Influence of nutrition education in paediatric coeliac disease: impact of the role of the registered dietitian: a prospective, single-arm intervention study.

BACKGROUND: The diagnosis of coeliac disease (CD) involves a change in the diet of the individual, which may influence their quality of life and nutritional status. The present study aimed to determine whether nutrition education by a registered dietitian is able to improve eating habits and body composition in children with CD. METHODS: Dietary, physical activity and body composition changes were analysed, comparing baseline assessments with those 1 year after receiving education on healthy eating. At both time points, a 3-day dietary survey, a food frequency consumption questionnaire, an adherence to the Mediterranean diet test (Kidmed), duration of activity and an electrical bioimpedance study were conducted. Student's paired t-test and the McNemar test were also employed. RESULTS: Seventy-two subjects (42 girls) with an mean (range) age of 10 (2-16) years were included. Before the intervention, an unbalanced diet was observed, rich in protein and fat, and deficient in complex carbohydrates. Only 14% consumed an adequate Mediterranean diet. After nutrition intervention, a significant increase in the consumption of plant-based foods and a concomitant decrease in meat, dairy and processed food intake (P < 0.001) were observed. Moreover, 92% of the patients (P < 0.001) managed to consume an adequate Mediterranean diet. Similarly, an increase was observed in the duration of physical activity undertaken [mean (SD) 1.02 (1.79) h, P < 0.001] and improvements in body composition were recorded, with a 17% decrease in fat mass percentage (P < 0.001). CONCLUSIONS: Nutrition intervention focused on healthy eating is effective with respect to improving the nutritional status and diet quality in CD patients.

Expansión del programa de cribado neonatal precoz de fibrosis quística en España / Expansion of early neonatal cystic fibrosis screening program in Spain

No disponible

Estatus vitamínico actual en pacientes pediátricos y adultos jóvenes con fibrosis quística / Current vitamin status in pediatric and young adult patients with cystic fibrosis

Introducción y objetivos: Las enfermedades como la fibrosis quística (FQ) que asocian malabsorción de grasas, precisan de suplementación de vitaminas liposolubles para evitar su deficiencia. El objetivo de nuestro objetivo fue conocer el estado de las vitaminas A, D y E en pacientes pediátricos y adultos con FQ. Pacientes y métodos. Estudio multicéntrico descriptivo y transversal, realizado en cinco hospitales nacionales, 126 pacientes con FQ sin agudización respiratoria, con edades entre 5 y 38 años. Los niveles de retinol y a-tocoferol se compararon con la población de referencia NHANES para establecer 3 categorías: deficientes (< P5), normales (P5-P95) y elevados (>P95); se consideró deficiencia de vitamina D si los niveles de 25 OH vitamina D fueron menores de 30 ng/ml. Resultados: La mediana de edad fue 14,2 años. El 51% eran varones y un 13% suficientes pancreáticos. El 56% presentaba deficiencia de al menos una vitamina liposoluble. La más frecuente fue la vitamina D (48%), seguida de la E (16%) y por último la vitamina A (11%). Los suficientes pancreáticos tenían niveles de vitamina D más elevados que los insuficientes. La proporción de pacientes con algún grado de deficiencia fue mayor en los pacientes adultos y sus niveles de vitamina D fueron más bajos. Conclusiones: A pesar de recibir una suplementacion adecuada, más de la mitad de los pacientes no alcanzaron niveles óptimos de vitaminas liposolubles. La función pancreática y la edad son dos factores a tener en cuenta a la hora de analizar el estatus vitamínico de estos pacientes

Introduction and objectives: Pancreatic insufficiency and fat and bile malabsoption render individuals with Cystic Fibrosis (CF) at risk for fat-soluble vitamin malabsoption. Our objective was to determine the status of vitamins A, D and E in pediatric and adult patients with CF. Patients and methods. Descriptive cross-sectional study. 126 patients without pulmonary exacerbation, aged between 5 and 38, were recruited in five national Cystic Fibrosis units. Retinol and a-tocopherol levels were compared with NHANES reference values to establish 3 categories: deficient (< P5), Normal (P5- P95) and elevated (> P95), vitamin D was considered deficient if 25 OH vitamin D < 30 ng/ml. Results: The median age was 14.2 years. 51% were male and 13% pancreatic sufficient. 56% had at least one vitamin deficiency. The most frequent was vitamin D (48%), followed by E (16%) and finally vitamin A (11%). Pancreatic sufficient had higher vitamin D levels than insufficient. The proportion of patients with any degree of vitamin deficiency was greater in adults and their vitamin D levels were lower. Conclusions: Despite adequate supplementation, more than half of patients did not reach optimal vitamins levels. Pancreatic status and age were two factors to take into account when analyzing vitamin status of these patients

Eficacia y seguridad de una pauta rush de inducción de tolerancia oral en pacientes con alergia a proteínas de leche de vaca: evolución clínica e inmunológica / Oral rush desensitization for cow milk allergy: Clinical and immunological follow-up

Introducción: Evaluar la seguridad y la eficacia de la inducción de tolerancia oral mediante pauta rush en pacientes con alergia a proteínas de leche de vaca persistente. Material y métodos: Estudio prospectivo realizado en 3 hospitales españoles. Se incluyó a niños mayores de 3 años con alergia IgE-mediada a proteínas de leche de vaca, a los que se administraron dosis crecientes de leche durante 5 días, desde 1 cc al 1% hasta 200 cc de leche pura en régimen de hospital de día. Las reacciones adversas a la administración de leche fueron registradas y se trataron atendiendo a la clasificación de Clark. Se realizó seguimiento clínico durante 2 años. Se determinaron los niveles de IgE específica basales y a los 6, 12 y 24 meses. Resultados: Se incluyó a 18 niños (13 varones) entre 3 y 14 años (media 5,96). De 271 dosis administradas, 55 presentaron algún tipo de reacción. Un 84% de las mismas fueron leves. Al finalizar el protocolo, el 100% presentaba algún grado de tolerancia (39% total). Tras 2 años, el 72% de los pacientes realizaba una dieta sin restricción de productos lácteos. Dos pacientes presentaron pérdida de la tolerancia alcanzada. Se observó un descenso significativo de los niveles de IgE específica a leche de vaca y α-lactoalbúmina a los 24 meses, y de caseína a los 6, 12 y 24 meses respecto de los niveles basales. Conclusiones: La desensibilización mediante pauta rush es una opción terapéutica eficaz y segura a medio plazo para pacientes con alergia persistente a proteínas de leche de vaca (AU)

Objective: The aim of this study was to evaluate the safety and efficacy of oral rush desensitization in children with cow milk allergy. Material and methods: Prospective study. We included IgE-mediated cow milk allergy children over 3 years in 3 Spanish hospitals. Increasing doses of cow milk for 5 days from 1 cc of 1% to 200cc of pure milk were administered. Clinical follow-up was conducted and we compared specific IgE levels at onset, 6, 12 and 24 months after desensitization. Results: We included 18 children (13 males) between 3 and 14 years (mean 5.96). A total of271 doses were administered; there were 55 adverse reactions (84% mild). At the end of the protocol, 100% showed some degree of tolerance (39% total). Full tolerance was achieved in 72%of patients after two years. Two children failed to achieve tolerance. There was a significant decrease in the levels of specific IgE to cow milk and alpha-lactalbumin at 24 months, and to casein at 6, 12 and 24 months, compared to baseline. Conclusions: Oral rush desensitization is a safe and effective therapeutic option for patients with persistent cow milk allergy to medium term (AU)

[Oral rush desensitization for cow milk allergy: Clinical and immunological follow-up]. / Eficacia y seguridad de una pauta rush de inducción de tolerancia oral en pacientes con alergia a proteínas de leche de vaca: evolución clínica e inmunológica.

OBJECTIVE: The aim of this study was to evaluate the safety and efficacy of oral rush desensitization in children with cow milk allergy. MATERIAL AND METHODS: Prospective study. We included IgE-mediated cow milk allergy children over 3 years in 3 Spanish hospitals. Increasing doses of cow milk for 5 days from 1 cc of 1% to 200 cc of pure milk were administered. Clinical follow-up was conducted and we compared specific IgE levels at onset, 6, 12 and 24 months after desensitization. RESULTS: We included 18 children (13 males) between 3 and 14 years (mean 5.96). A total of 271 doses were administered; there were 55 adverse reactions (84% mild). At the end of the protocol, 100% showed some degree of tolerance (39% total). Full tolerance was achieved in 72% of patients after two years. Two children failed to achieve tolerance. There was a significant decrease in the levels of specific IgE to cow milk and alpha-lactalbumin at 24 months, and to casein at 6, 12 and 24 months, compared to baseline. CONCLUSIONS: Oral rush desensitization is a safe and effective therapeutic option for patients with persistent cow milk allergy to medium term.

Analysis of the Spanish national registry for pediatric home enteral nutrition (NEPAD): implementation rates and observed trends during the past 8 years.

BACKGROUND/OBJECTIVES: The home enteral nutrition (HEN) provides nutritional support to children with chronic diseases who are nutritionally compromised and allows them to be discharged more quickly from hospitals. In 2003, a web-based registry (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition -NEPAD-) was created with the objective of gathering information about pediatric HEN practices in Spain. AIM: The aim of this study was to report the implementation of the NEPAD (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition) registry of pediatric HEN in Spain and to analyze data evolution trends from 2003 to 2010. SUBJECTS/METHODS: The data from the Spanish NEPAD registry were analyzed according to the following variables: demographic data, diagnosis, indication for HEN, nutritional support regime and administration route. RESULTS: Over the study period, 952 patients (1048 episodes) from 20 Spanish hospitals were included in the NEPAD registry. The most frequent indication for HEN was decreased oral intake (64%), and neurological disease was the most prevalent illness. HEN was delivered via a nasogastric tube in 573 episodes (54.7%), by gastrostomy in 375 episodes (35.8%), oral feeding in 77 episodes (7.3%) and by jejunal access in 23 episodes (2.2%). Significant differences in the mode of administration were observed based on the pathology of the child (χ(2), P<0.0001). The cyclic feeding was the most widely used technique for the administration of HEN. Most of the patients used a pump and a polymeric formula. Transition to oral feeding was the primary reason for discontinuation of this type of support. CONCLUSIONS: Since the NEPAD registry was established in Spain, the number of documented patients has increased more than 25-fold. Many children with chronic illness benefit from HEN, mainly those suffering from neurological diseases.

Alimentación y enfermedad inflamatoria intestinal. Tratamiento y control nutricional de la EII en pediatría. Parte 2 / Food and inflammatory bowel disease. Treatment and nutritional control in paediatric IBD. Part 2

La relación que se establece entre la enfermedad inflamatoria intestinal (EII) y la dieta es compleja. Las publicaciones sobre este aspecto son escasas y contradictorias, más aún, si cabe, en el ámbito de la pediatría. El presente trabajo, en una primera parte, ofrecía una revisión de la evidencia científica existente sobre el discutible papel de la dieta como factor de riesgo o causa de la aparición de la EII, así como sobre las consecuencias nutricionales que tiene dicha enfermedad en una población en periodo de desarrollo. La segunda parte de la revisión se centra en evaluar la eficacia de la nutrición enteral como tratamiento de la EII, especialmente en la enfermedad de Crohn, y el papel preponderante que en este caso desempeña frente al tratamiento farmacológico, así como su capacidad para revertir las complicaciones derivadas de esta patología en cuanto al estado nutricional y al crecimiento(AU)

The link which exists between inflammatory bowel disease (IBD) and diet is a complex one. Publications on this issue are scarce and contradictory, even more so in the paediatric field. This first paper provided a review of the existing scientific evidence concerning the debatable role of diet as a risk factor or cause of IBD, as well concerning as the nutritional consequences of this disorder for a population at this stage of development. The second part of the review concentrates on assessing the effectiveness of enteral nutrition in treating IBD, especially in cases of Crohn’s disease, and the predominant role it plays in such cases in comparison with pharmacological treatment, as well as its ability to reverse complications arising from this pathology in terms of nutritional status and growth(AU)

Alimentación y enfermedad inflamatoria intestinal. Tratamiento y control nutricional de la EII en pediatría. Parte 1 / Food and inflammatory bowel disease. Treatment and nutritional control in paediatric IBD. Part 1

La relación que se establece entre la enfermedad inflamatoria intestinal (EII) y la dieta es compleja. Las publicaciones sobre este aspecto son escasas y contradictorias, aún más, si cabe, en el ámbito de la pediatría. El presente trabajo, en una primera parte, ofrece una revisión de la evidencia científica existente sobre el discutible papel de la dieta como factor de riesgo o causa de la aparición de la EII, así como sobre las consecuencias nutricionales que tiene dicha enfermedad en una población en periodo de desarrollo. La segunda parte de la revisión se centra en evaluar la eficacia de la nutrición enteral como tratamiento de la EII, especialmente en la enfermedad de Crohn, y el papel preponderante que en este caso desempeña frente al tratamiento farmacológico, así como su capacidad para revertir las complicaciones derivadas de esta patología en cuanto al estado nutricional y al crecimiento(AU)

The link which exists between inflammatory bowel disease (IBD) and diet is a complex one. Publications on this issue are scarce and contradictory, even more so in the paediatric field. This paper first provides a review of the existing scientific evidence concerning the debatable role of diet as a risk factor or cause of IBD, as well concerning as the nutritional consequences of this disorder for a population at this stage of development. The second part of the review concentrates on assessing the effectiveness of enteral nutrition in treating IBD, especially in cases of Crohn’s disease, and the predominant role it plays in such cases in comparison with pharmacological treatment, as well as its ability to reverse complications arising from this pathology in terms of nutritional status and growth(AU)

Resistencia insulínica en pacientes pediátricos con fibrosis quística y sobrepeso / Insulin resistance in overweight cystic fibrosis paediatric patients

Introducción y objetivos: Conocer la prevalencia de sobrepeso en pacientes pediátricos con fibrosis quística (FQ) y su importancia como factor de resistencia insulínica diabetogénica y riesgo de hipertrigliceridemia. Material y métodos: Ciento nueve pacientes con FQ (47% varones) de 5-18 años se calificaron por su índice de masa corporal (IMC): desnutridos, normales o con sobrepeso. Se determinaron colesterol, triglicéridos y proteína C reactiva (PCR), glucohemoglobina y los índices HOMA-IR y QUICKI y en la sobrecarga oral de glucosa (SOG) su índice insulinogénico e ISI composite y áreas bajo la curva (ABC) de glucosa e insulina. Resultados: Seis pacientes (5,5%) tenían sobrepeso. Su edad, distribución sexual y base genética son similares al resto; aunque la proporción de suficientes pancreáticos (3/6; 50%) es superior (p=0,003). Los trastornos glucídicos no son más frecuentes que en el resto; pero la insulina basal, HOMA-IR, y la insulina durante la SOG (pico máximo y ABC) identifican su hiperinsulinismo, diferenciándolos de los otros dos grupos. Todos los pacientes muestran en la SOG respuesta insulínica tardía, sin diferencias en los índices derivados. Conclusiones: Entre los pacientes FQ con sobrepeso no hay mayor incidencia de trastornos glucídicos pero sí de hiperinsulinismo y resistencia insulínica, con el consiguiente riesgo añadido a la tendencia diabetogénica inherente a estos pacientes(AU)

Aim: To determine the prevalence of overweight in paediatric patients with cystic fibrosis (CF) and to analyse its role as diabetogenic insulin resistance factor and risk of hypertriglyceridaemia. Patients and methods: A total of 109 CF patients (47% males) between 5 and 18 years were divided into 3 groups according to body mass index (BMI): underweight, normal and overweight. Total cholesterol, triglycerides, C- reactive protein (CRP), glycosylated haemoglobin, HOMA-IR and QUICKI index were determined. Insulinogenic index, ISI composite and areas under the curve (AUC) for glucose and insulin were obtained by oral glucose tolerance test (OGTT). Results: Six patients (5.5%) were overweight. All groups had similar distribution by age, sex and CFTR mutation, although the proportion of pancreatic sufficient (3/6, 50%) was higher in overweight patients (P=.003). The prevalence of glycaemic disorders was similar between groups. Baseline insulin, HOMA-IR, and insulin during the OGTT (peak and AUC) were higher in overweight patients. All patients had a delayed insulin response in OGTT. Conclusions: Overweight CF patients do not have a higher incidence of glycaemic disorders, but their hyperinsulinism and insulin resistance may be additional diabetogenic risk factors(AU)

[Insulin resistance in overweight cystic fibrosis paediatric patients]. / Resistencia insulínica en pacientes pediátricos con fibrosis quística y sobrepeso.

AIM: To determine the prevalence of overweight in paediatric patients with cystic fibrosis (CF) and to analyse its role as diabetogenic insulin resistance factor and risk of hypertriglyceridaemia. PATIENTS AND METHODS: A total of 109 CF patients (47% males) between 5 and 18 years were divided into 3 groups according to body mass index (BMI): underweight, normal and overweight. Total cholesterol, triglycerides, C- reactive protein (CRP), glycosylated haemoglobin, HOMA-IR and QUICKI index were determined. Insulinogenic index, ISI composite and areas under the curve (AUC) for glucose and insulin were obtained by oral glucose tolerance test (OGTT). RESULTS: Six patients (5.5%) were overweight. All groups had similar distribution by age, sex and CFTR mutation, although the proportion of pancreatic sufficient (3/6, 50%) was higher in overweight patients (P=.003). The prevalence of glycaemic disorders was similar between groups. Baseline insulin, HOMA-IR, and insulin during the OGTT (peak and AUC) were higher in overweight patients. All patients had a delayed insulin response in OGTT. CONCLUSIONS: Overweight CF patients do not have a higher incidence of glycaemic disorders, but their hyperinsulinism and insulin resistance may be additional diabetogenic risk factors.

Estado nutricional en pacientes pediátricos con fibrosis quística / Nutritional condition in pediatric patients with cystic fibrosis

Introducción y objetivos. La importancia de un óptimo estado nutricional en la fibrosis quística es bien conocida, ya que incide en la mortalidad y en la función pulmonar de estos pacientes. Nuestro objetivo fue evaluar el estado nutricional de una muestra de pacientes pediátricos con fibrosis quística. Pacientes y métodos. Estudio multicéntrico descriptivo y transversal, se reclutó en cinco hospitales universitarios nacionales, 109 pacientes con fibrosis quística sin agudización respiratoria, con edades entre 5 y 18 años. Se clasificaron según el índice de masa corporal en: desnutridos, normales y sobrepeso. Análisis estadístico: ANOVA de un factor. Pruebas post-hoc de Bonferroni. Resultados. La edad media fue de 12,65 ± 3,21 años, el47% eran varones. Catorce pacientes (13%) estaban desnutridos, 89 (82%) tenían un estado nutricional normal y 6pacientes (5%) presentaban sobrepeso. Su edad, distribución sexual, base genética son similares en todos los grupos; aunque la proporción de suficientes pancreáticos (3/6; 50%)fue superior (p = 0,003) en los pacientes con sobrepeso. Los valores medios de peso, talla e índice de masa corporal expresados como puntuación Z fueron: -0,62 ± 0,78; -0,20± 1,18 y -0,32 ± 0,84 DS, respectivamente. Si analizamos los distintos datos antropométricos según el sexo y edad de los pacientes no encontramos diferencias estadísticamente significativas entre los mismos. Conclusiones. A pesar de los avances en el tratamiento, un porcentaje no desdeñable de pacientes con fibrosis quística presentan desnutrición. Por otra parte, las pautas de estilo de vida actuales y el soporte nutricional agresivo, podrían suponer en un pequeño porcentaje de casos la aparición de problemas nutricionales por exceso (AU)

Introduction and objectives. The importance of an adequate nutritional state in cystic fibrosis is well known and it is also known that it has an effect on the mortality and lung function of these patients. Our aim was to know nutritional status of a sample of pediatric cystic fibrosis patients. Patients and methods. Descriptive cross-sectional study.109 patients without pulmonary exacerbation, aged between5 and 18, were recruited in five national cystic fibrosis units. Were divided into 3 groups according body mass index: malnourished, normal and overweight. Statistical analysis: oneway ANOVA, Bonferroni Post-hoc tests. Results. The mean age was 12.65 ± 3.21 years, 47% were male. Fourteen patients (13%) were malnourished, 89 (82%)had normal nutritional status, and 6 patients (5%) were overweight. All groups had similar distribution by age, sex and CFTR mutation, although proportion of pancreatic sufficient (3/6, 50%) was higher in overweight patients (p=0.003). Mean weight, height and body mass index(expressed as Z score) were: -0.62 ± 0.78; -0.20 ± 1.18 and -0.32 ± 0.84 DS respectively. We don’t find differences in anthropometric data when we analyze by sex and age of the patients. Conclusions. Despite advances in treatment, a significant percentage of cystic fibrosis patients are malnourished. Moreover, current lifestyle and nutritional support, could lead in a small percentage of patients the appearance of overnutrition problems (AU)

Pancreatitis en la fibrosis quística: correlación con el genotipo y estado pancreático / Pancreatitis in cystic fibrosis: association with genotype and pancreatic status

Introducción: La pancreatitis es una rara complicación en la evolución de pacientes con fibrosis quística (FQ). Puede presentarse en forma de episodios agudos, aislados o repetidos, o evolucionar a cronicidad con progresiva destrucción de la glándula. El objetivo de este estudio fue describir las características de una cohorte de pacientes que habían padecido pancreatitis, conocer su frecuencia e intentar encontrar posibles factores de riesgo asociados. Metodología: Estudio retrospectivo descriptivo de pacientes controlados en unidades de FQ de cinco hospitales españoles que habían padecido pancreatitis. Se recogieron datos demográficos, clínicos y analíticos, y relativos al estado pancreático y el genotipo. Resultados: De 520 pacientes, 17 presentaron pancreatitis. Una prevalencia del 3,3%, superior a la descrita en la literatura. Analizando el estado pancreático, se observó que 8 de ellos eran insuficientes pancreáticos (47,06%), hecho que contrasta, en parte, con lo referido clásicamente al considerar esta complicación más típica de pacientes con cierto grado de reserva pancreática. No se encontraron factores de riesgo ni asociaciones significativas con la genética, edad, sexo u otras características. Conclusiones: En nuestra serie, la prevalencia de pancreatitis es superior a la descrita en la literatura, no tratándose de una complicación exclusiva de suficientes pancreáticos. Se produce más frecuentemente durante la adolescencia o en el inicio de la edad adulta. La enfermedad pulmonar es leve en la mayoría. La genética es variable, sin poder establecerse una clara relación genotipo-fenotipo. Se debe observar a largo plazo la evolución de esta patología intercurrente y diseñar estudios más amplios para obtener resultados más significativos (AU)

Introduction: Pancreatitis is an uncommon complication of cystic fibrosis (CF). Either single or recurrent acute episodes can occur and it occasionally may follow a protracted course with relentless destruction of the pancreas. Moreover mild mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) have been found in many cases of idiopathic chronic pancreatitis. We describe a group of patients with CF who had one or more episodes of pancreatitis. We have estimated its prevalence in a large population of patients with CF across Spain. Methods: A retrospective descriptive study was conducted by collecting the demographic, clinical and laboratory data, pancreatic status and genotype of CF patients who attended the CF Units in 5 Spanish hospitals. Results: The overall number of CF patients under follow-up in the five centres was 520, of which 17 cases with pancreatitis were identified. The prevalence of pancreatitis in this population was 3.3%, higher than previously reported. Noticeably eight of the 17 patients (47.06%) had pancreatic insufficiency. This appears to be, partly, in contrast with that classically found, as this complication is usually associated with patients with a certain level of pancreatic reserve. No associations with genotype, age, gender or other factors were found. Conclusions: The prevalence of pancreatitis in our CF patients was higher than that found in other CF populations, and was not limited to patients with pancreatic sufficiency. It occurred mostly in teenagers and young adults often with mild pulmonary disease. The CF genotype was variable. The course of the patients should be carefully monitored, and further information on the long-term outcome of larger cohorts of patients is needed (AU)

[Pancreatitis in cystic fibrosis: association with genotype and pancreatic status]. / Pancreatitis en la fibrosis quística: correlación con el genotipo y estado pancreático.

INTRODUCTION: Pancreatitis is an uncommon complication of cystic fibrosis (CF). Either single or recurrent acute episodes can occur and it occasionally may follow a protracted course with relentless destruction of the pancreas. Moreover mild mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) have been found in many cases of idiopathic chronic pancreatitis. We describe a group of patients with CF who had one or more episodes of pancreatitis. We have estimated its prevalence in a large population of patients with CF across Spain. METHODS: A retrospective descriptive study was conducted by collecting the demographic, clinical and laboratory data, pancreatic status and genotype of CF patients who attended the CF Units in 5 Spanish hospitals. RESULTS: The overall number of CF patients under follow-up in the five centres was 520, of which 17 cases with pancreatitis were identified. The prevalence of pancreatitis in this population was 3.3%, higher than previously reported. Noticeably eight of the 17 patients (47.06%) had pancreatic insufficiency. This appears to be, partly, in contrast with that classically found, as this complication is usually associated with patients with a certain level of pancreatic reserve. No associations with genotype, age, gender or other factors were found. CONCLUSIONS: The prevalence of pancreatitis in our CF patients was higher than that found in other CF populations, and was not limited to patients with pancreatic sufficiency. It occurred mostly in teenagers and young adults often with mild pulmonary disease. The CF genotype was variable. The course of the patients should be carefully monitored, and further information on the long-term outcome of larger cohorts of patients is needed.

La fibrosis quística en la actualidad (II): aspectos nutricionales / Cystic fibrosis at the present time (II): nutritional aspects

La desnutrición crónica ha sido un problema universal, y actualmente la vigilancia y el soporte nutricional son parte integral y esencial en el cuidado multidisciplinario de esta enfermedad. La importancia de un óptimo estado nutricional es bien conocida, y así se sabe que incide en la mortalidad, que disminuye la morbilidad y que podría favorecer un menor deterioro de la función pulmonar. Actualmente el objetivo es lograr mantener a los pacientes sobre el percentil 50 de índice de masa corporal. Si no se consigue alcanzar o mantener las metas propuestas con las modificaciones en la dieta, optimizando el aporte energético, se pueden aportar suplementos, y en algunos casos están indicadas otras medidas de soporte más agresivas, como la nutrición enteral. Además, son importantes los aportes de micronutrientes, como las vitaminas liposolubles, por lo que se recomienda su administración sistemática; en los últimos años, con el aumento de la expectativa de vida de las personas, se presentan otros desafíos nutricionales, como los déficit específicos de ácidos grasos o el mantenimiento de una buena salud ósea (AU)

Chronic malnutrition has been a universal problem and at the present moment the nutritional monitoring and support are major and essential parts within the disease. Are the importance of an adequate nutritional state is well known and it is also known that it has an effect on the mortality, that it diminishes the morbidity and that it could favor a better pulmonary function. Currently keeping the patients BMI around the 50th percentile is a reasonable target. The therapies to achieve this goal include diet monitoring ensuring a high caloric intake, energy supplements can be added and in restricted cases more aggressive intervention as enteral feeding might be needed. Adequate intake of micronutrients and liposoluble vitamins are important recommending a systematic administration in the last years, with the increase of life expectancy other nutritional challenges are brought up, such as the deficiency of essential fatty acids or the keeping of a good bone health (AU)

Prevención de la alergia a las proteínas de la leche de vaca / Prevention of cow's milk protein allergy

No disponible

La fibrosis quística en la actualidad (I): aspectos digestivos / Cystic fibrosis update time (I): digestive aspects

La fibrosis quística es una enfermedad genética, caracterizada fundamentalmente por neumopatía crónica progresiva e insuficiencia pancreática, pero presenta un amplio espectro de manifestaciones clínicas. En los últimos años se ha profundizado notablemente en su conocimiento y en el desarrollo de nuevas medidas terapéuticas, lo que ha conducido a la prolongación de la expectativa de vida y a una considerable mejora en la calidad de ésta. El pronóstico depende principalmente de la severidad de la afectación pulmonar; sin embargo, controlarlas diversas manifestaciones digestivas y evitar la desnutriciónes esencial para lograr una buena evolución. Los aspectos digestivos son diversos, y en este trabajo se revisan los más recientes tanto desde el punto de vista diagnóstico como terapéutico. La enfermedad hepática, relativamente frecuente, aparece como una complicación temprana; en la mayoría de los casos se desarrolla una afectación leve, aunque una pequeña proporción de pacientes cursan con una alteración severa con cirrosis, que conduce a hipertensión portal e hiperesplenismo. La insuficiencia pancreática exocrina aparece en aproximadamente el 90% de los pacientes, que precisan tratamiento sustitutivo enzimático. Otras complicaciones, como la esteatorrea rebelde, la inflamación intestinal y el síndrome de obstrucción intestinal distal, han adquirido especial relevancia en los últimos años (AU)

Cystic fibrosis is a genetic disorder characterized mainly by a chronic progressive pulmonary disease and pancreatic insufficiency but with a wide range of clinical manifestations. Over the last years breakthroughs in the understanding of its pathogenesis and the development of new therapies have lead to outstanding improvements both in survival and quality of life. Its prognosis mostly depends on the severity of the pulmonary impairment but adequate control of the gastrointestinal manifestations, ensuring a good nutritional condition is essential for achieving a good outcome. The digestive aspects are various and they will be examined from the most recent points of view of therapeutical diagnosis. Liver disease is quite common. It often has an early onset but most patients have competitive lymild impairment with only a few developing multifocal cirrhosis with conducts to a portal hypertension and hypersplenism. Pancreatic exocrine insufficiency occurs in over 90% of the patients and requires of a replacement enzyme therapy. Other complications like unresponsive steatorrhea control, intestinal inflammation and distal intestinal obstruction syndrome have acquired special relevance in the last years (AU)

Patología gastrointestinal en niños con parálisis cerebral infantil y otras discapacidades neurológicas / Gastrointestinal disorders in children with cerebral palsy and neurodevelopmental disabilities

A pesar de los constantes avances en la medicina perinatal, la prevalencia de los niños con parálisis cerebral infantil no ha disminuido en los últimos 20 años. La patología gastrointestinal constituye uno de los principales problemas de estos y otros pacientes con discapacidades neurológicas. El manejo multidisciplinar de estos pacientes, por parte de neurólogos, gastroenterólogos, enfermeras, dietistas y otros especialistas, contribuye a una mejora sustancial de su calidad de vida y la de sus cuidadores. En este artículo discutiremos sobre los métodos diagnósticos y las opciones terapéuticas disponibles para los principales problemas nutricionales y gastrointestinales en los pacientes con discapacidades neurológicas: el reflujo gastroesofágico, el estreñimiento y los trastornos de la deglución (AU)

Recent data suggest that, contrary to initial expectations with improvements in perinatal medicine, the prevalence of cerebral palsy has not decreased over the last 20 years. Gastrointestinal disorders are a major chronic problem in most of children with cerebral palsy and in children with neurodevelopmental disabilities. A multidisciplinary approach, with input from neurologists, gastroenterologists, nurses, dieticians and other specialists, can make a major contribution to the medical wellbeing and quality of life of these children and their caregivers. This article focuses on diagnostic methods and therapeutic options available for major nutritional and gastrointestinal problems in patients with neurological disabilities: gastroesophageal reflux, constipation and swallowing disorders (AU)

[Gastrointestinal disorders in children with cerebral palsy and neurodevelopmental disabilities]. / Patología gastrointestinal en niños con parálisis cerebral infantil y otras discapacidades neurológicas.

Recent data suggest that, contrary to initial expectations with improvements in perinatal medicine, the prevalence of cerebral palsy has not decreased over the last 20 years. Gastrointestinal disorders are a major chronic problem in most of children with cerebral palsy and in children with neurodevelopmental disabilities. A multidisciplinary approach, with input from neurologists, gastroenterologists, nurses, dieticians and other specialists, can make a major contribution to the medical wellbeing and quality of life of these children and their caregivers. This article focuses on diagnostic methods and therapeutic options available for major nutritional and gastrointestinal problems in patients with neurological disabilities: gastroesophageal reflux, constipation and swallowing disorders.

Enfermedad de Kawasaki. Experiencia en nuestro hospital / Kawasaki's Disease. Experience in our hospital

Objetivo: Conocer la situación de la enfermedad de Kawasaki en nuestro medio. Pacientes y métodos: Se revisaron de forma retrospectiva, entre 1995 y 2007, a los niños que cumplían los criterios diagnósticos de la enfermedad de Kawasaki en su forma completa o incompleta, en el Hospital Universitario Central de Asturias. Se realizó un análisis descriptivo de los datos. Resultados: Revisamos un total de 19 pacientes. El 84%fueron menores de 3 años y el 57% varones. La incidencia anual fue de 8,4 casos/100.000 niños menores de 5 años. El 31% (todos varones) presentaron alteraciones en la ecocardiografía inicial; sin embargo, todos se normalizaron en controles posteriores. No se registraron aneurismas. El75% presentaron una PCR >5 mg/dl y hasta el 77% una VSG>50 mm/h. Solo 2 niños no respondieron a gammaglobulinai .v., requiriendo una segunda dosis. Conclusiones: La enfermedad de Kawasaki presenta en nuestro medio una incidencia semejante a la comunicada en Europa y Estados Unidos. Las complicaciones coronarias son más frecuentes en varones y en pacientes con fiebre prolongada (AU)

Objective: The aim of this study was to analyze the incidence and clinical spectrum of Kawasaki’s disease in children attended in our hospital. Patients and methods: Retrospective review of all children with Kawasaki’s disease evaluated in the department of Pediatric of Hospital Central (Asturias), between 1995and 2007.Results: Nineteen patients with Kawasaki’s disease were analyzed. 84% were younger than 3 years and 57% men. The annual incidence rate in our health area was estimated at8.4 cases per 100,000 children < 5 years. 31 % (all male)showed abnormalities in the initial echocardiography, none that included coronary aneurysms, all them were normalized in later controls. Blood analysis showed alterations of high ESR (77% of cases) and C-reactive protein (75%). Only2 children did not respond to IV gamma globulin requiring a second dose. Conclusions: The annual incidence rate for Kawasaki’s disease in our area was similar to that in Europe and the United States. Coronary aneurysms were more common in men and in patients with prolonged fever (AU)