High serum retinol and lung function in young patients with cystic fibrosis.

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 < 0.05). The z score of SR correlated positively with FEV1 (r = 0.364; P = 0.000), after adjusting data for sex, age, body mass index, and pancreatic function. The odds ratio for a FEV1 >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.

Safety and efficacy of a new supplementation protocol in patients with cystic fibrosis and vitamin D deficiency.

OBJECTIVES: Based on the European and American Cystic Fibrosis (CF) consensus recommendations, an increase in vitamin D (VD) supplementation in patients with CF and insufficient or defficient levels was proposed. The objective of our study was to determine the safety and efficacy of this new protocol. MATERIAL AND METHODS: Multicentre nonrandomized uncontrolled experimental study. Patients with insufficient levels (<30 ng/mL) received increasing doses of VD (between 800 and 10 000 IU/day). Patients were followed up for 12 months, during which their vitamin and nutritional status, pulmonary function and calcium and phosphate metabolism were assessed. STATISTICAL ANALYSIS: t test for paired data and multivariate logistic regression analysis. RESULTS: Thirty patients aged 1-39 years (median, 9.1) completed the follow-up. Two patients were dropped from the study on account of 25-OH VD levels greater than 100 ng/mL at 3 months without clinical or laboratory signs of hypercalcaemia. At 12 months, we observed an increase of 7.6 ng/mL (95% CI, 4.6-10 ng/mL) in the mean 25-OH VD level and an improvement in vitamin status: 37% achieved levels of 30 ng/mL or greater, 50% levels between 20 and 30 ng/mL and 13% remained with levels of less than 20 ng/mL. We found no association between improved VD levels and pulmonary function. CONCLUSIONS: The proposed protocol achieved an increase in serum VD levels and a decrease in the percentage of patients with VD insufficiency, although it was still far from reaching the percentages of sufficiency recommended for this entity.

Differences between pediatric and adult celiac disease.

INTRODUCTION: 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. OBJECTIVES: To analyze clinical differences in disease expression between both groups, as well as findings at the time of diagnosis. METHODS: A retrospective study of a series of patients diagnosed with CD during childhood (< 14 years) versus a series of adult patients (> 14 years). RESULTS: 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%) (p < 0.001) (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%) (p < 0.01). Adults had a greater diagnostic delay with a mean 10 ± 9 years versus 1 ± 2 years in children (p < 0.001). In adults, we found a higher frequency of associated autoimmune diseases (24.3 versus 9.3% in children) (p < 0.05). Regarding serum markers, TGt-2 was more commonly positive among children (88%) as compared to adults (31%) (p < 0.001); (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%) (p < 0.001) (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. CONCLUSIONS: 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.

Gluten-Free Diet: Nutritional Strategies to Improve Eating Habits in Children with Celiac Disease: A Prospective, Single-arm Intervention Study.

BACKGROUND: Elimination of gluten-containing cereals and consumption of ultra-processed gluten-free foods might cause an unbalanced diet, deficient in fiber and rich in sugar and fat, circumstances that may predispose celiac children to chronic constipation. AIM: to evaluate if counseling with a registered dietitian (RD) was capable of improving eating and bowel habits in a celiac pediatric population. METHODS: Dietetic, lipid profile and stool modifications were analyzed, comparing baseline assessments with those twelve months after receiving heathy eating and nutrition education sessions. At both time points, 3-day food records, a bowel habit record and a lipid panel were conducted. Calculated relative intake of macro- and micro-nutrients were compared with current recommendations by the European Food Safety Authority (EFSA). Student's paired t-test, McNemar test, Mandasky test and Pearson correlation tests were used. RESULTS: Seventy-two subjects (58.3% girls) with a mean (standard deviation (SD)) age of 10.2 (3.4) years were included. Baseline diets were imbalanced in macronutrient composition. Significant improvements were observed in their compliance with dietary reference values (DRVs), where 50% of the subjects met fat requirements after the education and 67% and 49% with those of carbohydrates and fiber, respectively (p < 0.001). Celiac children decreased red meat and ultra-processed foods consumption (p < 0.001) and increased fruits and vegetables intake (p < 0.001), leading to a reduction in saturated fat (p < 0.001) and sugar intake (p < 0.001). Furthermore, 92% of the patients achieved a normal bowel habit, including absence of hard stools in 80% of children constipated at baseline (p < 0.001). CONCLUSIONS: RD-led nutrition education is able to improve eating patterns in children with celiac disease (CD).

Vitamin D Status in Pediatric and Young Adult Cystic Fibrosis Patients. Are the New Recommendations Effective?

INTRODUCTION: In recent years, guidelines for vitamin D supplementation have been updated and prophylactic recommended doses have been increased in patients with cystic fibrosis (CF). OBJECTIVE: To evaluate safety and efficacy of these new recommendations. RESULTS: Two cohorts of pancreatic insufficient CF patients were compared before (cohort 1: 179 patients) and after (cohort 2: 71 patients) American CF Foundation and European CF Society recommendations were published. Cohort 2 patients received higher Vitamin D doses: 1509 (1306-1711 95% CI) vs 1084 (983-1184 95% CI) IU/Day (p < 0.001), had higher 25 OH vitamin D levels: 30.6 (27.9-33.26 95% CI) vs. 27.4 (25.9-28.8 95% CI) ng/mL (p = 0.028), and had a lower prevalence of insufficient vitamin D levels (<30 ng/mL): 48% vs 65% (p = 0.011). Adjusted by confounding factors, patients in cohort 1 had a higher risk of vitamin D insufficiency: OR 2.23 (1.09-4.57 95% CI) (p = 0.028). CONCLUSION: After the implementation of new guidelines, CF patients received higher doses of vitamin D and a risk of vitamin D insufficiency decreased. Despite this, almost a third of CF patients still do not reach sufficient serum calcidiol levels.

[Analysis of health-related quality life in celiac patients]. / Análisis de la calidad de vida relacionada con la salud en pacientes celiacos.

INTRODUCTION: 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: 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.

Study of adherence to the gluten-free diet in coeliac patients.

INTRODUCTION: The following of a strict gluten-free diet (GFD) is essential in the control of coeliac disease. The aim of this study was to determine the adherence to a GFD in coeliac patients and to evaluate the factors that could influence this adherence. MATERIAL AND METHODS: A descriptive observational study was carried out, in which gluten immunogenic peptides (GIP) were determined in faeces using a semi-quantitative method, and the Coeliac Dietary Adherence Test was completed. Sociodemographic and clinical details were collected, and an ad hoc questionnaire was prepared. RESULTS: Of the 80 patients included, 92.5% were adherent according to the GIP and 86.3% according to Coeliac Dietary Adherence Test (acceptable agreement; Kappa: 0.31, P = .004). The large majority (83.3%) of patients with positive GIP gave negative anti-transglutaminase antibodies in the latest determination. Current age and time of onset were significantly associated with adherence. Those with a positive GIP had a mean age of 5 years more (P = .0001) and were 52 months more on a GFD (P = .025). One quarter of those surveyed considered the diet difficult to follow. Just under two-thirds (60%) considered that the variability in the eating site was an important factor in leading to infringements, with children's parties being the main area where they occurred (66.7%). The lack of variety (61.4%) and the increased cost (98.6%) of gluten-free foods is highlighted. CONCLUSIONS: The adherence to the GFD is generally good. The analysis of GIP helps to detect non-adherent patients that would pass unnoticed in other circumstances. Measures must be established in order to maintain good long-term adherence, taking into account the risk factors and difficulties detected.

Assessment of Two Nutritional Screening Tools in Hospitalized Children.

AIM: to evaluate validity and concordance of Screening Tool for the Assessment of Malnutrition in Pediatrics (STAMP) and Screening Tool for Risk On Nutritional status and Growth (STRONGkids) screening tools for assessment of nutritional risk in pediatric inpatients. METHODS: Prospective longitudinal observational multicenter study in children aged 1 month or older admitted as inpatients. Weight, height, cause of admission, demographic data, length of stay, and nutritional interventions were recorded. STAMP and STRONGkids were applied within the first 72 h of admission. Anthropometric measurements were recorded again 12-18 months after admission. RESULTS: Eighty-one patients with median age of 4.1 years completed the study. Agreement between tools was moderate (κ = 0.47). STAMP had a greater tendency to classify patients as high risk (12.3% vs. 2.5%). Both tools showed very weak correlation with height for age. All undernourished patients at the beginning and the end of the study were classified as medium or high risk by STAMP and STRONGkids (100% sensitivity), although specificity was below 50% in all cases. There were no differences in length of stay based on nutritional risk with any of the tools. CONCLUSIONS: STAMP and STRONGkids demonstrated moderate agreement, with high sensitivity but low specificity for the diagnosis of undernutrition. Further studies are required to analyze cost-effectiveness of these tools and nutritional interventions derived from them.

Manual Physical Therapy in the Treatment of Functional Constipation in Children: A Pilot Randomized Controlled Trial.

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). Design: A pilot randomized and controlled trial was conducted. Settings/Location: Subjects were recruited in a tertiary university hospital in the north of Spain. Subjects: Forty-seven children (2-14 years) were included. Participants were assessed by Pediatric Gastroenterologist. They were recruited in a tertiary university hospital. Subjects were randomly allocated. MPT was performed in a private clinic. Interventions: Control group (CG) received CPT and intervention group (IG) received MPT, consisting in nine sessions of MPT with a 45-min initial session and 30 min for the rest of sessions distributed weekly during the first and second months and biweekly in the third month. Outcome Measures: At times 0-1-3 months and 5 years, results obtained were checked and compared, according to the defecatory pattern ("Symptom Severity Score" SSS), quality of life (Pediatric Quality of Life Inventory Scale), Bristol Stool Form Scale (BSFS), and the defecation frequency (DF). Results: Results from SSS, BSFS, and DF revealed no statistically significant differences between groups in median values at any follow-up. However, there were significant changes over time. The full sample median for SSS was reduced from baseline 24 (interquartile range 20-27) to 11 (6-13) at month 1, 7 (3-13) at month 3, (8 CG; 5 IG), and 5 (1-12) at year 5. The BSFS scale increased from 2 (1-3) at baseline to 4 (3-4) at month 1 and year 5. DF increases progressively from 1 per week to 5 in the fifth year. Higher rates of quality of life were observed at month 3 in IG for the total, physical, and emotional functioning scores and at fifth year for total, emotional, social, and school functioning scores. Conclusions: This study gives preliminary evidence of no difference between MPT and CPT for improvement in measures of CPC, although there was some advantage for MPT in quality of life. Study results justify the conduct of a full-scale efficacy trial of MPT, as well as a noninferiority trial comparing MPT and CPT.

[Effect of healthy eating before intervention with a low FODMAP diet in pediatric patients with irritable bowel syndrome]. / Efecto de la alimentación saludable previa a la intervención con dieta baja en FODMAP en pacientes pediátricos con síndrome de intestino irritable.

INTRODUCTION: Background: 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.

INTRODUCCIÓN: Introducción: una dieta baja en oligosacáridos, disacáridos, monosacáridos y polioles fermentables (FODMAP) puede ser eficaz en el tratamiento de los pacientes pediátricos con síndrome de intestino irritable (SII). Su complejidad y efectos secundarios hacen de la misma una alternativa terapéutica secundaria. Objetivo: demostrar que la educación nutricional, dirigida a optimizar la dieta de los niños con SII, es capaz de mejorar sus síntomas gastrointestinales sin tener que realizar una dieta baja en FODMAP. Métodos: estudio de intervención prospectivo. Se analizaron los cambios de los síntomas gastrointestinales, mediante el cuestionario pediátrico de calidad de vida para síntomas gastrointestinales (PedsQL™ síntomas GI), tras recibir educación nutricional basada en alimentación saludable. Asimismo, se analizaron los cambios antropométricos y de hábitos dietéticos tras dicha intervención. Resultados: se incluyeron 21 pacientes (12 niñas) con edad media de 10,6 años (5-14 años). Se observó una dieta con un exceso de ingesta de azúcares simples, grasas saturadas y sal junto con un déficit de fibra. Tras la intervención, se apreció un aumento de 8,07 puntos en el cuestionario (IC del 95%: 13,42 a -2,73; p = 0,005). Además, se observó una pérdida de peso significativa en los pacientes con sobrepeso y obesidad (disminución del Z-score de índice de masa corporal [IMC] 0,62 DE; p = 0,001). Se observaron cambios significativos en los hábitos dietéticos: aumento de consumo de hidratos de carbono complejos, frutas y verduras y disminución de azúcares simples. Conclusiones: la alimentación saludable resulta eficaz para mejorar los síntomas gastrointestinales en pacientes pediátricos con SII, sin requerir la exclusión de FODMAP.

Treatment compliance in children and adults with cystic fibrosis.

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.

[Attitudes towards cow's milk protein allergy management by spanish gastroenterologist]. / Manejo de la alergia a proteína de leche de vaca por los gastroenterólogos españoles.

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.

[Malnutrition in children admitted to hospital. Results of a national survey]. / Malnutrición en el niño ingresado en un hospital. Resultados de una encuesta nacional.

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 (<8%) than previously reported, probably in relation to the variety of hospitals in the survey. Nevertheless, nutritional risk when evaluated with STAMP showed a high risk of malnutrition.

[Excess weight in patients with cystic fibrosis: is it always beneficial?]

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.

[Fat-soluble vitamins supplementation in patients with cystic fibrosis: is enough Aquadek's®?]. / Suplementacion con vitaminas liposolubles en pacientes con fibrosis quistica: ¿es suficiente con Aquadek's®?

INTRODUCTION AND OBJECTIVE: Evaluate the safety and efficacy of a novel polyvitaminic (Aquadek's®) in patients with Cystic Fibrosis (CF). MATERIAL AND METHODS: 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. STATISTICAL ANALYSIS: paired t tests. RESULTS: 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 (p <0.001) and decreased the percentage of patients with pro-vitamin A deficiency 46% (95% CI 22-64) (p <0.001). 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.

[VITAMIN D AND CHRONIC LUNG COLONIZATION IN PEDIATRIC AND YOUNG ADULTS CYSTIC FIBROSIS PATIENTS]. / VITAMINA D Y COLONIZACIONES PULMONARES CRÓNICAS EN PACIENTES PEDIÁTRICOS Y ADULTOS JÓVENES CON FIBROSIS QUÍSTICA.

INTRODUCTION AND OBJECTIVES: evaluate vitamin D status and its association with chronic lung colonisation in Cystic Fibrosis patients. MATERIAL AND METHODS: descriptive cross-sectional multicenter study. From November 2012 to April 2014, at 12 national hospitals, 377 patients with Cystic Fibrosis were included. Vitamin D levels < 30 ng/ml were classified as insufficient. Chronic colonisation was considered if they had at least two positive cultures in the past year. RESULTS: 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 p < 0.001). 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. CONCLUSIONS: 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 D < 30 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 meses­20 años). Un 65% presentaban niveles insuficientes de vitamina D. Se observó una correlación inversa entre edad y niveles de vitamina D (r = -0,20 p < 0,001). 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.

[Nutrition status on pediatric admissions in Spanish hospitals; DHOSPE study]. / Evaluación del estado nutricional de niños ingresados en el hospitalen en España; estudio DHOSPE (Desnutrición Hospitalaria en el Paciente Pediátrico en España).

UNLABELLED: Malnutrition among hospitalized patients has clinical implications and is associated with adverse outcomes: depression of the immune system, impaired wound healing, muscle wasting, longer length of stay, higher costs and increased mortality. Although the rate of malnutrition in hospitalized children varies in different studies, it seems to be lower than in adult population. Nevertheless, this is a population that has a higher risk of developing malnutrition during hospital stay. There is a need to find the most suitable nutrition screening tool for pediatric patients. AIM: As a first step, we have performed a nationwide study on the prevalence of malnutrition on admission, in order to further evaluate the results of employing a screening tool (STAMP). MATERIAL AND METHODS: The study is a multicenter, transversal study performed in 32 Spanish hospital between June and September 2011 in patients under 17 admitted to a the hospital longer than 48 hours. Weight, height and STAMP questionnaire were done on admission and repeated at day 7, 14 or at discharge. Nutritional status was classified according to Waterlow index for height and for weight. The study was approved by the Ethics Research Committee in each hospital and informed consent obtained prior to be included in the study. RESULTS: 991 patients were finally included. Mean age was 5.0 years (SD: 4.6), distributed uniformly among ages. Moderate to severe malnutrition was present in 7.8%, and overweight-obesity in 37.9%. We found a significant correlation between nutritional status and type of disease. There were no correlationship with age, or with plasmatic albumin levels. comments: This is the first nationwide study on the prevalence of malnutrition on admission in pediatric patients. Malnutrition in pediatric patients was present in around 8% of admissions, slightly inferior to other series. The most likely explanation is that the study included patients from different types of hospitals, mimicking real life conditions.

La desnutrición en los pacientes hospitalizados tiene repercusiones clínicas y se asocia con peores resultados: inmunodepresión, retraso en la cicatrización de las heridas, atrofia muscular, prolongación del ingreso hospitalario y mayor mortalidad. La tasa de desnutrición al ingreso en el paciente pediátrico varía con los estudios, aunque parece inferior a lo que ocurre en el paciente adulto. Sin embargo, es una población de mayor riesgo de desarrollar desnutrición durante el ingreso. Se precisa, por tanto, encontrar una buena herramienta de cribado nutricional. Objetivo: Como primer paso para alcanzar ese objetivo se realizó un estudio de ámbito nacional para determinar la tasa de desnutrición en el ingreso. Material y métodos: Se trató de un estudio transversal, multicéntrico realizado en 32 hospitales españoles entre junio y septiembre de 2011 en pacientes < 17 años que ingresaran en el hospital por un periodo > 48 horas. Se midieron peso y talla y se pasó el cuestionario STAMP en el momento del ingreso y a los 7, 14 días o en el momento del alta. El estado nutricional se clasificó de acuerdo con el índice de Waterlow para peso y talla. El estudio fue aprobado por el Comité Ético de Investigación de cada uno de los hospitales y se requirió la firma del consentimiento informado antes de su inclusión en el estudio. Resultados: 991 pacientes participaron en el estudio. La edad media fue de 5 años (DE: 4,6), distribuidos de forma uniforme entre todas las edades. Se encontró desnutrición moderada o grave en el 7,8% y sobrepeso-obesidad en el 37,9% de los ingresados. Encontramos una situación nutricional significativamente peor para todos los grupos de edad en función de la enfermedad de base. No encontramos correlación entre la desnutrición y la edad, o los niveles de albúmina sérica. Comentarios: Esta es la primera encuesta nacional para estudiar la prevalencia de desnutrición en el momento del ingreso. La cifra encontrada, 8%, fue ligeramente inferior a la encontrada en otros estudios, probablemente debido a la inclusión de pacientes de hospitales de distinto grado de complejidad, acercándose a lo que sería una muestra real de la población española.