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)

We compared the morphologic characteristics of muscle fiber necrosis and subsequent regeneration after injury induced by intramuscular injections of bupivacaine hydrochloride (BPVC) and a variety of solutions at acid and alkaline pH (acetic anhydride, citric acid buffer, and sodium carbonate buffer). After BPVC injection the necrotic muscle fibers were rapidly invaded by phagocytic cells, followed by active regeneration and very little fibrous scar formation. The regenerating muscle fibers increased rapidly in size and attained complete fiber type differentiation and regained their initial fiber diameter within 1 month. Both alkaline and acid solutions induced muscle fiber necrosis followed by regeneration. Fiber necrosis induced by alkaline buffers and acetic anhydride solutions above pH 5.0 produced changes quite similar to that induced by BPVC. However, injection with 0.1 M acetic anhydride at pH below 4.0 resulted in coagulative necrosis of the injured muscle with very little phagocytic infiltration with poor regenerative activity and dense fibrous tissue scarring. Thus, pH 4.0 appears to be the critical pH determining the type of muscle injury and subsequent poor phagocytic and regenerative activities. This model of acidic acetic anhydride injury may lead to the identification of factors which interfere with regeneration and cause fibrous tissue scarring in human muscular dystrophy.
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PMID:Comparison of behavior in muscle fiber regeneration after bupivacaine hydrochloride- and acid anhydride-induced myonecrosis. 163 76

Muscular dystrophy patients fall in respiratory failure in the terminal stage. Erythrocyte 2,3-diphosphoglycerate (2,3-DPG) is an important regulator of oxygen release, as it affects the position of the oxyhemoglobin dissociation curve. In order to survey the internal respiration in these patients, we studied the erythrocyte 2,3-DPG which regulates oxygen transport function. The concentration of erythrocyte 2,3-DPG was determined in 27 cases with Duchenne type muscular dystrophy and 10 cases with myotonic dystrophy (MyD). We analyzed the relation of erythrocyte 2,3-DPG to spirogram, arterial blood gas and acid-base analysis in these patients. 14 normal males were used as controls. In control subjects, the mean concentration ratio of 2,3-DPG and hemoglobin (DPG/Hb) was 0.880 +/- 0.072. 18 cases of DMD and 9 of MyD, which showed more than 45 torr of Pco2 in arterial blood gas, revealed 0.823 +/- 0.053 and 0.814 +/- 0.092 of DPG/Hb respectively. These values were significantly lower than that of controls. DPG/Hb correlated to % VC, Pao2, Paco2, pH, HCO3 and BE in DMD, but no relation to these parameters in MyD. The low ratio of DPG/Hb in erythrocyte was considered to be metabolic compensation of respiratory failure in DMD. On the other hand, 2,3-DPG of MyD seemed to be also affected by any other factors in addition to respiratory failure.
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PMID:[Erythrocyte 2,3-diphosphoglycerate in muscular dystrophy]. 275 52

We studied changes in blood gas data from 32 patients with Duchenne type muscular dystrophy (DMD), who were followed for more than 5 years. Regression lines for each blood gas variable were obtained by the least square regression method, from 276 observations. The mean change per year was calculated by averaging the individual slopes. All blood gas variables (PaO2, PaCO2, pH, and [HCO3-]) correlated significantly with age the regression lines were: PaO2 = 95.6-0.45x Age, PaCO2 = 34.7 + 0.70x Age, pH = 7.397-0.0023x Age, [HCO3-] = 20.7 + 0.28x Age. The rates of change were -0.74 Torr/year for PaO2, 1.07 Torr/year for PaCO2, -0.0028 per year for pH and 0.55 mEq/l/year for [HCO3-]. The slope of each regression line was within the average individual yearly change +/- one standard deviation. AaDO2 did not change significantly with age, but chronic respiratory acidosis worsened because of alveolar hypoventilation. As an index of the severity of DMD, PaCO2 was better than PaO2, because the former was more strongly correlated with age and the values of PaCO2 deviated less from the the regression line.
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PMID:[Blood gas changes in Duchenne type muscular dystrophy]. 769 62