Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function†

Sokol, Ronald J.; Heubi, James E.; McGraw, Catherine A.; Balistreri, William F.

doi:10.1002/hep.1840060607

Cited by 21 publications

(12 citation statements)

References 38 publications

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“…Recently. Sokol et al [27], who studied the variations in total plasma fatty acids in 5 cholestatic patients, reported only a mild decrease in C l8:2 n-6 for 2 of them.…”

Section: Discussionmentioning

confidence: 99%

“…After several months of intramuscular vi tamin E treatment, the fatty acid composi tion and the lipid peroxide levels were not generally improved. Similarly, Sokol et al [27] studied the effect of long-term vitamin E treatment in 5 cholestatic patients and showed no consistent changes in various liver functions. However, although in our treated patients plasma vitamin E returned to normal values, they might still have a latent vitamin E deficiency as suggested pre viously [32], Thus we observed a decrease in the vitamin E/total lipid ratio in several of our patients and we also noted a negative relationship between lipid peroxides and the vitamin E/total lipid ratio.…”

Section: Discussionmentioning

confidence: 99%

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Plasma Fatty Acid Composition and Lipid Peroxide Levels in Children with Paucity of Interlobular Bile Ducts

Babin

Lemonnier

et al. 1988

Ann Nutr Metab

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The total and free fatty acid composition of plasma and lipid peroxide concentrations was studied in 32 cholestatic children with syndromatic paucity of interlobular bile ducts (Alagille’s syndrome). The mean lipid peroxide value in these patients was 8.80 ± 3.70 nmol/ml, nearly 4 times higher than the mean control value. Compared to the control group, the patients exhibited significant variations in total fatty acids, and in particular a relative decrease in linoleic acid (from 29.5 ± 6.1 % in the controls to 19.1 ± 8.03% in the patients) compensated by an increase in saturated and monounsaturated fatty acids. The plasma lipid peroxide levels were inversely correlated with the unsaturated/saturated fatty acids ratio in total fatty acids, and with the vitamin E status (vitamin E/total lipids). Most of the total and free fatty acid variations observed were largest in patients with severe jaundice. Dietary fat malabsorption and the increase in lipid peroxidation partly explain these results. Furthermore, in free fatty acids, we observed a marked increase in arachidonic acid (from 1.43 ± 0.85% in the controls to 4.27 ± 2.24% in the patients), suggesting abnormal eicosanoid synthesis.

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“…Recently. Sokol et al [27], who studied the variations in total plasma fatty acids in 5 cholestatic patients, reported only a mild decrease in C l8:2 n-6 for 2 of them.…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Plasma Fatty Acid Composition and Lipid Peroxide Levels in Children with Paucity of Interlobular Bile Ducts

Babin

Lemonnier

et al. 1988

Ann Nutr Metab

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“…Recently, a protective effect of vitamin E on lipid peroxidation of hepatic mitochondria1 membranes in bile duct ligated rats was documented (67). Apart from these experimental studies, limited clinical data on patients with cholestatic liver disease suggest that efficient antioxidant supply improves parameters of hepatic function (68).…”

Section: Liver Diseasementioning

confidence: 99%

Oxygen free radicals and antioxidants in cystic fibrosis: the concept of an oxidant‐antioxidant imbalance

Winklhofer‐Roob

1994

Acta Paediatrica

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Patients with cystic fibrosis frequently exhibit increased oxygen free radical generation from activated neutrophils due to chronic lung inflammation on the one hand and antioxidant deficiencies due to exocrine pancreatic insufficiency on the other, resulting in an oxidant–antioxidant imbalance in favor of the former. As a consequence, free radical attack on unsaturated fatty acids of lipid structures leading to lipid peroxidation and damaging effects on proteins may occur. In the lung, antiproteases are thought to be inactivated by oxygen free radicals released from inflammatory cells. In the cholestatic liver, bile acids may propagate lipid peroxidation. An efficient antioxidant supply is suggested to control tissue injury by restoring the oxidant‐antioxidant balance. Mechanisms involved in the generation of oxygen free radicals are described and data on the antioxidant defense system in cystic fibrosis patients are presented, together with evidence of increased lipid peroxidation. Possible implications for disease processes are discussed as well as therapeutic concepts to reconstitute the oxidant‐antioxidant balance.

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“…These patients generally present with very severe vitamin E deficiency and early vitamin E treatment is essential to prevent or improve the neurological disorders which are secondary to prolonged cholestasis (Alvarez et at., 1985;Sokol et al, 1985). However, the usual vitamin E therapy does not appear to have any effects on the biochemical abnormalities observed in these patients (Sokol et al, 1986;Lemonnier et al, 1987). Consequently, various tissues might be subject to attack by the free radicals modifying membrane integrity.…”

Section: Introductionmentioning

confidence: 97%

2‐Deoxy‐d‐glucose uptake and fatty acid content in fibroblast cultures from children with syndromic paucity of interlobular bile ducts (alagille syndrome)

Couturier

Lemonnier

1990

J of Inher Metab Disea

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2-Deoxy-D-glucose (2-DOG) uptake was studied in skin fibroblast cultures from control children and children with Alagille syndrome or syndromic paucity of interlobular bile ducts (PILBD). No significant differences in uptake were observed between patients and controls. However, as the scatter of the results was larger in the fibroblasts from patients, we attempted to establish for these patients a relationship between 2-DOG uptake and some biochemical parameters. We observed an inverse relationship between this uptake and the levels of plasma cholesterol and phospholipids (r = -0.85). Compared to controls, 2-DOG uptake was significantly lower in cultures from patients who had very high levels of cholesterol (P2 group), but not in cultures from patients with moderately increased levels of cholesterol (P1 group). The level of total cellular cholesterol in cultured cells from the P1 and P2 groups was not significantly different from the control level, but we found marked differences between the concentrations of fatty acids. In the cultures from patients (especially the P2 group), we observed a significant increase in total fatty acids; among the saturated fatty acids, this increase chiefly concerned the 18:0 (14%) and among the polyunsaturated the n - 3 fatty acids (55%). The high concentrations of 20:5, 22:5 and 22:6, which enhance membrane fluidity, might explain the decrease in 2-DOG uptake found in the cultures from patients (P2 group) with PILBD. The nature of these abnormalities might be connected with the genetic origin of Alagille syndrome.

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Correction of vitamin E deficiency in children with chronic cholestasis. II. Effect on gastrointestinal and hepatic function†

Cited by 21 publications

References 38 publications

Plasma Fatty Acid Composition and Lipid Peroxide Levels in Children with Paucity of Interlobular Bile Ducts

Plasma Fatty Acid Composition and Lipid Peroxide Levels in Children with Paucity of Interlobular Bile Ducts

Oxygen free radicals and antioxidants in cystic fibrosis: the concept of an oxidant‐antioxidant imbalance

2‐Deoxy‐d‐glucose uptake and fatty acid content in fibroblast cultures from children with syndromic paucity of interlobular bile ducts (alagille syndrome)

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