Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0432222 (SEM)
 47,337 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The production of energy in muscle from long-chain fatty acid oxidation is dependent upon the presence of carnitine. An abnormally low level of muscle carnitine, as seen in patients with the carnitine deficiency syndrome, results in marked muscle weakness. Muscle from 83 consecutive patients undergoing diagnostic muscle biopsy was assayed for carnitine. Carnitine levels (mean +/- SEM, expressed as nmoles carnitine per mg noncollagen protein) in muscle from patients with Duchenne dystrophy (8.1 +/- 1.7) and possible Becker dystrophy (10.6 +/- 3.0) were significantly (P less than 0.001) different from histologically normal muscle (24.0 +/- 1.4). Carnitine levels in patients with limb-girdle dystrophy (16.1 +/- 3.1) and polymyositis/dermatomyositis (16.6 +/- 3.2) were also low, although not as low as in Duchenne dystrophy. Carnitine levels from patients with denervation atrophy (22.1 +/- 3.6), nonspecific fiber atrophy (21.3 +/- 1.3), and a group of miscellaneous neuromuscular diseases (20.4 +/- 1.4) were not significantly different from histologically normal muscle. The low values of carnitine seen in Duchenne dystrophy and a group of possible Becker dystrophy patients may be a nonspecific effect, related to severe muscle damage.
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PMID:Muscle carnitine levels in neuromuscular disease. 92 14

A simple, reliable method for the exposure of specific structures hidden within bulk tissue for viewing by SEM is described. The method employs dry fracture after critical-point drying and differs from other dry fracture methods in that the fracture plane is 'engineered' by the experimenter, thus overcoming the tendency for natural planes of weakness within the specimen exclusively to define the fracture plane. The technique retains the simplicity of the very high-quality in situ cellular relationships normally associated with random dry fracture. Attention is given to novel means of circumventing the artefacts that are normally a problem with dry fracture techniques.
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PMID:SEM studies of surfaces hidden within bulk tissue: a simple technique to control the position and orientation of dry fracture planes. 140 45

Maximum voluntary force and cross-sectional area (MVF and CSA) of the human adductor pollicis muscle were compared in groups of young (19-55 years, mean = 28, n = 53) and elderly (74-90 years, mean = 80, n = 39) subjects, of both sexes. Despite the elderly subjects being in good health and active outdoors, they were considerably weaker than the young subjects, their MVF/CSA being 26 +/- 3% (mean +/- SEM) lower. It was found that both young and elderly subjects could fully activate their muscles. Therefore the muscle weakness of old age does not appear to be due to reduced activation and must be caused by another mechanism, possibly biochemical in nature.
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PMID:The weakness of old age is not due to failure of muscle activation. 153 65

In untreated patients with uncomplicated essential hypertension, exercise induces an abnormal increase in blood pressure; the influences of this increase on exercise were evaluated by a cardiopulmonary exercise test (CPX) performed in control conditions (step 1) and during acute blood pressure reduction (step 2). Patients were classified as (1) normotensive (resting diastolic blood pressure [BPd] less than 90 mm Hg; n = 14), (2) mildly hypertensive (BPd of 90 to 104 mm Hg; n = 9), and (3) moderately to severely hypertensive (BPd greater than or equal to 105 mm Hg; n = 16). For the three groups, peak mean blood pressure during exercise was 125 +/- 5 mm Hg (mean +/- SEM), 144 +/- 3 mm Hg (p less than 0.01 vs normotensive), and 161 +/- 4 mm Hg (p less than 0.01 vs normotensive and p less than 0.01 vs mild hypertension), respectively. Oxygen consumption (VO2) at peak exercise and at ventilatory anaerobic threshold was 26.1 +/- 1.1 and 17.2 +/- 0.5 ml/min/kg, 25.4 +/- 1.1 and 16.9 +/- 0.8 ml/min/kg, and 26.4 +/- 1.3 and 17.5 +/- 1.2 ml/min/kg in normotensive subjects, those with mild hypertension, and those with moderate to severe hypertension, respectively. Fourteen normotensive subjects, six with mild hypertension, and nine with moderate to severe hypertension participated to step 2 (nifedipine vs placebo, double-blind crossover). Nifedipine reduced blood pressure at rest and at peak exercise in those with hypertension. Peak exercise VO2 was unaffected by nifedipine in both normotensive subjects and those with hypertension. With nifedipine, ventilatory anaerobic threshold occurred earlier and at a lower VO2 in mild and in moderate to severe hypertension (delta VO2 = -1.9 and -2.4 ml/min/kg, respectively). These findings might be due to nifedipine-induced redistribution of blood flow during exercise and might be the reason for the complaint of weakness after blood pressure reduction in hypertensive subjects.
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PMID:Exercise performance in patients with uncomplicated essential hypertension. Effects of nifedipine-induced acute blood pressure reduction. 160 Jul 77

After anesthesia employing nondepolarizing muscle relaxants, 30%-40% of adult patients demonstrate residual paralysis with a train-of-four ratio less than 70%, but it is not known if the same is true for children. This study was designed to investigate neuromuscular transmission in 91 ASA physical status I or II day-care children (aged 0-10 yr) after halothane anesthesia in which pancuronium (n = 34), atracurium (n = 32), or vecuronium (n = 25) was administered. Peripheral nerve stimulation was used clinically to assess neuromuscular blockade during surgery. In the recovery room, the evoked response of the adductor pollicis muscle was measured by train-of-four stimulation of the ulnar nerve. This measurement was made (mean +/- SEM) at 18.0 +/- 1.5, 15.0 +/- 1.3, and 15.0 +/- 1.7 min after pharmacologic antagonism with 0.02 mg/kg atropine and 0.06 mg/kg neostigmine in the pancuronium, atracurium, and vecuronium groups, respectively. There were no differences in the ages of the patients in the three groups at 4.3 +/- 0.4, 4.0 +/- 0.4, and 5.0 +/- 0.5 yr, with 17 children less than 2 yr. Recovery from neuromuscular blockade in all three groups was almost complete. The train-of-four ratio (height of fourth twitch compared with the first) was similar in patients who had received pancuronium (96.7% +/- 0.9%), atracurium (95.5% +/- 0.9%), or vecuronium (96.3% +/- 1.3%). Therefore, postoperative muscle weakness or respiratory impairment is unlikely in pediatric day-care surgical patients more than 2 yr old when these anesthetic techniques are used.
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PMID:Postoperative neuromuscular function in pediatric day-care patients. 167 89

Human recombinant erythropoietin (r-HuEPO) improves quality of life in patients on maintenance haemodialysis, but the haemoglobin (Hb) level necessary to achieve this improvement is unknown. In this study, quality of life, functional capacity and symptoms of 28 haemodialysis patients with an initial Hb of 67 +/- 2 (mean +/- SEM) g/L were assessed after 0, 6 and 12 months of r-HuEPO, the dose of which was titrated to achieve a stable Hb of between 90 and 100 g/L. At six and 12 months Hb was 97 +/- 2 and 93 +/- 2 g/L, and mean r-HuEPO dose between three and six, and between nine and 12 months was 88 +/- 6 and 62 +/- 9 U/kg/week intravenously respectively. There was a significant improvement in level of activity and satisfaction with various aspects of life, and a reduction in fatigue, weakness, dyspnoea, angina and restless legs. Patients were able to walk 50% further in six minutes. The improvement in quality of life and function was similar to that reported from other centres whose target Hb was between 100 and 120 g/L, and where the r-HuEPO dose was 75% higher than in this study. Costs of r-HuEPO therapy were assessed. The drug itself costs +A3681/yr/patient, to which was added the estimated cost of additional dialyses and medications, bringing the total to +A5177/yr/patient. There was, however, a reduction in both hospitalisation by 8.3 days/yr/patient and medical consultation by 3.9 hours/yr/patient. Five patients commenced full-time work, one took up full-time study aimed at finding work, three transferred to home haemodialysis and six fewer patients drew social security benefits. The net cost saving from using low dose r-HuEPO was more than +A1,000/yr/patient.
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PMID:Low dose erythropoietin in maintenance haemodialysis: improvement in quality of life and reduction in true cost of haemodialysis. 175 17

The effect of the 21-aminosteroid U74006F on neurologic recovery after a spinal cord compression trauma was investigated in rats. The compression was induced by a blocking weight technique, in which a 35 g (moderate injury) or a 50 g (severe injury) weight was applied for 5 minutes to an 11 mm2 plate over the midthoracic spinal cord. One hour after trauma, the severely injured animals were treated either with U74006F, 3 mg/kg, methylprednisolone, 30 mg/kg, or vehicle, whereas the moderately injured animals received U74006F, 3 mg/kg or vehicle. Neurologic hind limb function was evaluated by the inclined plane technique. On day 1 after trauma, subtotal paraparesis occurred in the 35 g group treated with vehicle (31 +/- 1 degrees, mean +/- SEM) on the inclined plane vs 64 +/- 1 degrees before trauma) and complete paraplegia in the 50 g group (22 +/- 1 degrees). Treatment with U74006F resulted in less hind limb weakness in the 35 g group (42 +/- 2 degrees) but had no beneficial effect in the 50 g group (25 +/- 2 degrees). Neurologic function gradually improved in the 35 g groups over the 9-day observation period. However, those animals treated with U74006F were significantly better over the entire period. In the 50 g group, no recovery from paraplegia was noted over the 4 day observation period in any of the three groups. These results suggest that after weight-induced spinal cord trauma, U74006F is associated with improved neurologic function in moderately injured, but not severely injured animals.
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PMID:Blocking weight-induced spinal cord injury in rats: therapeutic effect of the 21-aminosteroid U74006F. 180 32

Anal dilatation in response to gentle parting of the buttocks has been advocated as a sign of sexual abuse in children, but nothing is known of the physiology of this response or its existence in normal subjects, in patients with spinal disease, and in patients with a weak sphincter and whether it can be elicited after training. To answer these questions we investigated the effect of parting the buttocks on anal function. Combined anal manometry and electromyography was conducted in six normal subjects (five men, one woman, aged 19-53 years), in 18 patients with faecal incontinence (three men, 15 women, aged 30-80 years), and in seven paraplegic patients (six men, one woman, aged 25-36 years), in four of whom the posterior sacral roots had been cut. Parting the buttocks in normal subjects reduced the pressure in the anal canal from 102 (20) to 14 (3) cm H2O (mean (SEM), p less than 0.00001), but did not cause the anus to gape. This drop in pressure was associated with increased electrical activity in the external anal sphincter. Normal subjects could consciously relax the external anal sphincter and reduce the anal pressure but not so as to result in anal gaping during traction on the buttocks, even after anal dilatation. Stimulation of the anal lining by moving a probe in and out of the anal canal increased the activity of the external anal sphincter, raising anal pressures. Paraplegic patients who had lost conscious control of their external sphincters showed anal gaping when the buttocks were parted. A similar phenomenon was seen in patients with faecal incontinence who had weakness of the external anal sphincter, while incontinent patients with weakness of both sphincters showed anal gaping even at rest. Inasmuch as the results of our study can be applied to children, the data suggest that reflex anal dilatation should only be used to support a diagnosis of sexual abuse if sphincter function is otherwise normal and there is no evidence of cerebrospinal disease. Although our results do not support the notion that children could become so conditioned to repeated digital or penile penetration of the anus that they can cause the anus to gape when the buttocks are parted, neither do they exclude it.
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PMID:Reflex anal dilatation: effect of parting the buttocks on anal function in normal subjects and patients with anorectal and spinal disease. 206 Aug 76

Hypomagnesemia is a common clinical finding in hospitalized patients and can cause hypocalcemia, cardiac arrhythmias, muscular weakness, and hypokalemia. Hypomagnesemia usually implies cellular magnesium (Mg) depletion, but stress and some clinical conditions which raise serum catecholamine concentrations may lower serum Mg (sMg) concentrations. To help investigate the mechanism and degree of the effect of catecholamines on sMg concentration, we gave intravenous epinephrine (0.1 microgram/kg/min) to 12 normal volunteers for 2 hours. The sMg concentration fell from 1.86 +/- 0.04 mg/dl to 1.63 +/- 0.05 mg/dl (mean +/- SEM, p less than 0.01). Pre-infusion intracellular free Mg (Mg++) in red blood cells (RBC) as measured by nuclear magnetic resonance spectrophotometry (NMR) was 171 +/- 7.6 microM and did not differ significantly from post-infusion RBC Mg++, 186 +/- 12.6 microM. Total blood mononuclear cell Mg content and urine Mg excretion also did not change. These data suggest that epinephrine has a small but significant effect on the lowering of sMg concentrations. Endogenous catecholamine release during stress or acute illness may therefore contribute to the hypomagnesemia seen in acutely ill patients. Our data also suggest that hypomagnesemia seen under conditions of acute stress may not always imply depleted tissue Mg stores. As no absolute change in cellular Mg or in urinary Mg excretion was demonstrated, acute intracellular shifts of Mg into blood cells and/or urinary Mg losses may not account for the hypomagnesemia. The prevalence and clinical consequences of stress hypomagnesemia require further investigation.
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PMID:Effect of intravenous epinephrine on serum magnesium and free intracellular red blood cell magnesium concentrations measured by nuclear magnetic resonance. 218 26

The efficacy of pain relief and the maternal and neonatal effects of continuous epidural infusion of 0.0625% bupivacaine/0.002% butorphanol was compared with the infusion of 0.125% bupivacaine alone in a randomized, double-blind study of 32 women in labor. A test dose of 2 ml 0.5% bupivacaine was given to every patient and followed by two epidural regimens in randomized, double-blind manner. Group B-B (bupivacaine/butorphanol) patients received 7.5 ml 0.125% bupivacaine plus 1 mg butorphanol (0.5 ml) followed by an infusion of 0.0625% bupivacaine/0.002% butorphanol at a rate of 12 ml/hour; Group B (bupivacaine alone) patients received 8 ml 0.25% bupivacaine followed by an infusion of 0.125% bupivacaine at a rate of 12 ml/hour. A bolus of 5 ml 0.125% bupivacaine or 0.0625% bupivacaine was given to Group B or B-B, respectively, if additional pain relief was required. Infusion of B-B combination resulted in similar pain relief and fewer patients with motor block than bupivacaine alone; 12% versus 38% in Groups B-B and B, respectively, had motor weakness. A smaller dose of bupivacaine was used in the B-B group compared to the B group; 71 +/- 14 versus 99 +/- 13 mg (mean +/- SEM; p less than 0.05). Progress of labor and the mode of delivery did not differ significantly between the two groups. All infants were vigorous and had normal acid-base status and neurologic adaptive capacity scores. Butorphanol appears to be useful as an adjunct to epidural bupivacaine for continuous epidural infusion during labor without adversely affecting the mother or the neonate.
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PMID:Continuous infusion epidural anesthesia during labor: a randomized, double-blind comparison of 0.0625% bupivacaine/0.002% butorphanol and 0.125% bupivacaine. 229 85


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