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Biomedical subjects

L Sieverding

Publications and source records attributed to L Sieverding.

46 records · Page 3Linked to original sources
PubMed1989

[Secondary diseases of the heart muscle and their differential diagnosis in childhood].

Possible causes of specific cardiac muscle disease, diagnosis, follow-up and the therapeutic management are discussed on the basis of a series of cases. 8 out of 30 patients who showed the clinical picture of dilative cardiomyopathy (DCM) were found to have a specific cardiac muscle disease. 4 patients had DCM following adriamycin therapy. Fibromuscular dysplasia with renal hypertension, thalassaemia major with secondary haemosiderosis, long-overlooked and untreated athyroidism each caused one case of dilative specific cardiac muscle disease. Once DCM was preceded by the Kawasaki syndrome for over 2 years. Amongst 47 patients with hypertrophic cardiomyopathy there were two children who had undergone ACTH treatment, 6 children born of diabetic mothers, 4 cases of Pompe's disease, and one patient with hypothyroidism resulting in reversible hypertrophy of the cardiac muscle. Different neurodegenerative diseases were associated with cardiac muscle disease in 4 cases, partly dictating the clinical course. Extremely rare was the development over 6 years of cardiac hypertrophy following a burns injury.

Adolescent↗
PubMed1988

[Differential diagnosis and prognosis of primary and post-myocarditis cardiomyopathy in childhood].

It is still difficult to differentiate between the various stages of myocarditis and primary dilated cardiomyopathy (DCM). Hence, we analyzed the history, as well as the laboratory and virological data of 22 children with a dilated, poorly functioning left ventricle, aged 2 months to 16.7 years (m = 4.2 y), on whom we had performed endomyocardial biopsies about 4 months after the beginning of the illness. Specimens were investigated by light and electron microscopy and, in addition immunoserological (n = 15) and immunohistological investigations (n = 7) were performed. On the basis of cellular infiltration in the histological examination we diagnosed resolving/resolved myocarditis in 6 patients and DCM in 15 patients. Previous respiratory infection or sudden onset were found in 40-50% of patients in both groups. Antimyolemmal and antisarcolemmal antibodies showed no preference. Follow-up (3.1 +/- 2.8 years) of the patients showed equal mortality (33% vs. 38%) in the post-myocarditis and DCM group. Complete normalization of all findings in 3 patients of the DCM group makes the classification among the post-myocarditis group probable, which would, then give a lower mortality rate (22% vs. 46%) and a chance of restitution in 55% of cases. In any one specific case all the diagnostic methods do not allow undoubted distinction between both entities. The prognosis of myocarditis seems to be better than that of DCM in childhood.

Adolescent↗
PubMed1988

[Pentoxyverine poisoning via maternal milk in a fully breast-fed newborn infant].

A four-week-old male infant who had been exclusively breast-fed by his mother developed acute respiratory failure with intermittent arrest of breathing for up to 15 sec, in between sighing breaths and normal ones. For two weeks his mother had been taking pentoxyverine-containing cough drops for an upper respiratory tract infection. Pentoxyverine was demonstrated in maternal serum and breast milk, as well as the child's serum and urine, in some in very high concentrations. The course of the illness and comparison with reported instances of pentoxyverine poisoning in infants makes it likely that this, too, was such a case.

Adult↗
PubMed1986

[Hypertrophic cardiomyopathy in childhood: studies of the clinical picture and course].

30 children with hypertrophic cardiomyopathy and a mean age of 4.9 +/- 4.4 years, 11 infants and 19 children, were followed up for an average of 6.6 +/- 5.6 years. Apart from a systolic murmur in nearly all patients, only a few had symptoms. 7 patients from 5 families had a positive family history. The ECG often showed left ventricular hypertrophy, twice a deep Q wave, and no cases of giant T wave. A subaortic pressure gradient greater than or equal to 40 mm Hg was measured in 6 children, a secondary cardiomyopathy was excluded by biopsy in 8. Associated lesions were coarctation (n = 2) and patent ductus arteriosus (n = 2). 7 children died: 1 infant in congestive heart failure, 3 children suddenly, and 3 postoperatively. The annual mortality rate was 3.5%. Also in childhood, beta-blocker therapy may not prevent sudden cardiac death, nor may operation prevent late complications. In spite of the lack of pressure gradients, the children did not fulfil the criteria of hypertrophic non-obstructive cardiomyopathy.

Cardiac Catheterization↗
PubMedDate not supplied

Slice-selective fat saturation in MR angiography using spatial-spectral selective prepulses.

Presaturation of fat signals by frequency-selective radiofrequency (RF) pulses is often applied in MR angiography to improve the visualization of the blood vessels. Unfortunately, standard fat saturation methods might cause a considerable reduction of the blood signal in the measured slices. This effect is caused by an attenuation of blood magnetization in remote tissue regions with water protons showing a similar Larmor frequency as the fat protons in the recorded slice. The affected blood water protons subsequently flow into the recorded slice and provide low signal intensity. Suitable spatial-spectral selective methods for slice-selective fat saturation were developed to avoid this unwanted effect. A spatial-spectral fat saturation technique was compared with a corresponding only spectrally selective approach. Both saturation techniques were included in a standard two-dimensional (2D) cine sequence and applied in angiographic examinations of the thighs. The results indicate that spatial-spectral saturation (acting slice selectively) leads to a clearly higher blood signal intensity in fat-suppressed MR angiography compared with standard techniques, especially in measurements performed during the systolic phase of the cardiac cycle.

Adipose Tissue↗
PubMedDate not supplied

Increased turnover of serotonin in children with pulmonary hypertension secondary to congenital heart disease.

Serotonin (5HT) is a potent vasoconstrictor of the pulmonary vascular bed and may be involved in the pathophysiology of secondary pulmonary hypertension in children with a left-to-right shunt due to a congenital heart defect. To test this hypothesis we measured the total and free 5HT concentration in blood as well as the urinary excretion of its main metabolite 5-hydroxyindoleacetic acid (HIAA) in children showing a left-to-right shunt with (n = 10) and without (n = 18) pulmonary hypertension. 5HT and HIAA were also measured in children after corrective cardiac surgery using cardiopulmonary bypass (n = 14) and in controls without congenital heart disease (n = 18). The concentrations of total and free 5HT were not significantly different between controls and patients with a left-to-right shunt. After cardiac surgery total 5HT concentration was significantly reduced by about 65% owing to a postoperatively reduced platelet count. In patients with a left-to-right shunt the total 5HT content was similar in the right atrium (204.0 +/- 17.3 ng/ml), pulmonary artery (189.0 +/- 19.1 ng/ml), and aorta (195.0 +/- 19.3 ng/ml), as was the free 5HT concentration. Therefore no net release of 5HT from platelets occurred between these sampling sites. In patients with pulmonary hypertension, the urinary excretion of HIAA was significantly increased when compared with controls and patients without pulmonary hypertension. It is concluded that turbulent blood flow in children with a left-to-right shunt does not lead to a significant release of 5HT from platelets. However, the increased urinary excretion of HIAA in patients with pulmonary hypertension indicates an increased turnover of 5HT, probably due to an increased number of intrapulmonary neuroepithelial cells or a higher metabolic rate of 5HT within those cells.

Cardiopulmonary Bypass↗
PubMedDate not supplied

[Progress and change in nuclear magnetic resonance diagnosis of congenital and acquired heart defects].

Since 1987, the authors have examined 186 patients (76 girls, 110 boys) with a variety of congenital and acquired heart diseases by means of magnetic resonance imaging (MRI). The patients' ages ranged from 2 days to 20 years (mean age 3.97 years). During the study new techniques were developed, which extend the applicability of MRI. Especially two-dimensional and three-dimensional angiography are demonstrated and discussed. Using all diagnostic tools offered by MRI more than 90% of cardiovascular malformations are correctly diagnosed. MRI is of unquestionable value in the evaluation of the thoracic and abdominal aorta and in demonstrating the vascular status in pulmonary atresia and anomalous pulmonary venous return. It may be useful if question arise in patients with complex lesions, intra- and extracardiac tumours and cardiomyopathies. Although MRI is still under investigation, it may play a major role in diagnosing congenital heart diseases.

Adolescent↗
PubMedDate not supplied

[Combination of scimitar syndrome and horseshoe lung. A rare but typical finding--case report and review of the literature].

The scimitar syndrome is a rare congenital cardiopulmonary malformation. In association with a horseshoe lung it is extremely uncommon. We describe a child with typical scimitar syndrome (anomalous right pulmonary venous return, hypoplasia of the right lung with dextro-position of the heart and aberrant systemic arterial supply of the right lung), associated with a horseshoe lung. No clinical symptoms are present, surgical management is not necessary. This is the 20th case in addition to the 19 reported cases with scimitar syndrome associated with horseshoe lung. Eight other additional cases of horseshoe lung without scimitar syndrome are mentioned here. In horseshoe lung the pulmonary parenchyma extends from the right lung base across the midline and fuses with the left lung. In only 3 cases continuous parenchymal tissue could be found histological. In 8 cases the lungs were separated by fissurelike structures, seen in CT or chest film, and in 5 cases fissures were demonstrated histological. From 6 patients no findings are available. The various symptoms, the diagnostic tools and the therapeutic procedure will be discussed.

Bronchopulmonary Sequestration↗
PubMedDate not supplied

[Encephalomyelopathy, cardiomyopathy, cataract and changes in the retinal pigment epithelium resulting from a cytochrome c oxidase deficiency].

A 4 1/2 months old female baby was admitted to our hospital after an unexpected heart attack. Birth was in the 37th gestational week after an uneventful pregnancy and delivery by sectio, birth weight 1650 g, Apgar 9/10/10. In the following weeks the baby showed general muscle hypotonia, failure to thrive and sometimes an uncharacteristic heart murmur. Besides a chronic lactic acidemia we found a hypertrophic cardiomyopathy, cataract and small defects of the pigment epithelium of the retina. The CT-scan of the brain showed hypodense areas of both thalami and the mid-brain. Metabolic examination of two muscle specimens showed a deficiency of cytochrome-c-oxidase activity (I: 30, II: 20, normal: 73-284 mU/mg protein). So our patient may be the first case with an established defect in the respiratory chain suffering from cardiomyopathy, cataract and mitochondrial dysfunction. There is also a strong similarity to other encephalomyopathies especially to the Leigh-Syndrome.

Cardiomyopathy, Hypertrophic↗