Seguimiento del crecimiento y estado de nutrición de pacientes pediátricos con afectación neurológica severa. Desarrollo de una aplicación informática específica

INTRODUCCIÓN Los pacientes con enfermedades neurológicas severas con frecuencia presentan trastornos de la nutrición y del crecimiento de origen multifactorial, siendo estos más evidentes a mayores grados de afectación motora. La vigilancia nutricional debe formar parte del cuidado integral de estos pacientes porque una intervención adecuada, no sólo mejora el estado de nutrición, sino que también va a disminuir de forma considerable comorbilidades asociadas. HIPÓTESIS Los pacientes con enfermedades neurológicas crónicas experimentan un deterioro clínico y de nutrición paralelo a la gravedad de la afectación motora, lo que conduce a peor calidad de vida y a una sobrecarga psicológica de los cuidadores. La atención nutricional específica debe mejorar la evolución clínica y nutricional, repercutiendo positivamente en la calidad de vida del paciente y de sus cuidadores. OBJETIVO Analizar de forma longitudinal el estado de nutrición de niños con enfermedad neurológica y el estado psicosocial de sus cuidadores. METODOLOGÍA Estudio observacional prospectivo multicéntrico. Se incluyen pacientes menores de 16 años con afectación neurológica y motora moderada-severa (III, IV o V de la escala Gross Motor Function Classification System. Se realizó evaluación clínica, dietética, antropométrica, bioquímica y estudio de densidad mineral ósea (DMO) mediante densitometría, con seguimiento longitudinal durante un año. Los datos antropométricos y de DMO fueron normalizados a z-score para su edad y sexo. Se pasaron cuestionarios a los cuidadores para estudiar factores psicosociales. Se diseñó una aplicación informática para el seguimiento nutricional específico de estos pacientes. RESULTADOS Y DISCUSIÓN Se incluyeron un total de 68 pacientes (62% varones), con una edad media de 9,6 ± 4 años. Los diagnósticos incluidos fueron: parálisis cerebral infantil (PCI, 47), enfermedades neuromusculares (4), encefalopatías epilépticas (4), anomalías genéticas (10), enfermedades metabólicas (1) y daño cerebral adquirido (1). La mayor parte presentaban afectación motora de grado muy severo. 21 niños eran portadores de gastrostomía (31%). La valoración antropométrica inicial sugirió un estado de desnutrición crónica, con parámetros similares en los portadores de gastrostomía respecto a los alimentados vía oral excepto en la talla (mayor en los pacientes sin gastrostomía). El peso y el perímetro braquial fueron menores a mayor afectación motora. En la evaluación longitudinal antropométrica, los pacientes se encontraron estables. Respecto al soporte nutricional, el 57% de los niños portadores de gastrostomía recibían alimentación mediante fórmulas de nutrición enteral (FNE). Los pacientes alimentados vía oral recibían alimentos naturales, y 51% recibían algún tipo de suplemento en forma de FNE. Mostraron problemas de deglución un 73,5%, siendo más frecuentes a mayor afectación motora. La ingesta energética total fue muy heterogénea, siendo la distribución de macronutrientes similar a la población general. Destacaron bajas ingestas de calcio (69%) y vitamina D (92%). A nivel bioquímico, tuvieron déficit de vitamina D el 43% y de zinc el 17%. En el análisis de la salud ósea, un 16% tenía antecedente de alguna fractura. El 50% de los niños presentaron baja DMO (z score menor de -2). Encontramos correlación entre menor DMO y mayor afectación motora y nutricional. Los cuestionarios psicosociales fueron contestados mayoritariamente por las madres, que mostraron un nivel de estrés moderado-alto y rasgos de ansiedad y depresión en el 46%. La enfermedad del hijo tuvo un notable impacto familiar, seguido de personal, económico y social. Se desarrolló una aplicación web multicentro para el seguimiento nutricional de niños con afectación neurológica. CONCLUSIONES El estado nutricional más frecuente fue de desnutrición crónica, presentando con frecuencia trastornos de deglución. La ingesta energética fue muy variable y no se relacionó con el estado nutricional. La mitad presentaron baja DMO, empeorando a mayor afectación motora y nutricional. La enfermedad neurológica de los hijos tiene un importante impacto psicológico en sus cuidadores

Assessment of nutritional status and bone health in neurologically impaired children: a challenge in pediatric clinical practice

NTRODUCTION: neurologically impaired children frequently experience nutritional disorders and bone health complications. Our aim was firstly to analyze a method to interpret bone mineral density (BMD) accurately in neurologically impaired children. Secondly, to determine its relationship with the nutritional status and micronutrient levels in order to identify which factors are associated with low BMD. METHODS: a observational multicenter study was conducted in children with moderate-to-severe neurological impairment. Data collected included: medical records, anthropometric measures, hematologic and biochemical evaluation. BMD was measured with Dual-energy X-ray absorptiometry and z-scores were calculated adjusting for sex and chronological age. Secondly, BMD z-scores were calculated applying height age (age at which the child's height would be in 2nd percentile) instead of chronological age. RESULTS: fifty-two children were included (aged 4-16 years). Seventeen patients (32.7%) received feeding by gastrostomy tube. Height and BMI z-score were below 2SD in 64% and 31% of patients respectively, with normal mid upper arm circumference and skinfold thickness measurements. Low vitamin-D levels were found in 42% of cases. 50% of patients evidenced low BMD when calculated for chronological age, whereas only 34.5% showed BMD z-score <-2 when calculated for height age. No correlation was observed between BMD and vitamin-D levels, weight and height z-scores or age when BMD was calculated applying height age. CONCLUSIONS: the prevalence of low BMD is high in neurologically impaired children, and it is probably multifactorial. In these children, we suggest adjusting BMD for height age, in order not to over diagnose low BMD

Seguridad y eficiacia de una nueva pauta de suplementación en pacientes con fibrosis quística e insuficiencia de vitamina D

Cystic fibrosis; Vitamin D; Multicentre studyFibrosis quística; Vitamina D; Estudio multicéntricoFibrosi quística; Vitamina D; Estudi multicèntricObjectives 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.Objetivos Basándonos en los documentos de consenso europeo y americano de Fibrosis Quística (FQ) se propone un incremento de la suplementación de vitamina D (VD) en pacientes con FQ y niveles insuficientes. El objetivo de nuestro estudio fue conocer la seguridad y la eficacia de este nuevo protocolo. Material y métodos Estudio multicéntrico, experimental no aleatorizado ni controlado. A los pacientes con niveles insuficientes (<30 ng/mL) se les administró dosis crecientes de VD (entre 800 y 10000 UI/día). Se realizó seguimiento durante 12 meses analizando estatus vitamínico, nutricional, función pulmonar y metabolismo fosfo-cálcico. Análisis estadístico: pruebas t para datos apareados y regresión logística con análisis multivariable. Resultados 30 pacientes entre 1 y 39 años (mediana 9,1) completaron el estudio. Se retiraron 2 por niveles de 25 OH VD > 100 ng/mL a los 3 meses sin encontrarse signos clínicos ni analíticos de hipercalcemia. Tras 12 meses se observó un incremento de 7,6 ng/mL (IC 95% 4,6−10 ng/mL) de los niveles medios de 25 OH VD. El 37% alcanzaron niveles ≥30 ng/mL, un 13% <20 ng/mL y un 50% entre 20 y 30 ng/mL. No se observó asociación de la mejoría de los niveles de VD con la función pulmonar. Conclusiones Con el protocolo propuesto se consigue un incremento de los niveles séricos de VD y una disminución del porcentaje de pacientes con insuficiencia de la misma, aunque todavía muy lejos de alcanzar los porcentajes de suficiencia recomendados para esta entidad.This study received funding from the Fundación Ernesto Sánchez Villares (05/2015) and the Fundación Nutrición y Crecimiento

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

Cystic fibrosis; Multicenter study; Vitamin DFibrosis quística; Estudio multicéntrico; Vitamina DFibrosi quística; Estudi multicèntric; Vitamina DIntroduction: 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.This research was partially funded by Fundación Ernesto Sánchez Villares and Fundación Nutrición y Crecimiento

Predictors of Response to Exclusive Enteral Nutrition in Newly Diagnosed Crohn´s Disease in Children: PRESENCE Study from SEGHNP

Exclusive enteral nutrition (EEN) has been shown to be more effective than corticosteroids in achieving mucosal healing in children with Crohn´s disease (CD) without the adverse effects of these drugs. The aims of this study were to determine the efficacy of EEN in terms of inducing clinical remission in children newly diagnosed with CD, to describe the predictive factors of response to EEN and the need for treatment with biological agents during the first 12 months of the disease. We conducted an observational retrospective multicentre study that included paediatric patients newly diagnosed with CD between 2014–2016 who underwent EEN. Two hundred and twenty-two patients (140 males) from 35 paediatric centres were included, with a mean age at diagnosis of 11.6 ± 2.5 years. The median EEN duration was 8 weeks (IQR 6.6–8.5), and 184 of the patients (83%) achieved clinical remission (weighted paediatric Crohn’s Disease activity index [wPCDAI] 15 mg/L and ileal involvement tended to respond better to EEN. EEN administered for 6–8 weeks is effective for inducing clinical remission. Due to the high response rate in our series, EEN should be used as the first-line therapy in luminal paediatric Crohn’s disease regardless of the location of disease and disease activityS

Nutritional Outcome in Home Gastrostoy-Fed children with Chronic Diseasese

The aim of the study was to assess the anthropometric outcomes after gastrostomy tube (GT) placement in children with chronic diseases and the influence of primary diagnosis, age, and nutritional support. A longitudinal, multicenter, and prospective study was performed evaluating 65 children with GT feeding and chronic diseases (61.5% with neurological disease). Each child was evaluated three times (at baseline and at 6 and 12 months after GT placement) and the following data was collected: primary diagnosis, age at GT placement, anthropometry, and feeding regime. Repeated measures ANOVA were used to analyze the main effects (intra and intergroup) and the interactions effects on weight gain and linear growth at 6 and 12 months after GT placement. All patients significantly improved their body mass index (BMI)-for-age z-score (p < 0.001) and height-for-age z-score (p < 0.05) after 6 and 12-month of follow-up. BMI gain increased significantly the first 6 months, followed by a plateau, while height followed a linear trend. Children with GT placement before 18 months old experienced an accelerated growth rate during the first 6 months post-GT. This technique showed the effectiveness of GT placement improving nutritional status and growth catch up regardless of their primary diagnosis and the type of nutritional support

Association of Maternal Secretor Status and Human Milk Oligosaccharides With Milk Microbiota: An Observational Pilot Study

Background and Objectives: Breast milk contains several bioactive factors including oligosaccharides (HMO) and microbes that shape the infant gut microbiota. HMO profile is determined by secretor status, however their influence on milk microbiota is still uncovered. This study is aimed to determine the impact of the FUT2 genotype on the milk microbiota during the first month of lactation and the association with HMO. Methods: Milk microbiota from 25 healthy lactating women was determined by quantitative PCR and 16S gene pyrosequencing. Secretor genotype was obtained by PCR-RFLPs and by HMO identification and quantification. Results: The most abundant bacteria were Staphylococcus and Streptococcus, followed by enterobacteriaceae-related bacteria. The predominant HMO in secretor milk samples were 2FL and LNFP I whereas non-secretor milk was characterized by LNFP II and LNDFH II. Differences in microbiota composition and quantity were found depending on secretor/non-secretor status. Lactobacillus spp, Enterococcus spp., and Streptococcus spp. were lower in non-secretor than in secretor samples. Bifidobacterium genus and species were less prevalent in non-secretor samples. Despite no differences on diversity and richness, non-secretor samples had lower Actinobacteria and higher relative abundance of Enterobacteriaceae, Lactobacillaceae and Staphylococcaceae. Conclusions: Maternal secretor status is associated with the human milk microbiota composition and is maintained during the first 4 weeks. Specific associations between milk microbiota, HMO and secretor status were observed although the potential biological impact on the neonate remains elusive. Future studies are needed to reveal the early nutrition influence on the reduction of risk of disease.European Research Council (ERC) under the European Union's Horizon 2020 research and innovation program (ERC Starting Grant, 639226).Peer reviewe

Multicomponent Exercise Training Combined with Nutritional Counselling Improves Physical Function, Biochemical and Anthropometric Profiles in Obese Children: A Pilot Study

Aerobics or strength exercise plus diet interventions have been shown to counteract childhood obesity. However, little is known with regard to periodized multicomponent exercise interventions combined with nutritional counselling, which might be less demanding but more enjoyable and respectful of children and adolescents&rsquo; nature. In order to analyze the impact of such a multimodal approach, 18 obese children (10.8 &plusmn; 1.6 years; 63% females; z Body Mass Index 3 &plusmn; 0.4) trained for 60 min, twice weekly and were measured for body composition, biochemical parameters and physical function. We found that 16 weeks of multimodal intervention (14 of training), based on fun-type skill-learning physical activities and physical conditioning with challenging circuits and games, together with nutritional counselling, led to an attendance &gt; 80%, with significant overall health improvement. Body composition was enhanced (p &lt; 0.01 for z BMI, mid-upper-arm-circumference, waist-to-height ratio, tricipital and subscapular skinfolds, body-fat % by Slaughter equation and Dual energy X-ray absorptiometry body fat% and trunk fat%), as well as metabolic profile (LDL cholesterol, gamma-glutamyl transferase , alanine aminotransferase ; p &lt; 0.05), homeostatic model assessment of insulin resistance (HOMA-IR; p &lt; 0.05) and inflammatory response (C-Reactive Protein; p &lt; 0.05). Physical fitness was also improved (p &lt; 0.01) through better cardiovascular test scores and fundamental movement patterns (Functional Movement Screen-7, FMS-4). Tailoring multimodal supervised strategies ensured attendance, active participation and enjoyment, compensating for the lack of strict caloric restrictions and the low volume and training frequency compared to the exercise prescription guidelines for obesity. Nutritional counselling reinforced exercise benefits and turned the intervention into a powerful educational strategy. Teamwork and professionals&rsquo; specificity may also be key factors

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&ndash;1711 95% CI) vs 1084 (983&ndash;1184 95% CI) IU/Day (p &lt; 0.001), had higher 25 OH vitamin D levels: 30.6 (27.9&ndash;33.26 95% CI) vs. 27.4 (25.9&ndash;28.8 95% CI) ng/mL (p = 0.028), and had a lower prevalence of insufficient vitamin D levels (&lt;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&ndash;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