Hyperammonaemia is a metabolic derangement that may cause severe neurological damage and even death due to cerebral oedema, further complicating the prognosis of its triggering disease. In small ...children it is a rare condition usually associated to inborn errors of the metabolism. As age rises, and especially in adults, it may be precipitated by heterogeneous causes such as liver disease, drugs, urinary infections, shock, or dehydration. In older patients, it is often overlooked, or its danger minimized. This protocol was drafted to provide an outline of the clinical measures required to normalise ammonia levels in patients of all ages, aiming to assist clinicians with no previous experience in its treatment. It is an updated protocol developed by a panel of experts after a review of recent publications. We point out the importance of frequent monitoring to assess the response to treatment, the nutritional measures that ensure not only protein restriction but adequate caloric intake and the need to avoid delays in the use of specific pharmacological therapies and, especially, extrarenal clearance measures. In this regard, we propose initiating haemodialysis when ammonia levels are >200−350 µmol/L in children up to 18 months of age and >150−200 µmol/L after that age.
We present the results of our experience in the diagnosis of inborn errors of metabolism (IEM) since the Expanded Newborn Screening was implemented in our Region. Dried blood samples were collected ...48 h after birth. Amino acids and acylcarnitines were quantitated by mass spectrometry (MS)/MS. Newborns with alterations were referred to the clinical centers for follow‐up. Biochemical and molecular genetic studies for confirmation of a disease were performed. In the period 2011 to 2019, 592 822 children were screened: 902 of them were referred for abnormal results. An IEM was confirmed in 222 (1/2670): aminoacidopathies: 89 hyperphenylalaninemia (HPA) (51 benign HPA, 32 phenylketonuria, 4 DNAJC12 defect, and 2 primapterinuria), 6 hypermethioninemia, 3 tyrosinemia type 1 (TYR‐1), 1 TYR‐3, 4 maple syrup urine disease (MSUD), 2 branched‐chain amino acid transferase 2 deficiency, 2 homocystinuria, 1 cystinuria, 2 ornithine transcarbamylase (OTC) deficiency, 2 citrullinemia type I (CTLN1); FAO defects: 43 medium‐chain acyl‐CoA dehydrogenase deficiency (MCADD), 13 very long‐chain acyl‐CoA dehydrogenase deficiency, 2 long‐chain 3‐hydroxyacyl‐CoA dehydrogenase deficiency (LCHADD), 1 multiple acyl‐coA dehydrogenation deficiency, 11 systemic primary carnitine deficiency, 2 carnitine palmitoyltransferase type 2 (CPT‐II) deficiency, 1 CPT‐I deficiency; organic acidurias: 12 glutaric aciduria type 1 (GA‐1), 4 methylmalonic acidemia (MMA), 7 MMA including combined cases with homocystinuria (MMAHC), 6 propionic acidemia (PA), 7 3‐methylcrotonyl‐CoA carboxylase, 1 3‐hydroxy‐3‐methylglutaryl‐CoA lyase deficiency lyase deficiency. Only 19 infants (8.5%) were symptomatic at newborn screening result (1 LCHADD, 5 PA, 1 CPT‐II deficiency, 1 MMA, 3 MMAHC, 2 MSUD, 2 OTC deficiency, 1 CTLN1, 1 MCADD, 2 TYR‐1). No false negative cases were identified. Genetic diagnosis was conclusive in all biochemically confirmed cases, except for two infants with HPA, identifying pathogenic variants in 32 different genes. The conditions with the highest incidence were HPA (1/6661) and MCAD deficiencies (1/13 787).
In the last few years, there are an increased number of families following a vegetarian diet, including their children. In order to guarantee child heath, paediatricians need to know the ...characteristics of this diet and the main foods that are used.
There are few data on the medium and long-term health outcomes when removing all animal foods from the child’s diet, especially at younger ages. Nevertheless, new tools have recently become available to facilitate following a vegetarian diet and decreasing the risk of deficiencies.
In this document, the group of foods commonly used in vegetarian diets are reviewed, as well as recommendations for each age group. It also mentions the need to use B12 supplements at all ages, as well as other nutrients (iodine, iron, vitamin D3, poly-unsaturated fatty acid n-3), when required.
A vegetarian or a vegan diet, as in any other kind of diet, needs to be carefully designed. After reviewing current evidence, even though following a vegetarian diet at any age does not necessarily mean it is unsafe, it is advisable for infant and young children to follow an omnivorous diet or, at least, an ovo-lacto-vegetarian diet.
En los últimos años ha aumentado el número de personas que optan por una dieta vegetariana y que incluyen a sus hijos en esta opción de alimentación. Esto obliga a los pediatras a conocer sus características principales y la composición de los alimentos usados más comúnmente, con el fin de garantizar la salud de los niños.
Existe una escasez de datos sobre el impacto a medio y largo de eliminar los productos de origen animal en la dieta de los niños, en especial en la de los más pequeños. Sin embargo, en los últimos años se han publicado herramientas (tablas de intercambios de alimentos, recomendaciones de suplementación) que facilitan el cumplimiento de una dieta vegetariana disminuyendo el riesgo de deficiencias.
En este documento se revisan los grupos de alimentos que habitualmente forman parte de la dieta vegetariana, así como las recomendaciones para cada grupo de edad. Se señala también la necesidad de utilizar suplementos de vitamina B12 en todas las edades, y en aquellos otros nutrientes (yodo, hierro, vitamina D3, ácidos grasos polinsaturados n-3) en riesgo de deficiencia.
Una alimentación vegetariana o vegana, como cualquier otro tipo de alimentación, debe estar bien planificada. A la luz de la evidencia disponible, a pesar de que seguir una dieta vegetariana en cualquier etapa de la infancia no signifique necesariamente que sea insegura, es preferible aconsejar que durante el periodo de lactante y en el niño de corta edad se siga una dieta omnívora o, al menos, ovo o lactovegetariana.
the recent economic and financial crisis has affected most Western countries, especially families of low socioeconomic classes. We speculate that worsening of socioeconomic condition associated with ...the crisis would increase obesity, mainly in disadvantaged families.
cross-sectional study of the 290,111 children aged three to 12 years old attending public school during the term 2014-2015 in Madrid City, by means of a stratified weighted sample randomly chosen, taking into account age (grade), city district and schools. The questionnaire included weight and height (auto-reported), dietary report (weekly frequency of intake), as well as socioeconomic variables.
1,208 questionnaires were evaluated from 64 classes. Half of participants were boys; 42% were younger than five years old, 35% werebetween six and eight years old, and 23% older than eight. Undernutrition was present in 5.0%, and excess of weight (overweight + obesity) in 36.7%. Undernutrition was higher in children under the age of six (9.1%). No relationship was found between undernutrition and the characteristics of the families but was slightly higher in families where both parents were unemployed. Excess of weight was higher in children of non-Spaniard parents (44% vs 32%, p < 0.0001), as well as in those families with economic problems (41% vs 31%, p = 0.0005). Only for meat, grains and dairy, the weekly intake was close to the recommendations.
children from lower income households were at a higher risk of being overweight compared with their peers. Participation in a school-based food aid program may reduce food insecurity for children and their families.
En los últimos años ha aumentado el número de personas que optan por una dieta vegetariana y que incluyen a sus hijos en esta opción de alimentación. Esto obliga a los pediatras a conocer sus ...características principales y la composición de los alimentos usados más comúnmente, con el fin de garantizar la salud de los niños.
Existe una escasez de datos sobre el impacto a medio y a largo plazo de eliminar los productos de origen animal en la dieta de los niños, en especial en la de los más pequeños. Sin embargo, en los últimos años se han publicado herramientas (tablas de intercambios de alimentos, recomendaciones de suplementación) que facilitan el cumplimiento de una dieta vegetariana disminuyendo el riesgo de deficiencias.
En este documento se revisan los grupos de alimentos que habitualmente forman parte de la dieta vegetariana, así como las recomendaciones para cada grupo de edad. Se señala también la necesidad de utilizar suplementos de vitaminaB12 en todas las edades, y en aquellos otros nutrientes (yodo, hierro, vitaminaD3, ácidos grasos poliinsaturados n-3) en riesgo de deficiencia.
Una alimentación vegetariana o vegana, como cualquier otro tipo de alimentación, debe estar bien planificada. A la luz de la evidencia disponible, a pesar de que seguir una dieta vegetariana en cualquier etapa de la infancia no signifique necesariamente que sea insegura, es preferible aconsejar que durante el periodo de lactante y en el niño de corta edad se siga una dieta omnívora o, al menos, ovo o lactovegetariana.
In the last few years, there are an increased number of families following a vegetarian diet, including their children. In order to guarantee child heath, paediatricians need to know the characteristics of this diet and the main foods that are used.
There are few data on the medium and long-term health outcomes when removing all animal foods from the child's diet, especially at younger ages. Nevertheless, new tools have recently become available to facilitate following a vegetarian diet and decreasing the risk of deficiencies.
In this document, the group of foods commonly used in vegetarian diets are reviewed, as well as recommendations for each age group. It also mentions the need to use B12 supplements at all ages, as well as other nutrients (iodine, iron, vitaminD3, poly-unsaturated fatty acid n-3), when required.
A vegetarian or a vegan diet, as in any other kind of diet, needs to be carefully designed. After reviewing current evidence, even though following a vegetarian diet at any age does not necessarily mean it is unsafe, it is advisable for infant and young children to follow an omnivorous diet or, at least, an ovo-lacto-vegetarian diet.
Recomendaciones nutricionales para el niño deportista Sánchez-Valverde Visus, F.; Moráis López, A.; Ibáñez, J. ...
Anales de pediatría (Barcelona, Spain : 2003),
August 2014, Letnik:
81, Številka:
2
Journal Article
Recenzirano
Odprti dostop
El deporte tiene numerosos beneficios para la salud y una adecuada nutrición ayuda a conseguir un óptimo rendimiento. La mayor parte de la actividad deportiva realizada por la población infantil ...tiene lugar en el ámbito escolar, no incluye actividades de resistencia y con frecuencia no tiene carácter de alta competición. Las características de la dieta del niño deportista son similares a la de la población pediátrica general, debiendo asegurar una óptima cobertura de los requerimientos. Durante la realización de la actividad, debe asegurarse una correcta hidratación y para ello el agua resulta la bebida más adecuada en la mayor parte de las situaciones, quedando el uso de otros productos reservado en pediatría a situaciones especiales muy concretas. La utilización sistemática de suplementos energéticos, así como la de micronutrientes, no está justificada con carácter general. El pediatra debe conocer y monitorizar el estado nutricional y los hábitos dietéticos del niño deportista, vigilar estrechamente aquellas situaciones donde se pretenda una disminución del peso corporal y valorar los aspectos psicológicos relacionados con la práctica deportiva competitiva.
Several health benefits have been attributed to sports practice, and an adequate nutrition status helps to maintain an optimal performance. Children most frequently practice non-competitive and non-endurance activities in a school setting. The dietary intake of children who practice sports should be similar to the general population, properly meeting their energy and nutrient requirements. During the activity performance, correct hydration should be aimed for, with water appearing to be an adequate source in most cases. General calorie and micronutrient supplementation should not be commonly recommended in children. Paediatricians must control nutritional status and dietary habits of children who practice sports, especially in those cases when weight-loss is aimed for, as well as take into account the psychological implications of competitive sports practice.
Standardization of clinical procedures has become a desirable objective in contemporary medical practice. To this effect, the Spanish Society of Parenteral and Enteral Nutrition (SENPE) has ...endeavoured to create clinical practice guidelines and/or documents of consensus as well as quality standards in artificial nutrition. As a result, the SENPE´s Standardization Team has put together the "Document of Consensus in Enteral Access for Paediatric Nutritional Support" supported by the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SEGHNP), the National Association of Pediatric and Neonatal Intensive Care Nursery (ANECIPN), and the Spanish Society of Pediatric Surgery (SECP). The present publication is a reduced version of our work; the complete document will be published as a monographic issue. It analyzes enteral access options in the pediatric patient, reviews the levels of evidence and provides the team-members' experience. Similarly, it details general and specific indications for pediatric enteral support, current techniques, care guidelines, methods of administration and complications of each enteral access. The data published by the American Society for Parenteral and Enteral Nutrition (ASPEN) and several European Societies has also been incorporated.
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