Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Either simultaneous or separate dietary deficiencies of vitamin E and selenium in Atlantic salmon during first 4 weeks of feeding caused twice the mortality shown in fish fed both supplemental vitamin E (0.5 IU/g dry diet) and selenium (0.1 mug/g). Subsequent dietary repletion with both vitamin E and selenium significantly reduced mortality during the following 2 weeks. Larger salmon (0.9 g initial mean weight), with vitamin E deficiency with or without selenium resulted in the following deficiency signs: extreme anemia, pale gills, anisocytosis, poikilocytosis, elevated plasma protein, exudative diathesis, dermal depigmentation, in vitro ascorbic acid-stimulated peroxidation in hepatic microsomes, yellow-orange liver color, yellow-brown intestinal contents, enlarged gall bladder distended with dark green bile, low vitamin E in carcass and hepatic tissue, muscular dystrophy, increased carcass fat and water, and a response to handling characterized by a transitory fainting with interruption in swimming. A deficiency of dietary selenium suppressed plasma glutathione peroxidase activity. Supplemental selenium with vitamin E significantly increased tocopherol activity in hepatic, but not carcass tissues. Supplements of both vitamin E and selenium were necessary to prevent muscular dystrophy.
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PMID:Vitamin E and selenium interrelations in the diet of Atlantic salmon (Salmo salar): gross, histological and biochemical deficiency signs. 93 27

We have previously shown that oral diethylstilbestrol (DES) lowers the high serum enzyme levels characteristic of Duchenne's muscular dystrophy (DMD). The present studies were undertaken to assess the effect of DES on the efflux of enzymes from isolated mouse skeletal muscle. Thirty-four male mice were used. Half received daily subcutaneous injections of 10 mug diethylstilbestrol-diphosphate (DES-DP) in saline for up to 3 wk and half daily saline injections. Left gastrocnemii were isolated from control and treated mice, and placed in separate incubation media at 37 degrees C. The efflux of creatine phosphokinase (CPK) and lactate dehydrogenase (LDH) from each was compared over a 4- to 5-hr period. In 15 of 18 there was a reduction in efflux of both enzymes from muscles isolated from DES-DP-treated mice. The greatest effect was noted during the second hour, when the mean efflux of each enzyme was reduced about 30%. Minor differences in muscle weight, water content, and enzyme activities did not explain the reduced efflux. These results suggest that DES has either reduced the permeability of the sarcolemma or enhanced the intracytoplasmic stability of these enzymes. This is the first drug reported to reduce the spontaneous enzyme efflux from isolated skeletal muscle. It remains to be established that a similar effect accounts for the reduction in serum enzyme levels when DES is administered to persons with DMD.
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PMID:Reduction of enzyme efflux from skeletal muscle by diethylstilbestrol. 114 55

Thick round fibres common in cross sections of muscle biopsies from patients with muscular dystrophy are due to contracted and swollen segments of otherwise normal muscle fibres. This contracture leads to segmental fibre breakdown, which is identical with Zenker's waxy degeneration. In biopsies from 90 patients suspected of neuromuscular disease, segmental contracture was seen in all or nearly all patients with infantile muscular dystrophy, necrotic myopathy or acute alcoholic myopathy. It was present in half of the patients with polymyositis or myotonic dystrophy. In resticted forms of muscular dystrophy it was rare as it was in neurogenic atrophy. In 9 clinically normal patients it was absent. In electron micrographs of the initial stage sarcomeres were moderately shortened, the sarcoplasmic reticulum was distended and the mitochondria were normal. In the plasmalemma holes were found, through which glycogen granules were lost into the interstitial tissue. In later stages myofibrils were overcontracted and homogenized; in large areas the plasmalemma was absent. Based on these findings a hypothesis for the development of waxy degeneration is proposed: locally defects of the plasmamembrane cause segmental contracture, glycogen granules and water soluble enzymes are lost through holes in the plasma membrane, and finally the affected fibre segment becomes necrotic.
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PMID:Segmental fibre breakdown and defects of the plasmalemma in diseased human muscles. 120 96

Twenty-six domestic reports of suspected adverse reactions from the guar gum-containing diet pill, Cal-Ban 3000 (filed with the FDA) were reviewed. There were 18 instances of esophageal obstruction, seven instances of small bowel obstruction, and one individual who was reported to have died after ingestion of Cal-Ban 3000, but for whom insufficient details were provided to assess causation. There were 14 women and 11 men (mean age 46.3 yr; range 17 to 67 yr) for whom sufficient information was available. Preexisting esophageal or gastric disorders were present in 50% of those with esophageal obstruction, including peptic stricture, pyrosis, hiatal hernia, esophagitis, gastric stapling procedure, Schatzki ring, and muscular dystrophy. Fourteen of these 18 patients with esophageal obstruction were treated successfully by endoscopy, although the tenacious gel-like consistency of the material was often difficult to remove. Two patients required rigid esophagoscopy when flexible endoscopy was unsuccessful. This resulted in the death of one patient who developed a pulmonary embolism after surgical repair of an intraoperative esophageal tear. For the seven patients with small bowel obstruction, no specific predisposing factors were mentioned. One individual required exploratory laparotomy, and inspissated tablets were found in the ileum. These cases, spontaneously reported to the FDA, are very similar to those reported in the literature. The water-holding capacity and gel-forming tendency of guar gum permits it to swell in size 10- to 20-fold, and may lead to luminal obstruction, especially when an anatomic predisposition exists. Such products have been banned in Australia, and Cal-Ban 3000 has recently been removed from the market in the United States. However, unsuspecting patients who are still in possession of the product should be apprised of the potential complications that may arise with its use.
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PMID:Esophageal and small bowel obstruction from guar gum-containing "diet pills": analysis of 26 cases reported to the Food and Drug Administration. 132 94

Magnetization transfer contrast imaging is an MR technique that capitalizes on interactions between the protons of mobile and macromolecularly bound water molecules. Studies to date, conducted primarily on 4.7 T and 1.5 T MR systems, have yielded results unique from conventional T1- and T2-weighted imaging studies. In this study, performed on a 0.1 T device, a section of lower leg was imaged in 20 normal human subjects and one patient with muscular dystrophy, using both a standard 500/22 gradient-echo sequence and a 500/22 gradient-echo sequence combined with off-resonance radio frequency irradiation designed to elicit magnetization transfer contrast. Results of the two techniques were compared. Our findings suggest that magnetization transfer contrast imaging is feasible at 0.1 T, and that this technique allows reproducible tissue characterization and improves contrast between certain tissues.
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PMID:Magnetization transfer contrast imaging of the human leg at 0.1 T: a preliminary study. 140 86

We have studied the water permeability through membranes, the function of the Na pump, and glucose metabolism of erythrocytes of patients with myotonic muscular dystrophy (MyD) using 1H--, 23Na, and 13C-NMR techniques. A significant decrease in water permeability was recognized in the MyD erythrocyte membrane, and impaired Na pumping was suspected to be correlated with the former biochemical abnormalities in band III protein of MyD erythrocyte membrane. Significant acceleration of glycolysis in the erythrocyte for the first 160 minutes was also recognized in MyD; however, the production of lactate showed no difference between MyD and controls. The increased glucose uptake in MyD may be compensatory to the diminished pumping mechanism, but further information, such as inorganic phosphate permeability and the activity of the rate-limiting enzyme of erythrocyte glycolysis, is needed.
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PMID:Study on the erythrocytes from myotonic dystrophy with multi-nuclear NMR. 199 97

Brain MR examinations were performed on one normal and 30 abnormal neonates and infants with a variety of clinical problems using pulsed gradient spin echo (PGSE) sequences sensitized in different directions to detect changes due to anisotropically restricted diffusion of water within the brain. Anisotropically restricted diffusion was demonstrated within white matter, which appeared to be myelinated with T1-weighted inversion recovery or spin echo sequences and within white matter where the presence of myelin was not demonstrated with these sequences. Cysts and fluid collections were recognised by their low signal intensity using PGSE sequences in the subcortical regions, lentiform nuclei, and periventricular regions. An intracerebral hematoma and a subdural hematoma showed a high signal intensity with PGSE sequences consistent with isotropically restricted diffusion. Chronic infarction displayed a low signal intensity consistent with relatively free isotropic diffusion. Patients with leukodystrophy associated with congenital muscular dystrophy showed an anisotropic pattern with different components of the abnormality present on images with sensitization in different directions. The corticospinal tracts failed to show highlighting in three cases where conventional imaging was normal. In several patients with a history of birth asphyxia, asymmetry and a relative reduction in signal intensity were seen in the corticospinal tracts.
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PMID:MR imaging of anisotropically restricted diffusion in the brain of neonates and infants. 200 94

Skeletal muscles from healthy dogs and Labrador Retrievers with hereditary muscular dystrophy were examined morphologically and histochemically and were analyzed biochemically for Na+, K+, Ca2+, Mg2+, Zn2+, Cu2+, Cl-, total muscle water, and total neutral lipid content. Flame atomic absorption spectrophotometer was used for elemental quantitation of hydrochloric acid tissue extracts. Muscle samples from dystrophic dogs contained substantially increased concentrations of Na+, Ca2+, Zn2+, Cu2+, and Cl-, and a considerable reduction in the content of K+ and Mg2+ compared with samples from healthy dogs. Total muscle water and total fat content was higher in muscles from dystrophic dogs. Most muscle samples from dystrophic dogs had a type-2 fiber deficiency and an increase in number of fibers with internalized nuclei.
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PMID:Analysis of muscle elements, water, and total lipids from healthy dogs and Labrador retrievers with hereditary muscular dystrophy. 272 11

Skeletal muscles from normal dogs and labrador retrievers with a hereditary muscular dystrophy were examined morphologically and histochemically and were analysed for sodium (Na+), potassium (K+) and chloride (Cl-) ions and total muscle water. The partition of total muscle water and electrolytes between intracellular and extracellular phases was calculated on the basis of the chloride space method as the estimate of extracellular fluid volume. Muscle samples from dystrophic dogs contained significantly increased concentrations of Na+, Cl-, total muscle water, and a significant reduction in the level of K+ compared with normal values. There was a significant increase in the intracellular water and Na+ levels with a concomitant reduction of intracellular K+ content. Most dystrophic muscle samples had a pronounced type 2 fibre deficiency and a marked increase in numbers of fibres with internalised nuclei.
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PMID:Intracellular electrolytes and water analysis in dystrophic canine muscles. 277 3

Intermittent positive pressure ventilation (IPPB) is reported to improve lung compliance and decrease the work of breathing in subjects with kyphoscoliosis. These results suggest that IPPB may improve chest wall and lung compliance in patients with neuromuscular disease. We studied the short-term effects of IPPB on total respiratory system compliance in 14 subjects with neuromuscular disease. Seven were quadriplegics, and seven had muscular dystrophy. Vital capacity was reduced to 38 +/- 14 percent of the predicted normal values. Baseline measurements of total respiratory system compliance were 57 +/- 18 percent when compared to normal control values. After a 20 minute treatment of IPPB delivered with inspiratory pressures of 20 to 25 cm H2O that more than tripled resting tidal volume, there was no significant change in total respiratory system compliance in either group of patients. These findings indicate that patients with quadriplegia or muscular dystrophy do not derive immediate improvement in ventilatory mechanics from IPPB treatments.
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PMID:Intermittent positive pressure breathing in patients with respiratory muscle weakness. Alterations in total respiratory system compliance. 353 Jun 48


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