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A missense mutation in the FALDH gene identified in Sjögren-Larsson syndrome patients originating from the northern part of Sweden.

Sjögren-Larsson syndrome (SLS) is an autosomal recessive disorder characterized by congenital ichthyosis, spastic di- or tetraplegia, and mental retardation. SLS has been reported to occur in many populations but the highest incidence is in the north of Sweden. The gene causing SLS encodes a fatty aldehyde dehydrogenase (FALDH). In the present study, a point mutation in exon 7 of the FALDH gene was found in SLS patients of northern Swedish origin. The mutation consists of a C-to-T exchange at nucleotide position 943 in the cDNA. As a consequence, a highly conserved proline is replaced by a serine. The mutation was found in 49 out of 58 affected chromosomes and could be the most widely spread SLS mutation in the world.

Aldehyde Oxidoreductases↗

Biogas production from crop residues on a farm-scale level: is it economically feasible under conditions in Sweden?

Anaerobic digestion would enable the energy potential of agricultural crop residues such as sugar beet tops and straw to be harnessed. Sweden is so spread out that full utilisation of this potential by centralised slurry-based technology is difficult. It appears that simple but effective high-solids reactor systems have a better chance of being economically viable on a farm-scale level (50-500 kW). In the present study, the financial prospects of high-solids digestion, using either single-stage fed-batch or two-stage batch reactor systems, are compared on a farm-scale level (50 kW) with those of conventional slurry digestion, on the basis of experimental results and observations on a laboratory- and pilot-scale. The gas produced can be used for heat, combined heat and power or as vehicle fuel. The results indicate high-solids single-stage fed-batch operations to stand the best chances of being competitive, particularly in connection with organic farming. The methane yield, degree of gas utilisation, and operational costs were found to have the strongest impact on the financial success of the process.

Anaerobiosis↗

Biogas production from crop residues on a farm-scale level in Sweden: scale, choice of substrate and utilisation rate most important parameters for financial feasibility.

Anaerobic digestion would enable the energy potential of agricultural crop residues such as ley crops and sugar beet tops to be harnessed in Sweden. In the present study, the financial prospects of single-stage fed-batch high-solids digestion on three different scales, 51, 67, and 201 kW, were calculated on the basis of experimental results and observations. In addition to scale, the effects of methane yield and fertiliser recovery (compared to green manuring) was investigated by testing different substrate mixtures. The biogas was disposed as heat, combined heat and power, or as vehicle fuel. Besides the positive effect of scale, the results indicate the importance of choosing substrates with a high methane yield and high nitrogen content, and the necessity of fully utilising both the capacity of the equipment installed and the energy carriers produced. Net unit costs of 5.3 and 8.1 ct/kWh were achieved (201 kW), heat and vehicle fuel, respectively.

Anaerobiosis↗

Epidemiology of chronic renal failure in children: a report from Sweden 1986-1994. Swedish Pediatric Nephrology Association.

In a national survey, chronic renal failure (CRF) in Swedish children was studied during the period 1986-1994; 118 children (72 boys, 46 girls) with CRF, defined as a glomerular filtration rate below 30 ml/min per 1.73 m2 body surface area, were identified. The median annual incidence of CRF was 7.7 and that of terminal renal failure (TRF) 6.4 per million children. The prevalence of preterminal renal failure decreased from 29 to 21 per million children over the study period, while the prevalence of TRF increased from 17.8 in 1986 to 38 per million children in 1994. The increase in TRF prevalence was due to a lower incidence of deaths due to uremia and a slightly increased incidence of TRF compared with an earlier study period, 1978-1985. The results point to a more active treatment of uremia in Sweden now than during the period 1978-1985. The congenital causes of CRF (renal malformations, obstructive conditions, and hereditary disorders) accounted for 67.5% of all cases, which is high compared with data from other countries. No child with non-obstructive pyelonephritis as a cause of CRF was identified. Age at detection of CRF and time from detection of CRF to TRF were studied. As a high proportion of children, 42%, reached 16 years of age without entering TRF, the value of presenting time from CRF to TRF for the remaining individuals is questionable. There were only minor differences in primary renal disease, age at presentation, and time from CRF to TRF when the study results were compared with those from 1978-1985.

Adolescent↗

Thermal bioclimatic conditions and patterns of behaviour in an urban park in Göteborg, Sweden.

People in urban areas frequently use parks for recreation and outdoor activities. Owing to the complexity of the outdoor environment, there have only been a few attempts to understand the effect of the thermal environment on people's use of outdoor spaces. This paper therefore seeks to determine the relationship between the thermal environment, park use and behavioural patterns in an urban area of Sweden. The methods used include structured interviews, unobtrusive observations of the naturally occurring behaviour and simultaneous measurements of thermal comfort variables, i.e., air temperature, air humidity, wind speed and global radiation. The thermal environment is investigated through the mean radiant temperature (Tmrt) and the predicted mean vote (PMV) index. The outcome is compared to the subjective behaviour and thermal sensation of the interviewees. It is found that the thermal environment, access and design are important factors in the use of the park. In order to continue to use the park when the thermal conditions become too cold or too hot for comfort, people improve their comfort conditions by modifying their clothing and by choosing the most supportive thermal opportunities available within the place. The study also shows that psychological aspects such as time of exposure, expectations, experience and perceived control may influence the subjective assessment. Comparison between the thermal sensation of the interviewees and the thermal sensation assessed by the PMV index indicates that steady-state models such as the PMV index may not be appropriate for the assessment of short-term outdoor thermal comfort, mainly because they are unable to analyse transient exposure.

Adaptation, Physiological↗

Voluntary associations for cancer patients in Sweden: supportive activities.

Our aim was to describe voluntary associations for patients with cancer in Sweden (n=108) and their activities, type and extent of member support, and the associations' collaboration with the health care system. A specially constructed questionnaire with structured and open questions was used for the investigation. The frequency of answers was 89%. The results show in the broadest sense that the associations have two missions. The direct patient-related mission concerns providing patients with support in the form of close proximity, approachability and through distribution of knowledge together with financial and practical support; the indirect patient-related mission deals with activities aimed at improving conditions for patients in general within the health care system and by influencing authorities as well as supporting family members and significant others and providing financing. The associations collaborate with the health care system, although they face difficulties in becoming 'sanctioned' and in establishing positive relationships with the health care community. The associations display a positive attitude towards their cause and the personal gratification that the voluntary work brings. In general, the findings indicate that the associations have a potential to help patients live and cope with their cancer disease.

Health Care Coalitions↗

Spinal stenosis surgery in Sweden 1987-1999.

Despite being recognised for many years as a clinical diagnosis, no exact definition of spinal stenosis has yet been agreed, leading to difficulties in interpreting and comparing studies of the incidence, prevalence and treatment. This study presents the first analysis of national data to be reported. It is a retrospective population-based national register study, aimed at analyzing surgical interventions in patients with lumbar spinal stenosis, patient characteristics, subsequent development, and case fatality rate, based on Swedish national data for 1987-1999. Complete follow-up data were obtained of incidence and type of spinal stenosis surgery, rate of multiple operations, mortality, underlying causes of death, length of hospital stay, and case fatality rate by linkage of the National Inpatient Register and Swedish Death Register. The study cohort consisted of 10,494 patients. Laminectomy was performed in 89%, and additional fusion in 11%. The mean annual rate of operations was 9.7 per 100,000 inhabitants, the annual number of operations performed increased from 4.7 to 13.2 per 100,000 inhabitants per year. The case fatality rate within 30 days after surgery was 3.5 per 1000 operations. Cardiovascular disease was the most common cause of death (46%). Relative risk of dying within 30 days of admission was doubled in men, and for fusion surgery, and increased four fold in patients older than 80 years. The relative risk of dying decreased during the study period. The results show that spinal stenosis surgery in Sweden has increased, and is associated with a low risk. Within an ageing group of patients, mortality has declined.

Age Factors↗

Familial risks for migraine and other headaches among siblings based on hospitalizations in Sweden.

Migraine and other headaches are common disabling conditions, reducing the quality of life in the affected individuals. The unambiguous definition of familial risk for subtypes of migraine and other headaches will advance the search for the heritable causes of these conditions and their underlying mechanisms. We aim at defining familial risks for siblings to be hospitalized because of migraine or other headaches. An ad hoc migraine database was constructed by linking the Multigeneration Register on 0- to 69-year-old siblings to the Hospital Discharge Register for data on migraines of all hospitalized patients in Sweden from years 1987 to 2001. Standardized incidence ratios (SIRs) were calculated for affected sibling pairs by comparison with those whose siblings had no migraine. Among a total of 14,123 hospitalized patients, 205 affected siblings were identified, with a familial SIR of 2.84. SIRs for migraine with and without aura were 3.08 and 2.67, respectively. The highest familial risk of 3.71 was noted for unspecified migraine. The risk tended to be higher for concordant (both siblings sharing the migraine subtype, ranging from 4.0 to 6.0) than discordant subtypes, but even many discordant SIRs were significant. The SIR for spouse correlation was 1.14 for husbands and 1.22 for wives, far from being significant. All subtypes of hospitalized migraines and other headaches show an increased familial risk, at least in specific age groups. The different subtypes appear to share susceptibility, which may imply common disease mechanisms.

Adolescent↗

Disease manifestations and HLA antigens in psoriatic arthritis in northern Sweden.

The aim of this study was to identify potential markers of aggressive joint manifestations and HLA associations in patients with psoriatic arthritis (PsA) in northern Sweden. Patients with PsA were examined clinically, with laboratory tests and radiologically. The classification of the disease was based on peripheral and/or axial engagement. HLA B17, B37 and B62 were significantly increased in PsA patients. Univariate analyses suggest that the HLA antigens B37, B62 and some clinical variables were associated with disease course. However, in multivariate analyses distal interphalangeal joint affliction and polyarticular manifestations were the only variables remaining significantly associated with irreversible joint destruction or deformity. There were no significant effects of HLA antigens. In this cross-sectional study, clinical manifestations were more reliable predictors of aggressive joint damage than were specific HLA antigens. However, HLA antigens seemed to modify the expression of the joint disease rather than being involved in joint disease susceptibility.

Adult↗

Examining the quality of health economic analyses submitted to the Pharmaceutical Benefits Board in Sweden. The first year.

This study assessed the quality of health economic documentation submitted to the Pharmaceutical Benefits Board (PBB) in Sweden. Two different instruments were used in the evaluation: the PBB checklist, which was constructed by the authors from the PBB guidelines for health economic evaluations, and the QHES, a validated quality assessment instrument. Some areas that seem especially problematic, or where the quality was particularly low are identified and discussed. Also, we present the cost per quality-adjusted life-year that the companies have presented and how this related to the PBBs decisions.

Cost-Benefit Analysis↗

Reimbursement and clinical guidance for pharmaceuticals in Sweden: do health-economic evaluations support decision making?

Introduction of the new Pharmaceutical Benefits Board (LFN; 1 October 2002) has markedly changed the principles of pricing and reimbursement of drugs in Sweden. The Board is required to make decisions based on information on cost-effectiveness, and pharmaceutical companies must submit economic evaluations when relevant as part of their applications for reimbursement. This study examined experience to date regarding the use of health-economic evaluations and cost-effectiveness information by the LFN. We also describe activities and the use of cost-effectiveness analysis by Swedish local formulary committees organized by the 21 county councils. It is concluded that economic evaluations have supported decision making by LFN, although cost-effectiveness seems to be of varying importance in different situations. While the use of health-economic evaluations and the outcome of decision making by LFN are similar to comparable committees in other countries, there is presently a gap in this sense between the LFN and Swedish local formulary committees. Coordinated decision making is much needed but may be difficult to implement as the perspective, expertise, and objectives of the two public authorities differ.

Cost Control↗

Long-term absenteeism due to sickness in Sweden. How long does it take and what happens after?

In this paper, we analyze exits from long-term sickness spells in Sweden. Using data for more than 2,500 people, the aim is to analyze the transition to different states: return to work, full disability pension, partial disability pension, and other exits from the labor force. Given the complexity of the exit decision, which encompasses both the individual's choice, the medical evaluation and the decision of the insurance adjudicator, we consider the outcome as being the result of two aspects of the exit process: one that governs the duration of a spell prior to the decision to exit, and another that governs the type of exit. Therefore, the analysis is done in two steps: first, we analyze the duration of the sickness spells, and then we analyze the process that governs the type of exit. The results indicate that both individual characteristics and push factors, such as regional unemployment, are important for both components of the decision process.

Absenteeism↗

Repellency of MyggA Natural spray (para-menthane-3,8-diol) and RB86 (neem oil) against the tick Ixodes ricinus (Acari: Ixodidae) in the field in east-central Sweden.

In the field in south-central Sweden, we tested by randomised, standardised methodology the potential anti-tick repellent activity of two concentrations of MyggA Natural spray (containing PMD) (4.2 and 3.2 g/m2) and one of RB86 (with 70% neem oil containing azadirachtin) (3 g/m2) to host seeking nymphs of Ixodes ricinus. Each substance was applied separately to 1 m2 cotton flannel cloths. Nymphal ticks on the cloths, pulled over the vegetation, were recorded at 10-m stops. Nymphal numbers recorded differed significantly between treated cloths [4.2 or 3.2 g MyggA Natural spray/m2 and 3 g RB86/m2] and the untreated control (df = 3, chi2 = 112.74, P < 0.0001). Nymphal numbers also differed significantly among collectors (df = 3, chi2 = 15.80, P < 0.001). Repellency of treated cloths, i.e., 4.2 or 3.2 g MyggA Natural spray/m2 and 3 g RB 86/m2 declined from day 0 (i.e. the day of impregnation) to day 3 after impregnation from 77 to 24%, 58 to 16% and 47 to 0.5%, respectively. This study suggests that all three treatments have significant repellent activities against I. ricinus nymphs.

Animals↗

Quantification of hydroxamate siderophores in soil solutions of podzolic soil profiles in Sweden.

Concentrations up to 2 and 12 nM of the hydroxamate siderophores ferrichrome and ferricrocin, respectively, were identified in soil solutions of podzolic forest soils at four sites in both northern and southern Sweden. No ferrichrysin was detected. As with the dissolved organic carbon and low molecular mass organic acids, the highest concentrations of the siderophores were found in the upper layers i.e. the mor layer, the eluvial and upper illuvial horizons. At the southern sites, the concentrations of ferrichrome and ferricrocin were both of similar magnitude and did not differ between the two sites. In contrast, soil solutions at the two northern sites contained more ferricrocin than ferrichrome; the ferricrocin concentrations were also higher at the northern sites than at the southern sites. Analyses were performed by high performance liquid chromatography with a porous graphitic carbon column on which ferrichrome, ferricrocin and ferrichrysin were separated. Detection by electrospray ionization mass spectrometry (ESI-MS) combined with on-line sample pre-concentration, by means of column-switching, enabled detection limits of 0.1-0.2 nM for ferrichrome, ferrichrysin and ferricrocin. The structural identities of the siderophores were further verified by MS/MS fragmentation. Fragmentation of ferrichrome, ferricrocin and ferrichrysin occurred mainly via peptide cleavage. The most intense fragments were typified by the loss of one of the three iron(III) chelating hydroxamate residues, i.e N(5)-acyl-N(5)-hydroxy ornithine.

Chromatography, High Pressure Liquid↗

Cost-effectiveness of exemestane versus tamoxifen as adjuvant therapy for early-stage breast cancer after 2-3 years treatment with tamoxifen in Sweden.

Aromatase inhibitors are rapidly becoming the cornerstone of endocrine treatment for advanced disease and are now also used as adjuvant treatment in early-stage disease. The objective of this study was to assess the cost-effectiveness of adjuvant treatment with exemestane versus tamoxifen for early-stage breast cancer after 2-3 years treatment with tamoxifen in Sweden. The results are based on findings in the Intergroup Exemestane Study (IES). IES was a randomized controlled trial in which postmenopausal women who had received 2-3 years of tamoxifen therapy following primary treatment of early-stage breast cancer, were randomized to either continue on tamoxifen therapy or be switched to exemestane therapy. The results showed a disease-free survival hazard ratio of exemestane relative to tamoxifen in IES of 0.69. A Markov state-transition model was developed to simulate consequences after the end of the clinical trial, and to integrate the trial data with external data on mortality, costs and quality of life specific for Swedish women. The cost per QALY gained was about euro 20,000 in the base case analysis without inclusion of consequences of coronary heart disease. Inclusion of these events increased the cost-effectiveness ratio to about euro 31,000. This means that, based on our assumption, sequential exemestane treatment in early breast cancer is a cost-effective option compared with tamoxifen alone, although more long-term data on overall survival and consequences of adverse events would be valuable to increase the validity of the analysis further.

Aged↗

Prospective study of plasma enterolactone and prostate cancer risk (Sweden).

OBJECTIVES: Enterolactone, a phytoestrogen produced by the intestinal microflora from precursors in plant foods, has been postulated to protect against hormone-dependent cancers. We studied the association between plasma enterolactone and risk of prostate cancer. METHODS: In the Northern Sweden Health and Disease Cohort, enterolactone concentrations were measured by time-resolved fluoroimmunoassay in plasma taken from 265 men who were diagnosed with prostate cancer at a mean time of 5 years after blood collection, and in plasma from 525 control men, matched for age and date of blood collection. RESULTS: There was no significant association between quartiles of plasma enterolactone and risk of prostate cancer. Odds ratios for prostate cancer, estimated by conditional logistic regression for increasing concentrations of enterolactone in quartiles were 1.00 (referent), 0.81 (95% confidence interval 0.52-1.27), 1.03 (0.67-1.58), and 1.22 (0.80-1.86). Adjustments for body mass index (BMI), smoking status and stratification for age, lag time, storage time and tumour characteristics did not materially alter risk estimates. Men with very low enterolactone levels, however, had significantly higher risk of prostate cancer, odds ratio for bottom decile versus all other deciles was 1.68 (1.03-2.74). CONCLUSIONS: Our results do not support the hypothesis that enterolactone formed from dietary lignans protects against prostate cancer.

4-Butyrolactone↗

Concentrations of inorganic elements in 20 municipal waters in Sweden before and after treatment--links to human health.

The water chemistry of 20 municipal water treatment plants in southern Sweden, representing various bedrock situations, and water qualities, were investigated. Four water samples, raw and treated, were collected from each plant and analyzed by predominantly ICP-OES and ICP-MS at four occasions from June to December, 2001. The concentrations of Ca, Mg, K, Na, HCO(3) and a number of micronutrients, varied considerably in treated waters from the studied plants (ranges; Ca: 9.1-53.7 mg L(-1), Mg: 1.4-10.9 mg L(-1), K: 1.1-4.8 mg L(-1), Na; 5.4-75.6 mg L(-1), HCO(3): 27-217 mg L(-1)). The elimination of Fe and Mn from raw water was efficient in all treatments investigated, giving concentrations in treated waters below the detection limits at some plants. Softening filters gave waters with Ca-concentrations comparable to the softest waters in this study. Adjustment of pH by use of chemicals like lye, soda or lime, modified the consumer water composition significantly, besides raising the pH. It was estimated that drinking water contributed to approximately 2.2-13% of the daily Ca uptake, if the gastrointestinal uptake efficiency from food and water was estimated to be around 50%. The corresponding figures for Mg was 1.0-7% and for F 0-59%. None of the studied elements showed any significant time trends in raw or treated waters during the follow-up period. The concentrations of potentially toxic metals such as Al, Pb and U were low and did not indicate risks for adverse health effects (ranges; Al: 0.5-2.3 microg L(-1), Pb: 0-0.3 microg L(-1), U: 0.2.5 microg L(-1)).

Gastrointestinal Tract↗

The epidemiology of anophthalmia and microphthalmia in Sweden.

Infants with a clinical diagnosis of anophthalmia or microphthalmia were identified from four health registers in Sweden, covering different parts of the period 1965-2001. During the observation period, the rate of anophthalmia decreased from the early 1970s from 0.4 to 0.2 per 10,000 births. The registered rate of microphthalmia increased markedly during the observation period to reach a maximum in 1987 of about 1.5 per 10,000. About 10% of the 432 identified children had a chromosome anomaly. There was no geographical variation in prevalence and infants born in urban or rural districts had, if anything, a lower risk than infants born in cities (0.93 and 1.13 per 10,000, respectively). Non-eye malformations were more common at anophthalmia (63%) than at microphthalmia (30%). Sex ratio was normal and no statistically significant variation between sub-groups (anophthalmia, microphthalmia, isolated, associated with non-eye malformations) could be demonstrated. There was a marked risk increase with maternal age but no certain parity effect, no effect of maternal education, but a possible association with subfertility. Maternal smoking in early pregnancy seemed to increase the risk for anophthalmia or microphthalmia in the absence of a coloboma.

Adult↗