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PubMed1996

[Selenium concentration in blood and Duchenne-type progressive muscular dystrophy].

The concentration of selenium (Se) and the activity of glutathione peroxidase (GSH-Px) in plasma and erythrocytes were measured in healthy men and in patients with Duchenne-type progressive muscular dystrophy (DMD). In healthy men, the Se concentration in erythrocytes showed a steep rise with aging and ascended gradually in plasma. The GSH-Px activity in both plasma and erythrocytes clearly increased with aging. The relationship between the Se concentration and the GSH-Px activity in healthy men showed a parallel rise with aging, but the coefficients of correlation were not very high (r = 0.44 and 0.56 in plasma and erythrocytes, respectively. In DMD patients, on the other hand, the Se concentration in erythrocytes decreased steeply with aging, and it decreased gradually in plasma. The GSH-Px activity in both plasma and erythrocytes apparently increased as in healthy men with aging, but the level was about 80% of that of healthy men. When selenite (Se+4) is added to the whole blood in vitro at 25 degrees C, it is rapidly taken up by erythrocytes (within several minutes) and is then released into plasma (a period of 30 min), then subsequent reuptake by erythrocytes is proceeded slowly. Our attention was attracted to the pattern of selenite release from erythrocytes of DMD patients.

Adolescent↗

PubMed2002

Plasma levels of natriuretic peptide and echocardiographic parameters in patients with Duchenne's progressive muscular dystrophy.

We investigated the relationship between plasma atrial natriuretic peptide (ANP) and brain natriuretic peptide (BNP) levels and systolic and diastolic cardiac function, determined by echocardiography, in 63 patients with Duchenne's progressive muscular dystrophy (DMD) (age range 8-21 years). The relationship between shortening fraction of the left ventricle and ANP and BNP levels was curvilinear rather than linear: When the shortening fraction was >15%, increases in ANP and BNP levels were minimal. However, if the shortening fraction was <15%, both natriuretic peptide levels increased dramatically. Stepwise regression analysis revealed that only the deceleration time of the early diastolic filling wave predicted plasma BNP concentration among various diastolic echocardiographic parameters determined by mitral flow. Three patients died of cardiac dysfunction during a 2-year follow-up period. These patients had a severely decreased deceleration time (<65% of normal) in association with increases in both natriuretic peptide levels. In conclusion, plasma ANP and BNP levels are not sensitive markers for the early detection of cardiac systolic dysfunction in patients with DMD. However, in patients with systolic dysfunction, an increase in the concentrations of these peptides, associated with a decrease in the deceleration time of early diastolic filling, suggests poor prognosis.

Adolescent↗

PubMed1982

Histopathological study of the conduction system of the heart in Duchenne progressive muscular dystrophy.

The cardiac conduction systems including sinoatrial (SA) node, atrioventricular (AV) node, atrioventricular(His) bundle, and peripheral conduction system (left and right bundle branch, and Purkinje fiber) of 23 patients with Duchenne progressive muscular dystrophy(DMD) were studied with light microscope. Infiltration of fat tissue and mild fibrosis were occasional findings in SA and AV nodes. Degeneration of the conduction muscle fiber was hardly noted in SA node, AV node, and His bundle. Only the peripheral conduction system (Purkinje fiber) showed significant degenerations such as eosinophilic, necrotic and vacuolar changes with fibrosis. These necrobiotic changes resembled hyaline and vacuolar skeletal and cardiac muscular degenerations in DMD and were assumed to have occurred on the basis of the structural and constitutional characteristics of the peripheral conduction fiber as a striated muscle fiber. The vascular changes and amyloid deposit suggesting precocious aging in the conduction systems were not observed.

Adolescent↗

PubMed1981

[Regional wall motion of the left ventricle in congestive cardiomyopathy: in comparison with progressive muscular dystrophy of Duchenne type (author's transl)].

The features of regional wall motion abnormalities of the left ventricle were analysed in 11 patients of congestive cardiomyopathy (CCM) in comparison with 22 patients of progressive muscular dystrophy (DMD) of Duchenne type who showed an abnormal motion of the left ventricle by echocardiography. Real time two-dimensional echocardiographic study demonstrated the following results: I) In CCM, (1) only 2 or less of 11 cases preserved a normal motion in each left ventricular segment, and the depression of wall motion of the left ventricle were thought to be generalized; (2) there were 9 cases with segmental wall motion abnormalities and 3 of them demonstrated ventricular aneurysms, and (3) the localizations of the segmental abnormalities varied in each case, and there was no apparent accumulation to any segments. II) In DMD, (1) all the cases showed depressed motions and 8 of them demonstrated a ventricular aneurysm in the posterior wall of the left ventricle (LVPW), (2) while, there was no case showing ventricular aneurysm in the segments other than LVPW, and about one third of all cases showed normal motion in those segments. From these results, we concluded as follows: 1) Although the depression of a wall motion of the left ventricle was generalized in CCM, this was not always uniform and the segmental abnormalities of a wall motion were frequently observed. The localization of the most severely disturbed segment varied in each case. 2) On the other hand, in DMD, the wall motion was disturbed more frequently and more severely in LVPW than in other ventricular segments.

Adolescent↗

PubMed1993

[Molecular genetic analysis of deletions in the Duchenne and Becker types of progressive muscular dystrophy].

Methods of molecular genetics (Southern's hybridization and DNA amplification by the PCR method) were used to search the DNA of patients suffering from the Duchenne (DMD) and the Becker (BMD) type of progressive muscular dystrophy for deletions in the dystrophin gene. The series consisted of 29 patients with DMD and 2 patients with BMD. As hybridization probes cloned cDNA sections were used designated as CF56a, CF56b, 1-2a, 2b-3, 4-5a, 5b-7 and 8. With the PCR methods means for exons 8, 19, 45 and 48 were used. No deletion was found in either of the BMD patients. In 13 (44.8%) of the 29 DMD patients deletion with at least one cDNA probe was found. Most deletions were detected with the probes 8 (46.2%) and 1-2a (30.8%). The high proportion of deletions in the etiology of DMD/BMD has both a high differential diagnostic value and allows to make direct prenatal diagnosis as well as to determine transmission in these families with subsequent elimination of the risk of diagnostic error resulting from recombination in DNA diagnosis by means of binding. (Tab. 1, Fig. 2, Ref. 20.)

Chromosome Deletion↗

PubMed1982

Sequential changes in high frequency notches on QRS complexes in progressive muscular dystrophy of the Duchenne type--a 3-year follow-up study.

In a 3-year follow-up study, sequential changes in the number of high frequency notches (HFN) on QRS complexes were investigated on 72 cases with progressive muscular dystrophy of the Duchenne type (PMD). The patients were classified into eight stages from the mildest, S(1), to the most severe, S(8), according to Swinyard-Deaver's criteria. The number of HFN progressively increased with advancing Swinyard-Deaver's stage. In the terminal stage, however, the number of HFN tended to decrease. The present observations show that the patient tends to have a small number of HFN in the early stages of the disease; with increasing severity of the disease he develops a larger number; in the terminal stage he again exhibits relatively few notches. It is concluded therefore that significant increases or decreases in the count of HFN on QRS complexes can be useful indicators for estimating the extent and severity of cardiac involvement in PMD.

Adolescent↗

PubMed1954