Temporal and Cartographic Analyses of the Distribution within Spain of Mortality Due to Granulomatosis with Polyangiitis (1984⁻2016)

The aim is to conduct a descriptive, population-based study in order to assess temporal and spatial changes in mortality due to granulomatosis with polyangiitis (GPA) in Spain from 1984 to 2016. Mortality data were obtained from the Spanish Annual Death Registry. Deaths in which GPA was the underlying cause were selected using the 446.4 and M31.3 codes from the International Classification of Diseases, 9th and 10th revision. Annual average age at death and age-adjusted mortality rates were calculated. Geographic analysis was performed at municipality and district level. Variations in mortality according to the type of municipality (urban, agro-urban or rural), district and geographic location (degrees of latitude) were assessed using standardized mortality ratios (SMRs) and smoothed-SMRs. Over the whole period, 620 deaths due to GPA were identified. Age at death increased at an average annual rate of 0.78% over the period 1987-2016 (p < 0.05). Age-adjusted mortality rates increased by an annual average of 20.58% from 1984 to 1992, after which they fell by 1.91% a year (p < 0.05). The agro-urban category had the highest percentage (4.57%) of municipalities with a significantly higher GPA mortality rate than expected. Geographic analysis revealed four districts with a higher risk of death due to GPA, two in the North of Spain and two in the South. This population-based study revealed an increase in the age at death attributed to GPA. Age-adjusted mortality rates went up sharply until 1992, after which they started to decline until the end of the study period. Geographic differences in mortality risk were identified but further studies will be necessary to ascertain the reasons for the distribution of GPA disease.This research was supported by the Spanish Stategy Action for Health (AESI), project no. TPY1238/15. GS enjoys a research grant funded by the Spanish Ministry of Education, Culture and Sport (No. FPU14/03914).S

Familial Mediterranean Fever in Spain: Time Trend and Spatial Distribution of the Hospitalizations

Familial Mediterranean Fever (FMF) is a rare, hereditary, auto-inflammatory disease. The aims of this study were to explore the time trend and geographical distribution of hospitalizations in Spain from 2008 to 2015. We identified hospitalizations of FMF from the Spanish Minimum Basic Data Set at hospital discharge, using ICD-9-CM code 277.31. Age-specific and age-adjusted hospitalization rates were calculated. The time trend and the average percentage change were analyzed using Joinpoint regression. Standardized morbidity ratios were calculated and mapped by province. A total of 960 FMF-related hospitalizations (52% men) were identified across the period 2008-2015, with an increase in hospitalizations of 4.9% per year being detected (p 1) in 13 provinces (5 in the Mediterranean area), and lower (SMR < 1) in 14 provinces (3 in the Mediterranean area). There was an increase in hospitalizations of patients with FMF in Spain throughout the study period, with a risk of hospitalization that was higher, though not exclusively so, in provinces along the Mediterranean coast. These findings contribute to the visibility of FMF and provide useful information for health planning. Further research should take into account new population-based information, in order to continue monitoring this disease.S

Investigación de una agregación de casos de cáncer en las proximidades de una antena de telefonía móvil

Se presenta la investigaci&oacute;n de un cluster de casos de c&aacute;ncer en un &aacute;rea urbana que los vecinos asociaban con la presencia de una antena de telefon&iacute;a m&oacute;vil y un transformador el&eacute;ctrico. Se dise&ntilde;&oacute; un estudio de cohorte retrospectivo; el &aacute;rea de estudio fueron los edificios colindantes a la antena y al transformador en los que se hab&iacute;an denunciado casos de c&aacute;ncer; la informaci&oacute;n se obtuvo mediante encuesta domiciliaria. Las localizaciones tumorales m&aacute;s frecuentes fueron mama, colonrecto y pulm&oacute;n. Se calcul&oacute; la Raz&oacute;n de Incidencia Estandarizada (RIE) entre los casos observados y los casos esperados para todas las localizaciones y para las localizaciones tumorales m&aacute;s frecuentes. Los intervalos de confianza de las RIEs siempre inclu&iacute;an el valor nulo. Conclusi&oacute;n: no se ha encontrado un aumento significativo de casos de c&aacute;ncer en la cohorte y las localizaciones tumorales identificadas son las que se espera que aparezcan en la poblaci&oacute;n de estudio

A population-based study of mortality due to muscular dystrophies across a 36-year period in Spain

Muscular dystrophies (MD) are a group of rare hereditary degenerative diseases. Our aim was to analyze the mortality pattern in Spain from 1981 to 2016 to assess the temporal trend and discern possible geographic differences using population-based data. Annual deaths related to MD were obtained from the National Statistics Institute with codes 359.1 of the ICD-9 (1981-1998) and G71.0 of the ICD-10 (1999-2016). Age-adjusted mortality rates were calculated and changes in mortality trends were identified. The standardized mortality ratios (SMR) and their respective 95% confidence intervals were calculated by district for 1999-2016. Smoothed SMRs and posterior probability were also assessed and then mapped to look for patterns or geographic distribution. All rates were expressed per 1,000,000 inhabitants. A total of 2,512 deaths (73.8% men) were identified. The age-adjusted mortality rates varied from 0.63 (95% CI 0.40-0.95) in 1981 to 1.51 (95% CI 1.17-1.93) in 2016. MD mortality showed a significant increase of 8.81% per year (95% CI 5.0-12.7) from 1981 to 1990, remaining stable afterwards. Areas with risk of death higher than expected for Spain as a whole were identified, not showing a specific regional pattern. In conclusion, the rising trend in MD mortality might be attributable to advanced improvements in diagnostic techniques leading to a rise in prevalence. Further research on the districts with the highest mortality would be necessary.This research was funded by Instituto de Salud Carlos III, Spanish Strategy Action for Health (AESI), project PI14CIII/00067, TPY 1238/15.S

Epidemiology of varicella in spain pre-and post-vaccination periods

BACKGROUND: Varicella virus can cause two different diseases: chickenpox and herpes zoster. In 2005 varicella vaccine has been introduced in the Spanish national vaccination schedule for 10-14 years old non-immune people, in order to reduce the severity of the disease. In 2007 a new surveillance protocol with aggregate data for chickenpox and herpes zoster was approved in order to detect any change in age distribution, severity and complications of the chickenpox and herpes zoster cases. The aim of this study is to know the burden of diseases (in the last ten years). METHODS: Number of cases, hospitalization and incidence for chickenpox and herpes zoster were study for two periods 1997-2003 and 2005-2007. Analysis for 1996-2007 fatal cases was done too. We decided to remove year 2004 because the extremely high chickenpox incidence registered. SOURCES OF DATA: RENAVE (Spanish Surveillance Network), Spanish hospital surveillance system (CMBD), and mortality registries. RESULTS: Chickenpox incidence decreased since 2005, but an increasing trend was detected in hospitalisation with an average of 1,311 hospitalizations every year. For the 32%-36% of hospitalized cases, the main diagnosis was not chickenpox. 4-14 deaths per year have been detected; 80% of them were older than 14 years. Annual rate of herpes zoster hospitalization was 2.5 per 100,000 inhabitants, similar in both sexes. Case fatality rate per year was 0.31 per million inhabitants. No significant changes were detected in age and sex in complicated cases between the two periods. 88% of chickenpox cases were younger than 15 years old and 64% of herpes zoster older than 50 years in 2007. CONCLUSIONS: Chickenpox has been decreasing during 2005-2007 in Spain. The impact of vaccination is difficult to asses, because of a peak registered in 2004 but also because the lack of vaccination coverage information for this period and the case-data information is available only for the last year. Fundamento: El virus varicela zoster puede causar dos enfermedades, la varicela y el herpes zóster. La vacuna frente a la varicela se incorporó en España en 2005 para personas susceptibles de entre 10 y 14 años. En 2007 se aprobó una propuesta de vigilancia de la varicela y herpes zóster que permitiera detectar posibles cambios en los patrones de distribución por edad, en la gravedad y complicaciones. El objetivo de este trabajo es conocer la carga de enfermedad por varicela antes y después de la vacunación. Método: Se analizan los datos agregados (casos e incidencia) de varicela y herpes zóster en España en el sistema CMBD para 1997-2003 y 2005-2007, así como la mortalidad por esta enfermedad a nivel nacional para e período 1999-2006. Resultados: El 88,1% de los casos de varicela se da en personas menores de 15 años. En el CMBD se registró un promedio anual de 1.311 ingresos. No se observaron cambios significativos en la distribución por edad, sexo ni complicaciones durante los ingresos en ninguno de los períodos estudiados. El 32-36% anual ingresó por un motivo diferente a varicela. La mortalidad osciló entre 4 y 14 individuos/año, el 80% mayores de 14 años. El 64% de los casos notificados de herpes zóster fueron mayores de 50 años. La tasa media anual de ingresos por fue de 2,5 por 100.000 habitantes sin diferencias por sexo. La tasa media anual de defunciones fue 0,31 por millón de habitantes. Conclusiones: En España la varicela tuvo una disminución generalizada durante 2005-2007, pero es difícil valorar el impacto de la vacunación por la falta de cifras de cobertura vacunal y porque este período coincide con el inmediato a la última onda epidémica, cuyo máximo se registró en 2004

Mortality Due to Cystic Fibrosis over a 36-Year Period in Spain: Time Trends and Geographic Variations

The aim of this study is to analyze population-based mortality attributed to cystic fibrosis (CF) over 36 years in Spain. CF deaths were obtained from the National Statistics Institute, using codes 277.0 from the International Classification of Diseases (ICD) ninth revision (ICD9-CM) and E84 from the tenth revision (ICD10) to determine the underlying cause of death. We calculated age-specific and age-adjusted mortality rates, and time trends were assessed using joinpoint regression. The geographic analysis by district was performed by standardized mortality ratios (SMRs) and smoothed-SMRs. A total of 1002 deaths due to CF were identified (50.5% women). Age-adjusted mortality rates fell by -0.95% per year between 1981 and 2016. The average age of death from CF increased due to the annual fall in the mortality of under-25s (-3.77% males, -2.37% females) and an increase in over-75s (3.49%). We identified districts with higher than expected death risks in the south (Andalusia), the Mediterranean coast (Murcia, Valencia, Catalonia), the West (Extremadura), and the Canary Islands. In conclusion, in this study we monitored the population-based mortality attributed to CF over a long period and found geographic differences in the risk of dying from this disease. These findings complement the information provided in other studies and registries and will be useful for health planning.This research was funded by the Instituto de Salud Carlos III, Spanish Strategy Action for Health (AESI), project TPY1238/15. The author G.S.D. received a research grant from the Spanish Ministry of Education, Culture, and Sport, FPU14/03914.S

Rare diseases. Concept, epidemiology and state of the question in Spain

[ES] Enfermedad rara es aquella cuya prevalencia es inferior a 5 casos por cada 10.000 personas en la Comunidad Europea. La mayoría de los casos aparecen en la edad pediátrica, dada la alta frecuencia de enfermedades de origen genético y de anomalías congénitas. No obstante, la prevalencia es mayor en los adultos que en los niños, debido a la excesiva mortalidad de algunas enfermedades infantiles graves y a la influencia de ciertas enfermedades que aparecen a edades más tardías. La Red Epidemiológica de Investigación en Enfermedades Raras (REpIER) desarrolló el primer atlas de distribución geográfica de las enfermedades raras en España, evaluó la existencia de registros de enfermedades raras existentes, facilitó el ulterior desarrollo de planes autonómicos y acciones sociosanitarias y planteó un marco de necesidades a desarrollar, que más tarde han llegado a ser reconocidos como necesidades a resolver en el marco del Comunicado de la Comisión Europea sobre Enfermedades Raras y en la propia Ponencia del Senado. [EN] Rare diseases are those whose prevalence is below 5 cases per 10,000 inhabitants in the European Community. Most cases are diagnosed during paediatric age due to their genetic origin, while some others are congenital malformations. Nevertheless, a higher prevalence is seen during adulthood as most of the former diseases are very severe and patients die during childhood. At the same time, higher survival rates are related to some chronic rare diseases in adults. The Spanish Network of Research Epidemiology for Rare Diseases (REpIER) developed the first atlas showing the geographical distribution of rare diseases in Spain, assessed the Spanish rare disease registries, contributed to the further development of regional plans on rare diseases, as well as to social and health actions, and established the real group of needs to be solved. These have been included in the Communication of the European Commission on Rare Diseases as well as in the Spanish Senate Presentation.S

Rare Diseases in Spain: a nationwide registry-based mortality study

Poster presented at the 9th European Conference on Rare Diseases and Orphan Products (ECRD Vienna 2018). Vienna, Austria. May 10-12, 2018.Background: Rare diseases (RD) are still lacking of population-based data and epidemiological indicators. The aim of this study is to assess 15-years’ time trends of mortality attributed to RD in Spain. Methods: Mortality statistics from the Spanish National Statistics Institute provide population-based data [1]. Deaths due to RD were extracted from official annual databases (1999-2013). Only those ICD-10 codes considered as RD by SpainRDR experts were included in this study [2]. Annual sex- and age-specific adjusted mortality rates per 100,000 inhabitants were calculated and time trends were performed by joinpoint regression analysis. Results: RD mortality represents 1.2% of all registered deaths from 1999 to 2013 in Spain. Mortality attributed to RD is higher in males (51.2%) than females (48.8%). Children (<15 years old) account for 15.2% of deceases. Distribution of RD deaths according to main ICD-10 groups is displayed in Fig. 1. Regarding time trends of RD mortality (Fig. 2), there has been a 0.95% decline in the annual age-adjusted death rates due to all RD (-0.95%, p<0.001). In addition: Decrease trends were also observed in the following subgroups: RD of the blood and blood-forming organs and certain rare disorders involving the immune mechanism (-2.06%, p<0.001), RD of the circulatory system (-3.90%, p<0.01), and rare congenital malformations, deformations and chromosomal abnormalities (-5.39%, p<0.01). Increase trends of annual age-adjusted death rates were detected for RD of the nervous system (1.85%, p<0.01), RD of the respiratory system (2.39%, p<0.01), RD of the digestive system (1.83%, p<0.05) and those RD affecting the genitourinary system (9.38% p<0.05). Other RD groups have not showed any significant change in this period. Conclusion: Official mortality statistics share criteria for analysing uniform and robust time series, which is useful for studying low-prevalence diseases. Assessed RD mortality trends are valuable information for the health authorities in Spain.Spanish Strategy Action for Health (AESI) supported this research, project No. TPY1238/15.N

A Nationwide Registry-Based Study on Mortality Due to Rare Congenital Anomalies

This study aimed to analyse population-based mortality attributed to rare congenital anomalies (CAs) and assess the associated time trends and geographical differences in Spain. Data on CA-related deaths were sourced from annual mortality databases kept by the National Statistics Institute of Spain (1999&ndash;2013). Based on the ICD-10, only CAs corresponding to rare diseases definition were included in this study. Annual age-adjusted mortality rates were calculated and time trends were evaluated by joinpoint regression analysis. Geographical differences were assessed using standardised mortality ratios and cluster detection. A total of 13,660 rare-CA-related deaths (53.4% males) were identified in the study period. Annual age-adjusted mortality rates decreased by an average of &minus;5.2% (&minus;5.5% males, &minus;4.8% females, p &lt; 0.001). Geographical analysis showed a higher risk of rare-CA-related mortality in regions largely located in the south of the country. Despite their limitations, mortality statistics are essential and useful tools for enhancing knowledge of rare disease epidemiology and, by extension, for designing and targeting public health actions. Monitoring rare-CA-related mortality in Spain has shown a 15-year decline and geographical differences in the risk of death, all of which might well be taken into account by the health authorities in order to ensure equality and equity, and to adopt appropriate preventive measures

Mortality attributed to hereditary haemorrhagic telangiectasia and geographical variability in Spain (1981-2016).

Objetivo Conocer la mortalidad directamente atribuida a la telangiectasia hemorrágica hereditaria (THH) en España, su tendencia temporal y la posible variabilidad geográfica. Método El total de los fallecidos por THH de base poblacional se obtuvo del Instituto Nacional de Estadística, seleccionando los códigos 448.0 (CIE 9-MC, 1981-1998) y I78.0 (CIE 10, 1999-2016) como causa básica de defunción. Se calcularon las tasas de mortalidad específicas y ajustadas por edad para cada sexo, las razones de mortalidad estandarizadas (RME) por provincia y comarca, y las RME suavizadas. Resultados Se identificaron 327 fallecimientos por THH (el 49,5% eran mujeres), siendo la mortalidad más alta a los 80-84 años en los hombres (0,220 por 100.000 habitantes) y a los 75-79 años en las mujeres (0,147 por 100.000 habitantes). No se detectaron cambios temporales entre 1981 y 2016. Las provincias de Navarra, Cantabria, Guipúzcoa, Pontevedra y Las Palmas presentaron un riesgo significativamente superior con respecto a lo esperado para el total nacional, así como las comarcas de Monte Sur (Ciudad Real) y Ripollès (Girona). Conclusiones Este trabajo ha permitido identificar algunas regiones con mayor riesgo de defunción por THH, si bien se desconoce si estas diferencias se asocian a la distribución de los tipos THH1 y THH2, por lo que son necesarios estudios posteriores para profundizar en las causas de la variabilidad geográfica. Estos hallazgos complementan la información proporcionada por otros estudios y registros, además de ser útiles para la planificación sanitaria. To identify the mortality directly attributed to hereditary haemorrhagic telangiectasia (HHT) in Spain, and to analyze its time trends and geographic variability. Population-based deaths due to HHT were selected from the Spanish National Statistics Institute: codes 448.0 (ICD-9, 1981-1998) and I78.0 (ICD-10, 1999-2016) as the basic cause of death. Specific and age-adjusted mortality rates were calculated by sex, as well as standardized mortality ratios (SMR) by province and district, and smoothed SMR. We identified 327 deaths attributed to HHT (49.5% women), with the highest mortality at 80-84 years in men (0.220 per 100,000 inhabitants) and at 75-79 years in women (0.147 per 100,000 inhabitants). Age-adjusted mortality rates did not show any significant time trend between 1981 and 2016 in Spain. The provinces of Navarra, Cantabria, Guipúzcoa, Pontevedra and Las Palmas had higher than expected mortality, as well as the regions of Monte Sur (Ciudad Real) and Ripollès (Girona). This study has identified some regions with higher risk of death due to HHT in Spain. It is unknown whether these differences are associated with the distribution of types HHT1 and HHT2, and further studies will be necessary to know the determinants of this geographical variability. These findings are useful to complement the information provided by other studies and registries, and for health planning.Proyecto TPY 1238/15 financiado por la Acción Estratégica en Salud del Instituto de Salud Carlos III (AESI).S