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Biomedical subjects

H Forsberg

Publications and source records attributed to H Forsberg.

At least 37 records · Page 2Linked to original sources

Outcome of pregnancy in women with myotonic dystrophy and analysis of CTG gene expansion.

Pregnancy outcome was investigated in 32 women with clinically obvious myotonic dystrophy. The results indicated that there are two groups of women, those whose children have the adult type of myotonic dystrophy and those whose children have the congenital type. The overall perinatal mortality was 14%. Polyhydramnios was an obvious sign of the congenital type. No subclinical gene carrier was found among the children. We conclude that prenatal diagnosis should be offered to women with myotonic dystrophy, particularly to those who have previously given birth to a child with the congenital type.

Chromosomes, Human, Pair 19↗

Maternal diabetes induces increased expression of extracellular matrix components in rat embryos.

The mechanisms responsible for the increased incidence of congenital malformations in offspring of diabetic mothers are poorly understood. Because the abnormal metabolic milieu of diabetes induces in the adult organism increased synthesis of basement membrane components, and these molecules play a prominent role in morphogenesis, we investigated whether maternal diabetes or high glucose levels disturb extracellular matrix synthesis in rat embryos. In gestational day 11 embryos, maternal diabetes induced a small but significant increase in laminin B1 (127 +/- 40% of control, mean +/- SD, P < 0.02) but not in fibronectin mRNA (101 +/- 26% of control). Day 12 embryos from diabetic mothers showed a larger increment in laminin B1 (179 +/- 91% of control, P < 0.02) and also an increase in fibronectin mRNA (172 +/- 73% of control, P < 0.02). A similar increase in the expression of fibronectin was observed in the kidneys and hearts of day 20 fetuses dissected from diabetic rats. High glucose levels mimicked in vitro the effects of maternal diabetes. Day 9 embryos cultured for 48 h in 50 mM D-glucose showed, akin to the day 11 embryos in vivo, an increase in laminin B1 mRNA (129 +/- 47% of control) and no changes in fibronectin mRNA (106 +/- 35% of control). The finding that maternal diabetes induces increased expression of extracellular matrix components in developing embryos establishes a link with the abnormalities occurring in the chronic complications of diabetes and proposes a new path of investigation for the mechanism of teratogenicity of the diabetic milieu.

Animals↗

Increased level of HLA-DR-expressing T lymphocytes in peripheral blood from patients with idiopathic dilated cardiomyopathy.

Peripheral blood leukocytes from 14 patients with idiopathic dilated cardiomyopathy (IDC), 13 patients with ischemic congestive heart failure, and 12 controls were characterized using different antibodies. The proportions of B lymphocytes, T lymphocytes, and the different T lymphocyte subsets were estimated. No difference between the three groups could be found in the various T and B cells subpopulations. Using a two-color direct immunofluorescence technique, the occurrence of circulating T helper/inducer (Leu-3a) and T cytotoxic/suppressor cells (Leu-2a) expressing HLA-DR antigens was examined. Only IDC patients demonstrated increased levels of HLA-DR-positive T helper/inducer cells (2.8 +/- 2.4%) and T cytotoxic/suppressor cells (2.8 +/- 2.3%) as compared with patients with ischemic congestive heart failure (0.8 +/- 0.7 and 1.0 +/- 1.0%, respectively) and controls (0.6 +/- 0.5 and 0.9 +/- 0.6%, respectively). When individual IDC patients were studied, 4 out of 12 patients had an increased level of HLA-DR-expressing T helper/inducer cells, and 7 out of 12 patients had elevated HLA-DR-positive T cytotoxic/suppressor cells. The findings suggest that activation of the T lymphocytes may be of importance in the pathogenesis of IDC.

Aged↗

Diabetic embryopathy. Studies with animal and in vitro models.

Diabetic pregnancy is associated with an increased risk for fetal maldevelopment for a largely unknown reason. A decade ago, Norbert Freinkel suggested that the altered fuel mixture offered to the growing conceptus may be the key to most of the changes in the embryogenesis of diabetic pregnancy. He coined the term fuel-mediated teratogenesis. During early pregnancy, periods of maternal hyper- and hypoglycemia may cause marked changes in the availability of glucose to the conceptus. Also, increased concentrations of lipids, notably ketone bodies, and branched-chain amino acids in the maternal circulation contribute to a changed fuel mixture for the embryo. In a recent experimental study of diabetic rats, it was found that the maternal metabolism of all three major classes of nutrients and maternal somatic growth during gestation covaried with the development of the embryo. Consequently, the maintenance of normal concentrations of metabolites from all nutrient classes may be important for prevention of adverse fetal outcome in diabetic pregnancy. In vitro, a high glucose concentration causes embryonic dysmorphogenesis by generation of free oxygen radicals. An enhanced production of such radicals in embryonic tissues may be directly related to an increased risk of congenital malformations in diabetic pregnancy. Thus, the notion that alterations in the net transfer of cellular fuels from the diabetic mother to her offspring may cause embryonic dysmorphogenesis, which suggests that combustion of the fuel may produce compounds that impair embryonic development, has obtained experimental support. If this is also true for human diabetic pregnancy, it has therapeutic implications.

Animals↗

Multiple-beam equalization radiography in chest radiology. Image quality and radiation dose considerations.

The large difference in transmission between the mediastinum and the part of the chest mainly containing lungs causes major problems in chest radiography. A system for advanced multiple beam equalization radiography has been evaluated. Evaluation of image quality has been performed both using standard phantoms and from clinical radiographs. Measurements of radiation dose burden to the patient have been made both in clinical examinations and using an anthropomorphic phantom. The image quality, in areas with low transmission, is substantially increased using the equalization system. In parts of the chest mainly containing lung tissue, conventional systems show an equal or slightly better image quality. The radiation dose burden to the patient is increased by 25 percent using the equalization system, as compared to a low-dose air-gap system. In our opinion, the slight increase in radiation dose burden is well motivated by the high overall quality of the radiographs produced.

Humans↗

Assessment of autonomic nerve function in myotonic dystrophy.

The function of the autonomic nervous system was studied in 23 patients with myotonic dystrophy, from a defined population in northern Sweden with an extremely high prevalence of this disease. Heart rate variability tests showed only minor signs of parasympathetic dysfunction. Blood pressure and plasma noradrenaline measurements in recumbent and upright positions showed no signs of sympathetic neuropathy. Increased plasma levels of noradrenaline was an unexpected finding. Our study does not support the hypothesis that cardiac arrhythmias, orthostatic hypotension, gastrointestinal motility disturbances and urinary bladder dysfunction in myotonic dystrophy are caused by autonomic neuropathy, and we believe that these symptoms should rather be ascribed to a defective function of the target organs.

Adolescent↗

Cardiac involvement in congenital myotonic dystrophy.

Seven young patients (mean age 19 years 8 months) with congenital myotonic dystrophy and with defined symptoms at birth were investigated by electrocardiography and echocardiography. None had cardiovascular symptoms. Electrocardiograms or echocardiograms or both were abnormal in all patients. Atrioventricular and intraventricular conduction defects were the most common electrocardiographic abnormalities and were seen in five patients. The echocardiographic examinations showed impaired left ventricular systolic function in one patient. Other echocardiographic findings were a small left ventricle and atrium, minor valve defects, and mitral valve prolapse. This study shows that the heart is often affected in young patients with congenital myotonic dystrophy. The specialised conduction system is often affected and so too is the myocardium, causing impaired systolic function.

Adolescent↗

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↗

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↗

Dental health and dental care in severely mentally retarded children.

Dental health of 103 children with severe mental retardation living in the north of Sweden, has been surveyed and compared with that of a control group of healthy children of normal intelligence. Data has been related to medical diagnosis, medication, type of residence and previous dental care. The children with severe mental retardation had been offered dental care to the same extent as the control children. Lack of cooperation in treatment and treatment under general anaesthesia was common among the SMR-children. The SMR-children who lived in institutions had a lower prevalence of caries than either those SMR-children not living in institutions or the control group. The SMR-children had a considerably higher frequency of inflamed gingival surfaces and pathological gingival pockets than the control children. The highest frequency was found among children with Down's syndrome. Gingival hyperplasias were recorded in 21% of the SMR-children. The study showed that the children with SMR had shared the generally improved dental care resources and that regular dental care had ensured that there was no neglected need for caries treatment. Periodontal health was poor and the need for stronger and appropriate preventive measures against gingivitis and periodontitis was considerable.

Adolescent↗

Experience with a 36 cm image intensifier system in neuroradiological examinations.

A large-sized image intensifier-TV-fluorographic system in combination with 100 mm sheet-film camera was evaluated for use in neuroradiologic practice. The image intensifier tube had new features such as a large input screen diameter, metal input screen cover and fiber optics on the output screen. The system was evaluated both technically and clinically. With some modifications, the tested system can be recommended for use in neuroangiography provided that it is supplemented by facilities for full-size or magnification technique.

Brain Diseases↗

Oral conditions in Sjögren-Larsson syndrome.

A study was carried out of oral conditions, including caries, gingivitis and periodontitis, malocclusion and enamel hypoplasias, in 34 of the 35 living Swedish patients with Sjögren-Larsson syndrome (SLS). Their residential circumstances (institutional or non-institutional) and medical background variables were recorded. The results were compared with previously published epidemiological data concerning the dental health of fully healthy and also of mentally and physically handicapped individuals. Compared with fully healthy individuals the SLS-patients had higher caries indices, a higher frequency of gingivitis and periodontitis, a higher frequency of enamel hypoplasias, but a similar prevalence of malocclusions. When compared with other mentally retarded individuals, the SLS-patients did not differ significantly with regard to caries frequency and gingival and periodontal status. The frequency of toothlessness in SLS-patients over 40 years of age was high. SLS-patients residing in institutions had a lower average caries prevalence than those who did not reside in institutions. The degree of mental retardation and the occurrence of epilepsy and more severe forms of neuromuscular disorder appeared to influence the extent of gingival and periodontal disease. The frequency of enamel hypoplasias was higher than in patients with cerebral palsy and comparable with the frequency in patients with Down's syndrome.

Adolescent↗