Aggregated data from the same laboratories participating in two glucose external quality assessment schemes show that commutability and transfers of values to control materials are decisive for the biases found.

OBJECTIVES: We report the results of glucose measurements performed during one year by the same measurement procedures (MPs) in 58 Norwegian hospital laboratories using control materials provided by external quality assessment (EQA) schemes from two different providers. The providers used materials with presumed vs. verified commutability and transfers of values using reference material vs. using a highest-order reference MP. METHODS: Data from six Labquality and three Noklus glucose EQA surveys were aggregated for each MP (Abbott Alinity, Abbott Architect, Roche Cobas, and Siemens Advia) in each scheme. For each EQA result, percent difference from target value (% bias) was calculated. Median percent bias for each MP per scheme was then calculated. RESULTS: The median % biases observed for each MP in the Labquality scheme were significantly larger than those in the Noklus scheme, which uses verified commutable control materials and highest-order reference MP target values. The difference ranged from 1.2 (Roche Cobas, 2.9 vs. 1.7 %) to 4.4 percentage points (Siemens Advia, 3.2 % vs. -1.2 %). The order of bias size for the various MPs was different in the two schemes. In contrast to the Labquality scheme, the median % biases observed in the Noklus scheme for Abbott Alinity (-0.1 %), Abbott Architect (-0.5 %), and Siemens Advia (-1.2 %) were not significantly different from target value (p>0.756). CONCLUSIONS: This study underlines the importance of using verified commutable EQA materials and target values traceable to reference MPs in EQA schemes designed for assessment of metrological traceability of laboratory results.

Trends in overweight and obesity in Bergen, Norway, using data from routine child healthcare 2010-2022.

AIM: Trends in childhood overweight, obesity and severe obesity have been lacking in Norway. This study assessed pre-pandemic trends from 2010 to 2019 and evaluated differences in prevalence during the 2020-2022 pandemic years. METHODS: Routine height and weight measurements from child and school health centres were extracted retrospectively from children aged 2, 4, 6, 8 and 13 years. Overweight, obesity and severe obesity was classified according to the International Obesity Task Force cut-offs. Pre-pandemic trends were estimated using linear regression. The prevalence during the pandemic was compared to the 95% prediction interval of this model. RESULTS: We obtained 181 527 body mass index measurements on 78 024 children (51.0% boys). There was a decrease in the prevalence of overweight including obesity from 2010 to 2019 in boys and this was statistically significant at 4 and 13 years of age. We found no significant trends in girls during this period. During the pandemic, the prevalence of overweight including obesity exceeded the prediction intervals for boys aged 4, 6, and 8 years, and for 6-year-old girls. CONCLUSION: From 2010-2019, overweight including obesity plateaued in girls and decreased in boys but increased during the pandemic among prepubertal boys. Routine healthcare data is useful for estimating the prevalence of different weight status.

Direct-to-consumer laboratory testing (DTCT): challenges and implications for specialists in laboratory medicine.

In vitro diagnostics (IVD) testing is a powerful tool for medical diagnosis, and patients' safety is guaranteed by a complex system of personnel qualification of the specialist in laboratory medicine, of process control, and legal restrictions in healthcare, most of them under national regulation. Direct-to-consumer laboratory testing (DTCT) is testing ordered by the consumer and performed either by the consumer at home or analysis of self-collected samples in a laboratory. However, since DTCT are not always subject to effective competent authority oversight, DTCT may pose risks to lay persons using and relying on it for healthcare decision-making. Laboratory medicine specialists should be very cautious when new DTCTs are introduced. As qualified professionals, they should feel obliged to warn and educate patients and the public about the risks of inappropriate and harmful DTCT.

Parental income gradients in child and adolescent mortality: Norwegian trends over half a century.

BACKGROUND: Child mortality has declined rapidly over the last century in many high-income countries. However, little is known about the socio-economic differences in this decline and whether these vary across causes of death. METHODS: We used register data that included all Norwegian births between 1968 and 2010 (2.1 million), and we analysed how all-cause and cause-specific child (0-4 years) and adolescent (5-20 years) mortality rates vary with relative parental income the year before the birth. RESULTS: Child and adolescent all-cause mortality decreased with increasing parental relative income within all birth cohorts. Among children aged 0-4 years, the socio-economic gradient in all-cause mortality and in mortality due to external causes, sudden infant deaths and perinatal factors declined over the period, while there was no systematic decline in mortality from congenital malformations. Among children aged 5-20 years, the gradient did not weaken similarly, although there were indications of declines in the socio-economic gradient related to all-cause deaths and deaths because of suicides and other external causes. While the absolute differences in mortality declined over time, the relative differences remained stable. CONCLUSIONS: Although children of low-income parents still have elevated mortality, there has been a large reduction in child mortality in all socio-economic groups across 50 years for all causes combined and most of the groups of specific causes of death.

Livmorhalsprøvetaking i primærhelsetjenesten.

BACKGROUND: In Norway, approximately 360 000 cervical screening samples were taken in 2020, of which 11 000 were registered as inadequate. We therefore wished to investigate doctors' knowledge of cervical sample-taking in the primary health service. MATERIAL AND METHOD: An anonymous survey on cervical sample-taking was sent by email to around 4 700 members of the Norwegian College of General Practice in September 2021. RESULTS: Of the 1 039 doctors who responded to the survey, 820 (79 %) reported that they always indicate the reason for taking the sample in the requisition form, and 898 (86 %) reported that they avoid taking a sample during menstruation. Only one in three doctors (343) correctly indicated the location of the squamocolumnar junction in postmenopausal women. In response to a question aimed at users of the ThinPrep method, which is particularly sensitive to sampling errors, 426 out of 697 (61 %) answered that they either avoid using a lubricant or use a water-based lubricant, while only 35 % of the doctors responded that they stop taking the sample if bleeding occurs. INTERPRETATION: The results show that although many doctors have satisfactory knowledge, a continuous focus on cervical sample-taking is essential. Correct sampling and knowledge of anatomical factors in postmenopausal women may be significant for reducing the number of inadequate samples.

Throat infections and use of streptococcal antigen test and antibiotic treatment in general practice; a web-based survey.

OBJECTIVE: The aim of this study was to investigate the use of streptococcal antigen tests and antibiotic prescription in general practice in Norway in relation to the national guidelines for sore throat. DESIGN: This study was based on a web-based survey. SETTING: Norwegian general practice. SUBJECTS: 4700 members of the Norwegian College of General Practice received the survey by E-mail. MAIN OUTCOME MEASURES: General practitioner (GP) adherence to national guidelines. RESULTS: In total, 807 GPs responded and were included in the study. According to the guidelines, 20% and 30% of the GPs would perform unnecessary streptococcal antigen testing when presented with mild and severe infections respectively, while 52% would not perform the test at moderate infection. Phenoxymethylpenicillin was recommended by 95% of the GPs. CONCLUSION: In this survey of self-selected GPs, we identified some non-adherence to National guidelines for streptococcal antigen testing and antibiotic prescribing. However, when antibiotic treatment was offered, the correct antibiotics were prescribed.Key pointsNorwegian guidelines for diagnosis and treatment of throat infections include the use of Centor criteria as a clinical tool to limit the unnecessary use of antibiotics. In this web-based survey, we investigated the use of streptococcal antigen tests and antibiotic prescription in general practice in relation to the national guidelines.•Streptococcal antigen tests were not always performed according to Norwegian guidelines, causing inappropriate antibiotic prescribing.•National guidelines were followed in the choice of antibiotics for sore throat.

Parental income gradients in adult health: a national cohort study.

BACKGROUND: Disparities in health by adult income are well documented, but we know less about the childhood origins of health inequalities, and it remains unclear how the shape of the gradient varies across health conditions. This study examined the association between parental income in childhood and several measures of morbidity in adulthood. METHODS: We used administrative data on seven complete Norwegian birth cohorts born in 1967-1973 (N = 429,886) to estimate the association between parental income from birth to age 18, obtained from tax records available from 1967, linked with administrative registries on health. Health measures, observed between ages 39 and 43, were taken from registry data on consultations at primary health care services based on diagnostic codes from the International Classification of Primary Care (ICPC-2) and hospitalizations and outpatient specialist consultations registered in the National Patient Registry (ICD-10). RESULTS: Low parental income during childhood was associated with a higher risk of being diagnosed with several chronic and pain-related disorders, as well as hospitalization, but not overall primary health care use. Absolute differences were largest for disorders related to musculoskeletal pain, injuries, and depression (7-9 percentage point difference). There were also differences for chronic disorders such as hypertension (8%, CI 7.9-8.5 versus 4%, CI 4.1-4.7) and diabetes (3.2%, CI 3.0-3.4 versus 1.4%, CI 1.2-1.6). There was no difference in consultations related to respiratory disorders (20.9%, CI 20.4-21.5 versus 19.7%, CI 19.2-20.3). Childhood characteristics (parental education, low birth weight, and parental marital status) and own adult characteristics (education and income) explained a large share of the association. CONCLUSIONS: Children growing up at the bottom of the parental income distribution, compared to children in the top of the income distribution, had a two- to threefold increase in somatic and psychological disorders measured in adulthood. This shows that health inequalities by socioeconomic family background persist in a Scandinavian welfare-state context with universal access to health care.

Availability and analytical quality of hemoglobin A1c point-of-care testing in general practitioners' offices are associated with better glycemic control in type 2 diabetes.

Background It is not clear if point-of-care (POC) testing for hemoglobin A1c (HbA1c) is associated with glycemic control in type 2 diabetes. Methods In this cross-sectional study, we linked general practitioner (GP) data on 22,778 Norwegian type 2 diabetes patients to data from the Norwegian Organization for Quality Improvement of Laboratory Examinations. We used general and generalized linear mixed models to investigate if GP offices' availability (yes/no) and analytical quality of HbA1c POC testing (average yearly "trueness score", 0-4), as well as frequency of participation in HbA1c external quality assurance (EQA) surveys, were associated with patients' HbA1c levels during 2014-2017. Results Twenty-eight out of 393 GP offices (7%) did not perform HbA1c POC testing. After adjusting for confounders, their patients had on average 0.15% higher HbA1c levels (95% confidence interval (0.04-0.27) (1.7 mmol/mol [0.5-2.9]). GP offices participating in one or two yearly HbA1c EQA surveys, rather than the maximum of four, had patients with on average 0.17% higher HbA1c levels (0.06, 0.28) (1.8 mmol/mol [0.6, 3.1]). For each unit increase in the GP offices' HbA1c POC analytical trueness score, the patients' HbA1c levels were lower by 0.04% HbA1c (-0.09, -0.001) (-0.5 mmol/mol [-1.0, -0.01]). Conclusions Novel use of validated patient data in combination with laboratory EQA data showed that patients consulting GPs in offices that perform HbA1c POC testing, participate in HbA1c EQA surveys, and maintain good analytical quality have lower HbA1c levels. Accurate HbA1c POC results, available during consultations, may improve diabetes care.

Evaluation of eleven rapid tests for detection of antibodies against SARS-CoV-2.

Objectives: SARS-CoV-2, causing COVID-19, has emerged to cause a human pandemic. Detection of SARS-CoV-2 in respiratory samples by using PCR is the standard laboratory diagnostic tool. Our aim was to perform a limited evaluation of the diagnostic performance and user-friendliness of eleven rapid tests for detection of antibodies against SARS-CoV-2. Methods: All participants were tested with PCR against SARS-CoV-2 at a clinical microbiology laboratory. Comparing with results from PCR tests, we evaluated the rapid tests' performances in three arms; 1) 20 hospitalized patients with PCR-confirmed COVID-19, 2) 23 recovered outpatients with former PCR-confirmed COVID-19, and 3) 49 participants with suspected COVID-19 presenting at a primary care emergency room. Results: All eleven tests detected antibodies in hospitalized COVID-19 patients, though with varying sensitivities. In former outpatients recovered from COVID-19, there were differences between tests in the immunoglobulin type G (IgG) sensitivity, with five tests having a sensitivity below 65%. In participants with suspected COVID-19 infection, the rapid tests had very low sensitivities. Most rapid tests were easy to perform and interpret. Conclusions: Rapid tests were not suited as stand-alone tests to detect present infection in a Norwegian primary care emergency room population. All the rapid tests were able to detect SARS-CoV-2 antibodies, although sensitivities varied and were generally higher in the study arm of more severely affected participants. Rapid tests with high IgG sensitivity (and specificity) may be useful for confirmation of past infection. An independent evaluation should be performed in the intended population before introducing a rapid test.

Parental education and the risk of cerebral palsy for children: an evaluation of causality.

AIM: To explore whether increasing parental education has a causal effect on risk of cerebral palsy (CP) in the child, or whether unobserved confounding is a more likely explanation. METHOD: We used data from Norwegian registries on approximately 1.5 million children born between 1967 and 2011. We compared results from a traditional cohort design with results from a family-based matched case-control design, in which children with CP were matched to their first cousins without CP. In addition, we performed a simulation study to assess the role of unobserved confounding. RESULTS: In the cohort design, the odds of CP were reduced in children of mothers and fathers with higher education (adjusted odds ratio [OR] 0.67, 95% confidence interval [CI] 0.60-0.75 for maternal education, and adjusted OR 0.75, 95% CI 0.67-0.85 for paternal education). In the family-based case-control design, only an association for maternal education remained (adjusted OR 0.80, 95% CI 0.64-0.99). Results from a simulation study suggested that this association could be explained by unobserved confounding. INTERPRETATION: A causal effect of obtaining higher education on risk of CP in the child is unlikely. Results stress the importance of continued research on the role of genetic and environmental risk factors that vary by parents' educational level. WHAT THIS PAPER ADDS: Children of higher-educated parents had significantly lower odds of cerebral palsy (CP). There was no evidence of difference in risk of CP within first cousins whose mothers or fathers had different educational levels. Association between parental education and odds of CP did not reflect a causal effect.

Spirometry in chronic obstructive pulmonary disease in Norwegian general practice.

BACKGROUND: General practitioners (GPs) in Norway increasingly use spirometry diagnostically as well as in follow up of patients with respiratory complaints, but little is known about their skills and knowledge in this area. The aim of the present study was to investigate how GPs interpret a case history and spirometry recordings of a patient with chronic obstructive pulmonary disease (COPD), and their knowledge about their own spirometer. METHODS: A web-based survey, consisting of a case history and spirometry recordings of a patient with COPD, was distributed to the 4700 members of the Norwegian GP Association. In addition to background information about themselves and their spirometer, topics included whether they requested, and how they interpreted, a spirometry reversibility-test, identification of the of most likely diagnosis, and recognition of the spirometry parameters used to diagnose COPD and grade airway obstruction. Immediate feedback was provided for educational purposes. RESULTS: Six hundred thirty GPs responded. Twenty six percent would not request a reversibility test, but 81% identified COPD as the most likely diagnosis. Less than 50% correctly identified the spirometry parameters used for diagnosis of COPD and grading the airway obstruction. One in five (21%) did not know which spirometer was used in their own practice, and 49 and 61% did not know which reference values were used for adults and children, respectively. Participants evaluated the survey as useful (average 74 points on a 0-100 scale) and would like more case-based surveys concerning use of spirometry in the future (average 91 points). CONCLUSION: In this cohort of self-selected GPs, probably more interested in respiratory medicine than the average GP, we identified several problem areas and gaps in knowledge regarding the use of spirometry.

Association of Household Income With Life Expectancy and Cause-Specific Mortality in Norway, 2005-2015.

Importance: Examining causes of death and making comparisons across countries may increase understanding of the income-related differences in life expectancy. Objectives: To describe income-related differences in life expectancy and causes of death in Norway and to compare those differences with US estimates. Design and Setting: A registry-based study including all Norwegian residents aged at least 40 years from 2005 to 2015. Exposures: Household income adjusted for household size. Main Outcomes and Measures: Life expectancy at 40 years of age and cause-specific mortality. Results: In total, 3 041 828 persons contributed 25 805 277 person-years and 441 768 deaths during the study period (mean [SD] age, 59.3 years [13.6]; mean [SD] number of household members per person, 2.5 [1.3]). Life expectancy was highest for women with income in the top 1% (86.4 years [95% CI, 85.7-87.1]) which was 8.4 years (95% CI, 7.2-9.6) longer than women with income in the lowest 1%. Men with the lowest 1% income had the lowest life expectancy (70.6 years [95% CI, 69.6-71.6]), which was 13.8 years (95% CI, 12.3-15.2) less than men with the top 1% income. From 2005 to 2015, the differences in life expectancy by income increased, largely attributable to deaths from cardiovascular disease, cancers, chronic obstructive pulmonary disease, and dementia in older age groups and substance use deaths and suicides in younger age groups. Over the same period, life expectancy for women in the highest income quartile increased 3.2 years (95% CI, 2.7-3.7), while life expectancy for women in the lowest income quartile decreased 0.4 years (95% CI, -1.0 to 0.2). For men, life expectancy increased 3.1 years (95% CI, 2.5-3.7) in the highest income quartile and 0.9 years (95% CI, 0.2-1.6) in the lowest income quartile. Differences in life expectancy by income levels in Norway were similar to differences observed in the United States, except that life expectancy was higher in Norway in the lower to middle part of the income distribution in both men and women. Conclusions and Relevance: In Norway, there were substantial and increasing gaps in life expectancy by income level from 2005 to 2015. The largest differences in life expectancy between Norway and United States were for individuals in the lower to middle part of the income distribution.

Maternal thyroid disorder in pregnancy and risk of cerebral palsy in the child: a population-based cohort study.

BACKGROUND: Cerebral palsy is the most frequent motor disability in childhood, but little is known about its etiology. It has been suggested that cerebral palsy risk may be increased by prenatal thyroid hormone disturbances. The objective of this study was to investigate whether maternal thyroid disorder is associated with increased risk of cerebral palsy. METHODS: A population-based cohort study using two study populations. 1) 1,270,079 children born in Denmark 1979-2007 identified in nationwide registers, and 2) 192,918 children born 1996-2009 recruited into the Danish National Birth Cohort and The Norwegian Mother and Child Cohort study, combined in the MOthers and BAbies in Norway and Denmark (MOBAND) collaboration cohort. Register-based and self-reported information on maternal thyroid disorder was studied in relation to risk of cerebral palsy and its unilateral and bilateral spastic subtypes using multiple logistic regression. Children were followed from the age of 1 year to the age of 6 years, and cerebral palsy was identified in nationwide registers with verified diagnoses. RESULTS: In register data, hypothyroidism was recognized in 12,929 (1.0%), hyperthyroidism in 9943 (0.8%), and unclassifiable thyroid disorder in 753 (< 0.1%) of the mothers. The odds ratio for an association between maternal thyroid disorder and bilateral spastic cerebral palsy was 1.0 (95% CI: 0.7-1.5). Maternal thyroid disorder identified during pregnancy was associated with elevated risk of unilateral spastic cerebral palsy (odds ratio 3.1 (95% CI: 1.2-8.4)). In MOBAND, 3042 (1.6%) of the mothers reported a thyroid disorder in pregnancy, which was not associated with cerebral palsy overall (odds ratio 1.2 (95% CI: 0.6-2.4)). CONCLUSIONS: Maternal thyroid disorder overall was not related to bilateral spastic cerebral palsy, but maternal thyroid disorder identified in pregnancy was associated with increased risk of unilateral spastic cerebral palsy. These findings should be replicated in studies making use of maternal blood samples.

Disease burden in Norway in 2016. / Sykdomsbyrden i Norge i 2016.

BAKGRUNN: For å kunne møte helseutfordringer i befolkningen trenger vi oversikt over befolkningens helsetilstand. I Norge har vi tradisjonelt hatt god oversikt over dødsårsaker, men vi vet mindre om byrden fra tilstander som medfører sykelighet, såkalt ikke-dødelig helsetap. Vårt mål var å beskrive den totale sykdomsbyrden i Norge i 2016, utviklingen de siste ti årene samt kjønnsforskjeller i sykdomsbyrde. MATERIALE OG METODE: Vi brukte resultater fra det globale sykdomsbyrdeprosjektet Global Burden of Diseases, Injuries and Risk Factors Study (GBD), som kvantifiserer ikke-dødelig helsetap slik at det kan måles på samme skala som dødelighet i form av tapte leveår. Summen av tapte leveår og ikke-dødelig helsetap gir sykdomsbyrdemålet helsetapsjusterte leveår (DALY). RESULTATER: Ikke-smittsomme sykdommer som hjerte- og karsykdom, kreft, kronisk obstruktiv lungesykdom og demens var viktige årsaker til tapte leveår hos begge kjønn i Norge i 2016. Ikke-dødelig helsetap utgjorde 52 % av sykdomsbyrden målt i helsetapsjusterte leveår. Spesielt muskel- og skjelettsykdommer, psykiske lidelser og ruslidelser var viktige. De siste ti årene har sykdomsbyrden (i aldersjusterte rater) sunket for mange tilstander som medfører tapte leveår, men ikke for tilstander som gir ikke-dødelig helsetap. FORTOLKNING: Ikke-dødelig helsetap utgjør en stor og økende andel av sykdomsbyrden i den norske befolkningen, noe som vil gi nye utfordringer for helsevesenet.

Intake of Caffeinated Soft Drinks before and during Pregnancy, but Not Total Caffeine Intake, Is Associated with Increased Cerebral Palsy Risk in the Norwegian Mother and Child Cohort Study.

BACKGROUND: Postnatal administration of caffeine may reduce the risk of cerebral palsy (CP) in vulnerable low-birth-weight neonates. The effect of antenatal caffeine exposure remains unknown. OBJECTIVE: We investigated the association of intake of caffeine by pregnant women and risk of CP in their children. METHODS: The study was based on The Norwegian Mother and Child Cohort Study, comprising >100,000 live-born children, of whom 222 were subsequently diagnosed with CP. Mothers reported their caffeine consumption in questionnaires completed around pregnancy week 17 (102,986 mother-child pairs), week 22 (87,987 mother-child pairs), and week 30 (94,372 mother-child pairs). At week 17, participants were asked about present and prepregnancy consumption. We used Cox regression models to estimate associations between exposure [daily servings (1 serving = 125 mL) of caffeinated coffee, tea, and soft drinks and total caffeine consumption] and CP in children, with nonconsumers as the reference group. Models included adjustment for maternal age and education, medically assisted reproduction, and smoking, and for each source of caffeine, adjustments were made for the other sources. RESULTS: Total daily caffeine intake before and during pregnancy was not associated with CP risk. High consumption (≥6 servings/d) of caffeinated soft drinks before pregnancy was associated with an increased CP risk (HR: 1.9; 95% CI: 1.2, 3.1), and children of women consuming 3-5 daily servings of caffeinated soft drinks during pregnancy weeks 13-30 also had an increased CP risk (HR: 1.7; 95% CI: 1.1, 2.8). A mean daily consumption of 51-100 mg caffeine from soft drinks during the first half of pregnancy was associated with a 1.9-fold increased risk of CP in children (HR: 1.9; 95% CI: 1.1, 3.6). CONCLUSIONS: Maternal total daily caffeine consumption before and during pregnancy was not associated with CP risk in children. The observed increased risk with caffeinated soft drinks warrants further investigation.

Associations of maternal atopic diseases with adverse pregnancy outcomes: a national cohort study.

BACKGROUND: Maternal asthma has been associated with adverse pregnancy outcomes. Little is known about the influence of other atopic diseases on pregnancy outcomes. We assessed how various maternal atopic diseases might affect preterm birth, stillbirth, and neonatal death. METHODS: By linking Norwegian national registries, we acquired information on maternal health, socio-demographic factors, pregnancy, birth, and neonatal outcome on all births in Norway from 1967 to 2003. RESULTS: A total of 1 974 226 births were included. Of these, 1.8% had a record of maternal asthma, 3.4% of maternal atopic dermatitis, and 0.4% of maternal allergic rhinoconjunctivitis. Overall rates of preterm birth, stillbirth, and neonatal death were 6.0%, 0.6%, and 0.5%, respectively. After adjustments for possible confounders, maternal asthma was associated with increased risk of preterm birth (relative risk (RR), 1.15, [95% confidence interval (CI) 1.10, 1.21]). In contrast, maternal atopic dermatitis was associated with decreased risk of preterm birth (RR 0.90, [95% CI 0.86, 0.93]), stillbirth (RR 0.70, [95% CI 0.62, 0.79]), and neonatal death (RR 0.76, [95% CI 0.65, 0.90]). Similarly, maternal allergic rhinoconjunctivitis was associated with decreased risk of preterm birth (RR 0.84, [95% CI 0.76, 0.94]) and stillbirth (RR 0.40, [95% CI 0.25, 0.66]). CONCLUSIONS: We confirmed the previously reported association of maternal asthma with increased risk for preterm birth. Unexpectedly, maternal atopic dermatitis and allergic rhinoconjunctivitis were associated with decreased risk of preterm birth and stillbirth. Mechanisms for these protective associations are unclear, and our findings require confirmation in further studies.

Porphyrias in Norway.

BACKGROUND: Porphyria is an umbrella term for a group of largely hereditary diseases that are due to defective haem synthesis. The diseases have a varied and partly overlapping range of symptoms and presentations. The commonest forms of porphyria are porphyria cutanea tarda, acute intermittent porphyria and erythropoietic protoporphyria. The purpose of this study is to provide an overview of the prevalence and pathological manifestations of porphyrias in Norway. MATERIAL AND METHOD: Information on all patients registered with the Norwegian Porphyria Centre (NAPOS) up to 2012 was used to estimate the prevalence and incidence of porphyrias in Norway. Figures on symptoms, precipitating factors and follow-up routines were obtained from the Norwegian Porphyria Registry, which includes 70% of Norwegians registered with NAPOS as having porphyria. RESULTS: The prevalence of porphyria cutanea tarda was approximately 10 : 100,000 and that of acute intermittent porphyria approximately 4 : 100,000. The total incidence of all porphyrias was approximately 0.5-1 : 100,000 per year. Diagnostic delay, i.e. the time passing between the onset of symptoms and diagnosis, varied from 1-17 years depending on the type of porphyria. There was wide variation in the frequency with which patients with the various types of porphyria went for medical check-ups. INTERPRETATION: The prevalence of acute intermittent porphyria and porphyria cutanea tarda appears to be higher in Norway than in most other countries. Data from the Norwegian Porphyria Registry makes it possible to demonstrate differences in treatment and follow-up of porphyria patients and may be used to initiate necessary measures.

Porphyria cutanea tarda and patterns of long-term sick leave and disability pension: a 24-year nationwide matched-cohort study.

BACKGROUND: Porphyria cutanea tarda (PCT) is a skin disorder caused by a defect in the liver enzyme uroporphyrinogen decarboxylase and is associated with hepatitis C virus infection, high alcohol intake, smoking and iron overload. Data on the long-term morbidity of PCT is lacking. METHODS: We conducted a nationwide matched cohort study over a 24-year period. The study sample included 534 persons aged 18-67 years with a biochemically confirmed PCT diagnosis and a sample of 21,360 persons randomly selected from the working age population, matched on age, sex and educational attainment. We investigated if persons with sporadic and familial PCT had an increased risk of long-term sick leave (LTSL) or disability pension. We further assessed risk before (pre-PCT), during (during-PCT) and after (post-PCT) the typical period of first onset to diagnosis, treatment and remission. RESULTS: Overall, persons with PCT had a 40% increased risk (hazard ratio [HR] = 1.4, 95% confidence interval [CI] = 1.3, 1.5) of LTSL and a 50% increased risk (HR = 1.5, CI = 1.3, 1.7) of disability pension. Risk of disability pension was increased pre-PCT (HR = 1.3, CI 1.3 (1.0, 1.6), during-PCT (HR 1.5, CI 1.0, 2.2) and post-PCT (HR = 2.0, CI 1.5, 2.6). For LTSL, risk was increased pre-PCT (HR = 1.3, CI 1.1, 1.4) and during-PCT (HR = 1.5, CI 1.1, 2.1), but not post-PCT. Risk was greatest in persons with sporadic than familial PCT. Diagnostic reasons for disability pension that were increased compared to matched controls were PCT or skin disease in 11 of 199 cases (PCT: n = 7, incident rate ratios [IRR] = 49.2, CI = 38.8, 62.4; diseases of the skin and subcutaneous tissue, n = 4, IRR = 4.2, CI = 1.6, 11.0). The vast majority of diagnostic reasons for accessing disability pension were related to comorbidities, PCT susceptibility factors and more general health issues such as: malignant neoplasms (n = 12, IRR = 2.4, CI = 1.4, 4.2), substance and alcohol dependence (n = 7, IRR = 5.0, CI = 2.5, 10.1), neurotic and mood-disorders (n = 21, IRR = 1.7, CI = 1.1, 2.6), and diseases of the musculoskeletal system and connective tissue (n = 71, IRR = 2.5, CI = 1.9, 3.2). CONCLUSIONS: Persons with PCT have an increased risk of LTSL and disability pension indicating significant morbidity in this patient group. Appropriate long-term follow-up and monitoring for relapses and co-morbid diseases are recommended.

Excess risk of adverse pregnancy outcomes in women with porphyria: a population-based cohort study.

The porphyrias comprise a heterogeneous group of rare, primarily hereditary, metabolic diseases caused by a partial deficiency in one of the eight enzymes involved in the heme biosynthesis. Our aim was to assess whether acute or cutaneous porphyria has been associated with excess risks of adverse pregnancy outcomes. A population-based cohort study was designed by record linkage between the Norwegian Porphyria Register, covering 70% of all known porphyria patients in Norway, and the Medical Birth Registry of Norway, based on all births in Norway during 1967-2006. The risks of the adverse pregnancy outcomes preeclampsia, delivery by caesarean section, low birth weight, premature delivery, small for gestational age (SGA), perinatal death, and congenital malformations were compared between porphyric mothers and the rest of the population. The 200 mothers with porphyria had 398 singletons during the study period, whereas the 1,100,391 mothers without porphyria had 2,275,317 singletons. First-time mothers with active acute porphyria had an excess risk of perinatal death [adjusted odds ratio (OR) 4.9, 95% confidence interval (CI) 1.5-16.0], as did mothers with the hereditable form of porphyria cutanea tarda (PCT) (3.0, 1.2-7.7). Sporadic PCT was associated with an excess risk of SGA [adjusted relative risk (RR) 2.0, 1.2-3.4], and for first-time mothers, low birth weight (adjusted OR 3.4, 1.2-10.0) and premature delivery (3.5, 1.2-10.5) in addition. The findings suggest women with porphyria should be monitored closely during pregnancy.

Evaluation of 32 rapid tests for detection of antibodies against SARS-CoV-2.

AIMS: To evaluate the analytical performance of 32 rapid tests for detection of antibodies against coronavirus SARS-CoV-2. MATERIALS AND METHODS: We used at total of 262 serum samples (197 pre-pandemic and 65 convalescent COVID-19), and three criteria to evaluate the rapid tests under standardized and optimal conditions: (i) Immunoglobulin G (IgG) specificity "good" if lower limit of the 95% confidence interval was ≥ 97.0%, "acceptable" if point estimate was ≥ 97.0%, otherwise "not acceptable". (ii) IgG sensitivity "good" if point estimate was ≥ 90.0%, "acceptable" if ≥ 85.0%, otherwise "not acceptable". (iii) User-friendliness "not acceptable" if complicated to perform or difficult to read result, otherwise "good". We also included partial evaluations of three automated immunoassay systems. RESULTS: Sensitivity and specificity varied considerably; IgG specificity between 90.9% (85.9-94.2) and 100% (97.7-100.0), and IgG sensitivity between 53.8% (41.9-65.4) and 98.5% (91.0-100.0). Combining our evaluation criteria, none of the 28 rapid tests that detected IgG had an overall performance considered "good", seven tests were considered "acceptable", while 21 tests were considered "not acceptable". Four tests detected only total antibodies and were not given an overall evaluation. IgG sensitivity and/or specificity of the automated immunoassays did not exceed that of many rapid tests. CONCLUSION: When prevalence is low, the most important analytical property is a test's IgG specificity, which must be high to minimize false positive results. Out of 32 rapid tests, none had a performance classified as "good", but seven were classified as "acceptable".