The aim was to investigate triggering factors and insulin pump usage (continuous subcutaneous insulin infusion, CSII) at diabetic ketoacidosis (DKA). Data from 1999 and 2000 were collected retrospectively from Sweden. In 1999 and 2000, 7.4 and 11.0%, respectively, of children with diabetes used CSII. One hundred and forty-two episodes of DKA (pH
To investigate, in a population-based cohort of patients with juvenile chronic arthritis (JCA), onset characteristics, progression, outcome, and prognostic factors longitudinally for 5 years.
This cohort consisted of 132 incidence cases identified between 1984 and 1986 in southwestern Sweden followed for 5 years with annual reports of subgroup, joint assessment, disease activity, eye examinations, laboratory measurements, and medication. At the 5-year follow-up, the Childhood Health Assessment Questionnaire (Child-HAQ) was evaluated. European League Against Rheumatism (EULAR) criteria for diagnosis and disease activity were used.
During the 5 years only four patients were lost to follow-up, 34% changed subgroup and 8% developed uveitis. At the 5-year follow-up the disease was active in 12% of the patients, stable in 28%, inactive in 25%, and in remission in 34%. Among those examined, 24% had radiological changes, of whom half had advanced changes. The Child-HAQ median score at the 5-year follow-up was 0.13 (range 0.0-1.9). The number of involved joints at inclusion correlated positively with active disease at the 5-year follow-up. Age at disease onset, the number of involved joints, and the number of joints with arthritis correlated positively with continuous disease and Child-HAQ score. CONCLUSION. Our study shows a diverse disease course during the first 5 years of JCA where one-third changed subgroup and two-thirds did not reach remission. Age of disease onset, the number of involved joints, and the number of joints with arthritis at inclusion were associated with poor outcome at the 5-year follow-up.
Symmetric non-erosive polyarthritis is the most common clinical feature in systemic lupus erythematosus (SLE). We report on a 42-year follow-up of a 71-year-old woman who first had polyarthritis in 1963 at the age of 29 and continuously since 1975. SLE was diagnosed in 2000 at the age of 66 as anti-dsDNA (56 kIU/l), and antinuclear antibodies (1:2,560) turned positive. In 2005 hand and feet radiographs revealed severe Jaccoud's arthritis with subluxations but without erosions.
The objective was to test the hypothesis that a described association between homozygosity for a 50bp deletion in the SOD1 promoter 1684bp upstream of the SOD1 ATG and an increased age of onset in SALS can be replicated in additional SALS and control sample sets from other populations. Our second objective was to examine whether this deletion attenuates expression of the SOD1 gene. Genomic DNA from more than 1200 SALS cases from Ireland, Scotland, Quebec and the USA was genotyped for the 50bp SOD1 promoter deletion. Reporter gene expression analysis, electrophoretic mobility shift assays and chromatin immunoprecipitation studies were utilized to examine the functional effects of the deletion. The genetic association for homozygosity for the promoter deletion with an increased age of symptom onset was confirmed overall in this further study (p=0.032), although it was only statistically significant in the Irish subset, and remained highly significant in the combined set of all cohorts (p=0.001). Functional studies demonstrated that this polymorphism reduces the activity of the SOD1 promoter by approximately 50%. In addition we revealed that the transcription factor SP1 binds within the 50bp deletion region in vitro and in vivo. Our findings suggest the hypothesis that this deletion reduces expression of the SOD1 gene and that levels of the SOD1 protein may modify the phenotype of SALS within selected populations.
Investigate the incidence of multiple sclerosis during 1953-2013 and estimate the prevalence rate of MS on 1 January 2003 and 2013 in Hordaland County, Western Norway.
All patients with onset of disease in Hordaland 1953-2013 were identified in files from previous studies until 2003 and from patient records at the departments of Neurology, Haukeland University Hospital and Haugesund Hospital during 2003-2013. 1558 patients were assessed and 1402 of these were included, of whom 1035 were alive and living in Hordaland at prevalence day 1 January 2013. Annual incidence rates were calculated for 1953-2013.
On 1 January 2003, the crude prevalence rate was 191/100 000 population and on 1 January 2013, the crude prevalence rate was 211.4 (95% CI 198.3 to 224.2) per 100 000; 270.9 (95% CI 250.6 to 292.3) for women and 151.8 (95% CI 136.8 to 167.9) for men. Prevalence peaked at ages 55-59 years for women and 60-64 years for men. The annual incidence rate increased from 1.9 (95% CI 1.2 to 2.6) per 100 000 during 1953-1957 to 7.2 (95% CI 6.0 to 8.5) during 1978-1982 and to 8.5 (95% CI 7.3 to 9.7) during 2003-2007, thus indicating a stabilising incidence over the past 35 years. The female/male ratio ranged from 1.2:1 to 1.8:1 (p=0.381) during the period.
Stabilising rather than increasing incidence combined with the stable female/male ratio are indicative of non-fluctuating environmental factors in a geographical area otherwise characterised by lack of vitamin D effective sun exposure. The rising prevalence of MS could result from improved survival and follow-up methodology.
BACKGROUND: Wilson's disease is associated with heavy copper overload, primarily in the liver. Copper is a toxic metal, and might be expected to be associated with cancer induction, as iron is in haemochromatosis. However, liver cancer is currently believed to be extremely rare in this disease, and other intra-abdominal malignancies have not been reported. AIM: To assess the frequency of abdominal malignant disease in patients with Wilson's disease on long-term follow-up. DESIGN: Retrospective study in two specialist Wilson's disease clinics: Cambridge/London and Uppsala. METHODS: We reviewed the case records of 363 patients seen at three centres: Addenbrooke's Hospital, Cambridge, 1955-1987; the Middlesex Hospital, London, 1987-2000; and the University Hospital, Uppsala, Sweden, 1966-2002. Patients were grouped by length of follow-up: 10-19 years; 20-29 years; 30-39 years; and 40 years or more. RESULTS: No cancers were seen in patients followed for
This study aims to: (i) explore the relations between smoking initiation and different profiles of academic achievement trajectories in early to mid-adolescence; and (ii) to investigate whether background characteristics (gender, ethnicity, grade repetition, parental education) and proximal processes (parental practices, extra-curricular involvement) predicted class membership and smoking initiation.
Four-year longitudinal cohort study (7th-10th grade).
Adolescents completed the questionnaires during school hours.
At total of 741 adolescents with no history of smoking in grade 7 participating in the Montreal Adolescent Depression Development Project.
Self-report questionnaires were used to assess predictors and previous smoking in year 1, and smoking initiation by the end of the study. Grade point average (GPA) was obtained twice yearly from school records.
Three academic achievement trajectories were identified and found to differ significantly in rates of smoking initiation: persistently high achievers (7.1% smoking), average achievers (15.1% smokers) and unstable low achievers (49.1% smoking). Further, results showed that general parenting practices and parental education indirectly reduced the likelihood of smoking by reducing the risk of membership in classes with lower GPA.
Adolescents who do well in school are less likely to smoke and it may be cost-effective for smoking prevention to focus on the few (12%) easy to identify unstable low achievers who form 35% of smoking onsets. In addition, as parental support and democratic control reduced the likelihood of poor academic performance, promoting essential generic parenting skills from a young age may also prevent future onsets of smoking in adolescence.
Most epidemiologic studies concerned with Major Depressive Disorder have employed cross-sectional study designs. Assessment of lifetime prevalence in such studies depends on recall of past depressive episodes. Such studies may underestimate lifetime prevalence because of incomplete recall of past episodes (recall bias). An opportunity to evaluate this issue arises with a prospective Canadian study called the National Population Health Survey (NPHS).
The NPHS is a longitudinal study that has followed a community sample representative of household residents since 1994. Follow-up interviews have been completed every two years and have incorporated the Composite International Diagnostic Interview short form for major depression. Data are currently available for seven such interview cycles spanning the time frame 1994 to 2006. In this study, cumulative prevalence was calculated by determining the proportion of respondents who had one or more major depressive episodes during this follow-up interval.
The annual prevalence of MDD ranged between 4% and 5% of the population during each assessment, consistent with existing literature. However, 19.7% of the population had at least one major depressive episode during follow-up. This included 24.2% of women and 14.2% of men. These estimates are nearly twice as high as the lifetime prevalence of major depressive episodes reported by cross-sectional studies during same time interval.
In this study, prospectively observed cumulative prevalence over a relatively brief interval of time exceeded lifetime prevalence estimates by a considerable extent. This supports the idea that lifetime prevalence estimates are vulnerable to recall bias and that existing estimates are too low for this reason.
Two large prospective cohort studies of childhood epilepsy (Nova Scotia and the Netherlands) each developed a statistical model to predict long-term outcome. We sought to evaluate the accuracy of a prognostic model based on the two studies combined.
Analyses with classification tree models and stepwise logistic regression produced predictive models for the combined dataset and the two separate cohorts. The resulting models were then externally validated on the opposite cohort. Remission was defined as no longer receiving daily medication for any length of time at the end of follow-up.
The combined cohorts yielded 1,055 evaluable patients. At the end of follow-up (>or=5 years in >96%), 622 (59%) were in remission. By using the combined data, the classification tree model and the logistic regression model predicted the outcome correctly in approximately 70%. The classification tree model split the data on epilepsy type and age at first seizure. Predictors in the logistic regression model were: seizure number before treatment, age at first seizure, absence seizures, epilepsy types of symptomatic generalized and symptomatic partial, preexisting neurologic signs, intelligence, and the combination of febrile seizures and cryptogenic partial epilepsy. When the prediction models from each cohort were cross-validated on the opposite cohort, the outcome was predicted slightly less accurately than did the model from the combined data.
Based on currently available clinical and EEG variables, predicting the outcome of childhood epilepsy may be difficult and appears to be incorrect in about one of every three patients.