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

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

Nine children with Duchenne muscular dystrophy were given Sanorex (mazindol), a growth hormone inhibitor, daily for 6 months. There was no significant change in their muscle function, but there was a significant reduction in weight gain and in levels of growth hormone, somatomedin C, hair zinc, serum zinc, and serum LDH. Selenium and glutathione peroxidase in the serum increased significantly. Thirteen other children with growth hormone deficiency had a significant reduction in hair selenium following growth hormone administration. These results show a significant relationship between growth hormone and selenium nutritional status and confirm our previous reports indicating an effect of growth hormone on zinc nutritional status. It is possible that prolonged therapy with a growth hormone inhibitor would attenuate the course and improve the longevity of patients with muscular dystrophy.
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PMID:Growth hormone inhibition causes increased selenium levels in Duchenne muscular dystrophy: a possible new approach to therapy. 649 89

When blood selenium concentrations and glutathione peroxidase activity was measured in 30 standardbred horses a significant correlation was found (r = 0.97). A comparison between blood GSH-px activity in clinically healthy foals, foals affected by muscular dystrophy (MD) and their respective mares was also done. There was no difference in GSH-px activity between the healthy foals and the MD foals or between the corresponding mares.
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PMID:Glutathione peroxidase and selenium in the blood of healthy horses and foals affected by muscular dystrophy. 717 98

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 concentration of selenium (Se) and the activity of glutathione peroxidase (GSH-Px) in plasma and erythrocytes were measured in healthy men and in patients with Duchenne-type progressive muscular dystrophy (DMD). In healthy men, the Se concentration in erythrocytes showed a steep rise with aging and ascended gradually in plasma. The GSH-Px activity in both plasma and erythrocytes clearly increased with aging. The relationship between the Se concentration and the GSH-Px activity in healthy men showed a parallel rise with aging, but the coefficients of correlation were not very high (r = 0.44 and 0.56 in plasma and erythrocytes, respectively. In DMD patients, on the other hand, the Se concentration in erythrocytes decreased steeply with aging, and it decreased gradually in plasma. The GSH-Px activity in both plasma and erythrocytes apparently increased as in healthy men with aging, but the level was about 80% of that of healthy men. When selenite (Se+4) is added to the whole blood in vitro at 25 degrees C, it is rapidly taken up by erythrocytes (within several minutes) and is then released into plasma (a period of 30 min), then subsequent reuptake by erythrocytes is proceeded slowly. Our attention was attracted to the pattern of selenite release from erythrocytes of DMD patients.
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PMID:[Selenium concentration in blood and Duchenne-type progressive muscular dystrophy]. 858 80

The mechanisms primarily responsible for the degenerative processes occurring in dystrophic skeletal muscle remain unresolved. The identification of the mechanisms that lead to the complete sparing of extraocular muscle in dystrophinopathies is of particular interest. A number of studies have provided evidence to suggest that the muscle pathology that characterizes muscular dystrophy may be, in part, free radical mediated. In the present study, we examined the antioxidant enzyme status of extraocular, diaphragm and gastrocnemius muscles in control strain and mdx mice. Our results revealed that in the control strain, both extraocular and diaphragm muscles had higher copper/zinc superoxide dismutase, manganese superoxide dismutase and selenium dependent glutathione peroxidase activities as compared to the gastrocnemius. Furthermore, the diaphragm had higher glutathione reductase activity as compared to the gastrocnemius. These findings indicate that the highly aerobic extraocular and diaphragm muscles have higher antioxidant enzyme capacity than the gastrocnemius, a muscle more dependent on anaerobic energy metabolism. Changes in the antioxidant enzyme status of the mdx mouse correlated, in part, with the degree of histopathological involvement of the three muscle groups assessed.
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PMID:Extraocular, limb and diaphragm muscle group-specific antioxidant enzyme activity patterns in control and mdx mice. 885 50

Because of the very low concentrations of selenium in the dry matter of grass, grass silage, hay and maize silage Slovenian dairy herds need to be supplemented with selenium. Selenium in the form of mineral and feed mixtures maintained adequate mean (sd) blood serum selenium concentrations of 43.9 (27.6) to 65.3 (18.5) micrograms/litre in lactating cows, but in late lactation and in the dry period when only mineral mixtures were used, about 60 per cent of the cows had marginal serum selenium concentrations, mainly because of the low intake of the mineral supplement. In 18 herds which were either unsupplemented or irregularly supplemented with selenium, the mean (sd) concentrations in blood serum were 13.7 (5.5) micrograms/litre and 17.4 (9.2) micrograms/litre, respectively, for selenium and 2.98 (2.72) mg/litre and 1.62 (1.73) mg/litre for vitamin E, indicating that under extensive farming conditions in Slovenia the lack of both micronutrients may be responsible for nutritional muscular dystrophy in calves. Among 37 clinical cases, cardiorespiratory signs predominated in 25 of the calves and skeletal myopathy was dominant in 12. A very low mean serum selenium concentration [9.7 (7.2) micrograms/litre] and typically high activities of aspartate aminotransferase (AST) [1125 (373) U/litre] and creatine kinase (CK) [9169 (3681) U/litre) were observed for the myocardial form of the disease, and 2797 (550) U/litre and 22,650 (13,500) U/litre were observed for the skeletal form of the disease. A highly significant (P < 0.0001) difference in the selenium concentration of liver dry matter between the regularly supplemented [402 (207) micrograms/kg] and irregularly supplemented [173 (69) micrograms/kg] herds was observed. If a minimum value of 300 micrograms/kg of liver dry matter is accepted as the criterion for the determination of adequate selenium status, 93 per cent of the samples from the irregularly supplemented herds were selenium deficient. A similar proportion was estimated to be selenium deficient when the criterion was taken to be 30 micrograms selenium/litre of blood serum.
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PMID:Assessment of selenium and vitamin E deficiencies in dairy herds and clinical disease in calves. 891 12

Selenium (Se) was discovered 180 years ago. The toxicological properties of Se in livestock were recognized first; its essential nutritional role for animals was discovered in the 1950s and for humans in 1973. Only one reductive metabolic pathway of Se is well characterized in biological systems, although several naturally occurring inorganic and organic forms of the element exist. The amount of Se available for assimilation by the tissues is dependent on the form and concentration of the element. Se is incorporated into a number of functionally active selenoproteins, including the enzyme glutathione peroxidase, which acts as a cellular protector against free radical oxidative damage and type 1 iodothyronine 5'-deiodinase which interacts with iodine to prevent abnormal hormone metabolism. Se deficiency has been linked with numerous diseases, including endemic cardiomyopathy in Se-deficient regions of China; cancer, muscular dystrophy, malaria, and cardiovascular disease have also been implicated, but evidence for the association is often tenuous. Information on Se levels in foods and dietary intake is limited, and an average requirement for Se in the U.K. has no been established. Available data suggest that intake in the U.K. is adequate for all, except for a few risk groups such as patients on total parenteral nutrition or restrictive diets.
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PMID:Selenium in health and disease: a review. 914 18

We report a pair of siblings with non-Fukuyama type, merosin-positive congenital muscular dystrophy, born to unrelated parents. Patient 1 was a 16-year-old girl with myopathy, cardiomyopathy, severe mental retardation and epilepsy. Patient 2 was a younger brother of patient 1, a 10-year-old boy with myopathy, severe mental retardation and epilepsy. Their serum selenium levels were decreased to 25 micrograms/l and 55 micrograms/l, respectively (normal 97-147 micrograms/l). Their muscle biopsy findings were similar to those seen in selenium deficient myopathy, showing abnormal mitochondrial distribution and giant mitochondria. After oral administration of selenium for 3 months, their gait disturbance apparently improved, which was confirmed by a gait analysis system. Why their gait improved remain unclear, but a defect in selenium metabolism may play a role in the development of congenital muscular dystrophy and mental retardation.
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PMID:[Two sibling patients with non-Fukuyama type congenital muscular dystrophy with low serum selenium levels--therapeutic effects of oral selenium administration]. 1091 76

The causes of mortality in kids under extensive conditions in the Mexico plateau were studied. Perinatal mortality of kids (0-7 days) was lower (P<0.05) than mortality in the other age groups (8-90 days). The weights of the animals at death were extremely low, group (i) 0-7 days, 2.3kg; (ii) 7-30 days, 2.8kg and (iii) 30-90 days, 4kg. The principal finding at necropsy was white muscle disease (WMD). The WMD lesions, suggested selenium (Se) deficiency was present in 49 out of the 74 kids evaluated in this study (P<0.05). In many cases, muscular dystrophy was present with other pathological conditions. Enteric and pneumonic conditions were also important causes. In the small intestine villus atrophy was observed in 14 of the 25 kids that died with enteric problems between 8 and 30 days of age. Pneumonic lesions were present in 12 out of 74 kids. Miscellaneous conditions as starvation (five cases), hepatic conditions (three cases), omphalophlebitic infection (two cases) were also present in lower proportions. In conclusion, WMD was considered the main cause of deaths in goat kids between 8 and 90 days of age. Evaluation of Se status of soil, forages and goat tissues is recommended.
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PMID:Main causes of mortalities in dairy goat kids from the Mexican plateau. 1142 37


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