Epidemiology of Fabry disease in patients in hemodialysis in the Madrid community.

This study screened for Fabry disease (FD) in patients in hemodialysis (HD) in the region of Madrid (CAM) with a cross-sectional design to evaluate HD-prevalent patients, followed by a three-year period prospective design to analyze HD-incident patients. INCLUSION CRITERIA: patients older than 18 years on HD in the CAM, excluding patients diagnosed with any other hereditary disease with renal involvement different from FD, that sign the Informed Consent (IC). EXCLUSION CRITERIA: underaged patients or not agreeing or not being capable of signing the IC. RESULTS: 3470 patients were included, 63% males and with an average age of 67.9±9.7 years. 2357 were HD-prevalent patients and 1113 HD-incident patients. For HD-prevalent patients, average time in HD was 45.2 months (SD 51.3), in HD-incident patients proteinuria was present in 28.4%. There were no statistical differences in plasmatic alpha-galactosidase A (α-GAL-A) activity or Lyso-GL-3 values when comparing HD-prevalent and HD-incident populations and neither between males and females. A genetic study was performed in 87 patients (2.5% of patients): 60 male patients with decreased enzymatic activity and 27 female patients either with a decreased GLA activity, increased Lyso-Gl3 levels or both. The genetic variants identified were: p.Asp313Tyr (4 patients), p.Arg220Gln (3 patients) and M290I (1 patient). None of the identified variants is pathogenic. CONCLUSIONS: 76% of HD Centers of the CAM participated in the study. This is the first publication to describe the prevalence of FD in the HD-population of a region of Spain as well as its average α-GAL-A-activity and plasmatic Lyso-Gl3 levels. It is also the first study that combines a cross-sectional design with a prospective follow-up design. This study has not identified any FD patient.

Consulta de acceso vascular: resultados antes y después de la instauración de un programa multidisciplinar con realización de ecografía doppler de rutina / Vascular access clinic results before and after implementing a multidisciplinary approach adding routine Doppler ultrasound

ANTECEDENTES Y OBJETIVO: El abordaje multidisciplinar y el uso de ecografía doppler (ED) en la creación y vigilancia del acceso vascular (AV) puede mejorar la prevalencia y permeabilidad de las fístulas arteriovenosas (FAV) para hemodiálisis. El objetivo de este estudio es analizar el impacto de una nueva consulta multidisciplinar (CMD) de AV con ED de rutina. MATERIAL Y MÉTODOS: Evaluamos los resultados de la consulta de AV en 2014 (pre-CMD) y 2015 (CMD), antes y después de la implantación de un equipo multidisciplinar (cirujano vascular/nefrólogo) con ED de rutina en mapeo prequirúrgico y FAV prevalente. RESULTADOS: Se evaluaron 345 pacientes en 2014 (pre-CMD) y 364 pacientes en 2015 (CMD). En ambos periodos se realizó un número similar de cirugías, 172 vs. 198, p = 0,289, con tendencia a aumentar las cirugías preventivas de reparación de FAV en el periodo CMD, 17 vs. 29 (p = 0,098). En FAV de nueva creación (155 vs. 169), disminuyó la tasa de fallo primario en el periodo CMD, 26,4 vs. 15,3%, p = 0,015 y aumentó de forma no significativa la realización de FAV radiocefálicas distales, 25,8 vs. 33,2% (n = 40 vs. 56), p = 0,159. También aumentó la concordancia entre la indicación quirúrgica en la consulta y la cirugía realizada (81,3 vs. 93,5%, p = 0,001). En el periodo CMD se solicitaron menos exploraciones radiológicas desde la consulta, 78 vs. 35 (p < 0,001), con una reducción del gasto sanitario (81.716€ vs. 59.445€). CONCLUSIONES: El manejo multidisciplinar y la utilización del ED de rutina permiten mejorar los resultados de AV, con disminución de la tasa de fallo primario de FAV, más opciones de FAV distal nativa, mejor manejo de la FAV prevalente disfuncionante y menor coste en exploraciones radiológicas

BACKGROUND: A multidisciplinary approach and Doppler ultrasound (DU) assessment for the creation and maintenance of arteriovenous fistulas (AVF) for haemodialysis can improve prevalence and patency. The aim of this study was to analyse the impact of a new multidisciplinary vascular access (VA) clinic with routine DU. MATERIAL AND METHODS: We analysed the VA clinic results from 2014 and 2015, before and after the implementation of a multidisciplinary team protocol (vascular surgeon/nephrologist) with routine DU in preoperative mapping and prevalent AVF. RESULTS: We analysed 345 and 364 patients from 2014 and 2015 respectively. The number of surgical interventions was similar in both periods (p = .289), with a trend towards an increase in preventive surgical repair of AVF in 2015 (17 vs. 29, p = .098). 155 vs. 169 new AVF were performed in 2014 and 2015, with a significantly lower primary failure rate in 2015 (26.4 vs. 15.3%, p = .015), and a non-significant increase in radiocephalic AVF, 25.8 vs. 33.2% (n = 40 vs. 56), p = .159. The concordance between the indication at the clinic and the surgery performed also increased (81.3 vs. 93.5%, p = .001). Throughout 2015 fewer complementary imaging test were requested from the clinic (78 vs. 35, p < .001), with a corresponding reduction in costs (€87,716 vs. €59,445). CONCLUSIONS: Multidisciplinary approach with routine DU can improve VA results, with a decrease in primary failure rate, higher likelihood of radiocephalic AVF, better management of dis-functioning AVF and lower radiological test costs

Vascular access clinic results before and after implementing a multidisciplinary approach adding routine Doppler ultrasound. / Consulta de acceso vascular: resultados antes y después de la instauración de un programa multidisciplinar con realización de ecografía doppler de rutina.

BACKGROUND: A multidisciplinary approach and Doppler ultrasound (DU) assessment for the creation and maintenance of arteriovenous fistulas (AVF) for haemodialysis can improve prevalence and patency. The aim of this study was to analyse the impact of a new multidisciplinary vascular access (VA) clinic with routine DU. MATERIAL AND METHODS: We analysed the VA clinic results from 2014 and 2015, before and after the implementation of a multidisciplinary team protocol (vascular surgeon/nephrologist) with routine DU in preoperative mapping and prevalent AVF. RESULTS: We analysed 345 and 364 patients from 2014 and 2015 respectively. The number of surgical interventions was similar in both periods (p=.289), with a trend towards an increase in preventive surgical repair of AVF in 2015 (17 vs. 29, p=.098). 155 vs. 169 new AVF were performed in 2014 and 2015, with a significantly lower primary failure rate in 2015 (26.4 vs. 15.3%, p=.015), and a non-significant increase in radiocephalic AVF, 25.8 vs. 33.2% (n=40 vs. 56), p=.159. The concordance between the indication at the clinic and the surgery performed also increased (81.3 vs. 93.5%, p=.001). Throughout 2015 fewer complementary imaging test were requested from the clinic (78 vs. 35, p <.001), with a corresponding reduction in costs (€87,716 vs. €59,445). CONCLUSIONS: Multidisciplinary approach with routine DU can improve VA results, with a decrease in primary failure rate, higher likelihood of radiocephalic AVF, better management of dis-functioning AVF and lower radiological test costs.

Identificar situaciones de riesgo para los pacientes en hemodiálisis mediante la adecuada valoración de su composición corporal / Risk identification in haemodialysis patients by appropriate body composition assessment

Introducción Circunstancias como el género, la edad, la presencia de diabetes mellitus (DM) y la insuficiencia renal tienen impacto sobre la composición corporal de los pacientes. Sin embargo, a la hora de evaluar parámetros nutricionales como el tejido magro y graso de los pacientes en hemodiálisis (HD) se emplean valores de referencia provenientes de población sana. Objetivos: Analizar la composición corporal mediante bioimpedancia espectroscópica (BIS) de 6.395 pacientes en HD para obtener valores de referencia de índice de tejido magro (ITM) y de índice de tejido graso (ITG) procedentes de pacientes en HD y confirmar su validez al demostrar que aquellos con un ITM por debajo del percentil 10 calculado para su grupo tienen mayor riesgo de muerte. Material y métodos Usamos la BIS para determinar el ITM e ITG de nuestra cohorte de pacientes en HD en España. Calculamos el percentil 10 y el percentil 90 del ITM e ITG en cada decil de edad de pacientes, agrupados según su género y presencia de DM. Recogemos parámetros clínicos, analíticos y demográficos. Resultados: Objetivamos que los valores del percentil 10 y del 90 de ITM/ITG varían en función del grupo (edad, género y presencia de DM) y que, tras ajustar por otros factores de riesgo como la sobrehidratación, los pacientes con ITM inferior al percentil 10 tienen mayor riesgo relativo de muerte (OR 1,57) que aquellos con valores superiores. Conclusiones: Monitorizar el ITM e ITG de los pacientes en HD CON adecuados valores de referencia puede ser útil para identificar situaciones de riesgo en los pacientes en HD (AU)

Introduction: Circumstances such as gender, age, diabetes mellitus (DM) and renal failure impact on the body composition of patients. However, we use nutritional parameters such as lean and fat tissue with reference values from healthy subjects to assess the nutritional status of haemodialysis (HD) patients. Aims: To analyse body composition by bioimpedance spectroscopy (BIS) of 6395 HD patients in order to obtain reference values of lean tissue index (LTI) and fat tissue index (FTI) from HD patients; and to confirm its validity by showing that those patients with LTI below the 10th percentile calculated for their group have greatest risk of death. Material and methods: We used the BIS to determine the LTI and FTI in our cohort of HD patients in Spain. We calculated the 10th percentile and 90th percentile of LTI and FTI in each age decile for patients grouped by gender and presence of DM. We collected clinical, laboratory and demographic parameters. Results: The LTI/FTI 10 and 90 percentile values varied by group (age, gender and presence of DM) and, after adjusting for other risk factors such as fluid overload, those patients with LTI lower than percentile 10 had a higher relative risk of death (OR 1.57) than those patients with higher values. Conclusions: Monitoring the LTI and FTI of patients on HD using suitable reference values may help to identify risk in this patient population (AU)

Risk identification in haemodialysis patients by appropriate body composition assessment. / Identificar situaciones de riesgo para los pacientes en hemodiálisis mediante la adecuada valoración de su composición corporal.

INTRODUCTION: Circumstances such as gender, age, diabetes mellitus (DM) and renal failure impact on the body composition of patients. However, we use nutritional parameters such as lean and fat tissue with reference values from healthy subjects to assess the nutritional status of haemodialysis (HD) patients. AIMS: To analyse body composition by bioimpedance spectroscopy (BIS) of 6395 HD patients in order to obtain reference values of lean tissue index (LTI) and fat tissue index (FTI) from HD patients; and to confirm its validity by showing that those patients with LTI below the 10th percentile calculated for their group have greatest risk of death. MATERIAL AND METHODS: We used the BIS to determine the LTI and FTI in our cohort of HD patients in Spain. We calculated the 10th percentile and 90th percentile of LTI and FTI in each age decile for patients grouped by gender and presence of DM. We collected clinical, laboratory and demographic parameters. RESULTS: The LTI/FTI 10 and 90 percentile values varied by group (age, gender and presence of DM) and, after adjusting for other risk factors such as fluid overload, those patients with LTI lower than percentile 10 had a higher relative risk of death (OR 1.57) than those patients with higher values. CONCLUSIONS: Monitoring the LTI and FTI of patients on HD using suitable reference values may help to identify risk in this patient population.

Intervención educativa sobre VIH/SIDA en una escuela de nivel secundario / Educative intervention about HIV/AIDS in a Junior High School

Introducción: el síndrome de inmunodeficiencia adquirida constituye una amenaza para el desarrollo y como tal debe ser visto. Objetivo: modificar el nivel de conocimiento y actuación sobre virus de inmunodeficiencia humana y el síndrome de inmunodeficiencia adquirida. Métodos: se realizó un estudio cuasi experimental de tipo de intervención educativa, la muestra estuvo constituida por 74 estudiantes, el instrumento de trabajo se aplicó una encuesta estructurada. Las preguntas midieron: medidas de prevención, factores de riesgo, conducta a seguir y vías de obtención del conocimiento. Para conocer la posible significación estadística con la aplicación de X2. El desarrollo de la investigación se basó en tres etapas: diagnóstico, intervención y evaluación.Resultados: se encontró un predominio del sexo femenino y color de piel blanca, comprobándose que había desconocimiento sobre las vías de transmisión del virus de inmunodeficiencia humana y el síndrome de inmunodeficiencia adquirida, como evitarlo y brindar apoyo. Se realizó un programa educativo; durante la intervención se realizó la investigación-acción con la aplicación de palestra, debates, demostraciones, pancartas, observándose incremento en los conocimientos por lo que se consideró de utilidad que se comprobó durante la evaluación Se halló relación favorable estadísticamente significativa entre el antes y después de la intervención. Conclusiones: hubo un predominio del sexo femenino. Conocieron que las relaciones sexuales, el uso de sangre infectada, los tatuajes, compartir jeringuillas y el tránsito de madre a hijo como las formas más frecuentes de transmisión de la enfermedad además que la principal forma de evitarlo es el uso del condón y la actitud que debe tener es de apoyar a las personas con el virus de inmunodeficiencia humana y el síndrome de inmunodeficiencia adquirida (AU)

Introduction: acquired Immunodeficiency Syndrome is a threat and such, must be seen. Objective: to modify the level of knowledge and to action about Acquired Immunodeficiency Syndrome. Methods: a quasi experimental study in form of educative intervention was carried out, the sample was formed by 74 students, and a structured survey was applied. The questions measured: prevention measures, risk factors, management to follow and ways of obtaining knowledge. To know the possible statistic meaning with the application of X2. The development of the investigation was based on three stages: diagnosis, intervention and evaluation. Results: the female sex and the white race prevailed, proving that there was no knowledge about the ways of transmission of Human Immunodeficiency Virus and Acquired Immunodeficiency Syndrome, how to prevent it and giving support. An educative program was performed; during the intervention an investigation-action was carried out with the application of competitions, debates, shows, advertisements observing an increasing of the knowledge which was proved, so it was considered very useful. A favorable statistically significant relation was found before and after the intervention. Conclusions: there was a predominance of the female sex They knew that sexual relations, the use of infected blood, tattoos, sharing syringes, and the passing by from mother to child are the most common ways of transmission of the disease, besides, the main way of preventing it, is using condom and the attitude to have, must be the support to patients with Acquired Immunodeficiency Syndrome (AU)

Clinical, analytical and bioimpedance characteristics of persistently overhydrated haemodialysis patients.

BACKGROUND: Fluid overload is an important and modifiable cardiovascular risk factor for haemodialysis patients. So far, the diagnosis was based on clinical methods alone. Nowadays, we have new tools to assess more objectively the hydration status of the patients on haemodialysis, as BCM (Body Composition Monitor). A Relative Overhydration (AvROH) higher than 15% (it means, Absolute Overhydration or AWOH higher than 2.5 Litres) is associated to greater risk in haemodialysis. However, there is a group of maintained hyperhydrated patients. The aim of the present study is to identify the characteristics of patients with maintained hyperhydrated status (AvROH higher than 15% or AWOH higher than 2.5 liters). The secondary aim is to show the hemodynamic and analytical changes that are related to the reduction in hyperhydration status. METHODS: Longitudinal cohort study during six months in 2959 patients in haemodialysis (HD) that are grouped according to their hydration status by BCM. And we compare their clinical, analytical and bioimpedance spectroscopy parameters. RESULTS: The change in overhydration status is followed by a decrease in blood pressure and the need for hypotensive drugs (AHT) and erythropoiesis stimulating agents (ESA). The target hydration status is not reached by two subgroups of patients. First, in diabetic patients with a high comorbidity index and high number of hypotensive drugs (AHT) but a great positive sodium gradient during dialysis sessions; and, younger non-diabetic patients with longer time on hemodialysis and positive sodium gradient, lower fat tissue index (FTI) but similar lean tissue index (LTI) and albumin than those with a reduction in hyperhydration status. CONCLUSION: Those patients with a reduction in hyperhydration status, also show a better control in blood pressure and anemia with less number of AHT and ESA. The maintained hyperhydrated patients, diabetic patients with many comorbidities and young men patients with longer time on hemodialysis and non-adherence treatment, can profit from a constant monitoring of their hydration state as well as an individualized treatment (dialysis and drugs).