Safety and Efficacy of Botulinum Toxin in the Treatment of Self-Biting Behavior in Lesch-Nyhan Disease.

BACKGROUND: Lesch-Nyhan disease (LND) is a disease of purine metabolism linked to chromosome X due to the absence or near-absence of enzyme hypoxanthine-guanine phosphoribosyltransferase. Patients with LND have a compulsive autoaggressive behavior that consists of self-mutilation by biting. METHODS: The objective of this study was to explore the safety and efficacy of botulinum toxin (BoNT) injected into the masticatory muscles and biceps brachii to reduce self-mutilation in patients with LND. We retrospectively analyzed six patients with LND who were treated with BoNT to prevent automutilatory behavior. RESULTS: The patient ages when started on treatment with BoNT were 4, 4.5, 6.6, 7.9, 13.9, and 32.3 years. Patients received a mean number of injections of 20, ranging from 3 to 29, over a period that ranged from 1.5 to 7.1 years. The maximum total dose of Botox was 21.3 units/kg mean and the maximum total dose of Dysport was 37.5 units/kg mean. A total of 119 injections were performed. Of these 113 (95%) were partially or completely effective. Only three of 119 injections (2.5%) produced adverse effects. CONCLUSIONS: Botulinum toxin is useful and safe for the treatment of self-biting behavior in patients with LND.

Frequency and Prognosis of Treated Hypertensive Patients According to Prior and New Blood Pressure Goals.

United States and European guidelines have recommended new treatment goals for office blood pressure (BP). We examined 9784 hypertensives of the Spanish Ambulatory BP Monitoring (ABPM) registry with office BP treated to the prior goal (<140/90 mm Hg); and evaluated the frequency and all-cause mortality of 4 BP strata depending on whether or not they attained more conservative or new office BP goal (130-139/80-89 and <130/80 mm Hg, respectively) and whether or not BP was controlled according to ABPM criteria in the European and US guidelines (24-hour ambulatory BP <130/80 and <125/75 mm Hg, respectively). Whether achieving or not the new office BP goal, the total-mortality risk during a 5-year follow-up was only significantly higher than the reference (normal office BP and ABPM) when 24-hour ambulatory BP was above goal (hazard ratio from multivariable Cox models was in the range of 2.4-2.9; P<0.001). The frequency of patients achieving the new office BP goal was 34.4%, and the frequencies of those not achieving the ABPM goal were 31.6% and 53.7% using the 130/80 or the 125/75 ABPM goal, respectively. Mean office systolic BP was 129 mm Hg for patients not achieving the ABPM goal. In hypertensive patients controlled under prior office BP goal, the frequency of those achieving new office BP goal <130/80 was high, suggesting this goal can be attained. In addition, patients had a higher mortality risk only when ABPM was above goal despite having mean office systolic BP under control, a condition that was also common.

Automedición en el domicilio frente a lectura de la presión arterial en la consulta en el seguimiento de diabéticos tipo II: efecto sobre la presión arterial ambulatoria y la albuminuria. Estudio aleatorizado / Home blood pressure vs. clinic blood pressure measurement-based follow up in type II diabetics: Effect on 24-h ambulatory BP and albuminuria. Randomised trial

Fundamento y objetivo: Comparar la eficacia en el control de la hipertensión arterial (HTA) y la albuminuria de 2 estrategias de seguimiento basadas en diferentes métodos de evaluación de la presión arterial (PA). Pacientes y métodos: Estudio de intervención aleatorizado, abierto, con grupo control, de 2 años de duración, realizado en 19 centros de atención primaria y una consulta de medicina interna. Se incluyó a diabéticos adultos tipo 2 con PA sistólica (PAS) ≥ 140mmHg sin nefropatía relevante. Los pacientes fueron aleatorizados a: 1) grupo de seguimiento convencional, con un objetivo de PA en la consulta < 140/90mmHg; y 2) grupo de seguimiento basado en cifras de automedición domiciliaria de la PA (AMPA), con un objetivo de PA en el domicilio < 135/85mmHg. En las visitas basal, anual y final se realizaron un perfil bioquímico, medición de albuminuria y monitorización ambulatoria de la PA de 24h. La principal variable de eficacia fue la variación de la PAS ambulatoria de 24h. Como variable secundaria se analizó la albuminuria. Resultados: Fueron evaluados 116 pacientes (edad media: 66,8 años). El descenso medio de la PAS ambulatoria de 24h en 2 años fue de 3,9mmHg (IC del 95%: 1,8-6,1), sin observarse diferencias significativas entre los 2 grupos (p=0,706), así como tampoco entre el resto de valores de PA ambulatoria. La albuminuria inicial fue similar en ambos grupos y no se modificó significativamente durante el seguimiento. Conclusión: En pacientes diabéticos sin nefropatía relevante, un seguimiento basado en AMPA mostró una eficacia similar en el control de la HTA y la albuminuria que el seguimiento convencional

Bakground and objective: To compare the efficacy of two strategies of blood pressure (BP) measurement-based follow-up in hypertension and albuminuria control. Patients and methods: Multicentre, prospective, randomised, open trial with a parallel-group design. Nineteen primary care centres and a hospital clinic participated. Adult type 2 diabetics with systolic BP ≥140mmHg without relevant renal disease were randomised to one of two follow-up strategies: 1) standard follow up, with a clinic BP target <140/90mmHg and 2) self-monitoring home BP (SMHBP)-based follow up, with a BP target <135/85mmHg. Biochemical standard blood variables, albuminuria, and 24-h ambulatory BP monitoring were performed at entry, 12 and 24 months. The main outcome measurement was 24-h ambulatory systolic BP variation. Albuminuria change was analysed as a secondary outcome. Results: 116 patients were analysed (mean age: 66.8 years). Mean systolic ambulatory 24- h BP change in two years was 3.9mmHg (95% CI 1.8-6.1). We did not find significant differences between both groups (p=0.706). Similarly, no differences were found when we compared other ambulatory BP values. Initial albuminuria was similar in both groups and did not significantly changed throughout the follow-up period. Conclusion: In type 2 diabetics without relevant nephropathy a SMHBP- based follow up was equivalent to a standard clinic-based BP follow up in BP and albuminuria control

Implantación del Examen Clínico Objetivo y Estructurado en la Facultad de Medicina de la Universidad Autónoma de Madrid / Implementation of the Objective Structured Clinical Examination (OSCE) in the Madrid Autonomous University Medical School

El examen clínico objetivo y estructurado (ECOE) evalúa las competencias adquiridas por los estudiantes al terminar el grado de medicina. La competencia es el conjunto de conocimientos y habilidades para resolver un problema profesional, con una actitud adecuada. La Conferencia de decanos de las 40 facultades de medicina de España ha establecido que todos los estudiantes del grado de medicina que finalizasen sus estudios en el curso 2015/2016 deberían superar una prueba (ECOE) que evaluase 8 competencias clínicas: anamnesis; exploración física; comunicación; habilidades técnicas; juicio clínico, manejo diagnóstico y tratamiento; prevención y promoción de la salud; relaciones interprofesionales; y aspectos éticos y legales. La Facultad de Medicina de la Universidad Autónoma de Madrid se ha incorporado a este proyecto y ha organizado su primer ECOE para los 197 alumnos de 6.° curso, con 25 estaciones o pruebas. Este trabajo describe las 7 etapas o procesos esenciales que se han seguido para la instauración del ECOE y que puede resultar de utilidad para la comprensión de este complejo examen y su implantación

The Objective Structured Clinical Examination (OSCE) evaluates clinical skills of medical students at the end of medical school training. Professional competence is the set of knowledge and clinical skills that helps to solve professional problems with the correct attitude. The Conference of Deans from all 40 Schools of Medicine in Spain agreed that all students completing their medical training in the 2015/16 academic year should take an exam to assess 8 competency areas: medical history, physical examination, communication skills, clinical judgment, diagnosis, management and treatment, prevention and health promotion, inter-professional relationships, and ethical and legal aspects. The Madrid Autonomous University Medical School took part in this national project, and conducted its first OSCE, with 25 tests, for the 197 students that concluded their training in 2016. This paper describes the 7 stages, or essential processes followed in order to establish the OSCE. This experience may be useful for the understanding and implementation of this complex competence evaluation process

Atherosclerosis as a potential pitfall in the diagnosis of giant cell arteritis.

OBJECTIVES: To explore whether the increase in the intima-media thickness (IMT) in arteriosclerotic disease correlates with the increase in the IMT in temporal arteries (TAs) and if that could mimic the US GCA halo sign. METHODS: Consecutive patients ⩾50 years old with high vascular risk and without signs or symptoms of GCA were included. The carotid US IMT measurements were obtained using a standardized software radiofrequency-tracking technology. Colour Doppler US and grey-scale measurements of the IMT in the branches of both TAs were performed by a second sonographer using a 22 MHz probe. RESULTS: Forty patients were studied (28 men) with a mean age of 70.6 years. The carotid IMT exhibited significant correlation with the TA IMT. A carotid IMT >0.9 mm was associated with a temporal IMT >0.3 mm. Only one patient had an IMT >0.34 mm in two branches. CONCLUSIONS: Atherosclerotic disease with a carotid IMT >0.9 mm increases the TA IMT and might mimic the halo sign. As atherosclerosis is common in this age group, we propose a cut-off of TA IMT >0.34 mm in at least two branches to minimize false positives in a GCA diagnosis.

Home blood pressure vs. clinic blood pressure measurement-based follow up in type ii diabetics: Effect on 24-h ambulatory BP and albuminuria. Randomised trial. / Automedición en el domicilio frente a lectura de la presión arterial en la consulta en el seguimiento de diabéticos tipo ii: efecto sobre la presión arterial ambulatoria y la albuminuria. Estudio aleatorizado.

BAKGROUND AND OBJECTIVE: To compare the efficacy of two strategies of blood pressure (BP) measurement-based follow-up in hypertension and albuminuria control. PATIENTS AND METHODS: Multicentre, prospective, randomised, open trial with a parallel-group design. Nineteen primary care centres and a hospital clinic participated. Adult type 2 diabetics with systolic BP ≥140mmHg without relevant renal disease were randomised to one of two follow-up strategies: 1) standard follow up, with a clinic BP target <140/90mmHg and 2) self-monitoring home BP (SMHBP)-based follow up, with a BP target <135/85mmHg. Biochemical standard blood variables, albuminuria, and 24-h ambulatory BP monitoring were performed at entry, 12 and 24 months. The main outcome measurement was 24-h ambulatory systolic BP variation. Albuminuria change was analysed as a secondary outcome. RESULTS: 116 patients were analysed (mean age: 66.8 years). Mean systolic ambulatory 24- h BP change in two years was 3.9mmHg (95% CI 1.8-6.1). We did not find significant differences between both groups (p=0.706). Similarly, no differences were found when we compared other ambulatory BP values. Initial albuminuria was similar in both groups and did not significantly changed throughout the follow-up period. CONCLUSION: In type 2 diabetics without relevant nephropathy a SMHBP- based follow up was equivalent to a standard clinic-based BP follow up in BP and albuminuria control.

Clinic Versus Daytime Ambulatory Blood Pressure Difference in Hypertensive Patients: The Impact of Age and Clinic Blood Pressure.

Clinic blood pressure (BP) is usually higher than daytime ambulatory BP in hypertensive patients, but some recent studies have challenged this view, suggesting that this relationship is strongly influenced by age. We used the Spanish ambulatory BP monitoring cohort to examine differences between clinic and daytime BP by age among 104 639 adult hypertensive patients (office systolic/diastolic BP ≥140/90 mm Hg or treated) in usual primary-care practice, across the wide age spectrum. To assess the impact of age, cardiovascular variables, and clinic BP on the clinic-daytime BP differences, we built multivariable regression models of the average BP differences, white-coat hypertension (high clinic BP and normal daytime BP), and masked hypertension (normal clinic BP and high daytime BP). In most patients, mean clinic BP values were higher than daytime BP at all ages. Some 36.7% of patients had white-coat hypertension (amounting to 50% at clinic systolic BP of 140-159 mm Hg) and 3.9% had masked hypertension (amounting to 18% at clinic systolic BP of 130-139 mm Hg). Age explained 0.1% to 1.7% of the variance of quantitative or categorical BP differences (P<0.001). Cardiovascular variables explained an additional 1.6% to 3.4% of the variance (P<0.001). Finally, clinic BP generally explained ≥20% more of the variance (P<0.01). In this large study in usual clinical practice, clinic BP misclassified hypertension status in >40% of patients. This misclassification was not importantly influenced by age but was more evident in patients with borderline/grade 1 hypertension. These findings reinforce the importance of ambulatory BP monitoring for defining BP status in routine clinical practice.

Hiperuricemia y gota: impacto de la ecografía / Hyperuricemia and gout: The impact of ultrasonography

No disponible

Obesidad y episodios vasculares en la diabetes mellitus tipo 2 / Obesity and vascular events in type 2 diabetes mellitus

No disponible

Influence of immune activation and inflammatory response on cardiovascular risk associated with the human immunodeficiency virus.

Patients infected with the human immunodeficiency virus (HIV) have an increased cardiovascular risk. Although initially this increased risk was attributed to metabolic alterations associated with antiretroviral treatment, in recent years, the attention has been focused on the HIV disease itself. Inflammation, immune system activation, and endothelial dysfunction facilitated by HIV infection have been identified as key factors in the development and progression of atherosclerosis. In this review, we describe the epidemiology and pathogenesis of cardiovascular disease in patients with HIV infection and summarize the latest knowledge on the relationship between traditional and novel inflammatory, immune activation, and endothelial dysfunction biomarkers on the cardiovascular risk associated with HIV infection.

High prevalence of masked uncontrolled hypertension in people with treated hypertension.

AIM: There are limited data on the quality of treated blood pressure (BP) control during normal daily life, and in particular, the prevalence of 'masked uncontrolled hypertension' (MUCH) in people with treated and seemingly well-controlled BP is unknown. This is important because masked hypertension in 'treatment naïve' patients is associated with a high risk of cardiovascular events. We therefore conducted the first study to define the prevalence and characteristics of MUCH among a large sample of hypertensive patients in routine clinical practice in whom BP was treated and controlled to recommended clinic BP goals. METHODS AND RESULTS: We analysed data from the Spanish Society of Hypertension ambulatory blood pressure monitoring (ABPM) Registry and identified patients with treated and controlled BP according to current international guidelines (clinic BP <140/90 mmHg). Masked uncontrolled hypertension was diagnosed in these patients if despite controlled clinic BP, the mean 24-h ABPM average remained elevated (24-h systolic BP ≥130 mmHg and/or 24-h diastolic BP ≥80 mmHg). From 62 788 patients with treated BP in the Spanish registry, we identified 14 840 with treated and controlled clinic BP, of whom 4608 patients (31.1%) had MUCH according to 24-h ABPM criteria (mean age 59.4 years, 59.7% men). The prevalence of MUCH was significantly higher in males, patients with borderline clinic BP (130-9/80-9 mmHg), and patients at high cardiovascular risk (smokers, diabetes, obesity). Masked uncontrolled hypertension was most often because of poor control of nocturnal BP, with the proportion of patients in whom MUCH was solely attributable to an elevated nocturnal BP almost double that solely attributable to daytime BP elevation (24.3 vs. 12.9%, P < 0.001). CONCLUSION: The prevalence of masked suboptimal BP control in patients with treated and well-controlled clinic BP is high. Clinic BP monitoring alone is thus inadequate to optimize BP control because many patients have an elevated nocturnal BP. These findings suggest that ABPM should become more routine to confirm BP control, especially in higher risk groups and/or those with borderline control of clinic BP.

[Sugar and cardiovascular disease]. / Azúcar y enfermedades cardiovasculares.

Cardiovascular diseases are the leading cause of death in the Spanish population and may be a relationship between the prevalence of these and excessive sugar consumption. In recent years, researchers have focused on the properties of these nutrients. Although there are many studies examining this association, the results are not unanimous. In any case there is sufficient basis for designing public health strategies in order to reduce the consumption of sugary drinks as part of a healthy lifestyle. Therefore, the question we address is: sugar intake in abundant amounts, is associated with a higher risk of cardiovascular disease? We use as the focus of the discussion SAFO analysis model.

Las enfermedades cardiovasculares constituyen la principal causa de muerte en la población española y podría existir una relación entre la prevalencia de las mismas y el consumo excesivo de azúcar. En estos últimos años, los investigadores se han centrado en las propiedades de estos nutrientes. Aunque existen muchos estudios que analizan dicha asociación, los resultados no son unánimes. En cualquier caso, existe fundamento suficiente para diseñar estrategias de salud pública de cara a reducir el consumo de bebidas azucaradas, como parte de un estilo de vida saludable. Por lo tanto, la cuestión que abordamos es: ¿la ingesta de azúcar, en cuantía abundante, se asocia un mayor riesgo de padecer enfermedad cardiovascular? Para ello utilizamos como eje de la discusión el modelo de análisis DAFO.

Azúcar y enfermedades cardiovasculares / Sugar and cardiovascular disease

Las enfermedades cardiovasculares constituyen la principal causa de muerte en la población española y podría existir una relación entre la prevalencia de las mismas y el consumo excesivo de azúcar. En estos últimos años, los investigadores se han centrado en las propiedad desde estos nutrientes. Aunque existen muchos estudios que analizan dicha asociación, los resultados no son unánimes. En cualquier caso, existe fundamento suficiente para diseñar estrategias de salud pública de cara a reducir el consumo de bebidas azucaradas, como parte de un estilo de vida saludable. Por lo tanto, la cuestión que abordamos es: ¿la ingesta de azúcar, en cuantía abundante, se asocia un mayor riesgo de padecer enfermedad cardiovascular? Para ello utilizamos como eje de la discusión el modelo de análisis DAFO (AU)

Cardiovascular diseases are the leading cause of death in the Spanish population and may be a relationship between the prevalence of these and excessive sugar consumption. In recent years, researchers have focused on the properties of these nutrients. Although there are many studies examining this association, the results are not unanimous. In any case there is sufficient basis for designing public health strategies in order to reduce the consumption of sugary drinks as part of a healthy lifestyle. Therefore, the question we address is: sugar intake in abundant amounts, is associated with a higher risk of cardiovascular disease? We use as the focus of the discussion SAFO analysis model (AU)

Safety and efficacy of aliskiren/amlodipine/hydrochlorothiazide triple combination in patients with moderate to severe hypertension: a 54-week, open-label study.

Combination antihypertensive therapies are recommended to attain blood pressure (BP) targets especially in high-risk patients in whom rapid and pronounced BP control is essential. This 28- to 54-week, open-label, multicenter study evaluated the safety and efficacy of a triple combination, aliskiren with amlodipine and hydrochlorothiazide (HCTZ), in patients with moderate to severe hypertension. Following a washout period of up to 4 weeks, patients received aliskiren/HCTZ 300/12.5 mg for 1 week, followed by add-on amlodipine 5 mg for 1 week. Thereafter, the doses of amlodipine and HCTZ were doubled. The first 206 of 564 patients who completed 28 weeks of study continued for an additional 26 weeks. Safety was assessed by recording all adverse events. Efficacy variables included changes in BP from baseline to endpoint and BP control rate. Of 564 patients, 493 completed the study. Peripheral edema (9.4%), headache (5.7%), nasopharyngitis (4.1%), and bronchitis (3.7%) were reported frequently. Clinically significant reductions in mean sitting systolic BP/mean sitting diastolic BP from baseline (-34.2/-20.3 mm Hg and -37.3/-21.8 mm Hg at weeks 28 and 54, respectively) were observed. Corresponding BP control rates were 69.1% and 77.1%. The aliskiren/amlodipine/HCTZ combination in patients with moderate to severe hypertension was well tolerated and provided clinically significant BP reductions and effective BP control.

Knowledge of stroke warning symptoms and intended action in response to stroke in Spain: a nationwide population-based study.

BACKGROUND: Worldwide thrombolysis rates remain suboptimal. Ambulance transfer is associated with greater use of this time-dependent treatment. Information on public awareness of stroke symptoms is important for planning effective education programs to promote calling of emergency services for suspected stroke. However, there is a paucity of data on this subject in European countries. Our objectives were to explore the recognition of stroke symptoms, awareness of the need to activate the emergency medical services for acute stroke events, and the association between knowledge of warning symptoms and intent to call for an ambulance among a sample representative of the adult population of Spain. This is the largest study on this subject to date in Europe. METHODS: The data were taken from the Study on Nutrition and Cardiovascular Risk in Spain, a cross-sectional study conducted in a sample representative of the Spanish noninstitutionalized population aged ≥18 years in 2008-2010. Study participants were selected by multistage clustered random sampling. The households within each section were selected by random telephone dialing using the landline telephone directory as the sampling frame. Subjects in the households were selected proportionally to the distribution of the population of Spain by sex and age. The study included a computer-assisted telephone interview on stroke symptom knowledge and the first action to perform in a stroke event, based on the American Heart Association and American Stroke Association recommendations, and two home visits to perform a physical examination and to obtain blood samples. RESULTS: Among 11,827 adults, 7,711 (65.2%; 95% CI = 64.1-66.3) identified 4-6 stroke warning symptoms, considered as adequate knowledge. A total of 1,348 (11.4%) were unable to classify any of the symptoms correctly. In the multivariate analysis, higher education was significantly associated with better knowledge of symptoms, and age ≥65 years, fair/poor self-rated health, history of obesity and known diabetes were significantly associated with less knowledge of stroke symptoms. One in 5 individuals indicated they would do something other than calling for an ambulance if they thought someone was having a stroke. The number of specific stroke warning symptoms known was directly associated with the intent to call an ambulance in a stroke event (OR adjusted for sociodemographic and clinical variables = 1.06 per symptom, 95% CI = 1.03-1.09; p < 0.001). CONCLUSIONS: In this population-based study, stroke symptom knowledge was suboptimal and only modestly associated with the intent to call for an ambulance. Educational interventions are needed to link stroke recognition more strongly to an immediate need to call for an ambulance in order to increase stroke patients' access to thrombolysis.