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In this paper, we will review the dietary allowances of these fatty acids in the paediatric population, and also the indications in different pathologies within the field of pediatric gastroenterology. Finally, we will try to explain the reasons that may justify the difficulty in translating good results in experimental studies to the usual clinical practice. This “good results” may be too little to be detected or there may be other causes but misinterpreted as effects of DHA.

Resumen Introduccion La realizacion estricta de una dieta sin gluten (DSG) es fundamental para el control de la enfermedad celiaca. El objetivo del estudio fue analizar la adherencia a la DSG en celiacos y evaluar factores que pudieran influir en la misma. Material y metodos Estudio observacional descriptivo. Se realizo una determinacion de peptidos inmunogenicos del gluten (GIP) en heces con metodo semicuantitativo y se cumplimento el cuestionario Celiac Dietary Adherence Test. Se recogieron datos sociodemograficos, clinicos y se elaboro una encuesta ad hoc. Resultados Se incluyeron 80 pacientes. El 92,5% eran adherentes mediante GIP y 86,3% con Celiac Dietary Adherence Test (concordancia aceptable; Kappa: 0,31, p=0,004). El 83,3% de los pacientes con GIP positivos tenia la ultima determinacion de anticuerpos antitransglutaminasa negativos. La edad actual y el tiempo de evolucion se asociaron significativamente con la adherencia. Aquellos con GIP positivos tenian de media 5 anos mas (p=0,0001) y llevaban 52 meses mas de DSG (p=0,025). Una cuarta parte de los encuestados consideraba dificil realizar la dieta. El 60% consideraba que la variabilidad en el lugar de comida era importante para inducir transgresiones, siendo las fiestas infantiles el principal lugar donde sucedian (66,7%). Se destaca la escasa variedad (61,4%) y el elevado coste (98,6%) de los alimentos sin gluten. Conclusiones La adherencia a la DSG es en general, buena. El analisis de GIP permitio detectar a pacientes no adherentes que en otras circunstancias pasarian desapercibidos. Se deben establecer medidas para mantener una buena adhesion de manera prolongada, considerando los factores de riesgo y dificultades detectados.

Introduction: treatment of celiac disease is gluten-free diet for life. This can impact the quality of life (QoL) of patients. Objectives:the aim of this study was to evaluate the QoL and the factors with an impact on QoL in a sample of children with celiac disease. Methods and materials: a descriptive observational study. QoL was evaluated using the Celiac Disease Dux Questionnaire (CDDUX). Adherence to gluten-free diet was assessed with the Celiac Dietary Adherence Test (CDAT) and the presence of gluten immunogenic peptides (GIP) in the stools. Sociodemographic and clinical data were collected, and an ad-hoc survey was developed. Results: eighty patients were included. Median CDDUX score was 44.04 points (QoL: "neutral"). Subscale scores included: "communication", 58.3 points ("neutral"); "having CD", 25 points ("Bad"); and "diet", 41.6 points ("neutral"). QoL was worse among patients with celiac relatives (the result of the survey was "bad" vs. "neutral" with p = 0.02) and among those who found unsatisfactory the somatosensory characteristics and the price of gluten-free food (the result of the survey was "bad" vs. "neutral" with p = 0.02). Those who found unsatisfactory the texture of these food reported a worse QoL ("bad" vs. "neutral", p = 0.009). Those who reported eating outside the home as a transgression inducer reported a "bad" QoL; those who did not, reported a "neutral" QoL (p = 0.03). Conclusions: celiac patients report a "neutral" QoL. A poorer QoL was related to having celiac relatives, finding gluten-free food unsatisfactory, and considering eating outside the home as an inducer factor for transgressions.Introducción: el tratamiento de la enfermedad celiaca es una dieta sin gluten de por vida, lo cual puede repercutir en la calidad de vida (CV) de los pacientes. Objetivos: nuestro objetivo fue evaluar la CV de una muestra de niños celiacos y estudiar los factores que pueden influir en la misma. Material y métodos: estudio observacional descriptivo. Se estudió la CV con el cuestionario Celiac Disease Dux Questionnaire (CDDUX). Se estudió la adherencia con el cuestionario Celiac Dietary Adherence Test (CDAT) y la determinación de péptidos inmunogénicos del gluten (GIP) en heces. Se recogieron datos sociodemográficos y clínicos, y se elaboró una encuesta ad hoc. Resultados: se incluyeron 80 pacientes. La mediana del CDDUX fue de 44,04 puntos (CV “neutra”); la de la subescala “comunicación” fue de 58,3 (“neutra”), la de “tener EC” fue de 25 (“mala”) y la de “dieta” fue de 41,6 puntos (“neutra”). La CV fue peor en los pacientes con familiares celiacos (“mala” frente a “neutra”, p = 0,02) y en aquellos insatisfechos con las características somatosensoriales y el precio de los alimentos sin gluten (“mala” frente a “neutra”, p = 0,02). Los insatisfechos con la textura de estos alimentos tenían peor CV (“mala” frente a “neutra”, p = 0,009). Los que consideraban comer fuera de casa como factor inductor de transgresiones referían una CV “mala” y los que no, una “neutra” (p = 0,03). Conclusiones: los pacientes celiacos tienen una CV neutra. El hecho de tener familiares con enfermedad celiaca, la insatisfacción con los alimentos sin gluten y el considerar un factor inductor de transgresiones el comer fuera de casa se relacionaron con una peor calidad de vida.

Objectives: Make a preliminary assessment of the efficacy of manual physical therapy (MPT) compared to conventional pharmacologic treatment (CPT) in chronic pediatric functional constipation (CPC)....

Resumen: Introducción: La alergia alimentaria es un problema creciente, siendo la proteína de leche de vaca la principal causa en niños. Sin un proceso diagnóstico adecuado, existe un elevado riesgo de sobrediagnóstico e infradiagnóstico y, por lo tanto, de sobretratamiento e infratratamiento. El objetivo de nuestro estudio fue analizar la variabilidad en el manejo de la alergia a proteína de leche de vaca (APLV) por los gastroenterólogos pediátricos españoles. Métodos: Se envió un cuestionario de 50 preguntas a través de la lista de email de la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátricas. Resultados: Recibimos 73 cuestionarios de los 321 enviados. Solo 3 de las respuestas lograron más del 90% de acuerdo. El 33% considera que la provocación oral es necesaria para el diagnóstico de APLV siempre. El 25% considera que la mejoría clínica tras la retirada de las proteínas de leche de vaca es suficiente para el diagnóstico. La provocación oral es realizada en domicilio por el 83,5% de los encuestados en APLV no IgE mediada. Los hidrolizados extensos de caseína son el tratamiento de elección (69,9%). Las fórmulas de soja, la última opción. Casi todos los encuestados conocían la existencia de guías de manejo de APLV, siendo las de la Sociedad Europea de Gastroenterología, Hepatología y Nutrición Pediátrica las más utilizadas (64,4%). El 23% considera que su conocimiento sobre alergia es inadecuado. Conclusiones: Aunque la APLV es una patología prevalente que los gastroenterólogos pediátricos llevan décadas tratando, hemos encontrado una gran variabilidad en su manejo. Existe posibilidad de mejora en este campo en el futuro. Abstract: Introduction: Food allergy is an increasing health problem in the developed world. Cow's milk protein is the main cause of food allergy in infants. Without an appropriate diagnostic workup, there is a high risk of both over- and underdiagnosis and therefore, over and undertreatment. The objective of our study was to analyze the variability in cow's milk protein allergy (CMPA) management by pediatric gastroenterologists in Spain. Methods: A fifty item questionnaire, including open and closed items in a Likert's scale from 0 to 5, was drafted and distributed through the Spanish Society for Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) e-mail list. Results: Seventy-three questionnaires were received back out of 321. Only 3 of the items achieved concordance greater than 90%. Thirty-three percent considered oral challenge to be necessary for the diagnosis of CMPA under any circumstance. Twenty-five percent considered that symptom improvement after cow's milk removal was enough for the diagnosis. Oral challenge was performed at home by 83.5% in non-IgE mediated cases. Extensively hydrolyzed casein formulas were the treatment of choice for 69.9%. Soy formulas were the last option. Almost all respondents were aware of the existence of clinical guidelines on CMPA, being European Society of Pediatric Gastroenterology, Hepatology and Nutrition guidelines the most followed (64.4%). Twenty-three percent considered that their knowledge about allergy was inadequate. Conclusions: Although CMPA is a prevalent condition that pediatric gastroenterologists have been treating for decades, we found a huge variability on its management. There is potential for improvement in this field among pediatric gastroenterologist in the future.

Introduction: Food allergy is an increasing health problem in the developed world. Cow's milk protein is the main cause of food allergy in infants. Without an appropriate diagnostic workup, there is a high risk of both over- and underdiagnosis and therefore, over and undertreatment. The objective of our study was to analyze the variability in cow's milk protein allergy (CMPA) management by pediatric gastroenterologists in Spain. Methods: A 50 item questionnaire, including open and closed items in a Likert's scale from 0 to 5, was drafted and distributed through the Spanish Society for Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) e-mail list. Results: Seventy-three questionnaires were received back out of 321. Only 3 of the items achieved concordance greater than 90%. Thirty-three percent considered oral challenge to be necessary for the diagnosis of CMPA under any circumstance. Twenty-five percent considered that symptom improvement after cow's milk removal was enough for the diagnosis. Oral challenge was performed at home by 83.5% in non-IgE mediated cases. Extensively hydrolyzed casein formulas were the treatment of choice for 69.9%. Soy formulas were the last option. Almost all respondents were aware of the existence of clinical guidelines on CMPA, being European Society of Pediatric Gastroenterology, Hepatology and Nutrition guidelines the most followed (64.4%). Twenty-three percent considered that their knowledge about allergy was inadequate. Conclusions: Although CMPA is a prevalent condition that pediatric gastroenterologists have been treating for decades, we found a huge variability on its management. There is potential for improvement in this field among pediatric gastroenterologist in the future. Resumen: Introducción: La alergia alimentaria es un problema creciente, siendo la proteína de leche de vaca la principal causa en niños. Sin un proceso diagnóstico adecuado, existe un elevado riesgo de sobrediagnóstico e infradiagnóstico y, por lo tanto, de sobretratamiento e infratratamiento. El objetivo de nuestro estudio fue analizar la variabilidad en el manejo de la alergia a proteína de leche de vaca (APLV) por los gastroenterólogos pediátricos españoles. Métodos: Se envió un cuestionario de 50 preguntas a través de la lista de email de la Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátricas. Resultados: Recibimos 73 cuestionarios de los 321 enviados. Solo 3 de las respuestas lograron más del 90% de acuerdo. El 33% considera que la provocación oral es necesaria para el diagnóstico de APLV siempre. El 25% considera que la mejoría clínica tras la retirada de las proteínas de leche de vaca es suficiente para el diagnóstico. La provocación oral es realizada en domicilio por el 83,5% de los encuestados en APLV no IgE mediada. Los hidrolizados extensos de caseína son el tratamiento de elección (69,9%). Las fórmulas de soja, la última opción. Casi todos los encuestados conocían la existencia de guías de manejo de APLV, siendo las de la Sociedad Europea de Gastroenterología, Hepatología y Nutrición Pediátrica las más utilizadas (64,4%). El 23% considera que su conocimiento sobre alergia es inadecuado. Conclusiones: Aunque la APLV es una patología prevalente que los gastroenterólogos pediátricos llevan décadas tratando, hemos encontrado una gran variabilidad en su manejo. Existe posibilidad de mejora en este campo en el futuro.

Introduction: Malnutrition on admission is closely related to a longer hospital stay and a higher morbidity. The prevalence of hospital malnutrition has been reported as almost as high as 50%, with 6% being the lowest. DHOSPE study investigates nutrition status in Spanish hospitals and its outcome during the hospital stay. Patients and methods: A longitudinal, multicentre, descriptive, cross-sectional study, with a short follow-up period was conducted in 32 hospitals during 2011. A total of 991 patients were included, with ages from 0 to 17 years. Each patient was measured at admission (weight, length, weight for length -W/L-, length for age -L/A-), and at 7 and 14 days. The STAMP nutritional screening tool was completed on admission. Anthropometric measurements were reported as z-score, and nutrition status classified according to W/L and L/A for acute and chronic malnutrition, respectively. Results: The prevalence of malnutrition was 7.1% for moderate, and 0.7% for severe acute malnutrition. For chronic malnutrition, 2.7% was moderate, and 1.4% severe. There were significant differences according to the underlying condition but not according to age. Results of STAMP show that around 75% of patients had a moderate to high risk of malnutrition. Nutritional status changed during admission for weight, as well as W/L and L/A. A worse nutritional status at admission and a higher STAMP score were positively correlated with the need for nutrition support. Conclusions: The prevalence of undernutrition was slightly lower (

Resumen: Introducción: La desnutrición al ingreso se relaciona con hospitalizaciones más prolongadas y mayor morbilidad. La prevalencia varía entre un 6 y un 50%. El estudio DHOSPE se pregunta sobre la situación nutricional al ingreso y cómo se modifica durante el mismo. Pacientes y métodos: Estudio observacional, descriptivo, transversal, con un breve seguimiento longitudinal, multicéntrico, realizado en 32 hospitales en 2011. Se incluyó a 991 pacientes. En todos se realizó una valoración nutricional al ingreso, a los 7 y 14 días. Se utilizó la herramienta de cribado nutricional STAMP en el momento del ingreso. Las medidas antropométricas se evaluaron como puntuaciones Z y el estado nutricional con los índices de Waterlow de peso para la desnutrición aguda y de talla para la crónica. Resultados: La prevalencia de desnutrición fue del 7,1% para la aguda moderada y del 0,7% para la grave. Para la crónica: 2,7% moderada y 1,4% grave. No se encontraron diferencias significativas en función de la edad pero sí en función de la enfermedad. Algo más del 75% de los pacientes presentaban riesgo intermedio o elevado de desnutrirse durante el ingreso, valorado con STAMP. La peor situación nutricional al ingreso y una puntuación más elevada en el riesgo nutricional se correlacionaron positivamente con la necesidad de soporte nutricional. Conclusiones: La prevalencia de desnutrición fue sensiblemente inferior (< 8%) a lo publicado, probablemente en relación con la distinta complejidad de los hospitales. El riesgo nutricional valorado con la herramienta STAMP fue elevado (el 75% presentaba riesgo moderado o intenso de desnutrición). Abstract: Introduction: Malnutrition on admission is closely related to a longer hospital stay and a higher morbidity. The prevalence of hospital malnutrition has been reported as almost as high as 50%, with 6% being the lowest. DHOSPE study investigates nutrition status in Spanish hospitals and its outcome during the hospital stay. Patients and methods: A longitudinal, multicentre, descriptive, cross-sectional study, with a short follow-up period was conducted in 32 hospitals during 2011. A total of 991 patients were included, with ages from 0 to 17 years. Each patient was measured at admission (weight, length, weight for length -W/L-, length for age -L/A-), and at 7 and 14 days. The STAMP nutritional screening tool was completed on admission. Anthropometric measurements were reported as z-score, and nutrition status classified according to W/L and L/A for acute and chronic malnutrition, respectively. Results: The prevalence of malnutrition was 7.1% for moderate, and 0.7% for severe acute malnutrition. For chronic malnutrition, it was 2.7% moderate, and 1.4% severe. There were significant differences according to the underlying condition but not according to age. Results of STAMP show that around 75% of patients had a moderate to high risk of malnutrition. Nutritional status changed during admission for weight, as well as W/L and L/A. A worst nutritional status at admission and a higher STAMP score were positively correlated with the need for nutrition support. Conclusions: The prevalence of undernutrition was slightly lower (

espanolIntroduccion: una dieta baja en oligosacaridos, disacaridos, monosacaridos y polioles fermentables (FODMAP) puede ser eficaz en el tratamiento de los pacientes pediatricos con sindrome de intestino irritable (SII). Su complejidad y efectos secundarios hacen de la misma una alternativa terapeutica secundaria. Objetivo: demostrar que la educacion nutricional, dirigida a optimizar la dieta de los ninos con SII, es capaz de mejorar sus sintomas gastrointestinales sin tener que realizar una dieta baja en FODMAP. Metodos: estudio de intervencion prospectivo. Se analizaron los cambios de los sintomas gastrointestinales, mediante el cuestionario pediatrico de calidad de vida para sintomas gastrointestinales (PedsQL™ sintomas GI), tras recibir educacion nutricional basada en alimentacion saludable. Asimismo, se analizaron los cambios antropometricos y de habitos dieteticos tras dicha intervencion. Resultados: se incluyeron 21 pacientes (12 ninas) con edad media de 10,6 anos (5-14 anos). Se observo una dieta con un exceso de ingesta de azucares simples, grasas saturadas y sal junto con un deficit de fibra. Tras la intervencion, se aprecio un aumento de 8,07 puntos en el cuestionario (IC del 95%: 13,42 a -2,73; p = 0,005). Ademas, se observo una perdida de peso significativa en los pacientes con sobrepeso y obesidad (disminucion del Z-score de indice de masa corporal [IMC] 0,62 DE; p = 0,001). Se observaron cambios significativos en los habitos dieteticos: aumento de consumo de hidratos de carbono complejos, frutas y verduras y disminucion de azucares simples. Conclusiones: la alimentacion saludable resulta eficaz para mejorar los sintomas gastrointestinales en pacientes pediatricos con SII, sin requerir la exclusion de FODMAP. EnglishBackground: a diet low in fermentable oligosaccharides, disaccharides, monosaccharides and polyols (FODMAP) may be effective in the treatment of pediatric patients with irritable bowel syndrome (IBS). Its complexity and side effects make it a secondary therapeutic alternative. Objective: to demonstrate that nutritional education, aimed at optimizing the diet of children with IBS, is able to improve gastrointestinal symptoms of children without following a diet low in FODMAP. Methods: prospective intervention study. Changes in gastrointestinal symptoms were analyzed by means of the Pediatric Quality of Life Inventory ™ Gastrointestinal Symptoms (PedsQL™ GI Symptoms), after receiving nutritional education based on a healthy diet. Likewise, anthropometric changes and dietary habits were analyzed. Results: twenty-one patients were included (12 girls) with a mean age of 10.6 years (5-14 years). A diet with excess intake of simple sugars, saturated fats and salt along with fiber deficit was observed. After the intervention, an increase in 8.07 points was observed in the inventory (95% CI: 13.42 a -2.73, p = 0.005). Additionally, significant weight loss was observed in overweight and obese patients (decrease in body mass index [BMI]; Z-score 0.62 SD, p = 0.001). Significant changes in dietary habits were observed: increased consumption of complex carbohydrates, fruits and vegetables and reduction of simple sugars. Conclusions: healthy eating is effective to improve gastrointestinal symptoms in pediatric patients with IBS, without requiring the exclusion of FODMAP.

Objective: The aim of the present study was to explore the relation between lung function and serum retinol (SR) in cystic fibrosis (CF) patients. Methods: This was a cross-sectional study conducted in a group of 98 young patients with CF (6.8–22.3 years), after the exclusion of those with pulmonary exacerbation, vitamin A deficiency, or other risks, from an initial group of 124 cases. Results: Data of forced expiratory volume in 1 second (FEV1) were widely scattered (87.7% ± 16.9%). These were similar in the 78 pancreatic insufficient and 11 pancreatic sufficient patients. SR (56.6 ± 18.4 μg/dL) was >2.5th percentile of healthy people in the whole group, although 31 patients were situated above the 97.5th percentile (higher value: 110 μg/dL). The FEV1 was noticeably higher in these than in those within the normal range (93.6 ± 14.0 vs 85.0 ± 17.6 μg/dL; P 80% is 3.78 in patients with SR above the 97.5th percentile, versus only 0.26 in those within the normal range. There were no cases with retinol toxicity. Conclusions: FEV1 of young patients with CF correlates positively with SR, regardless of age, pancreatic function, or nutritional condition. Those with a moderately high retinol (up to 110 μg/dL) maintain the best respiratory function (FEV1 ≥80% in >90% of them) without any signs of toxicity.

evaluate vitamin D status and its association with chronic lung colonisation in Cystic Fibrosis patients.descriptive cross-sectional multicenter study. From November 2012 to April 2014, at 12 national hospitals, 377 patients with Cystic Fibrosis were included. Vitamin D levels30 ng/ml were classified as insufficient. Chronic colonisation was considered if they had at least two positive cultures in the past year.the median age was 8.9 years (2 months to 20 years). 65% had insufficient levels of vitamin D. There was an inverse correlation between age and vitamin D levels (r = -0.20 p0.001). Those diagnosed by screening, were younger and had higher levels of vitamin D. There was an inverse correlation between the number of colonisations and vitamin D levels (r = -0.16 p = 0.0015). Adjusting for age, pancreatic status and diagnosis by screening, colonization by S. aureus in 6 years, increased the risk of insufficient levels of vitamin D: OR 3.17 (95% CI 1.32 to 7.61) (p = 0.010) and OR 3.77 (95% CI 1.37 to 10 , 37) (p = 0.010), respectively.despite adequate supplementation, more than half of our patients did not achieve optimal levels of vitamin D. Regardless of age, diagnosis by screening or pancreatic status, chronic colonization by Pseudomonas sp. in children and adolescents and S. Aureus in infants and preschoolars increases the risk of developing vitamin D deficiency in these patients.Introducción y objetivos: conocer la situación en la que se encuentran los pacientes con fibrosis quística en relación con sus niveles de vitamina D y su asociación con las colonizaciones pulmonares crónicas. Material y métodos: estudio multicéntrico transversal. Participaron 12 hospitales nacionales. De noviembre a abril del 2012 al 2014 se incluyeron 377 pacientes con fibrosis quística. Se consideraron insuficientes niveles de vitamina D30 ng/ml. Presentar al menos dos cultivos positivos en el último año fue considerado un criterio de colonización crónica. Resultados: los pacientes tenían una mediana de edad de 8,9 años (2 meses—20 años). Un 65% presentaban niveles insuficientes de vitamina D. Se observó una correlación inversa entre edad y niveles de vitamina D (r = -0,20 p0,001). Los diagnosticados por cribado eran más jóvenes y tenían niveles de vitamina D más altos. Los niveles de vitamina D presentaron una correlación inversa con el número de colonizaciones pulmonares (r = -0,16 p = 0,0015). Ajustando por edad, función pancreática y diagnóstico mediante cribado, la colonización por S. Aureus en menores de seis años y por Pseudomonas sp. en los mayores de esa edad, incrementaban el riesgo de presentar niveles insuficientes de vitamina D: OR 3,17 (IC95% 1,32-7,61) (p=0,010) y OR 3,77 (IC95% 1,37- 10,37)(p = 0,010), respectivamente. Conclusiones: a pesar de una suplementación adecuada, más de la mitad de nuestros pacientes no alcanzan niveles óptimos de vitamina D. La colonización crónica por Pseudomonas sp. en escolares y adolescentes y por S. Aureus en lactantes y preescolares se asocia de forma independiente con la deficiencia de vitamina D.

Evaluate the safety and efficacy of a novel polyvitaminic (Aquadek's®) in patients with Cystic Fibrosis (CF).Prospective, longitudinal and non-randomized study. CF patients with pancreatic insufficiency and clinically stable were given Aquadek's® (two chewable tablets) daily for 12 months. Serum levels of retinol, beta-carotene, 25 OH vitamin D and α-tocopherol were evaluated twelve months before, at baseline and 12 months after.paired t tests.28 patients aged 6 to 39 years (median 18.5 years) were included. Aquadek's® supplementation led to an increase in vitamin A dose and a decrease in the number of tablets administered. At baseline, 89% had at least one vitamin deficiency (61% pro-Vitamin A and 54% vitamin D). After one year, serum beta-carotene levels were increases 160 (95% CI 98-222) mcg/l (p0.001) and decreased the percentage of patients with pro-vitamin A deficiency 46% (95% CI 22-64) (p0.001). The proportion of patients with vitamin D insuficiency increased 18%(95% CI 2-32) (p =0.025). In any case serum levels exceeded the upper limits used to assess the risk of toxicity.Conclusions: Two daily Aquadek's® chewable tablets are safe and effective for maintaining vitamin A and E status of CF patients older than 6 years, although it is insufficient to normalize serum 25OHvitaminD according to the current recommendations for this disease.Introducción y objetivo: Conocer si la suplementación un nuevo polivitamínico (Aquadek´s®) durante 12 meses es segura y eficaz en pacientes con Fibrosis Quística (FQ). Material y Métodos: Estudio prospectivo, longitudinal y no controlado en pacientes con FQ insuficientes pancreáticos, clínicamente estables, que recibieron suplementación con Aquadek´s® (2 comprimidos masticables) durante 12 meses en lugar de su suplementación habitual. Se evaluaron niveles séricos de retinol, betacarotenos, 25 OH vitamina D y α-tocoferol un año antes, al inicio y tras un año de tratamiento. Análisis estadístico: Pruebas t para datos pareados. Resultados: Se incluyeron 28 pacientes entre 6 y 39 años (mediana 18,5 años). La suplementación con Aquadek´s® supuso un incremento en la dosis de vitaminas A y una disminución del número de comprimidos administrados. Al inicio, un 89% presentaban algún tipo de déficit vitamínico: (61% pro-Vitamina A y 54% vitamina D). Tras un año, se produjo un incremento de los niveles de betacarotenos: 160 mcg/l (IC 95% 98-222) (p.

Cystic fibrosis-related diabetes is the most common comorbidity in subjects with cystic fibrosis. The hallmark of cystic fibrosis-related diabetes is the reduction of β-cell mass, leading to insulin insufficiency. However, insulin resistance (IR) may arise from decreased glucose uptake by muscle (peripheral IR) and through impaired suppression of hepatic glucose production (hepatic IR). Increased prevalence of overweight and obesity, sedentary lifestyle, high-calorie diets, fat-soluble vitamin deficiency, elevated oxidative stress, and increased life expectancy are reasons to believe that IR can achieve an important role in the development of glucidic disorders and macrovascular complications previously recognized in patients with cystic fibrosis. In this chapter we explain the principal mechanisms of IR in cystic fibrosis and propose preventive and nutritional management strategies for this problem.

Su incidencia es muy variable dependiendo de la definicion aceptada y de la poblacion estudiada. En algunas zonas rurales afecta al 10% de los ninos. En Estados Unidos, entre 19801989, el 5% de los ingresos hospitalarios y el 10% de los ninos vistos en centros de medicina primaria presentaban FM1,8. En los paises en desarrollo el FM y la malnutricion son mucho mas comunes. En poblacion indigente, se eleva al 15-25% de ingresos de ninos menores de 2 anos3-4. En cuanto al sexo parece ser mas comun en hembras2,8.

Introduction: The primary objective of this study was to find out the prevalence of overweight and obese status, as well as their association to pulmonary function, total cholesterol and vitamin D in patients with cystic fibrosis (CF). Materials and methods: This is a multicenter descriptive and cross-sectional study. Twelve Spanish hospitals participated. 451 patients with CF were included. Adults were classified according to body mass index (BMI) and children were classified according to BMI percentiles (WHO tables). Pearson’s correlation, Anova, Student’s t-test and multiple linear regression were conducted. Results: Mean age was 12.3 (range 4-57) years old, 51% were male and 18% had pancreatic sufficiency. Participants were classified in five nutritional status categories: 12% were malnourished; 57%, at nutritional risk; 24%, normally nourished; 6%, overweight; and 1%, obese. Pulmonary function in overweight or obese patients (91 ± 19%) was better than in malnourished patients (77 ± 24%) (p = 0.017). However, no difference was observed between those at nutritional risk (86 ± 19%) or normally nourished (90 ± 22%) groups. Overweight and obese patients had higher levels of total cholesterol (p = 0.0049), a greater proportion of hypercholesterolemia (p = 0.001), as well as lower levels of 25 OH vitamin D (p = 0.058). Conclusions: Prevalence of overweight and obese was 6 and 1%. Excess weight status does not offer any benefit in pulmonary function in comparison to normally nourished patients, Introducción y objetivos: conocer la prevalencia de sobrepeso y obesidad, así como su asociación con la función pulmonar, el colesterol total y la vitamina D en pacientes con fi brosis quística (FQ). Material y métodos: estudio multicéntrico descriptivo y transversal. Participaron 12 hospitales españoles. Fueron incluidos 451 pacientes con FQ, clasifi cados según el índice de masa corporal (IMC) en adultos y el IMC percentilado (tablas OMS) en niños. Análisis estadístico: C.Pearson, Anova, t de Student y regresión lineal múltiple. Resultados: la mediana de edad fue 12,3 (rango 4-57) años. Un 51% eran varones y el 18%, sufi cientes pancreáticos (SP). El 12% estaba desnutrido; el 57%, en riesgo nutricional; el 24%, normonutrido; el 6% presentaba sobrepeso; y un 1%, obesidad. La función pulmonar en los pacientes con sobrepeso (91 ± 19%) era mejor que en los desnutridos (77 ± 24%) (p = 0,017), sin embargo, no se observaron diferencias con respecto a los que estaban en riesgo nutricional (86 ± 19%) o normonutridos (90 ± 22%). Los pacientes con sobrepeso tenían más elevado el colesterol total (p = 0,0049), mayor proporción de hipercolesterolemia (p = 0,001), así como niveles más bajos de 25 OH vitamina D (p = 0,058). Conclusiones: la prevalencia de sobrepeso y obesidad fue del 6 y el 1%. El sobrepeso y la obesidad no ofrecen benefi cio sobre la función pulmonar en comparación con los normonutridos

The therapeutic requirements of patients with CF are highly complex. Many patients require continuous care at home with many prophylactic medications, such as nebulised or oral antibiotics, pancreatic enzymes, mucolytic agents, vitamin and nutritional supplements as well as daily physiotherapy and a healthy lifestyle with adequate nutrition and exercise. These treatments are intensified and become more complicated during the exacerbation of the disease. Throughout its evolution, other pathologies associated with CF may also occur which require extra treatment regimens.

Celiac disease (CD) is a common autoimmune condition (involves 1-2% of the general population) that develops at any age in life but manifests differently in children and adults.To analyze clinical differences in disease expression between both groups, as well as findings at the time of diagnosis.A retrospective study of a series of patients diagnosed with CD during childhood (14 years) versus a series of adult patients (14 years).a total of 187 patients were included, of which 43 were children and 144 were adults. Among clinical manifestations in children classic presentation forms predominated -34 patients(79%) versus 20 adult patients (14%) (p0.001) (OR = 23.4;95% CI: 9.8-56.1). In contrast, atypical forms were predominant in the latter, and anemia was the most common finding in 61 patients (42%) versus 8 pediatric patients (19%) (p0.01). Adults had a greater diagnostic delay with a mean 10 ± 9 years versus 1 ± 2 years in children (p0.001). In adults, we found a higher frequency of associated autoimmune diseases (24.3 versus 9.3% in children) (p0.05). Regarding serum markers, TGt-2 was more commonly positive among children (88%) as compared to adults (31%) (p0.001); (OR = 21.4: 95% CI: 7.2-63.6). We found similar results with regard to the presence of villous atrophy, which was more common in children (95%) than in adults (33%) (p0.001) (OR = 41.0;95% CI: 9.5-76.7). As regards genetic markers, DQ2 was somewhat more common in children (97.7%) than in adults (90.3%) whereas DQ8 wasless common in children (2.3%) than in adults (9.7%), with no significant differences between groups. Patients negative for both markers were not included.Pediatric CD has clear differences when compared to adult CD, with classic forms predominating in the former, who also display a higher occurrence of positive serology and villous atrophy, and less diagnostic delay. In contrast, atypical forms predominate in the adult, with a lower occurrence of positive serology and milder histological forms. In these patients associated autoimmune conditions are more common and diagnostic delay is longer.

Objectives To determine treatment compliance and how compliance was perceived by patients, parents and by a multidisciplinary team in Cystic Fibrosis (CF) patients. Also to analyse the relative importance given to each of the prescribed treatments, reasons for non-adherence and to investigate possible predictors of therapeutic compliance. Patients and methods 34 CF patients (21 females), aged between 1.6 and 40.6 years, attending an outpatient CF clinic. Design: cross-sectional. A self-administered questionnaire was given to all patients whilst attending a programmed visit which was used to determine compliance to different treatments (physiotherapy, nutritional supplements, respiratory and digestive medications). Patients were subjectively classified as compliant or non-compliant by medical staff involved in their care. Results Treatment compliance was greater for digestive (88.2%) and respiratory medication (61.8%), compared to physiotherapy (41.2%) or nutritional supplements (59%). CF patients considered digestive medication indispensable (94.1%), compared to nutritional supplements (44.1%). Whilst 26.4% of CF patients considered that respiratory medications or nutritional supplements influenced little or nothing in their quality of life. Comparing age groups younger patients were found to be more compliant (10.4 vs. 20.5 years p=0.008) and had less severe disease (Shwachman score 83.2 vs. 73.9 p=0.048). Conclusions CF patients had greater treatment adherence when prescribed digestive and respiratory medications, compared to physiotherapy or nutritional supplements. Therapeutic adherence was found to worsen with age and disease severity, however improved with treatments which were perceived by patients as more important or had a greater influence in their quality of life.