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Biomedical subjects

B O Eriksson

Publications and source records attributed to B O Eriksson.

At least 37 records · Page 2Linked to original sources
PubMed1994

Impaired ketogenesis in carnitine depletion caused by short-term administration of pivalic acid prodrug.

Long-term treatment with pivalic acid prodrug results in impaired ketone-body production. Therefore, it was of interest to investigate whether short-term treatment had any influence on the fatty acid oxidation. In this study six healthy males were given 1200 mg per day of pivmecillinam for 12 days to induce carnitine deficiency. The concentration of free carnitine in serum was reduced from a mean of 42.8 mumol/liter (range, 31-48) to 11.6 mumol/liter (range, 7.0-24), but the muscle carnitine concentration was not reduced. A 36-h fasting test was performed before and after drug administration to study the effect on ketone-body production. After treatment, the two subjects with the lowest level of serum free carnitine at the end of the fasting period had impaired ketogenesis. This indicates a carnitine deficiency in the liver which was reflected in the free carnitine concentration for by mobilization of muscle carnitine. We conclude that there is a substantial risk to develop carnitine deficiency and impaired fatty acid oxidation in the liver during short-term treatment with drugs conjugated with pivalic acid.

Adult↗
PubMed1994

Dilated cardiomyopathy and the dystrophin gene: an illustrated review.

Cardiomyopathy is often found in patients with Duchenne and Becker muscular dystrophy, which are X linked muscle diseases caused by mutations in the dystrophin gene. Dystrophin defects present in many different ways and cases of mild Becker muscular dystrophy have been described in which cardiomyopathy was severe. Female carriers of Duchenne muscular dystrophy can develop symptomatic skeletal myopathy alone or combined with dilated cardiomyopathy. They can also develop dilated cardiomyopathy alone. X linked dilated cardiomyopathy has been found in association with dystrophin defects. The relation between the molecular defects and the cardiac phenotypes has not yet been established. New mutations in the dystrophin gene are common and such mutations cause one third of the cases with Duchenne and Becker muscular dystrophy. This means that sporadic cases of cardiomyopathy caused by dystrophin defects are likely. This paper reports such a case in a boy of 14 who died of dilated cardiomyopathy. Before the cardiac investigation, which was performed one month before he died, he had not complained of muscular weakness. He had minor signs of limb girdle myopathy and slightly increased concentrations of serum creatine kinase. He was found to have an unusual deletion in the dystrophin gene.

Adolescent↗
PubMed1989

Transport of carnitine into cells in hereditary carnitine deficiency.

Carnitine uptake has been studied in fibroblasts from a case of hereditary carnitine deficiency and in relatives. There was no evidence for carrier-dependent uptake in cells from the patient. The mother and probably the healthy sister had an impaired uptake. The results show that the defect in this form of carnitine deficiency is an inability to establish a concentration gradient over the cell membrane.

Biological Transport↗
PubMed1989

Glucose metabolism and insulin secretion in infants with symptomatic ventricular septal defect.

Nineteen infants with symptomatic ventricular septal defect (VSD) were examined on, altogether, 26 occasions, when each was given an intravenous glucose tolerance test (IVGTT); concentrations of insulin- and C-peptide in plasma were determined. Comparisons were made with 14 healthy infants of the same age. The VSD infants were growth retarded with lower weight/age and length/age ratios. Glucose tolerance as indicated by glucose fasting levels and response to intravenous glucose tolerance test, including glucose disappearance rate, did not differ between the two groups. In response to the glucose load, insulin in plasma was significantly less in VSD infants. In response to the IVGTT, insulin secretion rate calculated from C-peptide levels in plasma was significantly elevated in the VSD group. We conclude that compared to healthy infants, those with symptomatic VSD have normal glucose tolerance, increased secretion rate of insulin, but decreased levels of circulating insulin in response to an intravenous glucose load. We suggest this is so because binding of insulin to peripheral receptors and/or insulin extraction in the liver somehow increases.

Blood Glucose↗
PubMed1988

Non-invasive evaluation of the long-term results of coarctectomy in childhood.

Nineteen young men operated upon for coarctation of the aorta in childhood were studied with pulse tracings from the femoral and carotid arteries and echocardiographic examination of the heart with estimation of the degree of hypertrophy and valve anomalies. The results were compared with intra-arterial blood-pressure measurements and angiographically measured width of the aortic anastomosis. Patients with markedly distorted pulse curves had the narrowest anastomoses although no uniform pattern could be detected. Although one of the selection criteria was 'no other known cardiovascular malformations', only three of the patients had completely normal aortic and mitral valves. Nine of the patients had left ventricular hypertrophy but the degree of hypertrophy could not be correlated to the degree of arterial hypertension. The importance of long-term follow-up of these patients, who in spite of surgery have a remaining cardiovascular excess mortality, is emphasized. Pulse tracings and echocardiography seem to be of value for this purpose.

Aortic Coarctation↗
PubMed1987

Mitochondrial encephalomyopathy. A variant with heart failure and liver steatosis.

We report the clinical and autopsy findings in a young man of 18 with a chronic progressive disorder comprised of lactic acidosis, mental deterioration, and epileptic seizures which were sometimes accompanied by stroke-like episodes with transient hemiparesis and cortical blindness. He died of congestive heart failure. The autopsy showed lesions of the gray matter of the brain. Both the putamen and parieto-occipital cortex showed loss of neurons and proliferation of macrophages, astrocytes and vessels. There was marked loss of neurons in the inferior olives, and slight reduction of the number of Purkinje cells. Skeletal muscle studies revealed ragged-red fibers and structurally abnormal mitochondria. The heart was enlarged: accumulations of mitochondria occurred in the muscle fibers. The liver exhibited marked fatty degeneration. Biochemical analyses showed normal activities of pyruvate dehydrogenase in thrombocytes, pyruvate carboxylase in lymphocytes, biotinidase in serum as well as succinate dehydrogenase and cytochrome c oxidase. The features of this disorder differ in many respects from cases of mitochondrial encephalomyopathy previously reported and cannot be assigned to any specific disease entity.

Acidosis, Lactic↗
PubMed1987

Neonatal surgery for coarctation of the aorta. The Gothenburg experience.

Thirty-four neonates (weights 0.97-4.47 kg) were operated on for coarctation of the aorta. They included five premature infants with birth weight less than or equal to 2.1 kg. All 34 were symptomatic, 31 severely so. Coarctation was "simple" in 12 cases and "complex" in 22; 12 with ventricular septal defect and 10 with more complex malformations. The mean age at operation was 11 days. Subclavian flap aortoplasty was used in 27 cases, a lusoric artery in two, and combined end-to-end anastomosis and flap repair in five. The main pulmonary artery was banded in 13 of the 22 complex coarctation syndromes. Absorbable suture was used in the last 22 cases. There was one early postoperative death. Serious ischaemic complications (bowel or limb gangrene) arose in four patients who were in severe heart failure preoperatively. During follow-up averaging 1.9 years there were two recurrences of coarctation. Resection with end-to-end anastomosis combined with subclavian flap aortoplasty when there is bulging ductal tissue or long, narrow isthmus and use of absorbable vascular suture may further lower the incidence of recurrent coarctation.

Aorta↗
PubMed1987

Subclavian flap angioplasty with absorbable suture polydioxanone (PDS). An experimental study in growing piglets.

The fate of the subclavian flap in aortoplasty was studied and a new synthetic monofilament, absorbable vascular suture (polydioxanone, PDS) was evaluated. In 11 piglets submitted to the aortic repair, the diameter of the aortic arch and descending aorta and the length and width of the subclavian flap were measured. The aortoplasty was performed with a continuous running suture of 6/0 PDS. All the animals survived and grew normally. They were sacrificed 6-26 weeks postoperatively, when the mentioned variables were reestimated. No aortic narrowing was found and no suture material was detectable in the lumen. The subclavian flap had grown uniformly in length and width. Histologic examination showed evening of the inner surface by intimal proliferation and healing of the anastomoses. There was no sign of flap destruction and tissue reaction to PDS suture was minimal, indicating normal growth and viability in all parts of the flap. The suture material was absorbed 26 weeks postoperatively. Continuous suture with absorbable PDS seems to be a good alternative for repair of aortic coarctation in early infancy.

Animals↗
PubMed1986

Estimation of stroke volume using Doppler echocardiography and left ventricular echocardiographic dimensions in infants and children.

To evaluate the accuracy of noninvasive determination of stroke volume in infants and children, 28 patients (age range 4 weeks to 19 years) were studied. Stroke volume was calculated according to Teichholtz from M-mode echocardiographic tracings of left ventricular dimensions in 8 subjects. Agreement with thermodilution performed within 60 min of echocardiography was good (r = 0.995, y = 0.91x + 1.59, SEE = 1.8 ml). Since stroke volume correlated to body size we corrected for (height)3. After this correction there was still good agreement to thermodilution (r = 0.88, y = 1.29x-7.13, SEE = 7.1 ml/H3). M-mode echocardiography was then used as a reference method for evaluating two different Doppler methods in the remaining 20 subjects. Continuous wave Doppler stroke distance, calculated from the mean velocity, was combined with aortic root area (Method I), and stroke distance calculated from maximum velocity was combined with the aortic interleaflet area (Method II). Good agreement was found with Method I (r = 0.95, y = 1.01x-0.14, SEE = 8.1 ml) and Method II (r = 0.95, y = 1.04x-1.14, SEE = 8.4 ml). However, when stroke volume was normalized for (height)3, Method I was found to be superior to Method II.

Adolescent↗
PubMed1983

Muscle substrate levels, muscle enzyme activities and muscle morphology in the vastus lateralis and deltoideus muscles in normal children and in children with coarctation of the aorta.

Muscle biopsies from the deltoideus dx and vastus lat. dx muscles were taken in 17 children with coarctation of the aorta, aged 5.0 to 13.8 years, prior to surgery. Higher concentrations of glycogen, ATP and CP were found in the vastus lat. muscle compared to the deltoideus muscle. The same differences between these two muscles were also found in healthy controls. No differences were found between the patients with coarctation of the aorta and the control group. Nor were any differences found for the other variables studied; glucose, glucose-6-phosphate, lactate, muscle enzyme activities (SDH, LDH and phosphorylase), muscle fibre composition or fibre sizes. It seems reasonable to assume that the differences in muscle substrate levels found between the vasus lat. and the deltoideus muscles in the two groups were due to a higher degree of activity during daily life for the legs as compared to the arms. Patients with coarctation of the aorta do not seem to be influenced by the altered haemodynamic situation with regard to the studied variables.

Adenosine Triphosphate↗
PubMed1983

Non-ionic contrast media in cardioangiography in infants. A comparison of iohexol and metrizamide.

Iohexol (300 mg I/ml) was compared with metrizamide (300 mg I/ml) in pediatric cardioangiography. Forty consecutive patients below one year of age were included in an open, randomized, comparative two-group investigation. Immediate extrasystoles occurred frequently at injections of contrast medium, but the frequency did not differ significantly between metrizamide and iohexol. Both contrast media caused only minor changes in heart rate. Iohexol caused a significantly larger increase in end diastolic pressure following injections in the left ventricle than did metrizamide. The pressure changes registered were, however, moderate in both contrast media groups and the difference between the two contrast media is probably of no clinical importance. A moderate plasma volume expansion was noted after injection of both contrast media. Metrizamide caused a slight increase of ASAT in the 10 min blood sample which differed significantly from that of iohexol, but no increase of enzyme activity was noted after 24 h and the change was considered to be of no clinical importance. Image quality was similar with both contrast media. Iohexol and metrizamide were well tolerated in pediatric cardioangiography.

Angiocardiography↗
PubMed1981

Blood flow resistance in the hand after coarctectomy.

Patients with established hypertension have an increased blood-flow resistance at maximal vasodilatation, which has been interpreted as being caused by a wall thickening in the resistance vessels. Hand blood-flow resistance at maximal vasodilatation was estimated from intra-arterial mean blood pressure and plethysmographically determined hand blood-flow in ten young men, operated upon for coarctation of the aorta during childhood. The patients were compared with different reference groups examined in the same way. The resting mean blood pressure in the coarctation group was only slightly elevated, to the level of a group of patients with mild blood pressure elevation, while the blood-flow resistance at maximal vasodilatation was markedly increased and equalled that found in a group of patients with established hypertension. This discrepancy between blood pressure and blood-flow resistance suggests a limited reversibility of structural changes in the resistance vessels, originating from the preoperative period with high blood pressure, and might be an argument in favour of early operation and/or antihypertensive medication in children with coarctation of the aorta.

Adolescent↗
PubMed1981

Baroreceptor reflexes after coarctectomy.

Patients with hypertension have a impaired baroreflex sensitivity, the reduction being proportional to the blood pressure elevation. Coarctation of the aorta is characterized by hypertension in the upper part of the body. In eight young men, operated upon for coarctation of the aorta during childhood, baroreflex sensitivity was estimated from the prolongation of the R-R interval during the transient rise of arterial blood pressure induced by intravenous injections of small doses of angiotensin. The patients were compared with normotensive and hypertensive reference groups, of the same age and sex, who were examined in the same way. The resting mean blood pressure in the coarctation group was slightly elevated, and the baroreflex sensitivity was reduced proportionally. The fact that the baroreceptors were more sensitive than would be expected from the patients pre-operative high blood pressure suggests that the baroreceptor-resetting in high blood pressure is to a considerable degree reversible when the blood pressure is reduced.

Adolescent↗
PubMed1981

Myocardial metabolism in relation to electrocardiographic changes and cardiac function during graded hypoxia in the fetal lamb.

Previous studies from this laboratory have demonstrated early and progressive alterations in the ST-T period of the fetal and neonatal electrocardiogram in relation to asphyxia. The aims of the present study were to investigate the metabolic background of these hypoxic ECG changes by means of serial myocardial biopsies in fetal lambs, relating these changes to the hypoxic depletion of glycogen, ATP and creatine phosphate stores in the heart and to the altered myocardial performance as measured by heart rate, mean arterial blood pressure, combined cardiac output and max. dP/dt. The experiments were performed on 21 fetal lambs, acutely exteriorized and subjected to graded hypoxia. During hypoxia there was a significant relationship between the degree of changes in the ST-T period according to a scoring system and the depletion of myocardial glycogen and ATP, a highly significant correlation between the rate of myocardial glycogenolysis and the rate of increase in T wave amplitude, and a parallelism between the amount of glycogen available and fetal cardiovascular function. The myocardium was capable of regenerating its glycogen stores under conditions of adequate oxygenation and in the absence of acidosis and hypoglycaemia.

Adenosine Triphosphate↗
PubMed1981

Comparisons of muscle substrate levels, muscle enzyme activities and muscle morphology in arm and leg muscles in normal subjects and in patients operated upon for coarctation of the aorta in childhood.

Muscle biopsies from the upper and the lower part of the body (m. deltoideus dx and m. vastus lat dx) were taken in young men, 17-28 years of age, who had been operated upon for coarctation of the aorta in childhood. Significant differences regarding SDH-activity and some muscle substrate levels were found, with higher values in the leg muscle. The same differences were found in ten healthy controls. It is concluded that arm and leg muscle differ in these respects but that the haemodynamic difference remaining after coarctectomy does not seem to influence this situation.

Adenosine Triphosphate↗
PubMed1981

Muscle metabolism during exercise in men operated upon for coarctation of the aorta in childhood.

Muscle biopsies were taken from the quadriceps muscle during rest and exercise in 16 young men (ages 16-28 years) operated upon for coarctation of the aorta during childhood and 10 healthy men (ages 19-25 years). Resting values and depletion after exercise for ATP, CP and glycogen were similar in the two groups. Muscle-lactate concentrations were higher at all levels of exercise in the operated group, while blood-lactate concentrations did not differ. It is concluded that the operated patients have a disturbed blood-flow regulation and a somewhat impaired blood-flow to the working leg muscles.

Adenosine Triphosphate↗