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

Muscle structure and blood enzyme activity were studied to 16 wk of age in lines of turkeys selected for rapid growth. The body and carcass weights were measured, frozen sections of breast and leg muscles examined, and plasma creatine kinase (CK) levels determined. Muscle weights were usually proportional to BW except for the relatively larger superficial pectoralis (SP) muscles in the most rapidly growing line. Damaged muscle fibers were found in all muscles examined, especially in the SP of the breast, the gastrocnemius (GA), and other muscles of the leg; these damages became more common from 10 to 16 wk of age. There were more degenerating muscle fibers and higher levels of plasma CK in the rapidly growing lines than in a slower growing unselected line. The findings support the idea that a focal myopathy, unrelated to deep pectoral myopathy or to inherited muscular dystrophy of the chicken, is associated with rapid growth of turkeys.
Poult Sci 1990 Sep
PMID:Turkey muscle growth and focal myopathy. 224 18

Cardiac involvement is common in patients with Duchenne type muscular dystrophy (DMD). However, published reports of long-term follow-up studies in the same patients are very rare. The purpose of this study was to clarify the natural course of cardiac dysfunction in DMD, and to analyze the relationship between the deterioration of cardiac function and skeletal muscular function. The cardiac function was serially evaluated for 2-12 years in 34 patients with DMD. The systolic time intervals and echocardiography were used for evaluating the cardiac function. The PEP/ET (pre-ejection period/ejection time) of systolic time intervals significantly deteriorated in 9-12 years (p less than 0.001). The maximal systolic and diastolic endocardial velocity (MSEV and MDEV) and the D/S ratio of echocardiography significantly deteriorated in 5 years (p less than 0.001). The D/S ratio is the ratio of the mean systolic velocity and the mean diastolic velocity of the left ventricular posterior wall. In almost half the patients, the deterioration of these parameters was correlated with that of Swinyard's stage. The Swinyard's functional ability stage demonstrates the grade of the skeletal muscular dysfunction. However, there was no correlation between the deterioration of cardiac function and Swinyard's stage in the other half of the patients. Correlation coefficient in the whole group was 0.32 (p = n.s.) between the PEP/ET and Swinyard's stage, and was -0.15 (p = n.s.) between the D/S ratio and Swinyard's stage. Although the mean left ventricular ejection fraction calculated using echocardiography was slightly decreased for 5 years, no significant change was detected in the whole group. One patient had progressive deterioration of left ventricular function despite mild skeletal muscular dysfunction, and died from congestive heart failure. The cardiac dysfunction was predominant. We named it 'cardiac type' DMD. Since deterioration of cardiac function was not always correlated with deterioration of skeletal muscular function (Swinyard's stage), cardiac function must be carefully monitored in patients with DMD.
Jpn Heart J 1990 Sep
PMID:Long-term observation of cardiac function in Duchenne's muscular dystrophy. Evaluation using systolic time intervals and echocardiography. 227 53

Enzyme histochemistry and acridine orange (AO) fluorescence techniques were used for studying muscle biopsy specimens of progressive muscular dystrophy in 75 cases. Five characteristic pathologic patterns for diagnosis were summarized. The level of serum CPK was be used as a marker for judging necrotic fibers. The result of AO staining showed that the number of regenerating IIc type fibers in DMD increased by 5-20%. This indicates that the numbers of the IIc type fibers are also related to necrotic fibers. The authors consider that the regenerative course is a compensatory repair reaction on necrotic fibers. But clinically, the speed of necrosis development is much higher than that of regeneration. Thus, enhancing the synthesis of proteins and promoting the capacity of regeneration should be considered as a new approach to effective therapy for DMD patients.
Zhonghua Bing Li Xue Za Zhi 1990 Sep
PMID:[Pathohistology of progressive muscular dystrophy and the relationship between necrotic and regenerative fibers]. 227 5

Variations in the content and translatability of the poly(A)+ RNA and mRNA molecules coding for myosin (M) were studied in the hind leg muscles of genetically dystrophic mice. The poly(A)+ RNA content of total skeletal muscle failed to increase normally during progression of the disease. M mRNA, isolated from dystrophic normally during progression of the disease. M mRNA, isolated from dystrophic murine muscle poly(A)+ RNA, was mostly found to be associated with the 26S RNA species. The translation of M mRNA in an in vitro heterologous wheat germ system was lower at 8 and 16 weeks in the dystrophic group as compared with the controls. Analysis of the translation products via sodium dodecyl sulfate-polyacrylamide gel electrophoresis, autoradiography, and densitometric autoradiographic tracing demonstrated the gradual disappearance of a protein band corresponding to M, the major component of skeletal muscle. cDNA was synthesized, using M mRNA that was isolated and purified from normal and dystrophic mouse muscle as a template. Total radioactivity was measured in some cDNA fractions produced from normal and dystrophic mouse muscle, while other fractions were utilized for separation and sizing of cDNA by disc gel electrophoresis. The cDNA from normal muscle was hybridized with M mRNA from normal and 16-week-old dystrophic mouse muscles. The cDNA probe, hybridization experiments, and studies involving the content and synthesis of M mRNA suggest that murine muscular dystrophy elicited a shorter species of mRNA or shorter sequences of the same species of mRNA coding for M. Not all poly(A)+ mRNA sequences coding for M, found in control mice, were present in their dystrophic counterparts.(ABSTRACT TRUNCATED AT 250 WORDS)
Biochem Cell Biol 1987 Sep
PMID:Biochemical changes in progressive muscular dystrophy. XIV. Skeletal muscle myosin mRNA translatability in dystrophic mice. 244 99

The genetic etiology, pathophysiology, natural progression, prognosis, and complications of Landouzy-Dejerine facio-scapulo-humeral benign muscular dystrophy are presented, as well as the risks of anesthesia and surgery. Patients with relatively benign myopathies may be candidates for reconstructive orthognathic surgery to improve oral function, facial esthetics, social interchange, and general quality of life. The surgery can be a gratifying experience for the patient, the family, and the surgeon, but the procedure, the anesthesia, and the postoperative care must be carefully planned to minimize the genuine risks.
J Oral Maxillofac Surg 1989 Sep
PMID:Correction of facial-skeletal deformities in two patients with facio-scapulo-humeral muscular dystrophy. 252 95

The degree of DNA-polyploidy of cardiac muscle cells was investigated by cytofluorometry in 4 cases of progressive muscular dystrophy (DMP) and 2 cases of myotonic dystrophy (DM), and the results were compared with those for non-dystrophic hearts of normal or increased weight. The heart muscle cells from all patients with these forms of dystrophy showed marked nuclear DNA hyperploidy reaching 16c, irrespective of cardiac hypertrophy or atrophy. In the non-dystrophic group, DNA hypertrophy corresponding to that of dystrophy was only detected in cases of marked cardiac hypertrophy exceeding a weight of 400 g. The wasting of the respiratory musculature, deformity of the thorax and cardiac muscular atrophy appeared to be the principal factors causing DNA polyploidy in patients with muscular dystrophy.
Acta Pathol Jpn 1989 Sep
PMID:Cytofluorometric determination of nuclear DNA in heart muscles of patients with muscular dystrophy. 253 66

In this report we describe the use of dystrophin analysis both in the diagnosis of Duchenne muscular dystrophy (DMD) in an aborted fetus and in genetic counseling. Our consultand's initial carrier risk, as based on family history and creatine kinase determinations, was calculated as 0.6%. DNA analysis of her family (and fetus) modified this risk to 8.5%. Skeletal muscle of the 23-wk male abortus was found to be histologically indistinguishable from that of age-matched controls. However, immunoblot testing for dystrophin indicated that the fetus had indeed inherited dystrophin deficiency. The carrier risk of the consultand was thus elevated to 100%. Dystrophin assays should be employed whenever the diagnosis of fetal DMD is equivocal (e.g., cases in which a gene deletion cannot be identified). Assay results are crucial for genetic counseling for subsequent pregnancies and for studies of the early pathogenesis of muscular dystrophy.
Am J Hum Genet 1989 Sep
PMID:Dystrophin analysis in duchenne muscular dystrophy: use in fetal diagnosis and in genetic counseling. 267

Inhibition of the enzyme, acetylcholinesterase (AChE), at the neuromuscular junction by pyridostigmine (PYR) results in breakdown of the postjunctional folds and dissolution of the Z-discs. It is hypothesized that excess activation of the acetylcholine (ACh) receptors by unhydrolyzed ACh results in a large influx of calcium ions. This could possibly lead to the activation of calcium-dependent proteases, resulting in the observed myopathy. Pretreatment with the calcium channel blocker, diltiazem, followed by administration of both PYR and the calcium blocker resulted in a significant reduction in the extent of muscle damage due to PYR alone. In order to ascertain whether the calcium blocker could reverse the myopathy previously induced by PYR, the AChE inhibitor was administered first, resulting in significant muscle damage, followed the next day by diltiazem. After 7 days of diltiazem treatment, with continued administration of PYR, the calcium blocker significantly reduced the myopathy at the neuromuscular junction. The results are discussed in terms of possible clinical application of diltiazem in neuromuscular diseases (i.e. muscular dystrophy).
Brain Res 1989 Sep 11
PMID:Calcium channel blocker reverses anticholinesterase-induced myopathy. 279 Apr 49

beta-Endorphin and alpha-melanotropin immunoreactivity was demonstrated in some motor nerves in histological sections of soleus and extensor digitorum longus muscles of the mouse. In two strains (C57BL/6J and 129/ReJ) which can inherit muscular dystrophy, the proportion of immunoreactive nerves was greater in the dystrophic individuals than in their healthy littermates. At 24 h after section of the sciatic nerve in the normal mice the proportion of immunoreactive nerve profiles had increased significantly in the denervated muscles even though most of the nerve axons had degenerated. Similar increases were also seen in nerves in the unoperated contralateral muscles.
Neurosci Lett 1988 Sep 23
PMID:Effect of nerve section on beta-endorphin and alpha-melanotropin immunoreactivity in motor nerves of normal and dystrophic mice. 284 88

In support of the widely held belief that membrane defects are present in the muscular dystrophies, alterations have been found in some transport-related enzymes of cells from affected donors. Cell membranes were isolated from cultured dermal fibroblasts of victims of myotonic muscular dystrophy, and of Duchenne's muscular dystrophy, and from cells of normal age- and sex-matched donors. Myotonic cells had an elevated Na+, K+ ATPase. gamma-Glutamyl transpeptidase was elevated in Duchenne cells. Among all cells' 5'nucleotide phosphatase exhibited a remarkably constant specific activity.
Life Sci 1985 Sep 09
PMID:Transport enzymes in the cell membranes of cultured fibroblasts; alterations in dystrophic cells. 286 29


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