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Introduction Cystic fibrosis is the most lethal autosomal recessive disease with higher prevalence in Caucasian population [1]. More than 1,800 mutations have been identified and can be clustered in six functional groups, therefore, altering the secretion and absorption in different tissues. Recurrent infections result in an increased damage to the lungs that leads to hypoxia in peripheric tissues such as skeletal muscle. Then, skeletal muscle weakness displays very early in patients with CF [2]. Recent studies have shown that CFTR protein is expressed in skeletal muscle, and therefore CF has a direct effect in this tissue [3]. Both respiratory damage and muscular weakness result in a reduced exercise capacity that will lead to exercise intolerance and reduction of expectoration and pulmonary function. Objective: Determine the effect of a strength exercise program on muscle function in children with CF. Methods: 17 pediatric patients with CF participated in a randomized, prospective, controlled design. They were divided in two groups: Control Group (CG) (n=9; Age 12.43 ± 3.78 years; follow standard medical care), and Exercise Group (EG) (n=8; Age 12.75 ± 3.06 years; follow 8 weeks of supervised exercise program). We measured muscle strength by 5RM test of both the upper and lower limbs in pediatric weight training machines. Mean differences between groups and measurements was determined using a mixed multivariate analysis of variance. Results: The EG showed a significant improvement in strength relative to body weight compared to the CG (p=0.015). A large and significant effect size was observed on the leg press strength (p=0.003; η2p =0.514), bench press (p=0.008; η2p=0.428) and seated lateral row (p=0.008; η2p =0.435). Discussion: In this preliminary data it seems that the muscles of the CF pediatric patient can respond to exercise and improve muscle strength despite having CFTR deficit. Practical application: To maintain a correct body composition and physical condition in CF it’s related with better disease prognosis. Strength exercise programs may be a useful and capable tool to maintain and improve the body muscle mass in CF. Sin financiación No data 2019 UEM

Introducción: La fibrosis quística es la enfermedad genética potencialmente fatal más común entre niños caucásicos, pese a la gravedad, gracias a los avances que ha habido en los últimos años en el diagnóstico, ha aumentado la esperanza de vida y esto hace necesario analizar su condición física (CF) en las distintas etapas que pueden atravesar en la edad adulta. Objetivo: Describir los niveles de fuerza muscular del tren inferior, la capacidad cardiorrespiratoria y la calidad de vida (CV) de sujetos adultos con FQ en tres etapas diferentes de la enfermedad. Material y Método. Las variables de este estudio descriptivo fueron: la fuerza muscular de las extremidades inferiores, medida a través del test “Chair Stand Test” (repeticiones/minuto)[1]; la capacidad cardiorrespiratoria fue analizada a través de un test de esfuerzo incremental en tapiz rodante con análisis de gases, valorando el consumo de oxígeno pico (VO2pico) alcanzado (ml·kg-1·min-1); y la CV, analizada utilizando el test “Cystic Fibrosis Questionnaire-Revised” (CFQ-R)[2]. La muestra se clasificó en tres grupos: grupo 1) espera de trasplante; grupo 2) post-trasplante; grupo 3) volumen espiratorio forzado (FEV) menor a 60%. El programa estadístico utilizado para valorar los resultados fue el “Statistical Programme Social Science” (SPSS-19). Los datos se presentan con media y desviación estándar. Resultados. 14 pacientes (71% mujeres) cumplieron los criterios de inclusión de este estudio. La media de edad fue de 36±1 años. Las características de los pacientes por grupos fueron: grupo 1 [n4; edad(años) 37±7; VO2pico(ml·kg-1·min-1) 27,5±5,2; fuerza(repeticiones) 21±6; dimensión física CV(%) 62,5±14,8]; grupo 2 [n5; edad(años) 38±7; VO2pico(ml·kg-1·min-1) 27,2±4,9; fuerza(repeticiones) 24±6; dimensión física CV(%) 82,5±25,9]; grupo 3 [n5; edad(años) 33±12; VO2pico(ml·kg-1·min-1) 32,8±7,8; fuerza(repeticiones) 24,6; dimensión física CV(%) 66,7±24,2]. Discusión. El grupo de pacientes con FQ con un FEV menor a 60% obtuvo resultados ligeramente superiores que el resto de los grupos analizados. Sheppard E et al. concluyeron que el test funcional utilizado que valora la fuerza muscular se puede aplicar con éxito en ámbito clínico [1]. Aplicación práctica: Tras los resultados obtenidos sería de gran interés poder hacer un estudio controlado aleatorizado aplicando un programa de entrenamiento y valorar sus efectos. Sin financiación No data 2019 UEM

Objetivo: Evaluar los efectos de un programa de entrenamiento sobre la fuerza muscular en un grupo de niños y adolescentes diagnosticados con tumores sólidos (TS) y linfomas (L) durante la fase de tratamiento quimioterápico neoadyuvante. Método: El diseño fue un ensayo controlado aleatorizado. La variable de fuerza fue medida en kilogramos a través del test 5repeticiones máximas (5RM), analizando diferentes grupos musculares. Esta variable se valoró en tres momentos diferentes:1) momento A, inicio del tratamiento; 2) momento B, fin del tratamiento; 3) momento C, dos meses después del cese delentrenamiento (desentrenamiento). La dosis de entrenamiento fue: a) la frecuencia de las sesiones fue tres entrenamientos semanales (60–70 minutos), realizando tres series de 8–15repeticiones en un circuito de cinco ejercicios, b) intensidad, 50%de 1RM estimado, c) duración del periodo de tratamiento quimioterápico; d) en máquinas pediátricas adaptadas. El testANOVA 3x2 [Statistical Programme Social Science (SPSS–v24)] fue utilizado para valorar el análisis estadístico. Resultados: 49 pacientes (71% varones) cumplieron los criterios de inclusión de este estudio, aleatorizados en 25 casos grupo control y 24 intervención. La duración y adherencia promedio del entrenamiento fueron de 19±2 semanas y del68±4%, respectivamente. Encontramos diferencias significativas en la interacción tiempo*grupo en todos los ejercicios de fuerza(p

INTRODUCTION: Physical inactivity and a low adherence to the Mediterranean diet produces alterations in body composition that may determinate a worse control of chronic diseases such as asthma (1), this is one of the most frequent diseases in childhood. Some investigation in animal show the exercise can be decreased an asthma inflammation and bronchial hyperresponsiveness (2), as well as systemic inflammation, which could help control asthma (3) Objectives: to analyze the effect of aerobic and strength exercise program in asthmatic children with symptoms of bronchospasm induced by exercise, with physical exercise on variables of physical condition and quality of life. METHODS: Randomized controlled trial with control group that compared their usual physical activity vs. exercise program for 12 weeks, 3 days/week, and 60 min/day. It is assessed the physical condition (ergospirometry on treadmill and strength of peripheral muscles) and quality of life with Pediatric Quality of Life Questionnaire (PAQLQ). RESULTS: The study included 43 children from 7 to 17 years old distributed in Control Group (CG, n = 27) and Intervention Group (IG, n = 16). The average adherence to the program was 82.8%. In VO2peak in ml · kg - 1 · min - 1 the IG had 3.31 ± 1.51 with respect to the CG that obtained 0.25 ± 0.71 in the interaction time per group was p(txg) = 0.064 with an increase in the IG of with respect to the CG, Statistically significant improvements were observed in all strength parameters evaluated, with more significance in High Row p(txg) = 0.006, Leg Press p(txg) = 0.002 and Hamstrings p(txg) = 0.003. We couldn’t see improves in quality of life. CONCLUSION: Similar studies in children with asthma observe improvements in VO2max in 1 METS approximately what, according to investigations is a considerable improvement for this population (4), also some investigation shows than the strength in the same population improves significantly and recent systematic review are demonstrating an adequate level of physical activity with a protective factor against asthma (5). In relation with PAQLQ the values weren’t changed probably because the sample has high results in PRE-PAQLQ, 53,3% of them has controlled asthma with at least two drugs. In conclusion 12 weeks of exercise program improves both, cardiorespiratory level and specially strength level in children with mild-moderate asthma. (1) Wanrooij VH, Willeboordse M, Dompeling E, van de Kant KD. (2014). Br J Sports Med. 48(13):1024-31. (2) Silva RA, Vieira RP, Duarte AC, Lopes FD, Perini A, Mauad T, Martins MA et al. (2010). EurRespir J. 35(5):994-1002. (3) Wärnberg J, Cunningham K, Romeo J, Marcos A.(2010). Proc NutrSoc. 69(3):400-6. (4) Counil FP, Varray A, Matecki S, Beurey A, Marchal P, Voisin M et al. (2003). J Pediatr. 142(2):179-84. (5) Eijkemans M, Mommers M, Draaisma JM, Thijs C, Prins MH. (2012). PLoSOne. 7(12): e50775. Sin financiación No data 2018 UEM

Sin financiación No data 2018 UEM

Sin financiación No data 2018 UEM

Sin financiación No data 2018 UEM

INTRODUCTION: Physical inactivity and a low adherence to the Mediterranean diet produces alterations in body composition that may determinate a worse control of chronic diseases such as asthma (1), this is one of the most frequent diseases in childhood. Some investigation in animal show the exercise can be decreased an asthma inflammation and ronchial hyperresponsiveness (2), as well as systemic inflammation, which could help control asthma (3) Objectives: to analyze the effect of aerobic and strength exercise program in asthmatic children with symptoms of bronchospasm induced by exercise, with physical exercise on variables of physical condition and quality of life. METHODS: Randomized controlled trial with control group that compared their usual physical activity vs. exercise program for 12 weeks, 3 days/week, and 60 min/day. It is assessed the physical condition (ergospirometry on treadmill and strength of peripheral muscles) and quality of life with Pediatric Quality of Life Questionnaire (PAQLQ). RESULTS: The study included 43 children from 7 to 17 years old distributed in Control Group (CG, n = 27) and Intervention Group (IG, n = 16). The average adherence to the program was 82.8%. In VO2peak in ml · kg - 1 · min - 1 the IG had 3.31 ± 1.51 with respect to the CG that obtained 0.25 ± 0.71 in the interaction time per group was p(txg) = 0.064 with an increase in the IG of with respect to the CG, Statistically significant improvements were observed in all strength parameters evaluated, with more significance in High Row p(txg) = 0.006, Leg Press p(txg) = 0.002 and Hamstrings p(txg) = 0.003. We couldn’t see improves in quality of life. CONCLUSION: Similar studies in children with asthma observe improvements in VO2max in 1 METS approximately what, according to investigations is a considerable improvement for this population (4), also some investigation shows than the strength in the same population improves significantly and recent systematic review are demonstrating an adequate level of physical activity with a protective factor against asthma (5). In relation with PAQLQ the values weren’t changed robably because the sample has high results in PRE-PAQLQ, 53,3% of them has controlled asthma with at least two drugs. In conclusion 12 weeks of exercise program improves both, cardiorespiratory level and specially strength level in children with mild-moderate asthma. Sin financiación No data 2018 UEM

SIN FINANCIACIÓN No data (2015) UEM

Tesis inédita presentada en la Universidad Europea de Madrid. Facultad de Ciencias de la Actividad Física y Deporte La fibrosis quística es una de las enfermedades genéticas más frecuentes en la raza caucásica, y afecta principalmente a los pulmones, y en menor medida al páncreas, hígado e intestino. Está producida por una mutación en el gen que codifica la proteína reguladora de la conductancia tras-membrana de la fibrosis quística (CFTR). Esta proteína interviene en el paso del cloro a través de las membranas celulares y su deficiencia altera la producción de sudor, jugos gástricos y moco. La esperanza de vida de los pacientes con fibrosis quística (FQ) raramente supera los 40 años y hasta la fecha, a pesar de los numerosos avances en el tratamiento y manejo de la enfermedad, no existe ninguna terapia eficaz para curarla. En niños afectados de FQ, el consumo pico de oxígeno (VO2pico) es el factor de predicción de esperanza de vida más importante y suele disminuir inexorablemente cada año (cuando los valores de esta variable son inferiores a 32 mLO2.kg-1.min-1, la tasa de mortalidad es muy elevada). El único modo disponible para incrementar significativamente el VO2pico de cualquier individuo, enfermo o no, es aumentar sus niveles de actividad física (o `ejercicio¿). [Resumen Teseo] UEM

Introducción: El ejercicio físico podría ser una herramienta eficaz para mantener alta la capacidad funcional en los pacientes con tumores sólidos (TS), pero antes de comenzar a analizar su efecto, debemos establecer si dicha capacidad funcional está disminuida en la población que padece esta enfermedad. El objetivo de este trabajo fue comparar la capacidad funcional y la agilidad de las tareas de la vida diaria en niños que padecen TS respecto a niños sanos controles (NS). Material/métodos: Se seleccionaron 52 niños, 24 pacientes de TS del HIUNJ (edad(años) 10±4,2; altura(cm) 146±21,9; peso(kg) 41,3±20,2; IMC(kg/m2) 18,1±4,9) y 28 niños sanos pareados en edad(años) 8 ±3,4; altura(cm) 137±21,4; peso(kg) 33,6±13,7; IMC(kg/m2) 17,1±2,1. Las variables analizadas fueron: capacidad funcional (VO2pico, VO2VT1) medida a través de prueba de esfuerzo con análisis de gases y agilidad (tiempo utilizado en los test funcionales “Test Up and Go 3m” (TUG-3) y “Test Up and Down Stairs12” (TUDS-12). Para el análisis de datos, se utilizó la prueba estadística T-student (SPSS.20). Resultados: La capacidad funcional, medida a través de VO2pico, en niños con TS fue significativamente más baja que en NS (25±8,8 ml/kg/min vs 40,9±8,9 ml/kg/min respectivamente), el VO2VT1 fue también menor para el grupo de niños de TS (17,1±6 ml/kg/min vs 25,7±4,9 ml/kg/min). No se encontraron diferencias significativas en la FCpico. Los test funcionales no mostraron diferencias entre ambos grupos. Discusión/Conclusión: Los niños que padecen TS tienen un descenso de un 38,8% en su capacidad funcional. Según Myers 2002 (N Engl J Med 2002; 4:793-801), un descenso de 3,5 ml/kg/min de la capacidad funcional se asocia con una reducción de un 12% en la tasa de longevidad en poblaciones enfermas. La capacidad funcional más baja de los niños con TS afecta al proceso evolutivo de su enfermedad, sugiriendo el ejercicio como herramienta capaz de incrementar dicha capacidad funcional. 0.125 SJR (2014) Q4, 198/223 Orthopedics and sports medicine, 143/169 Physical therapy, sports therapy and rehabilitation, 126/128 Sports sciences UEM

Children with cystic fibrosis (CF) often have to be hospitalized because of acute exacerbation of their respiratory symptoms. Given the fact that improved peak oxygen uptake (VO2peak) is positively associated with lung function and overall health in children with CF, this study examined the association between VO2peak and the need for hospitalization in a cohort of pediatric CF patients. In a 3-year study, 77 CF children with mild-to-moderately severe CF (forced expiratory volume in 1 sec [FEV1] ≥ 50%) underwent a maximal exercise test to determine VO2peak. Anthropometric, lung function and muscle strength measurements were also conducted and dates of hospitalization were recorded for the study period. Associations were then determined between the variables recorded and hospitalization by univariate and multivariate Cox proportional hazards regression analyses. VO2peak was 38.6 ± 6.7 ml kg−1 min−1 for boys and 31.9 ± 6.9 ml kg−1 min−1 for girls. In multivariate analyses, VO2peak was the only variable significantly associated with time to hospitalization (hazard ratio 0.91, P = 0.03). A significant association was detected between greater aerobic fitness, and lower risk of hospitalization. Because hospitalization due to respiratory exacerbation is a powerful prognostic factor, our findings provide further support for the importance of aerobic fitness evaluation in the management of children with mild-to-moderately severe CF. 2.704 JCR (2014) Q1, 20/119 Pediatrics; Q2, 25/57 Respiratory system UEM

Purpose: To investigate (1) the contributions of sex, age, nutritional status- and physical-fitness-related variables on health-related quality of life (HRQOL) in Spanish children with cystic fibrosis, and (2) the agreement on HRQOL between children and their parents. Methods: In 28 children aged 6-17 years, body mass index percentile, percentage body fat, physical activity, pulmonary function, cardiorespiratory fitness, functional mobility, and dynamic muscle strength were determined using objective measures. HRQOL was measured using the revised version of the cystic fibrosis questionnaire. Simple and multiple linear regression analyses were performed to determine the variables associated with HRQOL. To assess the agreement on HRQOL between children and parents, intra-class correlation coefficients (ICCs) were calculated. Results: Girls reported worse emotional functioning, a higher treatment burden, and more respiratory problems than boys. Greater functional mobility appeared associated with a less favourable body image and more eating disturbances. Agreement on HRQOL between children and parents was good to excellent, except for the domain of treatment burden. Conclusions: Sex and age were stronger predictors of HRQOL than nutritional status- or physical-fitness-related variables. Children reported a lower treatment burden than their parents perceived them to have. 2.412 JCR (2012) Q1, 18/82 Health care sciences & services; Q2, 42/158 Public, environmental & occupational health UEM

La Anorexia Nerviosa (AN) es un trastorno de la alimentación de predominio en mujeres jóvenes que se caracteriza por una pérdida de peso ponderal inducida y mantenida por el propio paciente, que le conduce a la malnutrición progresiva y rasgos psicopatológicos peculiares como la distorsión de la imagen corporal y el miedo a la obesidad (Moreno Villares, 2003). La AN tiene importantes repercusiones sobre múltiples órganos y sistemas. Uno de los sistemas más deteriorados es el tejido muscular, lo que a su vez repercute sobre el modelado y remodelado óseo. Todo ello se encuentra estrechamente relacionados con la cantidad y tipo de actividad física que se realiza durante la fase de crecimiento tal y como demuestran numerosos estudios transversales y longitudinales (Calbet y col., 2001; Valdimarsson y col., 2006; Vicente-Rodríguez y col., 2004, 2006). Se han observado niveles de actividad física elevados entre pacientes con AN que se pueden manifestar a través de diferentes formas como actividades colectivas, correr, caminar, conductas nerviosas de repetición, entre otras, sugiriendo un rol etiológico en el desarrollo de AN en algunas pacientes (Davis y col. 1997). El ejercicio es tratado como una forma de perder peso en AN y se asocia a una peor respuesta al tratamiento (Carter y col., 2004; Strober y col., 1997). El objetivo de este la estudio es describir los niveles de actividad física en pacientes de AN utilizando la metodología de acelerómetros. Anorexia Nervosa (AN) is an eating disorder which is predominant in young females and characterized by an induced weight loss and supported by the own patient. It induces progressive malnutrition and peculiar psychopathologic features like body-image distortion and fear of obesity (Moreno Villares, 2003). AN has important impacts in multiple organs and systems. One of the most deteriorated systems is the skeletal muscle tissue. Bone mass is also usually affected. The aforementioned problems are strongly related to the total amount and type of physical activity during the growth phase as shown in previous several cross and longitudinal studies (Calbet et al., 2001, Valdimarsson et al., 2006, Vicente-Rodriguez et al., 2004, 2006). Elevated physical activity levels are commonly observed among patients with AN manifested in several forms like collective activities, running, walking pacing and fidgeting behaviours among others, all of which could play an etiologic role in development of AN in some patients (Davis et al., 1997). Physical activity is assumed to facilitate weight loss in AN and is associated with poorer outcome to the treatment (Carter et al., 2004; Strober et al. 1997). The purpose to this study was to describe the physical activity levels of AN patients using accelerometry technology. Sin financiación No data 2008 UEM

This guide attempts to explain the role of exercise in the treatment of Cystic Fibrosis. When we think of exercise, we should think about an activity that involves some kind of stimulus, since it is the basis for achieving improvements in the different organic systems and improvements that will allow us to develop the activities of everyday life with better quality. In addition, this stimulus must be changed over time to continue achieving improvements and adaptations that will allow us to feel better and better. Federación Española de Fibrosis Quística No data (2017) UEM

Proyecto PS09/00194. Prescripción de un programa de ejercicio físico intrahospitalario para niños con fibrosis quística; Proyecto de investigación intrahospitalario para niños con fibrosis quística (Hospital Universitario Niño Jesús) No data (2015) UEM