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Biomedical subjects

L Dembo

Publications and source records attributed to L Dembo.

10 recordsLinked to original sources

Rare variant of truncus arteriosus with intact ventricular septum and hypoplastic right ventricle.

A three week old girl was admitted to hospital with severe congestive heart failure and cyanosis. Cross sectional and Doppler echocardiography and cardiac catheterisation showed a unique variant of truncus arteriosus with an intact ventricular septum. The trunk rose only from the left ventricle and was associated with a hypoplastic right ventricle with sinusoids to the right coronary artery.

Cardiac Catheterization

[Echocardiographic diagnosis of anomalous origin of left coronary artery].

Cross sectional and M-mode echocardiography is a well-established noninvasive technique in the diagnosis of dilated cardiomyopathy and is essential in the evaluation of its severity and progress. However, in most cases of dilated cardiomyopathy in children, it does not provide data suggestive of the etiology of the disease. In the rare congenital malformation, anomalous origin of the left coronary artery from the pulmonary artery, the echocardiographic techniques may suggest the diagnosis when the left coronary artery cannot be demonstrated to arise from its usual site, the left coronary sinus (as visualized in the short axis of the aortic root). A firm diagnosis can be made if the anomalous origin of the left coronary artery is identified in a cross-sectional view of the main pulmonary artery (left parasternal long axis view of the main pulmonary artery, Fig. 1). This was demonstrated in a 9-year-old girl by coronary and aortic angiography (Fig. 2a, b). Anatomical confirmation was made during corrective surgery 3 months later. The origin of the left coronary artery was at the left posterior aspect of the main pulmonary artery, just proximal to its bifurcation. This is an unusual origin in this anomaly in which the coronary artery has been reported to arise close to the pulmonary valve, but not distal, as in this case.

Child

Early detection of infantile endocarditis by gallium--67 scintigraphy.

An infant with suspected soft tissue infection of the knee was studied by 67Ga-scintigraphy. In addition to knee and hip joint increased activity, heart uptake was also demonstrated prior to the development of clinical signs of endocarditis. The early detection and treatment resulted in satisfactory clinical resolution.

Endocarditis, Bacterial

The scale of myocardial involvement in varicella myocarditis.

Two patients with varicella myocarditis are described. An arrhythmia associated with complete recovery occurred in the first patient whereas intractable congestive heart failure complicated by hemiplegia resulted in a fatal outcome in the other case. We stress the extent of myocardial involvement produced by the herpes zoster virus in the setting of varicella.

Cardiac Output

Hepatomegaly following short-term high-dose steroid therapy.

Children treated with large doses of corticosteroids were found to develop hepatomegaly within a few days. No relationship could be established between the condition for which steroids were given and the liver enlargement. Liver biopsy was thought to be indicated, and thus was performed in three children because of diagnostic uncertainty. The light and electron microscopic examinations revealed normal liver architecture, without edema, sinusoid engorgement, or inflammatory changes. The hepatocytes were distended by increased amounts of glycogen. In a pattern reminiscent of some glycogen storage diseases, mitochondria and other cytosol components were displaced toward the cell membrane or around the nucleus, which occasionally contained glycogen. Moderate sinusoidal compression, interhepatocytic free glycogen particles, and mild increase in lipid droplets were also found. It is concluded that the hepatomegaly noted in patients given short-term, high-dosage steroid therapy is due to excessive glycogen accumulation within parenchymal cells. This finding is in accordance with observations in animals, as well as with biochemical studies demonstrating hepatocytic glycogen deposition after steroid therapy. Being benign and reversible, early hepatomegaly following administration of high-dose corticosteroids should not influence the initial therapeutic plan required by the basic disease.

Adolescent

Sinus node dysfunction in a healthy pediatric population.

ECG recordings of 624 healthy children (age range 6 to 12 years) from a rural population were analyzed for evidence of sinus node dysfunction. Twelve children were found to have the disorder and they underwent further assessment in order to establish any etiological factors or anatomical abnormalities. All the children were asymptomatic and physical examination was completely normal. Standard ECG taken during 24-h monitoring demonstrated that the most common finding, seen in 10 of 12 patients, was that of sinus arrest. Second-degree sinoatrial exit block, Mobitz type I, occurred in four children and Mobitz type II was seen in three. Two of the children were found to have holosystolic mitral valve prolapse, which was in the normal frequency range for a population of healthy children. After a 2-year follow-up and reassessment, there was no change in the symptomatology, the ECG tracings or the physical findings of any of the children.

Arrhythmia, Sinus