Phenotype assessment in neurologically impaired paediatric patients: Impact of a nutrition intervention protocol.

BACKGROUND: Deficits in nutritional status and functional feeding disorders are common in Neurologically Impaired Paediatric Patients (NIPP). Interventions addressing these problems could offer better overall health status and quality of life in this group of patients, but the extent of their effectiveness is yet to be determined. Recent guidelines concerning the nutritional care of NIPP have been published from ESPGHAN but compliance to them has not been assessed. AIM: The study aimed to assess the phenotypic profile of a group of NIPP attending the outpatient clinic of a pediatric department, and to implement, for the first time to our knowledge, an individualized nutritional intervention protocol following ESPGHAN guidelines 2017 as well as to assess the impact on phenotypic parameters and nutritional status. PATIENTS AND METHODS: 68NIPP and their caregivers aged 1m-17 years (83.8% suffering from cerebral palsy (CP) were invited to assess their phenotypic parameters and to implement in a nutrition intervention protocol in order to improve their dietary intake and nutritional status. Anthropometry (weight, height, triceps skinfold thickness, mid upper arm circumference) was expressed as z-scores for age and sex using WHO Anthro software and classified following the WHO criteria. Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), Dysphagia Disorder Survey (DDS), Saliva Severity Scale (SSS), gastrointestinal complications, energy and nutrient intake were assessed at the beginning (zero point), after 6 (point 1) and 12 (point 2) months period. Intake to Requirement ratio (I/R) was derived. At zero point, following the baseline evaluation, caregivers were advised and educated on nutrition protocol and customized nutrition plans were handed out. The impact of the nutritional intervention on the phenotypic parameters was recorded on follow up visits (points 1, 2).The primary outcomes analyzed were anthropometric parameters (Waz), as indicators of nutritional status. GMFCS, MACS, DDS, SSS, FA were evaluated as possible predictors of this outcome. Secondary outcomes included the impact of the intervention protocol on the phenotypic parameters during the study period. RESULTS: Based on weight for age z-score (Waz ≤ -2) (WHO) 17 patients (32.1%) were undernourished, 5/68 (10, 4%) were with triceps skinfold thickness z-score (TSTz) <-2 and 3/68 (7%) with mid upper arm circumference z-score (MUACz) <-2. Z-scores (WHO) for weight (p1 = 0, 036) (p2 = 0, 003), body mass index (BMI) (p2 = 0,000), MUAC (p1 = 0, 029) and TST (p1 = 0, 021) (p3 = 0, 044) were significantly improved in follow-up evaluations compared to the baseline. Less NIPP were found to be underweight according to Waz from point 1 to point 2 (p3 = 0, 006), as well as stunding according to height for age z-score (Haz) from point 1 to point 2 (p ≤ 0,001). Patients with higher levels of GMFCS (p1 = 0,040), MACS (p1 = 0,028) DDS (p1 = 0,001) and SSS (p1 = 0,005) had significantly lower Haz. Patients with higher levels of SSS (p1 = 0,002) had significantly lower TSTz scores. There were no significant changes in the classification of NIPP according to DDS or the patients' feeding ability. The energy (kcals) intake/kg of body weight (bw) was significantly higher at point 2 compared to point zero (p3 = 0,028), protein intake/kg of body weight was significantly higher at points 1 and 2 compared to point zero (p1 = 0,026, p3 = 0,003), and fat intake/kg of body weight (bw) was significantly higher at point 2 compared to point zero (p3 = 0,012). Intake of energy (kcals)/bw (p1 = 0,026), (p2 = 0,046), (p3 = 0,048) carbs/bw (p1 = 0,014) (p2 = 0,042), I/R of pro (p1 = 0,032), (p3 = 0,013), and fat/kg (p2 = 0, 033) (p3 = 0,037) were found to be significantly lower in higher GMFCS levels. DQI did not improve during the study period nor correlated to any of the anthropometric parameters. Gastrointestinal complications correlated with Waz (r = -, 285 p1 = 0, 011). Feeding Ability (FA) was found to be the only strong predictor for Waz at baseline evaluation (p = 0,012) when a multiple regression was run along with DDS. CONCLUSION: Underweight was detected in one third of the patients, some degree of dysphagia in 69% and gastrointestinal complications in 58.8% of the sample. Height for age z-score (Haz) was the anthropometric parameter most sensitive to the changes in ranking on motor and functional feeding scores. The implementation of a customized nutrition intervention protocol in line with ESPGHAN's guidelines had a beneficial effect on improving dietary intake and nutritional status of NIPP after a 12 months period. Better results could be expected if dysphagia and feeding ability were also addressed by appropriate intervention protocols. Patients' feeding ability is of importance for predicting Waz.

Leucine Supplementation Increases Muscle Strength and Volume, Reduces Inflammation, and Affects Wellbeing in Adults and Adolescents with Cerebral Palsy.

BACKGROUND: Spastic cerebral palsy (CP) is characterized by muscle weakness owing, in part, to a blunted muscle protein synthetic response. This might be normalized by long-term leucine supplementation. OBJECTIVES: The study assessed the effects of 10 wk leucine supplementation in adolescents and adults with CP. METHODS: The study was a single-center randomized controlled trial. Twenty-four participants were randomly assigned to a control group (n = 12) or a leucine group (n = 12). l-Leucine (192 mg/kg body mass) was dissolved in water and administered daily for 10 wk. The primary outcome measures of elbow flexor muscle strength and muscle volume (measured by 3D ultrasound technique) and inflammation [C-reactive protein (CRP) concentration] were assessed before and after the 10 wk, alongside the secondary outcomes of body composition (measured by CP-specific skinfold assessment), metabolic rate (measured by indirect calorimetry), and wellbeing (measured by a self-reported daily questionnaire). Data were compared via a series of 2-factor mixed ANOVAs. RESULTS: Twenty-one participants completed the intervention (control group: n = 11, mean ± SD age: 18.3 ± 2.8 y, body mass: 48.8 ± 11.9 kg, 45% male; leucine group: n = 10, age: 18.6 ± 1.7 y, body mass: 58.3 ± 20.2 kg, 70% male). After 10 wk, there was a 25.4% increase in strength (P = 0.019) and a 3.6% increase in muscle volume (P = 0.001) in the leucine group, with no changes in the control group. This was accompanied by a 59.1% reduction in CRP (P = 0.045) and improved perceptions of wellbeing (P = 0.006) in the leucine group. No changes in metabolism or body composition were observed in either group (P > 0.05). CONCLUSIONS: Improvements in muscle strength and volume with leucine supplementation might provide important functional changes for adults and adolescents with CP and could be partly explained by reduced inflammation. The improved wellbeing highlights its capacity to improve the quality of daily living. This trial was registered at clinicaltrials.gov as NCT03668548.

Bone mineral density and biochemical and hormonal indicators in children with quadriplegic cerebral palsy / Densidad mineral ósea e indicadores bioquímicos y hormonales en niños con parálisis cerebral cuadripléjica

Introduction: children with cerebral palsy (CP) have multiple risk factors for low bone mineral density or osteoporosis. Objective: to explore the association between bone mineral density (BMD) and biochemical and hormonal indicators of bone metabolism in children with quadriplegic cerebral palsy (CP). Methods: a cross-sectional analytical study included 59 participants from six to 18 years of age with quadriplegic CP. Serum concentrations of calcium, phosphorus, 25OHD metabolite, parathyroid hormone (PTH), alkaline phosphatase, and thyroid hormones were determined using standardized methods. The BMD measurement was obtained from the lumbar spine expressed in g/cm2 and Z-score. Unpaired Student's t-test, Chi-square test, odds ratio and Pearson's correlation were performed. Results: participants with CP and malnutrition had lower serum concentrations of calcium, phosphorus and alkaline phosphatase. Those who had low BMD showed lower serum concentrations of calcium, phosphorus and alkaline phosphatase. Most participants with low and normal BMD had vitamin D deficiency (27.1% and 10%) and insufficiency (35.4% and 30%), respectively. There was a significant correlation between BMD and serum concentrations of calcium, phosphorus, alkaline phosphatase, vitamin D and thyroid-stimulating hormone (TSH). There were no differences in the biochemical and hormonal indicators by level of gross motor function, use of anticonvulsants and oral versus enteral feeding method. Conclusion: malnutrition and alteration of vitamin D nutritional status were associated with low BMD and alterations of biochemical indicators of bone metabolism in pediatric patients with quadriplegic CP. The relationship between BMD and biochemical indicators of bone metabolism in children with quadriplegic CP was also demonstrated

Introducción: los niños con parálisis cerebral (PC) presentan múltiples factores de riesgo de densidad mineral ósea baja u osteoporosis. Objetivo: explorar la asociación entre la baja densidad mineral ósea (DMO) e indicadores bioquímicos y hormonales del metabolismo óseo en niños con PC cuadripléjica. Métodos: un estudio transversal analítico incluyó a 59 participantes de entre seis y 18 años de edad con PC cuadripléjica. Las concentraciones séricas de calcio, fósforo, metabolito 25OHD, hormona paratiroidea (PTH), fosfatasa alcalina y hormonas tiroideas se determinaron utilizando métodos estandarizados. La medición de DMO se obtuvo de la columna lumbar expresada en g/cm2 y puntaje Z. Se realizaron pruebas t de Student no pareada, Chi-cuadrado, razón de momios y correlación de Pearson. Resultados: los participantes con PC y desnutrición tenían concentraciones séricas más bajas de calcio, fósforo y fosfatasa alcalina. Los participantes con DMO baja tuvieron menor concentración sérica de calcio, fósforo y fosfatasa alcalina. Los participantes con DMO baja y normal tenían deficiencia de vitamina D (27.1% y 10%) e insuficiencia (35.4% y 30%), respectivamente. Hubo una correlación significativa entre DMO y las concentraciones séricas de calcio, fósforo, fosfatasa alcalina, vitamina D y hormona estimulante de la tiroides. Conclusión: la desnutrición y la alteración del estado nutricio de la vitamina D se asociaron con DMO baja y alteraciones de los indicadores bioquímicos del metabolismo óseo. Se demostró una asociación entre DMO e indicadores bioquímicos y hormonales del metabolismo óseo en niños con PC cuadripléjica

Nutritional Status and Cardiometabolic Risk Factors in Institutionalized Adults with Cerebral Palsy.

Background and Objectives: Cerebral palsy (CP) is a set of permanent disorders that limit physical activity and increase the risk of developing other diseases, such as metabolic syndrome (MS). Adequate nutrition can contribute to the prevention of associated symptoms. The main objective of this study is to evaluate the nutritional status and the prevalence of cardiometabolic risk factors in adults with CP and Gross Motor Function Classification System (GMFCS) levels between IV and V. Materials and Methods: A sample of 41 adults with CP and GMFCS levels from IV to V were studied. The variables used in the study were age, sex, weight, height, mean age, and GMFCS level range. To evaluate nutritional status, body mass index and the Mini Nutritional Assessment (MNA), a nutritional screening tool, were used. To assess cardiometabolic risk, data on obesity, central obesity, blood pressure, fasting plasma glucose, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, and triglycerides were collected. Results: More than 80% of the population studied was malnourished or at risk of malnutrition, according to the MNA tool classification ranges, and around 35% of the studied population was within the underweight range. Regarding cardiometabolic risk factors, only one adult with CP was diagnosed with MS. Conclusions: The studied population of adults with CP and GMFCS levels between IV and V is not a population at risk of MS; however, the high prevalence of malnutrition, as well as some of the most prevalent cardiovascular risk factors, should be taken into consideration.

Does l-Tryptophan supplementation reduce chewing deficits in an experimental model of cerebral palsy?

Children with cerebral palsy commonly present with feeding difficulties that result from multiple orofacial sequelae, especially deficits in mastication. A previous study demonstrated that perinatal protein undernutrition accentuated the chewing impact in an experimental model of cerebral palsy. Therefore, the present study investigated whether nutritional manipulation reversed or minimized the chewing sequelae in cerebral palsy. We emphasized the relevance of evaluating the therapeutic potential of nutrients, especially tryptophan supplementation, to reduce the chewing deficits that are typical of this syndrome. Clarification of the role of nutrients may help in the development of new treatment strategies for these children.

Terapia oromotora y suplemento dietético, mejora en las habilidades de alimentación y en la nutrición de pacientes con parálisis cerebra / Oromotortherapy and dietary supplement, improve feeding skills and nutrition of patients with cerebral palsy

Fundamentos: La parálisis cerebral (PC)compromete los procesos de alimentación y el estado nutricional de los niños que la padecen. El objetivo de este estudio fue evaluar los resultados nutricionales y las habilidades de alimentación de niños con PC trasterapia oromotora y suplemento dietético. Métodos: Estudio de cohorte, longitudinal, prospectivo, cuasi experimental. Participaron 80 pacientes con PC, que se dividieron en 2 grupos (tratado en una institución y en su hogar) y tuvo una duración de 3 años 9 meses. Los participantes recibieron una evaluación nutricional al inicio y al término del estudio, todos recibieron dieta hipercalórica, terapia oromotora y suplemento dietético. Resultados: El mayor porcentaje de mejora se obtuvo en los casos clasificados con desnutrición moderada (GTI: 42,5% vs GTH: 30 %). En los pacientes GTH, la nutrición normal aumentó un 12,6 %.En los pacientes GTI, la desnutrición leve disminuyó un 15 %, la moderada 27,6 %; y la nutrición normal aumentó un 40%. Las habilidades de succión, masticación y deglución severas disminuyeron un 22,9%. Conclusiones: La terapia oromotora, dieta hipercalórica e hiperproteica y la suplementación dietética, son altamente eficaces para mejorar el estado nutricional de los niños con PC, siendo más efectiva cuando se aplica en una institución especializada

Background: Cerebral palsy (PC) compromises the feeding processes and the nutritional status of the children who suffer from it. The objective of this study was to evaluate the nutritional results and feeding abilities of children with CP after oral-motor therapy and dietary supplement. Methods: Longitudinal, prospective and quasi-experimental cohort study. 80 patients with PC, who were divided into 2 groups (treated in an institution and in their home), were included in this study thatlasted 3 years 9 months. Participants received a nutritional evaluation at the beginning and at the end of the study, all received a high calorie diet, oral-motor therapy and dietary supplement. Results: The highest percentage of improvement was obtained in the cases classified as moderate malnutrition (GTI: 42.5% vs. GTH: 30%). In GTH patients, normal nutrition increased by 12.6%. In GTI patients, mild malnutrition decreased 15%, moderate27.6%; and normal nutrition increased40%. Severe sucking, chewing and swallowing skills decreased22.9%. Conclusions: Oral motor therapy combined with a high calorie and high protein diet, and a dietary supplement, is highly effective in improving nutritional status of children with CP, and is most effective when applied in a specialized institution by professionals

Assessment of anthropometric indicators in children with cerebral palsy according to the type of motor dysfunction and reference standard / Evaluación de indicadores antropométricos en niños con parálisis cerebral de acuerdo con el tipo de disfunción motora y estándar de referencia

Aim: The study aimed to demonstrate that the assessment of the anthropomorphic measurements of children with cerebral palsy (CP) varies according to the type of motor dysfunction and references standard used for comparison. Method: In a cross-sectional design, 108 children 2 to 16 years were classified according to the type of motor dysfunction by gender and age group. Weight, mid-upper-arm-circumference (MUAC), and alternative measures for height were performed. Height/age and weight/age indexes and BMI were evaluated with percentiles and/or Z-scores with reference to a number of previously published references of growth, including those of the World Health Organization (WHO). Results: Fifty-three (49.1%) were females and 55 (50.9%) males. Spastic type was predominant (73.1%) and 26.9% were other types of dysfunction. Most of the children were located on level IV (14.6%) and level V (73.1%) of the Gross Motor Function Classification System (GMFCS). Significant differences were found, suggesting that weight (p = 0.002), height (p = 0.001), and MUAC (p = 0.05) are higher in the spastic group than in other groups. Conclusions: The anthropometric indicators were significantly higher in the spastic group than in other groups. Upper-arm length (UAL) seemed less appropriate than knee height (KH) and lower-leg length (LLL) for measuring height. The WHO reference standard was not useful to evaluate the majority of anthropometric indexes in children with CP, other references as the growth charts of Day and Brooks have been more suitable (AU)

Objetivo: demostrar que la evaluación de las mediciones antropomórficas de los niños con parálisis cerebral (PC) varía según el tipo de disfunción motora y la referencia estándar utilizada. Método: en un diseño transversal se incluyeron 108 niños de 2 a 16 años clasificados de acuerdo con el tipo de disfunción motora por sexo y grupos de edad. Se obtuvieron el peso, circunferencia media de brazo y mediciones alternas para la talla. Los índices talla/edad, peso/edad y el IMC fueron evaluados con los percentiles y/o puntuaciones Z con referencia a estándares de crecimiento previamente publicados, incluyendo los de la Organización Mundial de la Salud (OMS). Resultados: cincuenta y tres (49.1%) eran mujeres y 55 (50.9 %) hombres. Predominó la PC tipo espástico (73.1%) y 26.9% otros tipos de disfunción. La mayoría de los niños se encontraron en el nivel IV (14.6%) y en el nivel V (73.1 %) de la Gross Motor Function Classification System (GMFCS). Se encontraron diferencias significativas, lo que sugiere que el peso (p = 0,002), talla (p = 0,001), y la circunferencia media del brazo CMB (p = 0,05) son mayores en el grupo espástico que en otros grupos. Conclusiones: los indicadores antropométricos fueron mayores en el grupo espástico. La longitud del brazo pareció menos apropiada que la altura de la rodilla y la longitud de la pierna para la medición de la talla. El estándar de crecimiento de la OMS no resultó útil para evaluar los índices antropométricos en niños con parálisis cerebral; otras referencias como las tablas de crecimiento de Day y Brooks fueron más adecuadas (AU)

Nutritional assessment and intervention in children with cerebral palsy: a practical approach.

Cerebral palsy (CP) is associated with the presence of feeding disorders in almost 60% of the affected children with subsequent undernutrition reported in up to 46% of the subjects. Since undernutrition may have a detrimental impact on physical and cognitive development, the introduction of an adequate nutritional support should always be considered in children with neurological impairment. The aim of the present review is to provide a practical guide to the assessment of nutritional status in children with CP, in order to identify individuals at risk for malnutrition that need the introduction of an adequate and personalized nutritional support. This review summarizes the methods for the evaluation of oral-motor function, anthropometric parameters, body composition and energy balance in children with CP. Moreover, we reviewed the indications for the introduction of nutritional support, and the suggested modalities of intervention.

Dietary Intakes and Nutritional Issues in Neurologically Impaired Children.

Neurologically impaired (NI) children are at increased risk of malnutrition due to several nutritional and non-nutritional factors. Among the nutritional factors, insufficient dietary intake as a consequence of feeding difficulties is one of the main issues. Feeding problems are frequently secondary to oropharyngeal dysphagia, which usually correlates with the severity of motor impairment and presents in around 90% of preschool children with cerebral palsy (CP) during the first year of life. Other nutritional factors are represented by excessive nutrient losses, often subsequent to gastroesophageal reflux and altered energy metabolism. Among the non-nutritional factors, the type and severity of neurological impairment, ambulatory status, the degree of cognitive impairment, and use of entiepileptic medication altogether concur to determination of nutritional status. With the present review, the current literature is discussed and a practical approach for nutritional assessment in NI children is proposed. Early identification and intervention of nutritional issues of NI children with a multidisciplinary approach is crucial to improve the overall health and quality of life of these complex children.

Optimising nutrition to improve growth and reduce neurodisabilities in neonates at risk of neurological impairment, and children with suspected or confirmed cerebral palsy.

BACKGROUND: Neurological impairment is a common sequelae of perinatal brain injury. Plasticity of the developing brain is due to a rich substrate of developing neurones, synaptic elements and extracellular matrix. Interventions supporting this inherent capacity for plasticity may improve the developmental outcome of infants following brain injury. Nutritional supplementation with combination docosahexaenoic acid, uridine and choline has been shown to increase synaptic elements, dendritic density and neurotransmitter release in rodents, improving performance on cognitive tests. It remains elusive whether such specific 'neurotrophic' supplementation enhances brain plasticity and repair after perinatal brain injury. METHODS/DESIGN: This is a two year double-blind, randomised placebo controlled study with two cohorts to investigate whether nutritional intervention with a neurotrophic dietary supplement improves growth and neurodevelopmental outcomes in neonates at significant risk of neurological impairment (the D1 cohort), and infants with suspected or confirmed cerebral palsy (the D2 cohort). 120 children will be randomised to receive dietetic and nutritional intervention, and either active supplement or placebo. Eligible D1 neonates are those born <30(+6) weeks gestation with weight <9(th) centile, ≤ 30(+6) weeks gestation and Grade II, III or IV Intra-Ventricular Haemorrhage or periventricular white matter injury, or those born at 31-40(+28) weeks gestation, with Sarnat grade I or II or III Hypoxic Ischaemic Encephalopathy or neuroimaging changes compatible with perinatal brain injury. Eligible D2 infants are those aged 1-18 months with a suspected or confirmed clinical diagnosis of cerebral palsy. The primary outcome measure is composite cognitive score on the Bayley Scales of Infant and Toddler Development III at 24 months. Secondary outcomes include visuobehavioural and visual neurophysiological assessments, and growth parameters including weight, height, and head circumference. DISCUSSION: This is the first study to supplement neonates and infants with perinatal brain injury with the combination of factors required for healthy brain development, throughout the period of maximal brain growth. A further study strength is the comprehensive range of outcome measures employed. If beneficial, supplementation with brain phosphatide precursors could improve the quality of life of thousands of children with perinatal brain injury. TRIAL REGISTRATION: Current Controlled trials: ISRCTN39264076 (registration assigned 09/11/2012), ISRCTN15239951 (registration assigned 23/04/2010).

Enfermedades neurológicas en pediatría y su transición al experto en nutrición de adultos; ¿cómo hacerlo? / Transition to adult care for children with chronic neurological disorders; which is the best way to make it?

Las enfermedades neurológicas crónicas en los niños tienen gran repercusión en su vida y en su salud como adultos. La transición desde las consultas pediátricas a los servicios de adultos es un proceso complejo, y no existen datos objetivos sobre cuál es la aproximación más efectiva. El modelo, sin embargo, preferido es el de habilitar una consulta de transición con especialistas pediátricos y de adultos. La malnutrición, tanto desnutrición como sobrepeso, es frecuente entre los niños con enfermedad neurológica. La desnutrición es más prevalente y sus causas son múltiples: ingesta insuficiente, pérdidas aumentadas y un metabolismo energético alterado. La desnutrición se asocia a un aumento de morbilidad, mientras que la recuperación nutricional se asocia a una mejoría general del estado de salud y de su calidad de vida. No es sencillo determinar cuáles son las necesidades nutricionales en estos enfermos. A esto se asocian las dificultades para la alimentación oral, entra las que la disfunción oral motora es una de las más importantes. Los síntomas digestivos, reflujo gastroesofágico y estreñimiento, junto con la espasticidad, las deformidades posturales y la escoliosis contribuyen a esa dificultad. Por todas estas razones es necesario hacer valoraciones nutricionales periódicas y evaluar la seguridad y la eficacia de la alimentación oral. Si con las modificaciones de la dieta oral no es posible garantizar un aporte nutricional suficiente, recurriremos al empleo de sondas nasogástricas, pero sobre todo a la gastrostomía endoscópica. En muchas ocasiones esta técnica se asocia a una técnica antirreflujo. Las repercusiones sobre el estado nutricional y la calidad de vida en la mayoría de estos pacientes es evidente, aunque no es una decisión sencilla para las familias (AU)

Chronic neurological disorders in children have significant effects on adult medical and social function. Transition from pediatric to adult services is a complex process. No objective data are available to inform physicians about the most effective approach. Nevertheless the most recommended approach is a joint pediatric/adult transition clinic. Malnutrition, either under or overnutrition, is a common condition among neurologically impaired children.Undernutrition is most prevalent, and its causes are diverse: insufficient caloric intake, excessive nutrient losses and abnormal energy metabolism. Malnutrition is associated with significant morbidity, while nutritional rehabilitation improves overall health as well as quality of life. It is not easy to determine which the nutritional needs in these patients are. Besides, they often present difficulties for oral feeding, mainly due to oromotor dysfunction. Gastrointestinal symptoms, gastro esophageal reflux and constipation, as well as spasticity, scoliosis and joint deformities contribute to these difficulties. Because of that, an assessment of nutritional status should be performed periodically, and to assess efficacy and security of oral intake. If modifying oral diet we cannot confirm an adequate support, a nasogastric tube or a gastrostomy need to be considered. Often, a fundoplication is associated to the placement of a gastrostomy. Although the outcomes in a better nutritional status and quality of life are often obtained, it is not an easy decision for families (AU)

Psoriasis first presenting around an enteral feeding tube in three pediatric patients: an important consideration for timely diagnosis and management.

Pediatric dermatologists may care for patients with percutaneous enteral feeding tubes. Although ostomy complications such as allergic contact and irritant dermatitis are common, psoriasis may be misdiagnosed. We report three novel cases of childhood psoriasis first presenting around an enteral feeding tube site. Localized psoriasis is an important clinical consideration in children with ostomy site eruptions to ensure timely diagnosis and effective management.

An exploratory study of sodium, potassium, and fluid nutrition status of tube-fed nonambulatory children with severe cerebral palsy.

Children with severe cerebral palsy (CP) often have lower mineral intakes than healthy children. It is unknown if their lower nutrient intakes are adequate to meet their needs. The objective of this study was to examine the sodium, potassium, phosphate, and fluid status of primarily tube-fed nonambulatory children with severe CP. The design consisted of a cross-sectional exploratory study and a clinical trial of sodium supplementation. Nutritional status was determined among primarily tube-fed children (aged 2 to 17 years) with CP based on blood and urine samples, anthropometry, and 3-day food records. Mineral and fluid status was evaluated by a nephrologist blind to nutrient intakes. Twenty children supplied food records, blood samples, and anthropometric measurements, and 16 supplied urine samples. Six (37.5%) of those who provided urine samples were considered possibly dehydrated, as urine osmolality was >600 mmol·kg(-1). Six (60%) of the 10 children with satisfactory fluid status (low urine osmolality) were considered to have a possible dietary sodium deficiency based on a very low urine sodium concentration (<20 mmol·L(-1)). Those considered to have a possible dietary sodium deficiency had a significantly lower sodium intake (48% ± 15% Adequate Intake (AI)) compared with those considered sodium sufficient (73% ± 20% AI) (p = 0.031). One child was considered possibly phosphorus deficient, but none was assessed as likely potassium deficient. The conclusion was that sodium deficiencies were likely prevalent among the children. The findings from this small observational study suggest that sodium intakes for tube-fed children with CP should be maintained near the AI for their age. Hydration status of children receiving hypercaloric formulas should be monitored.

Nutritional rehabilitation increases the resting energy expenditure of malnourished children with severe cerebral palsy.

AIM: The aim of this study was to measure resting energy expenditure (REE) and energy intake in children with quadriplegic cerebral palsy (CP), to relate these to anthropometric measurements, and to determine the influence of nutritional rehabilitation on REE. METHODS: Fifty-six children (20 females, 36 males; age range 3y 11mo-18y; mean age 10y; SD 3y 11mo) with CP (Gross Motor Function Classification System level V) participated in this cross-sectional study. Children were excluded if they had a known metabolic disorder, genetic syndrome, or chromosomal abnormality. Thirty-three of the children were tube fed and 23 were fed orally. A comparison group comprised 111 (42 females, 69 males) healthy children who had undergone anthropometric and REE measurements and were of similar age to the children with CP (4-19y). REE was measured by indirect calorimetry and energy intake was determined from weighed food records. RESULTS: The REE in the children with CP was low (79.5%) compared with that predicted and highly variable (SD 38.4%). Fat-free mass was the strongest predictor of REE, accounting for 27% of the variation. Energy intake as a percentage of REE in was greatly overestimated in oral-fed children with CP (293%). In a subset of children with CP (n=14), an increased energy intake by gastrostomy tube feeding resulted in an increase in REE from 70.0% to 101.9% of that predicted. INTERPRETATION: The REE of children with CP is low and variable and is not strongly related to any one anthropometric measurement. Food records in oral-fed children with CP are of little value owing to their inaccuracy. This study provided support for the hypothesis that the low REE found in malnourished children with CP is partly due to a low energy intake.

Hypocaloric considerations in patients with potentially hypometabolic disease States.

The provision of nutrition has traditionally been driven by the desire to provide adequate calories. However, over the past decade it has become evident that provision of excess calories can be detrimental to patients' outcomes in both critical care and long-term care settings. This review examines patient populations for whom hypocaloric nutrition can be both appropriate and beneficial. In specific situations, critically ill patients, such as those with obesity, stroke, and spinal cord injury, may have decreased energy requirements. In patients with spinal cord injury, the level of injury significantly correlates with the extent of reduced caloric energy expenditure. Ventilator-dependent patients with amyotrophic lateral sclerosis (ALS) have decreased energy needs; energy expenditure for ALS patients is lower than the predictive equation value. Aging patients will have decreased energy needs relative to a reduction in lean body mass. Patients with cerebral palsy (CP) have significantly lower caloric requirements than anticipated using predictive equations. Patients with CP pose a particular challenge in nutrition assessment. Several studies demonstrate that patients with CP have significantly lower caloric requirements than anticipated using predictive equations; thus, patients with CP benefit from indirect calorimetry. Provision of nutrition for obese patients is briefly addressed, as this is an increasingly important consideration for nutrition support in both the critical care and long-term care settings. When indirect calorimetry is not available, clinicians should remember that most patients will have low resting energy expenditure regardless of functional status and require frequent evaluation during institution of nutrition recommendations to guard against overfeeding and obesity.

Growth in cerebral palsy.

Cerebral palsy is often accompanied by abnormalities of growth and nutrition; children with severe motor impairments are most at risk. Nutrition, neurological, and endocrine factors all contribute to suboptimal growth. Poor growth and nutrition are associated with poor general health outcomes and reduced levels of participation, and therefore warrant careful evaluation and appropriate intervention. The lack of normative data combined with the complex interaction of nutrition and nonnutrition factors contributing to growth in this population present real difficulties in management. Particular care is needed to avoid overfeeding and the resultant increase in fat mass and associated morbidity.

La alimentación del niño con parálisis cerebral un reto para el nutricionista dietista: perspectivas desde una revisión / Nutrition for children having cerebral paralysis is challenging for dietitians: review

Antecedentes: la parálisis cerebral es un término usado para describir el síndrome producido por lesión o daño del sistema nervioso central durante períodos críticos del desarrollo. Los niños con parálisis cerebral presentan diferentes manifestaciones clínicas, pero todos presentan deterioro de la función neurológica. Esta enfermedad, considerada altamente incapacitante, presenta una incidencia de 2,5 por cada mil nacidos vivos en países en desarrollo y de 2,0 en países desarrollados. Objetivo: identificar características alimentarias y nutricionales de los niños con parálisis cerebral para orientar a los profesionales de la salud con recomendaciones que contribuyan a la intervención efectiva de este grupo. Materiales y métodos: se realizó una búsqueda sistemática de artículos publicados en los últimos 10 años en bases de datos nacionales e internacionales. Resultados: la parálisis cerebral afecta el desarrollo de los diferentes sistemas reguladores de las funciones vitales del organismo: succión, masticación, deglución y respiración, que comprometen los procesos de alimentación y el estado nutricional de los niños que la padecen.Conclusiones: la atención alimentaria y nutricional de niños con parálisis cerebral, requiere que el nutricionista dietista, de forma interdisciplinaria, desarrolle modificaciones dietéticas acordes a las características del paciente para favorecer el consumo de alimentos y mejorar su estado nutricional.

Cerebral paralysis defined the syndrome for damage of nervous system, it could happen during critical periods of child development. Children having cerebral paralysis present different clinical manifestations, but all present damage of neurological function. Cerebral paralysis is an extremely disable disease, the world incidence is 2,5 per 1000 newborns in developing countries and 2,0 per 1000 newborns in developed nations. Objective: to identify feeding and nutritional characteristics in children having cerebral paralysis in order to develop guidelines for health professionals working with these children. Methods: a systematic search of articles published the last 10 years related to the topic was done, looking in scientific national and international data base. Results: cerebral paralysis affects physiological functions like suction, mastication, swallowing and breading system, that affect feeding process and nutritional status of children. Conclusion: the main purpose of this review was to understand the impact of cerebral paralysis on nutritional status in children having this syndrome, also this review provides information about recommendations that health professionals should apply to improve the quality of life for this population.

Nutrición del niño con enfermedades neurológicas prevalentes / Nutrition of children with selected neurological illnesses

In this paper, some neurological disorders are reviewed where nutritional management is relevant. These are cerebral palsy, epilepsy, autism and attention déficit disorder (ADHD). Nutrition is affected in children with Cerebral Palsy due to the illness, difficult in ingesting foods due to sensory, motor and/or GI disorders, and specific nutritional déficits secondary to insufficient intake. In epilepsy and ADHD, interaction with medications must be added. In autism there is additional controversy regarding nutritional management. Nutritional status will depend on the severity of the main disorder, time of evolution, adequate care. Nutritional vigilance by the multidisciplinbary treatment team. Good management contributes to improve the evolution of the illness, diminish medication adverse effect, prevent specific deficiencies, development of excess disorders (as in obesity), and non nutritional complications (such as infections).

En este artículo se revisan algunas enfermedades neurológicas en donde el manejo nutricional tiene un rol relevante, como son la parálisis cerebral, epilepsia, autismo y síndrome de déficit atencional (SDAH). La nutrición frecuentemente es afectada en niños con parálisis cerebral por factores como la enfermedad de base, la dificultad en ingerir alimentos por alteraciones sensoriales, motoras y/o gastrointestinales y las deficiencias nutricionales específicas relacionadas con la ingesta insuficiente lograda; se agregan para epilepsia y SDAH las interacciones fármaco-nutriente. En autismo hay controversias que involucran factores relacionados a la alimentación en su manejo. El estado nutricional del paciente dependerá de la severidad de su enfermedad de base, tiempo de evolución, adecuado cuidado y de la vigilancia nutricional por parte del equipo multidisciplinario tratante. Un buen manejo metabólico nutricional contribuye a mejorar la evolución de la enfermedad neurológica de base, a disminuir los efectos adversos de la medicaciones específicas, a prevenir carencias específicas de nutrientes, el desarrollo de enfermedades por exceso (obesidad) y complicaciones no nutricionales (ej infecciosas).

Avaliação dos hábitos alimentares de interesse odontológico em crianças com paralisia cerebral / Assessment of dietary habits of dental interest in children with cerebral palsy

Introdução - As dificuldades na mastigação e deglutição em crianças com Paralisia Cerebral (PC) são identificadas como fatores de risco para hábitos alimentares inadequados podendo estar associada a presença da doença cárie. O objetivo do estudo foi avaliar os hábitos alimentares de interesse odontológico em crianças com PC em comparação a crianças normoreativas (grupo controle). Material e Métodos - Participaram do estudo 60 crianças pareadas pela idade, 30 com diagnóstico médico de PC e 30 normoreativas. Foram incluídas crianças de ambos os gêneros, na faixa etária de 5 a 11 anos de idade (média = 7,45). Avaliou-se a presença visível de biofilme nos dentes anteriores, e um questionário foi aplicado ao responsável referente à preferência da consistência dos alimentos, necessidade de auxilio na alimentação, utensílio utilizado para alimentação e o conteúdo da dieta. Um diário alimentar foi entregue ao responsável, para ser preenchido durante 5 dias. A avaliação clínica visual da presença de biofilme e a análise dos diários foram realizadas por um único examinador. Resultados - As crianças com PC apresentaram uma frequência significantemente mais alta de alimentos líquidos ou pastosos, assim como maior uso de colher, maior necessidade de auxilio durante a alimentação e maiores índices de biofilme, em comparação ao grupo controle (p < 0,01). A frequência de ingestão de sacarose foi mais alta em crianças com PC, mas sem associação à presença de biofilme dentário. Conclusões - As crianças com PC são mais dependentes no momento da alimentação, possuem uma dieta de consistência mais líquida a pastosa e apresentam maior quantidade de biofilme dentário em comparação com as crianças normoreativas.

Introduction - The difficulties in chewing and swallowing in children with Cerebral Palsy (CP) are possible risk factors for an inadequate nutritional state. The objective of the study was to evaluate the dietary habits of dental interest in children with CP compared to normoreactive children(control group). Material and Methods - Sixty children matched by age participated; 30 with medical diagnosis of CP and 30 normoreactive. Children of both genders were included, aged 5 to 11years (mean = 7.45). The presence of biofilm in anterior teeth was evaluated, and a questionnaire was applied to the guardian regarding the consistency of food, need for assistance during feeding, utensil used for feeding and content of the diet. A food diary was given to the guardian to be completed for 5 days. Visual clinical evaluation of the presence of biofilm and the food diary analysis were performed by a single examiner. Results - Children with CP had a significantly higher frequency of liquid or paste foods, as well as a greater use of spoon, greater need for help during feeding and higher rates of biofilm compared to the control group (p < 0.01). The frequency of sucrose intake was higher in children with CP, but no association with the presence of dental biofilm was found. Conclusions - Children with CP are more dependent during feeding, they have a diet of liquid to paste consistency and they have a greater amount of dental biofilm compared to normoreactive children.