RESUMEN
Hereditary Fructose Intolerance (HFI) is an autosomal recessive inborn error of metabolism characterised by the deficiency of the hepatic enzyme aldolase B. Its treatment consists in adopting a fructose-, sucrose-, and sorbitol (FSS)-restrictive diet for life. Untreated HFI patients present an abnormal transferrin (Tf) glycosylation pattern due to the inhibition of mannose-6-phosphate isomerase by fructose-1-phosphate. Hence, elevated serum carbohydrate-deficient Tf (CDT) may allow the prompt detection of HFI. The CDT values improve when an FSS-restrictive diet is followed; however, previous data on CDT and fructose intake correlation are inconsistent. Therefore, we examined the complete serum sialoTf profile and correlated it with FSS dietary intake and with hepatic parameters in a cohort of paediatric and adult fructosemic patients. To do so, the profiles of serum sialoTf from genetically diagnosed HFI patients on an FSS-restricted diet (n = 37) and their age-, sex- and body mass index-paired controls (n = 32) were analysed by capillary zone electrophoresis. We found that in HFI patients, asialoTf correlated with dietary intake of sucrose (R = 0.575, p < 0.001) and FSS (R = 0.475, p = 0.008), and that pentasialoTf+hexasialoTf negatively correlated with dietary intake of fructose (R = -0.386, p = 0.024) and FSS (R = -0.400, p = 0.019). In addition, the tetrasialoTf/disialoTf ratio truthfully differentiated treated HFI patients from healthy controls, with an area under the ROC curve (AUROC) of 0.97, 92% sensitivity, 94% specificity and 93% accuracy.
RESUMEN
INTRODUCTION: Introduction: promoting healthy eating habits among childhood is one of the key aspects to improve medium and long-term health outcomes. Objectives: the main aims are to improve eating habits, promote the Mediterranean diet (MD) and prevent and/or reverse overweight and obesity in children from 3 to 12 years old. Methods: the program has a one-year follow-up and includes three to five visits with registered dietitians, one telephone control and one practical workshop. Anthropometric, body composition and eating habits data are collected, and nutritional education is carried out. A total sample of 1,000 children will be included. Results: until now, 622 participants have been included (51.6 % boys; median age 8.5 years). At the beginning, 32.2 % of participants were overweight or obese and 38.9 % had an adequate MD. Although no differences were found in the assessment of the Kidmed questionnaire regarding sex (p = 0.214) or body mass index (BMI) subgroups (p = 0.181), differences were found regarding age (p = 0.023) and BMI Z-score (p = 0.004), showing slightly lower values in those having and adequate MD. At the moment, 362 participants have made the six-month visit, of which 61.6 % presented an adequate MD, with statistically significant differences compared to the baseline visit (p < 0.0001). Conclusions: preliminary results show the need for nutritional education in children and suggest that Programa Nutriplato® can be effective in improving eating habits.
INTRODUCCIÓN: Introducción: promocionar hábitos de alimentación saludable durante la infancia es uno de los aspectos clave para fomentar un buen estado de salud a medio y largo plazo. Objetivos: los objetivos principales son mejorar los hábitos alimentarios, promocionar la dieta mediterránea (DM) y prevenir y/o revertir el sobrepeso y la obesidad en niños de 3 a 12 años. Métodos: el programa tiene un seguimiento de un año e incluye de tres a cinco visitas con dietistas-nutricionistas, un control telefónico y un taller práctico. Se recogen datos antropométricos, de composición corporal y de hábitos alimentarios, y se realiza educación nutricional. Se incluirán un total de 1.000 niños. Resultados: hasta el momento, se han incluido 622 participantes (51,6 % niños; mediana de edad de 8,5 años). Al inicio, el 32,2 % presentaba sobrepeso u obesidad y el 38,9 % seguía una DM óptima. No se encontraron diferencias en la valoración del cuestionario Kidmed en función del sexo (p = 0,214) ni del subgrupo de índice de masa corporal (IMC) (p = 0,181), pero sí en función de la edad (p = 0,023) y del Z-score del IMC (p = 0,004), mostrando valores ligeramente menores en aquellos que presentaban una DM óptima. Por ahora, 362 participantes han realizado la visita de los seis meses, de los cuales el 61,6 % presentó una DM óptima, con diferencias estadísticamente significativas en comparación con la inicial (p < 0,0001). Conclusiones: los resultados preliminares muestran la necesidad de realizar educación nutricional en los niños y sugieren que el Programa Nutriplato® puede ser efectivo en la mejora de hábitos alimentarios.
