Joint status in Spanish haemophilia B patients assessed using the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score.

AIM: The use of musculoskeletal ultrasound (MSK-US) following protocols for haemophilic arthropathy and the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score can help standardize monitoring in haemophilia. This study evaluated the joint status (elbows, knees and ankles) of patients with haemophilia B (HB) in Spain using MSK-US and the HEAD-US score. METHODS: Haemophilia B patients ≥14 years old were included in this observational, multicentre, cross-sectional study, regardless of their clinical condition, HB severity and treatment received. Two blinded observers were involved in image acquisition and scoring in each centre. RESULTS: Eighty-two patients from 12 centres were enrolled: 27% mild HB, 23% moderate, 50% severe HB. Mean age was 38.9 ± 16.4 years, 60% were treated on demand (OD) and 40% were on prophylaxis. HEAD-US was zero in all joints in 28.6% OD patients and 36.4% on prophylaxis. Mean scores significantly worsened with HB severity, except for the left knee. Patients on primary and secondary prophylaxis had significantly better joint health vs OD patients in all joints, except the right ankle. Among OD patients, those with severe disease presented significantly worse scores in all HEAD-US items related to permanent damage. CONCLUSION: Joint status of HB patients in Spain is influenced by severity and treatment modality, related to the development of arthropathy, which appears prevalent in OD patients with severe HB. Routine assessment with an imaging tool such as ultrasound and HEAD-US system may help to improve joint health by personalizing and adjusting treatment in this population.

Factor VIII delivery devices in haemophilia A. Barriers and drivers for treatment adherence / Dispositivos de administración de Factor VIII en la hemofilia A. Barreras y estímulos para la-adherencia

Introduction and objective: To capture the experience of haemophilia A patients with their devices for coagulation factor reconstitution, the barriers for treatment adherence; and to determine their preferences, presenting a new double-chamber syringe (DCS). Method: A cross-sectional research through a supervised survey and a DCS testing session. Results: Seventy-four (74) patients participated, 50% of them on prophylaxis treatment, and 7 years (median) with their usual treatment (IQR 17.25). In the survey, the DCS received the highest score (75/100, p < 0.001) and it was the most likely to be used in prophylactic treatment (p < 0.001). In the practical testing session (n = 29), 62.1% preferred the DCS, and required 43 seconds as median time (24.5-82) for preparation, vs. 4 minutes (1-15) with their usual treatment (p < 0.001). The favourable opinion of their doctor regarding prophylaxis was very influential on the attitude of participants towards treatment adherence (OR = 1.324, CI 95% = 1.040-1.685, p = 0.023). Conclusions: The DCS was the preferred device, and was likely to encourage prophylaxis (AU)

Introducción y objetivo: Recabar la experiencia de pacientes con hemofilia A con sus dispositivos de reconstitución de factor de coagulación, barreras para la adherencia y determinar sus preferencias, presentando una nueva jeringa de doble cá- mara (JDC). Método: Investigación transversal mediante encuesta dirigida y sesión de prueba de la JDC. Resultados: Participaron 74 pacientes, el 50% en tratamiento con profilaxis, y 7 años (mediana) con su tratamiento habitual (RIC 17,25). En la encuesta, la JDC recibió la mayor puntuación (75/100, p < 0,001) y la mayor probabilidad de uso en profilaxis (p < 0,001). En la sesión práctica (n = 29), el 62,1% prefirió la JDC y necesitaron de mediana 43 segundos (24,5-82) para la preparación, vs. 4 minutos (1-15) con el tratamiento habitual (p < 0,001). La opinión favorable del médico respecto a la profilaxis resultó muy influyente en la actitud de los participantes hacia la adherencia (OR = 1,324, IC 95% = 1,040-1,685, p = 0,023). Conclusiones: La JDC fue el dispositivo preferido y se mostró con probabilidad de favorecer la profilaxis (AU)

Factor VIII delivery devices in haemophilia A. Barriers and drivers for treatment adherence.

INTRODUCTION AND OBJECTIVE: To capture the experience of haemophilia A patients with their devices for coagulation factor reconstitution, the barriers for treatment adherence; and to determine their preferences, presenting a new double-chamber syringe (DCS). METHOD: A cross-sectional research through a supervised survey and a DCS testing session. RESULTS: Seventy-four (74) patients participated, 50% of them on prophylaxis treatment, and 7 years (median) with their usual treatment (IQR 17.25). In the survey, the DCS received the highest score (75/100, p < 0.001) and it was the most likely to be used in prophylactic treatment (p < 0.001). In the practical testing session (n = 29), 62.1% preferred the DCS, and required 43 seconds as median time (24.5-82) for preparation, vs. 4 minutes (1-15) with their usual treatment (p < 0.001). The favourable opinion of their doctor regarding prophylaxis was very influential on the attitude of participants towards treatment adherence (OR = 1.324, CI 95% = 1.040-1.685, p = 0.023). CONCLUSIONS: The DCS was the preferred device, and was likely to encourage prophylaxis.

Introducción y objetivo: Recabar la experiencia de pacientes con hemofilia A con sus dispositivos de reconstitucion de factor de coagulacion, barreras para la adherencia y determinar sus preferencias, presentando una nueva jeringa de doble camara (JDC). Método: Investigacion transversal mediante encuesta dirigida y sesion de prueba de la JDC. Resultados: Participaron 74 pacientes, el 50% en tratamiento con profilaxis, y 7 anos (mediana) con su tratamiento habitual (RIC 17,25). En la encuesta, la JDC recibio la mayor puntuacion (75/100, p < 0,001) y la mayor probabilidad de uso en profilaxis (p < 0,001). En la sesion practica (n = 29), el 62,1% prefirio la JDC y necesitaron de mediana 43 segundos (24,5-82) para la preparacion, vs. 4 minutos (1-15) con el tratamiento habitual (p < 0,001). La opinion favorable del medico respecto a la profilaxis resulto muy influyente en la actitud de los participantes hacia la adherencia (OR = 1,324, IC 95% = 1,040-1,685, p = 0,023). Conclusiones: La JDC fue el dispositivo preferido y se mostro con probabilidad de favorecer la profilaxis.

Evaluation of compliance with the EGS guidelines in Spain, using Achievable Benchmarks of Care (ABC®) methodology: the IMCA Study.

PURPOSE: To evaluate compliance with diagnostic and therapeutic recommendations of the European Glaucoma Society (EGS) for the management of glaucoma in Spain, as determined using the Achievable Benchmarks of Care approach. METHODS: A panel of 3 experts extracted quality and process-of-care indicators from the EGS guidelines. Investigators from all over the country, representative of Spanish ophthalmologists, were invited to participate in the study, completing a questionnaire on quality indicators (pertaining to the center) and process-of-care indicators (pertaining to all patients) for the first, diagnostic visit (retrospective) and the study visit. RESULTS: A total of 495 Spanish ophthalmologists included 5,060 patients with glaucoma or intraocular hypertension. The treatment was initiated with monotherapy in 88.5% of the patients. Overall, the percentage compliance with diagnostic examinations was 84.6% for intraocular pressure measurement, 96.3% for optic nerve head evaluation, 76.6% for visual field evaluation, 29.5% for central corneal thickness evaluation, and 49.3% at the study visit. The benchmark performance was close to 100% for most examinations. Only 32.8% of the centres had a written protocol for the management of the patients with glaucoma, and in these centers the recommendations of the EGS were followed in a higher proportion (p<0.0001) than in the rest. CONCLUSIONS: The use of local protocols for the management of glaucoma seems to enhance compliance. Benchmark performance was close to 100% in most indicators, suggesting that EGS recommendations are attainable in Spain. The overall performance suggests that certain diagnostic procedures are, in practice, not as widely used as recommended.

A six-month, multicenter, open-label, noncomparative, prospective, observational study of the efficacy and tolerability of atorvastatin in the primary care setting(estudio del control de las hiperlipidemiasen atención primaria): the cheap study.

BACKGROUND: A close relationship exists between high levels of total cholesterol (TC) (particularly low-density lipoprotein cholesterol [LDL-C]) and low levels of high-density lipoprotein cholesterol (HDL-C), which is associated with an increased risk for arteriosclerosis and cardiovascular disease (CVD). Evidence shows that atorvastatin produces significantly greater reductions in LDL-C and TC than other hydroxymethylglutaryl-coenzyme A reductase inhibitors. However, the results achieved in clinical studies could be different from those found in general clinical practice, where patient follow-up is less thorough and poorer compliance may reduce the effectiveness of the lipid-lowering therapy. OBJECTIVE: The aim of this study was to assess the effectiveness of atorvastatin in achieving the LDL-C levels recommended by several Spanish scientific societies, as well as its tolerability in standard clinical use. METHODS: This 6-month, open-label, noncomparative, prospective, observational study was conducted in 1351 primary care centers in Spain. All patients were aged 18 to 80 years and had primary hypercholesterolemia (TC >200 mg/dL and triglycerides [TG] 200 mg/dL and fasting TG 200-400 mg/dL). All patients also had LDL-C levels higher than those established by the Spanish Society of Arteriosclerosis (Sociedad Española de Arteriosclerosis [SEA]) according to baseline cardiovascular risk and previous use of lipid-lowering therapy (for patients with low, moderate, or high cardiovascular risk, the recommended LDL-C goals are ≤175 mg/dL, ≤155 mg/dL, and ≤135 mg/dL, respectively; for patients with CVD, the LDL-C goal is ≤100 mg/dL). None of the patients had creatine kinase activity ≥540 U/L or alanine aminotransferase (ALT) or aspartate aminotransferase (AST) levels ≥60 U/L. Study visits occurred at months 0, 2, and 6 of treatment. Patients received atorvastatin calcium 10 mg/d for 2 months. The dosage was then doubled to 20 mg/d in patients who did not achieve the SEA LDL-C goal and also in those patients whose primary care physicians (PCPs) deemed this higher dosage necessary; this dosage was continued for at least 4 additional months, to complete at least a 6-month course of treatment. The percentage of patients who achieved their goals was used to measure atorvastatin effectiveness. Percentages of change in LDL-C, TC, TG, and HDL-C from baseline to the final study visit also were used as measures of effectiveness. The incidence of adverse events (AEs) per 10,000 patient-months was used for the primary tolerability analysis. A secondary tolerability analysis was performed in all patients treated with atorvastatin who had some recorded follow-up, regardless of whether the patient met inclusion criteria. Information was obtained from data recorded in the case-report forms. RESULTS: A total of 5317 outpatients (2715 women, 2598 men, 4 sex unknown; mean [SD] age, 58.7 [10.5] years) were enrolled. Among patients receiving known dosages of atorvastatin, 1580 of 4033 (39.2%) and 2378 of 3585 (66.3%) patients met the SEA LDL-C goal after 2 and 6 months of therapy, respectively (P