Haemoglobinopathies and other rare anemias in Spain: ten years of a nationwide registry (REHem-AR).

REHem-AR was created in 2013. The progressive implementation of neonatal screening for haemoglobinopathies in Spanish autonomous communities where the registry had not been implemented, as well as the addition of new centres during this period, has considerably increased the sample of patients covered. In this study, we update our previous publication in this area, after a follow-up of more than 5 years. An observational, descriptive, multicentre and ambispective study of adult and paediatric patients with haemoglobinopathies and rare anaemias registered in REHem was performed. The data are from a cross-sectional analysis performed on 1 June, 2023. The study population comprised 1,756 patients, of whom 1,317 had SCD, 214 had thalassaemia and 224 were diagnosed with another condition. Slightly more than one third of SCD patients (37%) were diagnosed based on neonatal bloodspot screening, and the mean age at diagnosis was 2.5 years; 71% of thalassaemia patients were diagnosed based on the presence of anaemia. Vaso-occlusive crisis and acute chest syndrome continue to be the most frequent complications in SCD. HSCT was performed in 83 patients with SCD and in 50 patients with thalassaemia. Since the previous publication, REHem-AR has grown in size by more than 500 cases. SCD and TM are less frequent in Spain than in other European countries, although the data show that rare anaemias are frequent within rare diseases. REHem-AR constitutes an important structure for following the natural history of rare anaemias and enables us to calculate investment needs for current and future treatments.

Temporada de gripe y VRS 2018-19 y efectividad vacunal frente a la necesidad de atención hospitalaria por gripe en la provincia de Guadalajara / Flu and VRS 2018-19 Season and vaccine effectiveness against the need for hospital flu treatment in Guadalajara province, Spain

INTRODUCCIÓN: El objetivo fue estimar la efectividad de la vacuna (EV) trivalente inactivada para prevenir atenciones hospitalarias por gripe (AHG) en Guadalajara, Castilla-La Mancha (CLM), España, temporada 2018-2019. MATERIAL Y MÉTODOS: Estudio de cohortes retrospectivo; semanas 40/2018 a 13/2019. Fuentes: Programa Microbiología; historia clínica electrónica; censo poblacional (INE, 1/7/2018). Casos: AHG (urgencias y/u observación de urgencias o ingreso), confirmadas mediante prueba antigénica o PCR. Se calcularon: fracciones preventivas ([FPv(vacunados) y FPp(poblacional)]) y número necesario de pacientes a vacunar (NNV). RESULTADOS: Hubo 228 AHG (tasa incidencia [TI] acumulada=8,9/104; ≥ 65 años=65%; cobertura vacunal=13% [≥ 65 años=58%]; mortalidad=9%); con máxima incidencia en la semana 6.ª (TI=1,7/104) (en CLM, en la 4.ª). El mayor pico de VRS ocurrió en la 3.ª semana (en CLM, en la 52). La FPv entre 14-65 años fue del 96% (FPp=58%) y en ≥ 65, del 32% (FPp=21%). NNV=414. Como en España, predominó el virus A, siendo A(H3N2) un 13% más prevalente (cepa no concordante con la vacunal). CONCLUSIONES: La temporada se retrasó por una sostenida circulación del VRS. La EV resultó inferior a la nacional. Sería imprescindible impulsar próximas campañas para mejorar la cobertura

INTRODUCTION: The objective was to estimate the effectiveness of inactivated trivalent vaccine (VE) in preventing hospital flu care (HFC) in Guadalajara, Castile-La Mancha (CLM), Spain, 2018-19 season. MATERIAL AND METHODS: Retrospective cohort study (40/2018 to 13/2019 weeks). Sources: Microbiology programme; electronic medical history; population census (INE, 1/7/2018). Cases: Population requiring HFC (hospital emergencies and/or emergency observation unit and/or hospital admissions), confirmed by antigenic test and/or PCR. Preventive fractions [PFv(vaccinated) and PFp(population)] and Necessary number of patients to be vaccinated (NNV) were calculated. RESULTS: 228 HFT occurred [cumulative incidence rate (IR)=8.9/104; ≥65 years=65%; vaccination coverage=13% (≥65 years=58%); mortality=9%); maximum incidence in the 6th week (IR=1.7/104) (in CLM, in 4th)]. Highest peak of RSV occurred in the 3rd (in CLM, in the 52th). PFv (14-65 years) was 96% (PFp=58%) and in ≥65, 32% (PFp=21%). NNV=414. As in Spain, influenza virus A predominated, with A(H3N2) being 13% more prevalent (strain not included in the vaccine). CONCLUSIONS: The season was delayed by sustained VRS circulation. The VE was lower than the national one. It is be essential to promote future campaigns to improve vaccination coverage

Flu and VRS 2018-19 Season and vaccine effectiveness against the need for hospital flu treatment in Guadalajara province, Spain. / Temporada de gripe y VRS 2018-19 y efectividad vacunal frente a la necesidad de atención hospitalaria por gripe en la provincia de Guadalajara.

INTRODUCTION: The objective was to estimate the effectiveness of inactivated trivalent vaccine (VE) in preventing hospital flu care (HFC) in Guadalajara, Castile-La Mancha (CLM), Spain, 2018-19 season. MATERIAL AND METHODS: Retrospective cohort study (40/2018 to 13/2019 weeks). SOURCES: Microbiology programme; electronic medical history; population census (INE, 1/7/2018). CASES: Population requiring HFC (hospital emergencies and/or emergency observation unit and/or hospital admissions), confirmed by antigenic test and/or PCR. Preventive fractions [PFv(vaccinated) and PFp(population)] and Necessary number of patients to be vaccinated (NNV) were calculated. RESULTS: 228 HFT occurred [cumulative incidence rate (IR)=8.9/104; ≥65 years=65%; vaccination coverage=13% (≥65 years=58%); mortality=9%); maximum incidence in the 6th week (IR=1.7/104) (in CLM, in 4th)]. Highest peak of RSV occurred in the 3rd (in CLM, in the 52th). PFv (14-65 years) was 96% (PFp=58%) and in ≥65, 32% (PFp=21%). NNV=414. As in Spain, influenza virus A predominated, with A(H3N2) being 13% more prevalent (strain not included in the vaccine). CONCLUSIONS: The season was delayed by sustained VRS circulation. The VE was lower than the national one. It is be essential to promote future campaigns to improve vaccination coverage.