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Occurrence of serologically verified Mycoplasma pneumoniae infections in Finland and in Scandinavia in 1970--1977.

This study analyses the numbers of serologically verified Mycoplasma pneumoniae infections in Finland as a whole and by regions, and in Scandinavia in 1970--77 on the basis of reports collected in the countries concerned. Two peaks of occurrence were found to have extended over most parts of Finland and across Scandinavia; in addition, there were separate local epidemics. Thus, M. pneumoniae infections showed a periodicity of occurrence, but not a regular cyclic pattern. The figures do not necessarily reflect the true incidence of M. pneumoniae infections in the areas investigated.

Antibodies, Bacterial↗

A review of wildlife diseases from Scandinavia.

The epidemiological and historical aspects of some important and representative wildlife diseases from Scandinavia are discussed. In noninfectious diseases, examples include cataract in moose (Alces alces), atherosclerosis in hybrid hares (Lepus timidus X L. europaeus), and ethmoid tumors in moose. The epizootiological and historical aspects of the recent epizootics of myxomatosis in European rabbits (Oryctolagus cuniculus) and rabies and sarcoptic mange in red foxes (Vulpes vulpes) are reviewed. The decline and subsequent increase in population abundances of tetraonids including the capercaillie (Tetrao urogallus), black grouse (Lyrurus tetrix), and hazel hen (Tetrastes bonasia) are discussed, and an hypothesis on predation by foxes is presented as a possible explanation for these population fluctuations. The potential impact of environmental pollution on wildlife populations is emphasized with reference to mercury in wildlife from Sweden and the possible effects of cadmium and selenium resulting from acidification. A bibliography of important references is presented pertaining to these and other diseases of wildlife from Scandinavia.

Animal Diseases↗

Mutations of the cell cycle arrest gene p21WAF1, but not the metastasis-inducing gene S100A4, are frequent in oral squamous cell carcinomas from Sudanese toombak dippers and non-snuff-dippers from the Sudan, Scandinavia, USA and UK.

PCR and direct DNA sequencing methods were used to analyse the prevalence of mutations in exon 2 of the p21waf1 gene in 14 oral squamous cell carcinomas (OSCCs) and 8 non-malignant oral mucosal lesions from Sudanese toombak dippers. For comparison, OSCCs (14 from the Sudan, 16 from Norway, 11 from Sweden, 21 from the USA and 14 from the UK) and non-malignant oral mucosal lesions (3 from the Sudan) from non-snuff-dippers were included. The prevalence of mutations in exons 2 & 3 of the S100A4 gene were analysed in the 14 OSCCs from toombak-dippers and in 25 cases of OSCCs from the control non-snuff-dippers. Of the 14 OSCCs investigated from toombak-dippers, mutations in the p21waf1 exon 2 were found in 43% (6 out of 14), compared to 14% (2 out of 14), 22% (6 out of 27) and 14% (5 out of 35) found in those from non-snuff-dippers from the Sudan, Scandinavia and the USA/UK, respectively. OSCCs from toombak-dippers showed 13 different mutations distributed as 10 (77%) transitions and 3 (23%) transversions. OSCCs from non-snuff-dippers from the Sudan, Scandinavia, the USA and the UK showed 33 different mutations distributed as 14 (42%) transitions and 19 (58%) transversions. In the OSCCs examined, cases with mutations in the p21waf1 also had p53 gene mutations. Only exon 2 of the S100A4 gene was found mutated in 3 cases of OSCCs (one from a toombak-dipper and two from the non-snuff-dippers). The toombak-dipper OSCC had 4 mutations (one transition, 3 transversions), compared to the OSCCs from non-snuff-dippers which showed 3 mutations each (one transition, 2 transversions). All these 3 cases were negative for mutations in the p21waf1 and p53 genes. No mutations of p21waf1 or S100A4 were found in the non-malignant oral mucosal lesions from the snuff-dippers/non-dippers. These findings suggest that; (i) p21waf1, together with p53, is a target gene of oral carcinogenesis in OSCCs from toombak-dippers, with the tobacco specific nitrosamines present in toombak possibly acting as principal carcinogens in these OSCCs; (ii) findings of p21waf1 exon 2 mutations in the OSCCs unrelated to snuff use further demonstrate that this gene may play an important role during the pathogenesis of OSCCs caused by smoked tobacco use; (iii) mutations in the S100A4 gene are rare in OSCCs, but appears to be complementary to p21waf1 and p53 mutations. Since molecular analysis of OSCCs can provide clues to endogenous or environmental factors contributing to the high risk of OSCCs, further analysis of the role of the p21waf1 gene mutations as a biomarker of malignant transformation, which is linked to the p53 gene, is necessary, especially in habitual users of toombak from the Sudan.

Carcinoma, Squamous Cell↗

[Prediction of cancer mortality in Scandinavia in 2005. Effect of various interventions].

Approximate estimates have been made of the effect by the year 2005 of various preventive measures to reduce cancer mortality in Scandinavia. Figures have been calculated for changes in smoking, diet and exposure to sunlight (primary prevention), for earlier diagnosis (secondary prevention), and improved survival as a result of improved treatment (tertiary prevention). The calculations have been performed with the American program CAN TROL, the effect being expressed as the percentage reduction in cancer mortality by the year 2005. The results give promise of a substantial overall reduction in cancer mortality in Scandinavia.

Diet↗

The first human isolate of Puumala virus in Scandinavia as cultured from phytohemagglutinin stimulated leucocytes.

A virus isolate was recovered from blood leucocytes of a patient with nephropathia epidemica (NE). Leucocytes were isolated from EDTA-blood by dextran sedimentation and cultured on monolayers of Vero E6 cells in the presence of phytohemagglutinin (PHA) in roller tubes during the first 72 hours of incubation followed by rolling culture for three weeks in total. Thereafter the first subculture was done in a plastic flask and afterward at at least 6 week intervals. Antigen was first detected after 6 months and 2 weeks of culture. When tested by monoclonal antibodies and patient sera the isolate had the characteristics of a PUU virus. PCR amplification using PUU-specific primers and subsequent partial sequencing of the S and M segments revealed that the Umeå/305/human/95 virus differs from the Finnish PUU Sotkamo rodent prototype virus and is similar but not identical to rodent strains of PUU virus acquired from the same region as the patient isolate. It is we concluded that the first human isolate of the etiologic agent of NE in Scandinavia was recovered from blood leucocytes stimulated with PHA by long-term culture in Vero E6 cells. The isolate belongs to the PUU serotype of hantaviruses as shown by its serologic profile and partial sequencing data.

Adult↗

Genetically heterogeneous selective intestinal malabsorption of vitamin B12: founder effects, consanguinity, and high clinical awareness explain aggregations in Scandinavia and the Middle East.

Selective intestinal malabsorption of vitamin B(12) causing juvenile megaloblastic anemia (MGA; MIM# 261100) is a recessively inherited disorder that is believed to be rare except for notable clusters of cases in Finland, Norway, and the Eastern Mediterranean region. The disease can be caused by mutations in either the cubilin (CUBN; MGA1; MIM# 602997) or the amnionless (AMN; MIM# 605799) gene. To explain the peculiar geographical distribution, we hypothesized that mutations in one of the genes would mainly be responsible for the disease in Scandinavia, and mutations in the other gene in the Mediterranean region. We studied 42 sibships and found all cases in Finland to be due to CUBN (three different mutations) and all cases in Norway to be due to AMN (two different mutations), while in Turkey, Israel, and Saudi Arabia, there were two different AMN mutations and three different CUBN mutations. Haplotype evidence excluded both CUBN and AMN conclusively in five families and tentatively in three families, suggesting the presence of at least one more gene locus that can cause MGA. We conclude that the Scandinavian cases are typical examples of enrichment by founder effects, while in the Mediterranean region high degrees of consanguinity expose rare mutations in both genes. We suggest that in both regions, physician awareness of this disease causes it to be more readily diagnosed than elsewhere; thus, it may well be more common worldwide than previously thought.

Anemia, Megaloblastic↗

Incidence of male breast cancer in Scandinavia, 1943-1982.

Male breast cancer incidence was examined in a collaborative study of data from the cancer registries in Denmark, Finland, Norway and Sweden, comprising a total of 1,529 cases diagnosed from 1943 to 1982. Effects of age, time of diagnosis, birth cohort and country of residence were evaluated by using statistical models of the multiplicative Poisson type. The logarithm of incidence increased linearly with the logarithm of age and had a slope of about 5. In Denmark, male breast cancer incidence increased significantly with time, about 1% per year. No significant time effects were observed in the other countries. A significant effect of birth cohort appeared in Sweden only, but there was no particular trend in incidence by year of birth. In relation to Denmark, the risk of male breast cancer was lower in Sweden, Norway and Finland, the relative risk estimates with 95% confidence intervals being 0.82(0.72-0.94), 0.72(0.61-0.84) and 0.53(0.43-0.64) respectively. The variation within Scandinavia is similar for female breast cancer, pointing to common factors being involved in the etiology of breast cancer in both sexes.

Adolescent↗

Asphyxiophilia in Scandinavia.

Asphyxiophilia, the desire for a state of oxygen deficiency in order to enhance sexual excitement and orgasm, is a very precarious form of sexual gratification. Asphyxiophilia has been difficult to document since it is mostly practiced in secrecy and solitude and is usually not discovered until the practitioner has been found dead. Most of the information about asphyxiophilia is found in police reports and medicolegal investigations by doctors of forensic medicine. Our study is based on information from 18 doctors of forensic medicine in Denmark, Norway, and Sweden. Six sources indicate similar frequencies (0.5-1 cases per million inhabitants per year) of observed deaths in Scandinavia. Although the frequency of observed deaths is different from the true mortality rate, this study gives a good illustration of the estimated prevalence. This accuracy is enhanced by the length of the observation periods (10-40 years).

Adolescent↗

Congenital diaphragmatic hernia: a survey of practice in Scandinavia.

There is no consensus on the treatment of congenital diaphragmatic hernia (CDH), and practice seems to vary between centres. The main purpose of the present study was to survey current practice in Scandinavia. Thirteen paediatric surgical centres serving a population of about 22 million were invited, and all participated. One questionnaire was completed at each centre. The questionnaire evaluated management following prenatal diagnosis, intensive care strategies, operative treatment, and long-term follow-up. Survival data (1995-1998) were available from 12 of 13 centres. Following prenatal diagnosis of CDH, vaginal delivery and maternal steroids were used at eight and six centres, respectively. All centres used high-frequency oscillation ventilation (HFOV), nitric oxide (NO), and surfactant comparatively often. Five centres had extracorporeal membrane oxygenation (ECMO) facilities, and four centres transferred ECMO candidates. The majority of centres (7/9) always tried HFOV before ECMO was instituted. Surgery was performed when the neonate was clinically stable (11/13) and when no signs of pulmonary hypertension were detected by echo-Doppler (6/13). The repair was performed by laparotomy at all centres and most commonly with nonabsorbable sutures (8/13). Thoracic drain was used routinely at seven centres. Long-term follow-up at a paediatric surgical centre was uncommon (3/13). Only three centres treated more than five CDH patients per year. Comparing survival in centres treating more than five with those treating five or fewer CDH patients per year, there was a tendency towards better survival in the higher-volume centres (72.4%) than in the centres with lower volume (58.7%), p =0.065.

Cross-Sectional Studies↗

Familial defective apolipoprotein B-100: detection in the United Kingdom and Scandinavia, and clinical characteristics of ten cases.

Familial defective apolipoprotein B-100 (FDB) is a recently identified, dominantly inherited genetic disorder, which leads to increased serum concentration of low density lipoprotein (LDL) cholesterol with reduced affinity for the LDL receptor. This disorder is associated with a G to A mutation in exon 26 of the apolipoprotein B (apo B) gene which creates a substitution of glutamine for arginine in the codon for amino acid 3500. We have searched for this mutation in 374 unrelated individuals with hyperlipidaemia from the United Kingdom, and in 371 unrelated individuals with a primary clinical diagnosis of atherosclerosis from the United Kingdom and Scandinavia. Ten individuals, 9 from the U.K. and 1 from Denmark, were identified. The frequency of the mutation was 3% in individuals classified clinically as having familial hypercholesterolaemia (FH) and 3% in individuals with type IIa hyperlipidaemia without FH, and was not found in patients with types IIb and III hyperlipidaemia. The mutation was rare in individuals with a primary clinical diagnosis of atherosclerosis. Plasma lipid levels and clinical characteristics of the ten patients identified in the present study are similar to those reported for heterozygous FH. Thus, in our study, FDB is associated with moderate to severe hypercholesterolaemia, and appears to be a serious disorder causing premature cardiovascular disease. Individuals with this mutation can be identified unambiguously using routine molecular screening techniques.

Adult↗

Epidemiology of invasive Haemophilus influenzae type b disease in Scandinavia.

In all Scandinavian countries, most invasive Haemophilus influenzae type b (Hib) infections occur before the age of 5 years. The age-specific incidence of Hib meningitis in this age group is 26-35/100,000 per year in Denmark, Norway, Finland and Sweden and about 43/100,000 per year in Iceland. Of the meningitis patients, approximately 60% are younger than 2 years but Hib meningitis also occurs in older children and adults. The case fatality rate is low (1-3%) in children but the rate of neurological sequelae is considerable. The incidence of acute epiglottitis, the other main manifestation of Hib disease, varies considerably. In Sweden, four studies have shown a very high incidence in the age group 0-4 years, 21-34 cases/100,000 population per year compared to only 5-17 in the other Nordic countries. Epiglottitis is more common than Hib meningitis in older children and adults. In Scandinavia the age-specific incidence of all invasive Hib infections could be estimated to be about 50-65/100,000 per year in the most susceptible age group and the risk of contracting invasive Hib disease before the age of 15 years would be about 1/200-300 children.

Adolescent↗

Oral mucosal changes related to smokeless tobacco usage: research findings in Scandinavia.

Smokeless tobacco is used in all Scandinavian countries. By far the highest consumption is recorded in Sweden, where the highest sales figures in the world can be found. Moist non-fermented snuff with a pH value of 8-9 comprises over 99% of the products. Only a few tons per year of chewing tobacco are sold. Moist snuff as it is used in Scandinavia today gives rise to oral mucosal changes which are reversible after cessation of the habit. The use of portion-bags, and even more so the use of chewing tobacco, seems to be associated with less pronounced changes than the use of loose snuff. The daily amount of snuff used and hours of daily use seem to have a greater impact on the risk for development of more pronounced changes as recorded clinically and histologically than the number of years with the habit and/or age of the subject. Gingival recessions are much more frequently found among users of loose snuff than among users of portion-bag-packed snuff and they seem to be irreversible.

Gingival Recession↗

National databases and rheumatology research I: longitudinal databases in Scandinavia.

Nationwide population-based longitudinal databases provide excellent resources for medical research in Scandinavia. These include the Population Registry, the Cancer Registry, the Cause of Death Registry, the Hospital Discharge Registry, and other registers, and are linkable to each other by the personal identification code. The registers have long historical backgrounds, and are regulated strictly by law. This article describes features of the national databases and provides some examples of rheumatology research that use these databases.

Databases as Topic↗

Evidence that the Cys282Tyr mutation of the HFE gene originated from a population in Southern Scandinavia and spread with the Vikings.

Hereditary hemochromatosis has been recognized as a clinical disorder for more than 100 years. The common form of the disorder is caused by the Cys282Tyr mutation (C282Y) of the HFE gene. Hereditary hemochromatosis affects predominantly people of Northern European origin. The C282Y mutation probably occurred on a single chromosome carrying the ancestral hemochromatosis haplotype, which subsequently was spread by emigration and the founder effect. It has been estimated that the C282Y mutation appeared 60-70 generations ago. It was initially suggested that the ancestral C282Y mutation occurred within the Celtic group of peoples. However, we hypothesize that the distribution of the C282Y mutation in Europe is more consistent with an origin among the Germanic Iron Age population in Southern Scandinavia. From this area, the mutation could later be spread by the migratory activities of the Vikings. The aim of the present study was to evaluate the validity of these two hypotheses. Several arguments are in favor of the 'Viking hypothesis': first, the highest frequencies (5.1-9.7%) of the C282Y mutation are observed in populations in the Northern part of Europe, i.e. Denmark, Norway, Sweden, Faeroe Islands, Iceland, Eastern part of England (Danelaw) and the Dublin area, all Viking homelands and settlements. Second, the highest allele frequencies are reported among populations living along the coastlines. Third, the frequencies of the C282Y mutation decline from Northern to Southern Europe. Intermediate allele frequencies (3.1-4.8%) are seen in the populations in Central Europe, which is the original Celtic homeland. Low allele frequencies (0-3.1%) are recognized in populations in Southern Europe and the Mediterranean.

Amino Acid Substitution↗

Evidence for a common Spinocerebellar ataxia type 7 (SCA7) founder mutation in Scandinavia.

Spinocerebellar ataxia type 7 (SCA7) is a neuro-degenerative disorder characterised by progressive cerebellar ataxia and macular degeneration. SCA7 is one of the least common genetically verified autosomal dominant cerebellar ataxias (ADCAs) in the world (4.5 to 11.6%), but in Sweden and Finland SCA7 is the most commonly identified form of ADCA. In an inventory of hereditary ataxias in Scandinavia (Sweden, Norway, Denmark and Finland) we identified 15 SCA7 families, eight in Sweden and seven in Finland, while no cases of SCA7 could be found in Norway or Denmark. We examined whether the relatively high frequency of SCA7 families in Sweden and Finland was the result of a common founder effect. Only two out of 15 families could be connected genealogically. However, an extensive haplotype analysis over a 10.2 cM region surrounding the SCA7 gene locus showed that all 15 families studied shared a common haplotype over at least 1.9 cM. This strongly suggests that all Scandinavian SCA7 families originate from a common founder pre-mutation.

Ataxin-7↗

Neuronal ceroid lipofuscinoses in Scandinavia. Epidemiology and clinical pictures.

The epidemiology of neuronal ceroid lipofuscinoses (NCL) in Scandinavia was studied. For juvenile NCL 40 Swedish living patients were identified. The corresponding number for Finland was 61, for Norway 28, for Denmark 16 and for Iceland three. The prevalence of juvenile NCL was thus 4.6, 12.2, 6.5, 3.1 and 11 per million inhabitants in Sweden, Finland, Norway, Denmark, and Iceland, respectively. For calculating incidence the years 1976-85 were used. The incidence was 2.2 per 100,000 live births in Sweden, 4.8 in Finland, 3.7 in Norway, 2.0 in Denmark, and 7.0 in Iceland. Late infantile NCL was found in five Swedish children, including one variant form, CLN5. This gives a prevalence of about 0.6 per million. In Finland 13 cases gave a prevalence of 2.6 per million with the variant form in 80% of cases. In Norway, three children corresponded to a prevalence of 0.7 per million and one case in Iceland to 3.8 per million. No Danish cases were reported. As for infantile NCL, sixteen Swedish cases have been diagnosed during the 27-year period 1968-95, six are presently alive. This gives an estimated prevalence of 0.7 per million and an incidence of 0.6 per 100,000. The prevalence and incidence of infantile NCL in Finland were 5.4 per million and 5 per 100,000, respectively. The prevalence in Norway was 0.2 per million inhabitants. The variability of onset, clinical course and symptoms in the Swedish cases of juvenile and infantile NCL were analysed.

Adolescent↗

Regional differences in coeliac disease prevalence in Scandinavia?

BACKGROUND: According to investigations from the central region of Sweden (Linköping), Norway, and Finland based on antibody screening, the prevalence of coeliac disease (CD) is around 1:300 (0.33%). In Denmark surveys in paediatric departments have shown a prevalence of only 1:10,000. The aim of the present study was to study the prevalence of CD in southern Sweden. METHODS: From October 1996 to February 1997, 1970 healthy blood donors were screened for CD in a serial procedure: first IgA and IgG gliadin antibodies (GA) and then endomysial antibodies in those positive for GA. RESULTS: One patient had previously known CD. Two patients had gastrointestinal symptoms and an increased number of intraepithelial lymphocytes, with improvement on a gluten-free diet. Three of 185 GA-positive blood donors had endomysial antibodies and biopsy-verified CD. Thus, 4 of 1970 blood donors had classic CD, resulting in a prevalence of 1:492 (0.20%)--that is, rather similar to that found in Linköping, Sweden, and in Finland and Norway. If the two persons with gluten-sensitive diarrhoea were also included, the prevalence was 6:1970 = 1:328, or 0.30%. CONCLUSIONS: The prevalence of classic CD (1:492) in southern Sweden is comparable to that found in the rest of Scandinavia, except for Denmark.

Adolescent↗

Human granulocytic ehrlichiosis--a clinical case in Scandinavia.

A clinical case of human granulocytic ehrlichiosis in Scandinavia is presented. The patient developed high fever, myalgia, headache and dyspnoea. Doxycycline treatment resulted in a dramatic improvement. Laboratory confirmation included a fourfold change in anti-Ehrlichia equi IFA titre and a positive PCR confirmed by gene sequence analysis.

Adult↗