Trastornos adquiridos y congénitos de la deficiencia de vitamina B12 en niños / Acquired and inherited disorders of vitamin B12 deficiency in children

Vitamin B12 is an essential micronutrient for cell growth and the development of the central nervous system. Its deficiency can manifest clinically as megaloblastic anemia, peripheral neuropathy, myelopathy and neuropsychiatric disorders. Early detection and treatment are essential as it can cause irreversible neurological sequelae. Diagnosis is often challenging as it is based on clinical and biochemical features. Clinically, the symptoms are nonspecific and equivocal. Biochemically, there is no gold standard to detect Cobalamin deficiency. The available biomarkers do not have a defined cut-off value or are not sensitive or specific enough. This article exposes the different causes of vitamin B12 deficiency, analyzes the advantages and disadvantages of biochemical markers and, for the first time, proposes an algorithmic diagnosis using biomarkers and therapeutic tests. The ultimate goal is to alert pediatricians to the difficulties of diagnosing vitamin B12 deficiency and strategies are proposed to differentiate between acquired and congenital cobalamin conditions. Finally, the treatment according to the etiology is described in a practical manner, as well as the expected time for improvement of the biochemical parameters.

La vitamina B12 es un micronutriente fundamental para el crecimiento celular y el desarrollo del sistema nervioso central. Su deficiencia puede manifestarse clínicamente como anemia megaloblástica, neuropatía periférica, mielopatía y trastornos neuropsiquiátricos. La detección y el tratamiento tempranos son esenciales, ya que esta deficiencia puede generar secuelas neurológicas irreversibles. El diagnóstico suele ser un desafío, ya que se basa en pilares clínicos y bioquímicos. Clínicamente, los síntomas son inespecíficos y equívocos. Bioquímicamente no existe un gold standard para diagnosticar la deficiencia de cobalamina. Los biomarcadores existentes no presentan un valor de corte definido o no son lo suficientemente sensibles o específicos. Este trabajo expone las diferentes causas de deficiencia de vitamina B12, analiza las ventajas y desventajas de los marcadores bioquímicos y por primera vez se plantea un algoritmo diagnóstico mediante biomarcadores y pruebas terapéuticas. El objetivo último es alertar a los pediatras acerca las dificultades que representa el diagnóstico de deficiencia de vitamina B12 y se proponen estrategias para diferenciar cuadros adquiridos versus congénitos de la deficiencia de cobalamina. Por último, se describe de manera práctica el tratamiento según la etiología así como el tiempo esperado para la mejoría de los parámetros bioquímicos.

STRONGkids for congenital heart disease perioperative nutritional risk screening / 中华临床营养杂志

Objective To identify the nutritional risks of the hospitalized children with congenital heart disease (CHD) during perioperative period and the relationship between nutrition risks and the clinical outcomes.Methods Totally 780 CHD children (522 cases of ventricular septal defect,133 cases of atrial septal defect,70 cases of tetralogy of fallot,and 55 of patent ductus arteriosus) were recruited.The STRONGkids (Screening Tool for Risk on Nutritional Status and Growth) was used to analyze the nutritional risks of CHD children and to identify the nutritional risks of these children at different ages.Also,the potential effect of pulmonary hypertension on malnutrition and effects of the possible nutritional risks on the clinical outcomes were analyzed.Results Of these 780 CHD children,260 (33.3％) were found to be with high and moderate nutritional risks.The incidences of these risks were 49.2％ (187/380) in the 0-1-year-old group,which was significantly higher than that in the 1-3-year-old group (26.2％,60/229) (x2 =25.174,P ＜0.001) and the ＞ 3-year-old group (7.6％,13/171) (x2 =36.029,P ＜ 0.001).Both pulmonary hypertension and malnutrition showed synergic effects on the clinical nutritional risk,and higher nutrition risks were associated with poorer clinical outcomes.Conclusions STRONGkids can effective evaluate the nutritional risks of CHD children during the perioperative period and thus can inform the standardized nutrition support.

Vitamin A status of children and lactating mother in the communes with protein energy malnutrion (PEM) < 30% and < 15%

Children aged 6-36 months old and breastfeeding mother in 2 groups of communes with a malnutrition prevalence <15% (group 1) and <30% 9group 2). Measuring the height, the weight, using HPLC to analyse, to quantify serum vitamin A level of children and breast milk. Results showed that serum vitamin A was similar in 2 groups, while vitamin A level of breast milk in grup 1 is higher considerably than in group 2. In the group 1, diet regimen of the group 1 is better, concerning the quantity as the quality. However, there is a supply of only 50% of recommended vitamin A, so the programme of high doses of vitamin A in gel capsula can be continued and vitamin A supplement in food is need