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The distribution of inside-out and right-side-out erythrocyte membrane vesicles in Duchenne progressive muscular dystrophy.

In ten cases of Duchenne muscular dystrophy, the distribution of erythrocyte ghost vesicles in dextran 110 gradient was examined. When compared with controls a greater number of inside-out vesicles was observed. It is suggested that the tendency to form abnormally oriented vesicles could result from structural abnormalities of the erythrocyte membranes.

Erythrocyte Membrane↗

Intelligence level of patients with the Duchenne type of progressive muscular dystrophy (pmd-d).

The I.Q. of 129 patients with PMD-D and 27 patients suffering from Werdnig-Hoffmann disease were estimated. Among the patients with PMD-D there was one group without any complicating factors and 3 other groups with additional factors that might influence the intelligence level. Comparing mean values and distribution of I.Q. for all these groups, one can conclude that, besides additional unfavourable pathological and environmental factors in all cases, PMD-D itself causes a small decrease of the I.Q. by about 1 SD. The frequent changes of the EEG record in these patients could reflect involvement of the CNS by the pathological process.

Adolescent↗

Fluorescent probe analysis of muscle plasmalemma in Duchenne's progressive muscular dystrophy.

The relationship between fluorescence intensity and binding of 1-anilino-naphthalene-8-sulphonate (ANS) to muscle plasmalemma in patients with Duchenne's muscular dystrophy (DD) and controls was studied. The fluorescence of ANS was markedly enhanced in DD as compared with controls. The magnitude of this enhancement was increased by monovalent and divalent cations; treatment of DD plasmalemma with trypsin caused an opposite effect. Treatment with phospholipase A and C altered the ANS fluorescence in DD and controls equally. These findings may indicate an increase of the hydrophobic character in the apolar-polar interface of DD plasmalemma. The relationship of these changes to a lack of dystrophin in DD remains to be established.

Adolescent↗