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C Autore

Publications and source records attributed to C Autore.

35 records · Page 2Linked to original sources

Autoantibodies in myotonic dystrophy.

We evaluated some immunological parameters in a group of 24 patients affected with myotonic dystrophy (MD). IgG, IgA, IgM immunoglobulin serum levels resulted decreased in most of the patients. Anti-smooth-muscle antibodies have been found in 10 out of 24 patients (45.5%). Moreover in some of them decreased C3 and C4 complement fraction serum levels have been found. Our data confirm the existence of some immunological abnormalities in a great number of subjects affected with the disease. Besides, these data evidence for most of the subjects the presence of autoantibodies non-organ-specific direct against myocellular antigens. These autoantibodies could be considered the expression of suffering of muscular fibres.

Adolescent↗

Antimitochondrial autoantibodies in myocardial hypertrophy: comparison between hypertrophic cardiomyopathy, hypertensive heart disease, and athlete's heart.

Antimitochondrial autoantibodies (AMA) were tested by indirect immunofluorescence in three groups of subjects with different types of myocardial hypertrophy: 35 patients affected with hypertrophic cardiomyopathy (HC), 20 patients with cardiac hypertrophy secondary to essential hypertension, and 35 active endurance athletes with exercise-induced left ventricular hypertrophy. Forty-two healthy subjects served as a control group. Left ventricular hypertrophy was considered a left ventricular mass (LVM) echocardiographically calculated (Devereux formula), exceeding 244 gm or a LVM index exceeding 122 gm/m2 (greater than 2 SD from a previously studied normal population). AMA were found in 15 of 35 (43%) patients with HC and in 6 of 20 (30%) patients with hypertensive heart disease (p less than 0.01); in contrast, AMA were not present in the sera of athletes or in the sera of controls. Although the significance of AMA in subjects with pathologic myocardial hypertrophy has not yet been established, their absence in the sera of athletes strengthens the opinion that cellular changes, as a compensatory response of the myocardium to a work overload, have a physiologic fashion in these cases. Moreover, identification of AMA in the sera of athletes with disproportionate severe left ventricular hypertrophy of uncertain origin may be helpful to ensure a single diagnosis.

Adolescent↗

Equivocal and borderline myocardial hypertrophy in relatives of patients with hypertrophic cardiomyopathy: possible implications in genetics of the disease.

To determine the occurrence of familial and sporadic forms of hypertrophic cardiomyopathy (HC) 74 first-degree relatives of 21 patients with proven HC were studied by M-mode and two-dimensional echocardiography. A diagnosis of HC was made in 11 relatives (15%) while it was excluded in 61 of them (82%); 2 subjects (3%) were considered neither affected nor unaffected (borderline left ventricular hypertrophy suggestive of HC). Inspection of pedigrees revealed 38% of familial forms of HC with an autosomal dominant pattern of inheritance in 5/8 families (62%). Furthermore, among those relatives judged unaffected by means of full echocardiographic criteria for HC, an attempt was made to find out whether minor changes of left ventricular geometry were present for their possible implications in genetics of HC (latent or potential forms, low phenotypic expression of the disease). Eleven out of 61 unaffected relatives had a left ventricular wall thickness radius ratio greater than 0.50 (equivocal hypertrophy), a value that was higher than two standard deviations of the control group. Assessment of clinical significance of borderline and equivocal hypertrophy in relatives of patients with HC is required for a better understanding of genetic transmission of this disease. In this view the occurrence of sporadic and familial forms of HC might be revisited.

Adolescent↗

Clinical relevance of the electrocardiogram in relatives of patients with hypertrophic cardiomyopathy.

Eighty-nine first-degree relatives of 22 patients with an established diagnosis of hypertrophic cardiomyopathy underwent electrocardiographic and echocardiographic screening. Scalar electrocardiogram was abnormal in 30/89 (33.7%) relatives. Of these thirty, eleven had definite evidence of hypertrophic cardiomyopathy at echo; one had borderline hypertrophy and was considered neither affected nor unaffected; four had questionable signs of hypertrophy. The remaining 14 relatives had normal echo-cardiograms. Fifty-nine relatives (66.3%) had normal electrocardiograms; at echo 3 were considered to have borderline hypertrophy, 16 had questionable signs of hypertrophy and 40 were normal. In relatives of patients with hypertrophic cardiomyopathy an abnormal electrocardiogram may reflect different morphologic conditions: a real hypertrophic cardiomyopathy or a myocardial hypertrophy of uncertain significance. Furthermore, in these categories of subjects, an abnormal electrocardiogram with normal echo must be considered with caution.

Adult↗

Ambulatory electrocardiographic monitoring in myotonic dystrophy (Steinert's Disease). A study of 22 patients.

Ambulatory electrocardiographic monitoring (AEM) was performed in 22 patients (range 13-62 years; mean age 38.2 +/- 12.7) with grades I, II and III of myotonic dystrophy in order to evaluate the occurrence of potentially dangerous cardiac arrhythmias and conduction disturbances. All patients had previously undergone echocardiographic examination to determine whether structure and function abnormalities were present. In 6 patients with normal resting electrocardiogram, AEM revealed: first degree A-V block (4 cases), class IVa Lown ventricular arrhythmias (3 cases) and episodes of atrial fibrillation (4 cases). In 2 of 3 cases with abnormal scalar electrocardiogram new abnormalities (first degree A-V block and further prolongation of P-R interval) were demonstrated by AEM. Only 1 patient had mild signs of left ventricular dysfunction at echo. Disorders of cardiac conduction and rhythm are characteristic of myotonic dystrophy and can predispose to severe cardiac events. In this respect AEM is shown to be an early and sensitive tool in identifying patients at risk.

Adolescent↗

HLA-DR3 antigen linkage in patients with hypertrophic obstructive cardiomyopathy.

In order to investigate if genetic factors could be involved in the pathogenesis of hypertrophic obstructive cardiomyopathy, we determined HLA-A, HLA-B, HLA-C, and HLA-DR specificities in 12 Italian patients affected with the disease and in healthy family members of one of them. HLA-DR3 was found in 50% of patients as compared to 17.1% of normal control subjects (p = 0.023, relative risk = 4.82). The two relatives also had HLA-DR3 antigen and, in addition, showed equivocal signs of hypertrophy at echocardiographic examination. Thus hypertrophic obstructive cardiomyopathy is associated with genes in the HLA-DR region, and immunogenetic factors could be involved in the pathogenesis of the disease. Furthermore, the minimal target organ abnormalities in "healthy" relatives could represent a subclinical stage of the disease.

Adult↗

[Contribution of M-mode echocardiography and myocardial scintigraphy for study of ECG pattern of left ventricular hypertrophy with giant negative T waves (author's transl)].

Eight normotensive patients with electrocardiographic criteria for left ventricular hypertrophy with giant negative T waves were studied with Thallium-201 imaging and M-mode echocardiography. In all the patients Thallium scanning demonstrated increased thickness of the left ventricular walls. In five of the above cases areas of increased uptake were noted in the apical region which had increased thickness as compared to the rest of the left ventricular wall. Echocardiography showed in one subject obstructive hypertrophic myocardiopathy and in another two asymmetric septal hypertrophy. In the remaining patients there was always present septal and posterior wall hypertrophy. Reliable echocardiograms of the apex were done in five subjects and in these hypertrophy was noted. The results of the two techniques were compared and correlated clinically and with the literature. The authors conclude in agreement with other studies that the picture of electrocardiographic left ventricular hypertrophy with giant negative T waves is indicative of hypertrophic myocardiopathies. Specifically, for us, the apical hypertrophy may be the only feature of the myocardiopathy or be part of a generalized left ventricular hypertrophy which is usually asymmetric septal hypertrophy.

Adult↗

[On the genesis of the first heart sound: phono-echocardiographic study in patients with A-V block (author's transl)].

A phono-echocardiographic study of acustic and morphologic events was performed in three patients with atrioventricular block in order to assess the role of the mitral valve in the changes of the amplitude of the first heart and, more generally, in the genesis of the first heart sound. Simultaneous recording of the electrocardiogram, the apical phonocardiogram and the mitral echocardiogram showed: 1) the coincidence between the C point of the echocardiogram and the onset of the earlier high frequency vibrations of the first heart sound (M1); 2) a close correlation between the intensity of the first heart sound and the position of the mitral valve at the onset of ventricular systole (P less than 0.001); 3) longer duration of the first heart sound in those beats when there was superimposition of P wave in QRS. The authors illustrate the recent reports about the genesis of the first heart sound and emphasize the main role of the mitral valve suggesting that the position of the mitral leaflets at the onset of ventricular systole influences the mechanism of acceleration and deceleration of blood and vibrations of the "cardiohemic system".

Adult↗