PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “PROGRESSIVE MUSCULAR DYSTROPHY”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 631 records · Page 35Linked to original sources
PubMed1978

[Staircase phenomenon in children with progressive muscular dystrophy and dermatomyositis].

The electrical and mechanical activity of the isometric twitch of flexor carpi ulnaris muscle during two per second indirect supramaximal stimulation for 90 sec was examined in 14 children with muscular distrophy and 8 children with dermatomyositis. The muscle electrical responses show no significant changes in the amplitude of its first negative phase. The first derivative of the dinamogram shows some of the following abnormalities in 8 of the examined children with muscular distrophy and in 2 of these with dermatomyositis: 1. Prolonged and increased negative staircase; 2. Insufficient or absent positive staircase potentiation. These abnormalities of the staircase phenomenon disclose disorders of the contractile function of the examined muscle.

Adolescent↗
PubMedDate not supplied

[Cyclic 3',5'-AMP metabolism in progressive muscular dystrophy patients].

Concentration of cyclic AMP was distinctly decreased in muscular tissue of patients with Dushenn's myodystrophy. Simultaneously, in patients with the myopathy basal activity of adenylate cyclase as well as activation of the enzyme by adrenaline and sodium fluoride were decreased. As a compensation, activity of phosphodiesterase was also decreased. Content of cAMP, activity and activation of adenylate cyclase were similar to normal state in patients with Erb's and Landuzi--Dejerin's myopathies; activity of phosphodiesterase was increased in the latter impairment. Dissociation constant for binding of cAMP by protein kinases was more than 10-fold decreased in patients with these types of myopathy.

3',5'-Cyclic-AMP Phosphodiesterases↗
PubMed1990

[Epidemiology and clinical polymorphism of the humero-scapulo-facial form of progressive muscular dystrophy].

The paper is concerned with a clinico-genealogic analysis of 9 patients with the humeroscapular form of Landouzy-Dejerine myodystrophy. The patients come from 4 families united by the common ancestor. The following aspects of the illness are marked: a mild course and considerable clinical polymorphism manifesting itself in the age difference by the disease onset as well as in the intensity of the clinical symptoms. These characteristics are of practical importance for regional medicogenetic counselling.

Adult↗
PubMed1979

[Pathogenesis of several forms of progressive muscular dystrophy].

The level of cyclic 3,5-adenosine monophosphate (cAMP), the activity of adenylate cyclase (AC) and phosphodiesterase (PDE) were studied in bioptates of the muscles of 17 patients with different forms of myopathy. The cAMP level and AC activity were decreased while that of PDE was increased. These changes are particularly demonstrative in patients with Erba type of myopathy. It is recommended that beta-adrenomimetic drugs and PDE inhibitors may be tried in pathogenetic therapy.

3',5'-Cyclic-AMP Phosphodiesterases↗
PubMed1979

Progressive muscular dystrophy type Duchenne. I. Spin label studies on the physico-chemical state of erythrocyte membranes.

Electron spin resonance studies of erythrocyte membranes from patients with Duchenne muscular dystrophy exhibit changes in the physical state of lipids and proteins in membranes when compared to membranes from normal subjects. The results suggest that the alterations in membrane lipid-protein organization are present in this disease.

Electron Spin Resonance Spectroscopy↗
PubMed1990

[Selenium and vitamin E in patients with progressive muscular dystrophy].

Serum levels of selenium and vitamin E were prospectively studied in children with Duchenne de Boulogne muscular dystrophy of variable age and muscular status. In contrast with previous studies, we found no differences with controls. However, we believe that selenium and vitamin E, two natural antioxydants, may contribute to the pathophysiology of pseudohypertrophic muscular dystrophy. A study of the effects of supplementation is on-going.

Adolescent↗
PubMed1988

Biochemical changes in progressive muscular dystrophy. XV. Distribution of radioactive glutamate and proximate composition of various components of skeletal muscle and liver in vitamin E-deficient dystrophic rabbits and 129/ReJ (dy/dy) mice.

The proximate composition of skeletal muscle and liver and the incorporation of radioactive glutamate into their various components were investigated in vitamin E-deficient rabbits and genetically dystrophic (dy/dy) mice during various stages of the disease. Total fat content in skeletal muscle was decreased in the early phase and increased in the terminal stage of both types of dystrophy. This component altered only in the liver of dystrophic mice, showing a reduction in the mild stage but returning later to normal values. Carbohydrate content was diminished in the skeletal muscle of both species at each stage of the disease. In the liver, however, it was elevated considerably in vitamin E-deficient rabbits, but was depressed in dystrophic mice. Protein content was decreased in the skeletal muscle of both dystrophic models. Similar results were obtained for amino acids, except that they were increased in the terminal stages of nutritional dystrophy. Analogous values were recorded for hepatic amino acids in nutritionally-induced and hereditary dystrophy (in rabbits and mice respectively). Incorporation of C14-glutamate into various components of skeletal muscle and liver in both models revealed divergent responses, depending upon the nature of the dystrophy, and the component in which incorporation was measured. These data indicate a rapid turnover of C14-glutamate in the skeletal muscle and liver of vitamin E-deficient and genetically dystrophic animals. Also, it is clear that the changes in the various components of skeletal muscle and liver, as well as the pattern of C14-glutamate incorporation, are different in the two types of dystrophy.

Amino Acids↗