Testing the feasibility of a National Spina Bifida Patient Registry

BACKGROUND

The purpose of this study was to describe the development and early implementation of a national spina bifida (SB) patient registry, the goal of which is to monitor the health status, clinical care, and outcomes of people with SB by collecting and analyzing patient data from comprehensive SB clinics.

METHODS

Using a web‐based, SB‐specific electronic medical record, 10 SB clinics collected health‐related information for patients diagnosed with myelomeningocele, lipomyelomeningocele, fatty filum, or meningocele. This information was compiled and de‐identified for transmission to the Centers for Disease Control and Prevention (CDC) for quality control and analysis.

RESULTS

A total of 2070 patients were enrolled from 2009 through 2011: 84.9% were younger than 18 years of age; 1095 were women; 64.2% were non‐Hispanic white; 6.5% were non‐Hispanic black or African American; and 24.2% were Hispanic or Latino. Myelomeningocele was the most common diagnosis (81.5%).

CONCLUSIONS

The creation of a National Spina Bifida Patient Registry partnership between the CDC and SB clinics has been feasible. Through planned longitudinal data collection and the inclusion of additional clinics, the data generated by the registry will become more robust and representative of the population of patients attending SB clinics in the United States and will allow for the investigation of patient outcomes. Birth Defects Research (Part A), 2013. © 2012 Wiley Periodicals, Inc.     

Mast cell tumours and other skin neoplasia in Danish dogs - data from the Danish Veterinary Cancer Registry

Background  

The Danish Veterinary Cancer Registry (DVCR) was established in May 2005 to gather information about neoplasms in the Danish dog and cat populations. Practitioners from more than 60 clinics throughout Denmark have submitted data on these species. The objectives of the current study were, with a special focus on mast cell tumours (MCT) to investigate the occurrence, gender distribution, biological behaviour, locations, types, the diagnostic method used and treatment of skin neoplasms in dogs based on information reported to the DVCR.     

Low Incidence of Livestock-Associated Methicillin-Resistant Staphylococcus aureus Bacteraemia in The Netherlands in 2009

Methicillin-resistant Staphylococcus aureus (MRSA) is a worldwide problem in both hospitals and communities all over the world. In 2003, a new MRSA clade emerged with a reservoir in pigs and veal calves: livestock-associated MRSA (LA-MRSA). We wanted to estimate the incidence of bacteraemias due to LA-MRSA using national surveillance data from 2009 in the Netherlands. We found a low incidence of LA-MRSA and MRSA bacteraemia episodes, compared to bacteraemias caused by all S. aureus (0.04, 0.18 and 19.3 episodes of bacteraemia per 100,000 inhabitants per year, respectively). LA-MRSA and MRSA were uncommon compared to numbers from other countries as well. MRSA in general and LA-MRSA in specific does not appear to be a public health problem in the Netherlands now. The low incidence of LA-MRSA bacteraemia episodes may best be explained by differences in the populations affected by LA-MRSA versus other MRSA. However, reduced virulence of the strain involved, and the effectiveness of the search and destroy policy might play a role as well.     

Defining smallness for gestational age in the early years of the Danish Medical Birth Registry

Background

Being born small for gestational age (SGA) is associated with decreased insulin sensitivity and increased blood pressure in childhood, but the association with clinical disease in early adulthood is less certain. The Danish Medical Birth Registry has registered all births in Denmark since 1973, but due to variable data quality, data is most often used only from 1981 onwards, and birth registers in other countries may have similar problems for the early years. We wanted to examine whether the data can be used for identification of children born SGA and used in future research.

Methodology/Principal Findings

All persons born between 1974 and 1996 were identified in the Danish Medical Birth Registry (n = 1.704.890). Immigrants and children without data on gestational age and birth weight were excluded, and a total of 1.348.106 children were included in the analysis. The difference between the different variables used in the history of the registry were examined, and the quality of data in the birth registry from 1974-1981 was examined and compared to subsequent years.Data on birth weight and gestational age in the early years of the registry is inconsistent, and the identification of children born SGA is inaccurate, with 49% false-positives. The biggest source of error is due to the rough and inaccurate intervals used for gestational age. By using –3 standard deviations as a cut-off for the identification of children born SGA, the number of false-positives was reduced to 9%, while the amount of false-negatives were increased.

Conclusion

Choosing –3 standard deviations for identifying children born SGA is a viable, though not optimal solution for identifying children born SGA. Overall the data in the registry is of sufficient quality to be used in further medical research.     

Oral malignant melanomas and other head and neck neoplasms in Danish dogs - data from the Danish Veterinary Cancer Registry

Background  

Head and neck cancers (HNC) are relatively common and often very serious diseases in both dogs and humans. Neoplasms originating in the head and neck region are a heterogeneous group. HNC often has an unfavourable prognosis and the proximity of the tissue structures renders extirpation of tumours with sufficient margins almost incompatible with preservation of functionality. In humans oral malignant melanoma (OMM) is extremely rare, but represents a particular challenge since it is highly aggressive as is the canine counterpart, which thus may be of interest as a spontaneous animal model.     

The Danish Twin Registry: 127 birth cohorts of twins.

The Danish Twin Registry is the oldest national twin register in the world, initiated in 1954 by ascertainment of twins born from 1870 to 1910. During a number of studies birth cohorts have been added to the register, and by the recent addition of birth cohorts from 1931 to 1952 the Registry now comprizes 127 birth cohorts of twins from 1870 to 1996, with a total of more than 65,000 twin pairs included. In all cohorts the ascertainment has been population-based and independent of the traits studied, although different procedures of ascertainment have been employed. In the oldest cohorts only twin pairs with both twins surviving to age 6 have been included while from 1931 all ascertained twins are included. The completeness of the ascertainment after adjustment for infant mortality is high, with approximately 90% ascertained up to 1968, and complete ascertainment of all liveborn twin pairs since 1968. The Danish Twin Registry is used as a source for large studies on genetic influence on aging and age-related health problems, normal variation in clinical parameters associated with the metabolic syndrome and cardiovascular diseases, and clinical studies of specific diseases. The combination of survey data with data obtained by linkage to national health related registers enables follow-up studies both of the general twin population and of twins from clinical studies.     

New cases of melanoma as documented in the National Cancer Registry

The authors briefly survey general aspects of crucial importance for the proper functioning of the National Cancer Registry, such as legislation, collected data, identification of patients, the completeness and validity of its content in relation to the results and conclusions of a comprehensive, national supervision performed after the Melanoma Consensus Conference. Compared to earlier national controlling attempts, the present supervision was highly successful: doctors of various hospitals did perform the detailed control of diagnosis in 95.81% of 1361 melanoma patients announced in 2001. They checked whether patients given the C43 BNO code had melanoma indeed, searched for those who received a different BNO code and identified those not announced for any reason to the Registry. After correction the Registry included 1117 new cases of melanoma in 2001. The authors state that the conclusions from this supervision may enhance the reliability not only of the data base of the Registry but that of the hospitals as well.     

Familial recurrence-pattern analysis of nonsyndromic isolated cleft palate--a Danish Registry study.

The finding of an association between genetic variation at the transforming growth-factor alpha (TGFA) locus and nonsyndromic isolated cleft palate (CP) represents a potentially important breakthrough in our understanding of this condition. The present study was undertaken to assess the feasibility of detecting linkage to putative CP-susceptibility loci, such as TGFA. To this end, the familial recurrence pattern for CP was evaluated to determine the most likely mode of inheritance for this condition. The study took advantage of the high ascertainment and uniform registration of CP in Denmark. In addition, the study utilized estimates of familial recurrence that were obtained by register linkage and, hence, were not subject to either recall bias or the potentially biasing influence of nonresponders. The recurrence risks for first-, second-, and third-degree relatives of 1,364 nonsyndromic CP probands were estimated to be 2.74% (72/2,628), 0.28% (3/1,068), and 0.00% (0/360), respectively. These estimates are close to published estimates based on questionnaire and interview data. The population prevalence for nonsyndromic CP was, however, found to be considerable higher than usually reported (0.058% [1,456/2,523,023]). Analyses of these and previously published data, using the method presented by Risch, indicated that major-locus or additive multilocus inheritance of CP is unlikely. The familial recurrence pattern was, however, consistent with CP being determined by several interacting loci. Under such a model, a single locus accounting for more than a sixfold increase in the risk to first-degree relatives of CP probands is unlikely, whereas a single locus accounting for a threefold increase provided a good fit to the data. Such a locus could be detected in a realistic sample of affected sib pairs.     

Physical activity patterns using accelerometry in the National Weight Control Registry

The National Weight Control Registry (NWCR) was established in 1993 to examine characteristics of successful weight‐loss maintainers. This group consistently self‐reports high levels of physical activity. The aims of this study were to obtain objective assessments of physical activity in NWCR subjects and compare this to physical activity in both normal‐weight and overweight controls. Individuals from the NWCR (n = 26) were compared to a never obese normal‐weight control group matched to the NWCR group's current BMI (n = 30), and an overweight control group matched to the NWCR group's self‐reported pre‐weight‐loss BMI (n = 34). Objective assessment of physical activity was obtained for a 1‐week period using a triaxial accelerometer. Bouts of moderate‐to‐vigorous physical activity (MVPA) ≥10 min in duration, as well as nonbout MVPA (bouts of MVPA 1–9 min in duration) were summed and characterized. NWCR subjects spent significantly (P = 0.004) more time per day in sustained bouts of MVPA than overweight controls (41.5 ± 35.1 min/day vs. 19.2 ± 18.6 min/day) and marginally (P = 0.080) more than normal controls (25.8 ± 23.4). There were no significant differences between the three groups in the amount of nonbout MVPA. These results provide further evidence that physical activity is important for long‐term maintenance of weight loss and suggest that sustained volitional activity (i.e., ≥10 min in duration) may play an important role. Interventions targeting increases in structured exercise may be needed to improve long‐term weight‐loss maintenance.     

Staphylococcus aureus Bacteraemia in a Tropical Setting: Patient Outcome and Impact of Antibiotic Resistance

Background

Most information on invasive Staphylococcus aureus infections comes from temperate countries. There are considerable knowledge gaps in epidemiology, treatment, drug resistance and outcome of invasive S. aureus infection in the tropics.

Methods

A prospective, observational study of S. aureus bacteraemia was conducted in a 1000-bed regional hospital in northeast Thailand over 1 year. Detailed clinical data were collected and final outcomes determined at 12 weeks, and correlated with antimicrobial susceptibility profiles of infecting isolates.

Principal Findings

Ninety-eight patients with S. aureus bacteraemia were recruited. The range of clinical manifestations was similar to that reported from temperate countries. The prevalence of endocarditis was 14%. The disease burden was highest at both extremes of age, whilst mortality increased with age. The all-cause mortality rate was 52%, with a mortality attributable to S. aureus of 44%. Methicillin-resistant S. aureus (MRSA) was responsible for 28% of infections, all of which were healthcare-associated. Mortality rates for MRSA and methicillin-susceptible S. aureus (MSSA) were 67% (18/27) and 46% (33/71), respectively (p = 0.11). MRSA isolates were multidrug resistant. Only vancomycin or fusidic acid would be suitable as empirical treatment options for suspected MRSA infection.

Conclusions

S. aureus is a significant pathogen in northeast Thailand, with comparable clinical manifestations and a similar endocarditis prevalence but higher mortality than industrialised countries. S. aureus bacteraemia is frequently associated with exposure to healthcare settings with MRSA causing a considerable burden of disease. Further studies are required to define setting-specific strategies to reduce mortality from S. aureus bacteraemia, prevent MRSA transmission, and to define the burden of S. aureus disease and emergence of drug resistance throughout the developing world.     

Long-Term Weight Loss and Breakfast in Subjects in the National Weight Control Registry

Objective: To examine breakfast consumption in subjects maintaining a weight loss in the National Weight Control Registry (NWCR). Research Methods and Procedures: A cross-sectional study in which 2959 subjects in the NWCR completed demographic and weight history questionnaires as well as questions about their current breakfast consumption. All subjects had maintained a weight loss of at least 13.6 kg (30 lb) for at least 1 year; on average these subjects had lost 32 kg and kept it off for 6 years. Results: A large proportion of NWCR subjects (2313 or 78%) reported regularly eating breakfast every day of the week. Only 114 subjects (4%) reported never eating breakfast. There was no difference in reported energy intake between breakfast eaters and non-eaters, but breakfast eaters reported slightly more physical activity than non-breakfast eaters (p = 0.05). Discussion: Eating breakfast is a characteristic common to successful weight loss maintainers and may be a factor in their success.     

Incidence of Otitis Media in a Contemporary Danish National Birth Cohort

Objectives

In recent years welfare in Denmark has increased which might be expected to reduce otitis media (OM) incidence. We examined the age-specific incidence of OM in a nation-wide cohort of children aged 0–7 years born in 1996–2003 (Danish National Birth Cohort, DNBC). Only selection was ability to understand and speak Danish.

Methods

Information of OM and ventilation tubes (VT) was collected through three maternal interviews at 6-month, 18-month and 7-years of age and based on this age-specific and cumulative incidence of OM was calculated. As different numbers of the total population answered the different interviews, the calculations are done with different denominators. The information in DNBC was validated against two population based registries containing information of VT insertions.

Results

Cumulative incidence of OM at 7 years was 60.6% (31,982/52,755). For children with OM, 16.2% (7143/44194) had their first OM episodes between 0–6 months of age, 44.3% (19579/44194) between 7–18 months, and 39.5% (17472/44194) between 19 months and 7 years. Four or more OM episodes before 7 years were reported by 39.5% (12620/31982) and by 64.0% (2482/3881) of those who had their OM debut between 0–6 months; by 48.2% (4998/10378) with debut between 7–18 months; and by 28.7% (4996/17344) with debut between 19 months and 7 years. These figures are essentially unchanged from earlier figures from Denmark. VT insertion at least once was reported by 26,1% in the 7-year interview. Assuming recordings in the Danish National Patient Registry to be gold standard, maternal self-reportings in DNBC of insertion of VT showed high sensitivity (96.4%), specificity (98.2%), and positive (94.8%) and negative predictive values (98.8%).

Conclusion

OM affects nearly 2/3 of preschool children in Denmark despite reduction in known OM risk factors.     

Causes of death in men with prostate cancer: Results from the Danish Prostate Cancer Registry (DAPROCAdata)

BackgroundCurrent knowledge of the validity of registry data on prostate cancer-specific death is limited. We aimed to determine the underlying cause of death among Danish men with prostate cancer, to estimate the level of misattribution of prostate cancer death, and to examine the risk of death from prostate cancer when accounting for competing risk of death.Material and methodsWe investigated a nationwide cohort of 15,878 prostate cancer patients diagnosed in 2010–2014; with 3343 deaths occurring through 2016. Blinded medical chart review was carried out for 670 deaths and compared to the national cause of death registry. Five death categories were defined: 1) prostate cancer-specific death, 2) other unspecified urological cancer death, 3) other cancer death 4) cardiovascular disease death, and 5) other causes of death. Competing risk analyses compared Cox cause-specific and Fine-Gray regression models.ResultsChart review attributed 51.2% of deaths to prostate cancer, 17.0% to cardiovascular disease, and 16.7% to other causes. The Danish Register of Causes of Death attributed 71.7% of deaths to prostate cancer when including all registered contributing causes of death, and 57.0% of deaths when including only the primary registered cause of death. The probability of death by prostate cancer was 10% at 2-year survival.ConclusionsMore than half of the deceased men in our study cohort died of their prostate cancer disease within a mean of 2.4 years of follow up. Data from the death registry is prone to misclassification, potentially overestimating the proportion of deaths from prostate cancer.     

The French National Registry of patients with Facioscapulohumeral muscular dystrophy

Background

Facioscapulohumeral muscular dystrophy is a rare inherited neuromuscular disease with an estimated prevalence of 1/20,000 and France therefore harbors about 3000 FSHD patients. With research progress and the development of targeted therapies, patients’ identification through registries can facilitate and improve recruitment in clinical trials and studies.

Results

The French National Registry of FSHD patients was designed as a mixed model registry involving both patients and physicians, through self-report and clinical evaluation questionnaires respectively, to collect molecular and clinical data. Because of the limited number of patients, data quality is a major goal of the registry and various automatic data control features have been implemented in the bioinformatics system. In parallel, data are manually validated by molecular and clinical curators. Since its creation in 2013, data from 638 FSHD patients have been collected, representing about 21% of the French FSHD population. The mixed model strategy allowed to collect 59.1% of data from both patients and clinicians; 26 and 14.9% from respectively patients and clinicians only. With the identification of the FSHD1 and FSHD2 forms, specific questionnaires have been designed. Though FSHD2 patients are progressively included, FSHD1 patients still account for the majority (94.9%). The registry is compatible with the FAIR principles as data are Findable, Accessible and Interoperable. We thus used molecular standards and standardized clinical terms used by the FILNEMUS French network of reference centers for the diagnosis and follow-up of patients suffering from a rare neuromuscular disease. The implemented clinical terms mostly map to dictionaries and terminology systems such as SNOMED-CT (75% of terms), CTV3 (61.7%) and NCIt (53.3%). Because of the sensitive nature of data, they are not directly reusable and can only be accessed as aggregated data after evaluation and approval by the registry oversight committee.

Conclusions

The French National Registry of FSHD patients belongs to a national effort to develop databases, which should now interact with other initiatives to build a European and/or an international FSHD virtual registry for the benefits of patients. It is accessible at www.fshd.fr and various useful information, links, and documents, including a video, are available for patients and professionals.

     

Cancer incidence in Iran in 2014: Results of the Iranian National Population-based Cancer Registry

BackgroundWe aimed to report, for the first time, the results of the Iranian National Population-based Cancer Registry (INPCR) for the year 2014.MethodsTotal population of Iran in 2014 was 76,639,000. The INPCR covered 30 out of 31 provinces (98% of total population). It registered only cases diagnosed with malignant new primary tumors. The main sources for data collection included pathology center, hospitals as well as death registries. Quality assessment and analysis of data were performed by CanReg-5 software. Age standardized incidence rates (ASR) (per 100,000) were reported at national and subnational levels.ResultsOverall, 112,131 new cancer cases were registered in INPCR in 2014, of which 60,469 (53.9%) were male. The diagnosis of cancer was made by microscopic confirmation in 76,568 cases (68.28%). The ASRs of all cancers were 177.44 and 141.18 in male and female, respectively. Cancers of the stomach (ASR = 21.24), prostate (18.41) and colorectum (16.57) were the most common cancers in men and the top three cancers in women were malignancies of breast (34.53), colorectum (11.86) and stomach (9.44). The ASR of cervix uteri cancer in women was 1.78. Our findings suggested high incidence of cancers of the esophagus, stomach and lung in North/ North West of Iran.ConclusionOur results showed that Iran is a medium-risk area for incidence of cancers. We found differences in the most common cancers in Iran comparing to those reported for the World. Our results also suggested geographical diversities in incidence rates of cancers in different subdivisions of Iran.     

Characterization of Dietary Patterns in the Danish National Birth Cohort in Relation to Preterm Birth

Background

Dietary patterns better reflect eating habits as opposed to single dietary components. However, the use of dietary pattern analysis in nutritional epidemiology has been hampered by the complexity of interpreting and presenting multidimensional dietary data.

Methods

This study extracts and visualizes dietary patterns from self-reported dietary data collected in mid-pregnancy (25^th week of gestation) from nearly 60,000 mother-child pairs part of a prospective, longitudinal cohort (Danish National Birth Cohort) and further examines their associations with spontaneous and induced preterm birth (gestational age<259 days (<37 weeks)).

Results

A total of seven dietary patterns were extracted by principal component analysis, characterized and visualized by color-coded spider plots, and referred to as: Vegetables/Prudent, Alcohol, Western, Nordic, Seafood, Candy and Rice/Pasta/Poultry. A consistent dose-response association with preterm birth was only observed for Western diet with an odds ratio of 1.30 (95% CI: 1.13, 1.49) comparing the highest to the lowest quintile. This association was primarily driven by induced preterm deliveries (odds ratio = 1.66, 95% CI: 1.30, 2.11, comparing the highest to the lowest quintile) while the corresponding odds ratio for spontaneous preterm deliveries was more modest (odds ratio = 1.18, 95% CI: 0.99, 1.39). All based on adjusted analyses.

Conclusions

In conclusion, this study presented a simple and novel framework for visualizing correlation structures between overall consumption of foods group and their relation to nutrient intake and maternal characteristics. Our results suggest that Western-type diet, high in meat and fats and low in fruits and vegetables, is associated with increased odds of induced preterm birth.     

Cost-Effectiveness of a National Initiative to Improve Hand Hygiene Compliance Using the Outcome of Healthcare Associated Staphylococcus aureus Bacteraemia

Background

The objective is to estimate the incremental cost-effectiveness of the Australian National Hand Hygiene Inititiave implemented between 2009 and 2012 using healthcare associated Staphylococcus aureus bacteraemia as the outcome. Baseline comparators are the eight existing state and territory hand hygiene programmes. The setting is the Australian public healthcare system and 1,294,656 admissions from the 50 largest Australian hospitals are included.

Methods

The design is a cost-effectiveness modelling study using a before and after quasi-experimental design. The primary outcome is cost per life year saved from reduced cases of healthcare associated Staphylococcus aureus bacteraemia, with cost estimated by the annual on-going maintenance costs less the costs saved from fewer infections. Data were harvested from existing sources or were collected prospectively and the time horizon for the model was 12 months, 2011–2012.

Findings

No useable pre-implementation Staphylococcus aureus bacteraemia data were made available from the 11 study hospitals in Victoria or the single hospital in Northern Territory leaving 38 hospitals among six states and territories available for cost-effectiveness analyses. Total annual costs increased by $2,851,475 for a return of 96 years of life giving an incremental cost-effectiveness ratio (ICER) of $29,700 per life year gained. Probabilistic sensitivity analysis revealed a 100% chance the initiative was cost effective in the Australian Capital Territory and Queensland, with ICERs of $1,030 and $8,988 respectively. There was an 81% chance it was cost effective in New South Wales with an ICER of $33,353, a 26% chance for South Australia with an ICER of $64,729 and a 1% chance for Tasmania and Western Australia. The 12 hospitals in Victoria and the Northern Territory incur annual on-going maintenance costs of $1.51M; no information was available to describe cost savings or health benefits.

Conclusions

The Australian National Hand Hygiene Initiative was cost-effective against an Australian threshold of $42,000 per life year gained. The return on investment varied among the states and territories of Australia.