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Biomedical subjects

B O Olofsson

Publications and source records attributed to B O Olofsson.

At least 37 records · Page 2Linked to original sources
PubMed1989

Autonomic neuropathy in familial amyloidotic polyneuropathy. A clinical study based on heart rate variability.

Familial amyloidotic polyneuropathy (FAP) is characterized by both sensimotor and autonomic dysfunction. Autonomic disturbance involving the gastrointestinal tract, the urinary bladder, the cardiac conduction system, and the peripheral circulation has been described. In this study simple, non-invasive tests of autonomic function based on heart rate variability were applied to 12 patients with FAP and 12 healthy volunteers. The heart rate variation during normal breathing, deep breathing and during tilt from recumbent to standing position was measured. All tests showed significantly less heart rate variation in patients than in controls and the heart rate variation decreased with increasing severity of neurological disability, but the small number of patients in our study does not allow any further comparison between subgroups. Our study thus indicates impaired cardiovascular autonomic function in patients with FAP and we believe that these findings might also be of importance in other forms of systemic amyloidosis.

Adult↗
PubMed1988

Propafenone versus disopyramide for treatment of chronic symptomatic ventricular arrhythmias. A multicenter study.

The efficacy and safety of propafenone, 150 mg four times daily, were compared with those of disopyramide, 100 mg four times daily, in a randomized single-blind, cross-over study in 38 patients with symptomatic premature ventricular complexes (PVCs). The 24-hour ambulatory ECG, employed for assessing antiarrhythmic efficacy, was analyzed blindly. The median reduction in the number of PVCs was higher with propafenone than with disopyramide (91.4% vs. 63.5%, respectively, p less than 0.01). A reduction of at least 80% was achieved by propafenone in 22 (59%) and by disopyramide in 16 patients (43%) (NS). Ventricular tachycardias (VTs) were abolished by propafenone in eight out of 11, and by disopyramide in five out of nine patients with VTs (NS) a possible proarrhythmic effect was seen in three patients during disopyramide and in one patient during propafenone treatment. Micturition disturbances (p less than 0.001) and a dry mouth (p less than 0.01) were more commonly associated with disopyramide than with propafenone. In conclusion, in the given dosages, propafenone was superior to disopyramide in suppressing PVCs and had fewer side-effects.

Adult↗
PubMed1988

Electrocardiographic findings in myotonic dystrophy.

Sixty five patients with myotonic dystrophy, from a defined population in northern Sweden with an extremely high prevalence of this disease, were examined by electrocardiography. The patients were subdivided into three groups according to the severity of the disease. Abnormal electrocardiograms were found in 6 (35%) patients with mild disease, 12 (50%) patients with moderate disease, and 23 (96%) patients with severe disease. First degree atrioventricular block and left anterior hemiblock were the most commonly encountered abnormalities in patients with mild and moderate disease, whereas atrial fibrillation and flutter, abnormal Q waves, and repolarisation abnormalities were more common in patients with severe disease. This study shows that the heart is often affected by myotonic dystrophy. These effects can be detected by electrocardiography in early and mild forms of the disease. The effect on the heart is progressive and clinically important atrial arrhythmias and electrocardiographic abnormalities which are useful in differential diagnosis are common in severe forms of the disease.

Adolescent↗
PubMed1988

24-hour electrocardiographic study in myotonic dystrophy.

Thirty-eight consecutive adult patients with myotonic dystrophy were included in a study with electrocardiography at rest and 24-hour ambulatory electrocardiography. The patients were subdivided into three groups according to the severity of the disease. The prevalence of abnormal electrocardiograms at rest was 31, 50 and 100% in patients with mild, moderate and severe disease, respectively. The main characteristics observed at ambulatory electrocardiography were a high frequency of sinus bradycardia (58%) and intermittent atrioventricular block II (8%). These bradyarrhythmias were not correlated to the severity of the disease. Sustained atrial fibrillation or flutter was found in 3 patients (8%), all with the most severe form of the disease. Ambulatory electrocardiography should be used deliberately in the evaluation of patients with myotonic dystrophy and symptoms compatible with cardiac arrhythmias.

Adolescent↗
PubMed1987

Cardiac stimulation threshold in familial amyloidosis with polyneuropathy.

It has been suggested that a higher cardiac stimulation threshold reduces the applicability of pacemaker therapy in cardiac amyloidosis. We therefore reviewed threshold data in patients with familial amyloidosis with polyneuropathy (FAP), which is an inherited type of systemic amyloidosis, invariably involving the heart. Fourteen FAP patients treated with a pacemaker were studied. The mean (+/- SD) voltage stimulation threshold during implantation was 1.0 +/- 0.5 V, and noninvasive follow-up 6 months later revealed a mean Vario threshold of 1.9 +/- 0.5 V. Beyond this time, the threshold tended to be stable, and high threshold exit block did not occur in any patient. Several FAP patients did show a moderately elevated threshold, and a multiprogrammable pulse generator with a high output capability is recommended when pacemaker therapy is considered in these patients.

Adult↗
PubMed1987

Prealbumin: its association with amyloid.

In recent years prealbumin has been shown to be a major component of two forms of systemic amyloid, senile systemic amyloid (SSA), and familial amyloidotic polyneuropathy (FAP). Despite the fact that the amyloid fibril proteins associated with these two forms of amyloid, designated ASc1 and AF, respectively, share many similarities the clinical features of the two diseases are remarkably different. To understand better this paradox the clinical, histochemical, immunological and biochemical features of SSA and FAP were reviewed.

Adult↗
PubMed1987

Prealbumin variants in the amyloid fibrils of Swedish familial amyloidotic polyneuropathy.

Amino-acid sequence analysis of an amyloid fibril protein from a patient with Swedish familial amyloidotic polyneuropathy showed homology with prealbumin but with heterogeneous N-terminal deletions. One-third of the molecules had the same amino acid substitution, methionine for valine in position 30, as in familial amyloidosis of Portuguese, Japanese and Swedish-American type. A protein with the same antigenic properties and size was found in the fibrils of two other patients with Swedish FAP while the amyloid fibrils in two further patients predominantly contained a smaller prealbumin-derived protein. Cyanogen bromide cleavage of this protein revealed no evidence for a methionine residue in position 30.

Aged↗
PubMed1987

Differentiation of cardiac amyloidosis and hypertrophic cardiomyopathy. A comparison of familial amyloidosis with polyneuropathy and hypertrophic cardiomyopathy by electrocardiography and echocardiography.

The clinical and echocardiographic features of cardiac amyloidosis may closely resemble those of hypertrophic cardiomyopathy, and the disorders may thus be mixed up. The present study was undertaken in an attempt to identify features separating the two conditions by analysis of electro- and echocardiographic findings in patients with familial amyloid polyneuropathy and hypertrophic cardiomyopathy. Twenty-nine patients with familial amyloidosis and 22 with hypertrophic cardiomyopathy were studied. Particular attention was given to the sum of the S wave in V1 and R wave in V5 or V6, the echocardiographic left ventricular mass and cross-sectional area, the presence or absence of asymmetrical septal thickening, granular and sparkling myocardial appearance, thickened heart valves, systolic anterior motion of the mitral valve, and pericardial effusion. A granular and sparkling appearance of the myocardium and thickened heart valves were found to be the best predictors of cardiac amyloidosis, while low QRS amplitudes in relation to echocardiographic left ventricular mass and a pericardial effusion seemed less important. The presence of systolic anterior movement of the mitral valve, a large left ventricular mass and a sum of S in V1 and R in V5 or V6 greater than 35 mm indicated hypertrophic cardiomyopathy. When the four strongest predictors (left ventricular mass, thickened heart valves, a granular sparkling myocardial appearance, and systolic anterior movement of the mitral valve) were used to reclassify the present patients, 28 of 29 amyloidosis patients and 21 of 22 patients with hypertrophic cardiomyopathy were correctly categorized. Noninvasive methods may thus be useful for detecting the myocardial infiltrative process, and cardiac amyloidosis may be confidently diagnosed by typical noninvasive findings together with histopathological documentation of amyloid in an organ other than the heart.

Adult↗
PubMed1986

Heart valve involvement in familial amyloidosis with polyneuropathy.

Involvement of the heart is common in the various types of amyloidosis. Little attention has, however, been paid to the presence and significance of amyloid in cardiac valves. We examined the heart valves of twelve autopsy cases with familial amyloidosis with polyneuropathy. All leaflets showed more or less abundant amyloid infiltration with significant valvular aortic stenosis due to degenerative calcification in two cases. In familial amyloidosis with polyneuropathy, amyloid deposits are thus invariably present in the valves, but the valvular function is often preserved. Amyloid may, however, produce hemodynamically significant valvular lesions, and the importance of valvular involvement in the various types of amyloidosis may still be underestimated.

Adult↗
PubMed1986

Cardiac arrhythmias in familial amyloid polyneuropathy during anaesthesia.

There is an increased risk of anaesthetic complications in patients with myocardial disorders. The present paper concerns seven patients with familial amyloid polyneuropathy, who developed severe bradycardia or heart block during anaesthesia. Amyloid infiltration of the cardiac conducting tissues probably increases their susceptibility to anaesthetic agents. Dysfunction of autonomic nerves may also diminish cardiac impulse formation and conduction. In addition, the possibility of contributing succinylcholine-induced hyperkalaemia cannot be excluded. There was a prompt response to infusion of isoproterenol in all patients so treated, but the insertion of a temporary pacemaker prior to anaesthesia should, nevertheless, be seriously considered in this group of patients.

Adult↗
PubMed1985

Electrophysiologic evaluation of the cardiac conduction system and its autonomic regulation in familial amyloid polyneuropathy.

A high incidence of cardiac conduction disturbances is a salient feature in familial amyloid polyneuropathy (FAP) and amyloid infiltration of the conduction system has been found. In patients with FAP autonomic nervous dysfunction involving gastrointestinal, urogenital and cardiovascular systems has been described. However, the present study is the first to evaluate the results of autonomic tests and pharmacologic intervention in the autonomic nervous regulation of the cardiac conduction system during an invasive electrophysiologic study. Seven patients with FAP and severe disturbances of the conduction system, evaluated concerning indications for pacemaker treatment, were studied; in 3 of them the parasympathetic regulation was found to be abolished. In 1 patient the observations indicated either vagal overactivity or denervation oversensitivity of the receptors. The function of the beta adrenoceptors was intact in all patients, but a disturbance of sympathetic innervation could not be excluded. Dysfunction of autonomic nervous regulation of the cardiac conduction system was thus found in 4 of 7 patients. However, most conduction abnormalities were irreversible, indicating amyloid infiltration of the conduction system as the predominant pathophysiologic mechanism, in accordance with the findings of pathoanatomic studies.

Amyloidosis↗
PubMed1985

Highly refractile myocardial echoes in familial amyloidosis with polyneuropathy. A correlative echocardiographic and histopathological study.

Two-dimensional echocardiography in systemic amyloidosis has in most cases shown highly refractile myocardial echoes. The morphological basis for this finding has so far been little examined. In the present study, echocardiographic examinations were performed in vitro on autopsied hearts from ten patients with familial amyloidosis with polyneuropathy (FAP). An ultrasonically visible needle was used to localize the abnormal echoes, and samples were taken for histological analysis of the region of the echocardiographic abnormality. Our results indicate that the highly refractile myocardial echoes in FAP are due to nodules composed of collagen and amyloid.

Adult↗
PubMed1985

Prevalence of coronary artery disease in patients with valvular heart disease.

To evaluate the usefulness of preoperatie coronary angiography in patients undergoing preoperative investigation because of valvular heart disease, we performed coronary angiography in a consecutive series of 329 patients. The prevalence of significant coronary artery disease was 32%. Asymptomatic coronary artery disease was present in 13%. Angina pectoris proved to be a poor predictor of coronary artery disease in aortic valve disease. In mitral valve disease, however, the specificity was high. A cost-benefit calculation was carried out in order to assess what advantage routine coronary angiography might have. According to this, coronary angiography should be performed in all patients suffering from valvular heart disease with angina pectoris, whereas it can be omitted in younger patients without angina. A cut-off point of 60 years seems appropriate for aortic valve disease and 65 years for mitral valve disease.

Adult↗
PubMed1985

Prealbumin in Swedish patients with senile systemic amyloidosis and familial amyloidotic polyneuropathy.

A prealbumin (PA)-like protein was demonstrated in amyloid fibrils both from a patient with senile systemic amyloidosis (SSA) and from a patient in northern Sweden with familial amyloidotic polyneuropathy (FAP). The investigated properties of this protein were similar in the two types of fibrils. The protein had molecular weight, antigenic determinants, and at least one cysteinyl residue in common with the subunit of normal PA. In contrast to normal PA, it contained disulphide-linked subunits and was to some extent bound to the fibril via the cysteinyl residues. The noncovalent forces between its subunits were weaker than in normal PA. It constituted part of the previously described AScl protein of the SSA fibrils. Proteins with lower molecular weight than the PA monomer were major proteins in both SSA and FAP fibrils. These proteins had similar properties in the two kinds of fibril and may be derived from PA. PA in serum from Swedish patients with FAP and SSA was normal with regard to the isoelectric pH of the monomers and tetramers.

Adult↗
PubMed1985

Serum prealbumin and retinol-binding protein in the prealbumin-related senile and familial forms of systemic amyloidosis.

In a series of 13 elderly patients with proven prealbumin-related senile systemic amyloidosis (SSA), depressed serum prealbumin values (110.7 +/- 14.1 micrograms/ml) were found as compared to an age-matched control group (175.1 +/- 20.3 micrograms/ml). As expected, there was a significant correlation between serum prealbumin and serum retinol-binding proteins in both groups of patients. Patients with reactive amyloid protein AA amyloidosis had slightly depressed serum prealbumin concentrations, whereas patients with prealbumin-related familial amyloidosis of Swedish type had prealbumin values within normal limits. Since the serum levels of the acute phase reactants, haptoglobin and amyloid-related serum protein AA, were higher in the group of patients with reactive amyloidosis than in patients with SSA, the depression of the prealbumin levels in SSA is not a result of inflammation. Since SSA is known to contain prealbumin, it is possible that a disturbed prealbumin metabolism in old age results in low prealbumin serum values and deposition of amyloid.

Adult↗
PubMed1984

The sinoatrial node in familial amyloidosis with polyneuropathy. A clinico-pathological study of nine cases from northern Sweden.

The pathogenesis of disturbances of the initiation and conduction of the cardiac impulse in cardiac amyloidosis has been a matter of controversy. In this study, we have analyzed the histopathology of the sinoatrial node and the atrial myocardium in nine cases of familial amyloidosis with polyneuropathy. Our results indicate that in this disease, amyloid infiltration of the sinoatrial node and the atrial musculature per se may account for the electrophysiological disturbances of these regions.

Adult↗