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

Administration of inhibitors of prostaglandin synthetase to chicken embryos produced myopathies in their skeletal muscles which were characterized by ringbinden, hypercontraction, loss of Z disks, M bands, and thick and thin filaments. The effects of the inhibitors were reversed by administration of PGE1. The pathology of the myopathy induced in the chicken embryos was identical to that observed in human muscular dystrophy and had some features in common with other congenital myopathies. Altered prostaglandin function may therefore be directly or indirectly involved in the pathogenesis of congenital myopathies.
Exp Neurol 1985 Sep
PMID:Inhibition of prostaglandin synthesis produces a muscular dystrophy-like myopathy. 404 Aug 68

Muscle biopsies from 10 Japanese patients (9 females and 1 male) with congenital muscular dystrophy (CMD) were studied. Their clinical features varied remarkably in severity; one patient died at 6 years of age. Family history was negative in all but one patient who had an affected sibling. Muscle biopsy findings varied from mild myopathic to advanced dystrophic changes. Hypertrophic fibers associated with occasional fiber splitting were assumed to reflect a chronic dystrophic process. Histochemical examination revealed type 1 fiber predominance in 5 patients, and type 2 fiber predominance in one. Eight patients had a slight to moderate increase in the number of undifferentiated type 2C fibers suggesting a regenerating process after fiber necrosis. Type 2B fibers were fairly well preserved in 8 patients. The overall findings differed from those of the Fukuyama type congenital muscular dystrophy (FCMD) and Duchenne muscular dystrophy (DMD) in which more active fiber necrosis and regeneration are seen. We conclude that the present CMD patients suffered from a chronic dystrophic process similar to that in limb-girdle muscular dystrophy.
J Neurol Sci 1985 Sep
PMID:Congenital muscular dystrophy. A histochemical study with morphometric analysis on biopsied muscles. 405 19

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.
J Dairy Sci 1985 Sep
PMID:Effects of supplemental vitamin E on the performance and metabolic profiles of dairy calves. 406 45

Eleven patients diagnosed as having muscular dystrophy and who underwent posterior spinal fusion were reviewed: Becker dystrophy in one, limb girdle in two, facioscapulohumeral in one, myopathia unspecified in one, myotonia dystrophica in two, myotonia congenita in one, and hypotonia congenita in three. There were eight females and three males. The curve pattern was thoracic in four, thoracolumbar in three, double thoracic and thoracolumbar in three, and thoracolumbar lordosis in one. Scoliosis was associated with kyphosis in two, with lumbar lordosis in one, and thoracic lordosis in four patients associated with poor vital capacity and shortness of breath. Seven patients had nonoperative treatment, five showing increase of the curve, and two having control of the curve. All patients had posterior spinal fusion with instrumentation with a follow-up of 9-89 months (average, 41 months). Postoperative support was used in all but one. Major complications occurred in four patients: a symptom of vascular obstruction of the duodenum in two, extubation delayed until the 7th day postoperatively in one and pseudarthrosis in one resulting in an increasing curve and refusion. One patient (limb girdle), 6 years after surgery at 21 years died from cardiomyopathy. The second (limb girdle) lost ambulation at age 22 years, 6.6 years after spinal surgery. In conclusion, patients with muscular dystrophies other than Duchenne generally have slowly evolving curves, and the use of an orthosis in the juvenile years controlled the curve until the pubertal growth spurt, when progression occurred. Surgical treatment was successful in stabilizing the deformities.
Spine (Phila Pa 1976) 1985 Sep
PMID:Spinal deformities in patients with muscular dystrophy other than Duchenne. A review of 11 patients having surgical treatment. 407 Dec 69

Myocardial involvement in progressive muscular dystrophy of the Duchenne type was evaluated in 19 patients using thallium-201 myocardial perfusion imaging. A qualitative analysis was performed from five projection images by three experienced physicians. Distinct perfusion defects were shown in 13 patients, especially in the LV posterolateral or posterior wall (11 patients). There was no significant relationship between the presence of perfusion defects and the skeletal muscle involvements or thoracic deformities assessed by transmission computed tomography. Extensive perfusion defects were shown in 2 patients who died of congestive heart failure 1 to 2 years after the scintigraphic study. Progression of the myocardial scintigraphic abnormalities were considered to be minimal in 7 of 9 patients who underwent two serial scintigraphic studies over 2 to 3 years. It was concluded that thallium myocardial perfusion imaging is a useful clinical technique to assess myocardial involvement in Duchenne's progressive muscular dystrophy.
Jpn Heart J 1985 Sep
PMID:Evaluation of myocardial involvement in Duchenne's progressive muscular dystrophy with thallium-201 myocardial perfusion imaging. 408 70

We report on a 15 year old patient with Duchenne's progressive muscular dystrophy who demonstrated a narrowing of the left ventricular inflow and outflow tracts due to compression by a highly deformed thoracic spine. A systolic murmur (4/6) with thrill and a diastolic murmur (2/6) were heard, with these murmurs being louder in the expiratory phase as compared with the inspiratory phase. The second heart sound showed a paradoxical splitting. Echocardiograms revealed a compressed and narrowed left ventricle and a prolapsed mitral valve. The intensities of the heart murmurs changed synchronously with the chamber's narrowing due to respiration. A narrowed left ventricle occurring as a result of the compression by the deformed thoracic spine is thought to be the cause of these cardiac findings.
Jpn Heart J 1985 Sep
PMID:Compressed heart and mitral valve prolapse in a case of Duchenne's progressive muscular dystrophy with thorax deformity. 408 78

The total population of 11,865 children of compulsory school age resident on the Isle of Wight was studied to determine the prevalence of epilepsy, cerebral palsy, and other neurological disorders. With the use of reliable methods, children selected from screening of the total population were individually studied by means of parental interviews and questionaries, neurological examination and psychiatric assessment of each child, information from school teachers, and perusal of the records of hospitals and other agencies. The association between organic brain dysfunction and psychiatric disorder was studied by comparing the findings in the children with epilepsy or with lesions above the brain stem (cerebral palsy and similar disorders) with those in (1) a random sample of the general population, (2) children with lesions below the brain stem (for example, muscular dystrophy or paralyses following poliomyelitis), and (3) children with other chronic physical handicaps not involving the nervous system (for example, asthma, heart disease, or diabetes).Psychiatric disorders in children with neuro-epileptic conditions were five times as common as in the general population and three times as common as in children with chronic physical handicaps not involving the brain. It was concluded, on the basis of a study of factors associated with psychiatric disorder, that the high rate of psychiatric disorder in the neuro-epileptic children was due to the presence of organic brain dysfunction rather than just the existence of a physical handicap (though this also played a part). However, organic brain dysfunction was not associated with any specific type of disorder. Within the neuro-epileptic group the neurological features and the type of fit, intellectual/educational factors, and socio-familial factors all interacted in the development of psychiatric disorder.
Br Med J 1968 Sep 21
PMID:Organic brain dysfunction and child psychiatric disorder. 423 74

The ultrastructure of the semitendonosus muscle of lambs and the pectoralis major muscles of chickens suffering from nutritional muscular dystrophy were studied. Samples of tissue from histologically normal and mildly degenerate areas were examined. Ultrastructural examination of areas that appeared apparently normal histologically, revealed lesions in the small vessels, connective tissue elements and neuromuscular elements. The possible relationship of these findings to the etiology of this disease are discussed.
Am J Pathol 1972 Sep
PMID:Ultrastructure of muscular dystrophy. II. A comparative study in lambs and chickens. 505 55

The vaccination status of 98 physically handicapped children was examined to identify factors associated with an inadequate vaccination status. Of the 98 children, 57 had cerebral palsy, 14 had myelomeningocele, 3 had muscular dystrophy and 24 had myelomeningocele, 3 had muscular dystrophy and 24 had other motor disabilities. According to the available vaccination records, only 17 children had received all the recommended injections on schedule; 26 had missed at least one injection, and 3 of them had never been vaccinated. The overall rate of vaccination in our study group (63%) was lower than expected. The children with moderate to severe limitation of function due to cerebral palsy were significantly less likely (P less than 0.05) than those with less severe limitations to have received a basic series of vaccinations. Health departments must ensure that physically handicapped children receive the preventive health measures viewed normal and appropriate for other children.
Can Med Assoc J 1982 Sep 15
PMID:Vaccinations and the physically handicapped child. 621 4

The etiopathogenesis of myotonic muscular dystrophy is thought to involve a basic defect in muscle membrane. Biochemical investigations of human muscle membrane have been hampered by difficulty in obtaining large quantities of muscle at biopsy for the preparation of sarcolemma. We have determined [3H]ouabain binding to normal and myotonic dystrophy human skeletal muscle by using cryostat sections. The binding increased with increase in number of tissue sections (protein) and in concentrations of [3H]ouabain, ATP and Na+. The binding of [3H]ouabain in myotonic dystrophy patients was 2-3 fold higher than in normal and disease controls. Kinetic analysis revealed that the increased binding of ouabain to myotonic tissue sections was independent of low-affinity sites directed by ATP and Na+. These findings provide further evidence for the involvement of membrane abnormalities in myotonic muscular dystrophy.
J Neurol Sci 1981 Sep
PMID:Quantitative measurement of [3H]ouabain binding to human skeletal muscle cryostat sections. 626 59


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