by Carbon-13 Topical Magnetic Resonance Spectroscopy

Rj¹,

et al. 1988

Pediatr Res

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Section: Discussionmentioning

confidence: 99%

Adipose Tissue Abnormalities in Cystic Fibrosis: Noninvasive Determination of Mono- and Polyunsaturated Fatty Acids by Carbon-13 Topical Magnetic Resonance Spectroscopy

Rj¹,

et al. 1988

Pediatr Res

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“…Lloyd-Still et al (7) fed oral safflower oil (1 g/ kg/day) to 11 CF patients for 1 yr, but was unable to correct 18:2 deficiency and or to produce a significant change in sweat chloride concentration or sweat rate; there was also no clinical improvement when compared to a control group. Chase et al (34), in a double-blind study of twice monthly infusions of Intralipid, found clinical improvement of the supplemented children when compared to controls; however, 2 wk after each infusion, plasma and RBC linoleic acid levels were not increased.…”

Section: Discussionmentioning

confidence: 99%

Correction of Linoleic Acid Deficiency in Cystic Fibrosis

et al. 1986

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ABSTRACT. To identify evidence of essential fatty acid deficiency, we screened 64 patients with cystic fibrosis by analyzing total lipid extracts from plasma. Forty-three had an abnormal linoleate (18:2) level (less than 26%). Thirteen deficient patients (aged 10-24 yr) ingested for 1 yr 7% of their total calories as linoleate derived from a daily supplement of Microlipid. Five deficient patients (aged 10-37 yr) served as controls. Plasma and erythrocyte fatty acid composition were monitored by gas chromatography of total lipid extracts seven times during the twelve month period. Prostaglandins E2 and FZa and their 15 keto 13, 14 dihydrometabolite, 6-keto F,,, and thromboxane B2 were measured by radioimmunoassay. Sweat tests, oxygen saturation, growth indices, clinical severity scores, compliance, and possible side effects from taking Microlipid were followed. Results showed that oral supplementation with Microlipid can significantly increase plasma and erythrocytes %18:2. One compliant patient died during the study and had normal tissue 18:2 levels. Nine of 13 patients gained more weight while taking Microlipid than in the previous year. No significant changes in sweat electrolytes, clinical scores, or oxygen saturation were found during the study year. Prostaglandin metabolites prostaglandin E2 showed an upward trend in supplemented patients, compared to controls. Prostaglandin FZa remained unchanged over 1 yr but showed a trend significantly downward over the final 6 months in supplemented patients. We conclude that linoleate deficiency can be corrected with daily Microlipid supplements and that correction may alter prostaglandin metabolism (Pediatr Res 20: 36-41, 1986) Abbreviations CF, cystic fibrosis 18:2, linoleate PGM, 13,14-dihydro-15-PGFza PG, prostaglandin EFA, essential fatty acid RBC, red blood cells Several workers (1 -3) have demonstrated markedly decreased concentration of essential fatty acid (EFA) in plasma from CF patients. The major changes include a decreased level of the predominant EFA, linoleate (1 8:2), and increased palmitoleate

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“…Altered plasma and tissue concentrations of fatty acids in cystic fibrosis (CF) 4 patients have been well described (1)(2)(3)(4)(5)(6)(7). Changes in concentrations of selected essential fatty acids have been found in CF patients, notably decreases in the plasma and tissue concentrations of linoleic acid (18:2 n-6) and docosahexaenoic acid (DHA; 22:6 n-3).…”

confidence: 99%

“…Changes in concentrations of selected essential fatty acids have been found in CF patients, notably decreases in the plasma and tissue concentrations of linoleic acid (18:2 n-6) and docosahexaenoic acid (DHA; 22:6 n-3). Increases in the concentration of eicosatrienoic acid (Mead acid; 20:3 n-9) have been identified in several studies (5)(6)(7). These alterations in fatty acid concentrations are significantly magnified in patients with severe variations in the CF transmembrane conductance regulator (CFTR) 5 gene, suggesting an association between the basic defect and abnormal essential fatty acid metabolism in CF patients (3 ).…”

confidence: 99%

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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Essential fatty acid status in cystic fibrosis and the effects of safflower oil supplementation

Cited by 105 publications

References 22 publications

Adipose Tissue Abnormalities in Cystic Fibrosis: Noninvasive Determination of Mono- and Polyunsaturated Fatty Acids by Carbon-13 Topical Magnetic Resonance Spectroscopy

Adipose Tissue Abnormalities in Cystic Fibrosis: Noninvasive Determination of Mono- and Polyunsaturated Fatty Acids by Carbon-13 Topical Magnetic Resonance Spectroscopy

Correction of Linoleic Acid Deficiency in Cystic Fibrosis

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

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