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

Twenty-eight Holstein heifer calves were allotted at birth to one of four treatments: 1) 0 mg, 2) 1,400 mg, or 3) 2,800 mg of dl-alpha-tocopherol acetate given orally at weekly intervals, or 4) 1,400 IU of dl-alpha-tocopherol weekly by intramuscular injection in order for us to study their performance and metabolic profile. Calves were fed milk at 8% of birth weight until they were weaned at 6 wk of age and fed a complete calf starter ad libitum from birth. Calves were on experiment for 12 wk. There were no significant differences in weekly weight gains, starter consumption, and fecal scores among treatments. However, there was a trend toward greater starter consumption and weight gains in supplemental calves. Serum alpha-tocopherol concentration measured after 7 d of each administration was significantly higher at wk 4 in calves given the high oral supplementation and at wk 2, 4, 6, and 8 higher in injected calves than in unsupplemented calves. Creatine kinase activity was higher in unsupplemented calves and negatively correlated with serum alpha-tocopherol until wk 8, suggesting preclinical muscular dystrophy. Alkaline phosphatase activity was higher with the high oral supplementation. Serum carbon dioxide values showed a trend toward positive correlation with those for serum tocopherol; however, the values were within normal range. There were no significant differences in creatinine, glucose, phosphorus, calcium, urea nitrogen, chloride, sodium, potassium, albumin, and total protein among treatments. Serum glucose was higher in all calves at wk 10 and 12 than at wk 4, 6, and 8. Calves may not get enough vitamin E with conventional calf starters, and supplementation may be essential to obtain maximum performance.
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PMID:Effects of supplemental vitamin E on the performance and metabolic profiles of dairy calves. 406 45

Serial consecutive frozen sections from 164 muscle biopsy specimens were studied cytochemically. We localized calcium and albumin as endogenous markers of extracellular fluid penetration and C3 and C9 complement components as markers of muscle fiber necrosis. In both Duchenne dystrophy and congenital muscular dystrophy, a significant percentage of fibers were overloaded with calcium and penetrated by albumin. Most of these fibers appeared opaque with trichrome stain. C3 and C9 complement components appeared only in necrotic fibers, which invariably were also penetrated by albumin. These observations support previous findings that muscle fiber necrosis is linked to massive inflow of extracellular fluid and complement activation. In addition, in both Duchenne and congenital dystrophies, numerous nonnecrotic fibers are penetrated by calcium-rich extracellular fluid.
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PMID:Muscle fiber degeneration and necrosis in muscular dystrophy and other muscle diseases: cytochemical and immunocytochemical data. 652 76

Tears from myotonic muscular dystrophy (MMD) patients and normal controls were analyzed for their tear proteins by sodium dodecyl sulfate-polyacrylamide gel electrophoresis. Lactoferrin comprised about 18% of the total tear protein in MMD patients as opposed to about 27% in normals. The albumin content relative to total protein in MMD tears was about 25% while the same value for normals is 13%. The lactoferrin/albumin ratio, was about 0.8 for MMD patients and about 2.1 for normals.
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PMID:Decrease of lactoferrin concentration in the tears of myotonic muscular dystrophy patients. 665 10

Muscle damage is often associated with an influx of extracellular fluid containing albumin into the muscle. Muscles affected by muscular dystrophy undergo severe muscle damage; therefore, the hypothesis was tested that muscles of dystrophic (mdx) mice contain elevated levels of albumin. Albumin levels in diaphragm (DIA) and soleus (SOL) muscles of control and mdx mice were measured at 3 months and 1 year of age. Albumin in mdx DIA at 1 year of age was twice that of control. In mdx SOL at 1 year of age albumin was increased 25% compared with control. The increase in albumin correlates well with the decline in function in mdx DIA and SOL muscles. Electron microscopy of muscles suggests that albumin is co-localized with transverse tubules of muscle fibers and thus may be mainly located in extracellular fluid. We conclude that albumin is elevated in muscles affected by muscular dystrophy and suggest that this may be of clinical importance in view of substances bound to albumin under physiological conditions.
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PMID:Elevated levels of albumin in soleus and diaphragm muscles of mdx mice. 898 13

Membrane lesions play an early role in the pathogenesis of muscular dystrophy. Using a new albumin-targeted contrast agent (MS-325), sarcolemmal integrity of two animal models for muscular dystrophy was studied by MRI. Intravenously injected MS-325 does not enter skeletal muscle of normal mice. However, mdx and Sgca-null mutant mice, animal models for Duchenne and sarcoglycan-deficient limb-girdle muscular dystrophy, respectively, showed significant accumulation of MS-325 in skeletal muscle. The results suggest that contrast agent-enhanced MRI could serve as a common, noninvasive imaging procedure for evaluating the localization, extent, and mechanisms of skeletal muscle damage in muscular dystrophy. Furthermore, this method is expected to facilitate assessment of therapeutic approaches in these diseases.
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PMID:Contrast agent-enhanced magnetic resonance imaging of skeletal muscle damage in animal models of muscular dystrophy. 1102 24

EPIX is developing MS-325 (AngioMARK), an intravascular magnetic resonance contrast agent for use in the imaging of blood vessels and blood flow in patients with cardiovascular disease, including peripheral vascular disease (PVD). In June 1999, EPIX and Mallinckrodt began phase III trials of MS-325 for the detection of aortoiliac occlusive disease in patients with PVD or abdominal aortic aneurysm [328640]. NDAs for the peripheral and cardiac applications were expected in 1999 and 2000, respectively [275240], [325717]. MS-325 has also shown promise in demonstrating the presence of microscopic muscular dystrophy, as well as monitoring the effects of gene therapy in a mouse model of the disease [360974]. MS-325 is a stable complex of gadolinium and an organic chelating agent. It resembles approved agents in terms of stability, safety and elimination profile, but possesses novel chemical groups which allow it to bind reversibly to albumin. This retains the agent in the blood and, via a patented biophysical phenomenon, enhances the magnetic properties of the gadolinium ion approximately ten-fold.
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PMID:MS-325 EPIX. 1124 2

The spectrum of nonmuscular involvement in six children with merosin-negative congenital muscular dystrophy is described. In all children, biochemical, neuroradiologic, cardiac, and neurophysiologic studies were performed. Cerebral structures that were myelinated at gestation, including internal capsule, corpus callosum, brainstem, and cerebellar white matter, demonstrated no abnormalities, whereas the periventricular and subcortical white matter, which were myelinated in the first postnatal year, demonstrated signs of leukoencephalopathy. Cerebrospinal fluid analysis revealed an elevated albumin cerebrospinal fluid to serum ratio in the younger children. Electroencephalogram results were abnormal in the two elder children. One child suffered from congestive cardiomyopathy. The increase in nerve conduction velocity in these children over the years lagged behind those of healthy patients, pointing to a demyelinating neuropathy. We conclude that in merosin-negative congenital muscular dystrophy patients, nonmuscular involvement includes the central and peripheral nervous system and the heart. The pattern of myelination of the brain and nerve conduction slowing suggests a myelination arrest. Merosin deficiency can give rise to a congestive cardiomyopathy, which is of no clinical relevance in the majority of children.
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PMID:Nonmuscular involvement in merosin-negative congenital muscular dystrophy. 1181 32