Nutritional Outcome in Home Gastrostomy-Fed Children with Chronic Diseases.

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.

Birth Weight, Postnatal Weight Gain, and Childhood Adiposity in Relation to Lipid Profile and Blood Pressure During Early Adolescence.

BACKGROUND: Different pathways likely underlie the association between early weight gain and cardiovascular disease risk. We examined whether birth weight for length relationship and weight gain up to 2 years of age are associated with lipid profiles and blood pressure (BP) in early adolescence and determined whether childhood adiposity mediates these associations. METHODS AND RESULTS: Data from QUALITY (Quebec Adipose and Lifestyle Investigation in Youth), a cohort of white children with parental history of obesity, were analyzed (n=395). Sex-specific weight for length z scores from birth to 2 years were computed. Rate of postnatal weight gain was estimated using individual slopes of weight for length z-score measurements. Percentage of body fat was measured at 8 to 10 years. Fasting lipids and BP were measured at 10 to 12 years. Using path analysis, we found indirect effects of postnatal weight gain, through childhood adiposity, on all outcomes: Rate of postnatal weight for length gain was positively associated with childhood adiposity, which in turn was associated with unfavorable lipid and BP levels in early adolescence. In contrast, small beneficial direct effects on diastolic BP z scores, independent of weight at other time points, were found for birth weight for length (ß=-0.05, 95% CI, -0.09 to -0.002) and for postnatal weight gain (ß=-0.02, 95% CI, -0.03 to -0.002). CONCLUSIONS: Among children with at least 1 obese parent, faster postnatal weight gain leads to cardiovascular risk factors in early adolescence through its effect on childhood adiposity. Although heavier newborns may have lower BP in early adolescence, this protective direct effect could be offset by a deleterious indirect effect linking birth weight to later adiposity.

Pediatric parenteral nutrition: clinical practice guidelines from the Spanish Society of Parenteral and Enteral Nutrition (SENPE), the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH) / Guía de práctica clínica SENPE/SEGHNP/SEFH sobre nutrición parenteral pediátrica.

Introduction:Parenteral nutrition (PN) in childhood is a treatment whose characteristics are highly variable depending on the age and pathology of the patient. Material and methods: The Standardization and Protocols Group of the Spanish Society for Parenteral and Enteral Nutrition (SENPE) is an interdisciplinary group formed by members of the SENPE, the Spanish Society of Gastroenterology, Hepatology and Pediatric Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH) that intends to update this issue. For this, a detailed review of the literature has been carried out, looking for the evidences that allow us to elaborate a Clinical Practice Guide following the criteria of the Oxford Center for Evidence-Based Medicine. Results: This manuscript summarizes the recommendations regarding indications, access routes, requirements, modifi cations in special situations, components of the mixtures, prescription and standardization, preparation, administration, monitoring, complications and home NP. The complete document is published as a monographic number. Conclusions: This guide is intended to support the prescription of pediatric PN. It provides the basis for rational decisions in the context of the existing evidence. No guidelines can take into account all of the often compelling individual clinical circumstances.

Introducción: la nutrición parenteral (NP) en la infancia es un tratamiento cuyas características son muy variables en función de la edad y la patología que presente el paciente. Material y métodos: el grupo de Estandarización y Protocolos de la Sociedad Española de Nutrición Parenteral y Enteral (SENPE) es un grupo interdisciplinar formado por miembros de la SENPE, Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP) y Sociedad Española de Farmacia Hospitalaria (SEFH) que pretende poner al día este tema. Para ello, se ha realizado una revisión pormenorizada de la literatura buscando las evidencias que nos permiten elaborar una Guía de Práctica Clínica siguiendo los criterios del Oxford Centre for Evidence-Based Medicine. Resultados: este manuscrito expone de forma resumida las recomendaciones en cuanto a indicaciones, vías de acceso, requerimientos, modificaciones en situaciones especiales, componentes de las mezclas, prescripción y estandarización, preparación, administración, monitorización, complicaciones y NP domiciliaria. El documento completo se publica como número monográfico. Conclusiones: esta guía pretende servir de apoyo para la prescripción de la NP pediátrica. Constituye la base para tomar decisiones en el contexto de la evidencia existente. Ninguna guía puede tener en cuenta todas las circunstancias clínicas individuales

Guía de práctica clínica SENPE/SEGHNP/SEFH sobre nutrición parenteral pediátrica / Pediatric parenteral nutrition: clinical practice guidelines from the Spanish Society of Parenteral and Enteral Nutrition (SENPE), the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH)

Introducción: la nutrición parenteral (NP) en la infancia es un tratamiento cuyas características son muy variables en función de la edad y la patología que presente el paciente. Material y métodos: el grupo de Estandarización y Protocolos de la Sociedad Española de Nutrición Parenteral y Enteral (SENPE) es un grupo interdisciplinar formado por miembros de la SENPE, Sociedad Española de Gastroenterología, Hepatología y Nutrición Pediátrica (SEGHNP) y Sociedad Española de Farmacia Hospitalaria (SEFH) que pretende poner al día este tema. Para ello, se ha realizado una revisión pormenorizada de la literatura buscando las evidencias que nos permiten elaborar una Guía de Práctica Clínica siguiendo los criterios del Oxford Centre for Evidence-Based Medicine. Resultados: este manuscrito expone de forma resumida las recomendaciones en cuanto a indicaciones, vías de acceso, requerimientos, modificaciones en situaciones especiales, componentes de las mezclas, prescripción y estandarización, preparación, administración, monitorización, complicaciones y NP domiciliaria. El documento completo se publica como número monográfico. Conclusiones: esta guía pretende servir de apoyo para la prescripción de la NP pediátrica. Constituye la base para tomar decisiones en el contexto de la evidencia existente. Ninguna guía puede tener en cuenta todas las circunstancias clínicas individuales (AU)

Introduction: Parenteral nutrition (PN) in childhood is a treatment whose characteristics are highly variable depending on the age and pathology of the patient. Material and methods: The Standardization and Protocols Group of the Spanish Society for Parenteral and Enteral Nutrition (SENPE) is an interdisciplinary group formed by members of the SENPE, the Spanish Society of Gastroenterology, Hepatology and Pediatric Nutrition (SEGHNP) and the Spanish Society of Hospital Pharmacy (SEFH) that intends to update this issue. For this, a detailed review of the literature has been carried out, looking for the evidences that allow us to elaborate a Clinical Practice Guide following the criteria of the Oxford Center for Evidence-Based Medicine. Results: This manuscript summarizes the recommendations regarding indications, access routes, requirements, modifications in special situations, components of the mixtures, prescription and standardization, preparation, administration, monitoring, complications and home NP. The complete document is published as a monographic number. Conclusions: This guide is intended to support the prescription of pediatric PN. It provides the basis for rational decisions in the context of the existing evidence. No guidelines can take into account all of the often compelling individual clinical circumstances (AU)

Indicaciones de la nutrición parenteral / Indications for parenteral nutrition

No disponible

Long-term visual outcome of methylmalonic aciduria and homocystinuria, cobalamin C type.

OBJECTIVE: To describe the long-term ophthalmologic outcomes of patients with methylmalonic aciduria and homocystinuria, cobalamin C type (cblC). DESIGN: Retrospective case series. PARTICIPANTS: All patients with cblC referred to the Department of Ophthalmology of the Centre Hospitalier Universitaire Sainte-Justine from 1984 through 2012 were studied. Twelve such patients were identified. METHODS: Clinical ophthalmic examinations, neuroimaging, electroretinography, and the results of MMACHC mutation analysis were reviewed retrospectively. MAIN OUTCOME MEASURES: We examined visual acuity, ocular alignment, presence of maculopathy and peripheral retinopathy, optic atrophy, and nystagmus. Photopic and scotopic electroretinograms were reviewed. We examined and compared mutations in the MMACHC gene. Neuroimaging abnormalities were compiled when available. RESULTS: Twelve cblC patients were followed up from 2 to 23 years (average, 10 years). Eleven of 12 patients were diagnosed before the age of 1 year (range, birth-2 years). An initial ophthalmic examination was performed within the first year of age in 9 of 12 patients. Visual acuity at the time of presentation was variable, ranging from light perception to 20/20. Visual acuity was worse than 20/100 in 75% (9/12) of patients at last follow-up. Eight patients (67%) had obvious maculopathy on fundus examination. Other findings included peripheral retinopathy (8/12 [67%]), nystagmus (8/12 [67%]), strabismus (5/12 [42%]), and optic atrophy (6/12 [50%]). Funduscopic deterioration was documented in 1 patient, whereas electrophysiologic changes occurred in 4 patients. Neuroimaging results were available in 7 of the patients, revealing corpus callosum atrophy (7/7 [100%]) and periventricular white matter loss (6/7 [85%]). CONCLUSIONS: Most children in our series had early-onset disease with neurologic manifestations and abnormal ophthalmologic examination results. Despite early treatment, many early-onset cblC patients have poor visual function.

Satisfaction with gastrostomy feeding in caregivers of children with homenenteral nutrition; application of the SAGA-8 questionnaire and analysis of involved factors / Satisfacción con la alimentación mediante gastrostomía en cuidadores de niños con nutrición enteral domiciliaria; aplicación del cuestionario SAGA-8 y análisis de los factores implicados

Aims: To assess the degree of satisfaction of caregivers of children with gastrostomy tube (GT) feeding through the structured questionnaire SAGA-8. Secondly, to evaluate if the parental satisfaction degree was related to several independent variables. Methods: A cross-sectional observational study was performed in 92 caregivers of children with GT feeding and chronic diseases. The following data was obtained: caregiver satisfaction with GT feeding (SAGA-8), age at GT placement, anthropometric data, length and mode of nutritional support, family demographic characteristics and caregiver psychological status. Results: All primary caregivers were mothers. High satisfaction with GT feeding was expressed by 82.6% of families. The simplicity of the system was emphasized by 87%, and 85.9% were very satisfied with the support received from the hospital staff. 73.9% of mothers acknowledged their child's nutritional status had improved and 89.1% rated the enhancement family's overall situation. Moreover, 75% of mothers reduced feeding-time, and 68.5% reported less respiratory infections. Finally, 71.7% of mothers recognized that they would have implemented this technique earlier. Caregiver satisfaction was positively correlated with age at GT placement and length of treatment, and both variables explained the 19.4% of the satisfaction variance. No correlation with anthropometric data, nutrition support mode, family demographic characteristics or caregiver psychological status was observed. Conclusions: The SAGA-8 questionnaire is a simple, specific, straight-forward tool to evaluate parental/caregiver degree of satisfaction with GT feeding and facilitates effective monitoring of the intervention. Lengths of HEN and precocious age at GT placement are responsible for most of parental satisfaction (AU)

Objetivos: Valorar el grado de satisfacción de los cuidadores de niños con alimentación mediante gastrostomía aplicando el cuestionario estructurado SAGA-8. Segundo, evaluar si la satisfacción parental guarda relación con diversas variables independientes. Métodos: Se realizó un estudio observacional, transversal en 92 cuidadores de niños con enfermedades crónicas que recibían alimentación mediante gastrostomía. Se analizaron los siguientes datos: satisfacción de los cuidadores con la alimentación por gastrostomía (SAGA-8), edad de implantación de la gastrostomía, parámetros antropométricos, tiempo de soporte nutricional, características demográficas familiares y estado psicológico del cuidador. Resultados: Todos los cuidadores eran madres. La mayoría (82,6%) manifestaron una elevada satisfacción con la alimentación mediante gastrostomía. El 85,9% resaltó la sencillez de la técnica sintiéndose el 85,9% muy satisfecha con el apoyo ofrecido por el centro. Un 73,9% de las madres percibió mejoría nutricional en su hijo, señalando el 89,1% un mayor bienestar familiar. Además, el 75% de las madres comunicaron una disminución en el tiempo de alimentación y el 68,5% una reducción en la morbilidad respiratoria. Finalmente, el 71,7% de las madres reconoció que hubieran aceptado antes el procedimiento. La satisfacción se correlacionó significativamente con la edad de implantación de la gastrostomía y con la duración del soporte nutricional, explicando ambos factores el 19,4% de la varianza de la satisfacción. No se encontró correlación con datos antropométricos, tipo de soporte nutricional, características demográficas familiares ni con el estado psicológico del cuidador. Conclusiones: El cuestionario SAGA-8 es sencillo, específico y fácil de aplicar para evaluar el grado de satisfacción de los padres/cuidadores con la alimentación mediante gastrostomía contribuyendo a su monitorización. La duración del soporte nutricional y la precocidad en la implantación de la gastrostomía son los responsables principales de la satisfacción de los cuidadores (AU)

Satisfaction with gastrostomy feeding in caregivers of children with home enteral nutrition; application of the SAGA-8 questionnaire and analysis of involved factors.

AIMS: To assess the degree of satisfaction of caregivers of children with gastrostomy tube (GT) feeding through the structured questionnaire SAGA-8. Secondly, to evaluate if the parental satisfaction degree was related to several independent variables. METHODS: A cross-sectional observational study was performed in 92 caregivers of children with GT feeding and chronic diseases. The following data was obtained: caregiver satisfaction with GT feeding (SAGA-8), age at GT placement, anthropometric data, length and mode of nutritional support, family demographic characteristics and caregiver psychological status. RESULTS: All primary caregivers were mothers. High satisfaction with GT feeding was expressed by 82.6% of families. The simplicity of the system was emphasized by 87%, and 85.9% were very satisfied with the support received from the hospital staff. 73.9% of mothers acknowledged their child's nutritional status had improved and 89.1% rated the enhancement family's overall situation. Moreover, 75% of mothers reduced feeding-time, and 68.5% reported less respiratory infections. Finally, 71.7% of mothers recognized that they would have implemented this technique earlier. Caregiver satisfaction was positively correlated with age at GT placement and length of treatment, and both variables explained the 19.4% of the satisfaction variance. No correlation with anthropometric data, nutrition support mode, family demographic characteristics or caregiver psychological status was observed. CONCLUSIONS: The SAGA-8 questionnaire is a simple, specific, straight-forward tool to evaluate parental/caregiver degree of satisfaction with GT feeding and facilitates effective monitoring of the intervention. Lengths of HEN and precocious age at GT placement are responsible for most of parental satisfaction.

Objetivos: Valorar el grado de satisfacción de los cuidadores de niños con alimentación mediante gastrostomía aplicando el cuestionario estructurado SAGA-8. Segundo, evaluar si la satisfacción parental guarda relación con diversas variables independientes. Métodos: Se realizó un estudio observacional, transversal en 92 cuidadores de niños con enfermedades crónicas que recibían alimentación mediante gastrostomía. Se analizaron los siguientes datos: satisfacción de los cuidadores con la alimentación por gastrostomía (SAGA-8), edad de implantación de la gastrostomía, parámetros antropométricos, tiempo de soporte nutricional, características demográficas familiares y estado psicológico del cuidador. Resultados: Todos los cuidadores eran madres. La mayoría (82,6%) manifestaron una elevada satisfacción con la alimentación mediante gastrostomía. El 85,9% resaltó la sencillez de la técnica sintiéndose el 85,9% muy satisfecha con el apoyo ofrecido por el centro. Un 73,9% de las madres percibió mejoría nutricional en su hijo, señalando el 89,1% un mayor bienestar familiar. Además, el 75% de las madres comunicaron una disminución en el tiempo de alimentación y el 68,5% una reducción en la morbilidad respiratoria. Finalmente, el 71,7% de las madres reconoció que hubieran aceptado antes el procedimiento. La satisfacción se correlacionó significativamente con la edad de implantación de la gastrostomía y con la duración del soporte nutricional, explicando ambos factores el 19,4% de la varianza de la satisfacción. No se encontró correlación con datos antropométricos, tipo de soporte nutricional, características demográficas familiares ni con el estado psicológico del cuidador. Conclusiones: El cuestionario SAGA-8 es sencillo, específico y fácil de aplicar para evaluar el grado de satisfacción de los padres/cuidadores con la alimentación mediante gastrostomía contribuyendo a su monitorización. La duración del soporte nutricional y la precocidad en la implantación de la gastrostomía son los responsables principales de la satisfacción de los cuidadores.

Does parental body mass index status modify the associations among birth weight, early growth and childhood adiposity?

OBJECTIVES: To examine the associations among birth weight, infant growth and childhood adiposity, and to test whether parental weight status modifies these associations. METHODS: The sample was comprised of 423 participants born at term who were an appropriate size for their gestational age from the Quebec Adipose and Lifestyle Investigation in Youth (QUALITY) study, a cohort of 630 children with a parental history of obesity. Infant growth velocity from zero to two years of age was estimated using slopes from simple linear regression for weight and body mass index (BMI) Z-scores. Child anthropometrics and body composition, and parental BMI were measured from eight to 10 years of age. Associations were modelled using multiple linear regressions. RESULTS: Increased birth weight and growth velocity independently predicted increased childhood adiposity. Effects of infant growth velocity on later adiposity were stronger with higher maternal BMI but not with higher paternal BMI. Similar interactions with birth weight were not found. CONCLUSIONS: Early childhood measures of growth and the mother's BMI score should be included in investigations on obesity risk.

OBJECTIFS: Examiner les associations entre le poids à la naissance, la croissance du nourrisson et l'adiposité pendant l'enfance et vérifier si le poids des parents les modifie. MÉTHODOLOGIE: L'échantillon se composait de 423 participants nés à terme dont la taille correspondait à l'âge gestationnel et qui étaient extraits de l'étude QUALITY sur l'adiposité et le mode de vie chez les jeunes du Québec, menée auprès d'une cohorte de 630 enfants ayant des antécédents parentaux d'obésité. Les chercheurs ont estimé la vélocité de croissance des nourrissons de zéro à deux ans au moyen de l'écart réduit de la régression linéaire simple et de l'indice de masse corporelle (IMC). Ils ont mesuré les données anthropométriques et la composition corporelle des enfants de huit à dix ans, ainsi que l'IMC des parents. Ils ont établi les modèles d'association au moyen de régressions linéaires multiples. RÉSULTATS: Un poids de naissance et une vélocité de croissance élevés étaient des prédicteurs indépendants d'une adiposité plus importante pendant l'enfance. Les effets de la vélocité de croissance des nourrissons sur leur adiposité plus tard étaient plus marqués lorsque l'IMC de la mère, mais pas celui du père, était élevé. Les chercheurs n'ont constaté aucune interaction similaire par rapport au poids à la naissance. CONCLUSIONS: Il faudrait inclure les mesures de croissance de la petite enfance et l'IMC de la mère dans les explorations du risque d'obésité.

Feeling of burden, psychological distress, and anxiety among primary caregivers of children with home enteral nutrition.

OBJECTIVE: To examine the relationship between several psychological factors and the feeling of burden experienced by caregivers of children with home enteral nutrition. METHODS: Fifty-six mothers of pediatric patients with chronic diseases requiring long-term home enteral nutrition were recruited. They were asked to respond to specific questionnaires about their anxiety symptoms (State-Trait Anxiety Inventory), psychological distress (SCL-90-R) and feeling of burden (Zarit-scale). RESULTS: Caregivers' feeling of burden was found to be statistically associated to psychological distress (r = .516, p < .001) and trait anxiety (r = .376, p = .005). No significant differences were found between the type of diagnosis and caregiver burden. Regression analysis indicated psychological distress has a partial mediational effect in the relationship between trait anxiety and caregivers' burden. CONCLUSIONS: Psychological distress and anxiety show a positive correlation with caregivers' feeling of burden, and may disrupt family well-being. Early identification of high-risk situations is essential in order to plan specific psychosocial aid efficiently.

Quality of dietary control in phenylketonuric patients and its relationship with general intelligence.

OBJECTIVES: Assessment of the quality of dietary treatment of phenylketonuria (PKU) patients and investigation of its relationship with the general intelligence of the patients. METHODS: Cross-sectional and longitudinal study of 105 PKU treated patients. The index of dietary control (IDC) was calculated as the phenylalanine (Phe) data reduction in half-year medians and the mean of all medians throughout the patient's life. We calculated four different IDCs related to age: IDC-A (< 6 years), IDC-B (6-12 years), IDC-C (13-18 years) and IDC-D (> 18 years). To evaluate the fluctuation of Phe values we calculated the standard error of the estimate of the regression of Phe concentration over age. Development quotient was calculated with the Brunet-Lezine test (< 4 years). Intelligence quotient was evaluated with the Kaufman Bit Intelligence Test (K-Bit), Wechsler Intelligence Scale for Children-Revised (WISC-R) and Wechsler Adult Intelligence Scale Third Edition (WAIS III). RESULTS: Cross-sectional study: The IDC in age groups were significantly different and so were the number of patients with good, acceptable and poor IDC related to age (p < 0.001). Sampling frequency was good in 72, acceptable in 23 and poor in 10 patients. The general intelligence (101 +/- 10) correlated negatively with the IDC (p < 0.0001) and Phe fluctuations (p < 0.004). Longitudinal study: Significant differences were observed between the IDC through the patients' lifetime except in the adolescent/adult period. CONCLUSIONS: 85% of PKU patients showed good/acceptable quality of dietary control. General intelligence correlates with the IDC at all ages, which highlights the importance of good control to achieve good prognosis.

The monitoring of trace elements in blood samples from patients with inborn errors of metabolism.

Patients having inborn errors of intermediary metabolism (IEMs) may have element deficiencies related to dietary treatment. Our objective was to study several elements [cobalt (Co), copper (Cu), zinc (Zn), selenium (Se), manganese (Mn), molybdenum (Mo) and magnesium (Mg)] in patients with IEMs with and without dietary treatment and to compare these results with those established in a healthy paediatric population. We studied 72 patients with IEMs (age range 2 months-44 years; median 10.5 years), with and without protein-restricted dietary treatment. Control values were established in 92 subjects (age range 1 day-42 years; median 6.5 years). Dietary treatment consisted of a natural protein-restricted diet supplemented with a special formula, depending on the specific metabolic defect. Samples were analysed with an Agilent 7500ce-ICP mass spectrometer. Significant differences were observed when we compared patients under dietary treatment and control values for Se and Co (P < 0.0001). No differences were observed for the other elements when the different groups were compared, except for Co (IEM patients without dietary treatment vs control group; P = 0.003). For Se and cobalamin, the daily intake of our patients (Se 48 ± 16 µg/day; cobalamin 3.5 µg/day) was slightly higher than the recommended daily averages (RDAs) (40 µg/day and 1.8 µg/day, respectively). We concluded that IEM patients under dietary treatment showed significantly lower selenium values in spite of correct supplementation, reinforcing the idea that these patients should be regularly monitored, at least for this element. Further investigations seem advisable about Se and Co availability in special diets.

Long-chain polyunsaturated fatty acid status in phenylketonuric patients treated with tetrahydrobiopterin.

OBJECTIVES: To evaluate LCPUFA composition in PKU patients treated with BH(4). DESIGN AND METHODS: Cross-sectional study of plasma and erythrocyte LCPUFA composition of 13 PKU patients treated with BH(4) compared with data from 48 PKU patients on protein-restricted diet, and 17 mild HPA patients on free diet. PUFA were analysed by gas chromatography. RESULTS: Plasma and erythrocyte docosahexaenoic acid (DHA), and LCPUFA deficiency markers did not show significant differences in PKU patients on BH(4) compared with those with mild HPA and our reference values, but they did in comparison with PKU on protein-restricted diet (p<0.0001). Essential fatty acids and arachidonic acid composition were not significantly different in any of the studied groups. DHA values correlate with the index of dietary control only in PKU patients on protein-restricted diet (p=0.002). CONCLUSION: LCPUFA status is within the reference values in PKU patients treated with BH(4). This translates to a further advantage of BH(4) therapy.

Cerebellar hemorrhage in a patient with propionic acidemia.

Cerebellar hemorrhage (CH) is a well-known complication in newborns. Among metabolic patients, it has been classically described but rarely reported. This is the first description of a patient with propionic acidemia in whom magnetic resonance imaging (MRI) allowed diagnosis of asymptomatic CH. Due to the usual silent presentation of CH at early ages, we suggest the possibility of including a brain MRI study as part of the routine neurological evaluation in metabolic patients, especially when neurological signs appear.

Guía para la administración y los cuidados de la nutrición enteral a través de sonda nasogástrica

Guía que tiene como objetivo facilitar el proceso de la alimentación por sonda nasogástrica, así como permitir el conocimiento y cuidado de todos los dispositivos relacionados con ella.

Guía para la administración y los cuidados de la nutrición enteral a través de sonda o botón de gastrostomía

Guía que tiene como objetivo facilitar el proceso de la alimentación a través de una sonda o de un botón de gastrostomía, así como permitir el conocimiento y cuidado de todos los dispositivos relacionados con ellos.

Guía para la administración y los cuidados de la nutrición enteral a través de sonda nasogástrica

Guía que tiene como objetivo facilitar el proceso de la alimentación por sonda nasogástrica, así como permitir el conocimiento y cuidado de todos los dispositivos relacionados con ella.

Guía para la administración y los cuidados de la nutrición enteral a través de sonda o botón de gastrostomía

Guía que tiene como objetivo facilitar el proceso de la alimentación a través de una sonda o de un botón de gastrostomía, así como permitir el conocimiento y cuidado de todos los dispositivos relacionados con ellos.