Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Ths dissociation of gross congestive cardiomyopathy and muscle disease in different members of a family affected with Becker's benign X-linked muscular dystrophy is described. The possibility that cardiomyopathy and muscular dystrophy may be the expressions of the same mutant gene has been suggested.
 ...
PMID:Congestive cardiomyopathy in a family of Becker's X-linked muscular dystrophy. 56 17

(1) Narrowness of the early-diastolic and presystolic peaks on the echo curve of the anterior mitral leaflet and an abnormal step formation at the middle or higher level on the descending limb of the presystolic peak were observed in 7 cases with congestive cardiomyopathy or myocardial fibrosis, 1 case with myocardial infarction, and 1 case with cardiomyopathy due to progressive muscular dystrophy. (2) This abnormal pattern is considered to be closely related to the myocardial condition in the above-mentioned diseases. (3) Probable mechanisms for this pattern formation are considered as follows: (i) a sudden reduction of distensibility of the left ventricle after filling of the ventricle over a certain limit near full-filling, and impaired ventricular contractility, (ii) restriction in the mobility of the mitral valve and its chardae due to thier inability to adapt themselves to a developed dilatation of the left ventricle. (4) A similar presystolic step formation on the echo curve of the anterior mitral leaflet was observed in 3 of 25 cases of hypertrophic cardiomyopathy of Goodwin's sense. Differences between the myocardial state in congestive cardiomyopathy or in similar myocardial diseases and that in hypertrophic cardiomyopathy were also discussed.
 ...
PMID:An unusual pattern of the mitral echocardiogram observed in cases of congestive cardiomyopathy and other myocardial diseases. 117 35

The presence of myocardial involvement is rare in benign Duchenne type of progressive muscular dystrophy (Becker's muscular dystrophy). We describe two brothers suffering from Becker's muscular dystrophy, both of whom presented with dilated cardiomyopathy. The first case is a 39-year-old male who had suffered from gait disturbance from the age of 17. When 37 years old, he was found to have heart disease. When he first came to our hospital, pseudohypertrophy of the calves was present. Chest radiography, electrocardiogram, ultrasonocardiography and clinical feature indicated Becker's muscular dystrophy with dilated cardiomyopathy. The second case is the younger brother of the 37-year old male. He suffered from leg weakness. He came to our hospital with the chief complaint of discomfort of the anterior chest. Pseudohypertrophy of the calves was present. Chest radiography, electrocardiogram, ultrasonogram indicated dilated cardiomyopathy.
 ...
PMID:[The two brothers' case of dilated cardiomyopathy with benign Duchenne type of progressive muscular dystrophy (Becker's type)]. 157 Apr 30

Twenty patients with different types of muscular dystrophy (MD) were included in a cross-sectional study by means of electrocardiography and ultrasound cardiography. A manifest cardiomyopathy was detected in 8 patients; a latent cardiomyopathy was found in 4. A hypertrophic cardiomyopathy was especially frequent in facioscapulohumeral MD, a congestive cardiomyopathy in Becker-Kiener MD. The ECG showed a reduction in the QT interval and frequent block formers in the X-chromosomal inherited forms and the trunc-girdle form. Bradycardia and a prolonged QT interval were frequent in myotonic dystrophy and facioscapulohumeral MD. Signs of cardiac infarction in the ECG were most frequent in the trunc-girdle forms. A high cardiac output per minute in conjunction with increased left ventricular volume was frequent in Becker-Kiener and Landouzy MD. A left ventricular dysfunction with reduced ejection was characteristic of myotonic dystrophy and trunc-girdle MD. A mitral valve prolapse was more frequent with increasing severity of the muscle disease and was particularly frequent in myotonic dystrophic and Landouzy MD. The cardiac output per minute and the stroke volume were significantly lower (P less than or equal to 0.03) where a mitral valve prolapse was present.
 ...
PMID:The heart in muscular dystrophy: an electrocardiographic and ultrasound study of 20 patients. 179 Jan 64

The cardiomyopathies comprise a diverse group of illnesses that can be characterized functionally by several techniques. However, the delineation of derangements of regional perfusion and metabolism have been accomplished only relatively recently with positron emission tomography (PET). Regional myocardial accumulation and clearance of 11C-palmitate, the primary myocardial substrate under most conditions, demonstrate marked spatial heterogeneity when studied under fasting conditions or with glucose loading. PET with 11C-palmitate permits the noninvasive differentiation of patients with nonischemic from ischemic dilated cardiomyopathy, since patients with ischemic cardiomyopathy demonstrate large zones of intensely depressed accumulation of 11C-palmitate, probably reflecting prior infarction. Patients with hypertrophic cardiomyopathy and Duchenne's muscular dystrophy demonstrate relatively unique patterns of myocardial abnormalities of perfusion and metabolism. The availability of new tracers and techniques for the evaluation of myocardial metabolism (11C-acetate), perfusion (H2(15)O), and autonomic tone (11-C-hydroxyephedrine) should facilitate further understanding of the pathogenesis of the cardiomyopathies.
 ...
PMID:Metabolic imaging of patients with cardiomyopathy. 188 95

This study describes the late potentials (LPs) obtained by signal-averaged electrocardiography (SAECG) in 66 patients with Duchenne's progressive muscular dystrophy (DMD). It also assesses the possible relationships between LPs and the severity of DMD, and the findings of two-dimensional echocardiography, as well as ventricular arrhythmias examined with the Holter system. SAECGs were performed with a Marquette MAC-1 unit. Based on Swinyard-Deaver's system of stages, ranging from the mildest, S1, to the most severe, S8, one patient each could be assigned to S2 and S4, 6 to S5, 20 to S6, 21 to S7, and 17 to S8. LPs were observed in 21 of the 66 patients (32%), including 3 of the 20 assigned to S6 (15%), 10 of the 21 in S7 (48%), and 8 of the 17 in S8 (47%). The total wall motion index evaluated by the method of Hegar was significantly greater in the patients with LPs (8.4 +/- 4.4) than in those without LPs (5.8 +/- 3.1) (p less than 0.05). The incidence of LPs was found to be higher in the dilated cardiomyopathy (DCM) type (8 of 12;67%) than in the normal type (9 of 41;22%) (p less than 0.01). The incidence of ventricular premature complexes (VPCs) was significantly higher in patients with LPs (13 of 21;62%) than in those without LPs (13 of 45;29%) (p less than 0.05). No sustained ventricular tachycardia (VT) was observed, although nonsustained VT was noted in three patients with LPs. The LPs in patients with DMD were thus associated with left ventricular dysfunction, and the presence of LPs might be correlated with the extent of myocardial derangement in DMD.
 ...
PMID:Late potentials in progressive muscular dystrophy of the Duchenne type. 200 37

This is a clinical report of a rare case of Charcot-Marie-Tooth disease associated with dilated cardiomyopathy. A seventy-seven-year-old Japanese male first visited our outpatient clinic with a ten-year history of muscular weakness in his bilateral lower extremities and gait disturbance characterized by classical features of peroneal muscular atrophy and inverted champagne bottle legs. Biopsy findings of the m. quadriceps femoris and the n. gastrocnemius revealed clustered atrophy of myofibrils and segmental demyelinization mingled with remyelinization. Because of his other problem of dilated cardiomyopathy, he had been treated with salt restriction, digitalis, diuretics and vasodilators, until his third hospitalization, when he developed terminal stage of severe congestive heart failure. Despite our intensive cardiac care, the patient died because of profound pump failure. Autopsy findings disclosed a remarkably dilated left ventricular chamber and an increased total heart weight of 600 grams. Grossly, the cross sectional view of the left ventricle revealed diffuse, but not homogenous fibrosis that was most prominent in the posterior wall. On light microscopic examination, the left ventricular myocardium revealed diffusely scattered muscular degeneration interlaced with fibrosis. Although large epicardial coronary arteries revealed only mild intimal atheromatous thickening, most of the small intramuscular coronary arteries were free from atherosclerosis. Neither diabetic nor amyloid lesions could be detected. It has been well known that cardiomyopathy is often associated with various forms of muscular dystrophy and Friedreich's ataxia.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[Charcot-Marie-Tooth disease associated with dilated cardiomyopathy: an autopsy case report]. 204 12

A dilated cardiomyopathy with clear signs of left-ventricular functional abnormalities occurred in a 21-year-old man with known Duchenne's progressive muscular dystrophy. Echocardiography and magnetic resonance imaging delineated the regional disorder of ventricular wall motion and defined its segmental extent. With neither method was it possible to analyse texture with demonstration of differential regional involvement. As a noninvasive method echocardiography is suitable for diagnosing Duchenne's cardiomyopathy and monitoring its progression.
 ...
PMID:[Cardiomyopathy in progressive muscular dystrophy]. 220 35

A young woman with humeroperoneal muscular dystrophy and contractures received a heart transplant for a severe dilated cardiomyopathy. Cardiac histopathology consisted of myocyte hypertrophy, interstitial fibrosis, and nuclear hyperchromaticity without mitochondrial abnormalities. Myopathy and heart disease were not clinically evident in her family, although three relatives had unexplained shortened Achilles tendons without weakness. Tendon contractures may be a partial expression of this myopathic disorder, suggesting an autosomal dominant inheritance with variable penetrance. A muscular dystrophy clinically similar to that of the Emery-Dreifuss (EDMD) type can thus occur in women. Rather than the cardiac arrhythmias typical of EDMD, a dilated cardiomyopathy may occur and present with severe congestive heart failure. This is the first report of cardiac transplantation in such a case.
 ...
PMID:Cardiac transplantation in female Emery-Dreifuss muscular dystrophy. 223 Aug 49

A 42-year-old man with limb-girdle muscular dystrophy who showed prominent cardiac involvement is described. He was treated for and diagnosed with dilated cardiomyopathy before admission. Initially, no overt feature of muscular dystrophy was evidenced, but serum enzymes were elevated. During the 2-year follow-up period, distinct features of muscular dystrophy appeared, and muscle biopsy confirmed the diagnosis. He was regarded as experiencing a sporadic occurrence. Because some patients diagnosed with and treated for dilated cardiomyopathy may have similar results, this form of muscular dystrophy should be recognized as occasionally associated with cardiac muscle disease.
 ...
PMID:Marked cardiac involvement in limb-girdle muscular dystrophy. 235 53


1 2 3 4 5 6 7 8 9 10 Next >>