National Disease Register Research Articles

Spread and Development of Alveolar Echinococcosis in Germany, 1992-2018

Alveolar echinococcosis (AE) is a rare parasitosis caused by the pathogen Echinococcus multilocularis. There is an increase in the number of cases in many countries. The aim of the study was to investigate the current prevalence and the change in the geographical distribution pattern. Data were collected retrospectively for the period 1992-2018 using the registered cases in the national disease register for AE in Germany. Statistical analysis was performed using the SAS statistical analysis system version 9.4 (SAS Institute, Cary, N.C., USA). The study population of n=569 patients included n=322 (56,59%) women and n=247 (43,40%) men. The mean average age of patients with alveolar echinococcosis at first presentation was 53,90±17,54 years (median: 56,00 years). The Moran's I test statistic showed a positive spatial autocorrelation for the period 1992-2018 corresponding to a heterogeneous distribution of disease cases in Germany (I=0,4165; Z=10,9591, p=0,001). An increase in age- and sex-specific prevalence could be determined for the entire study period (1992-2018). The overall prevalence in the period 1992-2018 was 0,71 cases per 100,000 population. The determination of the prevalence for the period 1992-2018 resulted in 0,31 cases for men and 0,40 cases for women per 100,000 population. In the period 1992-1996, no AE cases had been registered in 11/16 (68,8%) federal states (Berlin, Brandenburg, Bremen, Hamburg, Mecklenburg-Vorpommern, Rheinland-Pfalz, Saarland, Sachsen, Sachsen-Anhalt, Schleswig-Holstein und Thüringen). The evaluation recently shows an increased occurrence of cases in the federal states of Hessen, Rheinland-Pfalz and Nordrhein-Westfalen. The analysis shows a rise in prevalence and an increasing number of cases outside the classic endemic areas of Baden-Württemberg and Bavaria.

POS1412 IDENTIFYING HIGH-COST DRUGS FOR RARE RHEUMATIC DISEASES IN ROUTINELY COLLECTED NHS DATA. RESULTS FROM A PILOT STUDY OF RITUXIMAB USE IN VASCULITIS USING DATA FROM THE NATIONAL DISEASE REGISTRATION SERVICE AND THE REGISTRATION OF COMPLEX RARE DISEASES-EXEMPLARS IN RHEUMATOLOGY (RECORDER) PROJECT

Background:Understanding real-world usage of high-cost drugs is crucial to support planning, adoption of innovation and reduce unwarranted variation in treatment. Hospital Episode Statistics (HES) contain diagnoses (coded by ICD-10) and procedures/treatments (coded by OPCS) for all daycase or inpatient care in England. However, OPCS codes are not specific for individual drugs, for example X921 (cytokine inhibitors band 1) includes rituximab (RTX) and 15 other drugs.Objectives:We aimed to validate the accurate identification of patients treated with RTX for ANCA-associated vasculitis (AAV) using HES data.Methods:We used data from the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) at Public Health England and their legal permissions (CAG 10-02(d)/2015). We extracted records from HES of all patients treated at two hospitals during financial year 2018/19 who ever had a coded diagnosis of granulomatosis with polyangiitis (GPA, M313), eosinophilic granulomatosis with polyangiitis (EGPA, M301), microscopic polyangiitis (MPA, M317), polyarteritis nodosa (PAN, M300) or arteritis unspecified (I776). Where people had multiple diagnoses of vasculitis, the most specific was considered their diagnosis. Enabled by data sharing agreements, we reviewed hospital records of those patients to validate diagnoses and whether X921 reliably identified RTX. We report the positive predictive value and sensitivity of the coding for X921 and GPA/EGPA/MPA for identifying people with AAV who are treated with RTX.Results:At Trust 1 records ever coded with GPA/EGPA/MPA identified 74 people, 69 of whom had AAV confirmed in their medical notes. Among these 74 patients there were 59 episodes coded with X921 procedure codes, of which 56 correctly identified a RTX infusion given for AAV. A total of 64 RTX infusions were given to people with AAV – 3 missed infusions were X921 procedures in patients who had coded diagnoses of PAN or I776 but never GPA/EGPA/MPA and 5 infusions were not coded as X921.The same analysis at Trust 2 identified 46 people, 44 of whom had AAV confirmed in their medical notes. Among patients identified with AAV there were 17 episodes coded as X921, of which 15 correctly identified a RTX infusion. A total of 23 infusions were given to people with AAV: 4 infusions were X921 procedures in patients who had coded diagnoses of PAN or I776 but never GPA/EGPA/MPA, and 4 infusions were not coded as X921.Table 1. Summary of Positive Predictive Values (PPV) applying our algorithm to identify AAV diagnoses and RTX useTrust 1Trust 2CombinedDiagnosis of AAV and coded as AAV6944113AAV coded7446120Diagnosis of AAV under any code7355128PPV AAV ascertainment (95% CI)93.2% (84.9-97.8)95.7% (85.2-99.5)94.2% (88.4-97.6)Sensitivity of AAV ascertainment (95% CI)94.5% (86.8-98.5)80.0% (67.0-89.6)88.3% (81.4-93.3)RTX given in people coded as AAV561571RTX coded in people coded as AAV591776RTX given for AAV under any diagnostic or procedure code642387PPV RTX ascertainment (95% CI)94.9% (85.9-98.9)88.2% (63.6-98.5)93.4% (85.3-97.8)Sensitivity of RTX ascertainment (95% CI)87.5% (76.8-94.4)65.2% (42.7-83.6)81.6% (71.9-89.1)Conclusion:HES data identified patients treated with RTX for AAV with a PPV of 93.4% (85.3-97.8) and sensitivity of 81.6% (71.9-89.1). This demonstrates the utility of national data to identify people receiving RTX for AAV. The RECORDER project, within the National Disease Registration Service plans to conduct real-world studies of the high-cost drug RTX, given for AAV, across the whole of England, and assess whether geography, demographics or socioeconomic factors influence frequency of prescription of this, and potentially other, high-cost drugs in line with the NHS long term plan.

P091 Can use of high cost drugs for rare rheumatic diseases be reliably identified within routinely collected NHS data? Results from a pilot study of rituximab use in vasculitis using data from the National Disease Registration Service (NDRS) and the Registration of Complex Rare Diseases - Exemplars in Rheumatology (RECORDER) project

Abstract Background/Aims Understanding real-world usage of high cost drugs and subsequent outcomes are crucial to support high quality care, adoption of innovation, and reduce unwarranted variation in treatment. Hospital Episode Statistics (HES) contain diagnoses (coded by ICD-10) and procedures/treatment (coded by OPCS) for admissions in England. However, OPCS codes are not specific for individual drugs, for example X921 (cytokine inhibitors band 1) includes rituximab and 15 other drugs. We aimed to validate the accurate identification of patients treated with rituximab for ANCA-associated vasculitis (AAV) using HES data. Methods We used data available to the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS), part of NDRS at Public Health England and their legal permissions (CAG 10-02(d)/2015). We extracted from HES all patients treated at one hospital who ever had a coded diagnosis of either granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa or 'arteritis unspecified’, which is applied by coders to several clinical terms including “systemic vasculitis” and “ANCA associated vasculitis”. Enabled by data sharing agreements, we reviewed hospital records of those patients who had a Finished Consultant Episode (FCE) during 2018/19, to validate diagnoses and whether X921 reliably identified rituximab. Results Initially using codes only for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, or microscopic polyangiitis we identified 65 people, 60 of whom had AAV confirmed in their medical notes (Positive predictive value (PPV) 92.3%; 95% CI 83.0-97.5). When we expanded our search to include in addition people with codes for polyarteritis nodosa or ‘arteritis unspecified’ who had rheumatology or renal follow up, we identified an additional 13 people with AAV and 10 who had other types of systemic vasculitis (PPV for AAV 79.5, 95% CI 69.6-87.4; PPV for systemic vasculitis 90.9%, 95% CI 82.9-96.0). Among patients identified only by the specific ICD-10 codes for the 3 subtypes of AAV there were 51 episodes coded as X921, of which 50 correctly identified a rituximab infusion (and 1 was tocilizumab). No additional infusions for these patients were missed by the coding. The PPV was 98.0% (95% CI 89.6-100). Among patients identified by the more inclusive algorithm, there were 61 episodes coded as X921 in that year. Of these, 59 were for rituximab treatment for AAV and an additional 4 rituximab infusions occurred but were not coded. The PPV was 96.7% (95% CI 88.7-99.6), and the sensitivity was 93.7% (95% CI 84.5-98.2). Conclusion This pilot study demonstrates how the use of novel algorithms within the NDRS RECORDER project can enable the identification and registration of people with AAV and their high-cost treatment at whole-population level. Further work is now underway to study this national cohort, and to apply this methodology to other rare autoimmune diseases and high cost drugs. Disclosure M. Chakravorty: None. F. Pearce: Grants/research support; Recipient of research grant from Vifor Phama. Vifor Pharma had no influence on the design, interpretation or reporting of this work. M. Rutter: None. P. Lanyon: Grants/research support; Recipient of research grant from Vifor Phama. Vifor Pharma had no influence on the design, interpretation or reporting of this work. J. Aston: None. M. Bythell: None. S. Stevens: None.

Overall survival of black and white men with metastatic castration-resistant prostate cancer (mCRPC): a 20-year retrospective analysis in the largest healthcare trust in England.

Prostate cancer in black men is associated with poorer outcomes than their white counterparts. However, most studies reporting this disparity were conducted in localized prostate cancer and primarily in the United States. Data regarding prostate cancer incidence and mortality for East London between 2008 and 2010 were obtained from the UK National Disease Registration Service. We further evaluated survival outcomes of 425 cases of mCRPC in St Bartholomew's Hospital, East London, between 1997 and 2016, and analyzed whether ethnicity impacted on responses to different treatment types. The incidence of prostate cancer in black men was higher than white men in East London. Prostate cancer-specific mortality was proportional to incidence based on ethnic groups. In the detailed analysis of 425 patients, 103 patients (24%) were black (B), and the remainder white (W). Baseline characteristics were comparable in both groups, although black patients had a lower baseline hemoglobin (p < 0.001). Median overall survival for the total cohort was 25.5 months (B) vs 21.8 months (W) (hazard ratio (HR) = 0.81, p = 0.08). There was prolonged survival in the black population in those who only received hormone-based treatment throughout their treatment course; 39.7 months (B) vs 17.1 months (W) (HR = 0.54, p = 0.019). Black men may do better than white men with mCRPC, in the context of equal access to healthcare. The study also suggests a greater margin of benefit of hormone-based therapy in the black subpopulation.

Treatable but not curable cancer in England: a retrospective cohort study using cancer registry data and linked data sets

ObjectivesThis study estimates the prevalence of cancers that are categorised as treatable but not curable (TbnC) in England. It provides a quantification of the population and a framework to aid...

Group A Streptococcal Infection, Acute Rheumatic Fever and Rheumatic Heart Disease in the Solomon Islands

Abstract Group A Streptococcal (GAS) infection of the upper respiratory tract (and possibly skin) can trigger Acute Rheumatic Fever (ARF), an autoimmune condition if left untreated. Severe or recurrent attacks of ARF that also remain untreated, can lead to a type of permanent cardiac damage known as Rheumatic Heart Disease (RHD). RHD remains a significant cause of morbidity and mortality globally and is rare in developed countries. In Australia, New Zealand (NZ) and the Pacific Region, the disease burden of ARF and RHD amongst Indigenous and Pacific communities remain one of the highest in the world, usually affecting children and young adults. Since a GAS vaccine remains unavailable, the most effective recommended treatment and management strategy to prevent further GAS infections that can lead to ARF and RHD constitute of painful monthly intramuscular injections of Benzathine Penicillin G (BPG). These BPG injections are administered for at least 10 years or more and are referred to as secondary prophylaxis (SP). Due to the pain, formulation and other issues associated with regular BPG injections, adherence to SP remains problematic globally. A new Penicillin that supports better adherence to SP is urgently needed. This work sought to further understand the local context in the Solomon Islands concerning GAS, ARF and RHD. Preliminary findings indicate very high ARF and RHD rates present in the local setting, in addition to issues with access to ARF/RHD prevention measures with BPG. An electronic database to monitor ARF and RHD as a national disease register will be of clinical relevance to the local setting in the Solomon Islands. Research efforts in the Pacific Region contributing towards the generation of a new Penicillin for BPG SP will be of benefit.

Assessing the History of Safe Use of Guayusa

Guayusa (Ilex guayusa Loes.) is a herbal tea that has been consumed for centuries as a traditional food in western Amazon regions where it is now valued as an antioxidant and a stimulant agent. Currently there is intense commercial activity to expand the consumption of guayusa as a healthy energy drink in Europe, where it is classified by law as a novel food. European Union novel food legislation permits traditional foods to be placed on the market if a history of safe use in a non-EU country can be established. However, a scientific assessment of the ‘safe’ use of guayusa is lacking. This study investigates the safety of guayusa consumption, analysing provincial hospital admissions data; national disease register data; national toxicology agency call centre data; and national food safety authority data. In doing so we present a crucial analysis for the novel food premarket risk assessment of dried guayusa leaves, based on the well-established use of this traditional food in Ecuador. Within a three-year period there was one minor adverse effect reported nationally, related to the stimulant properties of guayusa. However, there were no hospital presentations, no product safety notifications and no disease register records of guayusa-related illness. Comprehensive records of unrelated food safety risks demonstrate that Ecuador’s surveillance and reporting system has sufficient rigour to identify risks should they exist. We conclude that there is a history of safe use of guayusa in Ecuador. Establishing safe guayusa consumption is an important milestone for the authorisation of dried guayusa leaves as a novel food with claimed health benefits in Europe. Consequently, this study has significance for international product development of guayusa as an antioxidant energy drink.

Proposed model for a Romanian register of chronic diseases in children

Background and ObjectivesThe prevalence of chronic diseases at children is increasing due to the progress made in medicine, which radically changed the evolution and the prognostics. Disease registers became important tools to register and monitor the chronic patients in order to asses their needs and improve their care. Disease registers are also very important for adjusting to population needs the actions in the public health field.The main objective for this research is to develop a model for a chronic disease register of children in Romania, taking into account the current situation of chronic diseases’ registration and reporting, the specific requirements for such medical database and the particularities of the information systems in the Romanian healthcare system. MethodsOur study was based on interviews with specialists and Health Ministry representatives, field observations, and analysis of published and unpublished data). ResultsWe developed a platform for a chronic diseases register of children based on open EHR specifications and architecture, which respects the requirements for data security and the particularities of Romania's healthcare system. The model created is functioning on two levels. The first one is local and corresponds to a medical unit, while the second one represents the connection and the communication method within the entire national medical system. In order to achieve the highest level of interoperability at the semantic level, we used, for the communication module of a National Disease Register system, only EN 13606 or HL7 v3 and / or HL7 v3 CDA. ConclusionsThis model is functional and can be applied initially at a clinical level for medical and administrative purposes. Its design allows a future development, integrated with the current informational systems and implemented at regional and national level. This will significantly improve the knowledge that may support good medical practice in this field, will enhance the coherence of data collection and, as a consequence, will impact positevely on the health of children diagnosed with chronic diseases.

Validation of the Finnish national dairy disease register—Data transfer from cow health cards to the disease register

Information on diseases of dairy cows in Finland is entered into the national disease register. Before the data from such types of secondary database are used, the quality of the data needs to be validated. In this study, 7,324 veterinary records for culled cows were compared against records in the national disease register in the period 2002 to 2008. Evaluation of the national disease register data was done by calculating completeness and correctness values. Completeness is the proportion of events that were physically recorded in the secondary database, and correctness is the proportion of correctly recorded events of all recorded events. The use of both correctness and completeness is important when describing the accuracy of secondary data. The completeness and correctness values for the Finnish national dairy disease register were 83 and 92%, respectively. We found that 39% of all unmatched diagnostic events were unreported by the artificial insemination technician who transferred the data from cow cards to the register. Logistic regression models showed that diagnostic events for cows born into the herd had twice the odds of being transferred compared with events for purchased cows. Diagnostic events for reproductive diseases had higher odds of being transferred to the register compared with all other disease groups, and the odds for transfer of the diagnostic event decreased as the age increased. We also found that if the diagnostic event was the last diagnostic event on the cow card, then its odds of being transferred to the disease register were significantly lowered. Although the Finnish national dairy disease register has good completeness and excellent correctness values, different disease groups, age groups, origin of the cow, and timing of the diagnostic event affect how well diagnostic events are transferred to the Finnish national dairy register.

PTU-199 Variable reporting and diagnosis of eosinophilic oesophagitis across the UK: data from the BSG national disease register

IntroductionEosinophilic oesophagitis (EoE), a disorder characterised by intermittent dysphagia, was first described over 20 years ago. The true prevalence of this condition is not known. In 2010, the British Society...

Maternal inflammatory bowel disease and offspring body size: A prospective cohort study

Maternal inflammatory bowel disease (IBD) may influence intrauterine growth and hence size at birth, but the consequences for offspring in later life remain uncertain. This study investigated the growth of children of mothers with Crohn's disease (CD) or ulcerative colitis (UC). The Danish National Birth Cohort, comprising 40,640 mother-child pairs with 7-year follow-up of children's height and weight, were linked to the Danish National Disease Register, whereby 50 mothers with CD and 147 mothers with UC were identified. Associations were tested by regression analyses, taking several covariates into account. Children of mothers with IBD were significantly shorter at birth than children of unaffected mothers (adjusted; CD, difference in cm, -0.82; 95% confidence interval [CI], -1.39 to -0.25; UC, -0.41; 95% CI, -0.75 to -0.07) and they tended to be of lower birth weight (adjusted; CD, difference in grams, -119.7; 95% CI, -246.7 to 7.3; UC, -64.0; 95% CI, -138.7 to 10.7). However, during the first year of life children of IBD mothers reached similar body sizes as children of unaffected mothers. At the 7-year follow-up, girls, but not boys, of CD mothers tended to be overweight (adjusted odds ratio [OR], 2.47; 95% CI, 0.98-6.24) and had increased waist circumference (adjusted difference in cm, 3.48; 95% CI, 1.40-5.58) compared to the unaffected population. The present study confirms that maternal history of IBD leads to decreased birth size in offspring. Reassuringly, body size in children of IBD mothers approached body size in children of unaffected mothers during childhood.

Informing the development of a national diabetes register in Ireland: a literature review of the impact of patient registration on diabetes care

Research suggests that a structured approach to diabetes care can lead to improved patient outcomes. In order to enable greater organisation of care, an electronic patient registration system is required. As part of the development of a national disease register in Ireland, we conducted a review of literature relating to the impact of registration systems on processes and outcomes of care. The aim of the review is to establish the impact of a registration system on patient care and clinical outcomes. The review explores the role played by a patient registration system, particularly in the primary care setting. The literature review applied a search strategy to six identified databases. Included studies were those based on original research, including a patient registration system and published between 1999 and 2009 in the English language. Studies including only patients with type 2 diabetes or those with both type 1 and type 2 diabetes were included. Some papers did not specify which type of diabetes was included. In interventions of structured care which used a patient registration system, modest results for clinical outcomes were demonstrated as well as significant improvements in the processes of care. A patient register was a necessary step along the path towards improved patient clinical outcomes, notably glycated haemoglobin (HbA1c), blood pressure and cholesterol measurements. This review suggested that registers are generally assumed to be an essential element of quality improvement interventions rather than an optional addition. A diabetes register is central to the development of a comprehensive diabetes management system in primary care, which can lead to improvements in the processes and outcomes of diabetes care.

Increased prevalence of Down’s syndrome in individuals with type 1 diabetes in Denmark: a nationwide population-based study

In patients with Down's syndrome, dogma has long held that the prevalence of diabetes is increased. The aim of the present study was to determine the actual prevalence of Down's syndrome among type 1 diabetic patients. The background population included all children born in Denmark between 1981 and 2000. Registry-validated and clinical data on type 1 diabetes and Down's syndrome diagnoses were obtained from the National Disease Register and Danish Cytogenetic Central Register, respectively. The prevalence of Down's syndrome in the background population was 0.09%, whereas we identified a prevalence of Down's syndrome in type 1 diabetes patients of 0.38% (95% CI 0.17-0.75), corresponding to a 4.2-fold increased prevalence compared with the background population (p = 7.3 x 10(-5)). To the best of our knowledge this is the first population-based study addressing the prevalence of Down's syndrome among verified type 1 diabetes patients. A more than fourfold increased prevalence of Down's syndrome among type 1 diabetes patients supports the notion that genes on chromosome 21 may confer risk for type 1 diabetes, probably also in the general population.

Wasted Records of Disease

IN the three chapters of this book Mr. Paget reviews the attempts made to secure or establish permanent systems of disease registration; legislative recognition of the need for such registration, and its shortcomings; and steps advisable to secure a permanent and useful system of national disease registration. His plea for the establishment of a national system of notification and registration of disease, will have the support of most practitioners and officers of public health.