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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

For the purpose of clarifying the cause of white muscle disease (WMD) in calves, tocopherol and selenium levels and blood glutathione peroxidase (GSH-Px) activity were measured on 10 calves with WMD and nine of their dams. The main clinical symptoms of the 10 calves with WMD were motor disturbances including recumbency and stiffness. Serum enzyme activities (GOT, GPT, CPK, LDH) in calves with WMD increased markedly, and this increase was also observed in some of their dams. Serum tocopherol levels of calves with WMD were low, 70% of which showing deficient levels of less than 70 micrograms/100 ml. Serum selenium levels of all the calves were lower than 35 ppb, indicating a deficiency, and were accompanied by low blood GSH-Px activity. alpha-Tocopherol and selenium concentrations in organs were very low. Dams of calves with WMD showed low serum tocopherol levels, 22% of which indicating deficient levels below 150 micrograms/100 ml. Serum selenium levels in dams showed a marked decrease to under 20 ppb, and also low blood GSH-Px activity. Feedstuffs supplied in the farms to affected calves indicated very low alpha-tocopherol contents (below 3 mg/100g DM) and low selenium concentrations below 50 ppb in DM. It was concluded that WMD in calves was attributable to nutritional muscular dystrophy caused by deficiencies in tocopherol and selenium in feedstuffs supplied to their dams.
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PMID:Studies on serum tocopherol, selenium levels and blood glutathione peroxidase activities in calves with white muscle disease. 258 29

Studies of the basic biochemical mechanisms underlying muscle damage aimed at finding agents which might reduce the amount of damage occurring in muscular dystrophy and other severe myopathies have been performed. These have suggested three types of agent which might be useful for this purpose, namely calcium antagonists, phospholipase inhibitors, and antioxidants or scavengers of reactive-free radicals. Vitamin E falls into the latter of these three categories and has been shown to reduce the amount of damage which occurs in isolated skeletal muscles following a given stress. It is suggested that, in the absence of calcium antagonists having relatively specific and effective actions on skeletal muscle or suitable inhibitors of muscle phospholipases in man, therapy with vitamin E or other antioxidants may reduce the amount of muscle damage occurring in patients with severe myopathies.
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PMID:Vitamin E and muscle diseases. 393 Aug 47

Vitamin E-deficient rabbits with signs of muscular dystrophy showed accumulation of cholesterol in muscle as well as elevation of plasma cholesterol. The increase in plasma cholesterol was detected in low density lipoprotein (LDL) and very low density lipoprotein (VLDL) but not in high density lipoprotein (HDL) fractions of plasma lipoproteins. In liver, the activity of cholesterol 7 alpha-hydroxylase, the key enzyme involved in degradation of cholesterol, was approximately one-fifth that of control rabbits. Cytochrome P-450 level of liver microsomes was also reduced significantly. These results suggested that accumulation of cholesterol observed in dystrophic muscle of vitamin E-deficient rabbits may be due to an increase in LDL and VLDL cholesterol, the plasma lipoproteins carrying cholesterol to peripheral tissue, and to a decrease in cholesterol 7 alpha-hydroxylase activity, whose activity may have been affected by the reduced level of cytochrome P-450.
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PMID:Effects of vitamin E deficiency on the distribution of cholesterol in plasma lipoproteins and the activity of cholesterol 7 alpha-hydroxylase in rabbit liver. 398 Dec 65

Forty-eight weanling S.P.F. Yorshire pigs were used to study the influence of supplemental vitamin E (25 IU per kg of diet) selenium (0.5 ppm in diet) and methionine (0.1% in diet) on the incidence of hepatosis dietetica and mulberry heart disease when fed a torula yeast-corn diet. Vitamin E and/or selenium increased pig survival. Supplemental selenium resulted in increased liver selenium concentrations. No hepatosis dietetica was observed in any of the pigs. The addition of vitamin E and/or selenium at the levels used did not reduce the frequency of myocardial lesions; however, they prevented skeletal muscular dystrophy and exudative diathesis. The myocardial lesions were less severe in supplemented pigs compared with unsupplemented controls.
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PMID:Vitamin E, selenium and methionine supplementation of dystrophogenic diets for pigs. 426 22

Vitamin E refers to a family of fat-soluble phenolic compounds called tocopherols, which have been established as essential nutrients in vertebrates. In animals the deficiency state has resulted in diminished reproductive capabilities, muscular dystrophy, exudative diathesis, megaloblastosis, gastrointestinal and pulmonary degeneration, and nephrosis. In humans with low vitamin E levels a subclinical diminished erythrocyte life-span has been demonstrated by hydrogen peroxide hemolysis test. This effect may have clinical significance among premature infants. The metabolic function of vitamin E appears to be as a scavenger of lipid peroxides and free oxygen radicals which enter into chain reactions to cause breakdown of lipids. Normal levels of the vitamin serve to prevent this cellular oxidative breakdown. Laboratory measurement of vitamin E is chromatographic, with HPLC presently used in both research and clinical applications. The association between vitamin E levels and hemolytic anemia in humans is currently under investigation.
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PMID:Vitamin E and autoxidation. 634 59

Vitamin E levels were measured in the plasma of infants and children with various neuromuscular disorders. Seven of 8 infants with Werdnig-Hoffmann disease (WHD) had a significantly lower plasma vitamin E level (p less than 0.01) than age-matched normal controls, children with congenital myopathies, or children with muscular dystrophy. Vitamin E deficiency in WHD is not caused by malabsorption. A therapeutic trial of vitamin E in 3 patients with WHD did not change the natural course of the disease. Vitamin E deficiency may play a role in the pathogenesis of WHD.
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PMID:Vitamin E deficiency in Werdnig-Hoffmann disease. 729 34

Three experiments were carried out with male broiler chickens reared from day- old to 6 weeks of age on semi-purified diets containing 10% fresh (Expt. 1 and 3) or oxidized (Expt. 2) re-esterified triglycerides with a fatty acid composition similar to that of soya bean oil containing increasing concentrations of either a mixture of d-alpha-, gamma-, delta-tocopherylacetate (d-tocopherols) of natural source or dl-alpha- tocopheryl acetate (dl-tocopherol). In Expt. 1 and 2 the mixture of d-tocopherols consisted of 35.7% d-alpha-, 45.3% d-gamma- and 19.0% d-delta-, while in Expt. 3 the distribution was 25.3% d-alpha-, 28.1% d-gamma- and 10.8% d-gamma- in 35.8% re-esterified triglycerides. The relative biopotency of d-alpha-: gamma-: delta-tocopherol was anticipated to be 100:25:1, whereas that of dl-alpha-tocopherol was 74% relative to d-alpha-tocopherol. The experiments demonstrate that the results obtained for the biological activity depend on the response parameters chosen. With respect to gain in weight, feed conversion, relative organ weight, packed cell volume (PCV), ELP (erythrocyte lipid peroxidation), plasma activities of glutamate-oxaloacetate-transaminase (GOT), creatine kinase (CK) and glutathione peroxidase (GSH-Px) and plasma Na+ concentration, the mixture of natural source tocopherols was identical to that of dl-alpha-tocopheryl acetate, although the concentration of alpha-tocopherol was only about one third of that of dl-alpha-tocopherol. Differences between natural source and synthetic tocopherols were expectedly observed with respect to plasma concentrations of alpha-, gamma-, delta-tocopherol. Differences between the two forms as to muscular dystrophy, in vitro haemolysis and potassium concentration in plasma were ambiguous. It is suggested that the function of d-alpha-, gamma-, delta-tocopherol in erythrocyte fragility and skeletal muscle structure should be compared to that of dl-alpha-tocopherol in future investigations.
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PMID:The biological activity of natural source tocopherols in chickens fed fresh or oxidized fat rich in linoleic acid. 821 3

Many of the nervous and muscular locomotor disorders that affect sheep throughout Australia are commonly referred to as "staggers" syndromes. The range of clinical signs displayed by sheep suffering these disorders is sufficiently diverse to enable each syndrome to be graded into one of 5 progressive clinical groups. The first group, the limb paresis syndromes, includes the primary myopathies associated with the ingestion of Ixiolaena brevicompta, Malva parviflora, and Trachymene ochracea, as well as selenium and Vitamin E disorders, Paroo virus staggers, congenital progressive muscular dystrophy, humpy back, hypocalcaemic muscle weakness, Tribulus terrestris staggers and tetanus. The second group is characterised by limb paresis with knuckling of the fetlocks, and includes the plant-associated toxicities of Romulea rosea, Stachys arvensis, Trachyandra divaricata, and Tribulus micrococcus, together with haloxon toxicity, enzootic ataxia (copper deficiency), and the probably genetic disorders of segmental axonopathy, neuroaxonal dystrophy, and degenerative thoracic myelopathy. Other locomotor disorders that fit more loosely into this group are listerial myelitis (post-dipping staggers), vitamin A deficiency, cervico-thoracic vertebral subluxation Stypandra glauca toxicity, Ipomoea spp toxicity, ivermectin toxicity, and botulism. The third group, the falling syndromes, includes the probably genetic disorders of thalamic cerebellar neuropathy, cerebellar abiotrophy, and globoid cell leucodystrophy, together with Swainsona spp toxicity. The fourth group, the falling syndromes, includes the plant associated toxicities of phalaris staggers, perennial rye grass staggers and nervous ergotism (Claviceps paspali).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The clinical differentiation of nervous and muscular locomotor disorders of sheep in Australia. 852 19

The Food and Nutrition Board of the Institute of Medicine recently published a comprehensive evaluation of antioxidants in human diets that includes dietary reference intakes for vitamin E. The new dietary reference intake is 15 mg (35 mol)/d for adults, which is 50% greater than the generous allowance in the 10th edition of Recommended Dietary Allowances published in 1989. Much of the data interpreted in these publications came from studies sponsored by the Committee of Nutritional Studies at Elgin State Hospital (Elgin, IL) of an earlier Food and Nutrition BOARD: The 50% increase in the recommended dietary allowances for vitamin E is not supported by any new data. It is possible that the publication of the Institute of Medicine did not take into consideration the effects of the oxidized lipids in the diets used to promote the development of vitamin E deficiency. If lipids, oxidized to remove tocopherols, had not been a part of the experimental diets, the minimum requirement for vitamin E would have been too small for possible evaluation. Studies on the different effects of saturated and oxidized lipids in the production of encephalomalacia in chicks and muscular dystrophy in rats are reviewed. The tolerable upper intake level of vitamin E supplementation is reported to be 1000 mg/d. It is possible that the universal consumption of aspirin may not have been taken into consideration when this level was determined. Vitamin E plus aspirin may increase the tendency to hemorrhage, which makes a lower upper intake level worth consideration.
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PMID:Critique of the requirement for vitamin E. 1191 70

The prophylactic effect of parentral administration of a single dose of Vitamin E-selenium preparation given to pregnant ewes during the last third of pregnancy for protection against nutritional muscular dystrophy (NMD) in their lambs was studied. Based on determination of serum muscle-specific enzymes in the lambs, it was found that administration of Vitamin E-selenium preparation to pregnant ewes from a flock with a known history of NMD was associated with a significant decrease in serum muscle-specific enzymes levels in their lambs when compared with those from non-treated dams in the same flock. It was also observed that serum muscle-specific enzyme levels in lambs from non-treated dams in the flock with a known history of NMD were significantly higher than those in lambs from non-treated dams in a flock with no history of NMD (control group). The serum creatine phosphokinase (CPK) level was significantly higher in lambs of treated dams as compared to lambs from the control group, but no significant differences were seen between the two groups of lambs for the other serum muscle-specific enzymes measured. No lambs from the treated group showed clinical signs of NMD up through the time of weaning. It is concluded that administration of Vitamin E-selenium preparation to pregnant ewes in a flock with a known history of NMD was effective in preventing NMD in their lambs.
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PMID:Some studies on nutritional muscular dystrophy in Qassim region in Saudi Arabia. Effect of administration of Vitamin E-selenium preparation to pregnant ewes on serum muscle-specific enzymes in their lambs. 1142 39


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